|Year : 2022 | Volume
| Issue : 2 | Page : 99-139
34th annual conference of indian society of pediatric nephrology selected abstracts
|Date of Web Publication||31-Dec-2022|
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
. 34th annual conference of indian society of pediatric nephrology selected abstracts. Asian J Pediatr Nephrol 2022;5:99-139
| O1 Incidence of Acute Kidney Injury in Noncritically Ill Hospitalized Children and Role of Cystatin C in Detection of Acute Kidney Injury at Tertiary Care Center in North India|| |
Gunjan Gupta, Shobha Sharma, Kanika Kapoor, Anita Rani, Rani Gera
Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
Rationale: Acute kidney injury (AKI) refers to an acute decline in function and inability to regulate acid, electrolyte, and fluid balance. AKI can be classified into community acquired-AKI (CA-AKI), when child either comes with or develops AKI within 48 hours of admission and hospital acquired-AKI (HA-AKI) if AKI develops 48 hours after admission. Studies show a wide variability in the incidence of AKI, ranging from 0.8% in noncritically ill population, to up to 80% in critically ill children depending on the criteria used to define AKI, settings, and the population used for study. Most of the studies have been in critically ill population, taking into account chiefly the cases of HA-AKI. There is the dearth of studies of CA-AKI, especially in hospitalized noncritically ill children.
Methods: This prospective cohort study aimed to determine the incidence, risk factors, and acute outcome of AKI in noncritically ill children (community and HA), and to observe the trend of cystatin C in patients with and without AKI.
Results: This study in 505 children (1 month to 12 years) showed AKI in 15.6% children, out of which 83.5% patients had CA-AKI while 16.5% had HA-AKI [Table 1]. Out of all patients with AKI, 54.43% were exposed to nephrotoxic drugs and 53.49% (23) had received 2 or more nephrotoxic drugs. Sepsis was noted in 34.2% patients; 35.44% patients had dehydration. Patients with HA-AKI [Table 1] had significantly longer duration of stay (15.23 ± 5.42 days) as compared to CA-AKI patients (7.48 ± 6.42 days) and also had significant exposure to nephrotoxic drugs. Cystatin C had specificity of 88.50% and a negative predictive value of 93.80%.
|Table 1: Baseline characteristics and outcomes in community-acquired and hospital-acquired acute kidney injury|
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Conclusions: The incidence based on only HA-AKI [Table 1] leads to under reporting of CA-AKI. Using age-appropriate eGFR to back calculate baseline serum creatinine can be helpful in the early diagnosis. Cystatin-C has good specificity and negative predictive value for diagnosing AKI.
|Figure 1: Receiver operating characteristic curve for cystatin C in predicting acute kidney injury (AUC = 0.78). AUC: Area under the curve|
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| O2 The Incidence of Acute Kidney Injury in the Pediatric Age Group Admitted in Hospital Setting With COVID-19 Infection|| |
Neha Pandey, Alpana Ohri, Amish Udani
Bai Jerbai Wadia Hospital for Children, Mumbai, Maharashtra, India
Rationale: COVID-19 infection is known for multiorgan involvement of which kidney injury predominates. Acute kidney injury (AKI) is associated with raised mortality in the intensive care setting worldwide. The involvement ranges from transient proteinuria to severe AKI requiring renal replacement therapy (RRT). The adult counterparts have reported higher incidence of AKI with mortality but pediatric literature are limited.
Objectives: Our aim was to detect the affection of kidneys in the pediatric age group who were admitted in the hospital setting with COVID-19 infection (acute or remote). Due to the scarcity of data of renal involvement in pediatric COVID-19 population, we aimed to study its incidence in the pediatric population.
Methods: This unicentric observational study included 44 pediatric patients with SARS CoV2 positive reverse transcription polymerase chain reaction (RT-PCR), serology, or antigen test admitted to the hospital. Patients were grouped based on the presence of underlying renal ailment. The study included six chronic kidney disease (CKD) patients infected with COVID 19 to study their outcome.
Results: Of the 44 enrolled subjects, 31 (70.5%) developed AKI. The majority of these had severe or stage III AKI as per KDIGO criteria. Among patients with AKI, 41.9% required acute RRT, of which six patients were successfully discharged. In the AKI subgroup, 64.5% patients were discharged with complete renal recovery and 35.4% had died. Among the seven patients who did not develop AKI, all were discharged successfully. This study also had 6 CKD patients, of which 50% were positive by RT-PCR, two patients had shock, and one died during the study period.
Conclusions: AKI occurred in the significant proportion of study population with SARS-Co-V2 infection. AKI subgroup reports higher mortality rate which is proportional to the stage of AKI. CKD patients may be at a higher risk to develop COVID-19 infection due to their relative state of immunodeficiency.
| O3 The Clinicopathological Spectrum and Response to Mycophenolate Sodium in the Treatment of C3 Glomerulonephritis|| |
Apurva Shah, Kinnari Vala, Anshuman Saha, Shahenaz Kapadia, Himanshu Patel
Department of Pediatric Nephrology, Institute of Kidney Disease and Research Centre, GUTS, Ahmedabad, Gujarat, India
Rationale: C3 glomerulopathy (C3G) is a rare clinical entity caused by alternate complement pathway dysregulation. Limited published literature is available regarding the clinical picture and outcome in Indian children.
Objectives: To retrospectively analyze the clinicopathological spectrum and response to mycophenolate sodium (MPA) in the treatment of C3G.
Subjects and Methods: Records of all patients 18 years diagnosed with C3G between January 2017 and December 2020 were retrieved after the ethics committee clearance. C3 glomerulonephritis (C3 GN) and dense deposit disease (DDD) were diagnosed based on the electron microscopy. The clinical features at presentation and outcomes in terms of response to MPA and death/kidney failure were evaluated. Improvement in estimated glomerular filtration rate (eGFR) by 50% and spot urine protein creatinine ratio (UPCR) <0.5 g/g defined complete remission (CR). Remission was partial (PR) if eGFR improved by 50%-25% and spot UPCR was 0.5–3.5g/g. No response was defined as improvement in eGFR by ≤25% and spot UPCR >3.5g/g.
Results: Twenty-two children (9 boys) were diagnosed with C3G: C3 GN n = 15 (68%) and DDD) n = 7 (32%). Age at presentation (median, range) was 7.66, 10 years and 9.28, 7 years in C3GN and DDD, respectively (P = 0.24). Acute nephritic syndrome was the most common presentation in C3GN (8/15, 53%). Whereas rapidly progressive renal failure was more common in DDD (4/7, 57%). Nephrotic range proteinuria was more common in DDD (85%). All children received prednisolone. Eighteen children received MPA, 3 cyclophosphamide and 1 received cyclosporine. On MPA Ten (55%) had CR and 4 (22.3%) had PR. Four (22.2%) had no response. Out of 4 with no response 3 progressed to kidney failure, 1 expired. Response to treatment was poorer (NR 3, 75%) in children who did not receive MPA (P < 0.05).
Conclusions: Children with DDD had more severe presentation and poorer outcome. MMF was useful in treating C3G patients with a higher probability of remission and lower probability of kidney failure.
| O4 The Clinical Profile and Outcome of Acute Kidney Injury in Neonates Presenting with Hypernatremic Dehydration at a Tertiary Care Center|| |
Vasundhara Bakshi, Koushal Khajuria, G. S. Saini
Department of Paediatrics, SMGS Hospital, Government Medical College, Jammu, Jammu and Kashmir, India
Rationale: Very few studies, that too mainly case reports are available that mention about acute kidney injury (AKI) due to hypernatremic dehydration in neonates and there is the scarcity of data from our region on the same. Hence, this study was undertaken on neonates admitted with hypernatremia with AKI to estimate the incidence and outcome of the problem in our region.
Objectives: To study the clinical profile and outcome of AKI in neonates presenting with hypernatremic dehydration at a tertiary care center.
Methods: A prospective hospital-based observational study of 106 neonates presenting with hypernatremic dehydration with AKI was done in the department of pediatrics at a tertiary care hospital over a period of 1 year. AKI was assessed by KDIGO criteria and statistical analysis was performed using IBM SPSS 21. Data were tested for normality and analyzed using Student's t-test, Chi-square test, and Fisher exact test.
Results: One hundred and six neonates were included in the study. The incidence of hypernatremic dehydration with AKI was 2.07% among the hospitalized neonates, majority being males. Majority (44.43%) were fed with either cow/buffalo or goat milk and bottle fed. Poor feeding was the most common presenting complaint followed by fever, lethargy, loose stools, and regurgitation of feeds. 79.24% babies presented with urea levels >19 mg/dl, 29.24% with serum creatinine values of >1 mg/dl while 43.39% babies had urine output <1 ml/kg/min. Majority of the babies, 84.90% had Grade 1 AKI in which 91.25% presented with mild hypernatremia. Renal recovery rate was 99.1% while 2 died due to complications.
Conclusions: Hypernatremia, if severe can cause AKI in neonates which are not uncommon and are difficult to diagnose clinically. The presence of other factors such as feeding malpractices and sepsis results in poorer outcomes. High index of suspicion specially without predisposing factors may lead to early diagnosis and timely management.
| O5 Prevalence of Absolute and Functional Iron Deficiency in Nondialyzed Children with Chronic Kidney Disease|| |
Swati Rani, Mukta Mantan, K. Rajeshwari, Sarika Singh1, Vineeta V. Batra2
Departments of Pediatrics and 1Pathology, Maulana Azad Medical College and GIPMER and Associated Hospitals, New Delhi, India
Rationale: Limited data exist on the prevalence and correlates of absolute and functional iron deficiency in nondialysis-dependent chronic kidney disease (CKD) in India.
Objectives: To determine the prevalence of absolute and functional iron deficiency in children with CKD as measured by serum ferritin and TSAT levels and reticulocyte haemoglobin content (CHr) and percentage of hypochromic red blood cells (PHRC) for determination of functional deficiency.
Methods: This cross-sectional study was done at a tertiary care teaching hospital from July 2021 to July 22 and children (2–18 years) with CKD were included; those on dialysis, on erythropoietin/IV iron, with history of blood transfusion in preceding 3 months, chronic liver disease and with acute infections were excluded. Clinical details were noted, biochemical and hematological investigations were done for all.
Results: Fifty-five (47 Male: 8 Female) children with median age (interquartile range) 7 years (4; 10) were enrolled; 19 were on iron supplements for more than 3 months while 36 (65.5%) were treatment naive. Anemia was seen in 32 (58.1%) and increased from 39.3% in stage 1 to 100% in stage 4 and 5 CKD. Absolute iron deficiency was seen in 24 (43.6%) and functional in 8 (14.5%). The prevalence of absolute and functional iron deficiency was higher in later stages of CKD [Figure 1]. The PHRC and CHr values were significantly altered in later stages [Table 1], 40% and 1.8% had B12 and folate deficiency respectively and no significant difference was present in any parameters between those on iron supplements and without.
|Figure 1: Absolute and functional iron deficiency in different stages of chronic kidney disease. CKD: Chronic kidney disease|
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Conclusions: The prevalence of anemia and iron deficiency is high in children with CKD and the prevalence of functional iron deficiency increases significantly in later stages of CKD. Newer methods like PHRC and CHr can be used assessing functional iron deficiency especially in later stages.
| O6 The Diagnostic Performance of Furosemide Stress Test in Predicting Progression to Acute Kidney Injury Stage 3, Need for Kidney Replacement Therapy and Mortality|| |
Sudarsan Krishnasamy1, Aditi Sinha2, Arvind Bagga2, Pankaj Hari2
1Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, 2Department of Pediatrics, Division of Nephrology, All India Institute of Medical Sciences, New Delhi, India
Rationale: Furosemide stress test (FST) is a novel functional biomarker for predicting severe acute kidney injury (AKI). As there are no prospective pediatric studies, we aimed to examine the role of FST in predicting AKI progression in critically-ill children.
Objectives: To determine the diagnostic performance of FST in predicting progressionto AKI Stage 3, need for kidney replacement therapy (KRT) and mortality.
Methods: Children ≤18 years of age admitted to the intensive care (ICU) or high dependency unit (HDU) of a tertiary care hospital from November 2019 to July 2021 were screened for eligibility. Patients who developed AKI Stage 1 or 2 (KDIGO urine output or serum creatinine criteria) within 7 days of admission underwent FST (intravenous furosemide at 1 mg/kg in naïve and 1.5 mg/kg in exposed) after catheterisation and urine output was measured hourly for the next 6 hours; output >2 ml/kg within the first 2-hours was deemed furosemide responsive. Other biomarkers such as plasma neutrophil gelatinase-associated lipocalin (NGAL) and plasma proenkephalin (PENK) were also evaluated.
Results: Of the 480 admitted patients, 51 developed AKI Stage 1 or 2 within 7 days of ICU/HDU admission, and underwent FST. Twelve patients (23.5%) developed Stage 3 AKI within 7 days of FST, 9 (17.6%) of whom required KRT. FST emerged as a good biomarker for predicting stage 3 AKI and need for KRT with an area-under-the-curve (AUC) being 0.92 ± 0.05 (95% confidence interval 0.82–1.0; P < 0.0001) and 0.96 ± 0.03 (0.9–1.0; P < 0.0001), respectively. The corresponding AUC for NGAL and PENK were 0.75 ± 0.08 (0.59–0.91; P = 0.009) and 0.79 ± 0.08 (0.64–0.94; P = 0.003).
Conclusions: FST is a simple, inexpensive, and robust biomarker for predicting stage 3 AKI and KRT need in critically-ill children; larger prospective studies are recommended.
| O7 Incidence and Risk Factors of Acute Kidney Injury in Children with Idiopathic Nephrotic Syndrome|| |
Sailaja Gupta, Kanika Kapoor, Shobha Sharma, Anita Rani, Rani Gera
Departments of Pediatrics and 1Biochemistry, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
Rationale: Acute kidney injury (AKI) in children with idiopathic nephrotic syndrome (INS) is most often secondary to its complications such as hypovolemia, infections, nephrotoxic drug exposure and due to rapid progression of the disease. Prospective studies on incidence and risk factors in children with INS are limited.
Objectives: To determine incidence and risk factors of AKI in children with INS.
Methods: A prospective observational study was conducted at a tertiary care teaching hospital over a period of 18 months. All consecutive children aged 1 year to 12 years hospitalized with a diagnosis of NS were screened for eligibility. Children hospitalized with the first episode or relapse of INS were enrolled in the study. Those admitted for rituximab or cyclophosphamide infusion, renal biopsy, congenital nephrotic syndrome and secondary causes of NS were excluded. NS, its course and complications were defined as per recent ISPN guidelines. AKI was defined and staged as per KDIGO guidelines. Serial serum creatinine (S cr) was measured by modified Jaffe's method within 48 hours of admission and subsequently every 48 hours up to 7 days and/or at discharge. Baseline S cr was taken as the lowest available value in preceding 3 months if available or from estimated glomerular filtration rate of 120ml/min/1.73, m2 in >2 years and by back calculation using modified Schwartz formula in <2 year.
Results: 136 children with 160 episodes of hospitalization were screened for eligibility during the study period. Of these, 107 children with 121 episodes of hospitalizations were enrolled in the study. The median age was 5 years (3–7) with male to female ratio of 1.57. The median age of onset of NS was 3 years. [Figure 1] shows flow of study and outcome at discharge. The incidence of AKI was 11.6% (14 of 121 hospitalizations) in this study. As per KDIGO, 14.3% had stage 1, 50.0% stage 2 and 35.7% Stage 3 AKI. [Table 1] shows factors associated with AKI. On multivariate analysis, only use of ACE inhibitors (P = 0.015, odds ratio = 9.8) was found to be independent risk factor.
|Table 1: Risk factors of acute kidney injury in children with idiopathic nephrotic syndrome (n=121 hospitalizations)|
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Conclusions: AKI is common in children with INS. Longer duration of disease, hypoalbuminemia, hypercholesterolemia and use of nephrotoxic drugs increase risk of AKI. Majority of the patients with AKI recover completely.
| O8 Comparison between Patients Receiving Azithromycin Combined with Steroid and Steroid Therapy Alone with Respect to Time to Remission and Time to Next Relapse in Patients of Steroid Sensitive Nephrotic Syndrome|| |
Rajat Singh, Anubha Shrivastava
Moti Lal Nehru Medical College, Prayagraj, Uttar Pradesh, India
Rationale: Steroid sensitive nephrotic syndrome (SSNS) is one of the most common chronic diseases in children with annual incidence of 1.2 to 16.9 per 100,000 children. Around 52%–70% of relapses among children chiefly follow the upper respiratory tract infection. Studies have shown that patients who were given prednisolone in combination with azithromycin showed faster remission, but there is not enough research on reduction in the relapse rates.
Objectives: To compare time to remission and time to next relapse in patients receiving azithromycin combined with steroid in comparison to patients receiving steroid therapy alone in patients of SSNS.
Methods: A prospective randomly controlled trial was conducted. Randomization was performed to select the research subjects who were composed of children with SSNS and treated with azithromycin combined with prednisolone (the intervention group) and with prednisolone alone (the control group). A total of 42 randomly selected patients with SSNS received either azithromycin combined with prednisolone (n = 21) or prednisolone alone (n = 21) till BSUP nil/trace for three consecutive days and followed till next relapse or 1 year whichever was earlier. During follow-up period, four patients, 2 of each group lost to follow up.
Results: The baseline characteristics of the patients in intervention and control groups were similar. The median duration to remission was 8 days in the intervention group and 11 days in control group (P > 0.05). The median duration for time to next relapse in the intervention group was 87 days and in the control group 91 days (P > 0.05). The median duration for time to next relapse in the intervention group was 87 days and in the control group 91 days (P > 0.05).
Conclusions: No significant difference seen in time to remission and time to next relapse in both groups.
| O9 Clinical Utility of Home, Clinic, and Ambulatory Blood Pressure Measurement in Children with Chronic Kidney Disease|| |
Manju J. Inbaraj, Georgie Mathew, R. V. Deepthi, Indira Agarwal
Department of Paediatric Nephrology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India
Background: Hypertension is a significant modifiable factor that determines the progression of chronic kidney disease (CKD) among children. Clinic blood pressure (CBP) may over or underestimate BP during measurement. A reliable, convenient, cost-effective “out-of-office” method of monitoring BP for children is desirable.
Objective: To determine the clinical utility of home BP monitoring (HBP) in the management of hypertension in children with CKD.
Methods: This was a prospective study in a tertiary center wherein children aged 5–18 years with CKD underwent successive measurements of CBP, HBP (average of 2 readings taken thrice a day for 3–5 days), and ambulatory BP monitoring (ABPM).
Results: Fifty children (median age 12.5 years, interquartile range 6, 78% boys) successfully underwent measurements with all three methods. CAKUT was the predominant presentation (68%). CKD stage III was present in 32% followed by stage 2 (18%). Fifty-four percent were already on anti-hypertensives. HBP detected hypertension with greater specificity (92.6%) than CBP (74.1%) while the sensitivity of both was poor (47%). However, elevated BP in both “CBP and HBP” increased sensitivity from 47.8% to 78.3%. There was moderate agreement between the values of HBP and ABPM (κ = 0.46 [0.23–0.69, P < 0.001) while the agreement between CBP and ABPM was fair. Mean arterial pressure values of HBP were consistently lower than that of ABPM (−2.93 ± 8.10 (95% confidence interval [CI]-5.25, −0.6), P = 0.015, r = 0.72. Similarly, HBP was consistently lower than ABPM in systolic (−3.97 ± 8.64 [(95% CI-6.42, −1.51), P = 0.002], r = 0.69) and diastolic values (2.36 ± 7.95 [(95% CI −0.10, 4.62), P = 0.041], r = 0.69). White coat hypertension was seen in 25.9% with ABPM and 33.3% with HBP. Masked hypertension was detected in 52.15% while nocturnal hypertension was detected in 56%; 12% had isolated nocturnal hypertensionby ABPM.
