P0361 NEONATAL CHOLESTASIS, BILIARY ATRESIA AND LIVER TRANSPLANTATION FROM MALAYSIA

2004 ◽  
Vol 39 (Supplement 1) ◽  
pp. S193-S194
Author(s):  
W. S. Lee ◽  
C. C. M. Boey ◽  
T. M. Ramanujam
Keyword(s):  
Liver Transplantation ◽  
Biliary Atresia ◽  
Neonatal Cholestasis

scholarly journals De novo gastric cancer developing after liver transplantation from deceased donor for biliary atresia: a case report

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Naruki Higashidate ◽  
Suguru Fukahori ◽  
Shinji Ishii ◽  
Nobuyuki Saikusa ◽  
Naoki Hashizume ◽  
...  
Keyword(s):  
Gastric Cancer ◽  
Liver Transplantation ◽  
Biliary Atresia ◽  
De Novo ◽  
Epigastric Pain ◽  
Gastroesophageal Junction ◽  
Deceased Donor ◽  
Lymphoproliferative Disease ◽  
Endoscopic Examination ◽  
De Novo Malignancy

Abstract Background Apart from Kasai’s procedure, liver transplantation (LTx) has dramatically improved the outcome of children with biliary atresia (BA). However, de novo malignancy has been reported to be one of the major causes of late mortality after LTx among adults. We report a rare case of de novo gastric cancer developing after LTx for BA received during childhood. Case presentation A 21-year-old male patient who had undergone LTx for BA at age 2 years occasionally visited our outpatient clinic due to symptoms of epigastric pain and dysphagia. Endoscopic examination and computed tomography revealed advanced gastric cancer at the gastroesophageal junction with multiple liver metastases. Despite systemic chemotherapy, the disease progressed, resulting in patient’s death 2 years after the diagnosis. Conclusions De novo malignancy in the absence of post-transplant lymphoproliferative disease is rare in pediatric patients who received LTx. To the best of our knowledge, no report has been available on the development of gastric cancer after LTx for BA during childhood. Primary physicians should therefore establish a follow-up plan for patients receiving LTx for BA considering the potential for the development of de novo malignancy, including gastric cancer, despite its rarity.

scholarly journals Simultaneous pancreaticoduodenectomy and liver transplantation for biliary atresia complicated by choledochal cyst

2021 ◽  
Vol 68 ◽  
pp. 101837
Author(s):  
Yi Luo ◽  
Dong Zhao ◽  
Tao Zhou ◽  
Jianjun Zhu ◽  
Jianjun Zhang ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Biliary Atresia ◽  
Choledochal Cyst

scholarly journals Paralysis in the left phrenic nerve after living-donor liver transplantation for biliary atresia with situs inversus

2008 ◽  
Vol 14 (11) ◽  
pp. 1659-1663 ◽  
Author(s):  
Yukihiro Sanada ◽  
Koichi Mizuta ◽  
Youichi Kawano ◽  
Satoshi Egami ◽  
Makoto Hayashida ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Biliary Atresia ◽  
Situs Inversus ◽  
Phrenic Nerve ◽  
Living Donor ◽  
Living Donor Liver Transplantation ◽  
Donor Liver ◽  
Donor Liver Transplantation

Prognosis of biliary atresia in the era of liver transplantation: French national study from 1986 to 1996

Hepatology ◽  
1999 ◽  
Vol 30 (3) ◽  
pp. 606-611 ◽  
Author(s):  
Christophe Chardot ◽  
Matthieu Carton ◽  
Nathalie Spire-Bendelac ◽  
Christophe Le Pommelet ◽  
Jean-Louis Golmard ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Biliary Atresia ◽  
National Study

Liver transplantation for biliary atresia: A nationwide investigation from 1996 to 2013 in mainland China

2016 ◽  
Vol 20 (8) ◽  
pp. 1051-1059 ◽  
Author(s):  
Ping Wan ◽  
Dongwei Xu ◽  
Jianjun Zhang ◽  
Qigen Li ◽  
Ming Zhang ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Biliary Atresia ◽  
Mainland China

