本病属先天性脂肪代谢异常病。病理检查见全身网状内皮系统及各器官内有尼曼——匹克氏细胞。国内报告甚少,我院曾遇1例,现简报如下: 患儿张×,女,11个月。因发热、咳嗽半月,于1977年5月16日入院。患儿系第一胎,足月产,生后常有呕吐,一般情况良好。8个月后,经常有腹泻,每天稀便5～6次,进食少,日渐消瘦,皮肤苍白,曾 The disease is a congenital abnormalities in fat metabolism. Pathological examination, see the whole body reticuloendothelial system and various organs within the Niemann-Pick’s cell. Little domestic reports, our hospital had a case, the briefing is as follows: children × ×, female, 11 months. Due to fever, cough half months, on May 16, 1977 admission. Children with the first child, full-term birth, often vomiting after birth, the general situation is good. After 8 months, often have diarrhea, loose stool 5 to 6 times a day, eat less, getting thinner, pale skin, once