Masaki Nio - Introduction to Biliary Atresia

Until Kasai portoenterostomy was developed by Professor Morio Kasai in 1957, biliary atresia (BA) was believed to be an incurable and fatal disease. Kasai portoenterostomy made surgical treatment possible for patients with BA; however, approximately one-third of patients in Japan (and more in many other parts of the world) still require liver transplantation before 1 year of age. BA is a rare disease, and its treatment is so complicated that the centralization of cases has progressed only to a limited number of highly specialized pediatric institutions in select European countries. While the surgical outcome has gradually improved, it is still not optimal. In Japan, the centralization of specialties is not considered important; thus, Kasai portoenterostomy has been performed in more than 100 facilities, most of which deal with one or two cases per year. Why, then, are the overall surgical results in Japan equivalent to or even greater than those in Western countries? This question is a major concern for medical professionals worldwide involved in the treatment of BA for which this book will hopefully provide some insights.

In 1975, an academic organization (the Japanese Society of Biliary Atresia, JBAS) was founded at the behest of Dr. Kasai and continues to study and discuss BA. Around 100 pediatric facilities in Japan, where Kasai portoenterostomy is predominantly performed, participate in the JBAS and share their experiences with the disease. In addition, in 1989, the National Registration System of BA [Japanese Biliary Atresia Registry (JBAR)] was launched under the leadership of Professor Ryoji Ohi. JBAR data on initial, liver transplantation, and follow-up registries are presented at the annual JBAS meeting. In other words, a sound understanding and standard BA treatment have been shared nationwide for over three decades. These efforts have led to improved outcomes for patients with BA. To date, data on over 3500 patients with BA have been compiled, an extraordinary number of cases given the rarity of this disease.

In this book, pediatric experts in fields such as hepatology, surgery, pathology, and liver transplantation familiar with BA describe our latest knowledge regarding standard medical procedures, surgical techniques, perioperative care, long-term follow-up, transitions, and social support systems for BA. I believe that this book will be extremely useful for medical professionals involved in BA worldwide, especially pediatric colleagues.