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The Effects of Ivacaftor on Bone Density and Microarchitecture in Children and Adults with Cystic Fibrosis.

• Authors : Putman MS; Endocrine Unit, Department of Medicine, Massachusetts General Hospital, Boston, MA, USA.; Division of Endocrinology, Boston Children's Hospital, Boston, MA, USA.

Subjects: Cystic Fibrosis*/Cystic Fibrosis*/Cystic Fibrosis*/drug therapy ; Cystic Fibrosis*/Cystic Fibrosis*/Cystic Fibrosis*/genetics ; Cystic Fibrosis*/Cystic Fibrosis*/Cystic Fibrosis*/metabolism

• Source: The Journal of clinical endocrinology and metabolism [J Clin Endocrinol Metab] 2021 Mar 08; Vol. 106 (3), pp. e1248-e1261.Publisher: Oxford University Press Country of Publication: United States NLM ID: 0375362 Publication Model: Print Cited

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New Treatment for Cystic Fibrosis.

• Authors : Aschenbrenner DS; Diane S. Aschenbrenner is an assistant professor at Notre Dame of Maryland University in Baltimore. She also coordinates Drug Watch: .

Subjects: Aminophenols/Aminophenols/Aminophenols/*administration & dosage ; Benzodioxoles/Benzodioxoles/Benzodioxoles/*administration & dosage ; Cystic Fibrosis/Cystic Fibrosis/Cystic Fibrosis/*drug therapy

• Source: The American journal of nursing [Am J Nurs] 2020 Feb; Vol. 120 (2), pp. 21.Publisher: Lippincott Williams & Wilkins Country of Publication: United States NLM ID: 0372646 Publication Model: Print Cited

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Long-term safety and efficacy of lumacaftor-ivacaftor therapy in children aged 6-11 years with cystic fibrosis homozygous for the F508del-CFTR mutation: a phase 3, open-label, extension study.

• Authors : Chilvers MA; BC Children's Hospital, Vancouver, BC, Canada. Electronic address: .; Davies JC

Subjects: Aminophenols/Aminophenols/Aminophenols/*therapeutic use ; Aminopyridines/Aminopyridines/Aminopyridines/*therapeutic use ; Benzodioxoles/Benzodioxoles/Benzodioxoles/*therapeutic use

• Source: The Lancet. Respiratory medicine [Lancet Respir Med] 2021 Jul; Vol. 9 (7), pp. 721-732. Date of Electronic Publication: 2021 Jan 28.Publisher: Elsevier Country of Publication: England NLM ID: 101605555 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 2213-2619

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Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation.

• Authors : Guimbellot JS; GOAL-e2 Investigators

Subjects: Aminophenols/Aminophenols/Aminophenols/*therapeutic use ; Chloride Channel Agonists/Chloride Channel Agonists/Chloride Channel Agonists/*therapeutic use ; Cystic Fibrosis/Cystic Fibrosis/Cystic Fibrosis/*drug therapy

• Source: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society [J Cyst Fibros] 2021 Mar; Vol. 20 (2), pp. 213-219. Date of Electronic Publication: 2020 Nov Publisher: Elsevier Country of Publication: Netherlands NLM ID: 101128966 Publication Model: Print-Electronic Cited Medium: Internet ISSN:

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Changes in LCI in F508del/F508del patients treated with lumacaftor/ivacaftor: Results from the prospect study.

• Authors : Shaw M; Khan U ...

Subjects: Aminophenols/Aminophenols/Aminophenols/*therapeutic use ; Aminopyridines/Aminopyridines/Aminopyridines/*therapeutic use ; Benzodioxoles/Benzodioxoles/Benzodioxoles/*therapeutic use

• Source: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society [J Cyst Fibros] 2020 Nov; Vol. 19 (6), pp. 931-933. Date of Electronic Publication: 2020 Jun Publisher: Elsevier Country of Publication: Netherlands NLM ID: 101128966 Publication Model: Print-Electronic Cited Medium: Internet ISSN:

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Longitudinal changes in lung function following initiation of lumacaftor/ivacaftor combination.

• Authors : Loukou I; Cystic Fibrosis Department, 'Agia Sofia' Children's Hospital, Athens, Greece. Electronic address: .; Moustaki M

Subjects: Cystic Fibrosis*/Cystic Fibrosis*/Cystic Fibrosis*/diagnosis ; Cystic Fibrosis*/Cystic Fibrosis*/Cystic Fibrosis*/drug therapy ; Cystic Fibrosis*/Cystic Fibrosis*/Cystic Fibrosis*/genetics

• Source: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society [J Cyst Fibros] 2020 Jul; Vol. 19 (4), pp. 534-539. Date of Electronic Publication: 2019 Oct Publisher: Elsevier Country of Publication: Netherlands NLM ID: 101128966 Publication Model: Print-Electronic Cited Medium: Internet ISSN:

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Precision Medicine: At What Price?

• Authors : Ferkol T; 1 Washington University in St. Louis St. Louis, Missouri.; Quinton P

Subjects: Aminophenols/Aminophenols/Aminophenols/*economics ; Aminopyridines/Aminopyridines/Aminopyridines/*economics ; Benzodioxoles/Benzodioxoles/Benzodioxoles/*economics

• Source: American journal of respiratory and critical care medicine [Am J Respir Crit Care Med] 2015 Sep 15; Vol. 192 (6), pp. 658-9.Publisher: American Thoracic Society Country of Publication: United States NLM ID: 9421642 Publication Model: Print Cited

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New Drug Hailed as Major Breakthrough in Cystic Fibrosis.

Subjects: Drug Combinations*; Aminophenols/Aminophenols/Aminophenols/*therapeutic use ; Benzodioxoles/Benzodioxoles/Benzodioxoles/*therapeutic use

• Source: American journal of medical genetics. Part A [Am J Med Genet A] 2020 Jan; Vol. 182 (1), pp. 8-9.Publisher: Wiley-Blackwell Country of Publication: United States NLM ID: 101235741 Publication Model: Print Cited Medium:

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Disease progression in patients with cystic fibrosis treated with ivacaftor: Data from national US and UK registries.

• Authors : Volkova N; Vertex Pharmaceuticals Incorporated, 50 Northern Avenue, Boston, MA, USA. Electronic address: .; Moy K

Subjects: Cystic Fibrosis*/Cystic Fibrosis*/Cystic Fibrosis*/drug therapy ; Cystic Fibrosis*/Cystic Fibrosis*/Cystic Fibrosis*/epidemiology ; Cystic Fibrosis*/Cystic Fibrosis*/Cystic Fibrosis*/microbiology

• Source: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society [J Cyst Fibros] 2020 Jan; Vol. 19 (1), pp. 68-79. Date of Electronic Publication: 2019 Jun 10.Publisher: Elsevier Country of Publication: Netherlands NLM ID: 101128966 Publication Model: Print-Electronic Cited Medium: Internet ISSN:

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Elexacaftor/Ivacaftor/Tezacaftor: First Approval.

• Authors : Hoy SM; Springer Nature, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand. .

Subjects: Drug Approval*; Aminophenols/Aminophenols/Aminophenols/*pharmacology ; Benzodioxoles/Benzodioxoles/Benzodioxoles/*pharmacology

• Source: Drugs [Drugs] 2019 Dec; Vol. 79 (18), pp. 2001-2007.Publisher: Adis, Springer International Country of Publication: New Zealand NLM ID: 7600076 Publication Model: Print Cited Medium: Internet ISSN:

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