{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T16:38:27Z","timestamp":1787503107205,"version":"build-2736575974"},"reference-count":14,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2024,4,15]],"date-time":"2024-04-15T00:00:00Z","timestamp":1713139200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Dermatology"],"published-print":{"date-parts":[[2024,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    The Index for Facial Angiofibromas (IFA), a novel scoring system for angiofibromas, has been validated in patients with tuberous sclerosis complex (TSC). The objective of this analysis was to further validate the IFA using data from a clinical trial of topical sirolimus in patients with TSC. This was an analysis of photographs from a Phase III trial conducted in Japan (NCT02635789). Patients (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200962) were randomized 1:1 to receive sirolimus or placebo gel for 12\u2009weeks. Changes in angiofibromas were independently assessed using the primary composite endpoint, the Facial Angiofibroma Severity Index (FASI), and the IFA. Thresholds for a clinically meaningful change in IFA score were evaluated using receiver operating characteristic (ROC) analysis. The IFA scores had good\u2010to\u2010excellent inter\u2010assessor reliability, very high intra\u2010assessor reliability, and could be used to evaluate the distribution of disease severity at baseline. High correlations were observed between the categorized change from baseline in IFA scores and the primary composite endpoint (Kendall's coefficient of concordance,\n                    <jats:italic>W<\/jats:italic>\n                    \u2009=\u20090.8655,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.0001), and between the change from baseline in IFA and FASI scores (Kendall's coefficient of concordance,\n                    <jats:italic>W<\/jats:italic>\n                    \u2009=\u20090.745,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.0001). By ROC analysis, an optimal IFA cut\u2010off point of 1.667 was determined to distinguish patients with markedly improved or improved angiofibromas from those with slightly improved or unchanged angiofibromas (area under the curve 0.937) as determined by the primary composite endpoint. The IFA score is potentially clinically useful because of its high validity and reliability. A decrease in score from baseline of \u22651.667 may be considered clinically meaningful.\n                  <\/jats:p>","DOI":"10.1111\/1346-8138.17220","type":"journal-article","created":{"date-parts":[[2024,4,15]],"date-time":"2024-04-15T08:16:15Z","timestamp":1713168975000},"page":"752-758","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Validation of the Index for Facial Angiofibromas: Data analysis from a randomized controlled trial of sirolimus gel treatment in patients with tuberous sclerosis complex"],"prefix":"10.1111","volume":"51","author":[{"given":"Izumi","family":"Hamada","sequence":"first","affiliation":[{"name":"Research &amp; Development Division, Nobelpharma Co., Ltd  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshinori","family":"Yukutake","sequence":"additional","affiliation":[{"name":"Research &amp; Development Division, Nobelpharma Co., Ltd  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yusuke","family":"Morita","sequence":"additional","affiliation":[{"name":"Research &amp; Development Division, Nobelpharma Co., Ltd  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Norifumi","family":"Ishikawa","sequence":"additional","affiliation":[{"name":"Research &amp; Development Division, Nobelpharma Co., Ltd  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenji","family":"Shimizu","sequence":"additional","affiliation":[{"name":"Research &amp; Development Division, Nobelpharma Co., Ltd  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mari","family":"Wataya\u2010Kaneda","sequence":"additional","affiliation":[{"name":"Department of Neurocutaneous Medicine, Division of Health Sciences Graduate School of Medicine Osaka University  Osaka Japan"},{"name":"Department of Dermatology, Graduate School of Medicine Osaka University  Osaka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,4,15]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.2147\/JMDH.S266990"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(22)00213-7"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/ddg.13264"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2018.0464"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2021.07.011"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2018.1408"},{"key":"e_1_2_9_8_1","unstructured":"Hyftor (sirolimus topical gel). Prescribing information [Internet]. 2022 [cited 2023 April 7]. Available from:https:\/\/www.accessdata.fda.gov\/drugsatfda_docs\/label\/2022\/213478s000lbl.pdf"},{"key":"e_1_2_9_9_1","unstructured":"Sirolimus gel approved by China's National Medical Products Administration [Internet]. Nobelpharma press release 18\/4\/2023 [cited 2023 June 16]. Available from:https:\/\/www.nobelpharma.co.jp\/_cms\/wp\u2010content\/uploads\/2023\/04\/2d0bf870ba6ba2ec935638b713ecb690.pdf"},{"key":"e_1_2_9_10_1","unstructured":"Eurpean Medicines Agency.Hyftor (sirolimus) summary of product characteristics [Internet]. 2023 [cited 2023 June 16]. Available from:https:\/\/www.ema.europa.eu\/en\/documents\/product\u2010information\/hyftor\u2010epar\u2010product\u2010information_en.pdf"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/ced.12398"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2022.07.041"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1037\/0033-2909.86.2.420"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1037\/1040-3590.6.4.284"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcm.2016.02.012"}],"container-title":["The Journal of Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/1346-8138.17220","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,11,13]],"date-time":"2025-11-13T11:15:18Z","timestamp":1763032518000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/1346-8138.17220"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,4,15]]},"references-count":14,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2024,6]]}},"alternative-id":["10.1111\/1346-8138.17220"],"URL":"https:\/\/doi.org\/10.1111\/1346-8138.17220","archive":["Portico"],"relation":{},"ISSN":["0385-2407","1346-8138"],"issn-type":[{"value":"0385-2407","type":"print"},{"value":"1346-8138","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,4,15]]},"assertion":[{"value":"2023-12-14","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-03-26","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-04-15","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}