INTRODUCTION:Cell-free and concentrated ascites reinfusion therapy (CART) has been widely used for malignant ascites and decompensated cirrhosis, but its perioperative application remains unclear. We evaluated CART after pediatric living donor liver transplantation (LDLT). METHODS:We retrospectively reviewed pediatric LDLT recipients who underwent CART for refractory ascites. Changes in ascites volume, protein recovery rates including albumin and immunoglobulin G (IgG), and adverse events were analyzed. RESULTS:Ten recipients underwent 92 CART sessions. Median ascites volume decreased from 1785 mL to 362 mL after concentration. Median recovery rates were 70.1% for total protein, 71.8% for albumin, 72.0% for IgG, 76.2% for antithrombin III, and 109.4% for coagulation factor XIII. Larger ascites volumes were weakly associated with higher recovery efficiency. No serious adverse events occurred. CONCLUSIONS:CART may be a safe and useful option for managing refractory ascites and protein recovery after pediatric LT while minimizing reinfusion volume.
Background: The superiority of thoracoscopic repair (TR) over conventional open repair (COR) for esophageal atresia, especially in terms of long-term outcomes, remains to be investigated. The aim of this study was to compare shortand long-term results between TR and COR group. Methods: Patients who underwent TR or COR for esophageal atresia without other predispositions to musculoskeletal deformities (2003-2016) and had been followed up for a minimum of 5 years were retrospectively reviewed. Musculoskeletal deformities (e.g., scoliosis, chest wall asymmetry, and rib deformities) were mainly evaluated based on the most recent chest radiographs. Results: Nine and eight patients were included in the TR and COR groups, respectively; the mean followup period was 8.7 and 11.5 years, respectively (p = 0.14). These groups had similar epidemiological characteristics and rates of postoperative complications. Musculoskeletal deformities developed significantly less frequently in the TR group versus the COR group (11 % vs. 88 %, p < 0.05; scoliosis: 0 % vs. 38 %, p = 0.08; chest wall asymmetry: 11 % vs. 50 %, p = 0.14; and rib deformities: 11 % vs. 88 %, p < 0.05, respectively). Conclusion: TR was associated with a decreased incidence of musculoskeletal deformities and comparable complication rates versus COR for esophageal atresia repair. TR may achieve better long-term outcomes in this setting. (c) 2024 Asian Surgical Association and Taiwan Robotic Surgery Association. Publishing services by Elsevier B.V. This is an open access article under the CC BY license (http://creativecommons.org/licenses/ by/4.0/).
Liver cirrhosis due to secondary sclerosing cholangitis caused by Langerhans cell histiocytosis (LCH) has a poor prognosis, and liver transplantation is the definitive treatment. However, the optimal timing has not been established. We report a 2-year-old girl with LCH-related liver cirrhosis who successfully underwent liver transplantation before progressing to severe liver dysfunction. Physical examination revealed a tumor on her palate. Biopsy was performed, and a diagnosis of LCH was established, together with hepatomegaly, splenomegaly, and rashes. Percutaneous liver biopsy before treatment revealed extreme fibrosis and absence of LCH cells. After beginning chemotherapy, she experienced several delays in treatment and dose reductions because of unacceptable bone marrow suppression, worsening liver dysfunction, and cholangitis. However, tumor shrinkage was observed in both magnetic resonance imaging and BRAF V600E mutant allele titers in her plasma. Given the good treatment response, liver transplantation was conducted. The postoperative course was uneventful, and chemotherapy was resumed 34 days after liver transplantation. Subsequent maintenance treatment was completed with no severe adverse effects. To prevent perioperative complications due to exacerbation of liver dysfunction and possible discontinuation of chemotherapy, liver transplantation should be considered before development of end-stage liver failure, provided that the original disease is well controlled.
