Language：English  

DOI： 10.1186/s40792-023-01681-0

Web of Science

PubMed

Publisher：金原出版  

DOI： 10.18888/op.0000003117

CiNii Research

Language：English  

DOI： 10.1155/2023/9075184

PubMed

Language：English   Publisher：Experimental and Clinical Transplantation  

Erythropoietic protoporphyria is a rare inherited metabolic disorder involving the heme biosynthesis pathway and leads to the accumulation of protopo-rphyrin in the erythrocytes or liver. Although peripheral neuropathy is known to develop occa-sionally in other types of porphyria, it rarely occurs in patients with erythropoietic protoporphyria. A 16-year-old boy was transferred to our hospital due to end-stage liver disease secondary to erythropoietic protoporphyria. Severe systemic peripheral neuropathy, similar to that presented in Guillain-Barré syndrome, developed; it was promptly managed with mechanical ventilation. Electrophysiological assessment of the presented neuropathy showed no responsiveness, indicating severe axonopathy. Six weeks after the transfer, liver transplant was performed. Postoperatively, hepatorenal syndromes improved immediately, and his erythrocyte protoporphyrin level decreased from 6291 to 174 μg/dL red blood cells. The patient started to move his limbs gradually and was weaned from mechanical ventilation 2 months after liver transplant. Eventually, he was discharged from hospital and was able to ambulate with assistance 10 months after liver transplant. To our knowledge, this is the first report detailing the clinical course in a patient with erythropoietic protoporphyria who recovered from severe systemic peripheral neuropathy after liver transplant.

DOI： 10.6002/ect.2022.0157

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PubMed

Language：English   Publisher：Transplantation Proceedings  

Background: Early mobilization after liver transplant (LT) plays an important role in postoperative recovery and complication prevention; however, patients undergoing LT cannot achieve early mobilization because of mechanical ventilation and poor preoperative physical performance. We investigated the effect of neuromuscular electrical stimulation (NMES) on lower limb muscle strength after living donor liver transplant (LDLT). Methods: Adult patients who underwent LDLT between December 2016 and January 2019 at a university hospital were recruited. A consecutive series of patients who underwent LDLT without NMES therapy before the clinical trial (April 2014-May 2016) were enrolled as the non-NMES (control) group. Patients in the NMES group received NMES on the quadriceps muscles starting 1 day post LDLT for 4 weeks. The study was conducted in accordance with the Declaration of Helsinki, and all patients provided informed consent. Results: Twenty-four patients in the NMES group and 16 patients in the non-NMES group were analyzed. There was no significant difference between groups regarding changes in any outcome. Conclusions: The application of NMES in patients with LDLT did not yield greater improvement of muscle strength, functional capacity, activities of daily living, or length of hospital stay 4 weeks postoperatively compared with the control group. However, developing a novel NMES device and confirming whether additional NMES is effective for other body areas may yield different results.

DOI： 10.1016/j.transproceed.2022.01.028

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PubMed

Language：Japanese   Publisher：一般社団法人 日本移植学会  

<p>（はじめに）</p><p>COVID-19の流行、医師の働き方改革が喫緊の問題としてある中、リカバリー互助制度は人の移動の最小化のみならず、肉体的・精神的に負担を伴う脳死下臓器摘出チーム人員の</p><p>負担軽減に繋がると考える。島嶼県である沖縄へは長時間の航空機移動を伴い、発着が天候に左右される可能性や航空便の運行スケジュールに時間が規定される。当院は2020年3月に初の生体肝移植を行って以降、これまで18例の生体肝移植を実施してきた。そのような状況下、互助制度による脳死肝臓摘出術を経験したので報告する。</p><p>（症例報告）</p><p>他施設からの依頼があり、当科単独での肝臓摘出が可能かを検討。当院は脳死肝臓移植の認定施設では無い為、学会に承認を得た後、器材の確保を行った。近隣施設が提供施設であったため、当日の集合時間は1時間前で十分だった。肝臓摘出後はJOTスタッフが搬送を担うことで摘出後の負担も軽減された。現在は脳死膵臓移植認定施設となったため、器材準備が出来ている状況で更に負担なく互助制度に参加可能である。</p><p>（まとめ）</p><p>リカバリー互助制度の発展には手術手技、搬送法の標準化が必須であり、学会主導で浸透が進んできている。沖縄県は地理的特徴から互助制度が果たす役割が大きい地と考えた。</p>

