Radiological and long-term clinical response to elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis with advanced lung disease.

Aalbers, Bente L; Mohamed Hoesein, Firdaus A A; Hofland, Regina W; Bronsveld, Inez; Kruijswijk, Marian A; Schotman, Suzan; Slingerland, Corlien W; Panhuis, Hannah; van der Ent, C Kors; Heijerman, Harry G M

Aalbers, Bente L; Mohamed Hoesein, Firdaus A A; Hofland, Regina W; Bronsveld, Inez; Kruijswijk, Marian A; Schotman, Suzan; Slingerland, Corlien W; Panhuis, Hannah; van der Ent, C Kors; Heijerman, Harry G M.

Afiliação

Aalbers BL; Department of Pulmonology, UMC Utrecht, Utrecht University, Utrecht, The Netherlands.
Mohamed Hoesein FAA; Department of Radiology, UMC Utrecht, Utrecht University, Utrech, The Netherlands.
Hofland RW; Department of Pulmonology, UMC Utrecht, Utrecht University, Utrecht, The Netherlands.
Bronsveld I; Department of Pulmonology, UMC Utrecht, Utrecht University, Utrecht, The Netherlands.
Kruijswijk MA; Department of Pulmonology, UMC Utrecht, Utrecht University, Utrecht, The Netherlands.
Schotman S; Department of Pulmonology, UMC Utrecht, Utrecht University, Utrecht, The Netherlands.
Slingerland CW; Department of Pulmonology, UMC Utrecht, Utrecht University, Utrecht, The Netherlands.
Panhuis H; Department of Pediatric pulmonology, UMC Utrecht location Wilhelmina Childrens Hospital, Utrecht University, Utrech, The Netherlands.
van der Ent CK; Department of Pediatric pulmonology, UMC Utrecht location Wilhelmina Childrens Hospital, Utrecht University, Utrech, The Netherlands.
Heijerman HGM; Department of Pulmonology, UMC Utrecht, Utrecht University, Utrecht, The Netherlands.

Pediatr Pulmonol ; 58(8): 2317-2322, 2023 Aug.

Article em En | MEDLINE | ID: mdl-37222401

Assuntos

Fibrose Cística; Humanos; Aminofenóis/uso terapêutico; Benzodioxóis/uso terapêutico; Agonistas dos Canais de Cloreto/uso terapêutico; Fibrose Cística/complicações; Fibrose Cística/tratamento farmacológico; Fibrose Cística/genética; Regulador de Condutância Transmembrana em Fibrose Cística/genética; Regulador de Condutância Transmembrana em Fibrose Cística/uso terapêutico; Pulmão; Mutação; Qualidade de Vida

Palavras-chave

Brody score; CFTR modulators; chest CT; cystic fibrosis; quality of life

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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Fibrose Cística Limite: Humans Idioma: En Revista: Pediatr Pulmonol Assunto da revista: PEDIATRIA Ano de publicação: 2023 Tipo de documento: Article País de afiliação: Holanda

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