[Congenital hypopituitarism in agenesis of the anterior pituitary gland: immediate management and follow-up].

We report a case of an infant admitted to our division at 50 days of age, who presented acute abdomen, respiratory insufficiency, skeletal abnormalities, craniofacial dismorphism, low gain of weight. Despite negative at neonatal screening for hypothyroidism, laboratory analyses showed marked decrease of all pituitary hormones but ACTH, and low levels of cortisoL Magnetic resonance was indicative of adenohypophysis agenesis with maintained neurohypophysis. Results of substitutive therapy are reported.