Premium
WISKOTT‐ALDRICH SYNDROME A Study of 6 Cases with Determination of the Immunoglobulins A, D, G, M and ND
Author(s) -
Berglund G.,
Finnström O.,
Johansson S. G. O.,
Möller K. L.
Publication year - 1968
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1968.tb04658.x
Subject(s) - medicine , wiskott–aldrich syndrome , immunology , antibody , titer , vaccination , meningitis , pneumonia , pediatrics , biochemistry , chemistry , gene
Summary 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.