Publication: A progressive ANCA associated glomerulonephritis leading to chronic kidney disease and stroke in a child with congenital heart disease: a case report
Date
2025
Authors
Jha, Ashish
Shah, Shiv Kumar
Thakur, Baidyanath
Mandal, Abhay
Thakur, Jitendra
Journal Title
Journal ISSN
Volume Title
Publisher
Madhesh Institute of Health Sciences (MIHS)
Abstract
ABSTRACT 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.
Description
A 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 Thakur1