Jha, AshishShah, Shiv KumarThakur, BaidyanathMandal, AbhayThakur, Jitendra2025-08-172025-08-172025https://hdl.handle.net/20.500.14572/1702A progressive ANCA associated glomerulonephritis leading to chronic kidney disease and stroke in a child with congenital heart disease: a case report Ashish Jha1,*, Shiv Kumar Shah1, Baidyanath Thakur1, Abhay Mandal1, Jitendra Thakur1ABSTRACT Nephrotic-nephritic syndrome in children is a rare but significant cause of chronic kidney disease (CKD). This case highlights a challenging diagnostic and therapeutic course involving persistent hematuria, ANCA positiv- ity, refractory hypertension, and cerebrovascular complications. A 10-year-old female, post-patent ductus ar- teriosus (PDA) closure, presented with generalized swelling, hematuria, and proteinuria. Initial workup sug- gested post-infectious glomerulonephritis (PIGN) with persistently low complement levels (C3, C4), and she was managed with diuretics. However, recurrent episodes led to further evaluation, revealing PR3-ANCA positivity, nephrotic-range proteinuria, and progressive renal dysfunction. Despite treatment, she developed refractory hypertension and an intracranial hemorrhage. The absence of renal biopsy posed challenges in definitive diag- nosis, and the patient is now on dialysis. This case underscores the diagnostic challenges in differentiating between PIGN, ANCA-associated glomerulo- nephritis, and rapidly progressive glomerulonephritis (RPGN) in pediatric patients. The interplay between con- genital heart disease and renal dysfunction highlights the need for interdisciplinary management. Early biopsy in recurrent nephrotic-nephritic cases is crucial to prevent irreversible renal damage. The case emphasizes the need for early renal biopsy and comprehensive management of pediatric nephrotic-nephritic syndrome with persistent proteinuria and hematuria.en-USArticle