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At least 991 records · Page 55Linked to original sources
PubMed1993

Localization of expression of the cystic fibrosis gene in human pancreatic development.

Cystic fibrosis (CF) is a disease that affects the function of specialized epithelial cells within the lungs, gut, pancreas, sweat glands, and male genital ducts. Following the cloning of the CF gene, it is possible to examine tissue-specific expression of this gene. To investigate the onset of the CF disease process, expression of the CF gene in midtrimester human fetal tissues has been analyzed by in situ hybridization using antisense RNA probes. The major site of CF gene expression at this stage of development is in the pancreas. Within the pancreas, CF mRNA is seen to be largely restricted to the epithelium of intralobular and small interlobular ducts.

Cystic Fibrosis↗
PubMedDate not supplied

The physical, psychological, and social implications of caring for the pregnant patient and newborn with cystic fibrosis.

Cystic fibrosis (CF) involves the exocrine glands and epithelial tissues of the pancreas, sweat glands, and mucus glands in the respiratory, digestive, and reproductive tracts. Significant pulmonary disease, poor nutritional status, and pulmonary hypertension are results of chronic infections. When the woman with CF becomes pregnant, her body may find it difficult to adjust to the normal physiological changes of pregnancy, with the pulmonary, cardiovascular, and nutritional status being particularly stressed. Both the mother and the fetus are at a significant risk for increased morbidity and mortality. The woman whose life and pregnancy are compromised by CF and/or the newborn who is diagnosed with CF offer the nurse an extremely challenging situation--one in which physical and psychological care are proportional in importance. As part of the healthcare team, which includes a perinatologist, a pulmonary medicine specialist, a neonatologist, an anesthesiologist, a geneticist, a dietitian, a respiratory therapist, a social worker, an ethicist, and perhaps a clergyman, the nurse helps to coordinate and carry out the care needed for a successful outcome.

Cystic Fibrosis↗
PubMed1988

[Specificity and sensitivity of the 4-N-acetyl-L-tyrosyl-p- aminobenzoic acid test in childhood with serum p-aminobenzoic acid determination].

Specificity and sensitivity of ALTAB test have been estimated in 40 healthy children and 13 cystic fibrosis patients. The test has been carried out in the modification as 2-hour-serum PABA-test. The found specificity was 90%, the sensitivity 100% respectively. Therefore this test is suitable for evaluation of exocrine pancreas function.

4-Aminobenzoic Acid↗
PubMed1995

Two cystic fibrosis transmembrane conductance regulator mutations have different effects on both pulmonary phenotype and regulation of outwardly rectified chloride currents.

Cystic fibrosis (CF), a disorder of electrolyte transport manifest in the lungs, pancreas, sweat duct, and vas deferens, is caused by mutations in the CF transmembrane conductance regulator (CFTR). The CFTR protein has been shown to function as a cAMP-activated chloride channel and also regulates a separate protein, the outwardly rectifying chloride channel (ORCC). To determine the consequence of disease-producing mutations upon these functions, mutant CFTR was transiently expressed in Xenopus oocytes and in human airway epithelial cells lacking functional CFTR. Both G551D, a mutation that causes severe lung disease, and A455E, a mutation associated with mild lung disease, altered but did not abolish CFTR's function as a chloride channel in Xenopus oocytes. Airway epithelial cells transfected with CFTR bearing either A455E or G551D had levels of chloride conductance significantly greater than those of mock-transfected and lower than those of wild-type CFTR-transfected cells, as measured by chloride efflux. A combination of channel blockers and analysis of current-voltage relationships were used to dissect the contribution of CFTR and the ORCC to whole cell currents of transfected cells. While CFTR bearing either mutation could function as a chloride channel, only CFTR bearing A455E retained the function of regulating the ORCC. These results indicate that CF mutations can affect CFTR functions differently and suggest that severity of pulmonary disease may be more closely associated with the regulatory rather than chloride channel function of CFTR.

Animals↗
PubMed2003

[Cystic fibrosis transmembrane conductance regulator (CFTR) gene: mutations and clinical phenotypes].

Cystic fibrosis (CF) is caused by mutation in the CFTR (cystic fibrosis transmembrane conductance regulator) gene. CF is characterised by chronic lung infections, pancreas insufficiency and, in males, congenital bilateral absence of the vas deferens (CBAVD). Furthermore, mutations in the CFTR are associated with several isolated, CF-related symptoms such as chronic lung diseases, CBAVD idiopathic pancreatitis and asthma. These patients have a higher frequency of CFTR mutations than unaffected individuals. The mutations found are not typical for the CF patients and are classified as mild mutations. One of these mutations (IVS8-5T) is frequently found in patients with the CF-related diseases, and in particular in patients with CBAVD. When a CFTR mutation is identified, genetic counselling and a mutation analysis should be offered to the relevant family members.

Cystic Fibrosis↗