• 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.

• Pediatric Infection and Vaccine
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• v.24 no.3
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• pp.160-167
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• 2017

Purpose: Because children with asplenia have an increased risk of fulminant infection associated with a high fatality, chemoprophylaxis, and vaccinations against encapsulated bacteria are recommended. However, there have been few reports of the burden of severe bacterial infection and the current status of chemoprophylaxis and immunization among children with asplenia in Korea. Methods: We conducted a retrospective study including children with asplenia who were treated at our institute between January 1997 and December 2016. Results: From a total of 213 children with asplenia, 114 (53.5%) had congenital asplenia and 58 (27.2%) had functional asplenia. The remaining 41 (19.3%) had acquired asplenia with the median age at splenectomy being 12.2 years (range, 5.0 to 16.9 years); the most common cause of splenectomy was hereditary spherocytosis (39.0%). The chemoprophylaxis rate was 16.4%. The immunization rates were 44.1% for pneumococcus, 53.0% for Haemophilus influenzae type B, and 10.7% for meningococcus. The incidence of invasive bacterial infection among children with asplenia was 0.28/100 person-year; a total of six episodes (2.8%) were observed in five patients with congenital asplenia and one patient with functional asplenia. The median age for these infections was 15 months (range, 4 to 68 months). Five of the six episodes were bacteremia, and the other was meningitis. The most common pathogen was Streptococcus pneumoniae (n=3), followed by H. influenzae (n=1). Three of the six patients (50.0%) died, all of whom had pneumococcal bacteremia. None of the six had chemoprophylaxis or proper vaccinations. Conclusions: Although there is an increased risk of a severe infection proper vaccinations and chemoprophylaxis are still lacking. Physicians should be encouraged to implement appropriate chemoprophylaxis and immunizations for patients with asplenia.