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   Table of Contents - Current issue
Coverpage
July-December 2022
Volume 5 | Issue 2
Page Nos. 53-144

Online since Saturday, December 31, 2022

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EDITORIAL COMMENTARY  

Pediatric nephrology service in Nepal: Fellows' perspective from training to practice p. 53
Ajaya Kumar Dhakal, Devendra Shrestha, Vivek Kumar Todi
A trained workforce in pediatric nephrology subsubspecialty is critical to the prevention, treatment, and reduction of morbidity and mortality associated with kidney disease in the pediatric population. Currently, there is a disparity between the proportion of children with kidney disease and the workforce required to care for these children due to the lack of formal pediatric nephrology training programs in Nepal. As a result, some pediatricians are seeking pediatric nephrology training outside the country with the support of international nephrology or pediatric nephrology associations, whereas others are pursuing training opportunities on their own. This article describes the benefits and shortcomings of such training and the challenges of implementing the skills after returning to a low-resource setting. The article also offers recommendations for further improvements in such fellowship programs.
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REVIEW ARTICLES Top

Optimizing the hemodialysis prescription and assessment of dialysis adequacy in children p. 56
V Hamsa, Nivedita Kamath
Hemodialysis (HD) is an important modality of kidney replacement therapy in children with kidney failure. Vascular access in children can be challenging. Although central venous catheters are commonly used, arteriovenous fistulae provide an effective and sustainable access for HD. To provide optimal HD, the prescription should be tailored to each child. The size of the dialyzer and tubings and the extracorporeal circuit volume must be adapted to the size and weight of the child. Dialysate composition and duration of dialysis are altered to suit the metabolic profile, and the ultrafiltration volume is decided based on the hemodynamic status and interdialytic weight gain. To ensure that optimal dialysis is provided, parameters of dialysis adequacy are measured at regular intervals. Although clearance of urea (Kt/V) is the recommended measure of dialysis adequacy, it is important to assess growth, nutrition, blood pressure control, metabolic profile, and quality of life as measures of adequate dialysis in children.
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Neonatal hypernatremic dehydration p. 64
Mohd Ashraf, Umer Amin Qureshi, Nucksheeba Aziz Bhat
Hypernatremic dehydration is a medical emergency which adversely affects central nervous system and kidneys, resulting in high mortality and morbidity, especially in neonates. Neonates are typically affected within the first 2 weeks of life, with those born in hot summers to primigravida mothers with insufficient lactation being particularly prone. Clinical features are usually nonspecific, including weight loss, hyperthermia, irritability, lethargy, hyperbilirubinemia, poor oral intake, oliguria, seizures, and/or shock. Restoration of vascular volume by administering 10–20 ml/kg of isotonic intravenous fluid is essential in the presence of circulatory collapse, followed by controlled slow rehydration to prevent rapid changes in serum sodium (Na). The outcome of hypernatremia in neonates largely depends on its severity, the timeliness of intervention, and the rate of fall of serum Na during the management process, for which qualitative standards are lacking. This review aims to help pediatric nephrologists, neonatologists, other clinicians, and postgraduate scholars to recognize, understand, and treat neonatal hypernatremic dehydration in lucid manner based on available evidence.
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Evidence based management of primary monosymptomatic nocturnal enuresis p. 69
Arwa Bohra, Girish Chandra Bhatt
Nocturnal enuresis refers to bedwetting at night or during sleep. It may be either primary or have a complex presentation due to secondary etiologies. It is essential to differentiate primary enuresis from secondary enuresis, and monosymptomatic enuresis from polysymptomatic monosymptomatic enuresis, to guide therapy. For primary monosymptomatic enuresis, bladder training, urotherapy, alarm therapy, and desmopressin have been the mainstay of treatment. Choosing a suitable therapy for nocturnal enuresis warrants a thorough review of symptoms and voiding diary. Additionally, certain pretreatment and posttreatment response predictors have been found useful in management. Subclassification of primary monosymptomatic enuresis through bladder diary or ultrasonography helps select suitable candidates for anticholinergic therapy, which was previously reserved for refractory enuresis. The aim of this review is to integrate newer approaches with conventional therapy in diagnosing, classifying, and selecting therapies for nocturnal enuresis. Search Strategy: MeSH analysis was done on NCBI Platform with “enuresis” as MeSH major topic with several subtopics. The input for search builder was: (”Enuresis/classification”[Mesh] OR “Enuresis/diagnosis”[Mesh] OR “Enuresis/diagnostic imaging”[Mesh] OR “Enuresis/diet therapy”[Mesh] OR “Enuresis/prevention and control”[Mesh] OR “Enuresis/therapy”[Mesh])) AND (”Enuresis/etiology”[Mesh] OR). Six hundred and fifty-seven results were found from 75 clinical trials, 28 systematic reviews, and 4 review articles that were used to synthesize this review.
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ORIGINAL ARTICLES Top

