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Keratitis-ichthyosis-deafness (KID) syndrome patients showed a significantly higher risk of skin infections than patients with other phenotypes. Complete data on DLQI were obtained from 13 patients, whose median age was 21 (8–71) years. Nine patients were male, and 4 were female. Systemic retinoids were administrated to 2 of the 3 HI patients. The Spearman’s correlation coefficient between the clinical ichthyosis score and DLQI was 0.611 (P \u003c 0.05). Conclusion: We confirmed that Netherton syndrome and KID syndrome patients have a higher risk of allergy to food or environmental allergens and of skin infections, respectively. 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  1. C100 医学部/医学系研究科
  2. C100a 雑誌掲載論文
  3. 学術雑誌

Cross-sectional survey on disease severity in Japanese patients with harlequin ichthyosis/ichthyosis: Syndromic forms and quality-of-life analysis in a subgroup

http://hdl.handle.net/2237/00030155
http://hdl.handle.net/2237/00030155
b1a3b896-bc13-4f7a-8f85-b0f8b685aa4a
名前 / ファイル ライセンス アクション
Revise.pdf Revise (627.7 kB)
Item type 学術雑誌論文 / Journal Article(1)
公開日 2019-05-10
タイトル
タイトル Cross-sectional survey on disease severity in Japanese patients with harlequin ichthyosis/ichthyosis: Syndromic forms and quality-of-life analysis in a subgroup
言語 en
著者 Murase, Chiaki

× Murase, Chiaki

WEKO 91029

en Murase, Chiaki

Search repository
Takeichi, Takuya

× Takeichi, Takuya

WEKO 91030

en Takeichi, Takuya

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Shibata, Akitaka

× Shibata, Akitaka

WEKO 91031

en Shibata, Akitaka

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Nakatochi, Masahiro

× Nakatochi, Masahiro

WEKO 91032

en Nakatochi, Masahiro

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Kinoshita, Fumie

× Kinoshita, Fumie

WEKO 91033

en Kinoshita, Fumie

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Kubo, Akiharu

× Kubo, Akiharu

WEKO 91034

en Kubo, Akiharu

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Nakajima, Kimiko

× Nakajima, Kimiko

WEKO 91035

en Nakajima, Kimiko

Search repository
Ishii, Norito

× Ishii, Norito

WEKO 91036

en Ishii, Norito

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Amano, Hiroo

× Amano, Hiroo

WEKO 91037

en Amano, Hiroo

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Masuda, Koji

× Masuda, Koji

WEKO 91038

en Masuda, Koji

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Kawakami, Hiroshi

× Kawakami, Hiroshi

WEKO 91039

en Kawakami, Hiroshi

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Kanekura, Takuro

× Kanekura, Takuro

WEKO 91040

en Kanekura, Takuro

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Washio, Ken

× Washio, Ken

WEKO 91041

en Washio, Ken

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Asano, Masayuki

× Asano, Masayuki

WEKO 91042

en Asano, Masayuki

Search repository
Teramura, Kazuya

× Teramura, Kazuya

WEKO 91043

en Teramura, Kazuya

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Akasaka, Eijiro

× Akasaka, Eijiro

WEKO 91044

en Akasaka, Eijiro

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Tohyama, Mikiko

× Tohyama, Mikiko

WEKO 91045

en Tohyama, Mikiko

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Hatano, Yutaka

× Hatano, Yutaka

WEKO 91046

en Hatano, Yutaka

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Ochiai, Toyoko

× Ochiai, Toyoko

WEKO 91047

en Ochiai, Toyoko

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Moriwaki, Shinichi

× Moriwaki, Shinichi

WEKO 91048

en Moriwaki, Shinichi

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Sato, Tomotaka

× Sato, Tomotaka

WEKO 91049

en Sato, Tomotaka

Search repository
Ishida-Yamamoto, Akemi

× Ishida-Yamamoto, Akemi

WEKO 91050

en Ishida-Yamamoto, Akemi

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Seishima, Mariko

× Seishima, Mariko

WEKO 91051

en Seishima, Mariko

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Kurosawa, Michiko

× Kurosawa, Michiko

WEKO 91052

en Kurosawa, Michiko

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Ikeda, Shigaku

× Ikeda, Shigaku

WEKO 91053

en Ikeda, Shigaku

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Akiyama, Masashi

× Akiyama, Masashi

WEKO 91054

en Akiyama, Masashi

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アクセス権
アクセス権 open access
アクセス権URI http://purl.org/coar/access_right/c_abf2
権利
言語 en
権利情報 © 2018. This manuscript version is made available under the CC-BY-NC-ND 4.0 license http://creativecommons.org/licenses/by-nc-nd/4.0/
キーワード
主題Scheme Other
主題 Clinical ichthyosis score
キーワード
主題Scheme Other
主題 Harlequin ichthyosis
キーワード
主題Scheme Other
主題 Congenital ichthyosis
キーワード
主題Scheme Other
主題 Syndromic form ichthyosis
キーワード
主題Scheme Other
主題 Quality of life
抄録
内容記述 Background: Congenital ichthyoses (CIs) adversely affect quality of life (QOL) in patients. However, the effects of CIs on patient QOL have not been studied sufficiently. Objective: To investigate the association between disease severity and QOL in patients with harlequin ichthyosis (HI) and ichthyosis: syndromic forms (ISFs) Methods: Clinical information of patients with HI and ISFs from 2010 to 2015 were obtained from 100 dermatology departments/divisions of principal institutes/hospitals throughout Japan. We examined the relationship between disease severity and QOL in patients with HI and ISFs. Patients who were aged 8 years or older and participated in a multicenter retrospective questionnaire survey in Japan were assessed by dermatology life quality index (DLQI, range of 0–30) and clinical ichthyosis score (range of 0–100). Results: Netherton syndrome patients had a significantly higher risk of allergy to food or environmental allergens than patients with other phenotypes. Keratitis-ichthyosis-deafness (KID) syndrome patients showed a significantly higher risk of skin infections than patients with other phenotypes. Complete data on DLQI were obtained from 13 patients, whose median age was 21 (8–71) years. Nine patients were male, and 4 were female. Systemic retinoids were administrated to 2 of the 3 HI patients. The Spearman’s correlation coefficient between the clinical ichthyosis score and DLQI was 0.611 (P < 0.05). Conclusion: We confirmed that Netherton syndrome and KID syndrome patients have a higher risk of allergy to food or environmental allergens and of skin infections, respectively. QOL impairment correlates with disease severity in HI and ISFs patients.
言語 en
内容記述タイプ Abstract
内容記述
内容記述 ファイル公開:2019-11-01
言語 ja
内容記述タイプ Other
出版者
言語 en
出版者 Elsevier
言語
言語 eng
資源タイプ
資源タイプresource http://purl.org/coar/resource_type/c_6501
タイプ journal article
出版タイプ
出版タイプ AM
出版タイプResource http://purl.org/coar/version/c_ab4af688f83e57aa
ISSN(print)
収録物識別子タイプ PISSN
収録物識別子 0923-1811
書誌情報 en : Journal of Dermatological Science

巻 92, 号 2, p. 127-133, 発行日 2018-11
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