Conclusions: HBP is an economical, convenient, well-tolerated tool for use in children. It has high specificity for the confirmation of hypertension. Combined with CBP, it is also a good screening tool. Its good agreement with ABPM makes it a suitable alternative for diagnosing and monitoring hypertension. ABPM is preferred for the clinical suspicion of white coat, masked, or nocturnal hypertension.
| O10 Adrenocortical Suppression in Nephrotic Syndrome Children and Adolescents (2–18 Years) Treated with Corticosteroids|| |
M. Ganeshkrishna, Mukta Mantan, Akshay Kumar, Aashima Dabas, Binita Goswami
Departments of Pediatrics and 1and Department of Biochemistry*, Maulana Azad Medical College & and Associated Hospitals, New Delhi, India.
Rationale: Both steroid-sensitive nephrotic syndrome (SSNS) and steroid-resistant nephrotic syndrome (SRNS) patients receive alternate day steroids for prolonged periods and are at increased risk for adrenocortical suppression. While single morning cortisol values are used for screening, an ACTH stimulation test is more definitive to identify this suppression.
Objectives: The primary objective was to determine the prevalence of adrenocortical suppression in SSNS or SRNS patients (2-–18 years), who were on either low dose alternate day steroids(<1mg/kg/day) or who received any dose of daily steroids for ≥ 2 weeks in the last 1 year and currently in remission. Subjects and methods
Methods: In this cross-sectional study, children (2-–18 years) with both SSNS (n = 27) and SRNS (n = 25) were included; those on daily prednsiolone or having serious bacterial infections or hospitalized were excluded. Clinical details were elicited and examination was done; remission was confirmed by biochemical investigations. A baseline morning fasting sample of serum cortisol was taken and 25 IU of ACTH (Acton Prolongatum*) was injected intramuscularly for post stimulation test (serum cortisol sample taken after 1 hour). All patients with 1 hour post- ACTH cortisol <18.0 μg/dl were diagnosed as having adrenal insufficiency.
Results: Fifty-two (33 Male: 19 Female) children were enrolled with a median age of 10 years; 23.1% were steroid-dependent nephrotic syndromeSDNS, 17.3% were frequent relapse nephrotic syndromeFRNS, 11.6% were infrequent relapse nephrotic syndromeIFRNS and 48.1% were SRNS patients. The pPrevalence of adrenocortical suppression was 50% by baseline early morning cortisol levels and 69.2% after ACTH stimulation test. Twelve12 (23%) had short stature & and 2 (3.8%) had obesity, 44.2 % experienced weakness/fatigue, whereas 30% had cushingoid features. The total cumulative dose of corticosteroid in the last one year (>0.22 mg/kg/day) was statistically correlated with adrenocortical suppression.
Conclusions: The prevalence of adrenocortical suppression in children on low dose alternate day steroid is high. While baseline cortisol levels may be used for screening, stimulation test identifies at least 20% more patients. Cumulative steroid intake of > 0.22 mg/kg/day emerged as a risk factor for predicting adrenocortical suppression [Figure 1].
|Figure 1: Diagnostic value of total cumulative prednisolone dose for adrenocortical suppression. CI: Confidence interval|
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|Table 1: Comparison of baseline parameters between adrenocortical insufficiency and no insufficiency|
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| O11 Crescentic Glomerulonephritis in Children: Clinical Spectrum and Predictors of Renal Survival|| |
Nikita Gupta, Alpana Ohri, Amish Udani, Chintan Shah
Division of Pediatric Nephrology, B. J. Wadia Hospital for Children, Mumbai, Maharashtra, India
Rationale: Crescentic glomerulonephritis (CGN) is rare form of glomerulonephritis in children with grave prognosis. The literature on pediatric CGN is quite limited.
Objective: We demonstrate a single center study to evaluate the clinical and histopathological profile in children with CGN and determine the predictors of renal outcome. We also compared the renal survival in patients with 20%–50% crescents to those having >50% crescents.
Methods: In this retrospective cohort study, we reviewed all native kidney biopsies performed in patients <18 years over a period of 9 years (2011–2019). Those having ≥20% crescents with follow-up for at least 1 year were enrolled.
Results: Study comprised of 34 patients. The most common variety was immune-complex GN (type II CGN) (n = 21; 62%) including patients with Henoch-Schonlein purpura (n = 6), lupus nephritis (n = 6), postinfectious GN (n = 3), C3GN (n = 3), and dense deposit disease (n = 3). The second most common was pauci-immune GN (type III CGN) (n = 12; 35%) followed by anti-glomerular basement membrane disease (type I CGN) (n = 1; 3%). Hypertension (88%), hematuria (84.2%), and oliguria (64%) were the most common presenting features. Outcome predictors for poor renal survival were the presence of oliguria (HR 5.11, P=0.035), severe hypertension (HR 11.51, P = 0.019), estimated glomerular filtration rate <15ml/min/1.73m2 at presentation (HR 5.05, P = 0.007), percentage of crescents (HR 10.66, P = 0.001), presence of fibrous crescents (HR 6.34, P = 0.001), and interstitial fibrosis and tubular atrophy (HR 8.88, P = 0.0046). Overall outcome of study revealed complete recovery (n = 12), partial recovery (n = 6), CKD (n = 3), and ESRD (n = 13). There was no significant difference in renal survival in Type II and Type III CGN. Patients with ≥50% crescents had poor renal survival (P = 0.037) than those with 20%–50% crescents.
Conclusions: Renal survival can be predicted by the severity of presenting features and histopathological markers. Two third of patients had Type II CGN with renal survival outcome similar to type III CGN. Percentage of crescents is the most important predictor of renal survival.
| O12 Renal Complications and Short-Term Outcomes in Bilateral Wilm's Tumor – A Case Series|| |
Nisha Krishnamurthy, Uma S. Ali, Rasik Shah, Sajid Qureshi
Department of Pediatric Nephrology, Unit of Narayana Health, SRCC Childrens Hospital, Mumbai, Maharashtra, India
Rationale: Although accounting for 4%–7% of all Wilm's tumor, bilateral Wilm's tumor (BWT) poses a challenge in management because of the conflicting goals of removing the tumor completely and preserving maximal renal tissue and function.
Methods: All cases of BWT <18 years age, operated at our hospital, between 2017 and 2022.
Results: Seventeen children, 11 girls and 6 boys, all <5 years of age. Out of 17, 15 had synchronous tumor occurrence and 2 had metachronous. BWT surgery types: (a) bilateral nephrectomy in 1, (b) bilateral nephron sparing surgery (NSS) in 6, (c) unilateral nephrectomy with contralateral NSS in 9 and (d) unilateral nephrectomy in 1. Preoperatively 9 (52%) and postoperatively 13 (76%) had hypertension that was controlled with a single drug. Serum creatinine was normal in 15 (88%) patients preoperatively. Postoperatively, on D1, it rose to >1.5 times baseline in 3 (17%) and to >3 times baseline in 7 (41%). This subsequently declined to <1.5 times baseline in 6 (35%) patients at discharge. Two (12%) had >3 times baseline value at discharge. Preoperatively, 15 (88%) patients had eGFR >90 ml/min/1.73m2. At discharge, 10 (59%) had >90 ml/min/1.73m2 and 7 (41%) had <60 ml/min/1.73m2. Two patients required renal replacement therapy (RRT), of which 1 was due to bilateral nephrectomy, survived up to discharge but died within 6 months. Proteinuria was seen in all patients, with 13 (76%) having urine protein-creatinine ratio in the range of 0.2–2.0. All patients who had NSS were nonoliguric and none required RRT. Short-term patient survival and renal outcome without RRT, both were good at 88%. Multidisciplinary care helps in improving short-term outcomes; long-term outcome requires a close follow-up.
| O13 Etiology, Outcomes, and Predictors of Kidney Survival in Children with Acute Glomerulonephritis and Crescents from India|| |
Bobbity Deepthi, Sriram Krishnamurthy, Rajesh Nachiappa Ganesh1, Amar Murdeshwar, Sachit Ganapathy2, Sudarsan Krishnasamy
Departments of Pediatrics, 1Pathology, and 2Biostatics, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
Rationale: There is a paucity of information on the etiology and outcomes of children with acute glomerulonephritis and crescents from India.
Objectives: To determine the etiology, outcomes, and predictors of kidney survival in these children.
Methods: A cohort of patients aged 1–18 years, diagnosed with acute glomerulonephritis and crescents on kidney biopsy over a 6-year period were enrolled. The clinical and laboratory parameters were recorded. Renal outcomes and predictive risk factors for kidney survival were determined.
Results: A total of 68 subjects were included; all were immune-complex glomerulonephritis (Lupus nephritis 23 [33.8%], C3 glomerulonephritis 21 [30.9%] and postinfectious glomerulonephritis 15 [22.1%], immunoglobulin A (IgA) nephropathy 5 [7.4%], immune-complex membranoproliferative glomerulonephritis 3 [4.4%], and IgA vasculitis 1 [1.5%]). The median (interquartile range [IQR]) age at presentation was 10 (7, 12) years, with no gender predilection. Majority 64 (94.1%) presented with low estimated glomerular filtration rate (eGFR), 60 (88%) had oliguria, 64 (94.1%) were hypertensive, and 63 (92.6%) had proteinuria at presentation. The median (IQR) eGFR at admission was 19 (10.93, 38.60) mL/min/1.73m2, and it increased to 126 (102.7, 142) mL/min/1.73m2 at the last follow-up, P < 0.001. At the last follow-up (median [IQR] 24.5 [12, 48] months), 39 (60%) achieved complete renal recovery, 17 (26.1%) had partial renal recovery, 9 (13.8%) were in CKD stage 3 and beyond, 3 (4.4%) children died within 6 months of diagnosis. Kidney survival was 77.9% and 76.4% at 1-year and at the last follow-up respectively. The prevalence of end-stage renal disease was 7.3% at 1-year and 7.7% at the last follow-up. The factors predicting renal survival were the duration of symptoms prior to presentation >7 days, percentage of crescents >37.5%, presence of fibrous crescents and segmental sclerosis.
Conclusions: Immune-complex glomerulonephritis is the most common etiology of acute glomerulonephritis with crescents in children. Renal outcomes are determined by duration of symptoms prior to presentation, and percentage and stage of crescents.
|Figure 1: Etiology. eGFR: Estimated glomerular filtration rate, IgA: Immunoglobulin A, IC-MPGN: Immune-complex membranoproliferative glomerulonephritis|
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| O14 Etiology and Outcomes of Acute Kidney Disease in Children|| |
Amar Murdeshwar, Sriram Krishnamurthy, Narayanan Parameswaran, Medha Rajappa1, Bobbity Deepthi, Sudarsan Krishnasamy, Sachit Ganapathy2, Pediredla Karunakar
Departments of Pediatrics, 1Biochemistry and 2Biostatistics, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
Rationale: There is a paucity of information regarding the etiology and outcomes of acute kidney disease (AKD) in children.
Methods: The objectives of this cohort study were to evaluate the etiology and outcomes of AKD and analyze the predictors of kidney survival. Patients aged 1 month to 18 years who developed AKD over a 4-year-period (between January 2018 and December 2021) were enrolled. Survivors were followedup at the pediatric nephrology clinic and screened for residual kidney injury.
Results: Among 5710 children who developed AKI, 200 who developed AKD were enrolled. The median (interquartile range [IQR]) age at admission of the children with AKD was 7 (4, 11) years and the median (IQR) estimated glomerular filtration rate (eGFR) was 17.03 (10.98, 28) mL/min/1.73 m2. Acute glomerulonephritis, acute tubular necrosis (ATN), hemolytic uremic syndrome (HUS), sepsis-associated AKD, and snake envenomation comprised of 69 (34.5%), 39 (19.5%), 24 (12%), 23 (11.5%), and 15 (7.5%) of the patients, respectively. Overall, 88 (44%) children required kidney replacement therapy. There were 37 (18.5%) deaths within the AKD period (7–90 days after diagnosis of AKI). At a follow-up of 90 days, 32 (16%) progressed to chronic kidney disease stage-G2 or greater. At a median (IQR) follow-up of 24 (6, 36.5) months (n = 154), 27 (17.5%) had subnormal eGFR and 20 (12.9%) had persistent proteinuria and/or hypertension. Kidney survival was 92% at 1 year, 90% at 2 years and 90% at 3 years in glomerular disorders; in nonglomerular disorders, survival was 71%, 70.5%, and 70.5%, respectively. Time to resolution of oliguria ≥14 days (HR 3.08 [95% confidence interval (CI) 1.6, 5.9]; P = 0.001) and multiorgan dysfunction syndrome (MODS) during the initial AKI-initiating event (HR 3.32 [95% CI 1.21, 9.15]; P = 0.02) were the predictors of kidney survival.
Conclusions: Acute glomerulonephritis, ATN, HUS, sepsis-associated AKD, and snake envenomation were the common causes of pediatric AKD. Time to resolution of oliguria>14 days and MODS predicted poor kidney survival.
|Table 1: Predictors of kidney survival (free of chronic kidney disease G2, G3a, G3b, G4, G5 or death) among patients of acute kidney disease on univariate and multivariate analysis|
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| O15 To Study Presentation and the Risk Factors for Poor Outcomes in Neonatal Acute Kidney Injury|| |
Paediatric Nephrotic Society of Bangladesh, Bangladesh Shishu Hospital and Institute, Dhaka, Bangladesh
Rationale: Neonatal acute kidney injury (AKI) has been accounted as a leading cause of neonatal mortality and morbidity. The adverse outcome of neonatal AKI can be prevented by identifying the high-risk infants and managing them meticulously. This study was conducted to find out the outcome of neonatal AKI and to explore the risk factor behind poor outcome.
Objectives: To find out the presentation and the risk factors for poor outcomes in neonatal AKI.
Methods: This prospective study was carried out among the neonates admitted to the ICU of Bangladesh Shishu Hospital and Institute from January 2021 to January 2022. All the neonates >3 days of age and had AKI following the modified KDIGO criteria were included in the study. Those neonates with lethal chromosomal or kidney anomalies and those who died within 48 hours after hospital admission were excluded from the study. The outcome of the neonates was observed. All the neonates were divided into two groups depending upon their hospital outcome (favorable and nonfavorable) and the impact of risk factors on both the groups were compared and analyzed.
Results: Among 48 neonates with AKI, 30 (62.5%) patients were in stage III AKI, 13(27%) patients had stage II AKI and the rest 5 (10.4%) patient had stage I AKI. The possible causes were attributed to sepsis, hypovolemia, perinatal asphyxia, intraventricular haemorrhage, drug-induced, obstructive uropathy, renal vein thrombosis and unidentified. Regarding outcome, 30 (62.5%) patients were expired during hospitalization, 18 (37.5%) patients survived and were discharged. In multivariate regression model, sepsis (adjusted odds ratio [AOR] = 3.4; P < 0.001), cardiac comorbidities (AOR = 2.5; P < 0.001), volume overload (AOR = 2.605, P < 0.001), surgical intervention (AOR = 1.6, P < 0.004), mechanical ventilation (AOR = 1.463, P < 0.015), and multiple inotrope administration (AOR = 1.566, P < 0.008) were associated with the worst outcome of neonatal AKI.
Conclusions: Neonatal AKI has unfavorable outcome which is associated with sepsis, cardiac comorbidities, surgical intervention, cardiovascular instability, and use of mechanical ventilation.
| O16 To Evaluate the Clinicopathological Spectrum, Efficacy and Safety of Percutaneous Ultrasound Guided Renal Biopsies at a Tertiary Care Center|| |
Mritunjay Kumar, Amit Shukla, Namita Mishra
Department of Pediatrics, AIIMS Raebareli, SGRR Institute of Medical Sciences, Dehradun, Uttarakhand, India
Rationale: Renal biopsy is a well-established diagnostic modality for the assessment of kidney diseases in children. It can provide diagnostic precision, prognostic value and guide in therapeutic options for many renal diseases. As clinico-epidemiological profile of kidney diseases vary in different population, indications for renal biopsy could also be different. Efficacy and complication rate could also be varies depending upon the set up and skills available. We conducted a retrospective audit of the first 23 renal biopsies conducted over a period of 1 year at a newly developed paediatric nephrology center of a tertiary care teaching hospital in Uttarakhand.
Objectives: To evaluate the clinicopathological spectrum, efficacy, and safety of percutaneous ultrasound-guided renal biopsies at a pediatric nephrology clinic of a tertiary care teaching hospital in Uttarakhand.
Methods: A retrospective study was conducted based on medical records from all patients who had undergone percutaneous ultrasound-guided renal biopsy (PRB) over a period of 1 year. Demographic data (age and gender), clinical symptoms at presentation, indications for renal biopsy, laboratory findings, complications of the procedure, and histological diagnosis were obtained from all patients who underwent PRB. Biopsy was performed using 16 G or 18 G biopsy needles. At least 2 cores were taken for each biopsy except in those where 1 additional core was taken for electron microscopy (EM) evaluation.
Results: A total of 205 patients were registered at the pediatric nephrology clinic over the study period of 1 year out of which 139 (67.8%) were males and 66 (32.2%) were females. Twenty-three (11.2%) children underwent percutaneous ultrasound-guided renal biopsy. Commonest indication for renal biopsy was steroid resistant and steroid dependent nephrotic syndrome (before starting calcineurin inhibitors) followed by acute glomerulonephritis. Biopsy was conducted with 16 G (78.3%) and 18 G (21.7%) biopsy gun. All the biopsies were conducted under Midazolam and Ketamine sedation and local 2% lidocaine. Mean number of passes for each biopsy was 3.3 ± 2 standard deviation (SD) with a maximum of 5 passes and minimum of 2 passes for each patient. Three cores were obtained in 4 (17.4%) children and 2 cores were taken in 19 (82.6%) children with a mean of 3.3 ± 2 SD cores each biopsy. Two cores were sent for light microscopy (LM) and IF in each biopsy however in 4 children an additional core was obtained for EM. In LM mean number of glomeruli reported were 26.2 ± 2 SD with a maximum of 64 glomeruli and a minimum of 5 glomeruli for each biopsy sample. For IF, mean number of glomeruli reported were 10.02 ± 2 SD with a maximum of 20 glomeruli and a minimum of 3 glomeruli for each sample. Twenty-one (91.3%) LM tissues were labelled satisfactory; however, 2 (8.6%) were reported unsatisfactory samples. Focal segmental glomerulosclerosis was the most common histopathological diagnosis and it was found in 6 (26%) biopsies. Three (13%) biopsies were reported as lupus nephritis. Minimal change disease, infection related glomerulonephritis and acute tubular necrosis (ATN) was found in 2 (8.7%) biopsies each. C3 glomerulopathy, acute cortical necrosis, dense deposit disease, ANCA associated vasculitis, thrombotic microangiopathy, C1-9 nephropathy, immunoglobulin A nephropathy, ATN, and C3 glomerulopathy were reported in 1 (4.4%) biopsy each. All 23 (100%) children complained of pain at the biopsy site once the local anaesthesia and sedation effect was over. Perinephric hematoma was detected in 5 (21.7%) biopsies and post biopsy transient hematuria was observed in 3 (13%) biopsies. None of the children required blood transfusion following biopsy. Interventions such as coil embolization and nephrectomy were not required in any of the children who underwent biopsy.
