Survey on Neonatal hemochromatosis in Japan：2010-2014

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  • Nagasawa Junko
    Department of Neonatology, Center of Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development
  • Wada Yuka
    Department of Neonatology, Center of Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development
  • Sasaki Aiko
    Department of Obstetrics, Center of Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development
  • Motomura Kenichiro
    Center of Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development Department of Allergy and Clinical Immunology, National Research Institute for Child Health and Development
  • Ito Reiko
    Department of General Pediatrics & Interdisciplinary Medicine, National Center for Child Health and Development
  • Matsumoto Kenji
    Department of Allergy and Clinical Immunology, National Research Institute for Child Health and Development
  • Sago Haruhiko
    Center of Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development
  • Harada Eimei
    Department of Pediatrics and Child Health, Kurume University School of Medicine
  • Kanda Hiroshi
    Department of Pediatrics, Iizuka Hospital
  • Ueno Yasuhisa
    Department of Neonatology, Medical Center for Maternal and Child Health, Ishikawa Prefectural Central Hospital
  • Nakata Yuya
    Department of Neonatology, Medical Center for Maternal and Child Health, Ishikawa Prefectural Central Hospital
  • Kondo Sonoko
    Department of Pediatrics, Kagawa University
  • Koyano Kosuke
    Department of Pediatrics, Kagawa University
  • Takakura Masahiro
    Department of Obstetrics and Gynecology, Kanazawa University
  • Mitani Yusuke
    Perinatal Mother and Infant Care Center, Kanazawa University Hospital
  • Matsuura Toshiharu
    Department of Pediatric Surgery, Kyushu University Hospital
  • Taguchi Tomoaki
    Department of Pediatric Surgery, Kyushu University Hospital
  • Hayashida Shintaro
    Department of Pediatric Surgery and Transplantation, Graduate School of Medical Sciences, Kumamoto University
  • Matsumoto Shirou
    Department of Pediatrics, Graduate School of Medical Sciences, Kumamoto University
  • Nakamura Kuriko
    Department of Neonatology, Saiseikai Yokohamashi Tobu Hospital
  • Inui Ayano
    Department of Pediatric Hepatology and Gastroenterology, Saiseikai Yokohamashi Tobu Hospital
  • Okada Noriki
    Department of Transplant Surgery, Jichi Medical University
  • Mizuta Koichi
    Department of Transplant Surgery, Jichi Medical University
  • Masunaga Ken
    Department of Neonatology, Tokyo Metropolitan Ohtsuka Hospital
  • Horikawa Shinjiro
    Perinatal Mother and Infant Care Center, Toyama University Hospital
  • Tanaka Taihei
    Department of Neonatology, Japanese Red Cross Nagoya Daini Hospital
  • Hirooka Takako
    Department of Neonatology, Japanese Red Cross Nagoya Daini Hospital
  • Nakao Atsushi
    Department of Neonatology, Japanese Red Cross Medical Center
  • Tsurisawa Chisa
    Department of Neonatology, Japanese Red Cross Medical Center
  • Kenmochi Takahiro
    Perinatal Center, Yokohama City University Medical Center
  • Seki Kazuo
    Perinatal Center, Yokohama City University Medical Center
  • Ito Yushi
    Department of Neonatology, Center of Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development

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Other Title
  • 日本における新生児ヘモクロマトーシス実態調査：2010-2014年

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<p> Neonatal hemochromatosis（NH）is a disease with poor prognosis causing severe liver failure in both fetal and neonatal periods and is assumed to be an allogeneic immunological fetal liver injury which ultimately requires liver transplantation.</p><p> Up till date, no survey has outlined management of NH in Japan, due to its difficulty in diagnoses and treatment.</p><p> Here we reported the first nationwide survey for management of cases diagnosed clinically with NH in a 5 years period from 2010 to 2014.</p><p> From 275 hospitals included in the survey a total of 197 hospitals responded.</p><p> The responds included managements of 19 cases.</p><p> Only 2 cases were completely fulfilling the diagnostic criteria given by the Japan Society of Pediatric Nutrition and Gastroenterology Liver, suggesting the possibility that the given diagnostic criteria need to be revised. Based on this result, new diagnostic criteria were created.</p><p> A wide variation in diagnostic investigations and treatments for the included cases were observed among hospitals.</p><p> From this survey, a standardized management for NH is warranted.</p><p> Furthermore, NH diagnosis was less successful in postnatal period as many cases could not receive the diagnostic investigation. Additionally, suspected cases could not receive the ultimate treatment due to their poor condition. Therefore, it would be crucial to improve NH prognosis by focusing on diagnosis and treatment during fetal period.</p>

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