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   Table of Contents - Current issue
January-June 2022
Volume 5 | Issue 1
Page Nos. 1-51

Online since Tuesday, June 28, 2022

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Management of severe acute kidney injury p. 1
Sudarsan Krishnasamy, Sriram Krishnamurthy
Acute kidney injury (AKI) is encountered in approximately one-fourth of children admitted to the intensive care units (ICUs). As AKI is known to prolong ICU stay as well as increase the overall morbidity and mortality, it is important to identify it timely and take appropriate measures to curtail further injury. Infections continue to be the most common cause in developing countries. While pneumonia, diarrhea, and tropical infections such as dengue, malaria, scrub typhus, and leptospirosis are major causes of AKI in children; glomerular diseases, systemic disorders, envenomations, and drugs also account for a major proportion of AKI in low and low-middle income countries. Fluid overload is associated with adverse outcomes in multiple studies; hence proper assessment of volume status is vital. Novel prognostic markers such as renal angina index and furosemide stress test are increasingly being applied in routine clinical care. The current guidelines recommend against the usage of furosemide for the prevention and management of AKI, except in a situation of fluid overload. Kidney replacement therapy (KRT) should be initiated promptly in AKI when indicated. The timing of initiation of KRT in AKI continues to be debatable and has attracted considerable research. While peritoneal dialysis continues to be the modality most often used in infants and young children, continuous KRT and sustained low-efficiency dialysis are used in hemodynamically unstable patients. Timely identification and management of the various complications reduce mortality. Cutting-edge multinational trials over the past decade have significantly impacted our understanding in managing this complex disorder.
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Nutrition assessment tools in children with chronic kidney disease p. 7
Arpana Iyengar
Children with chronic kidney disease (CKD) are vulnerable to undernutrition and its accompanying consequences. Assessment of nutrition in these children is confounded by the presence of edema and overhydration. There is no single measure that can comprehensively reflect the underlying nutrition status. Hence, there is a need to explore nutrition assessment tools that reflect body composition without being affected by hydration status. Many tools of assessment that are widely studied in adults with CKD get extrapolated to children. Studies on nutritional assessment focusing on pediatric patients with CKD are needed for early recognition and long-term monitoring of nutrition status. This review attempts to provide an understanding of the utility and limitations of tools available for assessment of nutrition and body composition in the context of undernutrition in pediatric CKD.
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Pediatric tubular and inherited disorders in asia: results of preliminary survey of the asian pediatric nephrology association (aspna) tubular and inherited working group p. 14
Lourdes Paula Real Resontoc, Nozu Kandai, Nakisa Hooman, Anil Vasudevan, Jie Ding, Hee Gyung Kang
Background and Objective: The registries and guidelines for kidney diseases in children mostly do not include the Asian population and hence, its applicability is questionable. As a first step to address this question, the tubular and inherited disease working group of the Asian Pediatric Nephrology Association aimed to assess the current situation of pediatric tubular and inherited disorders in Asia. Methods: Our group conducted an online survey among the members of AsPNA from September to October 2020. Data collected included demographics, number of patients each physician cares for per year, methods of diagnosis, and access to genetic tests. Descriptive analysis was performed. Results: A total of 299 pediatric nephrologists from 21 countries in Asia participated. Distal renal tubular acidosis, Bartter syndrome, autosomal dominant polycystic kidney disease, autosomal recessive kidney disease, and Alport syndrome were the commonly reported diseases. Around 70% employed clinical history, radiologic imaging, and biochemical tests for diagnosis. More than half (55.4%) of the institutions have access to genetic testing. For future collaborative projects, 88% expressed interest to participate. Conclusions: The results highlight the diversity of disease prevalence, diagnostic practices, capability, and access to genetic tests across Asia. The data gathered from this preliminary survey can be used to address knowledge gaps, and improve management and outcomes.
