ヒラタ ヨシヒロ   Hirata Yoshihiro
  平田 義弘
   所属   医学部 医学科(東京女子医科大学病院)
   職種   非常勤講師
論文種別 原著
言語種別 英語
査読の有無 査読あり
表題 Outcomes of 50 Cases of Pediatric Liver Transplantation for Intrapulmonary Shunt While Considering the Deteriorated Prognosis of Severe Intrapulmonary Shunt Patients.
掲載誌名 正式名:Transplantation proceedings
略  称:Transplant Proc
ISSNコード:18732623/00411345
掲載区分国外
巻・号・頁 51(8),pp.2793-2797
著者・共著者 Hirata Yoshihiro, Sakamoto Seisuke, Yoshimura Shohei, Takeda Masahiro, Uchida Hajime, Shimizu Seiichi, Kitamura Masayuki, Irie Rie, Miyazaki Osamu, Yoshioka Takako, Nosaka Syunsuke, Kasahara Mureo
発行年月 2019/10
概要 BACKGROUND:Intrapulmonary shunt (IPS) is recognized in 10% of chronic liver disease patients. Liver transplantation (LT) is associated with a high risk of morbidity and mortality in patients with IPS.PATIENTS AND METHODS:Of 519 pediatric LT cases between November 2005 and October 2018, 50 patients with IPS were enrolled in this study. The patients were divided into 3 groups, according to the shunt ratio, calculated by scintigraphy: mild (15%-20%, n = 26), moderate (20%-40%, n = 19), and severe (> 40%, n = 5). We compared the patients' characteristics before LT and the outcomes of LT between these groups.RESULTS:The major original disease resulting in LT in the mild and moderate groups was biliary atresia (73.1% and 52.6%, respectively), while that in the severe group was congenital portosystemic shunt (60%). The median ages at LT were 7.5, 6.1, and 8.3 years in the mild, moderate, and severe groups, respectively. All of the mild and moderate IPS patients lived; however, 3 patients with severe IPS (60.0%) died within 3 months. The shunt ratios of the mild and moderate IPS patients normalized within 2 years after LT, while the 2 surviving severe IPS patients showed a slight improvement. The autopsy findings of the lung in 1 deceased severe IPS patient showed medial hypertrophy and proliferation of intimal cells of the pulmonary arteries, suggesting a diagnosis of portopulmonary hypertension.CONCLUSIONS:LT can be safely performed for mild and moderate IPS patients; however, LT for severe IPS patients should be carefully indicated because concomitant portopulmonary hypertension may be masked by IPS.
DOI 10.1016/j.transproceed.2019.03.079
PMID 31563247