DOI： 10.11386/jst.57.supplement_s304_3

CiNii Research

Language：English   Publisher：BMC Gastroenterology  

Background: There are long-standing controversies about the transplant indications for alcoholic liver disease (ALD), because of the recognition that ALD is fundamentally self-inflicted. However, it is unclear whether psychosocial characteristics of ALD are different from that of non-alcoholic liver disease (NALD) in the selection of liver transplantation (LT) recipients. We aimed to clarify the psychosocial characteristics of ALD recipients (ALD-R)/ALD recipient candidates (ALD-RC) and NALD recipients (NALD-R)/ NALD recipient candidates (NALD-RC). Methods: From 2011 to 2019, 75 patients were enrolled in this prospective observational study (ALD-RC, n = 19; NALD-RC, n = 56), LT were carried out as follow; ALD-R, n = 6; NALD-R, n = 52. We evaluated psychosocial characteristics in the preoperative period and 3, 12 months after LT (ALD-R, n = 3/3; NALD-R, n = 28/25). The following scales were used to evaluate psychosocial characteristics: Visual Analogue Scale, Alcohol Use Disorders Identification Test, Hospital Anxiety and Depression Scale, Beck Depression Inventory, Brief Evaluation of Medication Influences and Beliefs, Social Support Questionnaire (SSQ), Temperament and Character Inventory, Parental Bonding Instrument (PBI), the Short Form Health Survey (SF-36). Results: When evaluating on the basis of abstinence rule, a comparison of ALD-RC and NALD-RC in the preoperative period identified similar patterns of psychosocial characteristics, except that the NALD-RC scored higher on the PBI item “overprotection from mother” (P < 0.05). The only significant difference between ALD-R and NALD-R after liver transplantation was in SSQ scores at 3 months. Conclusion: The psychosocial characteristics of ALD-RC and NALD-RC may be similar when evaluated on the basis of Japan’s abstinence rule. This result also imply that the psychosocial characteristics of ALD-RC may differ from the previously reported psychosocial characteristics of alcohol dependent patients. These findings have the potential to provide helpful information for the evaluation of ALD-RC.

DOI： 10.1186/s12876-021-02032-9

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PubMed

Language：English   Publisher：Transplant Infectious Disease  

Background: Chronic high Epstein–Barr virus loads (CHEBV) are commonly observed in pediatric liver transplant patients. However, it is unclear how CHEBV impacts the liver graft. The aim of this study was to clarify the clinical and pathological impacts of CHEBV on the liver graft. Methods: From 2012 to 2020, we retrospectively investigated 46 pediatric liver transplant patients (under 16 years) who survived ≥6 months. The patients were divided into two groups: CHEBV group (EBV DNA >10 000 IU/ml of whole blood for ≥6 months) and nonchronic high EBV (NCHEBV) group (patients who did not meet CHEBV criteria). Tacrolimus was reduced to <3.0 ng/ml in patients with EBV DNA >5000 IU/ml. Blood biochemistry data and pathological findings, obtained at the time of protocol and episodic biopsies, were compared between the two groups. Results: Out of 46 patients, 28 CHEBV and 18 NCHEBV patients were enrolled. The blood biochemical examination did not show a significant difference between the two groups. In addition, no significant differences between the two groups were found in the pathological findings, including frequency of late acute rejection and the progression of fibrosis at the time of both protocol and episodic biopsies. Appropriate adjustment of immunosuppression for CHEBV management may have contributed to the prevention of the progression of fibrosis. Conclusion: CHEBV had little adverse effect on the liver graft. Graft fibrosis might have been avoided through optimal dose modification of tacrolimus. Further long-term monitoring is necessary because CHEBV may affect the pediatric liver graft in the long term.

