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@article{Faughnan:2020:10.7326/M20-1443,
author = {Faughnan, ME and Mager, JJ and Hetts, SW and Palda, VA and Lang-Robertson, K and Buscarini, E and Deslandres, E and Kasthuri, RS and Lausman, A and Poetker, D and Ratjen, F and Chesnutt, MS and Clancy, M and Whitehead, KJ and Al-Samkari, H and Chakinala, M and Conrad, M and Cortes, D and Crocione, C and Darling, J and de, Gussem E and Derksen, C and Dupuis-Girod, S and Foy, P and Geisthoff, U and Gossage, JR and Hammill, A and Heimdal, K and Henderson, K and Iyer, VN and Kjeldsen, AD and Komiyama, M and Korenblatt, K and McDonald, J and McMahon, J and McWilliams, J and Meek, ME and Mei-Zahav, M and Olitsky, S and Palmer, S and Pantalone, R and Piccirillo, JF and Plahn, B and Porteous, MEM and Post, MC and Radovanovic, I and Rochon, PJ and Rodriguez-Lopez, J and Sabba, C and Serra, M and Shovlin, C and Sprecher, D and White, AJ and Winship, I and Zarrabeitia, R},
doi = {10.7326/M20-1443},
journal = {Annals of Internal Medicine},
pages = {1--16},
title = {Second international guidelines for the diagnosis and management of hereditary hemorrhagic telangiectasia.},
url = {http://dx.doi.org/10.7326/M20-1443},
year = {2020}
}

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TY  - JOUR
AB  - DESCRIPTION: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease with an estimated prevalence of 1 in 5000 that is characterized by the presence of vascular malformations (VMs). These result in chronic bleeding, acute hemorrhage, and complications from shunting through VMs. The goal of the Second International HHT Guidelines process was to develop evidence-based consensus guidelines for the management and prevention of HHT-related symptoms and complications. METHODS: The guidelines were developed using the AGREE II (Appraisal of Guidelines for Research and Evaluation II) framework and GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodology. The guidelines expert panel included expert physicians (clinical and genetic) in HHT from 15 countries, guidelines methodologists, health care workers, health care administrators, patient advocacy representatives, and persons with HHT. During the preconference process, the expert panel generated clinically relevant questions in 6 priority topic areas. A systematic literature search was done in June 2019, and articles meeting a priori criteria were included to generate evidence tables, which were used as the basis for recommendation development. The expert panel subsequently convened during a guidelines conference to conduct a structured consensus process, during which recommendations reaching at least 80% consensus were discussed and approved. RECOMMENDATIONS: The expert panel generated and approved 6 new recommendations for each of the following 6 priority topic areas: epistaxis, gastrointestinal bleeding, anemia and iron deficiency, liver VMs, pediatric care, and pregnancy and delivery (36 total). The recommendations highlight new evidence in existing topics from the first International HHT Guidelines and provide guidance in 3 new areas: anemia, pediatrics, and pregnancy and delivery. These recommendations should facilitate implementation of key components of HHT care into
AU  - Faughnan,ME
AU  - Mager,JJ
AU  - Hetts,SW
AU  - Palda,VA
AU  - Lang-Robertson,K
AU  - Buscarini,E
AU  - Deslandres,E
AU  - Kasthuri,RS
AU  - Lausman,A
AU  - Poetker,D
AU  - Ratjen,F
AU  - Chesnutt,MS
AU  - Clancy,M
AU  - Whitehead,KJ
AU  - Al-Samkari,H
AU  - Chakinala,M
AU  - Conrad,M
AU  - Cortes,D
AU  - Crocione,C
AU  - Darling,J
AU  - de,Gussem E
AU  - Derksen,C
AU  - Dupuis-Girod,S
AU  - Foy,P
AU  - Geisthoff,U
AU  - Gossage,JR
AU  - Hammill,A
AU  - Heimdal,K
AU  - Henderson,K
AU  - Iyer,VN
AU  - Kjeldsen,AD
AU  - Komiyama,M
AU  - Korenblatt,K
AU  - McDonald,J
AU  - McMahon,J
AU  - McWilliams,J
AU  - Meek,ME
AU  - Mei-Zahav,M
AU  - Olitsky,S
AU  - Palmer,S
AU  - Pantalone,R
AU  - Piccirillo,JF
AU  - Plahn,B
AU  - Porteous,MEM
AU  - Post,MC
AU  - Radovanovic,I
AU  - Rochon,PJ
AU  - Rodriguez-Lopez,J
AU  - Sabba,C
AU  - Serra,M
AU  - Shovlin,C
AU  - Sprecher,D
AU  - White,AJ
AU  - Winship,I
AU  - Zarrabeitia,R
DO  - 10.7326/M20-1443
EP  - 16
PY  - 2020///
SN  - 0003-4819
SP  - 1
TI  - Second international guidelines for the diagnosis and management of hereditary hemorrhagic telangiectasia.
T2  - Annals of Internal Medicine
UR  - http://dx.doi.org/10.7326/M20-1443
UR  - https://www.ncbi.nlm.nih.gov/pubmed/32894695
UR  - https://www.acpjournals.org/doi/10.7326/M20-1443
ER  -

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Claire L. Shovlin PhD FRCP

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