Introduction to Biliary Atresia

Masaki Nio, M.D., Ph.D., ACSFMasaki Nio is Professor and Chief of Pediatric Surgery at Tohoku University Graduate School of Medicine and Director of Pediatric Medical Center of Tohoku University Hospital. In 1981, he completed a M.D. course at Tohoku University School of Medicine. In 1983, he joined the Second Department of Surgery, Tohoku University Hospital, which Professor Morio Kasai presided over from 1963 to 1986. In 1987, he completed a Ph.D. course in Pediatric Surgery at Tohoku University Graduate School of Medicine. From 1991 to 1993, he worked as a research fellow in Children’s Hospital of Los Angeles, USA. He served as the President of Japanese Society of Pediatric Surgeons, a Board Member of Japan Surgical Society, a Board Member of Pacific Association of Pediatric Surgeons, the Chairman of Japanese Biliary Atresia Society, and so on. He has been a secretary general of Japanese Biliary Atresia Society since 2008.

• Biliary Atresia: A Historical Overview.- History of The Japanese Biliary Atresia Society (JABS).- Japanese Biliary Atresia Registry (JBAR).- Pathogenesis: Overview.- Pathogenesis: Genetics/Epigenetics.- Pathogenesis: Viral Infection.- Pathogenesis: Maternal Microchimerism.- Epidemiology: Incidence and Gender Ratio.- Epidemiology: Ethnic Variations/Family Histories/Heredity.- Epidemiology: Gestational Age/Birth Weight /Associated Anomalies.- Classifications.- Mass Screening.- Stool Color Card System.- Prenatal Diagnosis and Signs/Symptoms.- Intracranial Hemorrhage.- Pathology.- Biomarkers.- Differential Diagnosis.- Diagnostic Modalities.- Preoperative Management and Direct Cholangiography.- Operative Procedures: Open Kasai Procedure.- Operative Procedures: Laparoscopic Kasai Procedure.- Operative Procedures: Re-do Kasai Procedure.- Operative Procedures: Cadaveric Liver Transplantation.- Operative Procedures: Living Donor Liver Transplantation.- Medical Treatment: UDCA/Steroid.- MedicalTreatment: Kampo Medicine.- Prevention/Treatment of Postoperative Cholangitis.- Nutritional Support.- Protocols of Follow-up Management.- Prognostic Indicators.- Long-term Complications: Gastro-Esophageal Varices.- Long-term Complications: Hypersplenism.- Long-term Complications: Cholangitis/Gall Stones.- Long-term Complications: Hepatopulmonary Syndrome.- Long-term Complications: Portopulmonary Hypertension.- Long-term Complications: Liver Cirrhosis/Hepatic Encephalopathy.- Long-Term Results, General.- Long-Term Results, Deformity of Intrahepatic Bile Ducts.- Long-Term Results, Social Performance.- Long-Term Results, Liver Transplantation.- Biliary Atresia and Pregnancy/Delivery.- Biliary Atresia and Malignancy.- Future Prospects.- Transition in Biliary Atresia.- Society of Patients and Families.- Clinical Guidelines for Biliary Atresia.