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伴有血清脂蛋白电泳图前β-组分缺失的黄斑部蝶形营养不良(作者译)

[Butterfly-shaped dystrophy of the macula with absent pre-beta-fraction of the electropherogram of the serum lipoproteins (author's transl)].

作者信息

Raitta C, Erkkilä H

出版信息

Klin Monbl Augenheilkd. 1979 Apr;174(4):561-6.

PMID:222937
Abstract

A case of butterfly-shaped dystrophy of the macula is described. Clinical and electrophysiological data are compared with those of 11 cases described in the literature. An aberrant electropherogram of the lipoproteins examined at two different laboratories was found to be present. The serum lipoproteins showed total lack of the pre-beta-fraction. Two children of the patient were examined, too. They showed minimal macular changes which however did not justify the diagnosis of butterfly-shaped dystrophy. Their pre-beta-lipoprotein fractions of the electropherogram were below the Finish standards. The proband is the first case of butterfly-shaped macular dystrophy described from Finland and therefore it was not possible to control the laboratory findings on other cases. The absence of the pre-beta-lipoprotein fraction might be an important feature of butterfly-shaped dystrophy of the macula.

摘要

本文描述了一例蝴蝶状黄斑营养不良病例。将临床和电生理数据与文献中描述的11例病例的数据进行了比较。发现在两个不同实验室检测的脂蛋白电泳图谱异常。血清脂蛋白显示前β-组分完全缺失。对该患者的两个孩子也进行了检查。他们的黄斑变化极小,但不足以诊断为蝴蝶状营养不良。他们的电泳图谱前β-脂蛋白组分低于芬兰标准。该先证者是芬兰描述的首例蝴蝶状黄斑营养不良病例,因此无法对照其他病例验证实验室检查结果。前β-脂蛋白组分的缺失可能是蝴蝶状黄斑营养不良的一个重要特征。

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