先天性脂肪营养不良性糖尿病或称脂肪萎缩性糖尿病是一种罕见的先天性疾病。国内尚未见报道,现报告一例: 患儿,男性,3岁2个月,因消瘦、肝脏渐进性增大三年多,体毛增多、皮肤变黑一个。多月,于1984年1月3日入院。患儿出生后四天即见消瘦,且日渐明显,二个月起发现肝脏渐进性增大。一岁后食量倍增,每餐能吃3—4两米饭,但营养状况并无改善,否认有多 Congenital lipodystrophy Diabetes or lipoatrophic diabetes is a rare congenital disease. There is no report in our country. One case is reported now: children and males, 3 years old and 2 months old, due to weight loss and progressive liver enlargement for more than three years, body hair increased and skin turned dark. Many months, on January 3, 1984 admission. Four days after birth, children see weight loss, and increasingly obvious, two months found that the gradual increase of the liver. One year old doubled appetite, 3-4 meals per meal can eat rice, but no improvement in nutritional status, denied