Pediatrics InternationalVolume 64, Issue 1 e15266 Clinical Note Spontaneous recovery from diaphragmatic paralysis after liver transplantation Takahiro Hosokawa, Corresponding Author Takahiro Hosokawa snowglobe@infoseek.jp orcid.org/0000-0001-9523-4077 Department of Radiology, Saitama Children's Medical Center, Saitama, JapanCorrespondence: Takahiro Hosokawa, MD, Department of Radiology, Saitama Children's Medical Center, 1-2 Shintoshin Chuo-ku Saitama, Saitama, 330-8777 Japan. Email: snowglobe@infoseek.jpSearch for more papers by this authorYutaka Tanami, Yutaka Tanami Department of Radiology, Saitama Children's Medical Center, Saitama, JapanSearch for more papers by this authorYoshiyuki Ihara, Yoshiyuki Ihara Department of Transplant Surgery, Saitama Children's Medical Center, Saitama, JapanSearch for more papers by this authorKoichi Mizuta, Koichi Mizuta Department of Transplant Surgery, Saitama Children's Medical Center, Saitama, JapanSearch for more papers by this authorEiji Oguma, Eiji Oguma Department of Radiology, Saitama Children's Medical Center, Saitama, JapanSearch for more papers by this author Takahiro Hosokawa, Corresponding Author Takahiro Hosokawa snowglobe@infoseek.jp orcid.org/0000-0001-9523-4077 Department of Radiology, Saitama Children's Medical Center, Saitama, JapanCorrespondence: Takahiro Hosokawa, MD, Department of Radiology, Saitama Children's Medical Center, 1-2 Shintoshin Chuo-ku Saitama, Saitama, 330-8777 Japan. Email: snowglobe@infoseek.jpSearch for more papers by this authorYutaka Tanami, Yutaka Tanami Department of Radiology, Saitama Children's Medical Center, Saitama, JapanSearch for more papers by this authorYoshiyuki Ihara, Yoshiyuki Ihara Department of Transplant Surgery, Saitama Children's Medical Center, Saitama, JapanSearch for more papers by this authorKoichi Mizuta, Koichi Mizuta Department of Transplant Surgery, Saitama Children's Medical Center, Saitama, JapanSearch for more papers by this authorEiji Oguma, Eiji Oguma Department of Radiology, Saitama Children's Medical Center, Saitama, JapanSearch for more papers by this author First published: 30 September 2022 https://doi.org/10.1111/ped.15266Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Supporting Information Filename Description ped15266-sup-0001-TableS1.docxWord 2007 document , 27.6 KB Table S1. Previous case reviews with phrenic nerve paralysis after liver transplantation. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article. Volume64, Issue1January/December 2022e15266 RelatedInformation
In a series of studies, using an identical rat intestinal transplantation model, we evaluated the effects of several drugs. FK-506 caused a significant attenuation in the proliferation of allogeneic CD4+ T cells and IFN-γ secreting effector functions. FYT720 resulted in a marked reduction in the numbers of lymphocytes, associated with a reduction of T cell recruitment, in grafts. An anti-MAdCAM antibody was next reported to significantly down-regulate CD4+ T cell infiltration in intestinal grafts by blocking the adhesion molecule, and could be useful as an induction therapy. Concerning TAK-779, this CCR5 and CXCR3 antagonist diminished the number of graft-infiltrating cells by suppressing the expression of their receptors in the graft. As a result, it reduced the total number of recipient T cells involved in graft rejection. As the next step, we focused on the participation of monocytes/ macrophages in this field. PQA-18 has been the focus of a novel immunosuppressant that attenuates not only the production of various cytokines, such as IL-2 & TNF-α, on T cells, but the differentiation of macrophages by inhibiting PAK2 as well. In this report, we summarize our previous studies not only regarding the above drugs, but on an anti-complement drug and a JAK inhibitor as well.
Early relaparotomy of adult recipients after living donor liver transplantation (LDLT) is significantly associated with poor prognosis. However, there are few reports focusing on pediatric recipients after LDLT. The aim of this study is to clarify the causes and outcomes of early relaparotomy after pediatric LDLT. A total of 265 pediatric recipients (272 LDLTs) transplanted from May 2001 to October 2015 were retrospectively analyzed. Early relaparotomy was defined as surgical intervention performed within 3 months after LDLT. Early relaparotomy was performed 49 times for 33 recipients (12.5%). The recipient and graft survival rates in the early relaparotomy group were significantly lower than those in the nonearly relaparotomy group, respectively (75.0% and 63.6% versus 96.6% and 95.8%; both P < 0.001). Left lateral segment grafts were used significantly more frequently in the nonrelaparotomy group (P = 0.01). According to the multivariate analysis, the preoperative Pediatric End-Stage Liver Disease (PELD)/Model for End-Stage Liver Disease (MELD) score of the early relaparotomy group was significantly higher than that of the nonearly relaparotomy group (13.7 versus 6.3; P = 0.04). According to the receiver operating characteristic curve, the preoperative PELD/MELD score cutoff point was 17.2. Early relaparotomy due to infectious causes led to significantly poorer graft survival than that due to noninfectious causes (P = 0.04). In conclusion, the recipient and graft survival rates of the early relaparotomy group were significantly lower than those of the nonearly relaparotomy group. A high preoperative PELD/MELD score was a risk factor for early relaparotomy. In particular, early relaparotomy due to infection showed a poor prognosis.
