{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T10:04:57Z","timestamp":1753869897214,"version":"3.41.2"},"reference-count":24,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2022,7,20]],"date-time":"2022-07-20T00:00:00Z","timestamp":1658275200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2022,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Genetically targeted drugs in vascular anomalies (VA) are used despite the absence of a validated severity score. The aim of this study was to evaluate the feasibility of grouping phenotypic VA clinical characteristics into a single severity score.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A systematic literature review including children treated with sirolimus accompanied by a detailed description of phenotype and management was conducted. Demographic data and clinical features were extracted to define distinct categories of phenotypes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Children with VA display two main phenotypes regardless of VA subtype, which may overlap. A <jats:italic>systemic phenotype<\/jats:italic> results from direct invasion and compression of vital structures generally leading to hospitalization and aggressive management in infancy. A <jats:italic>functional phenotype<\/jats:italic> is associated with chronic pain and disability manifesting mainly during early adolescence and managed in the outpatient setting.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The two distinct phenotypes described could be the basis for developing a unified scoring system for VA severity assessment.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.29869","type":"journal-article","created":{"date-parts":[[2022,6,22]],"date-time":"2022-06-22T14:53:02Z","timestamp":1655909582000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Defining vascular anomaly phenotypes in children based on a systematic literature search: A critical step in developing a single severity score for interventional clinical trials"],"prefix":"10.1002","volume":"69","author":[{"given":"Laurence","family":"Gari\u00e9py\u2010Assal","sequence":"first","affiliation":[{"name":"Pediatric Residency Program CHU Sainte\u2010Justine Universit\u00e9 de Montr\u00e9al  Montr\u00e9al Quebec Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jos\u00e9e","family":"Dubois","sequence":"additional","affiliation":[{"name":"Department of Radiology CHU Sainte\u2010Justine Universit\u00e9 de Montr\u00e9al  Montr\u00e9al Quebec Canada"},{"name":"Vascular Anomaly Team CHU Sainte\u2010Justine Universit\u00e9 de Montr\u00e9al  Canada"},{"name":"Vascular Anomalies Canada Anomalies Vasculaires  Montr\u00e9al Quebec Canada"},{"name":"Research Center CHU Sainte\u2010Justine Universit\u00e9 de Montr\u00e9al  Montr\u00e9al Quebec Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kelley","family":"Zwicker","sequence":"additional","affiliation":[{"name":"Vascular Anomalies Canada Anomalies Vasculaires  Montr\u00e9al Quebec Canada"},{"name":"Department of Pediatrics, Children's Hospital of Eastern Ontario University of Ottawa  Ottawa Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alix","family":"Pincivy","sequence":"additional","affiliation":[{"name":"Library, CHU Sainte\u2010Justine Universit\u00e9 de Montr\u00e9al  Montr\u00e9al Quebec Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julie","family":"Powell","sequence":"additional","affiliation":[{"name":"Vascular Anomaly Team CHU Sainte\u2010Justine Universit\u00e9 de Montr\u00e9al  Canada"},{"name":"Vascular Anomalies Canada Anomalies Vasculaires  Montr\u00e9al Quebec Canada"},{"name":"Division of Dermatology Department of Pediatrics CHU Sainte\u2010Justine Universit\u00e9 de Montr\u00e9al  Montr\u00e9al Quebec Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yang","family":"Zhang","sequence":"additional","affiliation":[{"name":"Canadian Economic Analysis Department, Bank of Canada  Ottawa Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vicky","family":"Breakey","sequence":"additional","affiliation":[{"name":"Vascular Anomalies Canada Anomalies Vasculaires  Montr\u00e9al Quebec Canada"},{"name":"Division of Pediatric Hematology\/Oncology McMaster University  Hamilton Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Victoria","family":"Price","sequence":"additional","affiliation":[{"name":"Vascular Anomalies Canada Anomalies Vasculaires  Montr\u00e9al Quebec Canada"},{"name":"Division of Pediatric Hematology\u2010Oncology Department of Pediatrics Dalhousie University  Halifax Nova Scotia Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Leonardo R.","family":"Brand\u00e3o","sequence":"additional","affiliation":[{"name":"Vascular Anomalies Canada Anomalies Vasculaires  Montr\u00e9al Quebec Canada"},{"name":"Dalla Lana School of Public Health University of Toronto  Toronto Ontario Canada"},{"name":"Division of Haematology\/Oncology Department of Paediatrics Hospital for Sick Children  Toronto Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Manuel","family":"Carcao","sequence":"additional","affiliation":[{"name":"Vascular Anomalies Canada Anomalies Vasculaires  Montr\u00e9al Quebec Canada"},{"name":"Division of Haematology\/Oncology Department of Paediatrics Hospital for Sick Children  Toronto Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Niina","family":"Kleiber","sequence":"additional","affiliation":[{"name":"Vascular Anomaly Team CHU Sainte\u2010Justine Universit\u00e9 de Montr\u00e9al  Canada"},{"name":"Vascular Anomalies Canada Anomalies Vasculaires  Montr\u00e9al Quebec Canada"},{"name":"Department of Pediatrics CHU Sainte\u2010Justine Universit\u00e9 de Montr\u00e9al  Montr\u00e9al Quebec Canada"},{"name":"Research Center CHU Sainte\u2010Justine Universit\u00e9 de Montr\u00e9al  Montr\u00e9al Quebec Canada"},{"name":"Department of Pharmacology and Physiology Universit\u00e9 de Montr\u00e9al  Montr\u00e9al Quebec Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,7,20]]},"reference":[{"key":"e_1_2_9_2_1","unstructured":"ISSVA Classification of Vascular Anomalies.Classification of the International Society for the Study of Vascular Anomalies (ISSVA); Published2018. Accessed March 6 2021.https:\/\/www.issva.org\/classification"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2014-3673"},{"volume-title":"GeneReviews","year":"1993","author":"Mirzaa G","key":"e_1_2_9_4_1"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.36552"},{"key":"e_1_2_9_6_1","unstructured":"International Society for the Study of Vascular Anomalies Mission Statement.ISSVA; Published2019.https:\/\/www.issva.org\/about\u2010issva\/mission. Accessed May 2022."},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/MOP.0000000000000794"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-016-4007-x"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1159\/000515980"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jvs.2019.06.217"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01402041"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1001\/archdermatol.2011.926"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/pde.13032"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-014-0188-3"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.29364"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-019-1118-1"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-018-0934-z"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2015-3257"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-015-2572-y"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.23124"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13643-020-01542-z"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1097\/SAP.0b013e31821453c8"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.16029"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0895-4356(02)00537-1"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.18619"}],"container-title":["Pediatric Blood &amp; Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.29869","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/pbc.29869","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.29869","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,23]],"date-time":"2023-08-23T11:39:07Z","timestamp":1692790747000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pbc.29869"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,7,20]]},"references-count":24,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2022,10]]}},"alternative-id":["10.1002\/pbc.29869"],"URL":"https:\/\/doi.org\/10.1002\/pbc.29869","archive":["Portico"],"relation":{},"ISSN":["1545-5009","1545-5017"],"issn-type":[{"type":"print","value":"1545-5009"},{"type":"electronic","value":"1545-5017"}],"subject":[],"published":{"date-parts":[[2022,7,20]]},"assertion":[{"value":"2022-03-19","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-06-16","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-07-20","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e29869"}}