高雪氏病是遗传性的少见病,由于糖脂代谢障碍所致。临床有肝、脾肿大,贫血,骨痛及中枢神经系统症状。自1948年钟惠澜报告第一例至1988年底国内报告100例左右,多数分布于山东、河北、辽宁及黑龙江等省。我院儿科1986年6月至1987年5月一年收治儿童高雪氏病3例,现报道如下: 例1,余△,男,5岁,四川仁寿县人。入院前5月四肢大关节游走性疼痛,一月前受凉后发热,咳嗽,反复鼻衄,出血量约400毫升,当地医院给予止血药和输血,患儿仍面色苍黄,乏力,发热38～39℃,因病情 Gaucher disease is a hereditary rare disease due to dyslipidemia. Clinical liver, splenomegaly, anemia, bone pain and central nervous system symptoms. Since 1948, Zhong Huilan reported the first case to the end of 1988, about 100 cases of domestic reports, mostly in Shandong, Hebei, Liaoning and Heilongjiang provinces. Pediatric hospital in our hospital from June 1986 to May 1987 one year treated children with Gaucher’s disease in 3 cases, are reported as follows: Example 1, Yu △, male, 5 years old, Renshou County, Sichuan. Pre-admission May limbs joints walk pain, a month ago, after the cold fever, cough, repeated epistaxis, bleeding about 400 ml, the local hospital hemostatic drugs and transfusions, children are still pale yellow, fatigue, fever 38 ~ 39 ℃, due to illness