Renal Granulomatous sarcoidosis in childhood : a report of 11 cases and a review of the literature

We analysed retrospectively 11 children with renal granulomatous sarcoidosis confirmed by renal histology in order to describe the course and prognosis of the disease. Symptomatic sarcoidosis was diagnosed at a mean age of 10.1 years. Nine children had renal involvement at the time of diagnosis. In...

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Veröffentlicht in:European journal of pediatrics 1999-02, Vol.158 (2), p.154-159
Hauptverfasser: COUTANT, R, LEROY, B, NIAUDET, P, LOIRAT, C, DOMMERGUES, J. P, ANDRE, J. L, BACULARD, A, BENSMAN, A
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container_end_page 159
container_issue 2
container_start_page 154
container_title European journal of pediatrics
container_volume 158
creator COUTANT, R
LEROY, B
NIAUDET, P
LOIRAT, C
DOMMERGUES, J. P
ANDRE, J. L
BACULARD, A
BENSMAN, A
description We analysed retrospectively 11 children with renal granulomatous sarcoidosis confirmed by renal histology in order to describe the course and prognosis of the disease. Symptomatic sarcoidosis was diagnosed at a mean age of 10.1 years. Nine children had renal involvement at the time of diagnosis. In the course of the disease, nine patients developed renal failure and mild proteinuria, seven had transient sterile leukocyturia, four showed microscopic haematuria, seven had a urinary concentrating defect, and enlarged kidneys were seen in three patients. One child had hypercalcaemia and hypercalciuria, none had hypertension. Light microscopy of the kidney showed interstitial infiltration by mononuclear cells in all children, interstitial fibrosis in nine patients, epithelioid granulomas in seven, tubular involvement in eight, and mild glomerular involvement in seven patients. Renal immunofluorescence was negative. Ten children received prednisone for 1-11 years. After a mean follow up of 5.5 years, three patients had entered end-stage renal failure and one had chronic insufficiency after interruption of medical supervision and prednisone therapy. Renal failure, proteinuria, leukocyturia, haematuria, and concentration defect are the prominent features of renal granulomatous sarcoidosis in children. Steroid therapy, adjusted according to disease activity, may prevent end-stage renal failure.
doi_str_mv 10.1007/s004310051038
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In the course of the disease, nine patients developed renal failure and mild proteinuria, seven had transient sterile leukocyturia, four showed microscopic haematuria, seven had a urinary concentrating defect, and enlarged kidneys were seen in three patients. One child had hypercalcaemia and hypercalciuria, none had hypertension. Light microscopy of the kidney showed interstitial infiltration by mononuclear cells in all children, interstitial fibrosis in nine patients, epithelioid granulomas in seven, tubular involvement in eight, and mild glomerular involvement in seven patients. Renal immunofluorescence was negative. Ten children received prednisone for 1-11 years. After a mean follow up of 5.5 years, three patients had entered end-stage renal failure and one had chronic insufficiency after interruption of medical supervision and prednisone therapy. Renal failure, proteinuria, leukocyturia, haematuria, and concentration defect are the prominent features of renal granulomatous sarcoidosis in children. Steroid therapy, adjusted according to disease activity, may prevent end-stage renal failure.</abstract><cop>Heidelberg</cop><cop>Berlin</cop><pub>Springer</pub><pmid>10048615</pmid><doi>10.1007/s004310051038</doi><tpages>6</tpages></addata></record>
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subjects Adolescent
Biological and medical sciences
Biopsy
Calcium Metabolism Disorders - complications
Calcium Metabolism Disorders - diagnosis
Child
Child, Preschool
Chronic Disease
Female
Follow-Up Studies
Granuloma - complications
Granuloma - diagnosis
Humans
Kidney - pathology
Kidney Diseases - complications
Kidney Diseases - diagnosis
Kidney Failure, Chronic - diagnosis
Kidney Failure, Chronic - etiology
Literature reviews
Male
Medical sciences
Retrospective Studies
Sarcoidosis - complications
Sarcoidosis - diagnosis
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
title Renal Granulomatous sarcoidosis in childhood : a report of 11 cases and a review of the literature
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