All those with a mutation and only one patient without one suffered from hypoglycemia; the two groups differed in the frequency of cholestatic jaundice associated with hepatomegaly in 4 cases which disappeared after Hydrocortisone® therapy (11/17 of those with a mutation and 1/10 without), mean ACTH (6.8 pg/mL with and 12.1 pg/mL without) and consanguinity (5/13 with and 1/8 without). Here, POMC is linked to Hypoglycemia.