Ans. b. Familial glucocorticoid deficiency Hypoglycemia, hyperpigmentation with normal BP and normal genitalia in a child points towards Familial glucocorticoid deficiency.Familial Glucocorticoid Deficiency* Familial glucocorticoid deficiency is a rare autosomal recessive condition.* It is characterized by adrenal insufficiency.Pathology:Pathological Examination of the Adrenal GlandZona glomerulosaWell preservedZona fasciculataAtrophiedZona reticularisAtrophiedThis causes low cortisol concentration because the zona fasciculata is primarily responsible for glucocorticoid production.* Zona glomerulosa is well preserved, mineralocorticoid action is usually unaffected.* Low circulating serum cortisol results in lack of feedback inhibition to the hypothalamus, which results in increased ACTH secretion from pituitary.Clinical features* Patients with familial glucocorticoid deficiency generally presents with signs and symptoms of''adrenal insufficiency" with the important distinction that mineralocorticoid production is always normal.* .VIC initial presenting sign is deep hyperpigmentation of the skin, mucous membrane or both as a result of the action of ACTH on cutaneous MSH receptors^.* The symptoms are compatible with glucocorticoid deficiency. May patients presents with recurrent hypoglycemia or severe infections.* In the neonatal period, frequent presenting signs include feeding problems, failure to thrive, regurgitation and hypoglycemia manifesting as seizures.