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Post-transplant patient survival. The post-transplant patient survival in cases with or without preceding Kasai procedure in Japan with a modified report of the Japanese Liver Transplantation Society (JLTS) 2013 (n = 1985)
Source publication
Purpose
Kasai portoenterostomy (KP) is the primary procedure for biliary atresia (BA). However, due to reports of poor outcomes of KP, primary liver transplantation (LT) is preferred in selected cases. In Japan, primary LT is limited with no study reports details, particularly regarding the indications. We conducted the first nationwide survey to a...
Citations
... Although early Kasai portoenterostomy (KP) may improve cholestasis to some extent, approximately 67% of the patients still require LT before adulthood [2]. And it is reported that the longterm survival rate with salvage LT is not as good as that of primary living donor liver transplantation (pLDLT) for BA patients [3,4]. Therefore, an increasing number of children in China with BA, aged below 1 year, are undergoing primary living donor liver transplantation (pLDLT) as a radical treatment. ...
Background
The survival rate of children with biliary atresia (BA) after liver transplantation (LT) is significantly improved, and their quality of life has attracted much attention.This study aimed to investigate the cognition and its influencing factors in children with BA after primary living donor LT (BA-pLDLT) during infancy.
Methods
Children with BA were recruited 6 months after pLDLT at Children’s Hospital of Chongqing Medical University (2018–2022). Demographic and clinical data were collected from the health information system. Cognition was assessed using the Chinese version of the Griffiths Mental Development scale (GMDS-C). Multivariate linear regression were used to analyze the influencing factors of their cognitive function.
Results
In total, 57 children with BA-pLDLT, aged 5.00(3.90–9.30) months at transplantation and 25.00(14.00-60.80) months at evaluation were included. The general developmental quotient (89.02 ± 12.07) and motor, language, eye-hand coordination, performance, and practical reasoning quotients of these children were significantly lower than the normative mean values of GMDS-C(P < 0.05). Of the 57 children, 16 (28.07%) had borderline developmental delay (DQ between 70 and 84), 3 (5.26%) had developmental delay (DQ < 70), and 11(19.29%) had language delay. Reoperation for biliary or vascular complications after pLDLT was a risk factor for decreased general development quotient and motor quotient and lower ZW at assessment was associated with decline motor quotient.
Conclusion
Children with BA-pLDLT have varying degrees of developmental delays in early life. Reoperation and nutritional deficiencies had adverse effects on cognitive development.
... There are studies that have compared the results of primary transplants to rescue transplants that do not show a higher mortality on the waiting list for the primary transplant group in spite of what may be a younger age or smaller size. In a small Japanese series of primary liver transplants done mostly for late diagnosis, no one died without receiving a transplant [54], and the post-LT survival was similar to the KPE group. Similarly, in an earlier comparison from 1997, of the two groups [55], children with primary transplants had a lower death rate while on the waiting list, shorter operative times for the transplant, less blood loss and had fewer post operative complications than the group with the KPE. ...
... However, the efficacy of KPE has been controversial for a long time, and the survival rates of 1,340 BA patients with native liver after KPE were 41%, 35%, 26% and 22% at 5, 10, 20 and 30 years in France, respectively (6). Therefore, it has been suggested that bypassing KPE and carrying out primary LT (pLT) may achieve a better prognosis (7,8), which has led to the increasing cases of BA children receiving pLT (BA-pLT) worldwide in the past 30 years (0.8%-15%) (9)(10)(11). In addition, given the liver source shortage in some regions due to ethical and religious reasons, the proportion of BA-pLT children receiving living donor LT is increasing dramatically. ...
Objective: To clarify the early growth and developmental characteristics of children with biliary atresia (BA) undergoing primary liver transplantation (pLT).
Methods: A prospective cohort study, which specifically focused on BA-pLT children, was conducted after the diagnosis of BA by following the children at the time of pLT and 1, 3, 5, 7 months and 1 year after pLT for growth and developmental monitoring. The growth parameters were calculated according to the WHO standard, and the developmental status was assessed using Denver Developmental Screening Tests.
