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Medical Genetics CLASSIFICATION, INHERITANCE, AND ASSOCIATED FEATURES OF THE PRIMARY IMMUNODEFICTENCY DISORDERS Disorder Associated Findings B-cell (antibody) deficiencies X-linked agammaglobulinemia Pyogenic infection after age 6 mo Ig deficiency with hyper-IgM (XL) Neutropenia, lymphadenopathy lgA deficiency Autoimmunity, respiratory or food allergy, respiratory infection; often asymptomatic Igg subclass deficiencies lgA deficiency Antibody deficiency with normal or elevated Igs Immunodeficiency with thymoma Aplastic anemia Common variable immunodeficiency Autoimmunity Transient hypogammaglobulinemia of infancy Prematurity T-cell(cellular) deficiencies Predominant T-cell deficiency DiGeorge anomaly Hypocalcemia, peculiar facies, aortic arch abnormalities heart disease Chronic mucocutaneous candidiasis Endocrinopathies Combined immunodeficiency with igs Bronchiectasis (Nezelof syndrome Nucleoside phosphorylase deficiency(AR)Medical Genetics CLASSIFICATION, INHERITANCE, AND ASSOCIATED FEATURES OF THE PRIMARY IMMUNODEFICIENCY DISORDERS
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