Translational Research in Biliary Atresia: News from Mice and Men

2019 ◽  
Vol 29 (04) ◽  
pp. 336-341
Author(s):  
Omid Madadi-Sanjani ◽  
Claus Petersen

AbstractBiliary atresia (BA) is a fibro-obliterative cholangiopathy of unknown etiology. While Kasai portoenterostomy achieves temporary biliary drainage in some cases, BA remains the most common indication for liver transplantation during childhood. During the last few decades, observations on BA, like cholestatic diseases in animals and the introduction of different animal models for BA, have not achieved the anticipated results, and we are still not able to translate the basic research to the patient's bedside. This article presents a review of the literature on available BA animal models and gives a glimpse of future developments.

2020 ◽  
Vol I (3) ◽  
pp. 43-49
Author(s):  
Julio César Moreno Alfonso

Biliary Atresia (BA) is an uncommon, progressive and idiopathic fibro-obliterative cholangiopathy. The typical clinical features should be identified prompt in order to realize a Kasai Hepatoportoenterostomy (HPE) for restore the bile flow. However, despite HPE the BA remains the most common indication for liver transplantation in children. The lasts studies have shown the importance of virus, immunity and other environmental substances in pathogenesis of BA that is important in order to look for new therapeutic and preventive strategies. This article is a systematic review of the literature about actual evidence in BA. Keywords: Biliary atresia; Neonatal jaundice; Kasai hepatoportoenterostomy; Liver transplantation


2016 ◽  
Vol 45 (2) ◽  
pp. 116
Author(s):  
Naresh P Shanmugam ◽  
Gomathy Narasimhan ◽  
Shaman Rajindrajith

2017 ◽  
Vol 28 (05) ◽  
pp. 439-444 ◽  
Author(s):  
Mauri Witt ◽  
Niels Bax ◽  
Henkjan Verkade ◽  
René Scheenstra ◽  
Ruben de Kleine ◽  
...  

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.


2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S354-S355
Author(s):  
Thomas M Tarro ◽  
Laurie Song ◽  
Beth A Carter ◽  
Meiyu Yeh ◽  
Pia S Pannaraj ◽  
...  

Abstract Background Biliary atresia (BA) is a rare, progressive, idiopathic, fibro-obliterative disease of the extrahepatic biliary tree seen in children. The current standard treatment is surgical management with Kasai portoenterostomy (KP). Bacterial cholangitis is a frequent complication following KP and an important determinant of long-term prognosis. Use of prophylactic antibiotics is common but not universal and efficacy is controversial. Methods A retrospective study was performed that included all patients with BA who underwent KP from November 2002 to July 2019. Chart review was conducted to collect demographic information and evaluate the use of antibiotic prophylaxis, number of cholangitis episodes, time to liver transplantation (LVT), and survival. Results Ninety-one patients with BA underwent KP during the study period. Seventy-two (79%) received prophylactic antibiotics, and 19 (21%) did not. The median duration of prophylactic antibiotics was 7 months (interquartile range [IQR] 8.5). Patients in the no-prophylaxis group had significantly fewer cholangitis episodes (median 0, IQR 1) than in the antibiotic prophylaxis group (median 1, IQR 2), p= 0.0201. The median time to LVT was 8 months (IQR 8.5) in the antibiotic prophylaxis group, compared to 7 months (IQR 6) in the no-prophylaxis group, p=0.8827. Of the patients who were on antibiotic prophylaxis, 57 (79.2%) received trimethoprim-sulfamethoxazole (TMP-SMX) alone and 15 (20.8%) received multiple/other antibiotics. Seven patients (7.7%) had culture-positive cholangitis. Six of 7 received prophylaxis with TMP-SMX and 5 of 7 grew bacteria that were resistant to TMP-SMX. No deaths occurred between the postoperative KP period to the time of LVT in both groups. Table 1. Characteristics of Patients in the Prophylaxis and No-prophylaxis Groups. Table 2. Impact of Prophylactic Antibiotics in the Period Between Kasai Portoenterostomy and Liver Transplantation. Table 3. Patients with Culture-positive Cholangitis after Kasai Portoenterostomy (n=7). Conclusion Antibiotic prophylaxis was frequently used after KP with TMP-SMX being the most common antibiotic used. Patients in the no-prophylaxis group had significantly fewer cholangitis episodes compared to those receiving antibiotic prophylaxis. Prophylactic antibiotics did not have an impact on time to LVT. Our findings suggest that antibiotic prophylaxis is not helpful in decreasing the frequency of cholangitis episodes after KP and may increase the risk for infections with resistant bacteria. Larger prospective randomized control studies are recommended. Disclosures Pia S. Pannaraj, MD, MPH, AstraZeneca (Grant/Research Support)Pfizer (Grant/Research Support)Sanofi Pasteur (Advisor or Review Panel member)


