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Secondary amyloidosis in glycogen storage disease type Ib (1990)

Kikuchi, M. ; Haginoya, K. ; Miyabayashi, S. ; [et al.]

Springer

European journal of pediatrics 149 (1990), S. 344-345

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ISSN: 1432-1076

Keywords: Glycogen storage disease Ib ; Secondary amyloidosis

Source: Springer Online Journal Archives 1860-2000

Topics: Medicine

Notes: 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.

Type of Medium: Electronic Resource

URL: http://dx.doi.org/10.1007/BF02171563

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