Cystic fibrosis

@article{Rosenstein1998CysticF,
  title={Cystic fibrosis},
  author={Beryl J. Rosenstein and Pamela L Zeitlin},
  journal={The Lancet},
  year={1998},
  volume={351},
  pages={277-282},
  url={https://api.semanticscholar.org/CorpusID:44627706}
}
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16 Citations
Background Citations
5

16 Citations

Inhaled anti-pseudomonal antibiotics for long-term therapy in cystic fibrosis.

It is found that inhaled antibiotics may improve lung function measured in a variety of ways and reduce the frequency of exacerbations and quality of life and adverse events (including drug-sensitivity reactions and survival).

Nebulised anti-pseudomonal antibiotics for cystic fibrosis.

Nebulised anti-pseudomonal antibiotic treatment in people with CF reduces frequency of exacerbations of infection, improves lung function, quality of life and survival and indicates the significance of development of antibiotic resistant organisms is still unclear.

Pneumocystis jirovecii among patients with cystic fibrosis and their household members

This study suggests for the first time the possible transmission of Pneumocystis in the family environment of patients with cystic fibrosis, indicating that patients and their household members are reservoirs and possible sources of this infection.

Abnormal functional lymphoid tolerance and enhanced myeloid exocytosis are characteristics of resting and stimulated PBMCs in cystic fibrosis patients

It is demonstrated that functional lymphoid tolerance and enhanced myeloid protease activity are key features of cystic fibrosis PBMCs.

The impact of MRSA infection in the airways of children with cystic fibrosis; a case-control study.

It is suggested that persistent MRSA infection in the airways of children with CF is associated with diminished lung function two years post acquisition, when compared to a matched control cohort without MRSA.

A potential strategy for reducing cysts in autosomal dominant polycystic kidney disease with a CFTR corrector

A role for VX-809, a corrector of cystic fibrosis transmembrane conductance regulator (CFTR), conventionally used to manage cystic Fibrosis in reducing renal cyst growth is demonstrated and this drug potentially offers a new way to treat patients with ADPKD.

CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR GENE MUTATIONS IN PATIENTS WITH MASSIVE NASAL POLYPOSIS

In massive nasal polyposis patients, CFTR gene mutations are not common and can be seen without classical cystic fibrosis clinical symptomps.

Dried blood spot-based metabolomic profiling in adults with cystic fibrosis.

The osmolyte (sorbitol) was remarkably down-regulated in CF patients compared to healthy controls indicating perturbation in the sorbitol pathway which may be responsible for the mucoviscidosis seen in patients with CF.

Exercise Capacity in Prepubertal Children with Cystic Fibrosis

In this group of prepubertal children with CF, nutritional status was comparable to healthy children of the same age and their aerobic exercise tolerance and peripheral muscle strength were relatively well preserved despite significantly lower lung function.

Prevalence and impact on FEV 1 decline of chronic methicillin-resistant Staphylococcus aureus ( MRSA ) colonization in patients with Cystic Fibrosis

It is shown that a MRSA episode in CF patients, chronically colonized with S. aureus, entails an FEV1 decline that is almost double that predicted for CF patients who can remain unaffected by MRSA.

3 References

Cystic Fibrosis

This issue discusses anxiety and depression and their impacts on disease outcomes and adherence to therapy, CFrelated diabetes, which differs from types 1 and 2 diabetes, and bone disease such as osteopenia and osteoporosis are more prevalent in CF patients than in the general population.

Update: cystic fibrosis.