Rodgers B M, Tribble C, Joob A
Ann Surg. 1987 Jun;205(6):693-9. doi: 10.1097/00000658-198706000-00011.
Gaucher's disease is an autosomal recessive disorder caused by deficiency of beta glucocerebrosidase, resulting in an accumulation of glucocerebroside in the reticuloendothelial system. These patients have massive splenomegaly and bone pain, but may have normal life expectancy. Traditionally, splenectomy has been used to treat hypersplenism, but may be associated with a high incidence of postsplenectomy sepsis and accelerated hepatic and bone lipid deposition. Two children are reported who had partial splenectomy for symptoms of Gaucher's disease. Both patients had laboratory evidence of hypersplenism. A 90% splenectomy was performed, and the residual splenic fragment was wrapped in Vicryl mesh. Both patients are currently asymptomatic with normal hematologic parameters. Postoperative radionuclide scans demonstrate increase in the size of the residual splenic fragment. Partial splenectomy may benefit patients with Gaucher's disease, but long-term follow-up care is necessary.
戈谢病是一种常染色体隐性疾病，由β-葡萄糖脑苷脂酶缺乏引起，导致葡萄糖脑苷脂在网状内皮系统中蓄积。这些患者有巨大脾脏肿大和骨痛，但预期寿命可能正常。传统上，脾切除术用于治疗脾功能亢进，但可能与脾切除术后败血症的高发生率以及肝脏和骨骼脂质沉积加速有关。报告了两名因戈谢病症状接受部分脾切除术的儿童。两名患者均有脾功能亢进的实验室证据。进行了90%的脾切除术，残余脾碎片用薇乔网包裹。两名患者目前均无症状，血液学参数正常。术后放射性核素扫描显示残余脾碎片大小增加。部分脾切除术可能对戈谢病患者有益，但需要长期随访护理。
