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Pulmonary hypertension, primary, fenfluramine or dexfenfluramine-associated

MedGen UID:
942109
Concept ID:
CN259073
Disease or Syndrome

Professional guidelines

PubMed

Ioannides-Demos LL, Proietto J, Tonkin AM, McNeil JJ
Drug Saf 2006;29(4):277-302. doi: 10.2165/00002018-200629040-00001. PMID: 16569079
Nisoli E, Carruba MO
Drug Saf 2003;26(14):1027-48. doi: 10.2165/00002018-200326140-00004. PMID: 14583064
Kolanowski J
Drug Saf 1999 Feb;20(2):119-31. doi: 10.2165/00002018-199920020-00003. PMID: 10082070

Recent clinical studies

Etiology

Knupp KG, Scheffer IE, Ceulemans B, Sullivan JE, Nickels KC, Lagae L, Guerrini R, Zuberi SM, Nabbout R, Riney K, Shore S, Agarwal A, Lock M, Farfel GM, Galer BS, Gammaitoni AR, Davis R, Gil-Nagel A
JAMA Neurol 2022 Jun 1;79(6):554-564. doi: 10.1001/jamaneurol.2022.0829. PMID: 35499850Free PMC Article
Nabbout R, Mistry A, Zuberi S, Villeneuve N, Gil-Nagel A, Sanchez-Carpintero R, Stephani U, Laux L, Wirrell E, Knupp K, Chiron C, Farfel G, Galer BS, Morrison G, Lock M, Agarwal A, Auvin S; FAiRE, DS Study Group
JAMA Neurol 2020 Mar 1;77(3):300-308. doi: 10.1001/jamaneurol.2019.4113. PMID: 31790543Free PMC Article
Lagae L, Sullivan J, Knupp K, Laux L, Polster T, Nikanorova M, Devinsky O, Cross JH, Guerrini R, Talwar D, Miller I, Farfel G, Galer BS, Gammaitoni A, Mistry A, Morrison G, Lock M, Agarwal A, Lai WW, Ceulemans B; FAiRE DS Study Group
Lancet 2019 Dec 21;394(10216):2243-2254. Epub 2019 Dec 17 doi: 10.1016/S0140-6736(19)32500-0. PMID: 31862249
Deitel M
Obes Surg 1997 Feb;7(1):3-4. doi: 10.1381/096089297765556114. PMID: 9730529
Sztuke-Fournier A
CMAJ 1997 Jan 1;156(1):89-90, 93-4. PMID: 9006574

Diagnosis

Knupp KG, Scheffer IE, Ceulemans B, Sullivan JE, Nickels KC, Lagae L, Guerrini R, Zuberi SM, Nabbout R, Riney K, Shore S, Agarwal A, Lock M, Farfel GM, Galer BS, Gammaitoni AR, Davis R, Gil-Nagel A
JAMA Neurol 2022 Jun 1;79(6):554-564. doi: 10.1001/jamaneurol.2022.0829. PMID: 35499850Free PMC Article
Nabbout R, Mistry A, Zuberi S, Villeneuve N, Gil-Nagel A, Sanchez-Carpintero R, Stephani U, Laux L, Wirrell E, Knupp K, Chiron C, Farfel G, Galer BS, Morrison G, Lock M, Agarwal A, Auvin S; FAiRE, DS Study Group
JAMA Neurol 2020 Mar 1;77(3):300-308. doi: 10.1001/jamaneurol.2019.4113. PMID: 31790543Free PMC Article
Souza R, Jardim C, Humbert M
Semin Respir Crit Care Med 2013 Oct;34(5):560-7. Epub 2013 Sep 13 doi: 10.1055/s-0033-1355439. PMID: 24037625
Michelakis ED, Weir EK
Am J Med Sci 2001 Apr;321(4):292-9. doi: 10.1097/00000441-200104000-00009. PMID: 11307870
Simonneau G, Fartoukh M, Sitbon O, Humbert M, Jagot JL, Hervé P
Chest 1998 Sep;114(3 Suppl):195S-199S. doi: 10.1378/chest.114.3_supplement.195s. PMID: 9741568