DOI： 10.1111/tid.13731

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Language：English   Publisher：International Journal of Surgery Case Reports  

Introduction: Anatomic variations of the biliary tree are common, making precise anatomic evaluation important before hepatobiliary surgery. Presentation of case: A 52-year-old woman with no medical history was admitted to our hospital for a live-liver donation to her husband. During her evaluation, magnetic resonance cholangiopancreatography (MRCP) revealed a previously unknown anatomic variation in her biliary system. Segment 2 of the bile duct (B2) independently drained into the posterior branch and formed a common channel (B2+posterior) before joining the anterior branch. Then, bile duct segments 3 and 4 (B3+4) drained into this B2+posterior+anterior channel to form a common hepatic duct. The computerized overlay features shown by MRCP and three-dimensional computed tomography clarified this anatomic variation. A right lobe donor graft was then obtained successfully, with intraoperative cholangiography confirming that the donated graft had two bile duct orifices (i.e., posterior and anterior branches). We thus avoided surgical missteps that would have disallowed bile drainage of B2 and B3+4 into the common hepatic duct. Discussion: Precise evaluation is mandatory for hepatobiliary surgical planning to rule out, or discover, challenging bile duct anatomy. Conclusion: Preoperative computerized overlay visualization of MRCP and computed tomography allowed definition of a previously unknown biliary tree variation.

DOI： 10.1016/j.ijscr.2021.01.042

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PubMed

Language：English   Publisher：Surgical Endoscopy  

Background: Native liver survival after laparoscopic Kasai portoenterostomy (Lap-PE) for biliary atresia (BA) is controversial. We examined whether a jaundice-free native liver survival rate is comparable between conventional Kasai portoenterostomy (Open-PE) and Lap-PE. Then, the impact of the two types of PE on subsequent living-donor liver transplantation (LTx) was addressed in this study. Methods: The jaundice-free rate in 1- and 2-year-old patients who underwent Open-PE and Lap-PE from January 2006 to December 2017 was investigated. Additionally, perioperative data (duration from the start of surgery to the completion of hepatectomy and others) of patients aged 2 years or younger who underwent LTx after either Open-PE or Lap-PE from 2006 to 2017 were evaluated. Results: Thirty-one (67%) out of 46 Open-PE patients and 23 (77%) out of 30 Lap-PE patients showed native liver survival with jaundice-free status at 1 year of age (p = 0.384); 29 (63%) out of 46 Open-PE patients and 19 (70%) out of 27 Lap-PE patients showed native liver survival with jaundice-free status at 2 years of age (p = 0.524); there were no significant differences. Additionally, there were 37 LTx cases after PE within 2 years of birth, including 29 Open-PE and 8 Lap-PE cases. The patients in the Lap-PE group had fewer adhesions and significantly shorter durations of surgery up to the completion of the recipient’s hepatectomy and durations of post-LTx hospital stay compared to the Open-PE group. There were no differences in blood loss or duration of stay in intensive care unit between the Lap-PE and Open-PE groups. Conclusions: Jaundice-free native liver survival rate has been comparable between Open-PE and Lap-PE. Lap-PE resulted in fewer adhesions, contributing to better outcomes of subsequent LTx compared to Open-PE.