Results: A total of 48 BA children who received pLT at the age of 5.00 ± 0.94 months were analyzed. The weight-for-age Z- value ( Z W ) and length-for-age Z -value ( Z L ) were higher than the head circumference-for-age Z- value ( Z HC ) at pLT ( P = 0.002 and 0.02), but they were all lower than the WHO growth standard ( Z = 0) ( P < 0.001). The Z W and Z HC decreased first and then returned to the population level at 1 year after pLT, while the Z L only returned to the preoperative status and was lower than the Z W and Z HC ( P < 0.001). Developmental screening showed that 35% (17/48) of the children were defined as suspicious and 15% (7/48) were abnormal at 1–4 months after pLT, the most likely time to be suspected of developmental delay. At 1 year after pLT, gross motor skill delay still existed (12/45, 27%), and language skill delay began to appear (4/45, 9%).
Conclusions: BA-pLT children suffer from growth and developmental problems. Low Z HC is the main growth problem before pLT, while low Z L is the problem after pLT. Developmental delays are significant after pLT, especially in motor and language skills. The current study suggested that further studies are warranted to clarify the long-term growth and developmental outcomes of BA-pLT children, to compare them with children undergoing the Kasai procedure and to explore their influencing factors and possible mechanisms.
... Primary liver transplant for children with biliary atresia is usually reserved for those children who present with advanced liver disease at the time of diagnosis. Reported incidences of pLT vary between 3-16% [9,[13][14][15][16][17][18], but have been as low as less than 1% in Japan [19,20] and as high as 40% in Brazil [11]. Comparing these incidences is challenging given the different rates of organ donation and organ availability in different cultures and countries. ...
Introduction:
Primary liver transplants (pLT) in patients with biliary atresia (BA) are infrequent, since most babies with BA undergo a prior Kasai portoenterostomy (KPE). This study compared transplant outcomes in children with BA with or without a prior KPE. We hypothesized that pLT have less morbidity and better outcomes compared to those done after a failed KPE.
Methods:
A retrospective review of patients with BA transplanted at our institution was performed. Patients were included if they received a pLT or if they were transplanted less than 2 years from KPE. Outcomes were compared between those groups. Comparisons were also made based on era (early: 1997-2008 vs. modern: 2009-2020). p < 0.05 was considered significant.
Results:
Patients who received a pLT were older at diagnosis (141.5 ± 46.0 vs. KPE 67.1 ± 25.5 days, p < 0.001). The time between diagnosis and listing for transplant was shorter in the pLT group (44.5 ± 44.7 vs. KPE 140.8 ± 102.8 days, p < 0.001). In the modern era, the calculated PELD score for the pLT was significantly higher (23 ± 8 vs. KPE 16 ± 8, p = 0.022). Two waitlist deaths occurred in the KPE group (none in pLT, p = 0.14). Both the duration of transplant surgery and transfusion requirements were similar in both groups. There was a significant improvement in graft survival in transplants after KPE between eras (early era 84.3% vs. modern era 97.8%, p = 0.025). The 1-year patient and graft survival after pLT was 100%.
Conclusions:
Patient and graft survival after pLT are comparable to transplants after a failed KPE but pLT avoids a prior intervention. There was no significant difference in pre- or peri-transplant morbidity between groups other than wait list mortality. A multicenter collaboration with more patients may help demonstrate the potential benefits of pLT in patients predicted to have early failure of KPE.
... Gunadi et al. reported that 4/7 cases (57.1%) of AGS who underwent KPE for the diagnosis of BA were classified into subgroup "o" [7]. This is a special subgroup that has been discussed in Japan, including the effectiveness of KPE [8]. However, according to a report from the Japanese Biliary Atresia Society in 2019, subgroup "o" was extremely rare, accounting for just 119 (3.5%) of 3356 registered patients with BA [9]. ...
