{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T02:02:31Z","timestamp":1790042551502,"version":"4.0.1"},"reference-count":17,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2016,7,19]],"date-time":"2016-07-19T00:00:00Z","timestamp":1468886400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Dermatology"],"published-print":{"date-parts":[[2016,9]]},"abstract":"Abstract<\/jats:title>Background\/Objectives<\/jats:title>Facial angiofibromas (AF<\/jats:styled-content>) have the potential to cause disfigurement in children with tuberous sclerosis complex (TSC<\/jats:styled-content>). Facial disfigurement can impact the quality of life (QoL) of individuals and their families, leading to negative psychosocial outcomes. QoL has not been studied in TSC<\/jats:styled-content> patients with AF<\/jats:styled-content>.<\/jats:p><\/jats:sec>Methods<\/jats:title>We conducted a cross\u2010sectional survey study to investigate QoL of TSC<\/jats:styled-content> patients with AF<\/jats:styled-content> and their caregivers and to explore the current state of access to treatment for AF<\/jats:styled-content>. TSC<\/jats:styled-content> patients and caregivers in TSC<\/jats:styled-content> clinic at Boston Children's Hospital and through the Tuberous Sclerosis Alliance were recruited to complete QoL surveys including the CADIS<\/jats:styled-content>, CDLQI<\/jats:styled-content>, and Skindex\u2010teen questionnaires, and a survey on access to treatment of AF<\/jats:styled-content>.<\/jats:p><\/jats:sec>Results<\/jats:title>Fifty\u2010eight patients with TSC<\/jats:styled-content> and 161 caregivers participated in the study. Caregivers of patients with AF<\/jats:styled-content> had significantly poorer QoL scores compared to caregivers of those without AF<\/jats:styled-content>, as measured by a modified CADIS<\/jats:styled-content> questionnaire (mean 31.7 vs. 11.7, p = 0.004). Among patients with AF<\/jats:styled-content>, those who received treatment had significantly better QoL scores compared with those without treatment, as measured by the CDLQI<\/jats:styled-content> (mean 3.8 vs. 9.5, p = 0.001). Forty\u2010one and two\u2010tenths percent of subjects reported never receiving treatment for AF<\/jats:styled-content>. Forty\u2010seven and three\u2010tenths percent of subjects were prescribed topical rapamycin, 47.7% of whom experienced difficulty with insurance coverage.<\/jats:p><\/jats:sec>Conclusions<\/jats:title>Presence and lack of treatment of AF<\/jats:styled-content> significantly impacts QoL in TSC<\/jats:styled-content> patients and their caregivers. Access to care for AF<\/jats:styled-content> is limited by multiple factors and should be addressed by clinicians working with this patient population.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pde.12933","type":"journal-article","created":{"date-parts":[[2016,7,20]],"date-time":"2016-07-20T01:22:44Z","timestamp":1468977764000},"page":"518-525","source":"Crossref","is-referenced-by-count":21,"title":["Effect of Angiofibromas on Quality of Life and Access to Care in Tuberous Sclerosis Patients and Their Caregivers"],"prefix":"10.1111","volume":"33","author":[{"given":"Cary","family":"Crall","sequence":"first","affiliation":[{"name":"Harvard Medical School Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Molly","family":"Valle","sequence":"additional","affiliation":[{"name":"Department of Neurology Boston Children's Hospital Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kush","family":"Kapur","sequence":"additional","affiliation":[{"name":"Harvard Medical School Boston Massachusetts"},{"name":"Department of Neurology Boston Children's Hospital Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kira A.","family":"Dies","sequence":"additional","affiliation":[{"name":"Department of Neurology Boston Children's Hospital Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marilyn G.","family":"Liang","sequence":"additional","affiliation":[{"name":"Harvard Medical School Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mustafa","family":"Sahin","sequence":"additional","affiliation":[{"name":"Harvard Medical School Boston Massachusetts"},{"name":"Department of Neurology Boston Children's Hospital Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer T.","family":"Huang","sequence":"additional","affiliation":[{"name":"Harvard Medical School Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,7,19]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2007.05.004"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.1996.d01-923.x"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.2011.211995"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/ced.12327"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1597\/06-072.1"},{"key":"e_1_2_6_7_1","volume-title":"The psychology of appearance and disfigurement","author":"Rumsey N","year":"2005"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/archdischild-2012-302470"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/ajd.12125"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.1995.tb16953.x"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1001\/archdermatol.2010.161"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.143.6.768"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.det.2012.12.010"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2014.07.029"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2012.01.038"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-3083.2011.04212.x"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.12567"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1177\/0883073813488664"}],"container-title":["Pediatric Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fpde.12933","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pde.12933","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,24]],"date-time":"2023-09-24T01:07:19Z","timestamp":1695517639000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pde.12933"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,7,19]]},"references-count":17,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2016,9]]}},"alternative-id":["10.1111\/pde.12933"],"URL":"https:\/\/doi.org\/10.1111\/pde.12933","archive":["Portico"],"relation":{},"ISSN":["0736-8046","1525-1470"],"issn-type":[{"value":"0736-8046","type":"print"},{"value":"1525-1470","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,7,19]]}}}