| Literature DB >> 2311631 |
M Kikuchi1, K Haginoya, S Miyabayashi, Y Igarashi, K Narisawa, K Tada.
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.Entities:
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Year: 1990 PMID: 2311631 DOI: 10.1007/bf02171563
Source DB: PubMed Journal: Eur J Pediatr ISSN: 0340-6199 Impact factor: 3.183