Long-term outcome of children with biliary atresia who were not transplanted after the kasai operation: >20-year experience at a children's hospital

Masato Shinkai, Youkatsu Ohhama, Hiroshi Take, Norihiko Kitagawa, Hironori Kudo, Kyoko Mochizuki, Tomoko Hatata

研究成果: Article査読

126 被引用数 (Scopus)

抄録

OBJECTIVE: Sequential strategies combining the Kasai operation as a first-line treatment and liver transplantation as a second-line option, if necessary, have been accepted for patients with biliary atresia (BA). To understand the role of the Kasai operation in the treatment of BA, it is necessary to analyze the long-term outcome of the operation alone and to evaluate the present status of survivors retaining their native livers. MATERIALS AND METHODS: A retrospective chart review was carried out for a group of 80 patients who had undergone the Kasai operation between 1970 and 1986 at the Kanagawa Children's Medical Center. RESULTS: The 5-, 10-, and 20-year survival rates of patients with their native livers were 63%, 54%, and 44%, respectively. The survival rates varied significantly depending on the type of BA, age at initial Kasai operation, era of surgery, and surgical method. By age 20, nearly half of the adult survivors had already developed liver cirrhosis and its sequelae. Episodes of cholangitis and gastrointestinal bleeding occurred after 20 years of age in 37% and 17% of the adult patients, respectively, and 20% of the adult patients died of liver failure or underwent living-related partial liver transplantation in their 20s. Five female patients gave birth to a total of 9 children, and 1 male patient fathered a child. CONCLUSIONS: Although increasing numbers of patients with BA survive 20 years or more after the Kasai operation, meticulous lifelong postoperative care should be continued for the survivors because of the possibility of hepatic deterioration.

本文言語English
ページ(範囲)443-450
ページ数8
ジャーナルJournal of Pediatric Gastroenterology and Nutrition
48
4
DOI
出版ステータスPublished - 2009 4月
外部発表はい

ASJC Scopus subject areas

  • 小児科学、周産期医学および子どもの健康
  • 消化器病学

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