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Repeated episodes of hypoglycaemia were observed in two girls with spinal muscular atrophy.During a 12 h fast blood glucose fell to 3.4 and 2.7 mmol/L,respectively.One girl developed hypoglycaemia and ketonuria.Reduced gluconeogenesis was probably the cause of hypoglycaemia in these patients who had a muscle mass of about 10%of bodyweight(normal 30-40%). Hypoglycaemia must be suspected and treated when patients with severe muscle wasting due to chronic neuromuscular disorders are admitted comatose.In our experience this condition is often regarded as respiratory insufficiency. |
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