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- Michigami Toshimi
- Department of Bone and Mineral Research, Research Institute, Osaka Women’s and Children’s Hospital, Osaka Prefectural Hospital Organization, Osaka, Japan Task Force for Hypophosphatasia Guidelines
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- Ohata Yasuhisa
- Department of Pediatrics, Osaka University Graduate School of Medicine, Osaka, Japan Task Force for Hypophosphatasia Guidelines
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- Fujiwara Makoto
- Department of Pediatrics, Osaka University Graduate School of Medicine, Osaka, Japan Task Force for Hypophosphatasia Guidelines
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- Mochizuki Hiroshi
- Division of Endocrinology and Metabolism, Saitama Children’s Medical Center, Saitama, Japan Task Force for Hypophosphatasia Guidelines
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- Adachi Masanori
- Department of Endocrinology and Metabolism, Kanagawa Children’s Medical Center, Kanagawa, Japan Task Force for Hypophosphatasia Guidelines
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- Kitaoka Taichi
- Department of Pediatrics, Osaka University Graduate School of Medicine, Osaka, Japan Task Force for Hypophosphatasia Guidelines
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- Kubota Takuo
- Department of Pediatrics, Osaka University Graduate School of Medicine, Osaka, Japan Task Force for Hypophosphatasia Guidelines
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- Sawai Hideaki
- Department of Obstetrics and Gynecology, Hyogo College of Medicine, Hyogo, Japan Task Force for Hypophosphatasia Guidelines
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- Namba Noriyuki
- Division of Pediatrics and Perinatology, Tottori University Faculty of Medicine, Tottori, Japan Task Force for Hypophosphatasia Guidelines
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- Hasegawa Kosei
- Department of Pediatrics, Okayama University Hospital, Okayama, Japan Task Force for Hypophosphatasia Guidelines
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- Fujiwara Ikuma
- Department of Pediatrics, Sendai City Hospital, Miyagi, Japan Task Force for Hypophosphatasia Guidelines
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- Ozono Keiichi
- Department of Pediatrics, Osaka University Graduate School of Medicine, Osaka, Japan Task Force for Hypophosphatasia Guidelines
この論文をさがす
抄録
<p>Hypophosphatasia (HPP) is a rare bone disease caused by inactivating mutations in the ALPL gene, which encodes tissue-nonspecific alkaline phosphatase (TNSALP). Patients with HPP have varied clinical manifestations and are classified based on the age of onset and severity. Recently, enzyme replacement therapy using bone-targeted recombinant alkaline phosphatase (ALP) has been developed, leading to improvement in the prognosis of patients with life-threatening HPP. Considering these recent advances, clinical practice guidelines have been generated to provide physicians with guides for standard medical care for HPP and to support their clinical decisions. A task force was convened for this purpose, and twenty-one clinical questions (CQs) were formulated, addressing the issues of clinical manifestations and diagnosis (7 CQs) and those of management and treatment (14 CQs). A systematic literature search was conducted using PubMed/MEDLINE, and evidence-based recommendations were developed. The guidelines have been modified according to the evaluations and suggestions from the Clinical Guideline Committee of The Japanese Society for Pediatric Endocrinology (JSPE) and public comments obtained from the members of the JSPE and a Japanese HPP patient group, and then approved by the Board of Councils of the JSPE. We anticipate that the guidelines will be revised regularly and updated.</p>
収録刊行物
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- Clinical Pediatric Endocrinology
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Clinical Pediatric Endocrinology 29 (1), 9-24, 2020
日本小児内分泌学会
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詳細情報
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- CRID
- 1390002184861996800
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- NII論文ID
- 130007783622
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- DOI
- 10.1297/cpe.29.9
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- ISSN
- 13477358
- 09185739
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- 本文言語コード
- en
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- データソース種別
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- JaLC
- Crossref
- CiNii Articles
- KAKEN
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- 抄録ライセンスフラグ
- 使用不可