Watanabe Y, Miyauchi K, Horiuchi A, Kikkawa H, Kusunose H, Kotani T, Kawachi K
Department of Surgery II, Ehime University, Shigenobu, Shitsukawa, Ehime 791-0295, Japan.
Surg Endosc. 2002 Oct;16(10):1495. doi: 10.1007/s00464-002-4503-z. Epub 2002 Jul 1.
A 4-year-old girl with pyruvate kinase deficiency underwent partial splenic embolization initially. However, even after this procedure, she still had to be transfused every 2 months and then every month. At 5 years of age, she was admitted to our hospital to undergo splenectomy. She underwent laparoscopic splenectomy and concomitant cholecystectomy for gallstones. The hemogram recovered to the normal range after surgery, and her postoperative course was uneventful. Considering the absence of morbidity, the short hospitalization, the quick return to normal activity, the good cosmetic result, and the improved clinical and hematologic results, we consider that simultaneous laparoscopic splenectomy and cholecystectomy is safe and effective for the management of hemolytic anemia resulting from pyruvatre kinase deficiency and associated with cholelithiasis.
一名患有丙酮酸激酶缺乏症的4岁女孩最初接受了部分脾栓塞术。然而，即使在该手术后，她仍需每2个月输血一次，之后变为每月输血一次。5岁时，她入住我院接受脾切除术。她接受了腹腔镜脾切除术及因胆结石而行的同期胆囊切除术。术后血常规恢复至正常范围，术后病程平稳。考虑到无并发症、住院时间短、能迅速恢复正常活动、美容效果良好以及临床和血液学结果改善，我们认为同期腹腔镜脾切除术和胆囊切除术对于治疗由丙酮酸激酶缺乏症导致并伴有胆石症的溶血性贫血是安全有效的。