Conclusions: Percutaneous ultrasound-guided renal biopsy is a safe, reliable, and effective technique in children and provides updated epidemiological data regarding renal disease pattern from a pediatric population in a geographic region never reported previously.
| O17 Use of Furosemide Stress Test and Cystatin-C as Predictive Markers of Acute Kidney Injury Progression in Children: A Prospective Cohort Study|| |
S. Dyvik, Daisy Khera, Aliza Mittal, Kuldeep Singh, Bharat Choudhary, SiyaramDidel, Purvi Purohit
All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
Rationale: Furosemide stress test (FST) is a simple, safe, noninvasive, low-cost tool for predicting progressive renal impairment. However, its usage is not validated in the pediatric population.
Objectives: To validate the use of FST in children as predictive marker of acute kidney injury (AKI) progression-from KDIGO stage-1/2 to stage-3. To evaluate the association between FST nonresponsiveness and need of renal replacement therapy (RRT), compare the predictive values of FST and Cystatin-C in the early detection of AKI worsening.
Methods: Prospective cohort study. One month-18 years old admitted in the pediatric intensive care unit and pediatric emergency with stage 1/2 AKI. FST (Dose: 1 mg/kg of furosemide in naive patients or 1.5 mg/kg in furosemide exposed) was performed followed by urine output and kidney function test monitoring to recognise progression to AKI stage 3. Serum and urine Cystatin-C levels were analyzed at 12 hours.
Results: Out of 92 evaluated for eligibility, 41 children were enrolled in the study. 7/41 (17.07%) progressed to KDIGO stage 3 AKI. 4/41 (9.76%) and 5/41 (12.19%) subjects were furosemide nonresponders at 2 hours and 6 hours post-FST, respectively. All of the furosemide nonresponders at 2 and 6 hours progressed to AKI stage 3, which accounted for sensitivity and specificity of 57.14% and 100% respectively at 2 hours and 71.43% and 100%, respectively, at 6 hours. Area under the receiver operating characteristic (AuROC) curve in predicting AKI progression with FST nonresponsiveness at 2 and 6 hours were 0.845 (P = 0.01) and 0.872 (P < 0.001), respectively. Out of 6 who received RRT, 4/6 (66.66%) children at 2 hours and 5/6 (83.33%) at 6 hours were nonresponders to FST (P = 0.0001). All furosemide nonresponders at 6 hours received RRT.3/4 (75.00%) furosemide nonresponders at 2 hours (P = 0.018) and 3/5 (60.00%) furosemide nonresponders at 6 hours (P = 0.042) died during the hospital stay. Total 9/41 (21.95%) were serum Cystatin-C positive, of which 5/9 (55.56%) progressed to stage 3 AKI indicating sensitivity of 71.43% and specificity of 88.24% with an AuROC curve of 0.752 (P = 0.084). 20/41 (48.78%) patients showed urine Cystatin-C positivity, of which 7/20 (35.00%) children progressed to AKI stage 3, accounting for sensitivity of 100% and specificity of 61.76% with AuROC of 0.918 (P = 0.003).
Conclusions: FST serves as a simple bedside tool which has robust predictive value in detecting renal impairment progression in children and also in determining requirement for RRT. The diagnostic value of FST at 6 hours is comparable with that of urine Cystatin-C, which has the best diagnostic accuracy in our study (AuROC = 0.918). Serum Cystatin-C has a lower predictive value than FST. To conclude, FST can be used in pediatric intensive care unit settings for tubular dysfunction assessment, while there is a need for larger studies to draw further conclusions in this regard.
|Figure 1: Comparison of AuROC curves of FST at 2 h, FST at 6 hours, serum cystatin C and urine cystatin C with progression to stage 3 AKI. AuROC: Area under the receiver operating characteristic, FST: Furosemide stress test, AKI: Acute kidney injury|
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| O18 Urinary Biomarkers Neutrophil Gelatinase-Associated Lipocalin and Beta-2 Microglobulin for Detection of Diabetic Nephropathy in Type 1 Diabetes Mellitus|| |
Nimisha Sachan, Aashima Dabas, Pradeep Kumar Dabla1, Mukta Mantan
Departments of Pediatrics and 1Biochemistry, Maulana Azad Medical College and Associated Hospitals, New Delhi, India
Rationale: Diabetic nephropathy is the most common chronic complication of TIDM that is screened by measuring microalbuminuria after 5 years of disease onset. Recent reports have shown that early tubular damage precedes glomerular damage in TIDM, necessitating the need to identify sensitive biomarkers, preferably noninvasive markers like urinary analytes.
Objectives: This study aimed to estimate the urinary levels of neutrophil gelatinase-associated lipocalin (NGAL) and beta-2 microglobulin in children with T1DM and correlate them with disease duration, glycemic control, and microalbuminuria.
Methods: This case−control study enrolled 40 children with T1DM (duration of diabetes more than 2 years) and 40 age-matched nondiabetic controls. Subjects with coexisting renal disorder and syndromic diabetes mellitus were excluded. Blood samples were evaluated for fasting plasma glucose, glycated hemoglobin A1c (HbA1c), renal functions (cases and controls). A 24-hour timed urine sample was evaluated for urine albumin-creatinine ratio (ACR), NGAL, and beta-2 microglobulin using ELISA.
Results: The mean age of cases was 10.58 (8.00, 14.15) and was comparable to controls (P = 0.799). Median (interquartile range) duration of T1DM was 4.00 (3.00, 6.75) years (18 cases had >5 years duration) and HbA1c 10.90 (9.03, 13.10) %. [Table 1] shows the comparison of biochemical parameters between cases and controls. Among cases, ratio of ACR with NGAL/creatinine (r = 0.38, P = 0.019) and beta-2 microglobulin/creatinine (r = 0.48, P = 0.004) was significant. On comparing cases with disease duration 2–5 years with controls shows elevated levels of NGAL (P = 0.014) and beta-2 microglobulin (P = 0.041). The receiver operating characteristic curve to predict ACR >30 mg/g showed area under the curve (AUC) as 0.569 (95% confidence interval [CI] 0.357, 0.781); P = 0.478 for NGAL/creatinine and AUC as 0.621 (95% CI 0.433, 0.809); P = 0.212 for beta-2 microglobulin/creatinine [Figure 1].
|Figure 1: ROC analysis of urinary biomarkers for microalbuminuria. ROC: Receiver operating characteristic|
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Conclusions: Urinary biomarkers NGAL and beta-2 microalbuminuria increased with duration of T1DM and showed good correlation with ACR suggesting their role in screening of diabetic nephropathy and importance of early screening.
|Table 1: Comparison of clinical and biochemical parameters between cases and controls|
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| O19 Role of Urinary Biomarkers in Diagnosing Uretero Pelvic Junction Obstruction – A Pilot Study|| |
Sangeetha Geminiganesan, Ramesh Babu, Mohanapriya
Departments of Pediatric Nephrology, Pediatric Urology and Genetics, SRIHER, Chennai, Tamil Nadu, India
Rationale: One of the major challenges in the management of antenatally diagnosed hydronephrosis is differentiation nonobstructive dilatation (NOD) from ureteropelvic junction obstruction (UPJO). While early selection and prompt surgical intervention is crucial in preserving renal function in UPJO, it is also essential to limit the time-consuming, costly and invasive investigations in those with NOD.
Objectives: In this pilot study, we aimed to analyze the role of biomarkers in diagnosing UPJO.
Methods: A prospective study was performed between January 2022 and September 2022 to analyze urinary biomarkers/creatinine ratio. The control (Group 1; n = 34) involved children with normal urinary tract ultrasound; while the study group involved children with hydronephrosis (n = 32): divided into NOD (Group 2a; n = 15) and UPJO (Group 2b; n = 17). While NOD was diagnosed based on the resolution of hydronephrosis on serial ultrasounds, UPJO was diagnosed when there was worsening of hydronephrosis grade or deterioration of renal function on renogram warranting a pyeloplasty. Urinary biomarkers (NGAL, KIM-1 and CA 19-9) were analyzed using the ELISA method.
Results: There was no significant difference in age or sex distribution between control and study groups. Among the UPJO group, there was significant elevation of biomarker/creatinine ratio compared to control or NOD groups. There was no significant difference in biomarker/creatinine ratio between controls and NOD.
Conclusions: Urinary biomarker assay is a promising noninvasive method in differentiation of UPJO from NOD. Further larger studies are warranted to support these findings.
| O20 Experience Of Renal Biopsy And Clinicopathological Correlation In Children At A Tertiary Care Center In Eastern India|| |
Amit Kumar Satapathy, Joseph John, Bhagirathi Dwibedi, Samarendra Mahaptro
AIIMS, Bhubaneswar, Odisha, India
Rationale: Renal biopsy is an important diagnostic tool for children with various kidney disease. It not only helps in diagnosis but also help in explaining the prognosis and guiding the management too. Here, we are presenting an experience of renal biopsy in children with various kidney disease from a tertiary care hospital.
Objectives: This study was conducted to retrospectively to investigate the indications for renal biopsy in native kidneys and to analyze the pathological findings in the last 5 years in a single tertiary pediatric hospital in Eastern India.
Methods: All children who underwent renal biopsy at our hospital between 2017 and 2021 were included in this study. Renal biopsy was performed after taking consent and assent wherever applicable. All children were admitted for renal biopsy in hospital. It was performed under ultrasound guided with a biopsy gun by radiologist taking all precautions. All renal biopsies were studied under light and immunofluorescent microscopy.
Results: In the present study, 54 children who underwent percutaneous native kidney biopsies during the study period. Out of 54, 24 (44%) were girls. Median age was 12 years (range 3–15 years). The most frequent indications for renal biopsy were nephrotic syndrome followed by secondary glomerulonephritis (GN). Podocytopathy was the most common histopathological finding (48%) children, followed by FSGS and C1q nephropathy was seen in 9% and 4% children, respectively. Mesangioproliferative GN was documented in (6%) children. Among the children with secondary GN, lupus nephritis was seen in 22% children followed by and immunoglobulin A nephritis in 11% of children.
Conclusions: The epidemiology of glomerular disease is children similar to the other parts of world where podocytopathy and minimal change disease remains the most common etiology. However, we have a higher number of secondary GN secondary to lupus nephritis which may be due to referral bias as our center is one of few public run hospital where renal biopsy is conducted for children eastern part of India.
| O21 Hypothalamic-Pituitary-Adrenal Axis Suppression in Children with Nephrotic Syndrome – An Observational Cross-Sectional Study|| |
Jyoti Bagla, Surbhi, Smriti Rohatgi, Ruchi Mishra, Sarika Arora1, Anand Prakash Dubey, Jagdish Chandra
Departments of Pediatrics and 1Biochemistry, ESI-PGIMSR Basaidarapur, New Delhi, India
Rationale: Prolong and recurrent use of steroids in children with nephrotic syndrome makes them vulnerable to steroid induced toxicity including hypothalamic-pituitary-adrenal axis (HPA) suppression [Figure 1].
Objective: To study HPA axis suppression in nephrotic children (1–13 years) who had received prolong steroid therapy (atleast treated for the 1st episode of nephrotic syndrome) and who were in remission at the time of recruitment and off steroid for 2–4 weeks.
Methods: Observational cross-sectional study from December 2020 to April 2022. The sample size calculated was 51 (G*Power 126.96.36.199 software). Serum cortisol level was measured at baseline (8 AM) and following ACTH Stimulation (Inj Acton Prolangatum-25 IU IM) at 60 and 120 min. Baseline cortisol <5mcg/dl was taken as suggestive of decreased adrenal reserve; however, peak cortisol <19.5 mcg/dl post stimulation was taken as confirmatory for adrenal suppression.
Results: Amongst study subjects, the median age was 6.11 years and male: female ratio was 1.55:1. Low baseline cortisol was observed in 39.20% (20/51); however, 27% (14/51) nephrotics had confirmed HPA axis suppression following ACTH stimulation test. Steroid dependant nephrotic syndrome children were having maximum HPA axis suppression 7/16 (43.75%) followed by FRNS 5/16 (31.25%). Cumulative dose of steroid in HPA axis suppressed group was higher than normal HPA axis nephrotics (6813.23 weeks vs. 3352.88 weeks) (P < 0.001). Comparing HPA axis suppressed group with normal axis nephrotics, duration of steroid therapy (234.64 vs. 57.36) (P = 0.060), number of relapses (6 vs. 3) (P = 0.018) and hospitalization (4 vs. 2) (P < 0.001) was significant in HPA axis suppressed nephrotics [Table 1]. HPA axis suppressed nephrotics showed higher body mass index (16.84 vs. 14.01) (P = 0.007), shorter height (107 vs. 131) (P = 0.030), and hypertension (64.28% vs. 32.43%) (P = 0.039) as compared to nonsuppressed nephrotics.
Conclusions: The high index of suspicion is required to diagnose HPA axis suppression in nephrotics, especially those who are steroid dependent and frequent relapsers. It is important to diagnose HPA axis suppression to reduce the burden of illness in terms of morbidity, mortality, and cost of care.
| O22 The Renal Consequences of Prematurity: Are We Screening Enough?|| |
Prijo Philip, Indira Agarwal, M. Suresh, J. P. Nithya1, Sridhar Gibikote2, Jayakumar Amrithraj3, Thenmozhi4
Departments of Pediatric Nephrology, 1Neonatology, 2Radiology, 3Clinical Biochemistry, 4Biostatistics, Christian Medical College, Vellore, Tamil Nadu, India
Rationale: Prematurity increases the risk for renal impairment due to lower nephron endowment. Further exposure to hypoxic damage, hemodynamic changes, nephrotoxic medications in the neonatal period increases the risk for glomerular and tubular damage. Early onset hypertension, elevated creatinine, and proteinuria may be the markers of these sequelae. Measurement of renal volume, rather than size and serum uromodulin, a marker of nephron endowment, may be the indicators of said renal consequences and warrant further study.
Objectives: To study the impact of prematurity on renal size, volume, and its effect on glomerular and tubular function and to study serum uromodulin levels and its correlation with renal function.
Methods: This cross-sectional study was performed on children born preterm between January 2014 and December 2016. Those fitting the inclusion criteria were called for follow up between January and December 2019. Blood pressure (BP) measurements, proteinuria and creatinine estimation, creatinine clearance calculation, and mean kidney volume (MKV) were calculated by renal ultrasound. Serum uromodulin was estimated by EUROIMMUN ELISA kit.
Results: The mean age at follow-up for the 37 children who responded was 5.8 years. Thirty-five percent (13/37) of children had BP at 90th centile and above; 3/37 (7%) had prehypertension and 5/37 (14%) had stage 1 hypertension. Twenty children (20/37, 54%) had an estimated glomerular filtration rate (eGFR) of between 90 and 120 ml/min/1.73 m2; none had pathological proteinuria. No correlation was observed between gestational age (GA) and renal parameters. MKV was 34.86 ± 8.64 ml, with lower MKV for those born at GA <28–33 weeks (P = 0.0.05). The mean serum uromodulin was 62.10 ± 45.84 ng/ml. Low serum uromodulin level had a linear relationship with hypertension and eGFR (P = 0.8 and 0.5). However, the correlation between uromodulin levels <60 ng/ml and reduced MKV was very significant (P = 0.002). At a uromodulin cut off of <52 ng/ml, there was significant reduction of MKV (<32 ml) (area under the curve 0.715, P = 0.02).
Conclusions: Preterm born children are at risk of developing hypertension. MKV rather than size is an early useful screening parameter. Lower serum uromodulin correlates with lower GA and MKV, both of which can be useful markers for assessing risk of renal insufficiency.
|Table 1: Characteristics associated with hypothalamic-pituitary-adrenal axis|
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| O23 Inflammatory Markers in Children on Dialysis: Does the Modality of Dialysis Make a Difference?|| |
Radhika Chemmangattu Radhakrishnan, Susan Uthup
Department of Pediatric Nephrology, SAT Hospital, Government Medical College, Thiruvananthapuram, Kerala, India
Rationale: Patients with end-stage kidney disease (ESKD) and especially those on dialysis are burdened with a high inflammatory load leading to endothelial dysfunction which results in high cardiovascular morbidity and mortality. There are very few studies comparing inflammatory markers in children on peritoneal dialysis (PD) and hemodialysis (HD).
Objectives: The primary objective was to compare the levels of fibroblast growth factor 23 (FGF 23), high sensitivity C-reactive protein (hsCRP), and interleukin-6 (IL-6) in children with ESKD undergoing maintenance HD and PD. The secondary objective was to find the correlation between the inflammatory markers and between inflammatory markers and clinical/laboratory parameters.
Methods: This was a cross-sectional hospital-based study involving children 1–18 years of age with ESKD on maintenance HD or PD for at least 3 months at our hospital. The inflammatory marker levels were compared between the two groups as well as correlated with clinical and laboratory parameters.
Results: A total of 16 children were included in the study (10 on PD and 6 on HD). All the patients on PD were on continuous ambulatory PD. The mean hsCRP was 3.14 ± 0.59 mg/L in HD group and 3.28 ± 0.87 mg/L (P = 0.515) in PD group. The mean FGF 23 was 1.81 ± 0.37 pg/ml in HD group and 1.50 ± 0.56 pg/ml in PD group (P = 0.913). The mean IL-6 was 821.7 ± 137.9ng/ml in HD group and 794.6 ± 121.6 ng/ml in PD group (P = 0.233). IL-6 showed positive correlation with dialysis vintage (r = 0.509, P = 0.044). There was positive correlation between FGF23 and IL6 levels (r = 0.547, P = 0.028). Conclusions: There is no difference in inflammatory markers namely hsCRP, IL-6 and FGF-23 in children on HD compared to those on peritoneal dialysis. IL 6 levels are positively correlated with dialysis vintage and FGF23 levels.
| O24 Clinical Profile and Outcome of Young Infants Admitted with Hypernatremic Dehydration|| |
Priya Tanwar, Kanika Kapoor, Ajay Kumar, Sukanya Gangopadhyay, Rani Gera
Departments of Pediatrics and Biochemistry, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
Rationale: Hypernatremic dehydration (HND) is a potentially lethal condition in young infants, which can lead to serious sequelae if undiagnosed and untreated. The mortality rate varies from 10% to 66% with increasing severity of HND.
Objectives: To study the clinical profile and outcome of young infants admitted with HND. SUBJECTS AND METHODS.
Methods: A prospective observational study was conducted at a tertiary care teaching hospital over a period of 18 months. All out born sick young infants <2 months who presented to ED with symptoms and signs of possible sepsis and/or dehydration were screened and those with hypernatremia were enrolled in the study. Those infants born at <37 weeks of gestation and gross congenital anomaly were excluded. Hypernatremic dehydration (HND) was defined as serum sodium levels (Se Na+) >145 mEq/L and classified as mild if Se Na + were 146-–149 mEq/L, moderate if 150-–169 mEq/L and severe >170 mEq/L. Dehydration was defined by any 2 of the following signs: lethargy, sunken eyes, depressed anterior fontanelle, prolonged CFT, doughy skin, decreased urine output, weight loss >10% body-weightBW in <7 days old and or >5 % in a day. Variables used in the study were defined as per the standard definitions. Acute kidney injuryAKI was defined and staged using serum creatinine as per the modified neonatal KDIGO guidelines. Clinical presentation, laboratory parameters, and co-morbidities were compared among outcome groups (survived and died).
|Figure 1: Association between platelet count (lakhs) and severity of hypernatremic dehydration|
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|Table 1: Comparison of study variables among those who survived and those who died (n=55)|
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| O25 Clinical Presentation and Outcomes of Acute Severe Hypertension in Children with Acute Glomerulonephritis|| |
Himesh Barman, B. Shakthi, Hameed Uddin Admed, Raj Rabidas, Rosina Ksoo
Department of Paediatrics, NEIGRIHMS, Shillong, Meghalaya, India
Rationale: This study was done with an objective to study the outcome of children with acute glomerulonephritis (AGN) who presented with acute severe hypertension and to study the association of severity of hypertension with presence of end organ involvement. Methodology.