ISRN Surgery ◽  
2012 ◽  
Vol 2012 ◽  
pp. 1-15 ◽  
Author(s):  
Barbara E. Wildhaber

Biliary atresia is a rare neonatal disease of unknown etiology, where obstruction of the biliary tree causes severe cholestasis, leading to biliary cirrhosis and death in the first years of life, if the condition is left untreated. Biliary atresia is the most frequent surgical cause of cholestatic jaundice in neonates and should be evoked whenever this clinical sign is associated with pale stools and hepatomegaly. The treatment of biliary atresia is surgical and currently recommended as a sequence of, eventually, two interventions. During the first months of life a hepatoportoenterostomy (a “Kasai,” modifications of which are discussed in this paper) should be performed, in order to restore the biliary flow to the intestine and lessen further damage to the liver. If this fails and/or the disease progresses towards biliary cirrhosis and life-threatening complications, then liver transplantation is indicated, for which biliary atresia represents the most frequent pediatric indication. Of importance, the earlier the Kasai is performed, the later a liver transplantation is usually needed. This warrants a great degree of awareness of biliary atresia, and the implementation of systematic screening for this life-threatening pathology.


2015 ◽  
Vol 21 (7) ◽  
pp. 922-927 ◽  
Author(s):  
Joao Seda Neto ◽  
Flávia H. Feier ◽  
Ana Luiza Bierrenbach ◽  
Cristiana M. Toscano ◽  
Eduardo A. Fonseca ◽  
...  

2012 ◽  
Vol 22 (4) ◽  
pp. 408-411 ◽  
Author(s):  
Christina Oetzmann von Sochaczewski ◽  
Claus Petersen ◽  
Benno M. Ure ◽  
Alexander Osthaus ◽  
Kai-Peter Schubert ◽  
...  

2003 ◽  
Vol 162 (9) ◽  
pp. 603-606 ◽  
Author(s):  
Chuen-Bin Jiang ◽  
Hung-Chang Lee ◽  
Chun-Yan Yeung ◽  
Jin-Cherng Sheu ◽  
Pei-Yeh Chang ◽  
...  

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Yi Ji ◽  
Xuepeng Zhang ◽  
Siyuan Chen ◽  
Yanan Li ◽  
Kaiying Yang ◽  
...  

Abstract Objective To determine whether revision laparoscopic Kasai portoenterostomy (RLKPE) is a viable treatment option for patients with biliary atresia (BA) who had undergone initially successful laparoscopic Kasai portoenterostomy (ILKPE). Methods The medical records of 312 patients with nonsyndromic BA who had undergone ILKPE between May 2009 and May 2017 were retrospectively reviewed. The patients were divided into three groups according to their outcomes after ILKPE: group A: 25 patients who had undergone RLKPE; group B: 203 patients who had undergone ILKPE and required no further surgical intervention; group C: 84 patients with failed ILKPE who had either died or required liver transplantation for survival. The 3-year and 5-year survival with native liver (SNL) rates were compared between groups A and B and between groups A and C. Among the 25 patients in group A, the perioperative data of RLKPE were compared with those of ILKPE. Results Of the 312 patients who had undergone ILKPE, 228 reached the normal bilirubin concentration range within 6 months postoperatively. Among them, 25 patients with a sudden cessation of bile flow had undergone RLKPE. Adequate biliary drainage, as evidenced by normalized conjugated bilirubin levels, was achieved in 80% of patients who had undergone RLKPE. The perioperative variables, including the operative time, blood loss, rate of conversion to open surgery and complications of RLKPE, were not significantly different between RLKPE and ILKPE. The 3-year and 5-year SNL rates in patients after RLKPE were 64.0% and 52.0%, respectively, which were not significantly different from the corresponding rates of 86.2% and 73.9%, respectively, in patients after unrevised ILKPE (P > 0.05). Conclusion Our data demonstrated that RPLKE is a viable and effective treatment option in patients with sudden cessation of bile drainage after ILKPE. RPLKE can delay the need for liver transplantation, yielding encouraging medium-term patient outcomes.


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