Critical infantile hepatic hemangioma: Results of a nationwide survey by the Japanese Infantile Hepatic Hemangioma Study Group

Tatsuo Kuroda, Masaaki Kumagai, Shunsuke Nosaka, Atsuko Nakazawa, Tetsuya Takimoto, Ken Hoshino

研究成果: Article

19 引用 (Scopus)

抄録

Background: The current survey aimed to describe the clinical features of critical infantile hepatic hemangioma (IHH) and the implications of recent treatments. Materials and Methods: A nationwide survey of critical IHH patients treated between 2005 and 2010 was performed in all 117 registered pediatric surgical hospitals in Japan. As a result, 19 patients were identified and reviewed using a statistical analysis. Results: Abdominal distention (47.4%), high-output cardiac failure (47.4%), coagulopathy (42.1%), and respiratory distress (31.6%) were the major symptoms. Three patients died (1 of coagulopathy, 1 of cardiac failure, and 1 of both). An accompanying portovenous shunt was also highlighted. Infantile hepatic hemangioma was totally insensitive to steroid treatment in 3 (23.1%) of the 13 patients, and 9 (47.4%) of the 19 patients required other treatments. Surgical resection and β-blocker improved the hematologic data, whereas hepatic arterial ligation and embolization seemed to produce a limited effect. Among the dead patients, several hematologic parameters were significantly worse: the thrombocyte count (pretherapeutic: 73 000 vs 300 000/mm 3, dead vs survivor, respectively [P <.03]; posttherapeutic: 66 000 vs 388 700/mm 3 [P <.003]) and the prothrombin time (posttherapeutic, 35.0 vs 12.1 seconds [P <.0001], dead vs survivor, respectively). Conclusion: For critical IHH cases with steroid-insensitive hematologic disorders, alternative treatments including β-blocker therapy, surgery, and liver transplantation should be considered.

元の言語English
ページ(範囲)2239-2243
ページ数5
ジャーナルJournal of Pediatric Surgery
46
発行部数12
DOI
出版物ステータスPublished - 2011 12

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Hemangioma
Liver
Survivors
Heart Failure
Steroids
Therapeutics
Pediatric Hospitals
Prothrombin Time
Liver Transplantation
Ligation
Surveys and Questionnaires
Japan
Blood Platelets

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

これを引用

Critical infantile hepatic hemangioma : Results of a nationwide survey by the Japanese Infantile Hepatic Hemangioma Study Group. / Kuroda, Tatsuo; Kumagai, Masaaki; Nosaka, Shunsuke; Nakazawa, Atsuko; Takimoto, Tetsuya; Hoshino, Ken.

:: Journal of Pediatric Surgery, 巻 46, 番号 12, 12.2011, p. 2239-2243.

研究成果: Article

Kuroda, Tatsuo ; Kumagai, Masaaki ; Nosaka, Shunsuke ; Nakazawa, Atsuko ; Takimoto, Tetsuya ; Hoshino, Ken. / Critical infantile hepatic hemangioma : Results of a nationwide survey by the Japanese Infantile Hepatic Hemangioma Study Group. :: Journal of Pediatric Surgery. 2011 ; 巻 46, 番号 12. pp. 2239-2243.
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abstract = "Background: The current survey aimed to describe the clinical features of critical infantile hepatic hemangioma (IHH) and the implications of recent treatments. Materials and Methods: A nationwide survey of critical IHH patients treated between 2005 and 2010 was performed in all 117 registered pediatric surgical hospitals in Japan. As a result, 19 patients were identified and reviewed using a statistical analysis. Results: Abdominal distention (47.4{\%}), high-output cardiac failure (47.4{\%}), coagulopathy (42.1{\%}), and respiratory distress (31.6{\%}) were the major symptoms. Three patients died (1 of coagulopathy, 1 of cardiac failure, and 1 of both). An accompanying portovenous shunt was also highlighted. Infantile hepatic hemangioma was totally insensitive to steroid treatment in 3 (23.1{\%}) of the 13 patients, and 9 (47.4{\%}) of the 19 patients required other treatments. Surgical resection and β-blocker improved the hematologic data, whereas hepatic arterial ligation and embolization seemed to produce a limited effect. Among the dead patients, several hematologic parameters were significantly worse: the thrombocyte count (pretherapeutic: 73 000 vs 300 000/mm 3, dead vs survivor, respectively [P <.03]; posttherapeutic: 66 000 vs 388 700/mm 3 [P <.003]) and the prothrombin time (posttherapeutic, 35.0 vs 12.1 seconds [P <.0001], dead vs survivor, respectively). Conclusion: For critical IHH cases with steroid-insensitive hematologic disorders, alternative treatments including β-blocker therapy, surgery, and liver transplantation should be considered.",
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