Thomas W E, Winfield D A
Department of Surgery, Royal Hallamshire Hospital, Sheffield, UK.
Postgrad Med J. 1991 Dec;67(794):1072-4. doi: 10.1136/pgmj.67.794.1072.
A 16 year old girl with Type 1 Gaucher's disease presented with massive splenomegaly, hypersplenism and abdominal discomfort. Traditionally hypersplenism has been treated with splenectomy, but this results in a high incidence of overwhelming sepsis and accelerated sphingolipid deposition in both liver and bone. A 90% partial splenectomy was therefore performed leaving a fully vascularized inferior segment of the spleen and resecting 5.8 kg of splenic tissue. The patient made an uneventful recovery with a marked improvement in her haematological parameters and general condition.
一名患有1型戈谢病的16岁女孩出现巨脾、脾功能亢进和腹部不适。传统上，脾功能亢进采用脾切除术治疗，但这会导致暴发性败血症的高发生率，并加速肝脏和骨骼中鞘脂的沉积。因此，实施了90%的部分脾切除术，保留了脾脏完全血管化的下段，并切除了5.8千克的脾组织。患者恢复顺利，血液学参数和总体状况显著改善。
