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KMID : 0377619670130030271
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Korean Jungang Medical Journal
1967 Volume.13 No. 3 p.271 ~ p.274
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Gaucher´s Disease
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Abstract
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This paper is to report a case of Gaucher´s disease which occurred in a ten year old Korean boy who had splenomegaly since six months of age.
At the age of ten years he had a splenectomy. The spleen weighed 2,550 grams and was the main site of massive infiltration of Gaucher´s cell. Mild to moderate infiltration of Gaucher´s cell was also noted in the accessory spleens, splenic hilar lymph nodes and sternal bone marrow. The bone marrow was minimally involved. There was no visible roentgenologic changes on a skeletal survey. The liver biopsy failed to show Gaucher´s cells although the patient had jaundice, hepatomegaly, and poor liver function tests. Two members of his family had histories of splenomegaly of unknown etiology in the past which may be significant in supporting the diagnosis.
The author believes this disease is in its chronic form and expects poor prognosis because of rapid deterioration of the hepatic function. This is probably the first case of Gaucher´s disease reported in Korea.
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