Portal venous velocity in the follow-up of patients with biliary atresia after Kasai portoenterostomy

2002 ◽  
Vol 37 (6) ◽  
pp. 873-876 ◽  
Author(s):  
P.L. Khong ◽  
C.G.C. Ooi ◽  
H. Saing ◽  
K.L. Chan ◽  
W.H.S. Wong ◽  
...  
Keyword(s):  
Biliary Atresia ◽  
Kasai Portoenterostomy

Laparoscopic Kasai portoenterostomy for cystic biliary atresia: midterm follow-up results of 35 patients

Surgery Today ◽  
2021 ◽  
Author(s):  
Yi Ji ◽  
Jianyuan Zhou ◽  
Xuepeng Zhang ◽  
Siyuan Chen ◽  
Zhicheng Xu
Keyword(s):  
Biliary Atresia ◽  
Kasai Portoenterostomy ◽  
Cystic Biliary Atresia

scholarly journals Long-Term Effects of Kasai Portoenterostomy for Biliary Atresia Treatment in Russia

Diagnostics ◽  
2020 ◽  
Vol 10 (9) ◽  
pp. 686
Author(s):  
Anna Degtyareva ◽  
Alexander Razumovskiy ◽  
Nadezhda Kulikova ◽  
Sergey Ratnikov ◽  
Elena Filippova ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Survival Rate ◽  
Biliary Atresia ◽  
Kasai Portoenterostomy ◽  
Critical Importance ◽  
Long Term Effects ◽  
Age Related

This prospective study enrolled 144 patients after surgical treatment of biliary atresia in early infancy. We analyzed the immediate effectiveness of the surgery and the age-related structure of complications in the up to 16-year follow-up. The immediate 2-year survival rate after the surgery constituted 49.5%. At the time of this writing, 17 of the patients had celebrated their 10th birthdays with good quality of life and no indications for transplantation of the liver. The obtained results underscore the critical importance of surgical correction of biliary atresia by Kasai surgery in the first 60 days of life and subsequent dynamic follow-up of patients for the purpose of the early detection and timely correction of possible complications.

Variceal Bleeds in Patients with Biliary Atresia

2017 ◽  
Vol 28 (05) ◽  
pp. 439-444 ◽  
Author(s):  
Mauri Witt ◽  
Niels Bax ◽  
Henkjan Verkade ◽  
René Scheenstra ◽  
Ruben de Kleine ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Biliary Atresia ◽  
Variceal Bleeding ◽  
Laboratory Tests ◽  
Subsequent Development ◽  
Kasai Portoenterostomy ◽  
Receive Treatment ◽  
Related Mortality

Introduction Portal hypertension often occurs in biliary atresia (BA). The subsequent development of esophageal varices and bleeding from these varices are a well-known complication. We aim to describe the incidence and severity of variceal bleeding in patients with BA. In addition, we describe the characteristics of patients who experienced variceal bleeds. Materials and Methods We included all infants treated for BA at our center between March 1987 and August 2015. Variceal bleeding was defined as hematemesis and/or melena with presence of varices at endoscopy. Findings at endoscopy and ultrasound, laboratory tests, clearance of jaundice, fibrosis-grade at Kasai portoenterostomy, and several varices prediction scores were documented. Routine endoscopies were not performed. Results In this study, 74 patients were included. During follow-up, 18 out of 74 patients (24%) developed variceal bleeding at an age of 9 months (range, 4–111). Twelve patients were listed for liver transplantation at the time of bleeding. Patients who did not clear their jaundice developed variceal bleeds more often and earlier in life. Bleeds were treated with sclerotherapy, banding, or octreotide. Four patients did not receive treatment. No bleeding-related mortality occurred. Conclusion One-fourth of the children diagnosed with BA experience variceal bleeds during follow-up. Most of these children are younger than 1 year and often already listed for transplantation. Major complications did not occur after variceal bleeding.

scholarly journals Genetic aspects of biliary atresia etiology

2020 ◽  
Author(s):  
MKh Isaeva ◽  
VA Belova ◽  
DO Korostin ◽  
AV Degtyareva
Keyword(s):  
Biliary Atresia ◽  
Genetic Predisposition ◽  
Stress Responses ◽  
Somatic Mutations ◽  
Kasai Portoenterostomy ◽  
Number Of Factors ◽  
Long Term Follow Up ◽  
Epigenetic Patterns

