Cystic fibrosis and exocrine pancreatic insufficiency

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00843989%3A_____%2F19%3AE0107994" target="_blank" >RIV/00843989:_____/19:E0107994 - isvavai.cz</a>

Alternative codes found

RIV/00216224:14110/19:00110723 RIV/65269705:_____/19:00071729

Result on the web

<a href="http://www.csgh.info/en/article/cystic-fibrosis-and-exocrine-pancreatic-insufficiency-" target="_blank" >http://www.csgh.info/en/article/cystic-fibrosis-and-exocrine-pancreatic-insufficiency-</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.14735/amgh2019303" target="_blank" >10.14735/amgh2019303</a>

Result language

angličtina

Original language name

Cystic fibrosis and exocrine pancreatic insufficiency

Original language description

Cystic fibrosis (CF) is a genetic disease affecting many organs, including the gastrointestinal tract. While the pulmonary damage is the most life threatening, the pancreas is one of the first organs affected by CF and one of the most strongly affected. Mutation in the CF transmembrane conductance regulator (CFTR) gene results in a reduced volume of pancreatic juice and hyperconcentration of macromolecules leading to precipitation in the duct lumina, causing obstruction and damage. The clinical presentation of individual cases depends on a combination of different CFTR mutations, the potential presence of modifier gene mutations and environmental factors. CFTR mutations are historically divided into 5 classes – severe mutations (classes 1–3) and mild mutations (classes 4–5). The CFTR functional status depends on the combined effects of both CFTR allels and the severity of the phenotype depends on the milder mutation. The majority of CF patients exhibit exocrine pancreatic insufficiency in early childhood because functional acinar tissue has been lost in utero or soon after birth. These patients rarely suffer from pancreatic complications such as recurrent acute pancreatitis and/or chronic pancreatitis which, however, can occur in the minority of patients who possess residual pancreatic exocrine function. CFTR mutations are found more frequently in idiopathic and alcoholic chronic pancreatitis but the data is conflicting. A combination with serine protease inhibitor Kazal-type 1 (SPINK-1) mutations can be found in the idiopathic chronic pancreatitis group, as well as the presence of environmental factors. Malnutrition is directly related to a worse prognosis of CF patients and the delivery of active digestive enzymes is a cornerstone of treatment, with acid supression and vitamin supplementation playing an important additional role.

Czech name

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Czech description

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Type

J_SC - Article in a specialist periodical, which is included in the SCOPUS database

CEP classification

—

OECD FORD branch

30219 - Gastroenterology and hepatology

Project

—

Continuities

V - Vyzkumna aktivita podporovana z jinych verejnych zdroju

Publication year

2019

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Gastroenterologie a hepatologie

ISSN

1804-7874

e-ISSN

1804-803X

Volume of the periodical

73

Issue of the periodical within the volume

4

Country of publishing house

CZ - CZECH REPUBLIC

Number of pages

5

Pages from-to

303-307

UT code for WoS article

—

EID of the result in the Scopus database

999