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}
}

Topics from this paper

The functional and physical relationship between the DRA bicarbonate transporter and carbonic anhydrase II.
TLDR
It is concluded that cytosolic CAII is required for full DRA-mediated bicarbonate transport, which differs from other bic carbonate transport proteins because its transport activity is not stimulated by direct interaction with CAII.
International prospective study of distal intestinal obstruction syndrome in cystic fibrosis: Associated factors and outcome.
TLDR
It is shown that delayed arrival at hospital after the initial symptoms causes significant morbidity and early recognition and treatment would improve the prognosis.
Characterization of a novel chemotactic factor for neutrophils in the bronchial secretions of patients with cystic fibrosis.
TLDR
It is reported that the chemotactic activity of this factor remained unaffected by a variety of physical treatments and could be distinguished from those of IL-8, formylmethionylleucylphenylalanine, leukotreine B4, and platelet-activating factor.
Pneumocystis jirovecii among patients with cystic fibrosis and their household members
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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.
The Mitochondrial Ca2+ import complex is altered in ADPKD.
TLDR
The goal here was to understand the role of components of the mitochondrial Ca2+ uptake complex in PC1-mutant cells in autosomal dominant polycystic kidney disease (ADPKD) and identify novel therapeutic targets for treating ADPKD.
Dried blood spot-based metabolomic profiling in adults with cystic fibrosis.
TLDR
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.
Nanostructure Drug Delivery System Is an Option to Solve Antimicrobial Drug Resistance
TLDR
The present chapter is focused on various aspects of nanostructureures used in antibacterial therapy, mainly recent advances in the research and applications of antimicrobial polymeric and other nanostructures.
A potential strategy for reducing cysts in autosomal dominant polycystic kidney disease with a CFTR corrector
TLDR
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.
The Q359K/T360K mutation causes cystic fibrosis in Georgian Jews.
TLDR
In this cohort, the Q359K/T360K mutation resulted in a severe CF phenotype, although with residual early CFTR function, and the CFTR2 database should consider defining this mutation as CF-causing.
Multisystem Imaging Findings of Cystic Fibrosis in Adults: Recognizing Typical and Atypical Patterns of Disease.
TLDR
The purpose of this article is to present the wide spectrum of CF in adults, including both classic and nonclassic variants, with an emphasis on the nonclassic imaging findings.
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