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Pulmonary arterial hypertension related to hereditary hemorrhagic telangiectasia

MedGen UID:
318651
Concept ID:
C1832529
Disease or Syndrome
Synonym: PULMONARY ARTERIAL HYPERTENSION, HEREDITARY HEMORRHAGIC TELANGIECTASIA-RELATED
 
OMIM®: 600376; 601284

Recent clinical studies

Etiology

Wits M, Becher C, de Man F, Sanchez-Duffhues G, Goumans MJ
Cardiovasc Res 2023 Oct 24;119(13):2262-2277. doi: 10.1093/cvr/cvad129. PMID: 37595264Free PMC Article
Bloom PP, Rodriguez-Lopez J, Witkin AS, Al-Samkari H, Kuter DJ, Mojtahed A, Luther J
Clin Transl Gastroenterol 2020 Jan;11(1):e00118. doi: 10.14309/ctg.0000000000000118. PMID: 31977452Free PMC Article
Circo S, Gossage JR
Curr Opin Pulm Med 2014 Sep;20(5):421-8. doi: 10.1097/MCP.0000000000000076. PMID: 25032812
Cottin V, Dupuis-Girod S, Lesca G, Cordier JF
Respiration 2007;74(4):361-78. doi: 10.1159/000103205. PMID: 17641482
Newman JH, Trembath RC, Morse JA, Grunig E, Loyd JE, Adnot S, Coccolo F, Ventura C, Phillips JA 3rd, Knowles JA, Janssen B, Eickelberg O, Eddahibi S, Herve P, Nichols WC, Elliott G
J Am Coll Cardiol 2004 Jun 16;43(12 Suppl S):33S-39S. doi: 10.1016/j.jacc.2004.02.028. PMID: 15194176

Diagnosis

Scarpato BM, McDonald J, Bayrak-Toydemir P, Elliott CG, Cahill BC, Emerson LL, Keenan LM
Chest 2023 May;163(5):e201-e205. doi: 10.1016/j.chest.2023.01.020. PMID: 37164583Free PMC Article
Dupuis-Girod S, Cottin V, Shovlin CL
Respiration 2017;94(4):315-330. Epub 2017 Aug 30 doi: 10.1159/000479632. PMID: 28850955
Circo S, Gossage JR
Curr Opin Pulm Med 2014 Sep;20(5):421-8. doi: 10.1097/MCP.0000000000000076. PMID: 25032812
Brenner L, Chung WK
Compr Physiol 2011 Oct;1(4):1721-8. doi: 10.1002/cphy.c100063. PMID: 23733703
Austin ED, Loyd JE
Clin Chest Med 2007 Mar;28(1):43-57, vii-viii. doi: 10.1016/j.ccm.2006.11.007. PMID: 17338927Free PMC Article

Therapy

Awdish RL, Mendez MP, MacDonald N, Yessayan L, Jennings JH, Albujoq K, Hegab S, Cajigas HR
J Healthc Qual 2017 Sep/Oct;39(5):e84-e90. doi: 10.1097/JHQ.0000000000000053. PMID: 27631708
Miyake R, Fujino T, Abe K, Hosokawa K, Ohtani K, Morisaki H, Yamada O, Higo T, Ide T
Int J Cardiol 2016 Jul 1;214:275-6. Epub 2016 Mar 31 doi: 10.1016/j.ijcard.2016.03.211. PMID: 27077548
Wang G, Fan R, Ji R, Zou W, Penny DJ, Varghese NP, Fan Y
BMC Pulm Med 2016 Jan 22;16:17. doi: 10.1186/s12890-016-0183-7. PMID: 26801773Free PMC Article
Amann A, Steiner N, Gunsilius E
Wien Klin Wochenschr 2015 Aug;127(15-16):631-4. Epub 2015 May 19 doi: 10.1007/s00508-015-0789-4. PMID: 25986996
Chaouat A, Coulet F, Favre C, Simonneau G, Weitzenblum E, Soubrier F, Humbert M
Thorax 2004 May;59(5):446-8. doi: 10.1136/thx.2003.11890. PMID: 15115879Free PMC Article

Prognosis

Bloom PP, Rodriguez-Lopez J, Witkin AS, Al-Samkari H, Kuter DJ, Mojtahed A, Luther J
Clin Transl Gastroenterol 2020 Jan;11(1):e00118. doi: 10.14309/ctg.0000000000000118. PMID: 31977452Free PMC Article
Sztrymf B, Yaïci A, Jaïs X, Sitbon O, Simonneau G, Humbert M
Sarcoidosis Vasc Diffuse Lung Dis 2005 Dec;22 Suppl 1:S91-100. PMID: 16457021
Harrison RE, Flanagan JA, Sankelo M, Abdalla SA, Rowell J, Machado RD, Elliott CG, Robbins IM, Olschewski H, McLaughlin V, Gruenig E, Kermeen F, Halme M, Räisänen-Sokolowski A, Laitinen T, Morrell NW, Trembath RC
J Med Genet 2003 Dec;40(12):865-71. doi: 10.1136/jmg.40.12.865. PMID: 14684682Free PMC Article

Clinical prediction guides

Bloom PP, Rodriguez-Lopez J, Witkin AS, Al-Samkari H, Kuter DJ, Mojtahed A, Luther J
Clin Transl Gastroenterol 2020 Jan;11(1):e00118. doi: 10.14309/ctg.0000000000000118. PMID: 31977452Free PMC Article
Lafyatis R
Nat Rev Rheumatol 2014 Dec;10(12):706-19. Epub 2014 Aug 19 doi: 10.1038/nrrheum.2014.137. PMID: 25136781
Sztrymf B, Yaïci A, Jaïs X, Sitbon O, Simonneau G, Humbert M
Sarcoidosis Vasc Diffuse Lung Dis 2005 Dec;22 Suppl 1:S91-100. PMID: 16457021
Harrison RE, Flanagan JA, Sankelo M, Abdalla SA, Rowell J, Machado RD, Elliott CG, Robbins IM, Olschewski H, McLaughlin V, Gruenig E, Kermeen F, Halme M, Räisänen-Sokolowski A, Laitinen T, Morrell NW, Trembath RC
J Med Genet 2003 Dec;40(12):865-71. doi: 10.1136/jmg.40.12.865. PMID: 14684682Free PMC Article
Humbert M, Trembath RC
Eur Respir J 2002 Sep;20(3):741-9. doi: 10.1183/09031936.02.02702002. PMID: 12358355

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