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A Clinical Study of Hereditary Spherocytosis.

Journal of the Korean Pediatric Society 1990;33(1):81-87.
Published online January 31, 1990.
A Clinical Study of Hereditary Spherocytosis.
Ki Ho Kim, Kun Soo Lee, Haeng Mi Kim, Doo Hong Ahn
Department of Pediatrics, School of Medicine, Kyungpook National University, Taegu, Korea
유전성구상적혈구증에 대한 임상적 고찰
김기호, 이건수, 김행미, 안두홍
경북대학교 의과대학 소아과학교실
Received: 11 May 1989   • Accepted: 24 July 1989
Abstract
The following results were obtained from eight cases of hereditary spherocytosis (HS) who were admitted at the Department of Pediatrics, Kyung-pook National University Hospital from July 1984 to January 1989. The percentage of HS among the pediatric hematology patients during the same period was 2.6%. Male amd female were one and seven respectively. The mean age at the diagnosis and at onset of symptom was 7.1 years (8 months-13.4 years) and 4.9 years (2 months-10.4 years). The duration from initial symptom to diagnosis was 2.2 years (5 months-5 years). The chief complaint was jaundice in 2, hepatosplenomegaly in 2, splenomegaly in 1, easy fatigability in 1, and pallor in 1. The liver and spleen were enlarged in 6 cases with 2.2 cm and 5.3 cm below the costal margin respectively. The mean hemoglobin was 8.2 g/dl, the reticulocyte count 12.3%, the mean reticulocyte production index 4.4, the mean MCV 78u3, the mean MCH 28 pg, the mean MCHC 34 g/dl. The mean spherocyte was 33% and the mean bilirubin 2.4 mg% with indirect predominant. The bone marrow aspiration was done in 7 cases and showed erythroid hyperplasia in all. In osmotic fragility test, the hemolysis was started in the mean concentration of 0.7%NaCl solution and was completed in 0.48%. The autohemolysis test at 48 hours showed 9.8%, 4.2%, 2.6%, 6.6%, 40.8%, and 60.8% of hemolysis in 6 cases and showed partial correction by adding glucose in 2 cases. Aplastic crisis was deveploped in one case and was recovered with packed cell transfusion after several days. Splenectomy was performed in 5 cases (10,11,13.4,5.9, and 10.6 years old) and cholecystectomy was done in one of five concomitantly.
Key Words: Hereditary Spherocytosis, Reticulocyte production index, Aplastic crisis, Splenectomy, Cholecystectomy


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