Noble-Jamieson G, Barnes N, Jamieson N, Friend P, Calne R
Department of Paediatrics, Addenbrooke's NHS Trust, Cambridge, England.
J R Soc Med. 1996;89 Suppl 27(Suppl 27):31-7.
About 10% of children with CF develop hepatic cirrhosis and progressive portal hypertension. As the portal hypertension worsens these children are likely to develop serious variceal bleeding and other complications including malnutrition and a decline in respiratory function. Indices of lung function may fall as much as 50% in a year and chest infections may require frequent admissions to hospital. The respiratory symptoms are often attributed to CF related lung disease and affected children may therefore be considered unsuitable for liver transplantation. We propose a simple scoring system which can help to select patients who should be referred for assessment of liver transplantation. After careful assessment and preparation children with lung function indices as low as 30% predicted can have a successful outcome after liver transplantation. With good graft function portal hypertension is relieved and absorption, nutrition and respiratory function all improve. The improved quality of life of these children is remarkable.
约10%的囊性纤维化（CF）患儿会发展为肝硬化和进行性门静脉高压。随着门静脉高压加重，这些患儿可能会出现严重的静脉曲张出血及其他并发症，包括营养不良和呼吸功能下降。肺功能指标一年内可能下降多达50%，胸部感染可能需要频繁住院。呼吸症状常被归因于与CF相关的肺部疾病，因此受影响的患儿可能被认为不适合进行肝移植。我们提出一种简单的评分系统，可帮助选择应转诊进行肝移植评估的患者。经过仔细评估和准备，肺功能指标低至预测值30%的患儿肝移植后也可获得成功结局。移植肝功能良好时，门静脉高压得以缓解，吸收、营养和呼吸功能均得到改善。这些患儿生活质量的改善非常显著。