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COVID-19 and anemia in children with nephrotic syndrome p. 21
Md Abdul Qader, Azmeri Sultana, Mohammed Maruf ul Quader, Jubaida Rumana, Tarannum Khondaker, Naito Kanon, Mohammed Hanif
Context: Children with COVID-19 present with less severe disease and require fewer hospitalizations than adults. Our previous study on children with renal disease and COVID-19, which included predominantly children with nephrotic syndrome, found anemia in a significant number of participants. Aims: This study aimed at evaluating the risk factors of anemia in children with nephrotic syndrome presenting with COVID-19 and the influence of anemia on hospital outcome. Methods: This case–control study was conducted at five pediatric nephrology centers in two major cities of Bangladesh. Consecutive patients with nephrotic syndrome and positive polymerase chain reaction (PCR) for severe acute respiratory syndrome coronavirus 2 were included as cases, and subsequently, two cases of nephrotic syndrome with negative PCR were enrolled as controls. Participants who presented between April 2020 and December 2020 were included, and demographic data, clinical features, and laboratory parameters were retrieved from hospital records for analysis. Results: A total of 22 children with nephrotic syndrome were positive with COVID-19 and subsequently, 44 children were included as control. The median age was 6.4 years in the cases and 5.2 years among the control. Most children presented with the initial episode of nephrotic syndrome. The children in the case group had a significantly lower hemoglobin level than the controls, and anemia was associated with raised inflammatory markers. In multivariate analysis, female sex and impaired renal function was associated with lower hemoglobin, but anemia did not have effect on hospital outcome. Conclusions: Anemia in children can be multifactorial. Anemia in nephrotic syndrome associated with COVID-19 does not appear to influence length of hospital stay and outcome.
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Utility of renal resistive index in children with nephrotic syndrome p. 27
Swarnim Swarnim, Mohsina Naj, Mukta Mantan, Anju Garg
Background: Renal resistive index (RRI) is one of the Doppler parameters that provide information about arterial impedance. While its utility has been explored in chronic kidney disease and obstructive uropathy, its role in glomerular diseases remains less defined. Aim: This study aimed to measure RRI in children (6–18 years) with nephrotic syndrome, both steroid sensitive (SSNS) and steroid resistant (SRNS), currently in remission. Setting and Design: This cross-sectional study was done over 1 year at a tertiary care teaching hospital from March 2017 to March 2018. Methods: Renal Doppler ultrasound was done and RRI was measured at the main renal, arcuate, and interlobar arteries for both the kidneys during disease remission. Detailed information of disease type, biopsy, and therapy was recorded. Baseline biochemical investigations were done to confirm disease remission. Results: Fifty patients (25 each with SSNS and SRNS) were enrolled; the mean age was 12.6 years. The mean RRI at the interlobar and arcuate arteries was elevated in SRNS in all three poles of both the kidneys as compared to the SSNS with a significant difference at midpole (P = 0.04). RRI values were higher in children having focal segmental glomerulosclerosis (FSGS) compared to those with minimal change disease. The percentage of patients having an RRI value >0.6 was significantly higher in those who had received cyclosporine for >2 years as compared to those who received it for <2 years. Conclusions: Doppler RRI values were elevated for patients with SRNS compared to SSNS; changes were significant at the midpole of both kidneys. Children with FSGS and those on calcineurin inhibitors for more than 2 years are more likely to have higher RRI values.
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Serum zinc level in patients on maintenance hemodialysis and its relationship to anemia p. 33
Nahideh Ekhlasi, Yosef Mojtahedi, Azadeh Afshin, Behnaz Bazargani, Arash Abbasi, Fahimeh Askarian, Dariush Fahimi, Mastaneh Moghtaderi
Hemodialysis patients are at risk for deficiency or excess of trace elements, both of which can affect their health. Zinc is an essential trace element and its deficiency results in the dysfunction of plasma membrane proteins and enhanced oxidative stress in cells, especially red blood cells, thus decreasing their lifespan. Zinc deficiency is reported in 40%–78% of hemodialysis patients and may be associated with anemia. Both anemia and zinc deficiency are common in patients with kidney failure. In this study, serum zinc levels and their relationship to anemia were evaluated in 24 patients on maintenance hemodialysis for 6 months or longer at either of two tertiary pediatric hemodialysis centers in Tehran, Iran. Serum zinc level and various blood indices were recorded before, during, and after the evaluation over 6 months. Patients receiving zinc-containing supplements in the 3 months before enrolment were ineligible. Patients with zinc deficiency received oral supplements of zinc sulfate at 1 mg/kg daily for 3 months, following which serum zinc level and other blood indices were retested to examine the influence of zinc supplementation on anemia. Our findings indicate that serum zinc levels may correlate with anemia in hemodialysis patients. Providing zinc supplements may improve anemia and the quality of life in patients with low zinc levels.
