Donohue Syndrome in a Neonate with Central Hypothyroidism

Donohue syndrome (Leprechaunism) is the most severe form of insulin resistance syndromes which is characterized due to specific symptoms. Congenital hypothyroidism is the most common reason of mental retardation while the normal neurological development can be provided by early and effective treatment. In this article، a case with Donohue syndrome and central hypothyroidism is reported. CASE: A 28 day old male newborn with clinical features of Donohue syndrome had fasting hypoglycemia، postprandial hyperglycemia، extreme hyperinsulinemia and low serum T4 and FT4، with lower limit TSH and T3U. Blood glucose was controlled and hypothyroidism was treated. The patient died one month after discharge In infants with Donohue syndrome، despite normal screening test for hypothyroidism، comprehensive thyroid function test is recommended.