Focal glomerulosclerosis in children: an Argentinian experience

Twenty-six children presenting with idiopathic nephrotic syndrome and a histological diagnosis of focal glomerulosclerosis were studied retrospectively to evaluate their response to treatment, outcome and clinicopathological correlations. Twenty-two patients (84.6%) were steroid resistant; of these,...

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Published inPediatric nephrology (Berlin, West) Vol. 6; no. 2; p. 158
Main Authors Tufro-McReddie, A, Alvarez, E, Arrizurieta, E, Repetto, H
Format Journal Article
LanguageEnglish
Published Germany 01.03.1992
Subjects
Adolescent
Argentina
Biopsy
Child
Child, Preschool
Cyclophosphamide - therapeutic use
Drug Resistance
Female
Follow-Up Studies
Glomerulosclerosis, Focal Segmental - drug therapy
Glomerulosclerosis, Focal Segmental - pathology
Humans
Infant
Longitudinal Studies
Male
Nephrotic Syndrome - drug therapy
Nephrotic Syndrome - pathology
Prednisone - therapeutic use
Retrospective Studies
Treatment Outcome
Argentina
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Summary:Twenty-six children presenting with idiopathic nephrotic syndrome and a histological diagnosis of focal glomerulosclerosis were studied retrospectively to evaluate their response to treatment, outcome and clinicopathological correlations. Twenty-two patients (84.6%) were steroid resistant; of these, 8 of the 19 with focal segmental glomerulosclerosis and 2 of the 3 with focal global glomerulosclerosis responded to cyclophosphamide (CY) within 16 weeks of starting therapy. Seven patients relapsed after a CY-induced remission, but 5 of them became steroid responsive. After an average follow-up of 83 months, 17 patients are in remission with normal renal function, 3 patients have persistent nephrotic range proteinuria and 6 patients are in chronic renal failure. Persistence of proteinuria, a high percentage of segmentally sclerotic glomeruli and diffuse mesangial proliferation were indicators of poor prognosis. We believe longer courses of CY therapy than those traditionally utilized are responsible for the relatively good results in our patients.
ISSN:0931-041X
DOI:10.1007/BF00866298