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Cantu and alport syndrome in a young girl p. 37
Chi San Wai, Lap Tak Alison Ma, Lai Ka Lee, Ho Ming Luk, Wai Ling Lau
We report a 9-year-old girl with nephritis, dysmorphism, and short stature. She was diagnosed to have Cantu syndrome and Alport syndrome by whole-exome sequencing. Diagnosis of patients with two or more syndromes is challenging due to blended phenotypes. This case illustrates the clinical utility of exome testing in patients with medical complexity.
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Laparoscopic Nephroureterectomy for Dysplastic Kidneys with Ectopic Ureters Opening Inside the Vagina p. 40
Mathula Hettiarachchi, Shenal Thalgahagoda
Urinary incontinence in girls could be due to ectopic ureteral insertion inside the vagina, a condition that is difficult to diagnose. In these patients, the urinary tract may be duplicated or single and be associated with a dysplastic kidney. We report two girls with dribbling of urine due to single ectopic ureteral insertion into the vagina. Computerised tomography urogram, retrograde contrast study and isotope scan revealed poorly functioning ectopic kidney. Hence, both patients underwent laparoscopic nephroureterectomy which led to the resolution of symptoms.
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An infant with cholestasis and refractory electrolyte abnormalities p. 43
Hadel Alsubaie, Yara S Kattan, Turki A Alshareef, Wajeeh Aldekhail, Weiam Almaiman
Arthrogryposis-renal dysfunction-cholestasis (ARC) is a rare multisystem disorder caused by mutations in the VPS33B gene, which in turn lead to a premature truncation of the gene product. This autosomal recessive disease has variations in phenotype. We report an infant girl who had severe presentation in the form of dysmorphic features, abnormal hearing assessment and refractory electrolytes disturbances. She was a product of consanguineous marriage with family history of similar presentation. The child passed away at age of 6 months due to aspiration pneumonia.
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Does the finding of horseshoe kidney in idiopathic nephrotic syndrome foresee prognosis or is it a co-incidental finding? p. 46
Walaa Abbas Alshammasi, Fakherah Alqahtani, Zainab Obaidi, Duaa Alsadeq, Abeer Bargawi, Ali Aldajani
A 6-year-old Yemini boy presented with nephrotic range proteinuria, hypoalbuminemia, edema, and hyperlipidemia, leading to a diagnosis of idiopathic nephrotic syndrome. Ultrasonography incidentally revealed a horseshoe kidney. While previous reports indicate the presence of congenital anomalies of the kidneys and urinary tract to be a poor predictor of remission in nephrotic syndrome, the present case responded to corticosteroids followed by sustained remission.
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Peritoneal dialysis via a central venous catheter in a Neonate with AKI p. 49
Tashi Tshering, Mimi Lhamu Mynak, Dorey A Glenn
At most centers, peritoneal dialysis (PD) is regarded as the optimal dialysis modality for neonates with acute kidney injury (AKI) who require kidney replacement therapy. Appropriately sized peritoneal catheters are not universally available in all countries and regions and are not currently available in Bhutan. We describe the successful use of a triple-lumen central venous catheter in an 18-day-old term infant with sepsis-related AKI and uremic encephalopathy. Considering the infant's deteriorating neurologic status and kidney function, the baby's fluid, electrolyte, and metabolic derangements were successfully managed by PD using a central venous catheter to access the peritoneal cavity. In regions where appropriately sized neonatal PD catheters are not available, a central venous catheter may be a useful alternative for short-term PD access.
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