Cystic fibrosis: insight into CFTR pathophysiology and pharmacotherapy

B Lubamba, B Dhooghe, S Noel, T Leal - Clinical biochemistry, 2012 - Elsevier
Cystic fibrosis is the most common life-threatening recessively inherited disease in
Caucasians. Due to early provision of care in specialized reference centers and more …

[HTML][HTML] Bacterial overgrowth, dysbiosis, inflammation, and dysmotility in the Cystic Fibrosis intestine

J Dorsey, T Gonska - Journal of Cystic Fibrosis, 2017 - Elsevier
Abstract Gastrointestinal disease in Cystic Fibrosis (CF) is caused by defective chloride and
bicarbonate transport in intestinal cells leading to reduced intraluminal fluidity, increased …

Multicenter prospective study showing a high gastrointestinal symptom burden in cystic fibrosis

B Moshiree, AJ Freeman, PT Vu, U Khan… - Journal of Cystic …, 2023 - Elsevier
Background and aims People with cystic fibrosis (PwCF) suffer from gastrointestinal (GI)
symptoms affecting their quality of life (QOL). Despite the relevance of GI symptoms to the …

Gastrointestinal complications of cystic fibrosis

D Gelfond, D Borowitz - Clinical Gastroenterology and Hepatology, 2013 - Elsevier
The cystic fibrosis transmembrane regulator protein (CFTR) is an ion channel in the apical
surface of epithelial membranes that regulates other ion channels. Dysfunction of CFTR …

The PACSYM questionnaire for chronic constipation: defining the minimal important difference

Y Yiannakou, J Tack, H Piessevaux… - Alimentary …, 2017 - Wiley Online Library
Summary Background The Patient Assessment of ConstipationSymptoms (PACSYM)
questionnaire is frequently used in clinical trials of constipation. However, the threshold for …

[HTML][HTML] Cystic fibrosis & disorders of the large intestine: DIOS, constipation, and colorectal cancer

JM Abraham, CJ Taylor - Journal of Cystic Fibrosis, 2017 - Elsevier
Abstract Since 1966 when the Cystic Fibrosis Foundation Patient Registry (CFFPR) was
founded, clinicians have witnessed significant advances in both the quality and quantity of …

Gastrointestinal disorders in cystic fibrosis

DN Assis, SD Freedman - Clinics in chest medicine, 2016 - chestmed.theclinics.com
Gastrointestinal (GI) abnormalities are integral to the clinical manifestations of cystic fibrosis
(CF). Indeed, the very first publication of CF in 1938 reported pediatric steatorrhea with …

Inhibition of Na+/H+ exchanger isoform 3 improves gut fluidity and alkalinity in cystic fibrosis transmembrane conductance regulatordeficient and F508del mutant …

Q Tan, G di Stefano, X Tan, X Renjie… - British Journal of …, 2021 - Wiley Online Library
Background and Purpose Constipation and intestinal obstructive episodes are major health
problems in cystic fibrosis (CF) patients. Three FDAapproved drugs against constipation …

Interventions for preventing distal intestinal obstruction syndrome (DIOS) in cystic fibrosis

J Green, FJ Gilchrist, W Carroll - Cochrane Database of …, 2018 - cochranelibrary.com
Background Cystic fibrosis (CF) is the most common, lifelimiting, genetically inherited
disease. It affects multiple organs, particularly the respiratory system. However …

Lubiprostone in constipation: clinical evidence and place in therapy

N Wilson, R Schey - Therapeutic advances in chronic …, 2015 - journals.sagepub.com
Constipation is one of the most common function bowel disorders encountered by primary
care providers and gastroenterologists. Disorders of chronic constipation, including irritable …