PurposeThe differential diagnosis between Alagille syndrome (AGS) with extrahepatic bile duct obstruction (EHBDO) and biliary atresia (BA) is difficult. We report a case series of AGS with EHBDO with detailed validation of the morphological and histopathological features for the differential diagnosis of BA.Methods
Six liver transplantations (LTs) were performed for AGS with EHBDO. All patients were diagnosed with BA at the referring institution and the diagnosis of AGS was then confirmed based on a genetic analysis before LT. We verified the morphological and histopathological findings of the porta hepatis and liver at the diagnosis of BA and at LT.ResultsAll patients had acholic stool in the neonatal period and were diagnosed with BA by cholangiography. The gross liver findings included a smooth and soft surface, without any cirrhosis. The gross findings of the porta hepatis included aplasia of the proximal hepatic duct, or subgroup “o”, in five patients. The histopathological examination of the EHBD also revealed obstruction/absence of the hepatic duct. There were no patients with aplasia of the common bile duct.Conclusions
Aplasia of the hepatic duct and the macroscopic liver findings may help in to differentiate between AGS with EHBDO and BA.
... However, in the lack of an accurate prognostic factor, we depend on clinical and follow-up of liver function test to decided when to made the referral. Recently, topics of primary liver transplantation in selected BA cases were debated and produced no consensus [14,15]. Therefore, any biomarker that can predict the result of Kasai operation and guide toward the next management, whether to go on Kasai portoenterostomy operation or primary liver transplantation would be of immense value [16]. ...
Background
The prognosis of biliary atresia (BA) remains difficult to predict. This study evaluated the roles of hepatocyte growth factor (HGF) and its receptor (C-met) towards clinical outcome and native liver survival.Methods
Hepatic HGF and C-met expression were determined using immunohistochemistry from liver biopsies of 41 BA patients during Kasai operation, and 17 non-cholestatic patients. The HGF and C-met expression was visually scored as per its intensity and percentage of stained area. BA patients were classified as high- and low-HGF and C-met receptor status. Native liver survival was compared between the two groups at 3-year follow-up. Data are shown as median and range.Main resultsMedian age of BA patients was 2 (1–6) months. Hepatic HGF and C-met staining scores of BA patients were higher than those of non-cholestatic patients (P < 0.0001). There was a correlation between HGF and C-met staining scores (spearman r = 0.77, P < 0.0001). However, there was no association between their expression and early outcome at 6 months post-op. Mean follow-up time was 68.6 months. Survival analysis revealed that native liver survival at 1 year and 3 years were 88% and 77%, respectively. Additionally, 82.6% (19/23) of patients in the low-HGF group survived with native liver, compared with 66.7% (10/15) of those in high-HGF group (P = 0.436). For C-met expression, 78.6% (22/28) of low-score and 70% (7/10) of high score groups survived with native liver (P = 0.673).Conclusions
Strong expression of hepatic HGF and its receptor in BA patients was demonstrated. However, the expression was not associated with the early outcome and native liver survival. These results suggest that HGF involved in the liver pathology of BA but its expression cannot be used as a prognostic indicator. Small sample size of patients was a main limitation. Further studies are warranted to validate our findings.
Background
Biliary atresia (BA) is a serious disease in infants that leads to liver cirrhosis and failure. Most patients die within 2 years of age in the absence of surgery. Therefore, we aimed to evaluate the cognitive function and explore influencing factors in young children with BA after primary living donor liver transplantation (BA-pLDLT) during infancy.
Methods
Children with BA were recruited 6 months after pLDLT at Children's Hospital of Chongqing Medical University (2018–2022). Demographic and clinical data were collected from the health information system. Cognitive function was assessed using the Chinese version of the Griffiths Mental Development scale (GMDS-C). Multivariate linear regression were used to analyze the influencing factors of cognitive function.
Results
In total, 57 children with BA -pLDLT, aged 5.00(3.90–9.30) months at transplantation and 25.00(14.00-60.80) months at evaluation were included. The general developmental quotient (89.02 ± 12.07) and motor, language, eye-hand coordination, performance, and practical reasoning quotients were significantly lower than the normative mean values of GMDS-C(P < 0.05). Of the 57 children, 16 (28.07%) had borderline developmental delay (70–84), 3 (5.26%) had developmental delay (< 70), and 11(19.29%) had language delay. Reoperation for biliary or vascular complications after transplantation was a risk factor for decreased general and motor developmental quotients (AQ) and low ZW at assessment was associated with decline AQ.
