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血管样条纹与异常血红蛋白病——一种新发现的关联。

Angioid streaks and AC hemoglobinopathy--a newly discovered association.

作者信息

McBrayer G M, Semes L, Stephens G G

机构信息

UAB School of Optometry, Birmingham, AL 35294.

出版信息

J Am Optom Assoc. 1993 Apr;64(4):250-3.

PMID:8315200
Abstract

It is estimated that approximately 2 percent of African-Americans possess one gene for C hemoglobin. This is usually demonstrated on hemoglobin electrophoresis. Hemoglobin C trait (AC hemoglobinopathy) is considered a clinically benign condition; there are no characteristic physical abnormalities. Angioid streaks in the ocular fundus represent a progressive, bilateral, degenerative disorder characterized by breaks in Bruch's membrane. The pathogenesis of angioid streaks in the hemoglobinopathies is thought to be linked to iron deposition in Bruch's membrane. This report describes what is believed to be the first reported case of angioid streaks associated with hemoglobin C trait.

摘要

据估计,约2%的非裔美国人携带一个C型血红蛋白基因。这通常可通过血红蛋白电泳检测出来。血红蛋白C性状(AC血红蛋白病)被认为是一种临床良性病症;没有特征性的身体异常表现。眼底的血管样条纹是一种进行性、双侧性、退行性疾病,其特征为布鲁赫膜破裂。血红蛋白病中血管样条纹的发病机制被认为与铁在布鲁赫膜中的沉积有关。本报告描述了据信是首例与血红蛋白C性状相关的血管样条纹病例。

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