Diagnosis and Treatment of Angiography Positive Medium to Large Vessel Childhood Primary Angiitis of Central Nervous System (p-cPACNS): An International Survey
Childhood Primary Angiitis of Central Nervous System (cPACNS) is rare, but can cause significant damage and result in disability or even death. Because of its rarity, the sometimes acute and variable presentation, limited awareness, and the absence of widely accepted diagnostic and therapeutic stand...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2021-03-01
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| Series: | Frontiers in Pediatrics |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fped.2021.654537/full |
| _version_ | 1818859360364789760 |
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| author | Angela S. Quan Jürgen Brunner Benjamin Rose Martin Smitka Gabriele Hahn Clare E. Pain Renate Häfner Fabian Speth Lucia Gerstl Christian M. Hedrich Christian M. Hedrich |
| author_facet | Angela S. Quan Jürgen Brunner Benjamin Rose Martin Smitka Gabriele Hahn Clare E. Pain Renate Häfner Fabian Speth Lucia Gerstl Christian M. Hedrich Christian M. Hedrich |
| author_sort | Angela S. Quan |
| collection | DOAJ |
| description | Childhood Primary Angiitis of Central Nervous System (cPACNS) is rare, but can cause significant damage and result in disability or even death. Because of its rarity, the sometimes acute and variable presentation, limited awareness, and the absence of widely accepted diagnostic and therapeutic standards, cPACNS is a diagnostic and therapeutic challenge. Three subcategories of cPACNS exist, including angiography-positive non-progressive p-cPACNS, angiography-positive progressive p-cPACNS which both affects the medium to large vessels, and angiography-negative small vessel sv-cPACNS. Diagnosis and treatment of cPACNS relies on personal experience, expert opinion and case reports/case series. To collect information on diagnostic and therapeutic approaches to transient and progressive cPACNS, a survey was shared among international clinicians (German Society for Pediatric Rheumatology, the Pediatric Rheumatology European Society, the German speaking “Network Pediatric Stroke,” and members of the American College of Rheumatology/CARRA Pediatric Rheumatology list server). Results from this survey will be used to define statements toward a consensus process allowing harmonization of diagnostic and therapeutic approaches and the generation of evidence in a rare condition. |
| first_indexed | 2024-12-19T09:10:57Z |
| format | Article |
| id | doaj.art-da6bbf417cad433bb5a00a6aff9b5ec3 |
| institution | Directory Open Access Journal |
| issn | 2296-2360 |
| language | English |
| last_indexed | 2024-12-19T09:10:57Z |
| publishDate | 2021-03-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj.art-da6bbf417cad433bb5a00a6aff9b5ec32022-12-21T20:28:11ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602021-03-01910.3389/fped.2021.654537654537Diagnosis and Treatment of Angiography Positive Medium to Large Vessel Childhood Primary Angiitis of Central Nervous System (p-cPACNS): An International SurveyAngela S. Quan0Jürgen Brunner1Benjamin Rose2Martin Smitka3Gabriele Hahn4Clare E. Pain5Renate Häfner6Fabian Speth7Lucia Gerstl8Christian M. Hedrich9Christian M. Hedrich10Department of Women's and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, United KingdomPädiatrische Rheumatologie, Department Kinder- und Jugendheilkunde, Klinisches Ethikkomitee, Medizinische Universität Innsbruck, Innsbruck, AustriaDepartment of Women's and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, United KingdomKlinik und Poliklinik fur Kinder- und Jugendmedizin, Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Dresden, GermanyInstitut und Poliklinik für Radiologie, Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Dresden, GermanyDepartment of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, United KingdomDeutsches Zentrum für Kinder- und Jugendrheumatologie, Garmisch-Partenkirchen, GermanyZentrum für Geburtshilfe, Kinder- und Jugendmedizin, Klinik und Poliklinik für Kinder- und Jugendmedizin, Universitätsklinikum Eppendorf, Hamburg, GermanyDivision of Paediatric Neurology, Developmental Medicine and Social Paediatrics, Department of Paediatrics, Dr. von Hauner Children's Hospital, Ludwig-Maximilians-University Munich, Munich, GermanyDepartment of Women's and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, United KingdomDepartment of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, United KingdomChildhood Primary Angiitis of Central Nervous System (cPACNS) is rare, but can cause significant damage and result in disability or even death. Because of its rarity, the sometimes acute and variable presentation, limited awareness, and the absence of widely accepted diagnostic and therapeutic standards, cPACNS is a diagnostic and therapeutic challenge. Three subcategories of cPACNS exist, including angiography-positive non-progressive p-cPACNS, angiography-positive progressive p-cPACNS which both affects the medium to large vessels, and angiography-negative small vessel sv-cPACNS. Diagnosis and treatment of cPACNS relies on personal experience, expert opinion and case reports/case series. To collect information on diagnostic and therapeutic approaches to transient and progressive cPACNS, a survey was shared among international clinicians (German Society for Pediatric Rheumatology, the Pediatric Rheumatology European Society, the German speaking “Network Pediatric Stroke,” and members of the American College of Rheumatology/CARRA Pediatric Rheumatology list server). Results from this survey will be used to define statements toward a consensus process allowing harmonization of diagnostic and therapeutic approaches and the generation of evidence in a rare condition.https://www.frontiersin.org/articles/10.3389/fped.2021.654537/fullvasculitisCNSchildhoodpediatrictreatmentdiagnosis |
| spellingShingle | Angela S. Quan Jürgen Brunner Benjamin Rose Martin Smitka Gabriele Hahn Clare E. Pain Renate Häfner Fabian Speth Lucia Gerstl Christian M. Hedrich Christian M. Hedrich Diagnosis and Treatment of Angiography Positive Medium to Large Vessel Childhood Primary Angiitis of Central Nervous System (p-cPACNS): An International Survey Frontiers in Pediatrics vasculitis CNS childhood pediatric treatment diagnosis |
| title | Diagnosis and Treatment of Angiography Positive Medium to Large Vessel Childhood Primary Angiitis of Central Nervous System (p-cPACNS): An International Survey |
| title_full | Diagnosis and Treatment of Angiography Positive Medium to Large Vessel Childhood Primary Angiitis of Central Nervous System (p-cPACNS): An International Survey |
| title_fullStr | Diagnosis and Treatment of Angiography Positive Medium to Large Vessel Childhood Primary Angiitis of Central Nervous System (p-cPACNS): An International Survey |
| title_full_unstemmed | Diagnosis and Treatment of Angiography Positive Medium to Large Vessel Childhood Primary Angiitis of Central Nervous System (p-cPACNS): An International Survey |
| title_short | Diagnosis and Treatment of Angiography Positive Medium to Large Vessel Childhood Primary Angiitis of Central Nervous System (p-cPACNS): An International Survey |
| title_sort | diagnosis and treatment of angiography positive medium to large vessel childhood primary angiitis of central nervous system p cpacns an international survey |
| topic | vasculitis CNS childhood pediatric treatment diagnosis |
| url | https://www.frontiersin.org/articles/10.3389/fped.2021.654537/full |
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