遗传性球形细胞增多症(简称HS)是一种红细胞膜有先天性缺陷的溶血性贫血。又称先天性溶血性黄疸,是一种遗传性溶血性疾病,为常染色体显性遗传,但约有1/3的病例无阳性家族史,系由基因突变引起。有人报道异常基因存在于第7或第8对染色体上,本病多于婴儿时期发病,国内已先后有报道,但新生儿期发病者较少见。本院在同家族中发现两例,现报道如下。例1,女婴,为第一胎第一产,于1987年6月13日足月自然分娩,生后一般情况好,20小时后皮肤轻度黄染,进行性加重,于生后38小时,总胆红素达20mg/dl,(直 Hereditary spherocytosis (referred to as HS) is a red blood cell membrane congenital defects of hemolytic anemia. Also known as congenital hemolytic jaundice, is a hereditary hemolytic disease, autosomal dominant inheritance, but about 1/3 of cases no positive family history, caused by gene mutations. Abnormal gene was reported in the first 7 or 8 pairs of chromosomes, the disease more than infancy, has been reported in China, but less common in neonatal onset. The hospital found two cases in the same family, are reported below. Example 1, the first baby for the first child, June 13, 1987 full-term natural childbirth after birth, generally good, mild yellowish skin after 20 hours, progressive increase in 38 hours after birth , Total bilirubin up to 20mg / dl, (straight