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无神经系统病变的Ⅰ型高歇氏病是一种常染色体隐性遗传的代谢紊乱病.是由于酸性β-糖苷酶缺乏活性而导致葡糖苷酰鞘氨醇在单核-巨噬细胞系统的细胞内堆积.患者长期健康不良并有继发于肝、脾、骨髓等受累引起的并发症.本文回顾了11例患有高歇氏病Ⅰ型的妇女21次妊娠的临床资料以说明高歇氏病和妊娠的相互影响问题.这11例患者种族来源不同,临床病情的严重程度亦不同,但妊娠期及产后经过良好,其中9人19次妊娠均无过多的并发症,甚至一例孕前有严重骨骼肌并发症的病人,在妊娠期及产后病情有相当改善.还有一例妊娠期骨骼及血液系统病情有急剧恶化,而产后则再次改善.仅有一例病人5年前行脾切除,孕期血象正常,因胎儿窘迫而行剖宫产,产后3周死于败血
Hodgkin’s disease with no neurological disease is an autosomal recessive metabolic disorder that results from the lack of activity of glucosylceramide in the mononuclear-macrophage system Intracellular accumulation of long-term patients with poor health and have secondary complications of liver, spleen, bone marrow involvement.This review reviewed the 11 cases of women with Type H of Sheep’s disease in 21 clinical data to illustrate the high resumption S disease and pregnancy.The 11 patients were from different races, the severity of the clinical condition is also different, but the pregnancy and postpartum well, nine of 19 pregnancies without excessive complications, and even a case of pre-pregnancy Severe skeletal muscle complications in patients during pregnancy and postpartum a considerable improvement in the condition, there is a case of pregnancy bone and blood system conditions have deteriorated rapidly, and then once again improve after childbirth.Only one patient underwent splenectomy 5 years ago, Blood during pregnancy is normal, due to fetal distress and cesarean section, 3 weeks postpartum died of septicemia