Ligamentum teres hepatis (LTH) abscess is extremely rare, with few case reports stating that surgical excision should be considered rather than conservative treatment. We report a case of LTH abscess in a child with successful conservative therapy. A 1-month-old girl was transferred from another hospital due to a space-occupying lesion in the liver. Contrast enhanced computed tomography and abdominal ultrasonography revealed an LTH abscess. Staphylococcus aureus was identified from the abscess contents. Cefazoline was administered for 14 days, after which the lesion could not be identified with ultrasonography. The abscess had not recurred by the 6-month outpatient clinic follow-up visit. Unlike other reported cases, in our case, the abscess decreased in size with conservative treatment, thus our case could be improved.
Niemann-Pick disease type C (NPC) is a rare autosomal recessive inherited disease characterized by lysosomal accumulation of free cholesterol in macrophages within multiple organs. Infantile-onset NPC often presents with jaundice and hepatosplenomegaly from birth, but these symptoms usually improve during early childhood, and it rarely progresses to liver failure. We report three cases from different hospitals in Japan; the patients developed neonatal-onset NPC, and liver transplantation (LT) was performed as a life-saving procedure. LT was performed at 19 days, 59 days, and 4 months of age, respectively. The last patient was diagnosed with NPC before LT, while the first two patients were diagnosed with neonatal hemochromatosis at LT. In these two patients, the diagnosis of NPC was made more than a year after LT. Even though oral administration of miglustat was started soon after the diagnosis of NPC, all patients showed neurological regression and required artificial respiratory support. All patients survived more than one year after LT; however, one patient died due to tracheal hemorrhage at 4.5 years of age, and another one patient was suspected as recurrence of NPC in liver graft. In conclusion, while LT may be a temporary life-saving measure in patients with neonatal-onset NPC leading to liver failure, the outcome is poor especially due to neurological symptoms. A preoperative diagnosis is thus critical.
BACKGROUND:We present retrospective analysis of our 15-year experience with pediatric living donor liver transplantation, focusing on the risk factors, treatments, and long-term prognosis for posttransplant biliary complications (BCs).METHODS:Between May 2001 and December 2017, 290 living donor liver transplantations were performed. The median age was 1.4 years old. The median observation period was 8.4 years. Biliary strictures were classified as anastomotic stricture (AS) or non-AS (NAS).RESULTS:Overall incidence of biliary complications was 18.6%, including AS in 46 cases, NAS in 6, and other classifications in 2. The mean period to diagnosis of the AS was 641 ± 810 postoperative days. The multivariate analysis showed that hepaticojejunostomy without external stent was an independent risk factor for AS (P = 0.011). The first treatments for AS were percutaneous transhepatic biliary drainage (PTBD) in 25 cases, double-balloon enteroscopy (DBE) in 19, and surgical reanastomosis in 2. The success and recurrence rates of PTBD treatments were 90.9% and 22.7%, respectively. The success and recurrence rates of endoscopic interventions under DBE were 93.6% and 75.3%, respectively. The 15-year graft survival rates in patients with and without AS were 95.7% and 89.1%, respectively (P = 0.255), but 2 patients with cholangitis due to multiple NAS underwent retransplantation.CONCLUSIONS:Posttransplant AS can be prevented by hepaticojejunostomy using external stent, and the long-term prognosis is good with early treatments using DBE or PTBD. However, the prognosis of multiple NAS is poor.
