Hormone-Replacement Therapy for Melanocyte-Stimulating Hormone Deficiency

The first law of endocrine disease treatment is that an absent hormone should be replaced. As they report in this issue of the Journal, Kühnen et al.1 successfully applied this principle to two adult patients with genetic proopiomelanocortin deficiency, and both patients had impressive hunger reduction and weight loss as well as improved insulin sensitivity.Proopiomelanocortin is a prohormone that is cleaved in different cells to produce multiple peptide ligands that act primarily on the five melanocortin receptors.2 The pituitary corticotroph product, adrenocorticotropic hormone, stimulates the adrenal gland through the melanocortin-2 receptor (MC2R). Clinically, proopiomelanocortin deficiency is dominated by a . . .