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. Chronic use of inhaled and oral corticosteroids can negatively affect bone health, growth, and glucose metabolism. This chapter provides practical guidelines regarding the screening, diagnosis, and treatment of iatrogenic adrenal insufficiency. As the guidelines are mainly derived from the asthma literature, this chapter also highlights the need for studies to evaluate the impact of CF therapies on adrenal function and other CF-endocrinopathies.

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. The exact mechanisms involved in female reproductive health conditions in CF are not clearly understood, but are thought to include cystic fibrosis transmembrane regulator (CFTR)-mediated abnormalities, changes in female sex hormones, and other CF health-related factors. In the era of CFTR modulator therapy, new data are necessary to understand the impact of CFTR modulation on contraceptive effectiveness, fertility, and pregnancy outcomes to help guide future clinical care. This article reviews the current scientific knowledge of major reproductive health issues for women with CF.

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. RESULTS: Ninety-seven percent (37/38) of OGTTs were appropriately ordered by providers, and 89% (34/38) of patients obtained the OGTT at the completion of the quality improvement period. Compared with the percentage of eligible patients completing the OGTT the year prior, the use of the algorithm significantly improved screening (P = 0.03). Data collected 1-year post-algorithm implementation revealed 97% (33/34) of OGTTs were ordered and 79% (27/34) of OGTTs were completed. The use of the algorithm 1-year post-implementation did not reveal a significant improvement in screening when compared to the reference year and implementation period (P = 0.08). CONCLUSIONS: Implementation of a clinical algorithm resulted in a statistically significant improvement in screening during the quality improvement period, but this improvement was not sustained the following year despite continued physician compliance with ordering the OGTT. Barriers to patient compliance need to be explored.