BACKGROUND:Intracranial and pulmonary vascular anomalies are well-known complications and causes of mortality in AGS; however, visceral artery anomalies are less commonly recognized. Herein, we present a retrospective analysis of our experience with pediatric LDLT that focuses on the current problems with and treatments for visceral artery anomalies in AGS after LDLT. METHODS:Between May 2001 and December 2017, 294 LDLTs were performed for 285 pediatric recipients. Of these, 13 LDLTs (4.4%) for 12 AGS patients were performed. We classified the visceral artery anomalies into aneurysms and stenosis. RESULTS:The overall incidence of visceral aneurysm was 2 of 12 recipients (16.7%) and included a SMA aneurysm in one patient and an IPDA aneurysm with a subsequent SPA aneurysm in one patient; the ages of the diagnosis of visceral aneurysm were 16.3, 21.1, and 21.7 y, respectively. An endovascular treatment was performed for a progressive IPDA saccular aneurysm (12.0 × 14.5 × 15.0 mm). The overall incidence of visceral artery stenosis was 7 of 12 recipients (58.3%) and the median age at the diagnosis of visceral artery stenosis was 15.5 y (range 1.7-22.9 y). All 3 AGS patients with RA stenosis suffered from renal dysfunction (eGFR of 51, 78, and 51 mL/min/1.73m2 ). CONCLUSION:The morbidity of visceral artery anomalies is not negligible. The performance of periodic imaging examinations is necessary, even for infants, because it is difficult to detect visceral vascular anomalies in the infant stage.
There are few long-term outcome reports for interventional radiology (IVR) treatments for vascular and biliary complications following pediatric living donor liver transplantation (LDLT). Herein, we presented our institution's experience and investigated the efficacy and issues of long-term outcome with IVR treatments. Between May 2001 and September 2016, 279 pediatric LDLTs were performed. The median age at LDLT was 1.4 years old, and the median observation period was 8.2 years. All the biliary reconstructions at LDLT were hepaticojejunostomy. The IVR treatments were selected as endovascular, radiological, or endoscopic interventions. Post-transplant hepatic vein, portal vein, hepatic artery, and biliary complications were present in 7.9%, 14.0%, 5.4%, and 18.3%, respectively. IVR treatment was the first treatment option in 81.8%, 94.9%, 46.7%, and 94.1%, respectively. The recurrence and cure rates following IVR treatment were 42.1%, 21.1%, 44.4%, and 34.0% and 84.2%, 97.4%, 100%, and 88.0%, respectively. The graft survival rates in patients with and without post-transplant vascular and biliary complications were 94.4% and 90.6%, respectively (P = 0.522). The IVR treatments for vascular and biliary complications following pediatric LDLT are the first choice option. Although the recurrence following IVR treatment is a major problem and it is necessary to carefully perform long-term follow-up, IVR treatments have good treatment outcomes.
[The copyright line for this article was changed on March 13, 2020 after original online publication.] Hironori Yamamoto consults for and received grants from Fujifilm Corp. Yukihiro Sanada is responsible for the study design. Yukihiro Sanada, Tomonori Yano, Taizen Urahashi, Yoshiyuki Ihara, Noriki Okada, Naoya Yamada, Yuta Hirata, and Takumi Katano are responsible for acquisition of data. Yukihiro Sanada and Tomonori Yano are responsible for analysis and interpretation. Tomonori Yano, Hironori Yamamoto, and Koichi Mizuta are responsible for the revision of the text. TO THE EDITOR: Hepaticojejunal anastomotic obstruction (HJO) is diagnosed when contrast medium delivered via percutaneous transhepatic cholangiography (PTC) does not flow into the jejunum or when the hepaticojejunal anastomotic site cannot be identified using enteroscopy. HJO after liver transplantation (LT) is a rare biliary complication,1 and intractable HJO can lead to severe complications, including graft failure. Although surgical revision is the first choice for the treatment of HJO, it is an invasive procedure that can cause additional injury. With the advances in and benefits of endoscopic instruments and techniques, however, endoscopic treatments for hepaticojejunal anastomotic stricture (HJS) offer a promising less‐invasive procedure.3 Since the development of instruments and techniques for double‐balloon enteroscopy (DBE),4 it has been possible to perform endoscopic retrograde cholangiography despite the length of the necessary passage, the strong adhesion of the Roux‐en‐Y limb to the peritoneum, and