Abstract
<jats:p>Inherited metabolic disorders (IMDs) are a group of over 750 known complex conditions that are genetic, inherited problems of metabolism caused by a biological enzyme deficiency. In the United Kingdom, six metabolic disorders are screened for on a newborn blood spot screening test, which is performed on all babies at 5 days of age. These disorders are PKU, MCADD, isovaleric acidaemia, glutaric aciduria type 1, homocystinuria and maple syrup urine disease. On diagnosis, the dietitian will discuss dietary management, prescribe any essential specialist dietary products (e.g. condition‐specific formula) and provide written information for the family and carers. Emergency regimens are given to patients and their families to prevent decompensation during periods of illness or fasting, including planned surgery. Idiopathic ketotic hypoglycaemia is a diagnosis of exclusion and the most frequent cause of hypoglycaemia in childhood.</jats:p>