WISKOTT-ALDRICH SYNDROME - A STUDY OF 6 CASES WITH DETERMINATION OF IMMUNOGLOBULINS A D G M AND ND

被引：88

作者：

BERGLUND, G

FINNSTRO.O

JOHANSSO.SG

MOLLER, KL

机构：

[1] Department of Pediatrics, University of Gothenburg

[2] the Department of Pediatrics, University of Umeå

[3] the Blood Center, University Hospital, Uppsala

来源：

ACTA PAEDIATRICA SCANDINAVICA | 1968年 / 57卷 / 02期

关键词：

immunoglobulins; Wiskott‐Aldrich syndrome;

D O I：

10.1111/j.1651-2227.1968.tb04658.x

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Six cases with Aldrich syndrome are presented, one of them splenectomized at the age of 3, died at the age of 6 years of adrenal haemorrhage. Another patient died at the age of 6 years of pneumonia with empyema and meningitis. The other 4 patients (1/2–6 years) are alive in a fairly good condition. Bleeding tendency dominates the symptoms in 2 cases, in the other 2, susceptibility to infections and/or eczema are the main symptoms. The bleeding tendency does not seem to be correlated to the number of platelets. Immunological studies in 5 of the patients have revealed a normal or high content of IgG in 4 of 5 cases and an increase of IgA in all. In 3 of 4 investigated patients elevated concentrations were demonstrated of a new immunoglobulin class, IgND. The immune response is changed; antibody production by vaccinations against viral diseases was poor, tuberculin reactions after BCG vaccination were negative, the pharyngeal lymphoid tissue was absent, and in 4 of the 5 investigated cases low titers of isoagglutinins were demonstrated. Copyright © 1968, Wiley Blackwell. All rights reserved

引用

页码：89 / &

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