Acute renal failure (ARF) is an uncommon but alarming complication of idiopathic nephrotic syndrome. The renal failure could be secondary to causes evident from the history and evaluation, such as severe intravascular volume depletion, acute tubular necrosis, allergic interstitial nephritis, bilateral renal vein thrombosis, acute pyelonephritis, or rapid progression of the original glomerular disease. It may be termed idiopathic if the underlying cause is undetermined. We present three children with idiopathic nephrotic syndrome who were admitted with acute renal failure. One case was due to drug-induced allergic interstitial nephritis. The other two were idiopathic in nature. Improvement in renal function occurred in the three patients over a variable period of 10 days to 4 weeks. After careful exclusion of well-known causes of acute renal failure, idiopathic acute renal failure (IARF) should be considered as a diagnostic possibility in these patients. The exact pathophysiology of IARF is not understood. Possible proposed explanations include interstitial edema, tubular obstruction, altered glomerular permeability, and unrecognized hypovolemia.

V esicoureteric reflux (VUR) is an important predisposing factor for urinary tract infection (UTI) and renal parenchymal scarring [1]. VUR describes retrograde flow of urine from urinary bladder into ureter and kidney. VUR is called primary when it is an isolated finding and referred to as secondary, when associated with other urological anomalies such as posterior urethral valve, renal agenesis, and multicystic dysplastic kidney [1].Accurate prevalence of VUR is not known as many are asymptomatic and diagnostic testing is performed only when clinically indicated. About 30-40% of children and 40-50% of neonates with UTI have VUR [2]. Of children with primary VUR, 30-50% may develop renal parenchymal scarring and risk of scarring is reported to be highest in young children [2]. About 5-10% of children with reflux nephropathy may progress to chronic kidney disease (CKD) in late childhood. Reflux nephropathy is also the most common cause of severe hypertension and CKD in childhood and adolescence [2]. The objectives were to study the clinical profile of children with primary VUR and to assess the relationship with grades of primary VUR and renal parenchymal scarring. MATERIALS AND METHODSThis was a hospital-based prospective observational study conducted over a period of 18 months, from December 2017 to June 2019. Fifty children between 1 month and 5 years of age,

Background: Cyclic neutropenia (CyN) is a rare inherited neutrophil disease characterized by a periodic neutropenia with an absolute neutrophil count (ANC) dropping below 0.5 × 109/L every 3–4 weeks, followed by normalization over the next few days. Clinical Description: A 3-year-old boy presented with recurrent episodes of fever, respiratory tract infections, diarrhea, gingivitis, and oral and cutaneous ulcers from 11 months of age, requiring multiple hospitalizations. He was immunized for age, normal development, with an unremarkable family history. An underlying primary immunodeficiency disorder was suspected in view of recurrent multi-site infections since infancy. Management: The hemogram revealed a normal total leukocyte count with severe, neutropenia, lymphocytosis, normal platelet count and severe anemia. Purified protein derivative test and human immunodeficiency virus test were negative. The chest Xray showed right lower lobe consolidation. Blood culture was sterile. Bone marrow study, immunoglobulin profile, and lymphocyte subtyping were within the normal limits. The child was treated symptomatically with parenteral antibiotics and packed red blood cells transfusion. The ANC increased to 2592/mm3 but, again fell by 27 days after admission. Review of previous blood counts showed a fall and rise of ANC every 3–5 weeks. Clinical exome sequencing revealed a heterozygous mutation in the ELANE gene, a pathogenic variant c. 1A>G (p.Met1), confirming the diagnosis of CyN. The child improved with Granulocyte – colony stimulating factor (G-CSF). Conclusion: Diagnosis of CyN can be challenging as it needs a high index of suspicion and meticulous monitoring of blood counts. If suspected, they should be screened for ELANE mutations.