• Tuberculosis and Respiratory Diseases
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• v.66 no.4
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• pp.295-299
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• 2009

Background: There are various etiologies causing bronchiectasis, but the cases without definite causes account for a quite high proportion. It is also uncertain that immunoglobulin G subclass deficiency (IgGSD) is associated with bronchiectasis. Therefore, we tried to measure the frequency of IgGSD in patients with bronchiectasis of unclear etiology, and to observe the clinical features of those patients with bronchiectasis and IgGSD. Methods: For the outpatients of a university hospital who were diagnosed as bronchiectasis by chest CT, we produced comprehensive history taking and physical examinations, and finally selected 31 patients with bronchiectasis of unclear etiology. Results: Two patients had total immunoglobulin G deficiency. The frequency of IgGSD was comparatively high (n=14). When we compared IgGSD group to normal immunoglobulin G subclass group, there were no significant differences in sex, age, and the frequency of sinusitis, bronchial asthma, and the abnormal lung function. Conclusion: In cases of bronchiectasis without definite causes, it can be considered to measure the level of immunoglobulin G subclass. It is also probably worthwhile to further evaluate the relationship between IgGSD and bronchiectasis.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.4 no.2
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• pp.243-248
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• 2001

In most cases, acute diarrhea in childhood heals spontaneously, but it may become the form of chronic diarrhea in immunodeficient children and then cause weight loss, dehydration, malabsorption and malnutrition. The immunodeficient diseases associated with chronic diarrhea include severe combined immunodeficiency syndrome, common variable immunodeficiency, acquired immunodeficiency syndrome, agammaglobulinemia or selective IgA deficiency. IgA deficiency is the most common primary immunodeficiency. Because many IgA deficient individuals seem to have compensated for their deficiency with increased IgM production and various nonimmunologic factors, the incidence of gastrointestinal involvement is not prominent. Some of those with IgA deficiency and recurrent infections have been found to also have IgG subclass deficiency. IgA deficiency with $IgG_2$ and $IgG_4$ subclass deficiency have high susceptability to infection and chronic diarrhea. IgG subclass deficiency, when present, is more likely to be found in association with a partial IgA deficiency rather than complete IgA deficiency. We report a 3-month-old male with intractable diarrhea accompanied by IgA, $IgG_2$, and $IgG_4$ deficiency.

• Clinical and Experimental Pediatrics
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• v.46 no.9
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• pp.883-888
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• 2003

Purpose : The etiology of hemolytic anemia can be classified as either cellular or extracellular defects of red blood cells. The aim of this study was to investigate the clinical and laboratory findings of hemolytic anemia concerning its etiological classification. Methods : Clinical and laboratory findings of the patients with hemolytic anemia treated from January 1987 to May 2002 at Severance Hospital were analyzed retrospectively. They were divided into two groups based on the types of red cell defects(group I : erythrocytic defect, group II : extraerythrocytic defect). Results : Twenty one cases were included in group I, thirty four cases in group II, and three cases were unclassified. In group I, nineteen cases(90.5%) were diagnosed as hereditary spherocytosis and were proved to have red cell membrane disorders while two cases(9.5%) were shown to have red cell enzyme deficiencies. In group II, thirteen cases(38.2%) were noted as autoimmune hemolytic anemia, eleven cases(32.4%) as traumatic or microangiopathic hemolytic anemia, four cases(11.8%) as drug induced hemolytic anemia, two cases(5.9%) were related with systemic lupus erythematosus and one case(2.9%) with malignancy. Hemoglobin at the time of diagnosis(7.5 g/dL vs. 6.2 g/dL, P<0.05) and the incidence of splenomegaly(85.7% vs. 18.2%, P<0.05) were higher in group I though blood urea nitrogen(9.0/0.4 mg/dL vs. 27.8/1.6 mg/dL, P<0.05) was higher in group II. Conclusion : Comparing the clinical features of pediatric hemolytic anemia, we concluded as following : In cases associated with extraerythrocytic defect, blood tests revealed significant initial lower hematocrit with higher level of BUN and Cr while cases with erythrocytic defect, splenomegaly were more common noted.