Childhood nephrotic syndrome: A single center experience p. 78
Sherif Mohamed El-Desoky, Dalal Abdullatif Alghamdi, Mashael Mahmoud Abujabal, Ghadeer Ghazi Alahmadi, Jameela Abdulaziz Kari
Background: Childhood nephrotic syndrome (NS) is the most frequently occurring chronic kidney disease (CKD) among children. Methods: This retrospective review aimed to determine the clinical profile, diagnostic procedures, therapeutic strategies, and outcomes of NS in children <18 years old diagnosed and followed up in the pediatric nephrology unit at King Abdulaziz University Hospital, Kingdom of Saudi Arabia, from 2000 to 2020. Children with an underlying secondary cause for NS were excluded. Results: Five hundred and sixteen children were enrolled into four groups of patients, including steroid-sensitive NS (SSNS, 276 patients), primary steroid resistant (SRNS, 138 patients), secondary SRNS (56 patients), and congenital and infantile NS (46 infants). The age at presentation was 5.29 ± 2.83 years and mean follow-up duration was 4 years. Kidney biopsy was indicated for 174 (33.7%) children, of whom 79 (45.4%) had focal segmental glomerulonephritis, 35 (20.1%) had IgM nephropathy, and 25 (14.3%) had minimal change disease. Seventy (13.5%) patients were found to have positive homozygous variants, chiefly in NPHS1 in 25 (35.27%), NPHS2 in 16 (22.8%), and LAMB2 in 6 (8.5%). Acute kidney injury and kidney failure were most commonly observed in patients with SRNS (P < 0.001), while sepsis and mortality were significantly predominant among patients with infantile NS (P < 0.001). Kidney failure was reported in 8.7% patients. Conclusion: NS in children remains a challenging chronic glomerular disorder with variable outcome. Progression of CKD is seen most commonly in children with SRNS, more so in those with genetic etiology. Patients with congenital NS have the highest mortality.
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The poor outcome of pediatric chronic kidney disease in a resource-limited country p. 86
Tochi Ada Uchenwa, Ifeoma Comfort Anochie
Introduction: There is a global rise in the incidence of chronic kidney disease (CKD), with the implication of increased demand for kidney replacement therapy (KRT). In resource-limited countries where there is a large deficit in renal support for such patients, this translates into poor clinical outcomes. Materials and Methods: This was a retrospective review of the prevalence, clinical outcomes of CKD patients that were managed in the renal unit of the University of Port Harcourt Teaching Hospital from June 2016 to June 2019. Results: Findings from the study showed a male preponderance of 1:0.6. Of all patients with kidney disease, CKD comprised 24.6% of cases, and among these, 75.6% had ESKD. Of 34 (75.6%) patients who required KRT, 10 (29.4%) patients received at least one session of hemodialysis; however, only one patient (2.9%) continued to receive once weekly sessions of hemodialysis, sustained for a year. Mortality among the patients with CKD was 60%, with 28.9% lost to follow-up, signed against medical advice, or absconded. Conclusion: Considering the burden of CKD in Nigeria, much is desired with regards to available KRT options. Government regulation is needed to improve access to available KRT.
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CASE REPORTS Top

Medullary nephrocalcinosis: The role of genetic analysis p. 91
Nidhi Gupta, Manoj Dhanorkar, Manas Ranjan Behera, Dharmendra Bhadauria
Nephrocalcinosis refers to the deposits of calcium within the renal parenchyma, usually detected on ultrasonography or computed tomography. The presence of medullary nephrocalcinosis may represent a variety of different etiologies. Early childhood onset of kidney stones and/or nephrocalcinosis often represent an inherited defect more frequently than in adults. The cases reported here highlight three different diseases that were diagnosed based on genetic analysis following presentation as medullary nephrocalcinosis in childhood.
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CORRESPONDENCE Top

Steroid dependent nephrotic syndrome with vesicoureteral reflux p. 95
Suchi Acharya, Biju M John
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Chronic kidney disease in a patient with large platelets and hearing loss p. 97
Georgie Mathew, John Gnanaraj, Sukesh Chandran Nair, Deepthi Raranveettil
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ABSTRACTS Top

34th annual conference of indian society of pediatric nephrology selected abstracts p. 99
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JOURNAL SCAN Top

Journal scan p. 140
Georgie Mathew, Priyanka Khandelwal
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