The MC4R agonist, setmelanotide, should be administered in patients with POMC, PCSK1, or LEPR deficiency and BBS, and the GLP-1 receptor agonists, semaglutide and liraglutide are also novel pharmacological agents that have been FDA approved for the treatment of childhood obesity. The gene discussed is GLP1R; the disease is Bardet-Biedl syndrome.