A Case of Fabry's Disease with Congenital Agammaglobulinemia
نویسندگان
چکیده
منابع مشابه
A Case of Fabry's Disease with Congenital Agammaglobulinemia
Fabry's disease is an X-linked lysosomal storage disorder caused by abnormalities in the α-galactosidase A (GLA) gene, which leads to a GLA deficiency and to the intracellular deposition of globotriaosylceramide (Gb3) within vascular endothelium and other tissues. It manifests as progressive multiple organ dysfunctions caused by the deposition of Gb3. On the other hand, congenital agammaglobuli...
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15 صفحه اولChest disease in patients with agammaglobulinemia.
As a new disease entity is studied it becomes apparent that certain clinical and laboratory features are particularly helpful to the clinician in the recognition of the disorder. A consideration of the recent literature indicates that recurrent pulmonary disease has assumed this role in the disease syndrome associated with agammaglobulinemia. Recurrent pulmonary infections including bronchitis,...
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ژورنال
عنوان ژورنال: Journal of Korean Medical Science
سال: 2011
ISSN: 1011-8934,1598-6357
DOI: 10.3346/jkms.2011.26.7.966