The Quality of Life of Long Term Survivors of Patients with Biliary Atresia

2021 ◽  
Author(s):  
Melanie Le ◽  
Konrad Reinshagen ◽  
Christian Tomuschat
Keyword(s):  
Quality Of Life ◽  
Liver Transplantation ◽  
Biliary Atresia ◽  
Long Term Results ◽  
Cochrane Library ◽  
Healthy Controls ◽  
Term Care ◽  
Associated Malformations

Abstract Purpose: Advances in surgical techniques and perioperative care have improved patients' short-and mid-term postoperative outcomes with Biliary Atresia (BA). However, the long-term results of these patients have not been thoroughly investigated. This systematic review aims to determine the long-term outcomes and the patients' health-related Quality of life (HrQoL) with their native livers or liver transplantation. Methods: A systematic literature-based search for relevant cohorts was performed using Pubmed/Medline, Cochrane Library from its inception to August 2021. Original studies reporting on BA, Hepatoportoenterostomie, portoenterostomy, Kasai, Liver transplantation, Quality of life, or HrQoL were included. Pooled prevalence has been calculated for cholangitis, secondary liver transplantation, or associated malformations using MetaXL (version 5.3). Subgroup analysis on HrQoL followed surgical treatment after BA was calculated by using RevMan (version 5.4).Results: 12 articles were considered for data synthesis. Nine studies compared biliary atresia patients to an age-matched healthy reference group. 4/9 (n = 338) of these studies indicated lower scores for biliary atresia patients; 5/9 (n = 127) stated similar health status. A Forest plot analysis including all studies with total HrQoL showed a tendency of higher scores towards healthy controls (MD -0.79, 95% CI: -6.00-4.41). Comparing patients after Kasai Hepatoportoenterostomy with healthy controls demonstrated favorable outcomes for the control group (MD -3.22, 95% CI: -7.20-0.75) with no statistical significance (p = 0.11). The pooled estimation of the prevalence of cholangitis, secondary liver transplantation and associated malformations are 0.33 (95% CI: 0.06–0.66), 0.59 (95% CI: 0,42–0.75) and 0.13 (95% CI: 0,01–0.33).Conclusion: Biliary atresia patients have an overall high prevalence of progressive liver-related complications and risk of lower HrQoL compared to their healthy peers. Furthermore, those patients who received liver transplantation appear to have the same Quality of life as those living with their native livers. Targeted and evidence-based follow-up procedures and transitional care are essential to meet these patients' long-term care needs. Prospective and multicenter research das focuses on the attributes and predictors of the long-term prognosis of patients with biliary atresia are necessary.

Successful living related liver transplantation in a case with biliary atresia associated with corrected transposition of the great arteries

2007 ◽  
Vol 11 (5) ◽  
pp. 540-542 ◽  
Author(s):  
Takuya Kimura ◽  
Toshimichi Hasegawa ◽  
Yoshiyuki Ihara ◽  
Sotaro Mushiake ◽  
Shigetoyo Kogaki ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Biliary Atresia ◽  
Living Related Liver Transplantation ◽  
Living Related ◽  
Corrected Transposition

scholarly journals Characteristics of Gut Microbiota in Children With Biliary Atresia After Liver Transplantation

2021 ◽  
Vol 12 ◽  
Author(s):  
Wei Song ◽  
Li-Ying Sun ◽  
Zhi-Jun Zhu ◽  
Lin Wei ◽  
Wei Qu ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Gut Microbiota ◽  
Biliary Atresia ◽  
Control Level ◽  
Control Group ◽  
Metagenomic Sequencing ◽  
Polyamine Biosynthesis ◽  
Fecal Samples ◽  
Linear Discriminant ◽  
Significant Difference