Therapy

Knupp KG, Scheffer IE, Ceulemans B, Sullivan JE, Nickels KC, Lagae L, Guerrini R, Zuberi SM, Nabbout R, Riney K, Shore S, Agarwal A, Lock M, Farfel GM, Galer BS, Gammaitoni AR, Davis R, Gil-Nagel A
JAMA Neurol 2022 Jun 1;79(6):554-564. doi: 10.1001/jamaneurol.2022.0829. PMID: 35499850Free PMC Article
Nabbout R, Mistry A, Zuberi S, Villeneuve N, Gil-Nagel A, Sanchez-Carpintero R, Stephani U, Laux L, Wirrell E, Knupp K, Chiron C, Farfel G, Galer BS, Morrison G, Lock M, Agarwal A, Auvin S; FAiRE, DS Study Group
JAMA Neurol 2020 Mar 1;77(3):300-308. doi: 10.1001/jamaneurol.2019.4113. PMID: 31790543Free PMC Article
Lagae L, Sullivan J, Knupp K, Laux L, Polster T, Nikanorova M, Devinsky O, Cross JH, Guerrini R, Talwar D, Miller I, Farfel G, Galer BS, Gammaitoni A, Mistry A, Morrison G, Lock M, Agarwal A, Lai WW, Ceulemans B; FAiRE DS Study Group
Lancet 2019 Dec 21;394(10216):2243-2254. Epub 2019 Dec 17 doi: 10.1016/S0140-6736(19)32500-0. PMID: 31862249
Haworth SG
J R Coll Physicians Lond 1998 May-Jun;32(3):187-90. PMID: 9670139Free PMC Article
Deitel M
Obes Surg 1997 Feb;7(1):3-4. doi: 10.1381/096089297765556114. PMID: 9730529

Prognosis

Patel S, Geenen KR, Dowless D, Bruno PL, Thiele EA
Dev Med Child Neurol 2023 Jul;65(7):961-967. Epub 2022 Dec 23 doi: 10.1111/dmcn.15492. PMID: 36562419
Humbert M, Deng Z, Simonneau G, Barst RJ, Sitbon O, Wolf M, Cuervo N, Moore KJ, Hodge SE, Knowles JA, Morse JH
Eur Respir J 2002 Sep;20(3):518-23. doi: 10.1183/09031936.02.01762002. PMID: 12358323
Archer SL, Djaballah K, Humbert M, Weir KE, Fartoukh M, Dall'ava-Santucci J, Mercier JC, Simonneau G, Dinh-Xuan AT
Am J Respir Crit Care Med 1998 Oct;158(4):1061-7. doi: 10.1164/ajrccm.158.4.9802113. PMID: 9769261
Simonneau G, Fartoukh M, Sitbon O, Humbert M, Jagot JL, Hervé P
Chest 1998 Sep;114(3 Suppl):195S-199S. doi: 10.1378/chest.114.3_supplement.195s. PMID: 9741568
Deitel M
Obes Surg 1997 Feb;7(1):3-4. doi: 10.1381/096089297765556114. PMID: 9730529

Clinical prediction guides

Knupp KG, Scheffer IE, Ceulemans B, Sullivan JE, Nickels KC, Lagae L, Guerrini R, Zuberi SM, Nabbout R, Riney K, Shore S, Agarwal A, Lock M, Farfel GM, Galer BS, Gammaitoni AR, Davis R, Gil-Nagel A
JAMA Neurol 2022 Jun 1;79(6):554-564. doi: 10.1001/jamaneurol.2022.0829. PMID: 35499850Free PMC Article
Geenen KR, Doshi SP, Patel S, Sourbron J, Falk A, Morgan A, Vu U, Bruno PL, Thiele EA
Dev Med Child Neurol 2021 Dec;63(12):1427-1432. Epub 2021 Jul 3 doi: 10.1111/dmcn.14965. PMID: 34216017
Lagae L, Sullivan J, Knupp K, Laux L, Polster T, Nikanorova M, Devinsky O, Cross JH, Guerrini R, Talwar D, Miller I, Farfel G, Galer BS, Gammaitoni A, Mistry A, Morrison G, Lock M, Agarwal A, Lai WW, Ceulemans B; FAiRE DS Study Group
Lancet 2019 Dec 21;394(10216):2243-2254. Epub 2019 Dec 17 doi: 10.1016/S0140-6736(19)32500-0. PMID: 31862249
Nisoli E, Carruba MO
Drug Saf 2003;26(14):1027-48. doi: 10.2165/00002018-200326140-00004. PMID: 14583064
Tellier P
Ann Med Interne (Paris) 2001 Nov;152(7):429-36. PMID: 11965083

Recent systematic reviews

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