Lu Brandy R, Esquivel Carlos O
Section of Pediatric Gastroenterology, Sutter Pacific Medical Foundation, California Pacific Medical Center, San Francisco, Stanford, CA, USA.
Pediatr Transplant. 2010 Dec;14(8):954-60. doi: 10.1111/j.1399-3046.2010.01412.x. Epub 2010 Oct 14.
With advances in medical treatments, patients with CF are having improved quality of life and living longer. Although pulmonary disease is still the leading cause of morbidity and mortality, this longevity has allowed for the development of other organ dysfunction, mainly liver and pancreas. This review discusses the abdominal organ complications and the role of abdominal organ transplantation in CF. Liver failure and portal hypertension complications are the most common indicators for liver transplantation in CF, and five-yr survival for isolated liver transplantation is >80%. Deficiency of pancreatic enzymes is almost universal and up to 40% of patients with CF can develop insulin-dependent diabetes, although the role of pancreas transplantation is less clear and needs further research. Finally, the need for lung transplantation should always be assessed and considered in combination with liver transplantation on a case-by-case basis.
随着医学治疗的进步，囊性纤维化（CF）患者的生活质量得到改善，寿命也得以延长。尽管肺部疾病仍是发病和死亡的主要原因，但这种寿命延长使得其他器官功能障碍得以发展，主要是肝脏和胰腺。本综述讨论了CF患者腹部器官并发症以及腹部器官移植的作用。肝衰竭和门静脉高压并发症是CF患者肝移植最常见的指标，单纯肝移植的5年生存率>80%。胰腺酶缺乏几乎普遍存在，高达40%的CF患者会发展为胰岛素依赖型糖尿病，尽管胰腺移植的作用尚不清楚，需要进一步研究。最后，应始终评估肺移植的必要性，并根据具体情况结合肝移植进行考虑。
