Title :

Digestive outcomes in Cystic fibrosis.

Author(s) :

Ley, Delphine [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Turck, Dominique [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Journal title :

Best Practice and Research: Clinical Gastroenterology

Abbreviated title :

Best Pract Res Clin Gastroenterol

Volume number :

56-57

Pages :

101788

Publication date :

2022-03-30

ISSN :

1532-1916

English keyword(s) :

Cystic fibrosis
Pancreatic insufficiency
Distal intestinal obstruction syndrome
Colorectal cancer
CFTR modulators
Acute recurrent pancreatitis

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

Cystic fibrosis (CF) is the most frequent life-limiting autosomal recessive disease in Caucasians, affecting the respiratory tract, but also the pancreas, gut, and hepatobiliary tract. CF is caused by variants in the Cystic ...
Show more >Cystic fibrosis (CF) is the most frequent life-limiting autosomal recessive disease in Caucasians, affecting the respiratory tract, but also the pancreas, gut, and hepatobiliary tract. CF is caused by variants in the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene. Prognosis of CF has markedly improved over the last 20 years because of the management in CF centers and recent introduction of CFTR modulators, aimed at correcting the defective CFTR protein. There are nowadays more CF adults than children, with a predicted median survival age of around 50 years in high-income countries. Around 85% of CF patients have pancreatic insufficiency present at birth. Gastroesophageal reflux disease (GERD) is more frequent in CF patients, but its role on decline in lung health is controversial. Distal small bowel obstruction syndrome (DIOS) caused by meconium-like stool plugs occurs at any age after the neonatal period, affecting up to 15–20% of CF patients. Because of increased life expectancy, most CF patients are expected to live to their fifties or beyond, when cancer is more frequent. In addition, CF is associated with a higher risk for GI malignancy as compared with the general population. Colorectal cancer represents the most significant risk, and colonoscopy-based screening is recommended from 40 years of age onwards. Other digestive outcomes in CF reviewed in this paper include meconium ileus, Clostridium difficile infection, intussusception, acute appendicitis, small intestinal bacterial overgrowth, appendiceal mucocele and rectal prolapse. Every CF Center should comprise a gastroenterologist with expertise in the care of CF patients.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

Université de Lille
Inserm
CHU Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Submission date :

2024-01-12T02:00:20Z
2024-03-04T10:49:08Z