Cystic Fibrosis Research Today is a free monthly online journal that collates and summarizes the latest research about Cystic Fibrosis, including details on symptoms, genetics, treatment, information.

Inhaled hypertonic saline in infants and children younger than 6 years with cystic fibrosis: the ISIS randomized controlled trial.

Rosenfeld M, Ratjen F, Brumback L, Daniel S, Rowbotham R, McNamara S, Johnson R, Kronmal R, Davis SD,

Division of Pulmonary Medicine, Seattle Children's Hospital, Seattle, Washington 98105, USA. margaret.rosenfeld@seattlechildrens.org

Published 19 June 2012 in JAMA, 307(21): 2269-77.
Full-text of this article is available online (may require subscription).

Articles on Cystic Fibrosis published 7 June 2012:

Multiple apical plasma membrane constituents are associated with susceptibility to meconium ileus in individuals with cystic fibrosis.   Nat Genet, 44(5): 562-9.

Variants associated with meconium ileus in cystic fibrosis were identified in 3,763 affected individuals by genome-wide association study (GWAS). Five SNPs at two loci near SLC6A14 at Xq23-24 (minimum P = 1.28 × 10(-12) at rs3788766) and SLC26A9 at 1q32.1 (minimum P = 9.88 × 10(-9) at rs4077468) accounted for ~5% of phenotypic variability and were replicated in an independent sample of affected individuals (n = 2,372; P = 0.001 and 0.0001, respectively). By incorporating the knowledge that ... [Abstract] [Full-text]

Articles on Cystic Fibrosis published 2 May 2012:

Ursodeoxycholate modulates bile flow and bile salt pool independently from the cystic fibrosis transmembrane regulator (Cftr) in mice.   Am J Physiol Gastrointest Liver Physiol, 302(9): G1035-42.

Cystic fibrosis liver disease (CFLD) is treated with ursodeoxycholate (UDCA). Our aim was to evaluate, in cystic fibrosis transmembrane regulator knockout (Cftr(-/-)) mice and wild-type controls, whether the supposed therapeutic action of UDCA is mediated via choleretic activity or effects on bile salt metabolism. Cftr(-/-) mice and controls, under general anesthesia, were intravenously infused with tauroursodeoxycholate (TUDCA) in increasing dosage or were fed either standard or UDCA-enriched ... [Abstract] [Full-text]

Articles on Cystic Fibrosis published 27 April 2012:

Vitamin D bioavailability in cystic fibrosis: a cause for concern?   Nutr Rev, 70(5): 280-93.

Despite the inclusion of extra vitamin D in their regimen of fat-soluble vitamin supplementation, cystic fibrosis patients remain chronically depleted of vitamin D. The persistence of suboptimal vitamin D status is often blamed on the maldigestion and malabsorption of fat. However, the mitigated success of recent clinical trials with high-dose vitamin D supplementation suggests that vitamin D bioavailability might be impaired in these patients. Given the growing understanding of the importance ... [Abstract] [Full-text]

Articles on Cystic Fibrosis published 16 April 2012:

Early cystic fibrosis lung disease detected by bronchoalveolar lavage and lung clearance index.   Am J Respir Crit Care Med, 185(8): 862-73.

[Abstract] [Full-text]

Future directions in early cystic fibrosis lung disease research: an NHLBI workshop report.   Am J Respir Crit Care Med, 185(8): 887-92.

Since the 1989 discovery that mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause cystic fibrosis (CF), there has been substantial progress toward understanding the molecular basis for CF lung disease, leading to the discovery and development of new therapeutic approaches. However, the earliest impact of the loss of CFTR function on airway physiology and structure and its relationship to initial infection and inflammation are poorly understood. Universal ... [Abstract] [Full-text]

Articles on Cystic Fibrosis published 12 April 2012:

Decade-long bacterial community dynamics in cystic fibrosis airways.   Proc Natl Acad Sci U S A, 109(15): 5809-14.

The structure and dynamics of bacterial communities in the airways of persons with cystic fibrosis (CF) remain largely unknown. We characterized the bacterial communities in 126 sputum samples representing serial collections spanning 8-9 y from six age-matched male CF patients. Sputum DNA was analyzed by bar-coded pyrosequencing of the V3-V5 hypervariable region of the 16S rRNA gene, defining 662 operational taxonomic units (OTUs) from >633,000 sequences. Bacterial community diversity ... [Abstract] [Full-text]

Articles on Cystic Fibrosis published 11 April 2012:

An update on the screening, diagnosis, management, and treatment of vitamin D deficiency in individuals with cystic fibrosis: evidence-based recommendations from the Cystic Fibrosis Foundation.   J Clin Endocrinol Metab, 97(4): 1082-93.

[Abstract] [Full-text]

Articles on Cystic Fibrosis published 6 April 2012:

Cystic fibrosis: a mucosal immunodeficiency syndrome.   Nat Med, 18(4): 509-19.

Cystic fibrosis transmembrane conductance regulator (CFTR) functions as a channel that regulates the transport of ions and the movement of water across the epithelial barrier. Mutations in CFTR, which form the basis for the clinical manifestations of cystic fibrosis, affect the epithelial innate immune function in the lung, resulting in exaggerated and ineffective airway inflammation that fails to eradicate pulmonary pathogens. Compounding the effects of excessive neutrophil recruitment, the ... [Abstract] [Full-text]

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