Progress in Definition, Prevention and Treatment of Fungal Infections in Cystic Fibrosis.

Cystic fibrosis (CF) is a chronic lethal multi-system condition; however, most of the morbidity and mortality is dependent on the status of the respiratory system. Progressive respiratory decline is mediated by chronic infection and inflammation, punctuated by important acute events known as pulmonary exacerbations which can lead to accelerated decline. The main bacterial species causing infections include Pseudomonas aeruginosa, Staphylococcus aureus, Haemophilus influenzae and Achromobacter xylosoxidans. In addition to bacteria, fungi are detected in a significant number of patients. The impact of fungal colonization of the airways is still not completely elucidated, but an increasing body of evidence suggests an important role for moulds and yeasts. Although fungal infections are rare, fungi can cause severe pneumonia requiring appropriate targeted treatment. The most common fungi in respiratory samples of patients with CF are Aspergillus fumigatus, Aspergillus terreus and Scedosporium species for filamentous fungi, and yeasts such as Candida albicans and Candida glabrata. Therapeutic strategies depend on the detected fungus and the underlying clinical status of the patient. The antifungal therapy can range from a simple monotherapy up to a combination of three different drugs. Treatment course may be indicated in some patients for two weeks and in others for up to six months, and in rare cases even longer. New antifungal drugs have been developed and are being tested in clinical studies offering the hope of therapeutic alternatives to existing drugs. Identifying relevant risk factors and diagnostic criteria for fungal colonization and infection is crucial to enabling an adequate prevention, diagnosis and treatment.

Androgens, exercise capacity, and muscle function in cystic fibrosis.

BACKGROUND: It is well established that muscle strength and exercise capacity are impaired in adults with cystic fibrosis (CF). Numerous factors have been implicated in this process including nutritional factors, disuse, and corticosteroid use. It is also known that serum testosterone levels are frequently low in adolescent male patients with CF. As testosterone is known to have an effect on peripheral muscle, we aimed to assess the relationship among circulating androgen levels, muscle strength, and exercise capacity in adults with CF. METHODS: A prospective observational study was performed. Participants underwent maximal exercise testing and peripheral muscle strength assessment (quadriceps, shoulder flexion, handgrip, maximal inspiratory pressure, and maximal expiratory pressure). Venous blood samples were obtained for the measurement of total testosterone, sex hormone-binding globulin, and albumin. The free testosterone (FT) level was calculated. RESULTS: A total of 15 subjects completed the study. The patients who were studied had impaired muscle strength and exercise capacity but normal mean androgen levels. Quadriceps strength, SF, and peak oxygen uptake (Vo(2)max) were 40.1 +/- 9.4%, 59.3 +/- 20.3%, and 77.4 +/- 22.2% predicted, respectively. One patient had a low testosterone level, with low FT levels evident in two subjects. There was no correlation between testosterone or FT level with any of the muscle strengths calculated. Neither testosterone or FT level was correlated with exercise capacity or pulmonary function. CONCLUSIONS: Male CF patients with impaired skeletal muscle strength and exercise capacity have normal testosterone levels. Hypogonadism was rare in this group of adult men with CF. These findings would not support the role of testosterone in muscle dysfunction or impaired exercise capacity in CF patients.