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At least 847 records · Page 47Linked to original sources
PubMed1981

Intrapancreatic common bile duct compression causing jaundice in an adult with cystic fibrosis.

A 29-yr-old man with cystic fibrosis had a 4-yr history of recurrent episodes of obstructive jaundice. Endoscopic retrograde cholangiography revealed a common bile duct smoothly narrowed in its intrapancreatic portion, with dilatation above. A choledochojejunostomy was performed, but the patient died of septic complications. At autopsy, extensive pancreatic fibrosis caused compression of the distal bile duct in a manner which is analogous to that seen in chronic pancreatitis. This complication of cystic fibrosis, not previously reported, may become more prevalent as more patients with cystic fibrosis are living into adulthood.

Adult↗
PubMed1981

Pancreatic sonography in cystic fibrosis.

Sonographic examination of the abdomen was performed in 20 patients with cystic fibrosis aged 3--41 years. The predominant acoustic architecture of the pancreas was one of moderate to markedly increased echogenicity when compared to the liver at the same depth. Increased echogenicity of the pancreas was independent of the presence of malabsorption. The usual reduced echo pattern associated with pancreatic inflammation was seen in only one of five cystic fibrosis patients with clinical evidence of pancreatitis. Other pancreatic abnormalities included pancreatic calcifications in one child with pancreatitis and a pseudocyst in an adult with pancreatitis. Eight patients had hepatosplenomegaly and gallstones were seen in two; one had a markedly contracted gallbladder. Sonography offers an effective method of following patients with cystic fibrosis.

Adolescent↗
PubMedDate not supplied

[Cystic fibrosis--a disease with many faces. The variable clinical picture versus the heterogeneity of molecular defect].

Cystic fibrosis (CF) is the most common genetic disorder in the Caucasian population with an autosomal recessive mode of inheritance. The disease is caused by mutations in the CFTR gene. So far, over 1300 of them have been identified. Primarily, the disease affects the epithelial cells of the airways, pancreas, intestines, gall bladder and sweat glands. However, cystic fibrosis is a clinically heterogeneous disorder. Especially the respiratory symptoms are significantly variable, even among patients with the same CFTR genotype. Therefore, there is an increasing interest in searching for the genetic modifiers of the clinical outcome in CF. This review presents the actual state of knowledge in terms of the potential genes-modifiers of the pulmonary form of cystic fibrosis.

Cystic Fibrosis↗
PubMed2026

Research updates in cystic fibrosis related diabetes: Understanding pathophysiology, expanding animal and human islet models, and advancing clinical and translational research.

In 2024-2025, the Cystic Fibrosis Foundation (US) and Cystic Fibrosis Trust (UK) hosted an International CFRD Consortium round-table webinar series for basic science, translational, and clinical researchers with the goal of sharpening mechanistic understanding of CFRD pathogenesis and prioritizing therapeutic development. This review summarizes the research priorities identified in the International CFRD Consortium, including (i) further investigation into the role of pancreatic fibrosis, vascular abnormalities, and α-cell dysfunction in the development of CFRD; (ii) the creation and refinement of novel animal and human cell- and tissue-based models to understand the complex interplay of exocrine and endocrine cells in the CF pancreas; (iii) development and validation of circulating and imaging biomarkers, together with dynamic glucose testing to explore β-cell function and kinetics in people with CF across the dysglycemia spectrum; and (iv) prospective clinical studies to guide CFRD treatment options and investigate the changing landscape of aging, increasing prevalence of obesity and diabetes and their complications in the era of cystic fibrosis transmembrane conductance regulator (CFTR) modulators. Collectively, these priorities aim to accelerate transition from mechanism to intervention and expand evidence-based care for people with CF at risk of, or living with, CFRD.

Humans↗
PubMed1999

Cationic lipid:pDNA complexes for the treatment of cystic fibrosis.

Cystic fibrosis (CF) is an autosomal recessive disease that results in damage to organs containing secretory epithelial cells, such as the lung, intestines, pancreas and liver. CF results from mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR), which functions as a chloride channel in the apical membrane of secretory epithelial cells. In the lung, the loss of CFTR activity results in abnormal epithelial ion transport, including defective cyclic AMP-mediated chloride (Cl-) efflux and the hyper absorption of sodium ions. These defects in ion transport affect the osmotic flow of water across the epithelium and result in thickened airway secretions which are not properly cleared from the lung. Patients with CF suffer from chronic pathogenic infection of the lungs which in turn results in the formation of purulent mucus, chronic inflammation and fibrosis, which eventually cause a loss of lung function. One approach to treat CF is to replace the defective copy of the gene encoding for CFTR with a correct copy employing one of a number of possible gene therapy vectors. This review summarizes the most recent progress towards gene therapy for CF employing cationic lipid:pDNA complexes. The results of recent clinical trials conducted in the nasal epithelium of CF patients are presented, followed by a description of preclinical studies conducted in order to support trials of aerosolized lipid:pDNA complexes into the lungs of CF patients. A brief overview of the results of these aerosol trials is presented, followed by a discussion of the lessons learned from these studies. More specifically, the issues of efficacy and toxicity of cationic lipid:pDNA complexes in the setting of the CF lung are discussed and recent findings which are relevant to these limiting issues are introduced. Finally, studies which point to the possibility of improved formulations capable of overcoming some of the barriers identified for the current generation of cationic lipid:pDNA complexes are introduced.

Cations↗
PubMed2003

The phenotypic consequences of CFTR mutations.

Cystic fibrosis is a common autosomal recessive disorder that primarily affects the epithelial cells in the intestine, respiratory system, pancreas, gall bladder and sweat glands. Over one thousand mutations have currently been identified in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene that are associated with CF disease. There have been many studies on the correlation of the CFTR genotype and CF disease phenotype; however, this relationship is still not well understood. A connection between CFTR genotype and disease manifested in the pancreas has been well described, but pulmonary disease appears to be highly variable even between individuals with the same genotype. This review describes the current classification of CFTR mutation classes and resulting CF disease phenotypes. Complex disease alleles and modifier genes are discussed along with alternative disorders, such as disseminated bronchiectasis and pancreatitis, which are also thought to result from CFTR mutations.

Cystic Fibrosis↗