Follow-up study of immune defects in patients with dysmorphic disorders
| Autor: | Sanaa A. Mahmoud, Mary Lowery-Nordberg, Harold Chen, Sami L. Bahna, Kristin Berlin, Narlito V. Cruz |
|---|---|
| Rok vydání: | 2009 |
| Předmět: |
Adult
Antigens Differentiation T-Lymphocyte Male Pulmonary and Respiratory Medicine medicine.medical_specialty Down syndrome Adolescent T-Lymphocytes Immunology Turner Syndrome Chromosome Disorders Lymphocyte Activation Gastroenterology Blood cell Young Adult Immune system Antigen Antigens CD Immunopathology Internal medicine Turner syndrome medicine Humans Immunology and Allergy Lectins C-Type Young adult Child Aged business.industry Immunologic Tests Genetic Diseases Inborn Middle Aged medicine.disease medicine.anatomical_structure Immune System Diseases Child Preschool Immunoglobulin G Female Down Syndrome business |
| Zdroj: | Annals of Allergy, Asthma & Immunology. 102:426-431 |
| ISSN: | 1081-1206 |
| DOI: | 10.1016/s1081-1206(10)60516-9 |
| Popis: | Background In a previous study, we noted immunologic abnormalities in 46 (54.8%) of 84 individuals with dysmorphic disorders. Objective To reevaluate patients with dysmorphic disorders and immunologic abnormalities 2 to 3 years after an initial study to determine any changes in those abnormalities. Methods Information was gathered regarding significant infections during the previous 12 months. Blood samples were drawn for the immunologic tests that were previously performed (IgG, IgA, and IgM level determinations; complete blood cell count; and lymphocyte subset enumeration) and for determination of IgG subclasses and T-cell activation by CD69 expression. Results In the 21 patients available, 26 (63.4%) of the previously noted 41 low immunologic values were still present. In 5 patients, all previously noted immunologic abnormalities resolved. Of the 17 low values noted in 6 patients with Down syndrome, 12 (70.6%) were still present. Also, the 2 patients with Turner syndrome continued to have low IgA and IgM levels. Two patients had a low IgG4 level. A history of significant clinical infections within the previous 12 months was noted in 10 (58.8%) of 17 patients; 8 (47%) had current immune defects. There was a significantly lower T-cell response to staphylococcal enterotoxin B than in healthy controls. The T-lymphocyte activation response was low in 8 (38.1%) of the 21 patients. Conclusions Our study revealed a high rate of immune defects in patients with dysmorphic disorders, both during the initial study and 2 to 3 years later, which may contribute to their increased susceptibility to infections. This association was most obvious in patients with Down syndrome and Turner syndrome. The findings should alert for early immunologic evaluation when such patients have infections. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|