A Pilot Study Evaluating Therapeutic Response of Different Dosage of Oral Glucocorticoid in Two Children with Familial Glucocorticoid Deficiency Presenting with Diffuse Mucocutaneous Hyperpigmentation.

Introduction: Familial glucocorticoid deficiency (FGD) is a rare autosomal recessive potentially life-threatening condition, characterized by glucocorticoid deficiency, preserved aldosterone/renin secretion, and secondary rise in plasma adrenocorticotropic hormone level. This occurs due to either mutation in adrenocorticotropic receptor (25%, FGD Type-1) or in the MC2 receptor accessory protein (15%-20%). However, in about 50% patients, no identifiable mutations have been identified.

Morgagni hernia presenting as obstructive jaundice.

Morgagnis hernia is rare in pediatrics, representing 1%-6% of all congenital diaphragmatic hernias (CDH). We report a young boy presented with obstructive jaundice caused by compression of common bile duct (CBD) due to stretching and rotation of second part of duodenum in right-sided Morgagni hernia. Such presentation is rarely reported in literature.