Arterial connective tissue alterations in thalassemia major: a biochemical study of a case with severe iron overload.
A biochemical analysis of glycosaminoglycans and collagen was performed in arteries of a 15-year old teenager who died of beta thalassemia major. The patient presented the severe clinical complications resulting from hemochromatosis, which was evidenced at autopsy and by histological examination. The arteries under study comprised the thoracic and abdominal aortas and the iliac and pulmonary arteries, which were compared with the same arteries from normal individuals. Data on total glycosaminoglycan and total collagen, including the determination of the relative contents of the different glycosaminoglycans, suggest an as yet undescribed fibrotic process in the thalassemic arteries. Also altered were the proportions of the disaccharides making up chondroitin sulfate and heparan sulfate. A reduction in the molecular weight of arterial heparan sulfate, presumably with free radical involvement, was also detected. These changes in the extracellular matrix may be ascribed to the presence of large amounts of iron in the tissue, and as such they should be expected in other disorders with chronic iron overload.
Cardoso
L E
LE
Departamento de Bioquímica, Universidade Federal do Rio de Janeiro, Brasil.
Mourão
P A
PA
D000293:Adolescent; D001158:Arteries / Q000737:chemistry; D003094:Collagen / Q000032:analysis*; D003240:Connective Tissue Diseases / Q000209:etiology*; D004591:Electrophoresis, Polyacrylamide Gel; D006025:Glycosaminoglycans / Q000032:analysis*; D006801:Humans; D007501:Iron / Q000097:blood*; D008297:Male; D017086:beta-Thalassemia / Q000097:blood / Q000150:complications* / Q000473:pathology / Q000628:therapy
D006025:Glycosaminoglycans; D003094:Collagen; D007501:Iron