Catalogo Articoli (Spogli Riviste)

OPAC HELP

Titolo:
Gastrointestinal complications in adult patients with cystic fibrosis
Autore:
Bargon, J; Stein, J; Dietrich, CF; Muller, U; Caspary, WF; Wagner, TOF;
Indirizzi:
Univ Frankfurt Klinikum, Zentrum Innere Med, Med Klin 2, D-60590 Frankfurt, Germany Univ Frankfurt Klinikum Frankfurt Germany D-60590 590 Frankfurt, Germany
Titolo Testata:
ZEITSCHRIFT FUR GASTROENTEROLOGIE
fascicolo: 8, volume: 37, anno: 1999,
pagine: 739 - 749
SICI:
0044-2771(199908)37:8<739:GCIAPW>2.0.ZU;2-Q
Fonte:
ISI
Lingua:
GER
Soggetto:
TRANSMEMBRANE CONDUCTANCE REGULATOR; URSODEOXYCHOLIC ACID THERAPY; LIVER-DISEASE; EPITHELIAL-CELLS; CLOSTRIDIUM-DIFFICILE; PANCREATIC FUNCTION; GENE; CFTR; IDENTIFICATION; EXPRESSION;
Keywords:
cystic fibrosis; DIOS; GERD; CFTR; pancreatic insufficiency;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
50
Recensione:
Indirizzi per estratti:
Indirizzo: Bargon, J Univ Frankfurt, Zentrum Innere Med, Med Klin 2, Theodor Stern Kai 7, D-60590 Frankfurt, Germany Univ Frankfurt Theodor Stern Kai 7 Frankfurt Germany D-60590 ny
Citazione:
J. Bargon et al., "Gastrointestinal complications in adult patients with cystic fibrosis", Z GASTROENT, 37(8), 1999, pp. 739-749

Abstract

Cystic fibrosis (CF) is the most common autosomal recessive disease in theCaucasian population. The primary cellular defect, the reduced expression of the cystic fibrosis transmembrane conductance regulator (CFTR), leading to a chloride secretory defect, is present in all epithelial cells of endodermal and mesodermal origin and has been described in sweat glands, the airway epithelium and the small intestine, the colon and rectum, including thepancreas. In the upper GI-tract the most troublesome complaints and symptoms originating from the esophagus are peptic esophagitis or esophageal varices. In the small intestinal wall, the clinical expression of CF largely depends on the decreased secretion of fluid and chloride ions and the sticky mucous covering the enterocytes. Although CFTR expression in the colon is lower, the large intestine may be the site of several serious complications such as rectal prolapse, distal intestinal obstruction (DIOS), and intussusception. in recent years an increase in colonic strictures after the use of high-dosepancreatic enzymes, has been increasingly reported. CF has also been reported to be increasingly associated with a number of hepatic and/or biliary abnormalities? of which chronic cholestatic liver disease is by far the mostrelevant. Plugging of intrahepatic bile ducts with inspissated secretions is thought to play a major role in the pathogenesis. It has been estimated that about 15% of cystic fibrosis patients reveal serum liver enzyme abnormalities, but prevalence of liver involvement is likely to be higher. Oral bile acid therapy is promising, but its long-term benefits in terms of survival and prevention of major complications by liver cirrhosis remain to be established. Pancreatic dysfunction in cystic fibrosis (CF) is characterizedby an insufficient pancreatic exocrine function. However, 10-15% of CF patients have pancreatic sufficiency and this status is genetically determinedby one or two >>mild<< mutations in the CFTR.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 02/12/20 alle ore 14:59:17