论文部分内容阅读
尼满—匹克氏病是一种类脂质代谢障碍引起的类脂质增多综合征。本病较为少见,现将我科近3年收治的5例报告如下。例1 王某,女,3岁,平武人,第三胎足月顺产。生后8月发现肝脾肿大(肝肋下2横指,脾肋下3横指),以后进行性长大,无明显触痛。近两年来反复患呼吸道感染和腹泻。其妹(1岁多)和患儿病情相同。查体:发育营养差,智力低于同龄小儿。腹膨隆,无移动性浊音。肝肋下8厘米,剑下10厘米,质硬;脾脏Ⅰ线15厘米,Ⅱ线16厘米,Ⅲ线1.5厘米,质硬有切迹。双眼底检查未见樱桃红斑点,余无异常。化验:血红蛋白10克,红细胞302万,白细胞2,250,中性多核粒细胞10％,带核粒细胞6％,淋巴细胞81％,单核细胞1％,
Hitman-Pick’s disease is a lipid-like lipid-induced lipid-like syndrome. The disease is relatively rare, now my department admitted to the past 3 years 5 cases are as follows. Example 1 Wang, female, 3 years old, Ping Wu, full-term third trimester of labor. After birth in August found that hepatosplenomegaly (hepatic ribs 2 transverse means, spleen ribs 3 transverse means), after the progressive growth, no obvious tenderness. Repeated over the past two years, respiratory infections and diarrhea. His sister (1 year old) and children with the same condition. Physical examination: development of poor nutrition, mental retardation than children of the same age. Abdominal bulging, no shifting dullness. Liver ribs 8 cm, 10 cm under the sword, hard; spleen Ⅰ line 15 cm, Ⅱ line 16 cm, Ⅲ line 1.5 cm, hard notch. Double check no cherry cherry spots, no abnormalities. Assay: 10 grams of hemoglobin, erythrocyte 3.02 million, white blood cells 2,250, 10% of neutral multinucleated granulocytes, 6% of nucleated granulocytes, 81% of lymphocytes, 1% of monocytes,