Al-Hussaini Abdulrahman, Taylor Rachel M, Samyn Marianne, Bansal Sanjay, Heaton Nigel, Rela Mohammed, Mieli-Vergani Giorgina, Dhawan Anil
Paediatric Liver Centre, King's College London School of Medicine at King's College Hospital NHS Foundation Trust, London, UK.
Pediatr Transplant. 2010 Mar;14(2):276-82. doi: 10.1111/j.1399-3046.2009.01218.x. Epub 2009 Aug 3.
We aim to report a single center experience of the management and long term outcome of HPS in pediatric liver transplant recipients. A retrospective review of children with HPS from 1990 to 2004.
liver disease or portal hypertension, hypoxemia (PaO(2) < 70 mmHg or SaO(2) < 95%) and intrapulmonary shunting documented by macroaggregated albumin scan ratio of >4% (classified mild group [<20%], moderate group [20-40%] and severe group [>40%]). Resolution of HPS post-liver transplant was defined as PaO(2) > 70 mmHg or SaO(2) > 95%. Eighteen children (six male [34%], median age at diagnosis of HPS 8.6 [1-15.5] yr) had HPS: biliary atresia (n = 8), idiopathic biliary cirrhosis (n = 4), progressive intrahepatic cholestasis (n = 2), miscellaneous (n = 4). The majority had mild shunting (n = 8). Fourteen underwent transplantation with resolution of HPS in 13. Six developed complications: hepatic artery thrombosis (n = 4), biliary (n = 2). Four children died (28%), two pretransplant. There was a tendency towards shunt fraction worsening to a slower degree over time. One-yr survival rate post-transplant was 93%. Median PaO(2) was significantly lower in non-survivors compared to survivors (43 vs. 55.2 mmHg, p = 0.03). There was correlation between oxygen parameters pretransplant and time to HPS resolution post-transplant. HPS is reversible after transplant, but is associated with increasing mortality and morbidity.
我们旨在报告小儿肝移植受者肝肺综合征（HPS）的管理及长期预后的单中心经验。对1990年至2004年患有HPS的儿童进行回顾性研究。
肝病或门静脉高压、低氧血症（动脉血氧分压[PaO₂]<70 mmHg或动脉血氧饱和度[SaO₂]<95%）以及经大颗粒聚合白蛋白扫描比值>4%证实存在肺内分流（分为轻度组[<20%]、中度组[20 - 40%]和重度组[>40%]）。肝移植后HPS的缓解定义为PaO₂ > 70 mmHg或SaO₂ > 95%。18名儿童（6名男性[占比34%]，HPS诊断时的中位年龄为8.6岁[1 - 15.5岁]）患有HPS：胆道闭锁（n = 8）、特发性胆汁性肝硬化（n = 4）、进行性肝内胆汁淤积（n = 2）、其他（n = 4）。大多数为轻度分流（n = 8）。1名儿童死亡（占比28%），2名在移植前死亡。随着时间推移，分流分数有向恶化程度减缓的趋势。移植后1年生存率为93%。与存活者相比，非存活者的中位PaO₂显著更低（43 mmHg对55.2 mmHg，p = 0.03）。移植前的氧参数与移植后HPS缓解时间之间存在相关性。HPS在移植后是可逆的，但与死亡率和发病率增加相关。