Methods: All children with Presenting with acute glomerulonephritis (AGN) with stage 2 hypertension were retrospectively identified and included. Those children who had stage 2 hypertension along with signs of end organ damage were categorised as hypertensive emergency and those without any sign of end organ damage were classified as urgency. Relevant clinic laboratory data were extracted by retrospective chart review.
Results: A total of 109 children qualified for inclusion. The mean age was 10.3 ± 4.4 and Male: Female ratio was 1.5:1. Out of these 73 (66.9%) presented with hypertensive urgency whereas 36 (33.1%) had hypertensive emergency. Among those presenting with hypertensive emergency, 91% had encephalopathy, 32% had retinopathy and 7.3% left ventricular failure. Mean blood pressure in hypertensive emergency group was significantly more than the urgency group. Children with hypertensive emergency tended to be older (9.8 + 4.4 vs. 11.5 + 4.1; P value < 0.05) and have higher mMean blood pressure (111.1 ±+ 10.9 vs. 119.7 ±+ 13.0; P value < 0.001). However, bBlood pressure normalized faster in hypertensive emergency group. (59.0 ±+ 42 vs. 89 ±+ 52.3 hours; P value< 0.001) compared to urgency group. However, excess mean blood pressure above 95% tile despite being high (21.1 ±+ 32.2 vs. 27.0 ±+ 12.0 mm of Hg; P value > 0.05) in emergency group did not meet statistical significance. Mean duration of hospitalization required was 12 days (4-–22). There was no Mortalitymortality.
Conclusions: Among children with AGNacute glomerulonephritis, children with hypertensive emergency tend have higher blood presser and tend to be older to children with hypertensive urgency. With proper treatment, outcome is good.
| O26 Diagnosis of Infection in Relapse of Nephrotic Syndrome: Development of a Personalized Decision-Analytic Nomogram and Incorporating Clinical Considerations into Statistical Model|| |
Soumya Tiwari, Y. Venkata Narayana, Viswas Chhapola, Ekta Debnath, Meenakshi Aggarwal, Anju Jain
Department of Pediatrics, Biochemistry and Microbiology, Lady Hardinge Medical College, New Delhi, India
Rationale: Infections associated with nephrotic relapses (NR) are often diagnosed and treated as per physician-preferences and instincts. A prediction tool to aid clinical decision-making would be valuable to avoid inappropriate use of antibiotics.
Objective: We aimed to develop a nomogram for the individualized prediction of the risk of infection based on common biomarkers and perform a decision curve analysis (DCA).
Methods: This prospective observational study included children (1–18 years) with NR. Children who were critically sick or had already received antibiotics were excluded. Infections were diagnosed using the standard clinical definitions and samples were obtained for total leucocyte count (TLC), absolute neutrophil count (ANC), quantitative C-reactive protein (qCRP), procalcitonin (PCT), and cultures (blood, urine, ascitic fluid). Logistic regression was used to identify biomarker-predictors of infection, and a probability nomogram was constructed. Model performance was checked using area under curve, Hosmer-Lameshaw statistic, Akaike information criterion, pseudo R2, and correct classification. DCA was done and net benefits calculated.
Results: We included 150 relapse episodes from 115 children (82 males, median age 66.5 months). Fifty-two children had clinically diagnosed bacterial infection. Uni-variate analysis showed PCT, TLC, ANC, and qCRP as the significant predictors of infection. On multivariate analysis, we tested two models: model-1: ANC + qCRP and model-2: TLC + qCRP. The performance of model-1 was superior to model-2, and qCRP or PCT based uni-variate models [Table 1]. Therefore, model-1 was chosen to develop the nomogram [Figure 1]a of predicted probabilities. DCA also revealed that model-1 was best for clinical decision making, and provided the maximum net benefit in the threshold probability range of 15%–60% when compared to CRP and PCT based models [Figure 1]b.
Conclusions: ANC and qCRP based nomogram can be conveniently used for precise visual prediction of the probability of infection in noncritically ill children with NR. Decision curves from this study will aid in decision-making of empirical antibiotic therapy, incorporating threshold probabilities as a surrogate of physician preference.
| O27 Age at Surgery Does not Affect the Outcome of Postnatally Diagnosed Posterior Urethral Valve: A Cross-Sectional Study|| |
Viswas Chhapola, M. Aditya, Soumya Tiwari, Ashna Kumar, Rajiv Chadha, Partap Singh Yadav, Subhasis Roy Choudhury
Departments of Pediatrics and 1Pediatric Surgery, Lady Hardinge Medical College, New Delhi, India
Rationale: Posterior urethral valve (PUV) is an important cause of congenital obstructive uropathy. The outcome of PUV may be different for boys who are diagnosed in the antenatal period compared to those diagnosed in the postnatal period.
Objective: We aimed to (1) know the profile of kidney function and urinary tract abnormalities in boys aged more than 3 years, who underwent PUV-fulguration after a postnatal diagnosis, and (2) compare these outcomes in boys who were operated before one year to those operated after 1 year of age.
Methods: The boys (>3 years) were included in the study, if, (1) they were diagnosed with PUV in their postnatal life, and (2) had a minimum of six months of follow-up after surgery. The outcome variables were estimated glomerular filtration rate, the proportion of boys with polyuria, and hypo-osmolality. The nadir serum creatinine, 24-hour urine volume (measured by bladder catheterization), and urine osmolality were noted.
Results: We enrolled 40 boys who had undergone either primary fulguration (n = 27), or primary vesicostomy (n = 13) followed later by fulguration [Table 1]. Fourteen boys had chronic kidney disease (≥CKD III), fifteen (37.5%) had polyuria, and nineteen (47.5%) had hypo-osmolar urine. Occurrence of polyuria (r = 0.03), hypo-osmolality (r = −0.17) and low GFR (r = −0.14) showed weak correlation with age. Seventy-nine percent of boys had renal scarring. A significant reduction (P = 0.031) of VUR was seen following surgery. Sixty-five percent boys had persistent moderate to severe hydronephrosis. About 50% had daytime incontinence. The nadir creatinine, CKD, polyuria, hypertension, proteinuria, residual VUR, scarring, change in the appearance of bladder outline, and incontinence were not significantly different in boys who were operated before one year of age compared to those operated at age ≥1 year [Table 2].
Conclusions: Boys with postnatally diagnosed PUV have a high prevalence of CKD, proteinuria, and hypertension. Polyuria and hypo-osmolar urine indicating nephrogenic diabetes insipidus are common. Most outcomes were not different with respect to the timing of surgery.
|Figure 1: (a) Nomogram for prediction of infection in children with relapse of nephrotic syndrome Physician instructions: Locate the scores for ANC and qCRP by drawing perpendicular lines from the respective values over the score line on the x-axis. Sum the scores for each predictor and locate this sum on the probability axis to find the likelihood of infection in a particular patient. In the example shown in the figure, ANC value of 5700 and CRP value of 13 correspond to scores of 1.9 and 1.1 respectively. Adding the two scores gives a total score of 3, which corresponds to 39% probability of infection. (b) Decision curves for ANC + qCRP (model-1), CRP, and PCT models. Threshold probability refers to the point at which a physician considers the benefit of treatment for infection equivalent to the harm of overtreatment for no-infection and thus reflects how the physician weights the benefits and harms associated with this decision. The highest curve at any given threshold probability is the optimal decision-making strategy to maximize net benefit. Net benefit was maximized with threshold probabilities of 1%–15% by the “treat all” approach; with threshold probabilities of 15%–60%, net benefit was maximized by the ANC + qCRP model. ANC: Absolute neutrophil count, qCRP: Quantitative C-reactive protein, PCT: Procalcitonin|
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| O28 Hyponatremia in Tubercular Meningitis: “The Fault Lies not in the Kidneys but in the Brain”|| |
Viswas Chhapola, Ivanderick Thongni, Soumya Tiwari, Pallavi Singh, Varinder Singh
Department of Pediatrics, Lady Hardinge Medical College, New Delhi, India
Rationale: The research on hyponatremia in children with tubercular meningitis (TBM) is limited. Most of the studiesin adults attribute hyponatremia mainly to the syndrome of inappropriate antidiuretic hormone secretion (SIADH) and cerebral salt wasting (CSW).
Objectives: We aimed to know the prevalence, aetiologies, predictors, and outcomes of hyponatremia in children with TBM.
Methods: Ours was a prospective observational study in children (up to 18 years) hospitalised with newly diagnosed TBM. Children with other known comorbidities causing hyponatremia were excluded. Serum sodium levels were checked once every 24–48 hours, and if hyponatremia was noticed further samples were sent for dextrose, serum sodium, potassium, uric acid, serum osmolality, urine osmolality, and spot urinary sodium. Assessment of fluid status and urine output was done. The aetiology was categorized as per “European clinical practice guideline on the diagnostic approach and treatment of hyponatremia algorithm” [Figure 1]. The primary outcome was percentage of children with TBM who develop hyponatremia during the hospital stay and relative percentage of different aetiologies.
Results: We included 92 children with TBM. Forty-seven (51.08%) developed hyponatremia (mild 27%, moderate 62%, severe 11%), 92% having euvolemic hyponatremia. SIADH (74.5%) was the most common aetiology, followed by osmotic agent-induced (13%), CSW (8.5%), and diuretics-induced hyponatremia. [Table 1] shows the clinical and laboratory data of children who developed hyponatremia. Being on oral feeds in the initial stages of hospitalisation (odds ratio [OR]: 7.04, P = 0.006), and low cerebrospinal fluid glucose (OR: 0.97, P = 0.009) were the only significant predictors of hyponatremia. Hyponatremic children had higher mortality (9 vs. 4); however, the difference was not statistically significant (P = 0.533).
|Table 2: Comparison of variables according to age at fulguration (≤1 vs. >1 year)|
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Conclusions: Moderate hyponatremia is common in TBM, most common aetiology being SIADH. Iatrogenic hyponatremia is often overlooked and misclassified. Treatment should be directed towards the specific aetiology as misdiagnosis may cause harm.
| O29 A Study of the Temporal Profile, Risk Factors and Outcomes of Catheter Associated Bloodstream Infections in Paediatric Hemodialysis patients|| |
Jovin Chris Antony, Georgie Mathew, R. V. Deepthi, V. Balaji1, Rani Diana Sahni1, Jeyaseelan2, Indira Agarwal
Department of Paediatrics Unit II, Division of Pediatric Nephrology, Christian Medical College, Departments of 1Microbiology and 2Biostatistics, Christian Medical College, Vellore, Tamil Nadu, India
Rationale: Catheter associated bloodstream infections (CABSIs) cause significant morbidity and mortality in patients undergoing short-term and long-term hemodialysis.
Objectives: To assess the temporal profile, risk factors and outcomes (mortality and morbidity patterns, antibiogram of the isolated pathogens) of CABSI in pediatrichemodialysis patients.
Methods: Study design: Observational study. Data of children, under 18 years of age, who underwent hemodialysis in a tertiary care pediatric hospital in South India, between January 2011 and December 2015 were collected retrospectively from the medical records. Various risk factors, temporal profile and outcomes of CABSI were analysed.
Results: During the 5-year time period, 166 children (61.5% boys) underwent HD catheter insertion; among these, 48 CABSIs were observed with an incidence rate of 12.5 infections per 1000 days. Mean time to infection from the insertion of catheter (temporal profile) was 13.6 days. Other than preexisting anemia (P = 0.049) and hemodialysis done in ward (P = 0.03; when compared to intensive care unit setting), no other significant risk factors could be identified regarding the incidence of CABSI. In patients with CABSI, hypotension was universal (p = 0.03) and CABSI resulted in catheter removal in 60.4% patients (34.4% with clinical symptoms). CABSI resulted in a significant prolongation of hospital stay by a mean 11.6 days (P and lt; 0.001), with total length of hospital stay being 24.6 days. Mortality rate of 18% was observed in the overall study population; and 14.5% in the CABSI group. No significant association between CABSI and mortality rates were observed. CABSIs were caused predominantly by nonfermenting Gram-negative bacilli; 33.3%, followed by coagulase negative Staphylococcus; 18.8% and Enterococcus; 16.7%.
Conclusions: CABSIs contributed to significant morbidity and longer hospital stay in pediatric hemodialysis patients. Aseptic precautions and an organized protocol for catheter care with constant re-training will help to decrease the incidence of CABSI.
|Table 1: Clinico-laboratory characteristics of children (n=47) who developed hyponatremia|
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| O30 To Understand the Psychological Effect of Long Term Nephrotic Treatment in Pediatrics Patients and Its Social Implications|| |
Nikhil Deo, Anubha Srivastva, Chitij Srivastva, Shahid Siddique, Mukesh Veer Singh
S.N. Children Hospital, M.L.N. Medical College, Prayagraj, Uttar Pradesh, India
Rationale: To understand the psychological effect of long term nephrotic treatment in pediatrics patients and it's social implications. Objectives: The study was to find out a short predesigned sheet including socio-demographic data sheet, clinical profile sheet, complication and social burden of disease, they seems to be depression, emotion, concentration, behavior, distress and psychogenic burden and outcome of patients with nephrotic syndrome (NS).
Subjects and Methods: A cross-sectional study carried out in Sarojani Naidu children hospital, Motilal Nehru Medical College, Prayagraj from 6-month period, a nonprobality Beck's depression Inventory (BDI-21), General Health Questionnaire (GHQ-12), Strengths and difficulties questionnaire.
Results: The majority of children were Hindu by religion (87%), male Preponderance (61%), locality wise 83% were rural, mostly caregiver were 66% father as relation with patients and most patients relationship with caregiver were son (51%). Majority of patients were URTI (49%) as precipitating factor present before on-set symptom of NS and most common symptom was periorbital swelling and pedal edema. Fifty-three percent patients were in primary School as education. Fifteen percent patients had school absenteeism were presents and 11% patients had concern from school. Forty-six percent patient had good response to prednisolone f/b 26% prednisolone with levamisole. Frequently relapsing NS (FRNS) was more common 33 Patients than INFRS (10%), most common complication was cushingoids (36%). BDI-21 scale, 14 out of 52, 11 patients (21%) were in borderline clinical depression and 3 (5.7%) modern had depression. GHQ-12 scale, 16 out of 52, 11 and five patients (9.6%) had score >15 and 20 respectively suggest having suggest distress and psychological distress respectively. SDQ-scale 42% children had difficulties in behavior, emotion and concentration interfere life. 32 children (76.2%) had minor difficulties and 7 children (16.7%) could had needed or not needed treatment and 3 children (7.1%) had severe difficulties.
Conclusions: Most common affected age group was 1–8 years with male prepondeeance 61%, hindu dominant 87%. Most relapse case were seen FRNS 33%, cross-sectional study suggest minor depression, psychological distress and presence of behavior problem in a significant proportion of children with NS.
| O31 A Study of Association of Lower Urinary Tract Dysfunction with Rectal Distension in Children|| |
Sangeeta, Jyoti Bagla, Jagdish Chandra, Smriti Rohtagi, Supriti Kohli
Departments of Pediatrics and 1Radiology, ESI PGIMSR, New Delhi, India
Rationale: Bladder and bowel dysfunction (BBD) is a term that covers lower urinary tract dysfunction (LUTD) and faecal elimination issues such as constipation and encopresis. LUTD includes overactive bladder, Underactive bladder, voiding postponement, dysfunctional voiding, extraordinary daytime urinary frequency and giggle incontinence etc.
Aims and Objectives: To study the association of LUTD with rectal distension and constipation in 5–15 years children.
Methods: This observational study was conducted in Department of Pediatrics from October 2019 to September 2021. All children with LUTD were enrolled and evaluated for constipation on basis of history, Rome III criteria, Bristol chart and rectal diameter on ultrasonography. Dysfunctional voiding symptom score (DVSS) was used to define the severity of LUTD. Response in various lower urinary tract symptoms (LUTS) i.e., storage symptoms (urgency, frequency, incontinence) voiding symptoms (hesitancy, straining, intermittency, giggle incontinence and dysuria) and holding maneuvers were assessed after 4 weeks of treatment for constipation and urotherapy. A sample size of 45 was calculated and enrolled. The data was analyzed using descriptive statistics.
Results: Mean age of subjects was 8.29 ± 2.1 years. Constipation was present in 22 (48.88%) patients. DVSS score ≥6 in females was in 86% and score ≥9 was in 31%. A higher DVSS is significantly associated with constipation i.e., 71.42% in males and 65% in females. Out of 45 cases, 8 had rectal distension. The mean value of rectal diameter in constipated children (25.55 ± 6.66) was significantly higher as compared to nonconstipated children (21.47 ± 5.15). Rectal distension was significantly associated with organic causes like hyrdonephrosis, reflux nephropathy (P = 0.002). The two most common LUTD were nocturnal enuresis in 64.44% and overactive bladder in 24.44%. A very good response to urotherapy and treatment for constipation was seen among various LUTS i.e., 82.76% to 100% except hesitancy (0%) and giggle incontinence (0%).
Conclusions: Children with LUTS should be evaluated for constipation and associated BBD, and if present, its treatment improves urinary symptoms in children with LUTD.
| O32 Serum Cystatin C as Early Predictor of Acute Kidney Injury in Preterm Neonates with Respiratory Distress Syndrome|| |
Jyoti Bagla, Rohan Kumar, Smriti Rohatgi, Lucky Manik, Sarika Arora1, Anand Prakash Dubey, Ruchi Mishra
Departments of Pediatrics and 1Biochemistry, ESI Hospital and PGIMSR, New Delhi, India
Rationale: Acute kidney injury (AKI) is associated with poor outcome in preterm neonates with respiratory distress syndrome (RDS), leading to focus on novel biomarkers for early diagnosis.
Objectives: To study diagnostic accuracy of serum cystatin C (sCysC) in early prediction of AKI in preterm newborns with RDS.
Methods: Prospective case control study in tertiary level teaching hospital in New Delhi (October 2019–April 2021). Total 90 preterms with gestation <37 weeks were enrolled and grouped as; case (n = 60) including preterms with RDS and control (n = 30) including healthy preterms. KDIGO classification was used for staging AKI. Serum creatinine (sCr; JAFFE method) and sCysC levels (nephlometric method) were measured at day 1, 3 and 7.Statistical Analysis was done by SPSS version 23.
Results: Mean gestational age and weight in cases was 33.87 ± 1.78 weeks and 1.68 ± 0.40 kg respectively compared to 35.10 ± 0.76 weeks and 2.01 ± 0.24 kg in controls. Amongst cases, 32 (53.3%) developed AKI (stage 1%–43.3%, stage 2%–8.3%, stage 3%-1.7%) in comparison to 6 (20%) in controls (all stage 1). Mean sCysC concentration (mg/l) in cases on day 1, 3, 7 was 2.39 ± 1.67, 2.62 ± 1.68, 3.07 ± 2.33 respectively (reference lab value 0.55–1.15). Mean sCysC levels (mg/l) were highest in stage 3 AKI (4.68 ± 2.82 in stage 1, 5.27 ± 2.41 in stage 2, 7.00 in stage 3). Receiver operatic characteristics for sCr and sCysC plotted on day 1, 3 and 7 to compare diagnostic performance of both. Area under curve was found to be 0.81 and 0.693 respectively (day 3). The sensitivity and specificity of sCysC on day 3 was 70% and 75% respectively (cut off 2.2 mg/l) compared to 87% sensitivity and 62% specificity of sCr (cut off 0.8mg/dl).