Background and AimsBiliary atresia (BA) is an idiopathic neonatal cholestasis and is the most common indication in pediatric liver transplantation (LT). Previous studies have suggested that the gut microbiota (GM) in BA is disordered. However, the effect of LT on gut dysbiosis in patients with BA has not yet been elucidated.MethodsPatients with BA (n = 16) and healthy controls (n = 10) were recruited. In the early life of children with BA, Kasai surgery is a typical procedure for restoring bile flow. According to whether BA patients had previously undergone Kasai surgery, we divided the post-LT patients into the with-Kasai group (n = 8) and non-Kasai group (n = 8). Fecal samples were collected in both the BA and the control group; among BA patients, samples were obtained again 6 months after LT. A total of 40 fecal samples were collected, of which 16 were pre-LT, 14 were post-LT (8 were with-Kasai, 6 were non-Kasai), and 10 were from the control group. Metagenomic sequencing was performed to evaluate the GM.ResultsThe Kruskal-Wallis test showed a statistically significant difference in the number of genes between the pre-LT and the control group, the pre-LT and the post-LT group (P < 0.05), but no statistical difference between the post-LT and the control group. Principal coordinate analysis also showed that the microbiome structure was similar between the post-LT and control group (P > 0.05). Analysis of the GM composition showed a significant decrease in Serratia, Enterobacter, Morganella, Skunalikevirus, and Phifllikevirus while short chain fatty acid (SCFA)-producing bacteria such as Roseburia, Blautia, Clostridium, Akkermansia, and Ruminococcus were increased after LT (linear discriminant analysis > 2, P < 0.05). However, they still did not reach the normal control level. Concerning functional profiles, lipopolysaccharide metabolism, multidrug resistance, polyamine biosynthesis, GABA biosynthesis, and EHEC/EPEC pathogenicity signature were more enriched in the post-LT group compared with the control group. Prior Kasai surgery had a specific influence on the postoperative GM.ConclusionLT partly improved the GM in patients with BA, which provided new insight into understanding the role of LT in BA.

scholarly journals Neuropsychological development of children with biliary atresia after liver transplantation

2021 ◽  
Vol 23 (3) ◽  
pp. 66-72
Author(s):  
A. V. Syrkina ◽  
I. E. Pashkova ◽  
A. R. Monakhov ◽  
O. V. Silina ◽  
E. V. Chekletsova ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Surgical Treatment ◽  
Biliary Atresia ◽  
Psychomotor Development ◽  
Two Stage ◽  
Neuropsychological Development ◽  
One Stage ◽  
The Difference ◽  
Group 2 ◽  
Group 1

Background. In young children, the most common liver disease leading to transplantation is biliary atresia. Liver transplantation has fundamentally improved the survival rate of children with biliary atresia. Studies on developmental outcomes in children are mostly limited to small samples; there are no such studies in the Russian Federation.Objective: to determine the cognitive outcomes in children undergoing one-stage or two-stage surgical treatment of biliary atresia.Materials and Methods. 83 children were divided into groups: 36 children underwent transplantation without previous surgical interventions (group 1), 47 children underwent the Kasai palliative portoenterostomy (group 2). Inclusion criteria: 24 months of age or younger at the moment of transplantation, no medical history of neurological pathology. All children were examined before transplantation and at 1, 3, 6 and 12 months after liver transplantation. Psychomotor development was assessed using the Griffiths Psychomotor Development Scale for children under 24 months (translated by E.S. Keshishian), the Griffiths Intellectual Development Scale for children aged 2 to 8 years, and the Modified Checklist for Autism in Toddlers, Revised, for children 16-30 months old.Results. All children had developmental delays at the time of transplantation. Up to 50% of the children had signs of cachexia, with a shoulder circumference of less than 3 percentile. Only two children showed obvious hepatic encephalopathy in the form of depressed consciousness. After liver transplantation, 94% of group 1 children recovered their preoperative psychomotor development levels, and only 68% in group 2 made these gains. At 3 and 6 months after transplantation, about 80% of group 1 children showed normal psychomotor development, whereas in group 2, only 61% did. By 12 months after liver transplantation, the difference between the groups was more evident: 83.3% of group 1 children and only 53.2% of group 2 children were developing according to age. The difference between the groups was statistically significant (p < 0.05).Conclusion. Children who received one-stage treatment of biliary atresia and underwent liver transplantation have better neuropsychological development within a year after surgery than children with two-stage surgical treatment.

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