Fat and Glucose Metabolism in Fed and Fasted State in Patients With Low Skeletal Muscle Mass

In a study from 2003 the investigators showed that adult patients with very low skeletal muscle mass (spinal muscular atrophy (SMA) type II, Duchenne muscular dystrophy, congenital muscular dystrophy) are prone to develop hypoglycemia during prolonged fasting. Since then case reports have described the same phenomenon with hypoglycemia and metabolic crises in children with low skeletal muscle mass provoked by infection, fasting and surgery. Pathophysiological mechanisms of metabolism have never been investigated in adults or children with SMA II. Thus the investigators studied fat and glucose metabolism during prolonged fasting in patients with SMA II and LAMA 2 and compared results to those found in healthy controls.