Conclusions: Serum cystatin C may be a good and reliable screening tool to predict AKI early in preterm neonates, in combination with sCr and other biomarkers.
| O33 Predictive Value of Risk Factors in Causing Acute Kidney Injury among Children with Nephrotic Syndrome|| |
Sanchari Ghosh1, Shakil Akhtar1, Subal Kumar Pradhan3, Subhankar Sarkar1, Deblina Dasgupta1, Ruhi Parween3, Shina Menon4, Rajiv Sinha1,2
Division of Pediatric Nephrology, Institute of Child Health, Kolkata, Apollo Gleneagles Hospital, Kolkata, West Bengal, SVPPGIP and SCB Medical College, Cuttack, Odisha, India, Seattle Children's Hospital, Seattle, USA
Rationale: Acute kidney injury (AKI) is an independent risk factor in increasing morbidity and mortality among hospitalised children. We hereby assess the predictive value of risk factors in causing AKI among children with nephrotic syndrome (NS).
Objectives: To formulate a predictive index for the risk of AKI (KDIGO definition) in children hospitalized with NS.
Methods: A prospective observational study was conducted from September 2020 to August 2021 in children (1–18 years) hospitalised with NS. Children with nephritic/secondary nephrotic features, preexisting chronic kidney disease, those admitted only for kidney biopsy/planned drug infusions/surgical procedure were excluded.
Results: Two hundred and sixty-five consecutive hospital admissions for idiopathic NS were screened. Two hundred admissions in 176 patients (63% female, median age 4 years [IQR: 3–7]) were included. AKI (KDIGO creatinine criteria) occurred in 18% (n = 36) admissions with none having AKI in repeat admissions. Fifty-three percent (n = 19) had AKI on day 1, 39% (n = 14) on day 3, 6% (n = 2) on day 5 and 1 (3%) on day 7 of admission. Multivariate analysis revealed the following risk factors to be significantly associated with AKI: FeNa% ≤0.2 (odds ratio [OR] 12.77; 95% confidence interval [CI] 3.5–46.4; P < 0.001), male gender (OR 6.38; 95% CI 2.76–14.74; P = 0.001), evidence of infection (OR 5.44; 95% CI 2.4 – 11.86; P = 0.03), exposure to other nephrotoxic drugs (OR 4.83; 95% CI 2.21 – 10.54; P < 0.001) and albumin ≤1.4g/dl [OR 4.35; 95% CI 1.55 – 12.8; P < 0.01, [Table 1]. In a subgroup analysisin children developing AKI after admission, we found following admission variables FeNa% ≤0.2, male gender, evidence of infection, nephrotoxic drug exposure and albumin ≤1.4g/dlto strongly predict subsequent development of AKI [Figure 1].
|Figure 1: ROC curve demonstrating accuracy of predictive equation including serum albumin ≤1.4 g/dl, FeNa ≤0.2%, male gender and evidence of infection (4a) and that of the predictive equation when FeNa ≤0.2% is excluded. ROC: Receiver operating characteristics|
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Conclusions: AKI is common in children hospitalized with NS. Male sex, serum albumin ≤1.4g/dl, FENa ≤0.2%, nephrotoxic drugs, and underlying infection correlated strongly with subsequent development of AKI and can be used in future studies to validate a “nephrotic renal angina index” to predict the risk of AKI.
| O34 To Study the Clinical Profile and Short-Term Outcome of Children Aged 5 to 18 Years Who had Enuresis at Presentation|| |
Chaudhari Nayan, Singhal Jyoti1, Sharma Jyoti1
Super Speciality Hospital, Amravati, 1KEM, Pune, Maharashtra, India
Rationale: Enuresis is defined as intermittent incontinence that occurs while a person is asleep. The term is used both as symptom and a diagnosis or condition. The aim of our study was to study the various etiologies in children with enuresis at presentation; to record, the prevalence of nonmonosymptomatic enuresis (NMNE) and their short-term outcome.
Objectives: To study the clinical profile and short-term outcome of children aged 5 to 18 years who had enuresis at presentation.
Methods: Clinical details of all patients aged 5 to 18 years enrolled with enuresis at presentation in the paediatric nephrology service over a period of 7 years were noted from case record forms, and course on follow up visits was recorded. We used all definitions and terminology as per the Standardization Committee of the International Children's Continence Society.
Results: Of the total 73 cases, enuresis was a symptom that led to the diagnosis of a congenital anomaly of the kidneys and urinary tract in 5 children (6.8%), diabetes mellitus and tubular disorder in 1 child (1.3%) each. Enuresis as a condition was seen in 65 (89.04%) children. It was primary in 49/65 (73.9%) and NMNE in 35/65 (53.9%). The most common comorbidity was constipation seen in 16/65 (24.6%) children. Details of subsequent course were available for 39 children. Improvement in children with MNE was seen by urotherapy alone in 15/16 (93.7%) and in NMNE by drugs 7/13 (53.8%). None of the children with MNE required pharmacotherapy.
Conclusions: Children presenting with the symptom of enuresis may have an underlying etiology that needs to be identified and addressed. Primary and NMNE are more common. A large majority of children improve with urotherapy alone.
| O35 Comparison of the Hypothalamic-Pituitary-Adrenal Axis Suppression between Single-Dose and Divided-Dose Prednisolone at Completion of 6 Weeks of Daily Steroid Therapy|| |
Tania Khan, Deblina Dasgupta, Prashanth Rajasekharan, Shakil Akhtar, Rajiv Sinha
Institute of Child Health, Kolkata, West Bengal, India
Rationale: Prednisolone is the standard therapy for first episode nephrotic syndrome (FENS). Early morning single dose (SD) has hypothetical advantage of less hypothalamic-pituitary-adrenal (HPA) axis suppression, but lack of robust evidence has resulted in variation in practice with divided dose (DD) prednisolone still commonly used.
Objectives: To compare HPA axis suppression between SD and DD prednisolone at completion of 6 weeks of daily steroid. Secondary objective included comparison of time to remission, time to first relapse, and frequency of relapse between SD and DD prednisolone.
Methods: Children with FENS were randomized (investigators were blinded to group assignment) 1:1 to receive prednisolone 2 mg/kg per day, either as SD or in two DD for 6 weeks, followed by single alternative daily dose of 1.5 mg/kg for 6 weeks. Short synacthen test (SST) was conducted at 6 weeks with HPA suppression defined as post ACTH cortisol <18 μmg/dl. After completion of 6 + 6 weeks steroid therapy, follow-up was continued for additional 180 days. Four children (SD = 1 and DD = 3) did not attend SST and were hence excluded from the analysis.
Conclusions: Among children with FENS, DD and SD prednisolone were equally effective in inducing remission with similar relapse rates, but SD had less HPA suppression and longer time to first relapse.
|Figure 1: First episode nephrotic syndrome: Single versus divided dose prednisolone|
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|Table 1: Comparison of baseline and outcome parameters between single dose and divided dose prednisolone groups|
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| O36 Assessment of Dietary Acid Load in Children with Chronic Kidney Disease Stages 2-5D-an Observational Study|| |
Madhileti Sravani, Sheeba Collins, Arpana Iyengar
Department of Pediatric Nephrology, St. John's Medical College Hospital, Bengaluru, Karnataka, India
Rationale: Dietary acid load (DAL) which reflects the balance between acid and alkali-inducing foods is a modifiable risk factor for metabolic acidosis in chronic kidney disease (CKD). With paucity of data in the Indian context, this cross-sectional study was undertaken.
Objectives: To estimate DAL and determine associated dietary factors in children with CKD2-5D.
Methods: Clinical profile, dietary assessment of energy, protein intake/deficits, and nutrient intake were computed for Indian foods from the United States Department of Agriculture in clinically stable children with CKD2-5D. DAL was estimated through potential-renal-acid-load (PRAL).
Results: Eighty-one children (mean age-122 ± 47 months, 69% boys, 39.5% on dialysis, 76.5% nonvegetarians, 30% on bicarbonate supplements) were studied. Positive PRAL (9.97 ± 7.66 meq/day) was observed in 74 (91.36%) children with comparable proportions in those with CKD 2–5 and 5D (90.1% vs. 93%, respectively, P > 0.05). On univariate analysis, protein intake and protein difference were observed to be significantly higher in the highest quartile as compared to lowest quartile of PRAL in CKD 2–5 (55 + 16g/day vs. 40+14g/day, P < 0.001) and in those with 5D (47 + 15g/day vs. 25 + 11g/day, P = 0.002). Higher calorie intake in the highest compared to the lowest quartile of PRAL was observed in those with 5D (1281 + 366Kcal/day vs. 875 + 320 Kcal/day, P = 0.03) but not in those with CKD 2–5 [1472 + 438Kcal/day vs. 1471 + 672 Kcal/day, P = 0.055, [Table 1]. Majority (74%) consumed highly acidic foods and limited formula: PRAL meq/day = (0.49 × protein-intake, g/day) + (0.037 × Po4−intake, mg/day) (0.02 × K+intake, mg/day)−(0.013 × Ca+2 intake, mg/day) − (0.027×Mg+2intake, mg/day). Positive dietary PRAL (>0) suggests acidic content and negative PRAL (<0) suggests alkali content. PRAL was stratified by quartiles for interpretation. Calorie, protein intake and their differences from reference values were calculated using KDOQI guidelines. Values of PRAL for Indian foods and Indian food pyramids for acid and alkali foods were created for reference [Figure 1].
|Table 1: Comparing dietary parameters and PRAL quartiles in children with chronic kidney disease stage 2-5D|
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Conclusions: In children with CKD2-5D, PRAL estimation revealed high DAL in the majority with a high consumption of acidic foods. These findings provide implications for appropriate dietary counseling to achieve negative PRAL.
| O37 To Assess Functional Capacity, Muscle Strength and Quality of Life in Children with Chronic Kidney Disease and Kidney Transplant Recipients at a Single Centre in Western India|| |
Himani Goswami, Harda Shah, Shahnenaz Kapadia, Kinnari Vala, Anshuman Saha
Department of Pediatric Physiotherapy and Pediatric Nephrology, IKDRC-ITS, GUTS Institute, Civil Hospital, Ahmedabad, Gujarat, India
Rationale: The relationship between functional capacity, muscle strength, quality of life and chronic kidney disease (CKD) has been scarcely studied in Indian children.
Objectives: This study aimed to assess the functional capacity, muscle strength and quality of life in children with CKD and kidney transplant recipients at a single centre in western India.
Methods: In a cross-sectional observational study, 60 children between 6 and 18 years of age (20 each with CKD stage 1–5, 5D and kidney transplant recipients) were included. The quality of life was measured by PEDSQL. Functional capacity was measured by six-minute walk test (6MWT) and muscle strength was assessed by hand grip strength (HGS) and time floor to stand (TFTS).
Results: Out of 60, 38 (63.3%) were male. Average age (mean ± standard deviation [SD]) in years was 12.54 ± 2.96. Average weight in kg (mean ± SD) were 27.27 ± 9.96, 29.02 ± 2.72 and 36.29 ± 11.12 for CKD 5D, CKD 1–5 and transplant groups respectively (P = 0.05). 6MWT in meter was significantly higher in transplant group (509.05 ± 43.37) followed by CKD 1–5 (465.9 ± 68.85) and CKD 5D (381.45 ± 50.88) (P < 0.00001). The HGS in kg was strongest in transplant group (19.75 ± 4.45) followed by CKD 1–5 (12.7 ± 3.85) and CKD 5D (10.4 ± 3.02) (P < 0.00001). TFTS in second was quickest in transplant group (7.68 ± 0.76) followed by CKD 1–5 (9.93 ± 1.77) and CKD 5D (10.36 ± 1.30) (P < 0.00001). There was a moderate positive correlation (r = 0.585) between 6MWT and estimated glomerular filtration rate (eGFR). HGS was strongly correlated with eGFR (r = 0.78). TFTS was negatively correlated with eGFR (r = −0.6). The self-reported PEDSQL score was highest for transplant group (93.22 ± 7.101) followed by CKD 1–5 (77.25 ± 5.14) and CKD 5D (55.64 ± 8.00) (P < 0.00001). The PEDSQL score positively correlated with eGFR (0.79).
Conclusions: Quality of life, functional capacity and muscle strength were worst in CKD 5D group. Kidney transplant recipients had best outcome in our cohort.
| O38 Clinico Pathological Profile of Pediatric Lupus Nephritis: ICH Kolkata Experience|| |
Debapoma Biswas, Priyankar Pal, Rajiv Sinha, Deblina Dasgupta
Department of Pediatric Rheumatology, Lupus Clinic, ICH, Kolkata, West Bengal, India
Rationale: Lupus nephritis (LN), with an overall incidence of about 70% in children, is the most important determinant of long-term prognosis in systemic lupus erythematosus.
Aims and Objectives: To study the clinicopathological profile and outcome of pediatric LN.
Methods: Cross-sectional observational study including children with biopsy proven LN, attending the lupus clinic at the Institute of Child Health Kolkata from January 2020 to October 2021.
Results: Total number = 60; female: male = 4:1; mean age at diagnosis: 10.6 ± 2.4 years. Duration of follow up: median 50 months (interquartile range [IQR]: 20–83 months). Most common extra-renal manifestation was mucocutaneous (90%) followed by hematological (48%). All 60 children had proteinuria >500 mg/day (median: 1327 mg/day; IQR: 862–2219), 54 (90%) had microscopic hematuria and 45 (75%) had estimated glomerular filtration rate (eGFR) <90 ml/min/1.73m3 (mean: 71.8 ± 23.2 ml/min/1.73m2). ANA was positive in all, antids DNA was positive in 38 (63%), C3 alone was low in 10% and with C4 in 82%. Anti-phospholipid antibody was positive in 18%. Proliferative LN was found in 51 (85%) with class 4 being the most prevalent (57%). Mycophenolate mofetil (MMF) was used for induction in most cases (29, 48%) followed by cyclophosphamide (CYC) (16, 27%). Rituximab was used in 10 (17%) as rescue agent. Complete remission was seen in 24 (44%) and partial remission in 22 (41%) at the end of one year. Mean time to CR: 8 ± 2months. Proteinuria at presentation was found to be significantly associated with remission at 1 year (P < 0.01). In children with proliferative LN, no significant difference was found in rates of remission between MMF and CYC. However, children receiving CYC had significantly worse eGFR at presentation.
Conclusions: Although a difficult disease to treat, early diagnosis and timely aggressive management leads to better response rates as seen in 85%. Efficacy of use of CYC vs MMF in paediatric LN needs further elaborative studies with larger sample size.
| O39 Vancouver Symptom Score for Dysfunctional Elimination Syndrome: Translation, Reliability-Testing and Validation of the Hindi Version|| |
Aishwarya Madaan, Soumya Tiwari, Viswas Chhapola
Department of Pediatrics, Lady Hardinge Medical College, New Delhi, India
Rationale: Voiding dysfunction (VD) is a common problem in school-age children and is commonly diagnosed using questionnaires. Vancouver symptom score for dysfunctional elimination syndrome (VSSDES) is a VD questionnaire and has been shown superior to other tools in both validity and reliability aspects. Apart from English the only other validated version is in Dutch language.
Objectives: To perform translation, reliability-testing, and validation of the Hindi version of the “VSSDES” in children aged 4–16 years with VD.
Methods: The VSSDES was translated into Hindi according to the standard translation protocol which included forward translations, synthesis of the translations, back-translation, independent reviews, harmonization, and pilot testing. One hundred and sixteen children (4–16 years) with symptoms suggestive of VD, and 106 healthy controls were recruited. We evaluated the Hindi-VSSDES for face validity, structural validity (factor analysis), construct validity, test-retest reliability, and internal-consistency reliability. Four prior hypotheses were formulated to assess construct validity. These hypotheses were based on convergent validity, discriminative validity, and subgroup analysis (criterion: ≥75% hypothesis accepted). The study methodology complied with COSMIN recommendations.
Results: The Hindi-VSSDES [Figure 1] had acceptable face validity and construct validity (fulfilled all 4 predefined hypotheses), excellent test-retest reliability (ICC = 0.96), and good internal consistency (Cronbach's alpha = 0.62) reliability. The factor analysis identified 4 factors related to three domains [Table 1]. The cut-off scores for screening and diagnosis of VD were 7 and 11 respectively. The smallest detectable change was 4.1 points. Scores were comparable if children self-filled the questionnaire or parents were the proxy. Scores in girls were significantly higher than boys. The average time to fill up the questionnaire was 6.54 ± 1.16 minutes. The questionnaire could be filled with ease by 91% population.
|Figure 1: Hindi version of “Vancouver symptom score for dysfunctional elimination syndrome”|
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|Table 1: The assessment of various parameters of English, Dutch and Hindi versions of Vancouver symptom score for dysfunctional elimination syndrome|
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Conclusions: The Hindi translated version of VSSDES is valid, reliable, easy to administer and can be used in clinical practice for screening and diagnosis of VD disorders. It is yet to be tested for responsiveness and interpretability.
| O40 Nephrolithiasis/Nephrocalcinosis in Children in a High Prevalence Region of Rajasthan: Clinical, Etiological and Genetic Profile|| |
Vivek Parihar, Aliza Mittal
Department of Pediatrics, AIIMS, Jodhpur, Rajasthan, India
Rationale: Incidence of nephrolithiasis and nephrocalcinosis in children and adolescents has significantly risen over past few years. Rajasthan is considered under stone belt of India.Approximately 50%–84% children have an underlying metabolic or genetic or systemic cause for kidney stone disease. Children with underlying disorder have 50% chances of recurrence. Mutations in atleast 30 genes have been found to cause monogenic form of nephrolithiasis. Identification of genetic cause help in formulating best suited treatment plan to prevent recurrence or end-stage renal disease.
Objectives: To study Clinical, etiological and genetic profile of children presenting with Nephrolithiasis/Nephrocalcinosis in a high prevalence region of Rajasthan.
Methods: This study is a hospital based Prospective and retrospective observational study conducted in the department of pediatrics of a tertiary care hospital in western Rajasthan from October 2021 to September 2022. Any child <18 years presenting with nephrolithiasis/ nephrocalcinosis were enrolled after taking written informed consent. Necessary investigations of blood, urine and imaging were done. Clinical exome of the patient was sent only after consent from parents wherever indicated. Clinical, etiological and genetic profile of eligible enrolled children was studied.
