Recurrent ketotic hypoglycemia
Dr Ira Shah
Medical Sciences Department, Pediatric Oncall, Mumbai, India
Address for Correspondence: Dr Ira Shah, 1, B Saguna, 271, B St Francis Road, Vile Parle {W}, Mumbai 400056, India.
Clinical Problem :
A 4 years old boy presented with recurrent ketotic hypoglycemic convulsions at 1½, 3½ and 4 years of age. Currently on examination, he was detected to have hepatomegaly {liver 2cm palpable with span 7 cm}. Liver function tests were normal. In this child, growth hormone, lipid profile, cortisol, thyroid functions were normal. Urine and plasma aminoacidogram were normal.
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Does this child have a liver cause of ketotic hypoglycemia_?
Discussion :
Usually with hypoglycemia, the normal physiological response is reduction of Insulin secretion, release of glucagon, adrenaline, initiation of glycogenolysis and gluconeogenesis with lipolysis. Lipids are metabolized to triglycerides in turn to fatty acids and then to ketone bodies which are used by the brain as alternate fuel. Thus absence of ketosis indicates excessive Insulin or fatty acid oxidation defect. Classical ketotic hypoglycemia is the commonest cause of hypoglycemia in children less than 4 years of age. Other causes of hypoglycemia with ketosis are Growth Hormone deficiency, hypopituitarism, adrenal insufficiency and organic acids. In this child, all other causes of ketotic hypoglycemia had been ruled out. Liver disease that can cause ketotic hypoglycemia are glycogen storage disease {GSD}. Since in this liver was not large, it is unlikely to be GSD. Other causes that are associated with decreased hepatic Glucose production include galactosemia, hereditary fructose intolerance and maple syrup urine disease, they usually have other manifestations also.
Thus in this child, it does not appear to be a liver cause of hypoglycemia but classical ketotic hypoglycemia.
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