CONGENITAL GH- AND ACTH DEFICIENCY: NEONATAL HYPOGLYCEMIA ASSOCIATED WITH MICROPENIS

A fullterm male infant admitted for symptomatic neonatal hypoglycemia (H) and micropenis was found to have abnormal GH-response to fasting and insulin-induced H (peak: 1.4 ng/ml; normal 6) and low somatomedin C levels (0.1 U/ml; normal 0.1-1). This plus an inadequate response of plasma cortisol and ACTH (94.6 nmol/L 102 and 12 pg/ml 13, respectively) to H,as well as abnormal urinary 17-hydroxycorticosteroid response to metapirone (basal 0.5 mg/24 hrs 0.5; normal > 3-fold rise) suggested both, GH- and ACTH-insufficiency. Plasma T4 (5.4 μg/dl), TRH+test (TSH: 3.2 21.4 μg/ml) and LH-RH-test (LH: 4.7 7.8 mU/ml) were normal. No or only a minute response to H noted for β-hydroxybutyrate (235 μMol/L 220), free fatty acids (0.1 0.3 mval/L), alanine (77 μMol/L 80) associated with low plasma C-peptide levels (0.11 0.16 nmol/L) suggest substrate deficiency as the primary cause of H.