Results: Total 29 children were enrolled over 1 year. Fourteen percent (n = 4) patients presented with UTI. Four patients presented with crystalluria. Forty-one percent (n = 12) patients were growth retarded and 14% (n = 4) had developmental delay. None of the patients had history of constipation but 14% (n = 4) patients had faecal loaded bowel loops on x ray. Rickets was present in 2 cases. Final diagnosis was confirmed in 21 patients. The most common identified aetiology was Idiopathic hypercalciurea (38%, n = 8). Distal renal tubular acidosis (RTA) is suspected aetiology in five patients out of which two patients subsequently are proven by genetics. Stones secondary to CAKUT were identified in two patients. Calcium oxalate was the most common stone retrieved. One child had pure uric acid stones. Clinical exome was sent in 10 patients. Genetics helped identify etiology in two patients of distal RTA. Genetics did not identify any known stone related genetic disease where phenotyping was in conclusive.
| O41 Genetic Defects in Patients with Congenital and Infantile Nephrotic Syndrome: Results from a Registry in Western India|| |
J. Sharma, A. Saha1, A. Ohri2, F. Shah3, P. Bhansali4, K. Sathe5, M. Matnani6, J. Dave7, B. P. Jain8,9, V. More10, P. Chhajed11, P. Deore12, C. Shah, V. Kinnari1, J. Singhal, N. Krishnamurthy13, M. Agarwal14, U. Ali13
KEM Hospital, Jehangir Hospital, KEM Hospital Pune, Wadia Hospital for Children, 5Department of Pediatrics, Sir H. N. Reliance Foundation Hospital and Research Centre, Namaha Hospital, Thunga Hospital, Tara Children's Kidney Care, Wockhardt Hospital and SRCC Children's Hospital, The Children's Hospital, 12Division of Nephrology, B. J. Wadia Hospital for Children, SRCC Children's Hospital, Mumbai, 4Orchid Pediatric Superspeciality Clinic, Aurangabad, Maharashtra, 1IKDRC-ITS-GUTS, Ahmedabad, 3Child's Kidney Care Center, Surat, Gujarat, 7Pediatric Nephrology, Vadodara, Gujarat, India
Rationale: Eighty-five percent of infants with congenital nephrotic syndrome (CNS, onset of NS at age <3 months) and 66% of infantile NS (INS, onset between 3 months to 1 year) are likely to have a monogenic etiology. As for all genetic disorders, there exists a significant genetic variability between different ethnic groups. This study aimed to determine the genetic defects in patients with CNS and INS by establishing a registry in Western India.
Methods: In this multicenter, cross-sectional study, pediatric nephrologists from 13 private and government institutions shared relevant clinical data and details of genetic evaluation of children presenting with NS within the first year of life. All patients but one (who underwent targeted sequencing for the WT1 gene) underwent large next generation panel testing for genes implicated in CNS/INS.
Results: The median age at presentation to the pediatric nephrology center was 9 months (range 1–23, interquartile range 3–13 months), history of consanguinity between parents existed in 14 (34%), family history of similar illness in 6 (15%), extra-renal manifestations in 17 (41%). Twenty-five (61%) were confirmed to have a monogenic etiology [Table 1]. The NPHS1 gene was the most commonly implicated (9/25) followed by PLCE1 (5/25). No definite genetic abnormality was found in 4 (25%) cases. Twelve variants of uncertain significance (VUS) involving 10 genes (10/25, 40%). A re analysis of these VUS attempted after 2–3 years of initial identification facilitated reclassification of 7/12 (58%); 3 possibly pathogenic, 3 possibly benign and one (in DSTYK gene) was discarded; increasing the diagnostic yield from 61% to 68.2%.
|Table 1: Genetic etiology of congenital and infantile nephrotic syndrome: comparison with other studies|
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Conclusions: Comparison of our data with similar studies reveals, that though variants in NPHS1 gene were the most common cause of NS in infancy, PLCE1 and NUP93 were implicated more frequently, NPHS2 conspicuous by its absence. Reclassification of VUS should be attempted, if feasible, since it may lead to a useful revision of diagnosis.
| O42 Kidney Morphometrics, and Centile Nomograms in Underweight Children|| |
Pallavi Singh, ViswasChhapola, Soumya Tiwari, Pooja Abbey1, Praveen Kumar, Abhinav Tyagi2
Department of Pediatrics and Radiodiagnosis, Lady Hardinge Medical College, New Delhi, India, University College Dublin, Dublin, Ireland
Rationale: Undernourished children are said to have smaller kidneys. Further research is required to understand the correlation of kidney size parameters with anthropometry and develop nomograms which are applicable in underweight children.
Objective: To measure kidney morphometric parameters and develop centile curves in underweight children.
Methods: It was a cross-sectional study on 152 (Males: 76) clinically stable children (6–59 months) with weight-for-age <−2 standard deviation. Children with–known kidney diseases or malformations, inborn errors of metabolism, history of prematurity or IUGR, urinary tract infections, tuberculosis and HIV-infection were excluded. Ultrasonography was performed in right and left lateral decubitus position. Kidney length, width, and depth were measured and volume was calculated using formula of ellipsoid. Kidney size parameters were compared with normative data from healthy Indian children. Correlations were checked, and quantile regression analysis done to identify significant predictors of kidney length and volume. Significant predictors were used to develop centile curves using Cole's Lambda-Mu-Sigma method.
Results: The pilot study (n = 21) revealed excellent interrater reliability (ICC 0.98 [95% CI: 0.98, 0.99]) between the two radiologists performing the ultrasound. Kidney length and volume were significantly smaller than normative values for healthy children. Kidney size was comparable between the two genders (P > 0.69), and in stunted versus nonstunted children (P > 0.05). Left kidney was significantly longer (P < 0.001). Kidney length (r: 0.6–0.63) had higher correlation with various anthropometric parameters than volume (r: 0.43–0.57). Age and weight-for-age z-scores were significant predictors of kidney size on univariate quantile regression. Multivariate analysis revealed that age was the only significant predictor (P < 0.001). Therefore, we constructed age-based centile curves of length and volume of right and left kidneys [Figure 1] and [Table 1].
|Table 1: Age based centiles chart of right renal length in underweight children|
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Conclusions: This study reiterates that underweight children have smaller kidneys. The nomograms derived from healthy children therefore cannot be used in these children. This is the first study to provide age-based centile curves for kidney size for use in underweight children.
| O43 Exploring the Utility of Telemedicine for Pediatric Nephrology at AIIMS Jodhpur: A Study of Patient Outcomes, Satisfaction and Compliance|| |
Vishnu Dev, Aliza Mittal, Kuldeep Singh, Siyaram Didel, Puneet Pareek, Sanjeev Misra
Department of Pediatrics, AIIMS, Jodhpur, Rajasthan, India
Rationale: During the COVID-19 pandemic, we adopted the provision of healthcare services through telemedicine. It is postulated that telehealth may be a good means of follow-up for patients with chronic diseases. This study aimed to evaluate patient and caregiver satisfaction with telemedicine service and determine the factors for poor patient satisfaction. We also assessed the compliance of patients and the monetary benefits of telemedicine.
Methods: Patients of the age group, 1 month to 18 years, who had a telemedicine consultation for renal-related disease from March 2021 to December 2021, were enrolled and excluded those on dialysis. Details of all patients who met the inclusion criteria were obtained from the Hospital information system every week. Patients were enrolled and followed up after one month from their first telemedicine consultation. We assessed patient satisfaction using a standard questionnaire-telemedicine utility questionnaire-total and subscale scores were calculated and classified as positive, neutral, and negative, based on responses on a 7-point Likert scale (1–7). Compliance of the patients to treatment was assessed using a questionnaire used by Ramay et al. in CKD children. This questionnaire contained 20 questions. Points were allotted from 1 to 5 for 17 questions and 0–2 for 1, and 0–1 for two questions. Total points were calculated, and adherence was represented as a percentage. The cost analysis of telemedicine was also assessed in terms of perceived cost savings by patients.
Results: Ninety-eight patients were enrolled as per inclusion criteria. The most common diagnosis was nephrotic syndrome (56%). 59.2% of the patients had a telemedicine consultation before enrollment. The median telehealth usability questionnaire score was 6 (Range: 1–7). During subscales evaluation, 89% of patients gave a positive response for components related to the use of telemedicine. (Maximum score was for “Telehealth saves me time traveling to a hospital or specialist clinic” - 100% agreed), the median score of 6. Reliability of telemedicine consultation received the lowest scores. (27.5% agreed to reliability, 65.6%-neutral response, and 12.5%-Not reliable mode of consultation). The system gave error messages that told me how to fix problems and received no positive scores (88.7% neutral, 11.3% negative response) followed by a component on “Visits provided over telehealth system are the same as in-person visit” received the most negative scores among all reliability components. (17.5% negative score, 18.6 neutral and 63.9% positive). Overall compliance of the patients was good, with mean compliance of 93%, median adherence of 96.6%, and the lowest was 73%. For single straight visit the total expenditure including lost wages of all patients was Rs 138,650 (food and transport-Rs. 106,270 [patients-Rs. 17,915, attendants-Rs. 88,355], lost wages-32,400). Multiple telemedicine visits incurred a total benefit of approx Rs. 345,390 (approx-Rs. 4000/patient over 6 months).
Conclusions: Our results suggest that most of our patients were satisfied with our telehealth services, excluding technical issues. However, telehealth services' reliability is not perceived to be as good. There are technical errors with the system, which most respondents were unsure if they could deal with. Even without a straight visit, there is reasonable compliance. Saving time and cost was a significant factor that contributed to good satisfaction with telehealth services.
|Figure 1: Age-based centile curves of right and left kidney length and volume in underweight children|
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| O44 Our Experience with Kidney Transplant in a Pediatric Chronic Kidney Disease Population from Indore, Central India|| |
Apollo Hospitals, Indore, Madhya Pradesh, India
Rationale: To analyze our experience with kidney transplant in pediatric chronic kidney disease (CKD) population from June 2016 to August 28, 2018 in Apollo Hospitals, Indore in Central India.
Design: Retrospective Study.
Methods: At our center, we performed 9 kidney transplants in children below 18 years of age from June 2016 to August 28, 2022. Mean age was 13.1 Year with Youngest was 7 Year and oldest 18 year. Ratio of male female was 2:1. Etiology of CKD showed Reflux Nephropathy in 44.4% (4/9); Glomerular disease in 30% (3/9) and congenital anomalies of the kidney and urinary tract in 22.2% (2/9). Two patients out of 9 (22.2%) underwent Preemptive kidney transplant. Mean duration from start of dialysis to transplant in seven patients was 124 days. All were live related kidney transplants with grandparents as donor in 30% (3/9); Mother in 44.4% (4/9); and father in 22.2% (2/9). Induction with Simulect was given in 22.2% (2/9); Grafalon in 22.2% (2/9); and all patients received Methyl Prednisone for 3 days staring from date of transplant at dose 15 mg/kg/day. Tacrolimus and Mycophenolate was started 2 days prior to transplant at dose with Tacrolimus 0.2 mg/Kg/day and mycophenolate mofetil (MMF) 1200 mg/m2/day in 2 divided doses.
Results: Follow up ranged from 1 month to 72 months with Mean 36 months. Postoperative: Mean postoperative stay in KTU was 7.1 day. Postoperative complications-In 1st month, Posterior Reversible encephalopathy Syndrome was seen in 2/9 patients in 1st month which improved with switch over from Tacrolimus to Cyclosporine and leveracetam. Two patients developed urinary tract infection which was treated appropriately. In first 3 months, CMV infection was seen in 11.1% (1/9) patient who received Grafalon as induction which was treated with Valgancyclovir and reduction in MMF dose. At 1 year, 11.1% (1/9) patient developed uncontrolled hypertension which improved well by switching over from CNI to Everolimus. During COVID pandemic, 33.3% (3/9) patients developed COVID infection 2 required hospitalization and 1 died with function graft. Mean creatinine was at Discharge 0.71 mg %, at 3 month 0.86 mg % and at 1 Year 1.1 mg %. Kidney Biopsy done in two patients both showed Transplant Glomerulopathy 1 year Graft and patient survival is 100%. Five year Graft survival is 100%, patient survival is 88% as one died with COVID Pneumonia with Functioning graft at 3 year Posttransplant.
Conclusions: Kidney transplant in children in Central India in Tier 2 cities have shown acceptable results compared to elsewhere in India.
| O45 Hypothyroidism in Chronic Kidney Disease: An Unrecognized Entity in Children|| |
Aditi Das, Abhijeet Saha, Rachita Singh Dhull
LHMC and Kalawati Saran Children's Hospital, New Delhi, India
Rationale: Thyroid status of children with chronic kidney disease (CKD) is not well studied and data is scarce. In overt hypothyroidism there is reduced serum-free T4 (FT4) and raised thyroid stimulating hormone (TSH) levels. Subclinical hypothyroidism (SCH) is associated with raised serum TSH levels and normal FT4.
Objectives: To evaluate the prevalence of hypothyroidism in children with CKD and the relationship between hypothyroidism and degree of proteinuria.
Methods: Observational cross-sectional study, conducted in a tertiary care teaching hospital, north India from January 2020 to January 2022. The data of the patients (1 years - 18 years) with diagnosed cases of CKD (stages 2–5), who visited multi-disciplinary CKD clinic were reviewed. We recorded anthropometric measurements, baseline laboratory investigations including spot urine protein creatinine ratio (UP: UC), thyroid function test (electrochemiluminescence method), and antiTPO (thyroid peroxidase) antibody level. SPSS version 23.0 was used for analysis.
Results: Fifty cases were enrolled. The mean age of presentation was 102 ± 53 months), and 76% were boys. [Table 1] Overall, 13 cases (26%) had hypothyroidism. The prevalence of hypothyroid cases increased as CKD stages advanced (23% of hypothyroid cases [3/13] in CKD stage 2–3 vs. 73% of hypothyroid cases [10/13] in CKD stage 4–5). No hypothyroid case was found when spot UP: UC was <0.2. Higher UP: UC was associated with an increased prevalence of hypothyroidism. CKD cases in sub-nephrotic proteinuria group, 20% had hypothyroidism, while in nephrotic-range proteinuria group this proportion was 39.1%. The Pearson correlation between TSH and spot UP: UC in overt hypothyroid cases was moderate positive (r = 0.56).
|Table 1: Demographic, clinical, baseline laboratory parameters of all patients|
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Conclusions: We observed that 26% children with CKD had hypothyroidism (subclinical or overt) especially in the presence of higher degree of proteinuria. Large multicentric studies are necessary to establish the correlation.
| O46 Serum Metabolomic Profile in Children with Sepsis Associated Acute Kidney Injury|| |
Nisha Chaudhary, Praveen Singh, Abhijeet Saha, Virendra Kumar, Shantanu Sengupta
Lady Hardinge Medical College and Associated Kalawati Saran Children's Hospital, Institute of Genomics and Integrative Biology, CSIR, New Delhi, India
Rationale: Sepsis associated acute kidney injury (SA-AKI) is associated with increased mortality and pooroutcome. Metabolomics can be used to discover novel pathways and metabolites which can detect AKI at an early stage and also serve as therapeutic targets.
Objectives: To identify the differentially expressed serum metabolites in children aged 1–18 years with SA-AKI compared to controls.
Methods: Fifteen children each with stage-1, 2 and 3 AKI respectively (n = 45), 15 children with sepsis without AKI and 15 healthy children were enrolled. Sepsis was defined by Surviving sepsis guidelines and AKI by KDIGO guidelines. Two millilitre serum sample in fasting state was collected and centrifuged. Supernatant was stored at −80°c and subjected to metabolomic analysis using liquid chromatography-mass spectroscopy. C-18 and HILIC (hydrophilic liquid interaction) chromatography were done in both positive and negative polarity. Data was analysed using appropriate statistical tests.
Results: We discovered novel metabolites belonging to glycerophosphocholine and glycerophosphoethanolamine sub-group of glycerophospholipid class of lipids in children with SA-AKI. Levels of 1-Palmitoyl-2-hydroxy-sn-glycero-3-phosphoethanolamine and 1-Oleoyl-sn-glycero-3-phosphoethanolamine were significantly upregulated in SA-AKI patients and their levels increased with stage of AKI as compared to healthy controls and patients with sepsis without AKI. Levels of 1-Palmitoyl-2-hydroxy-sn-glycero-3-phosphoethanolamine, 1-Oleoyl-sn-glycero-3phosphoethanolamine, 1-Stearoyl-2-hydroxy-sn-glycero-3-phosphoethanolamine and 1-(5Z, 8Z, 11Z, 14Z-Eicosatetraenoyl)-sn-glycero-3 - phosphoethanolamine were upregulated in severe AKI (Stage 2 and 3) as compared to healthy as well as sepsis controls whereas levels of decylbenzenesulfonic acid was significantly downregulated [Table 1]. Glycerophospholipid, linoleic acid and alpha linolenic acid pathways were significantly altered in stage 3 AKI patients [Figure 1]. Most metabolites were discovered in C-18 mode and were hence nonpolar. A large number of discovered metabolites have not been annotated till date and are presented by their m/z (mass/charge) values and will be presented in conference.
Conclusions: Serum metabolomics is a safe and accurate technology for discovery of early differentially expressed biomarkers in SA-AKI which can aid in early detection and therapeutics of SA-AKI.
| O47 Effect of Rituximab in Childhood Onset Steroid-Dependent Nephrotic Syndrome in a Tertiary Care Teaching Hospital|| |
Adarsh S. Kumar, H. R. Niyas, Susan Uthup1, Christy Cathreen Thomas1, C. R. Radhika1
Departments of Pediatrics, and 1Pediatric Nephrology, SAT Hospital, Government Medical College, Thiruvananthapuram, Kerala, India
Rationale: A high proportion of children with idiopathic nephrotic syndrome have steroid dependence (SDNS) with a relapsing course, drug toxicity and multiple infections. Rituximab, a chimeric monoclonal anti-CD20 antibody is documented as a steroid sparing agent that helps drug withdrawal and improved growth. As the data in our part of population is scarce, we planned to study the same in children with SDNS.
Objectives: Primary objective to assess effect of rituximab in inducing sustained remission in SDNS. Secondary objectives to assess effect of rituximab in reducing immunosuppressants. O compare cumulative steroid dose, growth velocity and side effects pre and post Rituximab.
Methods: Anambiceptive study was conducted in a tertiary care teaching hospital in children between 1 and 18 years with SDNS, who had received rituximab two doses, (375 mg/m2) as per standard protocol during a 2-year period from January 2017. Secondary/Congenital NS and estimated glomerular filtration rate (eGFR) ≤60 ml/mt were excluded. Demographic and clinical profiles were noted. One year follow up was done to assess for sustained remission, relapses, cumulative steroid dose, alternate immunosuppressants, growth, body mass index and eGFR. Categorical and quantitative variables were expressed as frequency and mean ± SD respectively. Paired t-test to compare quantitative parameters between time periods.
Results: Thirty-six patients with were enrolled with a mean age of 11.8 ± 4.1 years with male preponderance. Thirty-four patients had a renal biopsy diagnosis and 78% had minimal change disease. After rituximab therapy remission was sustained in 69%. Number of relapses/patient/year reduced from 3 (2, 3.75) to 0 (0, 1) post rituximab. Forty-two percent were withdrawn from immunosuppressants at 1-year postrituximab. Mean cumulative steroid dose was 224.5 ± 133 mg/kg and 43 ± 47.2 mg/kg pre and post rituximab respectively (P < 0.01). Mean height velocity was 2.3cm/year prerituximab as compared to 7.5cm/year postrituximab with increment in height velocity of 5.2cm (P < 0.01). There was significant reduction in obesity (61% pre-vs. 25% post) and antihypertensive drug requirement.
Conclusions: Rituximab induced sustained remission in SDNS. There was significant reduction in relapses, immunosuppressant requirement, cumulative steroid dose, obesity improvement in height velocity.
| O48 Lupus Nephritis in Children: Bangladesh Perspective|| |
Afroza Begum, Sabina Sultana, Amina Akter, Sharmin Sultana, Shanjida Sharmim, Samina Masud, Shamsun Nahar, Abdullah Mamun, Tahmina Jesmin, Saimul Haque, Ranjit Roy
Department of Paediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
Rationale: Renal involvement is the most important prognostic factor of Systemic Lupus Erythematosus (SLE). Renal involvement, its severity, treatment facilities and outcome varies in different countries. As there is very few data on this regard from this region, we prospectively studied the children with Lupus Nephritis (LN) who were admitted or visited in the out-patient department of the Paediatric Nephrology of Bangabandhu Sheikh Mujib Medical University.