Conclusion
Children with BA-pLDLT have varying degrees of developmental delays during early life. Reoperation and nutritional deficiencies had adverse effects on cognition.
Background:
Several surgical strategies, including split donor transplantation and living donor transplantation, have been used to increase the donor liver pool. This report focuses on the effects of whole, split, and LDLT on recipient outcomes.
Methods:
We retrospectively analyzed the records of all patients with biliary atresia at Tianjin First Central Hospital between April 2013 and December 2019.
Results:
A total of 882 patients were included and divided into three groups by graft type, with 198 in the whole-liver-transplantation group, 78 in the split liver transplantation group, and 606 in the LDLT group. The median follow-up time was 39 months, patient survival rates of three groups were 94.4%, 88.5%, and 95.0%, respectively, and graft survival rates were 90.2%, 83.3%, and 94.7%, respectively. We divided the split liver transplantation group into two subgroups according to the donor's age, and patient survival rates exhibited a significant difference only in the group whose donor age was over 45 years. The postoperative complication rates were significantly higher with respect to hepatic artery thrombosis, portal stenosis, and AR; and lower in hepatic venous stenosis, PTLDs, CMV virus, and EBV infection in the WLT group. Our multivariate model showed that donor age ≥45 years, RBC transfusion, pneumonia, and HAT were the independent predictors of allograft loss.
Conclusions:
The survival of split liver transplantation group was slightly lower. The types of complications are different from different graft types. Therefore, postoperative monitoring and treatment need to be adjusted according to the different graft types used.
Introduction Age at Kasai portoenterostomy (KPE) has been identified as a predictive factor for native-liver survival in patients with biliary atresia (BA). Outcomes of pediatric liver transplantation (LT) have improved over recent years. It has been proposed to consider primary LT as a treatment option for late-presenting BA infants instead of attempting KPE. We present our experience with patients older than 90 days undergoing KPE.
Materials and Methods A retrospective chart review of patients with BA undergoing KPE at our institution between January 2010 and December 2020 was performed. Patients 90 days and older at the time of surgery were included. Patients' characteristics, perioperative data, and follow-up results were collected. Eleven patients matched the inclusion criteria. Mean age at KPE was 108 days (range: 90–133 days).
Results Postoperative jaundice clearance (bilirubin < 2 mg/dL) at 2-year follow-up was achieved in three patients (27%). Eight patients (73%) received a liver transplant at a mean of 626 days (range: 57–2,109 days) after KPE. Four patients (36%) were transplanted within 12 months post-KPE. Two patients died 237 and 139 days after KPE due to disease-related complications. One patient is still alive with his native liver, currently 10 years old.
Conclusion Even when performed at an advanced age, KPE can help prolong native-liver survival in BA patients and offers an important bridge to transplant. In our opinion, it continues to represent a viable primary treatment option for late-presenting infants with BA.
The Japanese Biliary Atresia Registry (JBAR) was launched in 1989 by the Japanese Biliary Atresia Society (JBAS). The Japanese Biliary Atresia Registry (JBAR) used an initial questionnaire, a questionnaire for liver transplantation, and a follow-up questionnaire. Each patient is to be followed up for 40 years. An annual report of the JBAR has been published in the Journal of the Japanese Society of Pediatric Surgeons. In addition, aggregation data can be browsed on the JBAS website (https://jbas.net/en/). Between 1989 and 2018, 3483 patients were registered in the JBAR. Among them, 1446 patients underwent liver transplantation. The 20-year overall and native-liver survival rates were 86.4% and 42.5%, respectively. The age at Kasai portoenterostomy had a significant impact on the long-term native liver survival rate (p < 0.001). Surgical outcomes of biliary atresia have markedly improved, owing to the cooperation of Kasai portoenterostomy and liver transplantation. JBAR also functions as a research infrastructure for biliary atresia. To date, several research projects have been conducted using this function.
