High bone mass associated with berardinelli lipodystrophy.

Objective: To describe an unusual case of Berardinelli-Seip syndrome with high bone mineral density (BMD).

Methods: We report the case of a 16-year-old girl presenting with dehydration, fatigue, and myalgia, associated with severe hyperglycemia, hypernatremia, and dramatically increased levels of liver enzymes, lactate dehydrogenase, and creatine kinase in the absence of ketosis. The clinical findings and pertinent medical literature are reviewed.