Combined immunodeficiency with hyper-gamma-globulinaemia.

Author:

Webster A D,Slavin G,Strelling M K,Asherson G L

Publisher

BMJ

Subject

Pediatrics, Perinatology and Child Health

Reference18 articles.

1. Duodenal ulcers in children;Robb, J.D.A.; Orszulok, T.J.; Odling-Smee, G.W.;Archives of Disease in Childhood,1972

2. Infants with major defects in cell-mediated immunity and hypo-y-globulinaemia (severe combined immunodeficiency) usually die in the first few years of life from infections unless they receive a successful bone marrow transplant;Nezelof et al; described an infant with a major defect in cell-mediated immunity and normal serum immunoglobulin levels who died at 16 months from a lung infection. This paper describes a similar infant with absent cell-mediated immunity and hyper-y-globulinaemia in whom we were unable to show any functional antibody,1964

3. It is therefore possible that the excessive production of IgA in our patient was related to the absence of suppressor T lymphocytes;raised levels of serum IgA (Humphrey, Parrott; East, 1964

4. Discussion munoglobulins does not rule out a severe and The clinical course, normal serum immunoglobulin levels, gross deficiency in cell-mediated immunity, and the presence of enlarged mediastinal lymph nodes containing plasma cells parallels the case described by Nezelof;et al.,;et al; The WHO Committee on Primary Immunodeficiency (Fudenberg,1964

5. Summary cently been suggested by Lawlor;et al; that An infant is described with absence of cell this condition warrants a separate classification. mediated immunity and apparent failure to produce It is likely that the rise in serum immunoglobulins functional antibody despite raised serum imwas related to the Pn. carinii infection, and intense munoglobulin concentrations. A marked rise in antigenic stimulation from the lungs probably serum IgA occurred during the terminal phase of accounts for the plasma cell infiltrate of the mediahis illness. We draw attention to this subgroup stinal lymph nodes. The hyper-y-globulinaemia, of severe combined immunodeficiency where there particularly the rise in IgA, was clearly out of is a tendency to spontaneously develop hyper-yproportion to that seen in normal individuals after globulinaemia,1974

Cited by 8 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. A practical approach to neutrophil disorders;Pediatric Clinics of North America;2002-10

2. Severe combined immunodeficiency with T lymphocytes retaining functional activity;Clinical Immunology and Immunopathology;1988-03

3. The role of eosinophil in regulation of granulopoiesis;Hematological Oncology;1986-10

4. Laboratory Investigation of Primary Deficiency of the Lymphoid System;Clinics in Immunology and Allergy;1985-10

5. Familial bronchiectasis;The Journal of Pediatrics;1983-02

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