DOI： 10.1007/s00464-019-07108-y

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PubMed

Language：English   Publisher：Journal of Gastrointestinal Surgery  

Background: Portal vein stenosis develops in 3.4–14% of split liver transplantation1–3 and its early detection and treatment are essential to achieve long-term graft survival,2–5 although the diagnostic capability of conventional modalities such as Doppler ultrasound and computed tomography is limited.1,4,5 Methods: This study used computational fluid dynamics to analyze portal vein hemodynamics in the management of post-transplant portal vein stenosis. To perform computational fluid dynamics analyses, three-dimensional portal vein model was created using computed tomographic DICOM data. The inlet flow condition was set according the flow velocity measured on Doppler ultrasonography. Finally, portal vein flow was simulated on a fluid analysis software (Software Cradle, Japan). Results: An 18-month-old girl underwent liver transplantation using a left lateral graft for biliary atresia. At the post-transplant 1-week evaluation, the computational fluid dynamics streamline analysis visualized vortices and an accelerated flow with a velocity ratio < 2 around the anastomotic site. The wall shear stress analysis revealed a high wall shear stress area within the post-anastomotic portal vein. At the post-transplant 6-month evaluation, the streamline analysis illustrated the increased vortices and worsening flow acceleration to reach the proposed diagnostic criteria (velocity ratio > 3:1).3,5 The pressure analysis revealed a positive pressure gradient of 3.8 mmHg across the stenotic site. Based on the findings, the patient underwent percutaneous transhepatic portal venoplasty with balloon dilation. The post-treatment analyses confirmed the improvement of a jet flow, vortices, a high wall shear stress, and a pressure gradient. Discussion: The computational fluid dynamics analyses are useful for prediction, early detection, and follow-up of post-transplant portal vein stenosis and would be a promising technology in post-transplant management.

DOI： 10.1007/s11605-019-04279-w

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PubMed

Language：English   Publisher：Annals of Transplantation  

Background: In the field of living donor liver transplantation (LDLT), it is important to ensure donor’s psychological well-be-ing. We report on clinical features and long-term outcomes of LDLT donors who developed psychiatric disorders after their donor operations. Additionally, we compare patient backgrounds, as well as surgical and perioperative aspects between LDLT donors with and without postoperative psychiatric complications. Material/Methods: Between November 1998 and March 2018, we identified 254 LDLT donors at our hospital. Among these, we investigated those who had newly developed psychiatric complications and required psychiatric treatment after donor operation. Results: The median duration of follow-up was 4 years. Sixty-five donors were lost to follow-up. Eight donors (3.1%) developed postoperative psychiatric complications, including major depressive disorder in 4, panic disorder in 2, conversion disorder and panic disorder in 1, and adjustment disorder in 1. The median duration from donor surgery to psychiatric diagnosis was 104.5 days (range, 12 to 657 days) and the median treatment duration was 18 months (range, 3 to 168 months). Of those, 3 donors required psychiatric treatment over 10 years, and 4 donors remained under treatment. The duration of hospital stay after donor operation was significantly longer and perioperative complications with Clavien classification greater than grade IIIa were more frequent in donors with psychiatric complications than in those without psychiatric complications (P=0.02 and P=0.006, respectively). Conclusions: Accurate diagnosis and appropriate treatment for psychiatric disorders by psychiatrists and psychologists are important during LDLT donor follow-up. Minimization of physiological complications might be important to prevent postoperative psychiatric complications in LDLT donors.

DOI： 10.12659/AOT.918500

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Scopus

PubMed

Language：English   Publisher：Clinical Transplantation  

Background: Long-term outcomes after endoscopic treatment of post-transplant biliary complications have not been fully understood. This study aimed to evaluate the impact of biliary complications on graft survival after right-lobe living-donor liver transplantation (R-LDLT). Method: From a single-institutional prospectively maintained database, all patients who underwent R-LDLT between 1999 and 2017 were included. Data on patient demographics, complications, endoscopic treatment, and graft survival were retrieved for analyses. Results: Among 111 patients who underwent R-LDLT, 33 (29.7%) developed biliary complications; of these, 19 (17.1%) were treated with biliary stenting, and the stent was removed following resolution of biliary complications in 8 of the 19 (42.1%) patients. The graft survival rate was 88.0% and 85.6% at 5- and 10-year follow-up, respectively, in patients without biliary complications, which was similar to that of the patients with resolved biliary complications (81.3% at 5- and 10-year follow-up, P =.68) but higher than that of patients having persistent (unresolved) biliary complications (61.4% and 49.1% at 5- and 10-year follow-up, respectively, P =.04). Conclusion: Post-transplant persistent biliary complications, unresolved after endoscopic management and requiring prolonged biliary stenting, are associated with inferior graft survival. However, patients with resolved biliary complications achieve a favorable long-term survival similar to patients without biliary complications.

