POMC and Aarskog-Scott syndrome, X-linked: All these patients had high levels of ACTH, and 79% of patients (73) had hyperpigmentation as the major sign of FGD; 77% showed hypoglycemia (72), 20% had seizures (19), 30% had jaundice (28), 14% had vomiting (13), 10% had respiratory stress (9), and 12% had recurrent infection (11) (Figure 3).