Biliary atresia (BA) is a cholestatic disorder of infancy that is fatal if untreated. Despite years of study the etiology of BA remains unknown. Three etiopathogenic mechanisms may be involved, such as immune dysregulation, environmental factors and genetic susceptibility. Genetic predisposition is being actively studied. Candidate genes associated with BA in certain populations, genes affecting the cholangiocyte cilia function, as well as genes involved in stress responses have been identified. However, the long-term follow-up of twins with BA suggests that genotype is not of paramount importance for the disease development. Both epigenetic patterns and postzygotic somatic mutations may contribute to etiology of the disease. Recently, some evidence is being accumulated on the possible genetic predisposition to certain outcome of Kasai portoenterostomy performed in patients with BA. However, the presence of a number of factors contributing to the development of the disease makes it difficult to identify the genetic markers.

Stretching the Limit of Native Liver Survival in Biliary Atresia After Kasai Portoenterostomy – a 37-year Territory-wide Study

2021 ◽  
Author(s):  
Patrick Ho Yu Chung ◽  
Edwin Kin Wai Chan ◽  
Fanny Yeung ◽  
Albert Chi Yan Chan ◽  
Jennifer Wai Cheung Mou ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Liver Transplant ◽  
Biliary Atresia ◽  
Kasai Portoenterostomy ◽  
Term Survival ◽  
Native Liver ◽  
The Mean ◽  
Native Liver Survival

Abstract ObjectiveWe present a 37 years’ experience in the management of biliary atresia (BA) and discuss long-term complications after Kasai portoenterostomy (KPE).MethodsA retrospective territory-wide study from 1980 to 2017 on 231 patients with open KPE from three tertiary paediatric surgical centres was performed. The jaundice clearance (JC) rate, native liver survival (NLS) rate and complications were analyzed.ResultsThe mean follow up period was 15.4 +/- 6.2 years. Over 60% of patients remained JC within 2 years after KPE. Seventy patients (30.3%) received liver transplant (LT) at a median age of 6.2 years (range: 0.5 to 25 years). The NLS rates at 10, 20 and 30 years were 70.7%, 61.5% and 53.0% respectively with no significant change over the study period. The median bilirubin level among all native liver survivors (n=153) was 24 µmol/L (2 to 162 µmol/L). Portal hypertension (PHT) and recurrent cholangitis were found in 51.6% and 27.5% of them respectively.ConclusionWith a vigilant follow up program, more than 60% of BA patients could remain stable with the disease and achieve long term survival without LT. Although portal hypertension and recurrent cholangitis are common, they do not need to be the indications for LT if managed properly.

CO4 THREE YEAR FOLLOW-UP AFTER KASAI PORTOENTEROSTOMY FOR BILIARY ATRESIA: OUTCOMES AND ROLE OF HIGH DOSE STEROID THERAPY

2009 ◽  
Vol 41 ◽  
pp. S200
Author(s):  
M. Colusso ◽  
E. Zaranko ◽  
C. Terzi ◽  
M. Cheli ◽  
P. Stroppa ◽  
...  
Keyword(s):  
Biliary Atresia ◽  
Steroid Therapy ◽  
High Dose ◽  
Kasai Portoenterostomy ◽  
High Dose Steroid

scholarly journals Long-Term Effects of Kasai Portoenterostomy for Biliary Atresia

2020 ◽  
Author(s):  
Anna Degtyareva ◽  
Alexander Razumovskiy ◽  
Nadezhda Kulikova ◽  
Sergey Ratnikov ◽  
Elena Filippova ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Survival Rate ◽  
Biliary Atresia ◽  
Kasai Portoenterostomy ◽  
Critical Importance ◽  
Long Term Effects ◽  
Age Related

The prospective study enrolled 144 patients after surgical treatment of biliary atresia in early infancy. We analyze the immediate effectiveness of the surgery and the age-related structure of complications in the up to 16-year follow-up. The immediate 2-year survival rate after the surgery constituted 49.5%. At the time of this writing, 17 of the patients have celebrated their 10th birthdays with good quality of life and no indications for LT. The obtained results underscore the critical importance of surgical correction of BA by Kasai surgery during the first 60 days of life and subsequent dynamic follow-up of the patients for the purpose of the early detection and timely correction of possible complications.

