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

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dc.contributor.authorWang, Ling-Jenen_US
dc.contributor.authorYang, Yao-Hsuen_US
dc.contributor.authorLin, Yu-Tsanen_US
dc.contributor.authorChiang, Bor-Luenen_US
dc.date.accessioned2009-05-27T17:17:35Z
dc.date.available2009-05-27T17:17:35Z
dc.date.issued2004-03-16en_US
dc.descriptionPublished by the Allergy and Immunology Society of Thailand.en_US
dc.description.abstractWe retrospectively reviewed the clinical and immunological features as well as the outcome of children with a diagnosis of primary hypogammaglobulinemia, who were treated at the National Taiwan University Hospital between 1984 and 2001. A total of 33 patients were enrolled: seventeen patients with common variable immunodeficiency (CVID), six patients with selective immunoglobulin deficiencies (one subclass IgA and five IgG), four patients with severe combined immunodeficiency (SCID), three patients with transient hypogammaglobulinemia of infancy (THI) and three patients with X-linked (Bruton) agammaglobulinemia (XLA). In addition to recurrent sinopulmonary infections and prolonged fever, allergic diseases are noted in 76% of CVID patients and 100% of patients with selective immunodeficiencies. Immunoglobulin levels were extremely low in XLA and decreased in CVID patients. Three SCID patients had decreased mean absolute lymphocyte counts of 290/mm3. Long-term complications included bronchiectasis in 2 XLA patients, 2 CVID patients and 1 patient with selective immunodeficiency; short stature in one of each XLA, SCID, and CVID patients respectively; poor school performance in 2 SCID patients and 1 XLA patient; and hemolytic anemia in 1 CVID patient. We concluded that in addition to a thorough physical examination, a family history of early death from infection and past history of neonatal hyperbilirubinemia, are crucial in evaluating a patient with suspicious primary hypogammaglobulinemia. The associated symptoms of primary hypogammaglobulinemia, such as recurrent sinopulmonary infections, prolonged fever and allergic diseases, are also diagnostic clues. In the treatment of hypogammaglobulinemia, early and regular high doses of Intravenous immunoglobulin (IVIG) supplement may avoid the development or decrease the severity of bronchiectasis.en_US
dc.description.affiliationDepartment of Pediatrics, Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan.en_US
dc.identifier.citationWang LJ, Yang YH, Lin YT, Chiang BL. Immunological and clinical features of pediatric patients with primary hypogammaglobulinemia in Taiwan. Asian Pacific Journal of Allergy and Immunology. 2004 Mar; 22(1): 25-31en_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/36873
dc.language.isoengen_US
dc.subject.meshAgammaglobulinemia --complicationsen_US
dc.subject.meshChild, Preschoolen_US
dc.subject.meshFemaleen_US
dc.subject.meshHumansen_US
dc.subject.meshImmunologic Deficiency Syndromes --complicationsen_US
dc.subject.meshInfanten_US
dc.subject.meshMaleen_US
dc.subject.meshRetrospective Studiesen_US
dc.subject.meshTaiwanen_US
dc.subject.meshTreatment Outcomeen_US
dc.titleImmunological and clinical features of pediatric patients with primary hypogammaglobulinemia in Taiwan.en_US
dc.typeJournal Articleen_US
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