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Secondary amyloidosis in glycogen storage disease type Ib
M Kikuchi1, K Haginoya, S Miyabayashi
1Department of Paediatrics, Tohoku University School of Medicine, Sendai, Japan.
European Journal of Pediatrics
|February 1, 1990
Abstract:
We observed the development of generalized amyloidosis in a girl with glycogen storage disease type Ib (GSD-Ib) who showed neutropenia, neutrophil dysfunction and recurrent infections. Renal and thyroid biopsies showed secondary amyloidosis, characterized by the presence of potassium permanganate sensitive Dylon positive deposits in glomeruli, renal vessels and thyroid interstitium. Immunohistochemistry showed that the deposits were composed of amyloid A (AA) protein. Possibly neutrophil abnormalities are involved in the pathogenesis of amyloidosis.