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[猎狐梗中与人类沃尔曼病相似的脂质贮积病]

[Lipid storage disease similar to Wolman's disease in humans in the fox terrier].

作者信息

von Sandersleben J, Hänichen T, Fiebiger I, Brem G

出版信息

Tierarztl Prax. 1986;14(2):253-63.

PMID:3738899
Abstract

In three young fox terriers a lipid storage disease is reported. This thesaurosis is almost identical to Wolman's disease in children. Clinically and pathologically hepatosplenomegaly is the most striking feature. Morphologically the disease is characterized by extensive lipid deposition mainly in liver, spleen, lymphnodes, intestinal mucosa, and bone marrow. Circular corneal lipid deposit (Arcus lipoides corneae) is of diagnostic significance. Some of the material has the typical structure of cholesterol crystals. Under polarized light the deposits consist of birefringent and non-birefringent lipids. All three dogs with lipid storage disease have one common ancestor. In addition their pedigrees reveal close relationship amongst the nearer forefathers and the affected animals themselves. From analogy with human Wolman's disease it is concluded, that in the Foxterrier, too, this lipid storage disease is caused by an inheritable deficiency of acid esterase. The mode of inheritance of this inborn lysosomal disease is probably autosomal recessive.

摘要

据报道,三只幼年猎狐梗患有一种脂质贮积病。这种贮积症与儿童的沃尔曼病几乎完全相同。临床和病理上,肝脾肿大是最显著的特征。形态学上,该病的特征是广泛的脂质沉积,主要在肝脏、脾脏、淋巴结、肠黏膜和骨髓中。角膜环状脂质沉积(角膜脂环)具有诊断意义。部分物质具有胆固醇晶体的典型结构。在偏振光下,沉积物由双折射和非双折射脂质组成。所有三只患有脂质贮积病的狗都有一个共同的祖先。此外,它们的谱系显示,较近的祖先与患病动物本身之间关系密切。通过与人类沃尔曼病类比得出结论,在猎狐梗中,这种脂质贮积病也是由酸性酯酶的遗传性缺乏引起的。这种先天性溶酶体疾病的遗传方式可能是常染色体隐性遗传。

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