岡田 憲樹

小児肝移植患者は、成人に比べ、グラフト不全や患者予後に直結する血管合併症や重症感染症のリスクが高い。小児肝移植患者の呼吸管理では、術前からの呼吸器合併症、過大グラフト、術中のover volumeなどの影響で抜管は遅れる傾向にある。また抜管可能であっても、術後に肝血流障害を認める症例では、肝血流が安定するまで抜管を遅らせるのが望ましい。循環管理では血管内脱水による肝機能障害や血栓症を防ぐため、心肺機能が許す範囲でハイドレーションをかける。大量腹水をもたらす重症急性拒絶反応の出現に注意する。肝移植周術期の敗血症は重篤であるため、発熱時の速やかな起炎菌・感染源の同定と抗菌薬治療、術後早期経腸栄養による感染症の予防が重要である。低体重の新生児や乳児における肝移植の成否には、肝移植のあらゆる術後合併症を理解し、新生児への血液浄化療法に精通したICU、PICUのスタッフの存在が不可欠である。(著者抄録)

小児肝移植における服薬アドヒアランスの現状と課題について検討した。肝移植を施行した中学生以上で移植後1年以上経過した125例(男性48名、女性77名、現年齢13〜37歳、移植時0.8〜22.6歳)を対象とした。電子カルテに記載された内容をもとに後方視的に収集した。服薬アドヒアランス良好群は83例、服薬ノンアドヒアランス群は42例であった。服薬ノンアドヒアランス群において、移植時年齢と現年齢は有意に高く、服薬数に関しては、免疫抑制薬服薬数と全服薬数が有意に多かった。服薬ノンアドヒアランス群において有意に肝機能障害を認め、グラフト不全症例が多い傾向にあった。免疫抑制薬3剤内服開始時年齢は服薬ノンアドヒアランス群で高い傾向にあり、服薬ノンアドヒアランス群20例の全例に肝機能障害を認めた。社会的背景に関しては、服薬ノンアドヒアランス群において不定期通院、内服自己管理、家庭環境・家族形態の変化、不快な服薬体験を有意に多く認めた。現年齢19歳以上、移植時年齢中学生以上において服薬ノンアドヒアランス率は有意に高かった。

Hepatocellular nodules caused by congenital extrahepatic portosystemic shunts (CEPS) occur as a result of abnormal portal blood flow, and are mostly cases of benign focal nodular hyperplasia (FNH). However, hepatocellular adenomas (HCA) and hepatocellular carcinomas have been documented in the CEPS patients. HCA can now be immunohistochemically diagnosed; therefore, the concept of hepatocellular nodules resulting from CEPS should be revisited. In this study, we performed a retrospective immunohistochemical investigation of hepatocellular nodules from livers isolated from the CEPS patients undergoing living donor liver transplantation (LDLT).<br /> Hepatocellular nodules from livers of five patients with CEPS who underwent LDLT between June 2004 and October 2012 at our institution were immunohistochemically investigated. HCA were classified into four subtypes (HNF1α-inactivated HCA (H-HCA); inflammatory HCA; β-catenin-activated HCA (b-HCA); unclassified HCA).<br /> Sixteen hepatocellular nodules were collected from livers of five patients with CEPS who underwent LDLT. Ten hepatocellular nodules were categorized as FNH (62.5%), five were categorized as b-HCA (31.3%), and one was categorized as

BackgroundAlthough endotoxin (Et) has been used as a biological index of bacterial infections, Et can also be used to evaluate liver functions because Et present in the portal vein blood is processed by the hepatic reticuloendothelial system. In the field of posttransplant management, it is important for liver transplant recipients to monitor the presence of posttransplant bacterial infections and graft liver functions because these results are directly correlated with a graft prognosis. Therefore, the measurement of Et during liver transplantation (LT) may be the detection of posttransplant infections and graft liver functions. This retrospective study investigated whether Et measured by the Et activity assay (EAA) in the peripheral venous blood during living donor LT (LDLT) can predict the incidence of posttransplant bacterial infections and graft liver functions. Materials and MethodsThe study subjects consisted of 21 patients who underwent LDLT between April 2010 and February 2011. Et activity (EA) was measured using the EAA in peripheral venous blood samples collected 1 or 2 days before LDLT, and on postoperative days (PODs) 1, 5, 7, and 14. We included LDLT recipients with intra-abdominal infections, respiratory infections, and bacteremia in the group with posttransplant bacterial infections. ResultsThe incidence rates of posttransplant bacterial infections or hyperbilirubinemia after LDLT were 57.1%. The LDLT recipients with posttransplant bacterial infections or hyperbilirubinemia had significantly higher levels of EA in comparison with patients without complications before LDLT (0.220.10 vs. 0.07 +/- 0.05, p&lt;0.001), but they had no statistically significant increase of EA between PODs 1 and 14. Based on a receiver operating characteristic curve analysis of pretransplant levels of EA in patients with posttransplant bacterial infections or hyperbilirubinemia, the recommended cutoff value to diagnose posttransplant bacterial infections or hyperbilirubinemia was set at 0.16 (sensitivity 83.3%, specificity 88.9%, and area under the curve 0.940). ConclusionAt a pretransplant level of EA greater than 0.16, patients had an augmented risk for developing posttransplant bacterial infections or hyperbilirubinemia.

The use of donors with coagulation FIX deficiency is controversial, and there are no current protocols for peri-transplant management. We herein describe the first reported case of a pediatric LDLT from an asymptomatic donor with mild coagulation FIX deficiency. A 32-yr-old female was evaluated as a donor for her 12-month-old daughter with biliary atresia. The donor's pretransplant coagulation tests revealed asymptomatic mild coagulation FIX deficiency (FIX activity 60.8%). Freeze-dried human blood coagulation FIX concentrate was administered before the dissection of the liver and 12 h afterwards by bolus infusion (40 U/kg) and was continued on POD 1. The bleeding volume at LDLT was 590 mL. On POD 1, 3, 5, and 13, the coagulation FIX activity of the donor was 121.3%, 130.6%, 114.6%, and 50.2%, respectively. The donor's post-transplant course was uneventful, and the recipient is currently doing well at 18 months after LDLT. The FIX activity of the donor and recipient at nine months after LDLT was 39.2% and 58.0%, respectively. LDLT from donors with mild coagulation FIX deficiency could be performed effectively and safely using peri-transplant short-term coagulation FIX replacement and long-term monitoring of the plasma FIX level in the donor.