Thevenot P, Gottrand F, Tassin E, Launay V, Loeuille G A, Razemon M, Turck D, Bonnevalle M, Pruvot F R, Hue V, Farriaux J P
Service de pédiatrie, CHRU, hôpital Claude-Huriez, Lille, France.
Arch Pediatr. 1996 Dec;3(12):1248-52. doi: 10.1016/s0929-693x(97)85936-2.
Orthotopic liver transplantation (OLT) is an effective treatment for patients with cystic fibrosis end stage liver disease, especially those with only mild pulmonary involvement. Long-term follow-up in such transplanted patients is still lacking.
A 15-year-old girl with cystic fibrosis received an OLT because of severe decompensated cirrhosis. She had been colonized by Pseudomonas aeruginosa for 3 years and had pancreatic insufficiency; she also had mild glucose intolerance. Postoperatively she developed diabetes mellitus requiring insulin therapy for 9 months. Oral cyclosporin was poorly absorbed so that she was given a new emulsion of cyclosporin (Neoral) that was better absorbed. A rapid pubertal catch-up was obtained but the patient remained colonized by Pseudomonas aeruginosa.
This 3-year postoperative follow-up confirms that OLT can represent a good alternative in those patients with severe liver disease and mild pulmonary involvement.
原位肝移植（OLT）是治疗囊性纤维化终末期肝病患者的有效方法，尤其是那些仅有轻度肺部受累的患者。目前仍缺乏对此类移植患者的长期随访。
一名15岁的囊性纤维化女孩因严重失代偿性肝硬化接受了原位肝移植。她已被铜绿假单胞菌定植3年，并有胰腺功能不全；她还存在轻度糖耐量异常。术后她发展为糖尿病，需要胰岛素治疗9个月。口服环孢素吸收不佳，因此给她使用了吸收更好的新型环孢素乳剂（新山地明）。患者青春期快速追赶，但仍被铜绿假单胞菌定植。
这例术后3年的随访证实，原位肝移植对于那些患有严重肝病且肺部受累较轻的患者而言是一个很好的选择。