the difficult angulation of the hepaticojejunal anastomosis. The penetration and balloon dilatation of the HJO using combined percutaneous transhepatic cholangioscopy (PTCS) and DBE is performed.2 The anastomotic penetration procedure for HJO combined with PTCS and DBE compose the so‐called “rendezvous technique.” Our institution has reported a pediatric case study of the rendezvous technique for HJO after living donor liver transplantation (LDLT).2 However, the longterm patency of the hepaticojejunal anastomosis after the rendezvous technique is still unclear. Therefore, in this study, we described our experience with the rendezvous technique for HJO after pediatric LDLT and the longterm outcomes after HJO treatment. Patients and Methods This study included 370 outpatients who underwent LT between October 1988 and December 2016 at Jichi Medical University and other facilities. Between October 2005 and February 2009, 9 endoscopic interventions using the rendezvous technique were performed for 6 (1.6%) patients with HJO after pediatric LDLT. We evaluated 6 patients (3 males and 3 females) with a median age of 12.5 years (range, 5.4‐17.2 years), and a median body weight (BW) of 33.3 kg (range, 15.1‐47.5 kg). The original disease was biliary atresia (BA) in all 6 patients. The posttreatment observation period ranged between 7.5 and 10.9 years. Approval to conduct this study was obtained from the ethics committees of Jichi Medical University (ethics committee approval case number 15‐106). DIAGNOSIS OF HJO HJO is diagnosed when the contrast medium delivered via PTC does not flow into the Roux‐en‐Y limb,5 when no real‐time moving images are obtained under fluoroscopy, or when the hepaticojejunal anastomotic site cannot be confirmed with DBE. THERAPEUTIC STRATEGY FOR HJO We present the therapeutic strategy for HJS or HJO in Fig. 1. The indication for DBE is more than 15 kg of BW because of instrumental and technical limitations. Therefore, observation and oral administration of ursodeoxycholic acid is the recommended strategy for patients who weigh <15 kg and for those with no dilatation of the intrahepatic bile duct (IHBD).Figure 1: Therapeutic strategy for HJS/HJO after LT.When HJO is diagnosed, treatment using the rendezvous technique with DBE and PTCS is performed after the percutaneous transhepatic biliary drainage (PTBD). In patients with HJO, if the treatment by the rendezvous technique is unsuccessful, surgical revision is performed. Results A total of 9 endoscopic interventions using the rendezvous technique were performed for 6 patients with HJO after pediatric LDLT (Table 1). The median period between the HJO treatment and the LDLT was 3.1 years (range, 0.7‐13.0 years). The rendezvous techniques employed were PTCS plus DBE in 5 patients and PTCS plus jejunography using DBE in 1 patient. Table 1 - Clinical Findings of the Patients Who Underwent the Rendezvous Technique for HJO After LDLT Case and Year Original Disease/BW (kg) Age at HJO Treatment (year) Period Between HJO Treatment and LDLT (year) Rendezvous Technique Outcome Prognosis and the Second Treatment Case 1 2005 BA/37.5 13.7 0.7 PTCS plus jejunography using DBE (EN‐450P5/20) Success Recurrence‐free for 10.9 years (10.5 years after removal of tube) Case 2 2006 BA/27.3 12.2 0.8 PTCS plus DBE (EC‐450BI5) Success Recurrence at 7 months 2007 BA/33.3 12.8 1.3 PTCS plus DBE (EC‐450BI5) Success Recurrence at 7 months 2008 BA/35.0 13.3 1.9 PTCS plus DBE (EC‐450BI5) Success Recurrence‐free for 8.6 years (7.9 years after removal of tube) Case 3 2007 BA/47.5 17.2 13.0 PTCS plus DBE (EN‐450P5/20) Success Recurrence‐free for 9.4 years (7.2 years after removal of tube) Case 4 2007 BA/38.7 12.5 7.6 PTCS plus DBE (EN‐450P5/20) Failure Surgical revision Case 5 2008 BA/18.9 6.9 3.1 PTCS plus DBE (EN‐450P5/20) Success Recurrence‐free for 7.7 years (7.2 years after removal of tube) Case 6 2009 BA/15.1 5.4 4.2 PTCS plus DBE (EN‐450P5/20) Failure Re‐rendezvous penetration method 2009 BA/15.1 5.5 4.3 PTCS plus DBE (EC‐450BI5) Success Recurrence‐free for 7.5 years (5.9 years after removal of tube) The initial successful intervention and recurrence‐free rates were 66.7% and 71.4%, respectively. The final successful intervention and recurrence‐free rates were 83.3% and 83.3%, respectively. The median recurrence‐free period was 8.7 years (range, 7.5‐10.9 years). Case 4, the patient in whom the hepaticojejunal anastomotic site could not be identified, underwent a surgical revision. Two episodes of HJO recurrence after the rendezvous technique occurred in case 