Noble-Jamieson G, Valente J, Barnes N D, Friend P J, Jamieson N V, Rasmussen A, Calne R Y
Department of Paediatrics and Surgery, Addenbrooke's NHS Trust, Cambridge.
Arch Dis Child. 1994 Oct;71(4):349-52. doi: 10.1136/adc.71.4.349.
Five children with cystic fibrosis complicated by hepatic cirrhosis received liver grafts. They all had portal hypertension with varices and three had variceal bleeding; respiratory function was only moderately impaired, but four were colonised with pseudomonas and one with aspergillus. Liver transplantation was well tolerated and there was no increase in respiratory or other early postoperative complications. Four of the children were fully well from 14 to 35 months after transplantation; the most recently transplanted had problems from a biliary stricture. In spite of the need for immunosuppression there was no increase in infection and respiratory function improved or remained stable. Once the children were stabilised after transplantation their nutrition and general health were greatly improved.
五名患有囊性纤维化并伴有肝硬化的儿童接受了肝移植。他们都有门静脉高压伴静脉曲张，其中三人发生过静脉曲张出血；呼吸功能仅受到中度损害，但四人感染了假单胞菌，一人感染了曲霉菌。肝移植耐受性良好，术后呼吸或其他早期并发症并未增加。四名儿童在移植后14至35个月完全康复；最近接受移植的那名儿童出现了胆管狭窄问题。尽管需要进行免疫抑制，但感染并未增加，呼吸功能得到改善或保持稳定。移植后患儿病情稳定后，他们的营养状况和总体健康状况有了很大改善。
