Justification: Steroid-sensitive nephrotic syndrome (SSNS) is one of the most common chronic kidney diseases in children. These guidelines update the existing Indian Society of Pediatric Nephrology recommendations on its management. Objective: To frame revised guidelines on diagnosis, evaluation, management, and supportive care of patients with the illness. Process: The guidelines combine evidence-based recommendations and expert opinion. Formulation of key questions was followed by review of literature and evaluation of evidence by experts in two face-to-face meetings. Recommendations: The initial statements provide advice for evaluation at onset and follow-up and indications for kidney biopsy. Subsequent statements provide recommendations for the management of the first episode of illness and of disease relapses. Recommendations on the use of immunosuppressive strategies in patients with frequent relapses and steroid dependence are accompanied by suggestions for step-wise approach and plan of monitoring. Guidance is also provided regarding the management of common complications including edema, hypovolemia, and serious infections. Advice on immunization and transition of care is given. The revised guideline is intended to improve the management and outcomes of patients with SSNS and provide directions for future research.

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. Process: The guidelines combine evidence-based recommendations and expert opinion. Formulation of key questions was followed by systematic review of literature, evaluation of evidence by experts, and two face-to-face meetings. Recommendations: Patients with SRNS should be managed under supervision of a pediatric nephrologist. Fourteen statements provide updated advice for defining steroid resistance, and underscore the importance of estimating proteinuria and baseline kidney function and the need for kidney biopsy and genetic screening. Calcineurin inhibitors are recommended as most effective in inducing remission of proteinuria, the chief factor associated with long-term renal survival. Advice on managing allograft recurrence, congenital nephrotic syndrome, and monitoring and supportive care, including the transition of care is described. This revised practice guideline is intended to improve management and patient outcomes and provide direction for future research.

Pediatric systemic lupus erythematous (SLE) is a multisystem disease chiefly affecting skin, kidneys, joints, and hematologic system. However, no organ of the body is immune to this condition. Ocular manifestations are seen in one third of adult patients. The most common ocular manifestation is keratoconjunctivitis sicca, whereas the most severe forms are optic neuritis and retinal vasculitis. An isolated unilateral ocular manifestation is a rare initial presentation of lupus. Here, we described a 9-year-old girl who presented to us with orbital preseptal cellulitis and was later diagnosed as diffuse proliferative lupus nephritis, which responded well to immunosuppression.

A 15-year-old child presented to our center with acute glomerulonephritis (GN) preceded by history of sore throat and fever. He had features of rheumatic carditis and echocardiographic evidence of severe eccentric mitral regurgitation and aortic regurgitation. Renal biopsy showed features consistent with diffuse proliferative GN. Simultaneous occurrence of acute GN and rheumatic fever in the same patient is very rare. He responded to treatment with steroids and renal function recovered over 3 months.

Atypical hemolytic–uremic syndrome (aHUS) in neonates is a rare entity. We report a neonate with aHUS presenting at 16 days of life. She was diagnosed to have a heterozygous pathogenic variant in the gene encoding factor H and was treated with repeated plasma infusions.