2, and the patient underwent endoscopic interventions using the rendezvous technique 3 times. These HJO recurrences occurred after the PTBD tube was pulled out of the IHBD. During the first treatment, a guidewire presented via the PTC route penetrated the intra‐abdominal cavity on the first attempt; during the second attempt, a guidewire presented via the PTC route penetrated to the Roux‐en‐Y limb, and the hepaticojejunal anastomotic site was dilated. The PTBD tube was placed beyond the hepaticojejunal anastomotic site. The first recurrence of HJO occurred 5 months after the PTBD tube was placed. During the second treatment, a guidewire presented via the PTC route penetrated to the Roux‐en‐Y limb directly, and the hepaticojejunal anastomotic site was dilated on the first attempt. The second episode of HJO recurrence took place 5 months after the first recurrence. During the third treatment, a guidewire via the PTC route penetrated to the Roux‐en‐Y limb directly, and the hepaticojejunal anastomotic site was dilated on the first attempt. The PTBD tube was placed for 8 months and removed, and no further HJO recurrence was reported. Five patients who underwent the rendezvous technique were able to have their PTBD tube removed. The median PTBD tube‐free period was 7.5 years (range, 5.9‐10.5 years). The graft and patient survival rates were both 100%. Discussion The “rendezvous technique” has been reported as the anastomotic penetration procedure used for severe duct‐to‐duct biliary anastomotic stricture using combined PTCS and endoscopic retrograde cholangiopancreatography, but there are few case reports of HJO using combined PTCS and DBE.2 It took a median of 3.1 years after LDLT to treat HJO in the present study. The HJS might have occurred at an early stage after LDLT. However, the mild liver dysfunction or mild dilatation of IHBD is observed for a longterm period. Therefore, HJS potentially progresses to HJO, and the treatment for HJO may be intractable and difficult. In the present study, the final successful and recurrence‐free rates when the rendezvous technique is used to treat HJO after LDLT were 83.3% and 83.3%, respectively, and the median recurrence‐free period was 8.7 years (range, 7.5‐10.9 years). When the rendezvous technique was unsuccessful in the present study (cases 4 and 6), it was because we could not reach the hepaticojejunal anastomotic site using DBE. However, if 3‐dimensional identification of the hepaticojejunal anastomotic site using real‐time moving images obtained with combined PTC and jejunography had been possible for case 1, the rendezvous technique could have been safely performed. In case 4, we could not identify the hepaticojejunal anastomotic site by jejunography. In case 6, we could not reach the hepaticojejunal anastomotic site using DBE (EN‐450P5/20), and therefore, we changed the scope of DBE to EC‐450BI5 and succeeded. The recurrence of HJO after the rendezvous technique is an important problem, but its cause is unclear. Two episodes of HJO recurrence occurred after the PTBD tube was pulled out of the IHBD in case 2. We hypothesized that the cause of recurrence in the case 2 patient was mispenetration1 and the short‐term placement of a PTBD tube.2 During the first treatment, the contrast agent was administered to the Roux‐en‐Y limb under direct vision with DBE to image the precise anastomosis site using fluoroscopy. To avoid mispenetration when using the rendezvous technique, a clip should be placed adjacent to the anastomosis site. Regarding the short‐term placement of a PTBD tube, the incidence rate of recurrent HJS after PTBD treatment for posttransplant HJS was 11.1%. We performed 279 LDLTs between May 2001 and December 2016 and performed PTBD treatment for 18 patients with HJS. Only 2 patients out of 18 (11.1%) patients developed recurrent HJS after PTBD tube removal, and the median duration of PTBD tube placement was 8 (4‐25) months. Therefore, we think that the cause of the second recurrence in the case 2 patient was the short‐term placement of the PTBD tube. In addition, the PTBD tube was placed for 8 months after the third treatment; after it was removed, there was no HJS recurrence. In conclusion, it is important for the patients with HJO to penetrate the anastomosis site under direct vision and three‐dimensional identification using combined PTCS and DBE. The rendezvous technique for HJO after LDLT provides a safe and less‐invasive treatment than surgical revision and offers a high success rate and longterm patency of the hepaticojejunal anastomosis.
