Urahashi Taizen, Mizuta Koichi, Sanada Yukihiro, Umehara Minoru, Wakiya Taiichi, Hishikawa Shuji, Hyodo Masanobu, Sakuma Yasunaru, Fujiwara Takehito, Yasuda Yoshikazu, Kawarasaki Hideo
Department of Transplant Surgery, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, Tochigi, 329-0498, Japan.
Pediatr Surg Int. 2011 Aug;27(8):817-21. doi: 10.1007/s00383-011-2866-8. Epub 2011 Feb 18.
Hepatopulmonary syndrome (HPS) is a progressive, deteriorating complication of end-stage liver disease (ESLD) that occurs in 13-47% of liver transplant candidates. Although LT is the only therapeutic option for HPS, it has a high morbidity and mortality, especially in patients with severe hypoxemia before transplantation, but the course of HPS after living donor liver transplantation (LDLT), especially for biliary atresia (BA) patients is not well established.
The present study evaluated 122 patients who received an LDLT for BA and of these, 3 patients had HPS at the time of LDLT in a single-center series.
Two patients of the HPS patients them had biliary and/or vascular complications, but they recovered uneventfully with interventional treatment. None of the patients required supplemental oxygen and had no residual cardiopulmonary abnormalities at a follow-up of more than 24 months.
Although a series of three patients is too small for definitive conclusion and further investigations must be conducted, pediatric LDLT can be a favorable therapeutic option for HPS.
肝肺综合征（HPS）是终末期肝病（ESLD）的一种进行性、恶化性并发症，在13%至47%的肝移植候选者中出现。虽然肝移植（LT）是治疗HPS的唯一选择，但其发病率和死亡率很高，尤其是在移植前有严重低氧血症的患者中，然而活体供肝肝移植（LDLT）后HPS的病程，特别是对于胆道闭锁（BA）患者，尚不明确。
本研究评估了122例因BA接受LDLT的患者，在一个单中心系列研究中，其中3例患者在LDLT时患有HPS。
HPS患者中有2例出现了胆道和/或血管并发症，但经介入治疗后顺利康复。在超过24个月的随访中，所有患者均无需补充氧气，且无残留心肺异常。
尽管3例患者的系列样本过小，无法得出确定性结论，必须进行进一步研究，但小儿LDLT可能是治疗HPS的一个有利选择。
