CGM patterns in adults with cystic fibrosis-related diabetes before and after elexacaftor-tezacaftor-ivacaftor therapy.

Cystic fibrosis-related diabetes (CFRD) is a common complication of cystic fibrosis that is associated with worse outcomes and higher mortality rates. CF transmembrane conductance regulator gene (CFTR) modulators have shown favorable effects on lung function, pulmonary exacerbations, and nutrition status. However, data regarding effects of CFTR modulators on glycemic control among those with CFRD is lacking.

Romosozumab used to treat a patient with cystic fibrosis-related osteoporosis.

Bone disease is a known complication of cystic fibrosis (CF). To date, there have been no reports on the effectiveness of romosozumab, monoclonal antibody to sclerostin, to treat CF-related bone disease. We report a case of a 46-year-old premenopausal female with CF-related bone disease and multiple fractures who was treated with romosozumab.

Trabecular bone score in people with cystic fibrosis.

Unlabelled: People with cystic fibrosis (CF) are at increased risk of fractures. Our study found that low trabecular bone score (TBS) (a measure of bone strength) may help identify people with CF at risk of fractures especially when combined with bone density measured by DXA, age, hemoglobin A1c, and transplant status.

Introduction: People with cystic fibrosis (CF) are at increased risk of fractures.

Hyperglycaemia in cystic fibrosis adversely affects BK channel function critical for mucus clearance.

Large-conductance, Ca-activated, voltage-dependent K (BK) channel function is critical for adequate airway hydration and mucociliary function. In airway epithelia, BK function is regulated by its γ-subunit, leucine-rich repeat-containing protein 26 (LRRC26). Since patients with cystic fibrosis (CF)-related diabetes mellitus (CFRD) have worse lung function outcomes, this study determined the effects of hyperglycaemia on BK function in CF bronchial epithelial (CFBE) cells and evaluated the correlation between glycaemic excursions and mRNA expression of in the upper airways of CF and CFRD patients.

Hemophagocytic Lymphohistiocytosis in a Patient With Advanced HIV and Cytomegalovirus Infection.

Hemophagocytic lymphohistiocytosis (HLH) is a rare, aggressive, and, if not treated, fatal disorder that is characterized by excessive immune system activation. This disorder can be precipitated by different triggers including malignancies, infections, and autoimmune disorders. Diagnosis is made by fulfilling criteria that was last updated in 2004, and treatment frequently includes management of the underlying trigger but can also include chemotherapy.