Immunoglobulin Levels in Patients with Carbohydrate-Deficient Glycoprotein Syndrome Type I
| Autor: | B. Kristiansson, J.E.M. Björklund, S.G.O. Johansson, H. Stibler, C.G.M. Magnusson |
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| Rok vydání: | 1997 |
| Předmět: |
Adult
Male medicine.medical_specialty Adolescent Immunoglobulin levels Immunology Immunoglobulin E Congenital Disorders of Glycosylation Reference Values Internal medicine medicine Humans Immunology and Allergy In patient Child Immunoglobulin Allotypes Glycoproteins Sweden chemistry.chemical_classification biology Carbohydrate-deficient glycoprotein syndrome Infant General Medicine Middle Aged Allotype Immunoglobulin Isotypes Endocrinology chemistry Child Preschool biology.protein Female Antibody Glycoprotein |
| Zdroj: | International Archives of Allergy and Immunology. 114:116-119 |
| ISSN: | 1423-0097 1018-2438 |
| DOI: | 10.1159/000237655 |
| Popis: | The characteristic feature of carbohydrate-deficient glycoprotein syndrome (CDGS) type I, a multisystemic disease, is underglycosylation of many serum glycoproteins, such as transferrin. A few cases of severe infections during childhood have been reported and an underlying immunodeficiency has been suggested. Because of this and the fact that all immunoglobulin (Ig) isotypes are glycoproteins we analysed the Ig levels in patients with CDGS I.The serum concentrations of IgG1, IgG2, IgG3, IgG4, IgA, IgM, IgD and IgE, and the frequency of the G2m(23) allotype were measured by enzyme immunoassay in 15 patients with CDGS type I.Ten (67%) patients had an elevated level of at least one Ig, when compared to age-related reference ranges. No particular isotype was involved although a tendency towards high IgE levels was registered. The frequency of homozygous G2m(23)-negative CDGS patients (33%) was not different from that of blood donors (34%).We conclude that CDGS I patients have no major changes in the serum levels of any specific Ig isotype. The severe infections observed in some CDGS patients are therefore unlikely to involve any Ig deficiency. Our results do not exclude that Ig of patients with CDGS may have altered physiological functions because of abnormal glycosylation. |
| Databáze: | OpenAIRE |
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