DOI： 10.1111/ctr.13771

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PubMed

Language：Japanese   Publisher：一般社団法人 日本移植学会  

<p>1998年11月から2020年4月までに再移植を含め339例の肝移植を実施した。グラフト機能不全のため再移植を行った15例（初回移植が自施設11例、他施設4例）と再移植に至らず初回移植後に死亡した52例のうち明らかな他病死（癌やリンパ腫などの悪性疾患10例、重症感染症17例、クモ膜下出血などのその他の疾患9例）を除くグラフト機能低下のあった16例の計31例を検討した。一般的に再移植がグラフト機能不全の救命手段とされるが、再移植実施は半数以下であった。さらに、非再移植症例の16例のうち、2例は再移植の脳死登録待機中に死亡、残りの多くの症例が感染症や出血、腎不全を合併し、再移植に至らず死亡した。1年以内に再移植となった早期グラフト機能不全症例が4例、晩期グラフト機能不全症例が11例であったが、早期グラフト機能不全で1例、晩期グラフト機能不全で7例、計8例が再移植後に死亡した。死因は、敗血症が5例、難治性の拒絶反応による肝不全が2例、PNFが1例であった。初回移植の症例と比較して、再移植の症例は手術時間や術前ＭＥＬＤスコアに有意差は認めないものの、術中出血量、温阻血時間で有意差を認めた。肝移植後グラフト機能不全に陥った場合、再移植施行症例も非施行症例も予後不良であるため、再移植に至らないように術後管理が必要であると同時に、適切な再移植時期、適応基準などの検討が重要と考えられた。</p>

DOI： 10.11386/jst.55.supplement_271_1

CiNii Research

Language：English   Publisher：Transplantation Proceedings  

Introduction: Budd-Chiari syndrome (BCS) associated with hypereosinophilic syndrome (HES) is very rare, and only a few reports have described its treatment. Furthermore, no report to date has described the performance of liver transplantation for the treatment of BCS associated with HES. We herein describe a 54-year-old man who underwent deceased-donor liver transplantation (DDLT) for treatment of BCS associated with HES. Case: A 54-year-old man was found to have an increased eosinophil count during a medical check-up. After exclusion of hematopoietic neoplastic diseases and secondary eosinophilia, idiopathic hypereosinophilia was diagnosed. Oral prednisolone was administered to the patient, and his eosinophil count immediately decreased to a normal level. He had an uneventful course without complications for 11 months but then presented with bloating and malaise. Imaging studies including ultrasonography, enhanced computed tomography, and angiography revealed BCS associated with HES. Transjugular intrahepatic portosystemic shunt failed because of complete obstruction of the hepatic veins. Therefore, the patient was introduced to our hospital for liver transplantation. DDLT was performed with venovenous bypass 1 month after the patient was placed on the DDLT waiting list. The explanted hepatic veins were completely occluded and organized. The patient's eosinophil count was maintained at a normal level with prednisolone treatment after DDLT. Conclusions: Liver transplantation can be a treatment option for BCS associated with HES if neoplastic diseases and secondary eosinophilia have been excluded. Life-long oral steroid therapy is required to control HES even after liver transplantation.

DOI： 10.1016/j.transproceed.2019.08.003

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Language：Japanese  

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Language：English   Publisher：Hepatobiliary and Pancreatic Diseases International  

