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Alport's Hereditary Nephritis with the Nephrotic Syndrome
Author(s) -
Okada Kaname,
Shimokawa Tatsuo,
Ichioka Takao,
Yano Ichiro,
Kawakami Koichiro,
Kagami Shoyoji,
Miyao Masuhide
Publication year - 1980
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.1980.tb00534.x
Subject(s) - mesangium , medicine , pathology , nephritis , thickening , nephrotic syndrome , glomerular mesangium , interstitial nephritis , renal biopsy , glomerular basement membrane , glomerulonephritis , biopsy , kidney , chemistry , polymer science
Histopathological changes were studied in renal biopsy specimens from a ten‐year‐old boy diagnosed as having Alport's hereditary nephritis with the nephrotic syndrome. Biopsy specimens of the kidney showed mild hypercellularity with an increase of mesangial matrix, irregular thickening of the capillary walls in the glomeruli, and numerous clusters of interstitial foam cells by light microscopy. Granular deposits of IgG, IgM, C3 and fibrinogen were seen along the capillaries and partially in the mesangium by immunofluoroscopy. Ultrastructurally, the lesions had various abnormalities of the glomerular basement membrane, including splitting, thinning, disruption, reticulation and irregular thickening. The mesangial matrix was increased and intramembranous deposits were observed. Foam cells were seen among both tubular and interstitial cells.