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Hypoglycaemia and Lactic Acidosis

Neonatal hypoglycaemia and lactic acidosis

Hypoglycaemia and lactic acidosis in response to infection

Boys with Barth Syndrome are prone to developing a low blood sugar (hypoglycaemia, hypoglycemia [US]) and acidic blood due to a build-up of lactic acid, especially during neonatal life and infancy. Lactic acidosis is particularly common when severe heart failure occurs.However, it is well recognised that boys with Barth Syndrome can develop hypoglycaemia and/or lactic acidosis without heart failure being apparent, especially during viral infections which cause vomiting or prevent feeding. This is especially the case with viral gastroenteritis which can be a very important precipitating factor and may require early hospital admission for intravenous supplements. The potential for hypoglycaemia is probably partly due to the low muscle mass present in many boys.

Hypoglycaemia can be life-threatening, causing drowsiness or coma, when the blood glucose falls to less than 1.5 mmol/l (27mg/dl). It can also make boys wake up in the morning feeling unwell with headache.

Barth Syndrome could potentially be confused with other biochemical diseases which produce hypoglycaemia in response to infective stress. This includes mitochondrial diseases and Medium Chain Acyl-CoA Dehydrogenase Deficiency (MCADD) and related disorders.