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多腺体缺陷综合征:波斯犹太人中的一种新变体。

The polyglandular deficiency syndrome: a new variant in Persian Jews.

作者信息

Shapiro M S, Zamir R, Weiss E, Radnay J, Shenkman L

出版信息

J Endocrinol Invest. 1987 Feb;10(1):1-7. doi: 10.1007/BF03347139.

Abstract

Five Persian Jews were detected with the polyglandular deficiency syndrome (PDS). Primary hypoparathyroidism and hypogonadism were present in each, adrenal insufficiency in two, and insulin-dependent diabetes mellitus and latent hypothyroidism in single subjects. The percentage of T and B cells, and the mononuclear cell response to phytohemagglutinin and Concanavalin A were normal in all five. IgG and IgA levels and the OKT4+/OKT8+ cell ratio were low in one subject. Antinuclear and antithyroid antibodies were present in one subject. HLA-DR5 was present in 4/4, HLA-24 and B5 (B51) in 3/4 subjects. A single case of isolated hypoparathyroidism (IHP) was detected among 12 first degree relatives. HLA antigens B8, DR3, were absent in all of these subjects. Seven non-Iranian Jews with IHP were also examined. HLA A26 or A25 were present in all seven. Persian Jews appear to have a unique variant of PDS.

摘要

发现五名波斯犹太人患有多腺体功能减退综合征(PDS)。每个人都存在原发性甲状旁腺功能减退和性腺功能减退,两人有肾上腺功能不全,单个人有胰岛素依赖型糖尿病和潜在甲状腺功能减退。所有五人的T细胞和B细胞百分比以及单核细胞对植物血凝素和刀豆球蛋白A的反应均正常。一名受试者的IgG和IgA水平以及OKT4 + / OKT8 +细胞比率较低。一名受试者存在抗核抗体和抗甲状腺抗体。4/4的受试者存在HLA - DR5,3/4的受试者存在HLA - 24和B5(B51)。在12名一级亲属中检测到一例孤立性甲状旁腺功能减退(IHP)。所有这些受试者均不存在HLA抗原B8、DR3。还检查了七名患有IHP的非伊朗犹太人。所有七人都存在HLA A26或A25。波斯犹太人似乎有一种独特的PDS变体。

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