Methods: We prospectively evaluated 89 children with LN in our department from January 2017 to July 2022. All patients fulfilled the SLICC criteria (2012) for the diagnosis of SLE and had evidence of renal involvement. Renal biopsy was done in 81 (91.01%) patients. Renal histology was classified according to ISN-2003 criteria. Immunosuppressive agents were given according to histological grading and clinical criteria. Patients were followed up for at least 1 year.
Results: Mean age of presentation was 12.11 ± 3.645 years and male to female ratio was 1:3. Fifty eight percent patient was from rural area and 72% parents had monthly income below 20,000 taka/month (198 US dollar). Significant proteinuria, haematuria, hypertension and impaired renal function was present in 65%, 53%, 56.7% and 40% patients respectively. Histopathological evaluation showed class IV (52%), class III (21%), class V (12%) and class II (5%). Inadequate sample was obtained in 5% cases. After one year follow up 51% patient had complete remission, 17% partial remission. Four patient (4%) developed end stage renal disease (ESRD), and 15% patient were lost to follow up. Death occurred in eight patients (9.0%) due to neuropsychiatric disease, septicaemia and COVID-19 infection.
Conclusions: Majority of the patients with LN were from low socio-economic status and from rural area. Proteinuria and hypertension were the main presenting feature. Class IV nephritis was the main histopathology. Response to immunosuppressive therapy was high. A great number of patients were lost to follow-up.
|Figure 1: Box whisper plots showing the no. of relpases, height velocity and cumulative steroid dose 1 year before and after giving rituximab in children with steroid dependent nephrotic syndrome|
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|Table 1: Percentage distribution of various parameters considered in the study|
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| O49 Genetic Profile of Children with Steroid Resistant Nephrotic Syndrome-A Multicentric Study from Western India|| |
U. S. Ali, J. Sharma1, M. Matnani2, P. Bhansali3, V. More, K. Sathe4, H. Vora5, P. Deshpande6, J. Singhal1, N. Krishnamurthy, N. Pandey1, P. Chhajed7, P. Inamdar8, V. Keskar9, F. Shah10, P. Deore11, K. P. Mehta12
NH-SRCC Children's Hospital, H. N. Reliance Foundation Hospital, Global Hospitals, P. D. Hinduja Hospital, The Children's Hospital, DY Patil Medical College, Fortis Hospital, Jaslok Hospital, Mumbai, KEM Hospital, Jehangir Hospital, Pune, 3Orchid Super Specialty Clinic, Aurangabad, Nelson Hospital, Nashik, Maharashtra, 10Child's Kidney Care Centre, Surat, Gujarat, India
Rationale: Genetic mutations affecting podocyte structure and function contribute to 20% to 30% of steroid resistant nephrotic syndrome (SRNS) in children. Due to paucity of studies in India we undertook this pilot study to identify the frequency and identity of the genetic mutations in Indian children from Western India.
Methods: Data of children with SRNS who had undergone genetic studies was obtained from medical records available with 13 participating centres and included clinical, histopathological findings and results of genetic studies obtained by next generation or whole exome sequencing, Categorical data is expressed as percentages and the association of variables in groups were compared using the Chi-Square test; P < 0.05 was considered significant.
Results: Sixty-seven children (1–18 years), 35 boys and 32 girls were included. Pathogenic or likely pathogenic mutations in a gene known to cause NS was seen in 18 (26.9%). Genetic mutations were seen in 15/47 (32%) children <5 years and in 3/20 (15%) children >5 years, equally in boys and girls. The commonest mutation was in NPHS2-gene in 8 cases, WT1-mutations in 3 (all females), COL4A5 in 2 (all boys) and 1 each of NPHS1, PLCE1, ACTN4, MYOEI mutation. One patient with an IPEX syndrome had a FOXP3 mutation. Two children had novel variants. Variants of uncertain significance were seen in 18. Seven were in chronic kidney disease 3–5. FSGS was commoner in children with a genetic etiology15/18 (83%) when compared to nongenetic etiology 25/49 (51%) (P < 0.05).
Conclusions: The frequency of genetic mutations (26.9%) is similar to those reported in other studies and was twice as common in younger children compared to those above 5 years. NPHS2-mutations were the commonest seen in 44% followed by WT1 mutations. COL4A5 mutations were seen in 2 but not as common as reported in Eastern India, suggesting genetic heterogeneity in different regions of India.
| O50 Comparision of Readings of Mercury Sphygmomanometer with 2 Nonmercury Blood Pressure Apparatus; Aneroid and Digital Sphygmomanometer in Normal Children and with Kidney Disease|| |
K. R. Arpitha, Sudha Ekambaram
Department of Paediatric Nephrology, Dr. Mehta's Multispeciality Hospital, Chennai, Tamil Nadu, India
Introduction: Increasing prevalence of hypertension (HTN) in children and adolescents is a significant public health issue and remains undetected unless routinely screened. The HTN prevalence in children is 0.96%–11.4%.
Rationale: Early recognition with routine blood pressure (BP) measurement is the key to diagnose HTN. The mercury sphygmomanometer has been the gold standard for BP measurement but is impractical to be used in community setting. Aneroid and digital manometers portability are higher compared to mercury device, but the reliability is still in doubt.
Objective: To compare readings of mercury sphygmomanometer with 2 nonmercury BP apparatus; aneroid and digital sphygmomanometer in normal children and with kidney disease.
Methods: Consented children aged 5–18 years were included and the participants were seated for 5 mins and BP measured with an appropriate cuff on the right upper arm, with the back supported and with both feet on the floor and arm at heart level. BP was measured by mercury, aneroid and then oscillatory methods respectively for all children.
Results: A total of 118 children were enrolled and the mean age is 9.8 years. The most common diagnosis is nephrotic syndrome-38.9% (46/118) followed by congenital anomalies of the kidney and urinary tract-23.72% (28/118). The prevalence of overweight and obesity in our study is 6.7% (8/118) and 1.69% (2/118) respectively. There is no significant difference in mean systolic BP (SBP) measured by all 3 apparatus among the study participants (P = 0.54) However, there is a statistically significant difference in mean diastolic BP (DBP) by all 3 apparatus (P = 0.05) and DBP by mercury and aneroid are comparable but not with mercury and digital.
Conclusions: The SBP measurements are reliable by all 3 methods whereas the DBP measurements are not comparable especially by digital method. So, more studies are needed to assess the reliability and accuracy of aneroid and digital devices.
| O51 Culture Positive Urinary Tract Infections in Children: Clinical Profile and Adequacy of Radiological Tests|| |
Sachin George, R. V. Deepthi, Georgie Matthew
Christian Medical College, Vellore, Tamil Nadu, India
Rationale: Urinary tract infections (UTIs) are common in children and have the risk of renal scarring. A retrospective study was carried out to analyse the clinical profile of children below 18 months of age with culture positive UTI and adequacy of radiological investigations.
Objectives: Primary objective was to assess the clinical profile of culture positive UTI children and adequacy of radiological tests. The secondary objectives were to assess the prevalent uro-pathogens and their antibiogram.
Methods: Clinical details regarding radiological investigations, prevalent uro-pathogens and antibiogram, common antibiotics used in treatment and uro-prophylaxis were obtained through electronic medical records in children <18 months of age admitted with culture positive UTI.
Results: In 225 children seen over a period of 36 months, a slight male predominance (56%) was observed. USG was done in nearly all children, with hydronephrosis being reported in nearly a sixth of them. MCU was performed in only 40% of patients with any degree of VUR noted in one-fifth of patients. DMSA, performed after 6 months of the index UTI, in one-fifths of the patients, demonstrated scarring in a small proportion. All three investigations were performed in one out of six children. Escherichia More Details coli was the predominant uropathogen identified. Significant meropenem resistance (52%) was observed in the community acquired UTI.
Conclusions: The evaluation and follow up of infants and young children with UTI shows inadequate radiological evaluation, which could increase the risk of undetected and untreated renal sequelae.
|Table 1: Comparing all three methods-systolic blood pressure and diastolic blood pressure|
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| O52 Clinical-Epidemiological Profile of Chronic Kidney Disease in Children Attending Tertiary Care Centre in Western India during pre and post COVID-19|| |
Suchi Acharya, Suprita Kalra, K. M. Adhikari
Department of Pediatrics, AFMC and CH (SC), Pune, Maharashtra, India
Rationale: Chronic kidney disease (CKD) is an important cause of mortality, morbidity and impaired quality of life in children. COVID-19 pandemic has arisen a significant global threat. Its impact on patients suffering from CKD is predicted to be severe and enduring, but the absolute magnitude is still largely unclear. As there have been constant interruptions to everyday life owing to social distancing and lockdowns during COVID-19, CKD patients were lost to follow up, were not on regular health check up and was also not able to access the health care system in emergencies. While children constitute a small proportion of patients with COVID-19, those with chronic disorders like CKD constitute a high-risk group and at-risk for adverse outcomes. A better understanding about the epidemiology, etiology and preventable factors can help in better management of these children amid the pandemic situation.
Objectives: To study the epidemiology, etiology and clinical profile of CKD in children who attended the tertiary care centre in Western India during pre and post COVID-19 period.
Methods: The demographic, clinical and biochemical profile and outcome data of children aged 0 to 15 years, diagnosed with CKD, attending our institute from September 2017 to September 2022 were analysed retrospectively using hospital based records. CKD was defined and classified using the KDIGO 2012 Clinical Practice Guideline for the Evaluation and Management of CKD. “Pre COVID-19” period was between September 2017 and March 2020, while “Post COVID-19” was between April 2020 and September 2022, as WHO declared COVID-19 as global pandemic on March 11, 2020.
Results: A total of 217 children diagnosed with CKD were included in our study. Among them 124 presented in pre COVID-19 period and 93 presented post COVID-19. [Table 1] Out of 217 CKD patients, 72% were male while 27.6% were females with male: female ratio of 2.6:1, which remained almost same in pre and post COVID-19 period. Most common age group affected were between 5 and 10 years (41%) in pre pandemic period while postpandemic it was under 5 years children (65%) who were affected the most. Stage 1 CKD (49.7%) was the most prevalent stage of CKD in our cohort followed by Stage 2 and 3 in both pre and post pandemic period. Out of 18 end stage renal disease (ESRD) children 10 presented in pre COVID-19 time while 8 presented post COVID-19. Congenital anomaly of kidney and urinary tract (CAKUT) was the most common underlying cause of CKD in more than 50% children in both pre and post COVID-19. Anemia and growth failure was the most prevalent clinical findings in our cohort. Among the 18 ESRD patients, almost 38% patients were lost to follow up in the post COVID-19 period and there were one mortality in the post-COVID-19 period. In our study more number of children presented at advanced stages of CKD during post COVID-19 compared to the prepandemic period because of poor follow up, poor access to health care facility due to social distancing and lockdowns.
|Table 1: Clinical-epidemiological profile of chronic kidney disease in children in pre- and post-COVID-19|
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Conclusions: COVID-19 has definitely impacted the children with CKD. Our study helps highlight the need for a national childhood CKD registry and public health measures to enhance early diagnosis, management, and prevention of progression of CKD in children, especially in these tiring times of COVID-19 pandemic. Prompt diagnosis, treatment, and prevention of progression can be life-saving in our setting.
| O53 Acute Kidney Injury in Children Hospitalised with Nephrotic Syndrome: Incidence, Etiology, Short-Term Outcome and Its Comparison in Steroid Sensitive and Steroid Resistant Nephrotic Syndrome|| |
Uma Maheshwari, AlpanaOhri, Amish Udani, Chintan Shah
Bai Jerbai Wadia Hospital for Children, Mumbai, Maharashtra, India
Rationale: Acute kidney injury (AKI), a severe complication of nephrotic syndrome (NS) is being more prevalent in recent times.
Objectives: (1) Determine incidence, etiology, short-term outcome of AKI in children hospitalized with NS. (2) Compare above variables in steroid sensitive versus steroid resistant NS (steroid-sensitive nephrotic syndrome [SSNS] vs. Steroid-resistant nephrotic syndrome [SRNS]).
Methods: Prospective observational study over 1 year including all consecutive nephrotics hospitalized with AKI. AKI defined according to the KDIGO criteria with baseline creatinine taken as the lowest value in last 6 months/ nadir during stay. Secondary NS were excluded. Calcineurin inhibitors, angiotensin-converting enzyme inhibitor/angiotensin receptor blockers excluded as nephrotoxins as most SRNS received these drugs and causality could not be established. Demographics, clinical features, labs, hospital course, treatment and follow-up at 3 months were recorded. SSNS and SRNS were compared.
Results: The incidence of AKI in hospitalized nephrotics (25/120) was 20.8%. Twenty-five patients (10 girls) with a mean age of 5 years (±4) developed AKI. Common etiologies of AKI were infection (56%), Nephrotoxic drugs, severe hypovolemia, and thrombosis in the decreasing order. Amongst drugs-use of radio contrast (3), indigenous drugs (2), aminoglycosides (2), and NSAIDS (1) were seen. Peak creatinine of 2.3 mg/dl (±1.59 standard deviation) was seen on day 4 of admission. Nephrotics developing AKI had a longer hospital stay versus those without AKI (P < 0.001). 2/25 patients died (8%), 10/25 (78.9%) required pediatric intensive care unit (PICU), 6/25 (46%) needed renal replacement therapy (RRT). Among discharged patients 4/23 (17.39%) had persistent deranged renal functions at 3 months' follow up, all of whom belonged to SRNS category. Comparison of 11 patients in SSNS (Group 1) with 14 in SRNS (Group 2) showed UTI (35.5%), drugs (28.6%), systemic infection (21.4%), thrombosis (14.2%) as the etiologies of AKI in SRNS. Systemic infection (45.5%), drugs (27.3%), and hypovolemia (18.2%) were causes in SSNS. Mortality rate, need for PICU (P = 1.0), and RRT (P = 0.7) were similar (4%, 42%, 28%) versus (4%, 36%, 18%).
Conclusions: AKI is seen in up to a fifth of children with NS equally in SSNS and SRNS. While etiology and severity of AKI in the two groups is similar, short-term outcome in SRNS is more ominous.
| O54 Genetic Profile and Genotype-Phenotype Correlation of Children with Primary Distal Renal Tubular Acidosis|| |
Jaya Verlani, K. Shivakumar1, Sachin George2, Georgie Mathew2, Indira Agarwal2, Susan Uthup3, Anil Vasudevan
St. John's Medical College Hospital, Bengaluru, Karnataka, Christian Medical College, Vellore, Tamil Nadu, Government Medical College, Thiruvananthapuram, Kerala, India
Rationale: Primary distal renal tubular acidosis (dRTA) is one of the common renal tubular disorders in children. Variants in ATP6V0A4, ATP6V1B1, SLC4A1, WDR72, and FOX11 gene constitute a monogenic causation in 50%–70% of dRTA.
Objectives: The aim of the study was to (a) determine the genetic yield of clinical exome sequencing assessing relevant disease genes in dRTA and (b) correlate the genetic variants with the phenotypic spectrum of dRTA.
Methods: In this cohort study, which is ongoing, children (<18 years) clinically diagnosed with dRTA were recruited from three centers after ethical approval. We obtained clinical data, pedigree information, and DNA samples at recruitment and during follow up. Genetic analyses were performed by clinical exome sequencing (CES) using Illumina HiSeq platform. Variant were filtered using GATK best practices pipeline and annotated with ensemble variant effect predictor data and were classified in accordance with ACMG/AMP guidelines. To facilitate the identification of potential genotype–phenotype associations in the interim analysis, the cohort was divided according to the genetic information into four groups: (i) SLC4A1 (ii) ATP6V0A4 (iii) ATP6V1B1 and (iv) others.
Results: Of the 48 children recruited (male/female ratio 27/21), 9/48 (18.7%) had positive family history and 23/48 (48%) were born from consanguineous marriage. The median age (range) at time of manifestation and recruitment were 12 (2 to 24.5) months and 78 (34 to 120) months, respectively. Failure to thrive at diagnosis was documented in 25/38 (64%) children. Severe hypokalemia (<3.5 meq/L) at time of diagnosis was noted in 21/45 (46%) children. Sensorineural hearing loss and nephrocalcinosis were seen in 5/45 (11%) and 22/47 (46.8%), respectively. The overall followed up duration was 16.5 (9 to 23.75) months. The average estimated glomerular filtration rate (eGFR) (n = 33) at last follow up was 108.48 (Range-65.08 to 174.37) ml/min/1.73m2 with one third (11/33, 33%) patients having eGFR <90ml/min/1.73m2. CES identified disease causing variants in 26 patients (54.1%) while variants of uncertain significance were detected in 14/48 (29.1%) patients. Disease-causing variants were most commonly identified in SLC4A1 (9/26; 34.6%) and ATP6V0A4 (9/26; 34.6%) genes followed by, ATP6V1B1 gene (5/26; 19.2%). Causal variants were less commonly identified in WDR72 (2/26; 7.7%) and FOX11 (1/26; 3.8%) genes. Genetic testing changed the diagnosis in three patients (6.2%). Children with SLC4A1 variants had a trend towards older age of presentation in patients compared to other genetic variants with median age 31 (19–36 months, P = 0.28); ATP6V1B:12 (12–34); ATP6V0A4: 2 (1.5–2) and others 14 (4–18). Children with SLC4A1 variants also had poor growth at last follow-up compared to others, mean height standard deviation score (SD) −3.31 (1.72); ATP6V1B: −1.37 (0.5); ATP6V0A4: 0.7 (3.36) and others: −0.87 (2.12). There was trend towards lower potassium in children with ATP6V0A4 variant compared to others (Mean [SD] 3.11 [0.49]; ATP6V1B: 3.23 [0.62]; SLC4A1: 3.8 [0.5] and others: 4 [0.28] mEq/L). There was no statistically significant difference in deafness among the variants. There was no statistically significant difference (P = 0.31) in the distribution of patients with impaired eGFR among the groups.
| O55 The Prevalence of Antineutrophil Cytoplasmic Antibody during therapy with Levamisole for Steroid Sensitive Nephrotic Syndrome|| |
Puneet Singh, Aditi Sinha, Pankaj Hari, Priyanka Khandelwal, Uma Kumar1, Vineeta Batra2, Arvind Bagga
Department of Pediatrics, Division of Pediatric Nephrology, AIIMS, 1Department of Rheumatology, AIIMS, 2Department of Pathology, GB Pant Hospital, New Delhi, India
Rationale: While antineutrophil cytoplasmic antibody (ANCA) vasculitis is a known rare adverse effect of levamisole, there is limited information of the prevalence, types and determinants of ANCA in children receiving therapy with levamisole for nephrotic syndrome.
Objectives: To determine, in patients with steroid-sensitive nephrotic syndrome (SSNS), the prevalence of ANCA (by indirect immunofluorescence, IIF), its specificity by ELISA against six antigens, its association with presence of palpable purpura, neutropenia, antinuclear antibodies (ANA), anticardiolipin (aCL) antibodies, and hypocomplementemia.
