Immunological and clinical features of pediatric patients with primary hypogammaglobulinemia in Taiwan.

摘要: We retrospectively reviewed the clinical and immunological features as well outcome of children with a diagnosis primary hypogammaglobulinemia, who were treated at National Taiwan University Hospital between 1984 2001. A total 33 patients enrolled: seventeen common variable immunodeficiency (CVID), six selective immunoglobulin deficiencies (one subclass IgA five IgG), four severe combined (SCID), three transient hypogammaglobulinemia infancy (THI) X-linked (Bruton) agammaglobulinemia (XLA). In addition to recurrent sinopulmonary infections prolonged fever, allergic diseases are noted in 76% CVID 100% immunodeficiencies. Immunoglobulin levels extremely low XLA decreased patients. Three SCID had mean absolute lymphocyte counts 290/mm3. Long-term complications included bronchiectasis 2 patients, 1 patient immunodeficiency; short stature one each XLA, SCID, respectively; poor school performance patient; hemolytic anemia patient. concluded that thorough physical examination, family history early death from infection past neonatal hyperbilirubinemia, crucial evaluating suspicious hypogammaglobulinemia. The associated symptoms such infections, fever diseases, also diagnostic clues. treatment regular high doses Intravenous (IVIG) supplement may avoid development or decrease severity bronchiectasis.