Crescentic glomerulonephritis in children: A retrospective review of data from Chris Hani Baragwanath Academic Hospital

Abstract

Background. Crescentic glomerulonephritis (CGN) as a cause of progressive renal failure is rare. Crescent formation on kidney biopsy represents a response to injury of the glomerular capillary walls. There are limited published reports on paediatric CGN in Africa.

Objectives. To describe the clinical presentation and outcome of children with CGN in Soweto, South Africa, over a 22-year period.

Methods. A retrospective study was conducted at the Paediatric Renal Unit at Chris Hani Baragwanath Academic Hospital. Children younger than 14 years with crescent formation in more than 50% of glomeruli on renal biopsy were included in the study. Kidney biopsy specimens were examined by light microscopy, immunofluorescence and electron microscopy. Demographic and clinical data were extracted from the patient files.

Results. During the study period, 961 kidney biopsies were performed. Fourteen patients (1.5%) met inclusion criteria of crescents in >50% of glomeruli. Common clinical findings were oedema (n=13; 93%), microscopic haematuria (n=12; 86%), hypertension (n=11; 79%) and proteinuria (n=10; 71%). The median estimated glomerular filtration rate (eGFR) at presentation was 23.9 mL/min/1.73m². Thirteen patients (93%) had immune-complex-mediated glomerulonephritis. The underlying cause was acute post-infectious glomerulonephritis in 9 patients (64%), membranoproliferative glomerulonephritis and IgA nephropathy in 2 patients each (14%) and global sclerosis in 1 patient (7%). Prolonged duration of symptoms resulted in a lower eGFR at follow-up. Treatment included peritoneal dialysis, methylprednisolone and cyclophosphamide. Seven (54%) patients had a normal eGFR at a median (range) follow-up of 36.7 (4.5 - 61.5) months. Six (46%) patients had progressed to chronic kidney disease stages 2 - 5. One patient was followed up for less than 3 months, and therefore was not included in the follow-up results.

Conclusion. Poor outcomes were observed in patients who presented late. The clinical findings of haematuria, hypertension and acute kidney injury warrant early referral and kidney biopsy to determine management.