Scholarlink

Purpose <p>This study aimed to clarify the incidence of portal hypertension (PH) in biliary atresia (BA), its impact on long-term native liver survival rates (NLSRs), and the role of PH treatment.</p> Methods <p>Data were analyzed from 3,777 patients in the Japanese Biliary Atresia Registry (1989–2021). Incidence and treatment of PH were examined in all patients and in 20-year native liver survivors. A subgroup of 596 jaundice-free native liver survivors was divided into three groups: without PH (A), untreated PH (B), and treated PH (C). Patient characteristics, clinical outcomes, and survival analyses were performed.</p> Results <p>PH occurred in 46.4% of all patients, with gastroesophageal varices (35.1%) and hypersplenism (36.8%) being most common. Among 759 20-year native liver survivors, 49.8% had PH, largely diagnosed between 2 and 15 years. Group C patients were older at Kasai portoenterostomy and had more cholangitis. Survival analysis showed best outcomes in group A and worst in group C. Conditional native liver survival analysis revealed group B initially had better NLSRs than group C, but differences diminished beyond 15 years.</p> Conclusion <p>In native liver survivors without jaundice following KP, PH influenced prognosis, but successful treatment enabled long-term survival comparable to patients with mild or no PH.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Portal hypertension in biliary atresia: a Japanese biliary atresia registry study

  • 期刊论文

  • 出版模式：

    开放获取

  • 发表日期：

    2025年10月31日

查看全文
  • Ryuji Okubo,
  • Masaki Nio,
  • Hideyuki Sasaki,
  • Taichi Fukuzawa,
  • Hironori Kudo,
  • Ryo Ando,
  • Tsuyoshi Sakurai,
  • Masatoshi Hashimoto,
  • Keisuke Tada,
  • Motoshi Wada

摘要

Purpose

This study aimed to clarify the incidence of portal hypertension (PH) in biliary atresia (BA), its impact on long-term native liver survival rates (NLSRs), and the role of PH treatment.

Methods

Data were analyzed from 3,777 patients in the Japanese Biliary Atresia Registry (1989–2021). Incidence and treatment of PH were examined in all patients and in 20-year native liver survivors. A subgroup of 596 jaundice-free native liver survivors was divided into three groups: without PH (A), untreated PH (B), and treated PH (C). Patient characteristics, clinical outcomes, and survival analyses were performed.

Results

PH occurred in 46.4% of all patients, with gastroesophageal varices (35.1%) and hypersplenism (36.8%) being most common. Among 759 20-year native liver survivors, 49.8% had PH, largely diagnosed between 2 and 15 years. Group C patients were older at Kasai portoenterostomy and had more cholangitis. Survival analysis showed best outcomes in group A and worst in group C. Conditional native liver survival analysis revealed group B initially had better NLSRs than group C, but differences diminished beyond 15 years.

Conclusion

In native liver survivors without jaundice following KP, PH influenced prognosis, but successful treatment enabled long-term survival comparable to patients with mild or no PH.