Evidence-based clinical practice guideline for management of urinary tract infection and primary vesicoureteric reflux.

We present updated, evidence-based clinical practice guidelines from the Indian Society of Pediatric Nephrology (ISPN) for the management of urinary tract infection (UTI) and primary vesicoureteric reflux (VUR) in children. These guidelines conform to international standards; Institute of Medicine and AGREE checklists were used to ensure transparency, rigor, and thoroughness in the guideline development. In view of the robust methodology, these guidelines are applicable globally for the management of UTI and VUR.

Combined saposin deficiency: A rare occurrence.

Combined saposin deficiency (OMIM #611721), an exceedingly rare lysosomal storage disorder, is caused by a mutation in the gene . This gene encodes a protein, prosaposin, that cleaves into four constituent proteins, each of which has a role as a cofactor for the enzymes whose deficiency results in Krabbe disease, metachromatic leukodystrophy, Gaucher disease, and Farber disease, respectively. Intact prosaposin itself is essential for neuronal survival.

Consensus Guidelines on Management of Steroid-Resistant Nephrotic Syndrome.

Justification: The management of steroid resistant nephrotic syndrome (SRNS) is challenging. These guidelines update existing 2009 Indian Society of Pediatric Nephrology recommendations on its management.

Objective: To frame revised guidelines on diagnosis and evaluation, treatment and follow up, and supportive care of patients with the illness.

Enuresis, Lower Urinary Tract Dysfunction and Teachers' Perceptions: A School-based Survey.

Objective: To determine the prevalence of enuresis and lower urinary tract dysfunction among Indian school children, and describe teachers' perceptions regarding toilet requests.

Methods: Anonymous survey of students of a secondary school in Visakhapatnam, India by a modified version of the Dysfunctional voiding and incontinence scoring system (DVISS) in 2518 parents. Two questionnaires - the Bathroom behaviour scale and Teachers' hassle scale for toilet requests were designed, validated and administered to 138 teachers.

Current Management of Urinary Tract Infection and Vesicoureteral Reflux.

Urinary tract infection (UTI) is defined as the growth of a significant number of microorganisms of a single species in the urine, in the presence of symptoms. Symptoms in young children are non-specific such as fever without focus; young infants may manifest with irritability, failure to thrive, jaundice, vomiting and diarrhea. Older children usually have symptoms of cystitis or pyelonephritis.

Hemolytic uremic syndrome in a developing country: Consensus guidelines.

Background: Hemolytic uremic syndrome (HUS) is a leading cause of acute kidney injury in children. Although international guidelines emphasize comprehensive evaluation and treatment with eculizumab, access to diagnostic and therapeutic facilities is limited in most developing countries. The burden of Shiga toxin-associated HUS in India is unclear; school-going children show high prevalence of anti-factor H (FH) antibodies.

Posttransplant Lymphoproliferative Disorder: Experience from a Pediatric Nephrology Unit in North India.

Posttransplant lymphoproliferative disorder (PTLD) is reported in 1%-3% among pediatric renal allograft recipients. We report the experience of PTLD among pediatric renal allograft recipients at a pediatric nephrology center in North India. Four cases of PTLD were identified from among records of 95 pediatric renal allograft recipients over a period of 21 years.

Effect of atorvastatin on dyslipidemia and carotid intima-media thickness in children with refractory nephrotic syndrome: a randomized controlled trial.

Background: Dyslipidemia is an important cardiovascular risk factor in steroid-resistant nephrotic syndrome (SRNS). Efficacy of statins for treatment of hyperlipidemia in children with SRNS is unclear.

Methods: This prospective, randomized, double-blind, placebo-controlled, parallel-group clinical trial enrolled 30 patients with SRNS, aged 5-18 years, with serum low-density lipoprotein cholesterol (LDL-C) levels between 130 and 300 mg/dl, to receive a fixed dose of atorvastatin (n = 15, 10 mg/d) or placebo (n = 15) by block randomization in a 1:1 ratio.

Targeted exome sequencing in anti-factor H antibody negative HUS reveals multiple variations.

Background: Genetic susceptibility to atypical hemolytic uremic syndrome (aHUS) may lie within genes regulating or activating the alternate complement and related pathways converging on endothelial cell activation.

Methods: We tested 32 Indian patients of aHUS negative for antibodies to complement factor H for genetic variations in a panel of 15 genes, i.e.

Phenotype of Dent Disease in a Cohort of Indian Children.

Objective: To describe the clinical and genotypic features of Dent disease in children diagnosed at our center over a period of 10 years.

Design: Case series.

Setting: Pediatric Nephrology Clinic at a referral center in Northern India.

Experience with Continuous Renal Replacement Therapy.

Information on provision of continuous renal replacement therapy (CRRT) in critically ill children from developing countries is limited. The authors describe their experience in 17 children with hypotension and acute kidney injury (AKI) with fluid overload or electrolyte imbalance managed by 20 sessions of CRRT. The median (range) age and weight were 6 y (0.