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Approach to child with primary immunodeficiency

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Approach to child with primary immunodeficiency

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    1. Approach to child with primary immunodeficiency By Dr. Mufarreh Al-Zahrani Consultant pediatrician, allergist and immunologist 14/5/2007

    2. primary Hereditary Secondary acquired

    4. multiple infections despite aggressive treatment. infections with unusual or opportunistic organisms. failure to thrive or poor growth. positive family history.

    5. more than 80 primary immunodeficiencies are recognized by the World Health Organization. males and females about equally .

    11. History: name age: early late Sex Type of infction Site of infecion

    12. Other associated infections Oral thrush Chronic diarrhea Abscesses Suppurative otitis media Chest infections Lymph nodes enlargement

    13. FTT Recurrent meningitis Bleeding Delayed separation of the cord Skin rash hair color

    14. Neurological symptoms Eye symptoms Vaccination Family history

    15. Examination dysmorphic features DiGeorge Syndrome hypertelorism, micrognathia, short philtrum with fish-mouth appearance, antimongoloid slant Coarse features Hyper IGE

    16. Hair color Albinism Giant granules

    17. Telangiectasia Ataxia

    18. Eczema

    19. Oral thrush

    20. Tonsils

    21. Lymph nodes

    22. hepatosplenomegaly

    23. Ears Heart murmur

    24. CBC Blood morphology isohemagglutinine CXR Quantitive immun. Specific antibody respose

    25. CMI test Lymphocyte markers, MHC Blastogenesis NBT Cd 40 Cd 11/18, Fas

    26. Complement FISH Chromosomal studies

    27. Treatment General measures Prevention Treat infection Prophylaxis screening Vaccine nutrition Specific Replacement BMT Gene therapy

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