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ヒラタ ヨシヒロ
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 |
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| 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 |