Background: Graft inflow modulation (GIM) during adult-to-adult living donor liver transplantation (LDLT) is a common strategy to avoid small-for-size syndrome, and some transplant surgeons attempt small size graft strategy with frequent GIM procedures, which are mostly performed by splenectomy, in LDLT. However, splenectomy can cause serious complications such as portal vein thrombosis and overwhelming postsplenectomy infection. Methods: Forty-eight adult-to-adult LDLT recipients were enrolled in this study and retrospectively reviewed. We applied the graft selection criteria, which routinely fulfill graft-to-recipient weight ratio ≥ 0.8%, and consider GIM as a backup strategy for high portal venous pressure (PVP). Results: In our current strategy of LDLT, splenectomy was performed mostly due to hepatitis C and splenic arterial aneurysms, but splenectomy for GIM was intended to only one patient (2.1%). The final PVP values ≤ 20 mmHg were achieved in all recipients, and no significant difference was observed in patient survival or postoperative clinical course based on whether splenectomy was performed or not. However, 6 of 18 patients with splenectomy (33.3%) developed postsplenectomy portal vein thrombosis (PVT), while none of the 30 patients without splenectomy developed PVT after LDLT. Splenectomy was identified as a risk factor of PVT in this study (P < 0.001). Our study revealed that a lower final PVP could be risk factor of postsplenectomy PVT. Conclusions: Using sufficient size grafts was one of the direct solutions to control PVP, and allowed GIM to be reserved as a backup procedure. Splenectomy should be avoided as much as possible during LDLT because splenectomy was found to be a definite risk factor of PVT. In splenectomy cases with a lower final PVP, a close follow-up is required for early detection and treatment of PVT.

DOI： 10.1016/j.hbpd.2019.06.011

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Language：Japanese  

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1111/tid.12911

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1016/j.ijscr.2018.04.026

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.12659/AJCR.906446

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Language：English   Publishing type：Research paper (scientific journal)  

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1016/j.transproceed.2017.06.009

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.12659/AOT.902791

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Language：English   Publishing type：Research paper (scientific journal)  

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.3748/wjg.v23.i5.869

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：公益社団法人 日本理学療法士協会  

<p>【はじめに，目的】末期肝障害患者の多くは，筋力や運動耐容能などの身体機能が低下している。身体機能の低下は肝移植術により数ヵ月から数年後に改善すると報告されているが，移植術後短期間の在院中にどのように変化するかは明らかではない。肝移植周術期における理学療法介入は，他の外科周術期と同様，術後合併症の予防や身体機能回復の促進を目的に行われており，筋力や運動耐容能の経過を把握することは重要である。肝移植による身体機能に関する報告の多くは脳死肝移植術を対象とした海外からのものであり，わが国で多く行われている生体肝移植術の報告は無い。そこで本研究は，生体肝移植術前後の身体機能の変化と，それらに関連する因子を検討することを目的とした。【方法】2014年4月から2015年12月までに当院で生体肝移植術を施行され，周術期理学療法が実施された成人21例を対象とした後方視的観察研究を行った。うち術前と術後4週目で評価が可能であった12例については，移植前後での身体機能を比較し，筋力の評価として握力ならびに等尺性膝伸展筋力，運動耐容能の評価として6分間歩行距離（6MWD）を測定した。術前後の評価指標についてはWilcoxonの符号順位検定を用いて比較し，各指標の相関はSpearmanの順位相関係数を用いて算出した。統計学的解析では有意水準を危険率0.05未満とした。【結果】評価群（n=12）と非評価群（n=9）では術前肝障害重症度の指標であるMELD scoreに有意差はみられなかった。評価群は全例が移植後自宅退院であったが，非評価群では2例が死亡退院であった。評価群の移植後の歩行開始は平均5.7日（2-17日），術後在院日数は平均54.2日（31-125日）であった。術前6MWDとMELD score（r=-0.646，P=0.023）ならびに呼吸機能の%VC（r=0.757，P=0.004），%FVC（r=0.776，P=0.003），%FEV1（r=0.734，P=0.007）には有意な相関がみられた。術前後の比較において6MWDに有意差はみられなかった（365.9±141.3 m vs. 341.1±139.7 m，P=0.182）が，握力（21.4±5.4 kgf vs. 17.3±5.9 kgf，P=0.015）と等尺性膝伸展筋力（19.1±5.4 kgf vs. 13.5±5.8 kgf，P=0.012）において有意な低下を認めた。術前MELD scoreと握力減少率（r=-0.604，P=0.038），等尺性膝伸展筋力減少率（r=-0.786，P=0.002）との間には有意な負の相関がみられた。【結論】生体肝移植術後4週の時点では，筋力は術前のレベルまで十分に回復しなかった。今後，移植後の筋力低下の軽減，早期回復を促す理学療法の確立が望まれる。</p>

DOI： 10.14900/cjpt.2016.0763

CiNii Research