Methods: This cross-sectional observational study enrolled patients, aged 2–18 years, with SSNS receiving levamisole at doses 2–2.5mg/kg on alternate days for ≥6 months with concurrent prednisolone dose ≤0.5 mg/kg/day. All patients underwent testing for ANCA by both IIF and ELISA, positive threshold being, perinuclear/cytoplasmic pattern at serum dilution ≥1:10 and >9.6 U/ml (myeloperoxidase [MPO])/>6.7 U/ml (proteinase 3 [PR3]) for respective methods. Patients with ANCA by either method were screened for its specificity against six antigens, namely PR3, MPO, anti-neutrophil elastase/HNE, lactoferrin, cathepsin G and bactericidal/permeability-increasing protein with positive threshold at extinction ratio >1.0. Conventional statistical tests were used to determine the association of ANCA with the presence of palpable purpura, leukopenia (total leukocyte count <4000/μL), neutropenia (absolute neutrophil count <1500/μL), (ANA, by IF, in serum diluted ≥1:100), aCL antibodies, hypocomplementemia (complement C3 <70 mg/dL), clinical features, and intensity of exposure to levamisole and prednisolone therapy.
Results: During October 2020–June 2022, 117 patients (68.4% boys) with median (interquartile range) age 90 (75–107) months were enrolled. ANCA were present in 20 (17.1%) patients (95% confidence interval [CI] 7.93–20.08 for both IIF and ELISA). Of these 20 patients, 13 (65%) had anti-MPO antibodies, 6 (30%) had anti-HNE, and 4 (20%) had anti-PR3 antibodies. Nineteen (16.2%) patients tested positive for ANA, eight (6.8%) had aCL antibodies, four (3.4%) had leukopenia, and one had neutropenia; however, these were not associated with presence of ANCA. Patients with ANCA tended to be older, more often female, had relatively prolonged levamisole use with lower concomitant prednisolone dose [Table 1] and [Figure 1]. The only independent determinant of ANCA positivity was a concomitant prednisolone dose <0.15 mg/kg/day (adjusted odds ratio 0.001; 95% CI 0.00003–0.045).
|Figure 1: Receiver operating characteristic curves for the presence of ANCA and (a) duration of levamisole use (threshold 28 months; sensitivity 80%; specificity 61.9%; area under the curve 0.696), and (b) dose of concomitant prednisolone dose (threshold 0.15 mg/kg/day; sensitivity 76.3%; specificity 85%; area under the curve 0.807). ANCA: Antineutrophil cytoplasmic antibody|
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|Table 1: Comparison of parameters between patients receiving levamisole with and without ANCA|
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Conclusions: ANCA is present in one in six patients receiving levamisole for nephrotic syndrome and is most commonly generated against MPO, HNE, and PR3. ANCA appears to be associated with reduced corticosteroid dose (<0.15 mg/kg/day) and prolonged levamisole therapy (>28-months).
| O56 Assessment of the Agreement between OCM-Derived Kt/V and Equilibrated Kt/V, Measured Conventionally Using urea Reduction Ratio in Children on Maintenance Hemodialysis|| |
Srinivasavaradan Govindarajan, Durgam Yashwanth, Kratik Agarwal, Madhur Singhal, Anand Tiwari, Aditi Sinha, Arvind Bagga
Department of Pediatrics, Division of Pediatric Nephrology, All India Institute of Medical Sciences, New Delhi, India
Rationale: Assessment of adequacy of delivered dialysis dose helps titrate the prescription of maintenance hemodialysis (MHD). Methods to measure dialysis efficacy rely on Kt/V using urea-kinetic modelling, and recently, online monitoring (OCM) using ionic dialysate. While OCM-derived Kt/V minimizes blood sampling and provides dynamic assessment, it has not been validated against conventional measures of dialysis adequacy in children. Further, the optimal method to estimate urea distribution volume (V) in children on MHD remains uncertain.
Objectives: The primary objective was to assess the agreement between OCM-derived Kt/V and equilibrated Kt/V (eKt/V), measured conventionally using urea reduction ratio, in children on MHD. Secondary objectives were to assess the agreement of (i) OCM-derived Kt/V measured using four equations for V and eKt/V, and (ii) Kt/V predicted based on the dialyser coefficient (provided by the manufacturer) and blood flow rate, similarly measured using different equations for V (predicted Kt/V), and eKt/V.
Methods: This prospective observational study enrolled all patients, age 3–18 years, undergoing MHD at a single center over 6-months. Patients with acute kidney injury or electrolyte or coagulation abnormalities, and sessions that were short (<4-hour) or infrequent (<3/week) were excluded. Parameters recorded included pre and postdialysis weight and blood pressure, dialysate and blood flow rates, net ultrafiltrate, and duration of session. Pre-and post-procedure blood samples were collected as described conventionally for calculating eKt/V. OCM-derived Kt/V was recorded at end of session. Predicted Kt/V was calculated from KoA and BFR as described by manufacturer. Median bias (95% confidence interval [CI]) and levels of agreement were calculated using Bland-Altman analysis; correlation coefficient between measurements was estimated using Lin's method. Agreement was compared among OCM-derived Kt/V and predicted Kt/V using four pediatric formulae for V.
Results: During April–September 2022, Kt/V was measured, OCM-derived and predicted, during 100 sessions of MHD in 15 patients (88% boys) with median (interquartile range) age of 14 (11, 17.8) years. Mean eKt/V was 1.45 ± 0.33, while the OCM-derived value, using the Mellits-Cheek equation for V, was 1.37 ± 0.27. [Table 1] provides the median bias with 95% CI and limits of agreement for OCM-derived Kt/V and predicted-Kt/V, using different equations for V, compared to eKt/V. The agreement between eKt/V and Kt/V appeared to be clinically acceptable when using the Mellits-Cheek equation (75 children within ±20%), but agreement was poor when the Watson formula was used [Figure 1] and [Table 1].
Conclusions: Online clearance monitoring can provide generally acceptable data on delivered dialysis dose in children. Generalizability for children with AKI and shorter sessions needs further observation.
| O57 Efficacy of Daily Plasma Exchange in Reducing Clinical Severity and Improving Laboratory Parameters in Patients with Acute Liver Failure or Acute on Chronic Liver Failure|| |
Srinivasavaradan Govindarajan, Sagar Tungal1, Sanjeevani Kaul2, Rohan Malik2, Anand Tiwari, Priyanka Khandelwal, Pankaj Hari, Aditi Sinha, Arvind Bagga
Department of Pediatrics, Division of Nephrology, All India Institute of Medical Sciences, 1Department of Pediatrics, Division of Intensive Care and Pulmonology, All India Institute of Medical Sciences, and 2Department of Pediatrics, Division of Gastroenetroelogy and Hepatology, All India Institute of Medical Sciences, New Delhi, India
Rationale: High volume plasma exchange (PEX) has been shown to be useful as a bridge to transplant in acute liver failure (ALF). Information on the role of PEX in pediatric ALF and acute on chronic liver failure (ACLF) is scarce.
Objectives: We assessed the efficacy of daily PEX in reducing clinical severity and improving laboratory parameters in patients with ALF or ACLF.
Methods: We retrospectively reviewed clinical records of all children with ALF or ACLF in whom daily PEX was performed in addition to usual standard of care, for hepatic encephalopathy and coagulopathy during 2015–2022. Membrane-based PEX was performed with 1–1.5 times the plasma volume using fresh-frozen plasma without anticoagulation, daily until indicated or demise. Outcomes included change in neurological status, laboratory features of ALF, scoring/staging for organ dysfunction (Chronic liver failure-sequential organ failure assessment [CLIF-SOFA], APASL-ACLF Research Consortium [AARC] grading) at day 7, and transplant-free survival.
Results: Fifteen patients (53.3% boys) with median (interquartile range) age 8 (6, 9) years underwent median 3 (range 2–7) sessions of PEX with plasma volume of 57.1 ml/kg (55, 63) ml/kg. Indications for PEX were ALF (n = 7) or ACLF (n = 8), chiefly secondary to hepatitis A and Wilson disease, respectively. Fourteen (93.3%) patients had stage 3 hepatic encephalopathy that met King's College criteria for liver transplant. The median CLIF-SOFA score at admission was 12 (11, 13); 62.5% patients with ACLF had AARC Grades 3–5. [Table 1] shows significant improvement in neurological status and laboratory parameters following PEX sessions. The median hospital stay was 11 (8, 16) days. Transplant-free survival by day 7 and 28 was 53.3% and 33.3%, respectively; one child underwent transplantation. Patients with ALF had significantly better outcomes in terms of neurological status, total bilirubin, improved coagulation profile, and survival (57.1% vs. 25%). There were no access - or procedure-related complications.
|Table 1: Agreement between Kt/V, for that estimated online (online monitoring-derived Kt/V) and that predicted from the dialyser KoA co-efficient and blood flow rate (predcited Kt/V), each using V estimated by different equations, and equilibrated Kt/V (eKt/V; based on urea reduction ratio)|
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| O58 Serum Magnesium Levels and Fractional Excretion of Magnesium in Children and Adolescents with Nephrotic Syndrome|| |
Neha Garg, Mukta Mantan, Urmila Jhamb, Binita Goswami1
Departments of Pediatrics and 1Biochemistry, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi, India
Rationale: Hypomagnesemia and increased fractional excretion of magnesium (FeMg) have been identified in patients with tubular damage. In this study we explore the possibility of using these parameters to identify tubular damage in children with nephrotic syndrome.
Objectives: The primary objective of the study was to estimate FeMg and serum magnesium levels in children and adolescents (2–18 years) with nephrotic syndrome (both steroid-sensitive nephrotic syndrome [SSNS] and steroid resistant nephrotic syndrome [SRNS]), currently in complete or partial remission.
Methods: This cross-sectional study was done from July 2021 to July 2022 (n = 50); children with CKD stage 3 or more, receiving drugs like diuretics, aminoglycosides, proton-pump inhibitors, secondary and congenital nephrotic syndrome were excluded. Clinical details were elicited and examination was done. Remission was confirmed with biochemical investigations and serum magnesium and creatinine levels were estimated; estimation of urinary magnesium and creatinine was done on freshly voided urine sample using Vitros 5600 integrated auto analyzer machine with a photometric kit.
Results: Fifty (31 Male: 19 Female) children (25 SSNS and 25 SRNS) with median age (interquartile range) 10 years (7; 11) were enrolled. Cyclosporine was used for more than 2 years in 40% of SRNS patients and tacrolimus was used for more than 2 years in 20%. The median value of serum magnesium for SSNS and SRNS group were 1.9 and 1.7 mg/dl respectively (P = 0.25) and FeMg were 1.76% and 1.39% respectively (P = 0.45). Hypomagnesemia (<1.8 mg/dl) was seen in 36% and 52% of SRNS and SSNS group, respectively (P = 0.45). The prevalence of increased FeMg (>2.2%) was similar in both the groups i.e., 28% (P = 1). Compared to MCD (47.4%), patients with FSGS (83.3%) had higher incidence of hypomagnesemia.
Conclusions: Prevalence of hypomagnesemia in NS was 44% (52% in SRNS and 36% in SSNS) while elevated FeMg was seen in 28% and comparable in both groups. Hypomagnesemia appears to be a common electrolyte abnormality in NS, especially SRNS and underlying tubular damage may be contributory both in SSNS and SRNS.
|Table 1: Pre-post comparison of clinical and laboratory parameters in 15 patients (53.3% boys), median age 8 (interquartile range 6-9) years|
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| O59 Determination of Arterial Stiffness in Children with Idiopathic Nephrotic Syndrome Compared to Apparently Healthy Children: A Cross-Sectional Study|| |
AIIMS, Bhubaneswar, Odisha, India
Rationale: Studies on the evaluation of endothelial function and its predictive risk factors in children with nephrotic syndrome (NS) are limited. There is a paucity of evidence regarding these parameters in determining the risk of cardiovascular disease. Such studies are important from both preventive and therapeutic point of view. This study is the first of its kind in India aimed to determine the combined physiological and vascular integrity parameters for predicting the risk of premature atherosclerosis.
Objectives: To determine arterial stiffness by measuring carotid intimal medial thickness (cIMT) in children with Idiopathic NS as compared to apparently healthy children of age group 2–14 years. Also compare the following parameters, flow mediated dilatation (FMD), carotid-radial pulse wave velocity (crPWV), Reactive hyperemia index, central BP monitoring, and Augmentation index.
Methods: A cross-sectional study was done from January 2020 to December 2021 in apparently healthy and NS children in remission in a tertiary care hospital. Relevant history, physical examination, anthropometric measurements, and laboratory investigations were done. Early markers of atherosclerosis were measure during the radiological and physiological parameters.
Results: Dyslipidemia was seen in more than 50% of children during remission. The mean cIMT in the common carotid artery was higher in children with NS. There was a significant correlation of the percentage change in FMD with body mass index, total cholesterol, triglycerides, LDL, and the duration of disease. However, there was neither any significant correlation between age with crPWV and Reactive hyperemia index (RHI) nor between physiological and radiological parameters between the study and control groups.
Conclusions: Dyslipidemia persists even in the remission phase in NS. No significant difference was noted in the absolute change in FMD or the % proportionate change in FMD pre and postdilatation in both the study and control groups. There was a trend of lower RHI in children with NS.
|Table 1: Distribution of patients according to serum magnesium and fractional excretion of magnesium|
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| O60 Comparative Study of Efficacy of oral Cyclophosphamide verses Mycophenolate Mofetil in Frequently Relapsing Steroid Dependent Nephrotic Syndrome|| |
Gurdeep Singh Dhooria, Deepak Bhat
Dayanand Medical College and Hospital, Ludhiana, Punjab, India
Rationale: Both oral cyclophosphamide (CYC) and mycophenolate mofetil (MMF) have been used in frequently relapsing/steroid dependent (FR/SD) nephrotic syndrome to prevent relapses and prevent steroid toxicity. Efficacy of oral CYC and MMF has not been compared previously.
Objectives: To study of efficacy of oral CYC verses MMF in FR SD nephrotic syndrome.
Methods: This single-center, nonrandomized, open-label trial conducted in the Pediatric Nephrology clinic of a tertiary care center enrolled children aged between 3 months and 18 years with FR/SD nephrotic syndrome. Participants included, received therapy with MMF (45 cases, 20% female) (800–1200 mg/m2 daily) for one year or oral CYC (100 cases, 25% female) (2–2.5 mg/kg for 8–12 weeks, cumulative dose <168 mg/kg); prednisolone was discontinued by 2–3 months. Responders were children in whom steroids were stopped for at least 6 months or more.
Results: Median age at onset of NS in CYC group was 3 years (interquartile range [IQR] 2–5.2) versus 2.6 years (1.6–3.45) in MMF group. Median age at CYP was 5.7 years (IQR 3.7–7.9) versus 5 years (IQR 3.65–7.33) in MMF group. Fifty percent of patients had sustained response at 6 months in both CYC and MMF group. Relapse-free survival post CYP therapy was 31% at 1 year versus 35% in MMF group. Median time to first relapse post CYP was 4 months versus 5 months (IQR 3–7) in MMF group. Steroids were stopped after median of 6 months (IQR 4–8) of starting MMF, whereas steroids were off in most patients at completion of course of CYC itself. Frequency of relapses were similar in both groups 1 (IQR 0–2).
Conclusions: Therapy with oral CYC was not superior to MMF in the frequency of relapses or sustained remission in children with FR or steroid-dependent nephrotic syndrome.
| O61 Bioavailable Vitamin D Levels in Children with First Episode Nephrotic Syndrome: A Longitudinal Analytical Study|| |
V. Sai Charan, Abhijeet Saha, Rachita Singh Dhull, Rachita Singh Dhull, Anita Nangia, Rajeev Goel
Lady Hardinge Medical College and Associated Kalawati Saran Children's Hospital, New Delhi, India
Rationale: Paucity of studies on bioavailable vitamin D levels in idiopathic first episode nephrotic syndrome (FENS) in children.
Objectives: Primary: To assess the levels of serum bioavailable vitamin D in children aged 1 to 12 years with idiopathic FENS and in healthy controls. Secondary objectives: (1) To measure the levels of bioavailable Vitamin D in FENS and after 4 weeks of standard steroid therapy induced remission. (2) To compare levels of serum and urine Vitamin D binding protein (VDBP) in FENS and after 4 weeks of steroid therapy induced remission.
Methods: Study design: Longitudinal analytical study. Study period: January 2021 to June 2022. Place of study: Division of Paediatric Nephrology, LHMC and KSCH, New Delhi. Department of Pathology and Biochemistry, SSK Hospital, LHMC, New Delhi. Study population - Children between 1 and 12 years with idiopathic FENS. Age and gender matched healthy children (1–12 years) were taken as controls. Inclusion criteria - 1 – 12-year-old children having idiopathic FENS. Exclusion criteria - (1) Apparent clinical rickets. (2) INS associated with other hypoalbuminemic states i.e., liver disease. Treatment protocol - As per ISPN protocol.
Methods: After the diagnosis of FENS (ISPN definition) was established additional investigations were done: (1) 25(OH) Vitamin D (2) PTH (3) Serum VDBP (ELISA) (4) Urine VDBP (ELISA). Bioavailable Vitamin D was calculated using serum albumin, 25 hydroxy Vitamin D and VDBP using formula suggested by Powe et al. is summarized below, Free 25(OH)D = (−b+√[b2−4ac])/2a
a = Kdbp∗Kalb∗albumin+Kdbp
b = (Kdbp∗DBP)−(Kdbp∗25(OH)D) + (Kalb∗albumin)+1
c = −(25[OH]D)
Kdbp = affinity constant between 25(OH)D and DBP (7 × 108 M-1)
Kalb = affinity constant between 25(OH)D and albumin (6 × 105 M-1)
Bioavailable 25(OH)D = (Kalb∗albumin+1)∗(free 25[OH]D)
Patients were followed up after 4 weeks of steroid induced remission and bioavailable Vitamin D was calculated again.
Results: The mean bioavailable Vitamin D was significantly lower during relapse (0.75 [0.84]) than during remission (1.11[0.84]). Other results are summarized in table attached.
Conclusions: This is the first known study to prove bioavailable Vitamin D as a better marker of Vitamin D status in children with FENS, than total 25 hydroxy Vitamin D, thereby proving free hormone hypothesis and adding emphasis on supplementation with maintenance doses of Vitamin D3 and calcium in all children with FENS will improve the bone health.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13], [Figure 14], [Figure 15], [Figure 16], [Figure 17], [Figure 18]
[Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6], [Table 7], [Table 8], [Table 9], [Table 10], [Table 11], [Table 12], [Table 13], [Table 14], [Table 15], [Table 16], [Table 17], [Table 18], [Table 19], [Table 20], [Table 21], [Table 22], [Table 23], [Table 24], [Table 25], [Table 26]
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|O16 To Evaluate ...|
|O17 Use of Furos...|
|O18 Urinary Biom...|
|O19 Role of Urin...|
|O20 Experience O...|
|O22 The Renal Co...|
|O24 Clinical Pro...|
|O25 Clinical Pre...|
|O26 Diagnosis of...|
|O27 Age at Surge...|
|O29 A Study of t...|
|O30 To Understan...|
|O31 A Study of A...|
|O32 Serum Cystat...|
|O33 Predictive V...|
|O34 To Study the...|
|O35 Comparison o...|
|O36 Assessment o...|
|O37 To Assess Fu...|
|O38 Clinico Path...|
|O39 Vancouver Sy...|
|O41 Genetic Defe...|
|O42 Kidney Morph...|
|O43 Exploring th...|
|O44 Our Experien...|
|O46 Serum Metabo...|
|O47 Effect of Ri...|
|O48 Lupus Nephri...|
|O49 Genetic Prof...|
|O50 Comparision ...|
|O51 Culture Posi...|
|O53 Acute Kidney...|
|O54 Genetic Prof...|
|O55 The Prevalen...|
|O56 Assessment o...|
|O57 Efficacy of ...|
|O58 Serum Magnes...|
|O60 Comparative ...|
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