Diabetic Ketoacidosis in a Pediatric Patient with Cystic Fibrosis–related Diabetes

Cystic fibrosis (CF), a genetic disorder caused by pathogenic variants in the <i>CFTR</i> gene, is associated with various complications including cystic fibrosis-related diabetes (CFRD). CFRD is an entity distinct from type 1 or type 2 diabetes. We report a rare case of diabetic ketoacidosis (DKA) in a pediatric patient with CFRD, occurring during a significant pulmonary exacerbation. The patient's management involved addressing fluid and electrolyte imbalances, careful monitoring of nutritional status, and correction of hyperglycemia with insulin. This case serves as a reminder to consider DKA in the differential diagnosis of patients with CF presenting with respiratory distress, even in the absence of typical symptoms such as polyuria and polydipsia.