Adverse eventA drug interaction leading to greater exposure to tacrolimus.Drug implicatedTacrolimus and Beni‐Madonna (a new cultivar citrus categorized as ‘Tangor’).The patientA 9‐month‐old girl with biliary atresia (body weight, 7.5 kg) taking tacrolimus after liver transplantation.Evidence that links the drug to the eventThe time course was consistent with the appearance of the interaction, which was confirmed by an increase in the blood concentration of tacrolimus. Dihydroxybergamottin was detected in peel of Beni‐Madonna and in peel and fruit pulp of grapefruit.ManagementAvoiding Beni‐Madonna intake.MechanismInhibition of activity of CYP3A4, P‐glycoprotein, or both, by Beni‐Madonna.Implication for therapyClinicians should be aware of this potential interaction, and patients taking drugs such as tacrolimus (the kinetics of which are affected by grapefruit juice) should avoid Beni‐Madonna intake.Hypothesis to be testedFurther study is required to determine if other Citrus species categorized as Tangor contain furanocoumarins.
Background and AimSerum Mac-2 binding protein glycosylation isomer (M2BPGi) is a novel fibrosis marker for various chronic liver diseases. We investigated the ability of M2BPGi to predict liver fibrosis in liver transplant (LT) recipients. MethodsA total of 116 liver biopsies were performed in 113 LT recipients. The serum level of M2BPGi was also measured on the same day. The median age at LT and liver biopsy was 1.1 and 11.8years, respectively. Serum M2BPGi levels and liver fibrosis status using METAVIR fibrosis score were compared. Immunohistological evaluation by anti--smooth-muscle actin (SMA) was performed, and the relationship between SMA positive rate and serum M2BPGi levels was investigated. ResultsThe median M2BPGi level was 0.78 (range, 0.22-9.50), and 65, 29, 16, 5, and 1 patient(s) had METAVIR fibrosis scores of F0, F1, F2, F3, and F4, respectively. In patients with F0 fibrosis, median M2BPGi level was 0.69 and was significantly lower than in patients with F1 (median 0.99, P<0.01), F2 (median 1.00, P=0.01), and F3 fibrosis (median 1.53, P<0.01). Area-under-the-curve analysis of the ability of M2BPGi level to predict liver fibrosis grade were>F1: 0.716,>F2: 0.720, and>F3: 0.900. Three patients with acute cellular rejection showed high levels of M2BPGi, which decreased after the treatment. A positive correlation existed between M2BPGi levels and SMA positive rate (r(2)=0.715, P<0.01). ConclusionMac-2 binding protein glycosylation isomer is a novel liver fibrosis marker in LT recipients and is also increased in patients with acute liver injuries, especially acute cellular rejection, even when fibrosis is absent.
When living donor liver transplantation (LDLT) is performed on small infant patients, the incidence of hepatic artery complications (HACs) is high. Here, we present a retrospective analysis that focuses on our surgical procedure for hepatic arterial reconstruction and the outcomes of monosegmental LDLT.
We report a case involving a rescued low birth weight infant (LBWI) with acute liver failure.CASE:The patient was 1594 g and 323/7 gestational wk at birth. At the age of 11 d, she developed acute liver failure due to gestational alloimmune liver disease. Exchange transfusion and high-dose gamma globulin therapy were initiated, and body weight increased with enteral nutrition. Exchange transfusion was performed a total of 33 times prior to living donor liver transplantation (LDLT). Her liver dysfunction could not be treated by medications alone. At 55 d old and a body weight of 2946 g, she underwent LDLT using an S2 monosegment graft from her mother. Three years have passed with no reports of intellectual disability or liver dysfunction. LBWIs with acute liver failure may be rescued by LDLT after body weight has increased to over 2500 g.