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An autopsy case of an adult woman with Rapid-Onset Obesity with Hypoventilation, Hypothalamic, Autonomic Dysregulation, and Neuroendocrine Tumors (ROHHAD(NET)) syndrome developing nonalcoholic steatohepatitis and hepatocellular carcinoma: A case report
http://hdl.handle.net/10458/0002001065
http://hdl.handle.net/10458/00020010650bca0e93-22fd-4798-8715-e3b0715c6951
| 名前 / ファイル | ライセンス | アクション |
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| Item type | 学術雑誌論文 / Journal Article(1) | |||||||||||||||
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| 公開日 | 2025-03-26 | |||||||||||||||
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| タイトル | An autopsy case of an adult woman with Rapid-Onset Obesity with Hypoventilation, Hypothalamic, Autonomic Dysregulation, and Neuroendocrine Tumors (ROHHAD(NET)) syndrome developing nonalcoholic steatohepatitis and hepatocellular carcinoma: A case report | |||||||||||||||
| 言語 | en | |||||||||||||||
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| 言語 | eng | |||||||||||||||
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| 資源タイプ | journal article | |||||||||||||||
| アクセス権 | ||||||||||||||||
| 著者 |
蓮池, 悟
× 蓮池, 悟× 大園, 芳範× 内田, 圭祐× Ogawa, Souichiro
× 田村, 穗高× 内山, 尚美× 畑田, 紘志× Komaki, Yuri
× 中村, 憲一× 岩切, 久芳× 末田, 光恵× 永田, 賢治× Toyoki Nishimura
× Matsuyama, Misayo
× 澤田, 浩武× Oguri, Toshiyuki
× 佐藤, 勇一郎× 河上, 洋 |
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| 抄録 | ||||||||||||||||
| 内容記述タイプ | Abstract | |||||||||||||||
| 内容記述 | BACKGROUND Nonalcoholic steatohepatitis (NASH) is an important etiology of hepatocellular carcinoma (HCC), and there is no established therapy for this syndrome. Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, autonomic dysregulation, and neural crest tumor (ROHHAD(NET)) is an extremely rare syndrome considered to be life-threatening, with death occurring around 10 years of age. We present the oldest known autopsy case of this syndrome that developed HCC. This case provided important information on not only improving the course of this syndrome, but also understanding the natural history and therapeutic modalities of NASH and HCC. METHODS The patient was diagnosed with ROHHAD(NET) syndrome in childhood, and liver cirrhosis due to NASH was diagnosed at age 17. HCC was detected at age 20, and embolization and irradiation were performed. At age 21, she died from accidental acute pancreatitis and subsequent liver failure and pulmonary hemorrhage. RESULTS Rapid onset of obesity, hypoventilation, and hypothalamic disturbance appeared in childhood and was diagnosed as this syndrome. At age 17, liver cirrhosis due to NASH was diagnosed by liver biopsy, and at age 20, HCC was diagnosed by imaging. Transarterial chemoembolization and irradiation were performed, and the HCC was well controlled for a year. CONCLUSION At age 21, she died from accidental acute pancreatitis, subsequent liver failure and pulmonary hemorrhage. Autopsy revealed that the HCC was mostly necrotized. This case was valuable not only for other ROHHAD(NET) syndrome cases, but also in improving our understanding of the natural history of NASH and HCC. |
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| 言語 | en | |||||||||||||||
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| 内容記述タイプ | Other | |||||||||||||||
| 内容記述 | Citation: Satoru Hasuike, Yoshinori Ozono, Keisuke Uchida, Souichiro Ogawa, Hotaka Tamura, Naomi Uchiyama, Hiroshi Hatada, Yuri Komaki, Kenichi Nakamura, Hisayoshi Iwakiri, Mitsue Sueta, Kenji Nagata, Misayo Matsuyama, Hirotake Sawada, Toshiyuki Oguri, Yuichiro Sato, Hiroshi Kawakami, An autopsy case of an adult woman with Rapid-Onset Obesity with Hypoventilation, Hypothalamic, Autonomic Dysregulation, and Neuroendocrine Tumors (ROHHAD(NET)) syndrome developing nonalcoholic steatohepatitis and hepatocellular carcinoma: A case report, Medicine, 103(22), e38383-e38383, 2024-05-31, https://doi.org/10.1097/MD.0000000000038383 | |||||||||||||||
| 言語 | en | |||||||||||||||
| bibliographic_information |
en : Medicine 巻 103, 号 22, p. e38383, 発行日 2024-05-31 |
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| 出版者 | ||||||||||||||||
| 出版者 | Ovid Technologies (Wolters Kluwer Health) | |||||||||||||||
| 言語 | en | |||||||||||||||
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| 収録物識別子タイプ | PISSN | |||||||||||||||
| 収録物識別子 | 0025-7974 | |||||||||||||||
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| 収録物識別子タイプ | EISSN | |||||||||||||||
| 収録物識別子 | 1536-5964 | |||||||||||||||
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| 関連タイプ | isVersionOf | |||||||||||||||
| 識別子タイプ | DOI | |||||||||||||||
| 関連識別子 | https://doi.org/10.1097/md.0000000000038383 | |||||||||||||||
| 権利 | ||||||||||||||||
| 言語 | en | |||||||||||||||
| 権利情報 | Copyright © 2024 the Author(s). | |||||||||||||||
| 出版タイプ | ||||||||||||||||
| 出版タイプ | VoR | |||||||||||||||
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蓮池, 悟, 大園, 芳範, 内田, 圭祐, Ogawa, Souichiro, 田村, 穗高, 内山, 尚美, 畑田, 紘志, Komaki, Yuri, 中村, 憲一, 岩切, 久芳, 末田, 光恵, 永田, 賢治, Toyoki Nishimura, Matsuyama, Misayo, 澤田, 浩武, Oguri, Toshiyuki, 佐藤, 勇一郎, 河上, 洋, 2024, An autopsy case of an adult woman with Rapid-Onset Obesity with Hypoventilation, Hypothalamic, Autonomic Dysregulation, and Neuroendocrine Tumors (ROHHAD(NET)) syndrome developing nonalcoholic steatohepatitis and hepatocellular carcinoma: A case report: Ovid Technologies (Wolters Kluwer Health), e38383– p.
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