Characterization of glucose metabolism in youth with vs. without cystic fibrosis liver disease: A pilot study.

Background: Diabetes and liver disease are life-threatening complications of cystic fibrosis (CF). CF-liver disease is a risk factor for CF related diabetes (CFRD) development, but the underlying mechanisms linking the two co-morbidities are not known. The objective of this pilot study was to characterize glucose metabolism in youth with CF with and without liver disease.

Female reproductive health in cystic fibrosis.

Women with cystic fibrosis (CF) are living longer and healthier lives, and opportunities for childbearing are increasingly promising. However, this population can also face sexual and reproductive health concerns, including menstrual irregularities, unplanned pregnancies, infertility and pregnancy complications. Additionally, more women are entering menopause and are at risk for the consequences of estrogen deficiency.

Adrenal function in cystic fibrosis.

Cystic fibrosis (CF) is not known to directly affect the adrenal gland, but commonly used CF therapies do impact the function of the hypothalamic-pituitary-adrenal (HPA) axis. By binding to the glucocorticoid receptor, medications such as inhaled and oral corticosteroids can enhance the systemic effects of cortisol and result in iatrogenic Cushing syndrome. Prolonged use suppresses the body's ability to make cortisol, resulting in iatrogenic adrenal insufficiency upon medication discontinuation.

Improved screening for cystic fibrosis-related diabetes by an integrated care team using an algorithm.

Objective: To determine whether implementation of a standardized, clinic-based algorithm improves compliance with cystic fibrosis-related diabetes (CFRD) screening guidelines.

Study Design: A CFRD screening algorithm was developed as part of a quality improvement initiative through collaboration between the pediatric pulmonary and endocrine divisions and implemented prospectively to children aged 8-17 years in our CF center for a 6-month period. The primary outcome measure was the percentage rate of CF patients who were appropriately screened with an oral glucose tolerance test (OGTT) during the quality improvement period as compared to the year prior.