Clinical spectrum and outcome of crescentic glomerulonephritis in children in developing countries |
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Authors: | Deepak Dewan Sanjeev Gulati Raj K Sharma Narayan Prasad Manoj Jain Amit Gupta Alok Kumar |
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Institution: | (1) Department of Nephrology, Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Rae Bareily Road, Lucknow, 226014, India;(2) Department of Pathology, Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Lucknow, India |
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Abstract: | Crescentic glomerulonephritis (CsGN) is an uncommon entity in children. This prospective study was conducted to evaluate the
aetiology, clinical spectrum and outcome in children with crescentic glomerulonephritis. The single-centre prospective study
comprised of 22 children with biopsy proven CsGN who had been referred to our institute over the period January 2000 to December
2005. These patients were subjected to detailed clinical and biochemical examinations. The diagnosis of underlying renal disease
was based on various criteria, including the clinical picture, serology and histopathology. The patients received intravenous
methyl prednisolone, oral steroid treatment, and oral cyclophosphamide with or without plasmapheresis. All patients received
supportive care, including control of hypertension and oedema and supportive management of renal insufficiency. During this
5-year period, CsGN accounted for 5.1% of all biopsies done in children. The mean age was 12.27 years (range 4 years to 18 years).
There were eight girls and 14 boys. The mean duration of symptoms prior to referral was 2.47 months (range 5 days to 21 months).
Aetiology was immune complex in 19 cases, anti-glomerular basement membrane (anti-GBM) antibody disease in two cases and pauci-immune
(Wegener’s granulomatosis) in one case. The percentage of crescents ranged from 50% to 100% (mean 70.6%). Twenty-one out of
22 (95.5%) children in our series had hypertension at presentation that required treatment with antihypertensive medications.
The serum creatinine level at presentation ranged from 1.5 mg/dl to 11.4 mg/dl (mean 5.5 mg/dl). Of the 22 children, two were
lost to follow-up, while the mean follow-up period of the rest of the 20 children was 8.13 months (range 1 month to 43 months).
At the last follow-up of the 22 children, ten had stage 5 chronic kidney disease (CKD) and three had stage 4 CKD, while seven
children had a calculated glomerular filtration rate (GFR) of >60 ml/min per 1.73 m2 body surface area. Persistent proteinuria was seen on follow-up in the majority 13/20 (65%)] of patients. The outcome of
CsGN in children continues to be poor, in our experience, due to delayed referral and delayed diagnosis. This was correlated
histologically by the presence of fibrocellular crescents in the majority of our patient. Thus CsGN should be treated as a
renal emergency. A greater awareness of this disease needs to be created amongst the referring paediatricians in developing
countries to facilitate early diagnosis and prompt treatment. |
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Keywords: | Crescentic glomerulonephritis Chronic kidney disease Proteinuria |
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