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平田 義弘
Department School of Medicine(Tokyo Women's Medical University Hospital), School of Medicine Position |
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| Article types | Original article |
| Language | English |
| Peer review | Peer reviewed |
| Title | Outcomes of 50 Cases of Pediatric Liver Transplantation for Intrapulmonary Shunt While Considering the Deteriorated Prognosis of Severe Intrapulmonary Shunt Patients. |
| Journal | Formal name：Transplantation proceedings Abbreviation：Transplant Proc ISSN code：18732623/00411345 |
| Domestic / Foregin | Foregin |
| Volume, Issue, Page | 51(8),pp.2793-2797 |
| Author and coauthor | Hirata Yoshihiro, Sakamoto Seisuke, Yoshimura Shohei, Takeda Masahiro, Uchida Hajime, Shimizu Seiichi, Kitamura Masayuki, Irie Rie, Miyazaki Osamu, Yoshioka Takako, Nosaka Syunsuke, Kasahara Mureo |
| Publication date | 2019/10 |
| Summary | 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 |