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Immunoglobulin deficiency with increased immunoglobulin M in three siblings: effect of long-term immunoglobulin therapy.

Abstract：

:Diagnosis of immunoglobulin deficiency with increased IgM (hyper-IgM syndrome) was made in three siblings (two girls and a boy) on the basis of history, physical findings, and laboratory data. The prominent clinical findings were recurrent viral and bacterial infections of the respiratory tract. The most severe infections affected the male patient, who died at the age of 8 years. Family history and the lack of clinical signs in the parents and relatives indicated no immunodeficiency which, together with the occurrence of the disease in both sexes, indicated an autosomal recessive inheritance. The two female patients (18 years old and 3 years old) have been treated with intravenous acid-treated immunoglobulin for 2 years, resulting in significant clinical improvement with respect to the frequency and severity of infections.

journal_name

Eur J Pediatr

journal_title

European journal of pediatrics

authors

Maródi L,Szabó I,Kalmár A

doi

10.1007/BF00441406

subject

Has Abstract

pub_date

1988-12-01 00:00:00

pages

215-7

issue

3

eissn

0340-6199

issn

1432-1076

journal_volume

148

pub_type

杂志文章

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