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KMID : 0371319660080050291
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Journal of the Korean Surgical Society
1966 Volume.8 No. 5 p.291 ~ p.296
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Hereditary Spherocytosis
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徐相賢/Suh, Sang Hyon
李世淳/洪弼勳/金秀子/Lee, Sae Soon/Hong, pill Whoon/Kim, Soo Ja
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Abstract
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Hereditary spherocytosis is a congenital hemolytic disease which has Mendelian dominant inheritance. It has known
to be one of the most rare disease in Korean, while it occurs predominantly in English and Northern European.
A case of hereditary spherocytosis in 2 year and 10 month old Korean girl has been treated in Severance Hospital and reported here. She had abdominal distension, abdominal mass in left upper quardrant, intermittent abdominal pain and severe anemia for 1 year duration.
Diagnosis was based on hemolytic anemia, splenomegaly, increased os:n)tic fragility of erythrocytes, negative Coo mb´s test, typical spherocytes in peripheral blood smear, erythrocytic hyperplasia in bone marrow study and acholuria. Splenectomy was performed after correction of severe anemia by blood transfusion, and resulted in clinical cure.
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KEYWORD
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