Secondary amyloidosis in glycogen storage disease type Ib
- 1 February 1990
- journal article
- research article
- Published by Springer Nature in European Journal of Pediatrics
- Vol. 149 (5) , 344-345
- https://doi.org/10.1007/bf02171563
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.Keywords
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