Cystic fibrosis related diabetes in an extremely young patient.

Diabetes in patients with cystic fibrosis (CF) is common but typically does not occur before the age of 10 years. We describe a male with cystic fibrosis who developed diabetes, consistent with cystic fibrosis related diabetes, at the age of 2 years and treated successfully with insulin.

Altered O-glycosylation and sulfation of airway mucins associated with cystic fibrosis.

Cystic fibrosis (CF) is the most lethal genetic disorder in Caucasians and is characterized by the production of excessive amounts of viscous mucus secretions in the airways of patients, leading to airway obstruction, chronic bacterial infections, and respiratory failure. Previous studies indicate that CF-derived airway mucins are glycosylated and sulfated differently compared with mucins from nondiseased (ND) individuals. To address unresolved questions about mucin glycosylation and sulfation, we examined O-glycan structures in mucins purified from mucus secretions of two CF donors versus two ND donors.