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Secondary amyloidosis in glycogen storage disease type Ib.

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.
AuthorsM Kikuchi, K Haginoya, S Miyabayashi, Y Igarashi, K Narisawa, K Tada
JournalEuropean journal of pediatrics (Eur J Pediatr) Vol. 149 Issue 5 Pg. 344-5 (Feb 1990) ISSN: 0340-6199 [Print] Germany
PMID2311631 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Dimethyl Sulfoxide
Topics
  • Amyloidosis (drug therapy, etiology)
  • Child
  • Dimethyl Sulfoxide (therapeutic use)
  • Female
  • Glycogen Storage Disease Type I (complications)
  • Humans
  • Neutropenia (etiology)

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