Centralization of Biliary Atresia: Has Germany Learned Its Lessons?

2021 ◽  
Author(s):  
Omid Madadi-Sanjani ◽  
David Fortmann ◽  
Udo Rolle ◽  
Burkhard Rodeck ◽  
Ekkehard Sturm ◽  
...  
Keyword(s):  
Biliary Atresia ◽  
Data Transfer ◽  
Previous Analysis ◽  
Outcome Data ◽  
Free Survival ◽  
Change In Practice ◽  
Native Liver ◽  
Survival With Native Liver ◽  
Pediatric Liver Transplant

Abstract Introduction The majority of pediatric surgeons and hepatologists recommend the centralization of biliary atresia (BA) treatment within experienced liver units. We aimed to investigate whether voluntary self-restriction and acceptance of the need for this change in practice changed the BA referral policy in Germany during the last decade. Materials and Methods In cooperation with pediatric surgeons, gastroenterologists or hepatologists, and pediatric liver transplant units, the 2-year follow-up data of infants with BA born in Germany between 2010 and 2014 were collected using www.bard-online.com or pseudonymized data transfer. Results were compared with our previous analysis of the outcome data of infants with BA born between 2001 and 2005 in Germany. Result Overall, 173 infants with BA were identified, of whom 160 underwent Kasai portoenterostomy (KPE; 92.5%) and 13 (7.5%) underwent primary liver transplantation at 21 German centers. At 2-year follow-up, overall survival was 87.7% (vs. 81.9% in 2001–2005 [p = 0.19]), survival with native liver post-KPE was 29.2% (vs. 22.8% in 2001–2005 [p = 0.24]), and jaundice-free survival with native liver post-KPE was 24.0% (vs. 20.1% in 2001–2005 [p = 0.5]). Compared with the 2001–2005 analysis, all criteria showed improvement but the differences are statistically not significant. Conclusion Our observation shows that KPE management requires improvement in Germany. Centralization of BA patients to German reference liver units is not yet mandatory. However, European and national efforts with regard to the centralization of rare diseases support our common endeavor in this direction.

scholarly journals Correctable biliary atresia and cholangiocarcinoma: a case report of a 63-year-old patient

2019 ◽  
Vol 5 (1) ◽  
Author(s):  
Masaki Nio ◽  
Motoshi Wada ◽  
Hideyuki Sasaki ◽  
Hiromu Tanaka ◽  
Masatoshi Hashimoto ◽  
...  
Keyword(s):  
Biliary Atresia ◽  
Liver Tumors ◽  
Malignant Tumors ◽  
Porta Hepatis ◽  
Case Presentation ◽  
Cancer Occurrence ◽  
Long Term Survivors ◽  
Near Future

Abstract Background Although cancer occurrence following surgery for biliary atresia has gradually increased, the development of cholangiocarcinoma in a native liver survivor of biliary atresia is extremely rare. Case presentation A 3-month-old female patient with the correctable type of biliary atresia underwent a cystoduodenostomy. At 16 years of age, she underwent multiple surgeries including lysis of intestinal adhesions, ileostomy, and gastrojejunostomy at another hospital. At 54 years of age, she underwent lithotomy at the porta hepatis, resection of the residual cystic bile duct with gallbladder, and hepaticojejunostomy in Roux-en-Y fashion. As she approached the age of 63, her computed tomography scan showed no liver tumors. In the following year, she developed cholangiocarcinoma at the porta hepatis and underwent chemotherapy. However, the cancer progressed, and she died before she reached the age of 64 years. Conclusions Cholangiocarcinoma is extremely rare in patients with biliary atresia. However, physicians should follow up patients with biliary atresia as closely as possible, as malignant tumors secondary to biliary atresia may increase in number in the near future because of the growing number of long-term survivors with biliary atresia.

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