The use of fructosamine in cystic fibrosis-related diabetes (CFRD) screening.

Objective: To determine whether serum fructosamine correlates with glycemic control and clinical outcomes in patients being screened for cystic fibrosis-related diabetes (CFRD).

Methods: Fructosamine and percent predicted forced expiratory volume in 1s (FEV) were measured in patients undergoing a 2h oral glucose tolerance test (OGTT) for CFRD screening. Fractional serum fructosamine (FSF) was calculated as fructosamine/total protein.

Fatty acids in blood and intestine following docosahexaenoic acid supplementation in adults with cystic fibrosis.

Unlabelled: The objective of this study was to investigate the effect of docosahexaenoic acid (DHA) supplementation on blood and intestinal DHA levels and lung function in mild/moderately affected adult CF patients with the DeltaF508 genotype.

Background: Cystic Fibrosis (CF) patients often present with plasma fatty acid levels indicating low levels of linoleic (18:2n-6) and docosahexaenoic (22:6n-3) acids and an increased level of arachidonic acid (20:4n-6). Improved dietary fat intake or reducing fat malabsorption with pancreatic enzymes has failed to normalize this biochemical deficiency of DHA.

Low bone mineral density in cystic fibrosis patients.

The goals of this practice-based, observational study were to describe the prevalence of low bone mineral density in patients at the Edmonton Cystic Fibrosis Centre, and to determine if body mass index and previous systemic corticosteroid use of over one month's duration were predictors of low bone mineral density. One hundred and thirteen pediatric and adult patients were studied. Bone mineral density of the lumbar spine region was measured using dual-energy X-ray absorptiometry.

An unusual case of pulmonary invasive aspergillosis and aspergilloma cured with voriconazole in a patient with cystic fibrosis.

The development of pulmonary aspergilloma and invasive aspergillosis is a rare complication of cystic fibrosis. We describe a 29-year-old patient with cystic fibrosis who had invasive pulmonary aspergillosis that was not cured by amphotericin B, liposomal amphotericin B, or itraconazole. This patient was subsequently successfully treated and cured with the novel antifungal agent voriconazole.