@entity1120 , a @entity2315 , is not a common disease in Thailand. During the period 1966-1998 we saw 20 @entity1 with @entity1120 at the Department of Pediatrics. Siriraj Hospital. The @entity1 came from different regions of the country but mostly from the central part of Thailand. There were 8 males and 12 females from 13 families of Thai, Thai-Chinese, Thai-Laos and Chinese-Chinese in origin. A history of consanguinity was present in 2 families. The age of onset was 2 months-4 years and the age when they were diagnosed was 4 months-15 years. The most common clinical features included @entity381 , @entity382 , @entity851 , pallor, @entity3459 and @entity16 . The diagnosis was made by the clinical manifestations, hematologic complications and demonstration of Gaucher cells in the bone marrow and/or other tissues. In one family, the diagnosis was confirmed by evaluation of glucocerebrosidase activities in skin fibroblasts. The management of these @entity1 was symptomatic ie packed red cell and platelet transfusion, splenectomy and other supportive measures. Most @entity1 died of @entity548 or @entity281 at an early age.

XXXX ;thirty-two years experience at Siriraj Hospital.

@entity2315 :: ('MESH:D016464', 'Disease') :: ['lysosomal disorder'] @entity1 :: ('9606', 'Species') :: ['patients'] @entity851 :: ('MESH:D006130', 'Disease') :: ['growth retardation'] @entity548 :: ('MESH:D006470', 'Disease') :: ['bleeding'] @entity16 :: ('MESH:D009422', 'Disease') :: ['neurological abnormalities'] @entity3459 :: ('MESH:D025861', 'Disease') :: ['bleeding disorders'] @entity281 :: ('MESH:D007239', 'Disease') :: ['infection'] @entity381 :: ('MESH:D013163', 'Disease') :: ['splenomegaly'] @entity1120 :: ('MESH:D005776', 'Disease') :: ["Gaucher's disease"] @entity382 :: ('MESH:D006529', 'Disease') :: ['hepatomegaly']
@entity1120 :: (MESH:D005776,Disease) :: ['Gaucher's disease']
