Biliary atresia – where are the problems?

Late referral
Biliary atresia (BA) is usually diagnosed too late. Although the best time to operate is six weeks after birth, only 20% of the affected infants fully recover. In the majority of the children, the bile flow remains insufficient and the liver fibrose worsens. A liver transplant then becomes – sooner or later – the only option for these children to survive. Because of this, BA is the most common reason for liver transplants in children.

Insufficient cooperation
Biliary atresia is a rare disease affecting approximately 700 infants in Europe every year (incidence approx. 1:12,000 births). All disciplines concerned with the specialised aspects of BA do not cooperate enough with each other. This also applies to basic research.

Unknown etiology
Although BA has been known since the middle of the last century, the etiology still remains unclear. One theory states that BA is a congenital defect, where the extrahepatic biliary ducts are not properly developed, and the second theory is based on the assumption that an inflammation causes the biliary ducts to shrink; this is then known as an acquired deficiency.

Biliary atresia – what to do?

European biliary atresia registry (EBAR)
National registries have shown that therapy results of BA greatly improve when this rare disease is diagnosed early and treated in specialized centers. However, the incidence of BA in each country is too small to drawn any major conclusions. Because of this, representatives of all three disciplines involved in the treatment of biliary atresia (pediatric gastroenterology, pediatric surgery and transplant surgery), from at least ten European countries, have already confirmed their participation.

The registry will record the following information:

• The individual course of the disease for each child with BA from diagnosis to, if necessary, liver transplantation. National and international data protection laws will be taken into consideration.
• Histological, infectological and immunological test results of each registered patient.
• The diagnostic and treatment standards for each participating center.
  
  The continual evaluation of the collected data will take place under the following parameters:
• How and when BA will be diagnosed and treated in each European center.
  How the registered cases will be distributed in the various centres.
• Can conclusions be drawn from data collected which are based on regional, time or another basis, where the unknown etilogy plays a part.
  
  The anonymized results, analyzed yearly, will be published promptly in the registry’s homepage
  
  biliary-atresia.com.
  

Scientific access to gathered material would be controlled by an executive committee and be subject to the rules of each national ethics commission. Further scientific research into the etiology of BA (including animal models) would be controlled by an interdisciplinary executive committee.
We realise that involvement in this register will increase your workload, but we are working on ways to keep this to a minimum, e.g. through online documentation of the anonymised data.
The aims of EBAR

Short-term

• All doctors, who undertake pediatric screenings, should be better informed about BA, especially the time factor between diagnosis and referral to a BA specialist.
• The clinical course of each patient can be greatly improved by early diagnosis and correct treatment, thereby lessening the need for liver transplants.
• The three specialist disciplines involved with BA should cooperate closely.
  Medium-term
• All children with BA should be treated at centers specialized in the treatment of this disease.
• The evaluation of the EBAR will help to establish standards for diagnosis and treatment of BA, offering – for the first time – the possibility of establishing criteria for quality controls.
• The necessity for liver tranplants is o continually reduced.
• A continual development of the internet presentation will support the abovementioned processes and bring specialists, amateurs, affected patients and their families closer together.

Long-term

• Basic research into BA, both clinical and scientific, will be coordinated and intensified, so that the etiology of this rare disease can be clarified.

Additional benefits to be derived from EBAR

• When, through the establishment of EBAR, the early diagnosis of BA can be so improved that the quota of curative first operations can be doubled (from currently 20% to 40%), approximately 130 liver transplants in Europe would not be necessary, and the pressure on the donor organ problem in Europe would be decreased.
• The treatment of every EHBA patient, who receives a timely operation, saves further costs, including those for a liver transplant.
  
  If you are interested in joining the register, please complete the attached initial questionnaire and return it to us by mail or fax. Please also indicate if you want to be included in EBAR’s list of participating clinics.
  
  We also need your help in contacting other centers in your country, which are involved in the treatment of children with biliary atresia. Interested centers will then be sent an initial questionnaire to complete. It would be helpful if you could differentiate between the disciplines involved (pediatric gastroenterology, pediatric surgery and transplant surgery).
  

EBAR organizing board

C. Petersen, MD, Dept. of Pediatric Surgery (Head: Prof. B. Ure, MD)
D. Harder, PhD, secretary
B. Rodeck, MD, Marienhospital Osnabrück
M. Melter, MD, Dept of Pediatric Gastroenterology and Nutrition, (Head: Prof. J.H.H. Ehrich, MD)
J. Klempnauer, MD, Professor and Head of the Dept. of Transplantation
Contact for all: Medical School Hannover

EBAR referee board

M. Davenport, MD Consultant Pediatric Surgeon, London (United Kingdom)
D. Kelly, MD, Reader in Pediatric Hepatology, Birmingham (United Kingdom)
J.B. Otte, MD, Dept. of Pediatric Surgery, Brussels (Belgium)
Advisers
P. Ramani, MD, Consultant Pediatric Pathologist, Birmingham, UK
W.F. Balistreri, MD, Director of the Divn. of Pediatric Gastroenterology & Nutrition, Cincinnati, USA

Overseas scientific cooperation
C.E.L. Tan, MD, Senior Consultant Dept. of Pediatric Surgery, Singapore
P.W. Dillon, MD , Ass. Professor of Pediatrics and Surgery, Hershey, PA, USA

Cara Mack ,MD, Dept. of Pediatric Gastroenterology, (Head: Prof. P.F. Whitington, M.D.), Chicago,IL, USA

Minju Li, MD, Dept. of Pediatric Surgery, Hangzhou, P R China