Congenital Hyperinsulinism Pipeline Shows 4+ Active Players and Late-Stage Drug Candidates
The congenital hyperinsulinism pipeline includes 4+ active companies and 4+ therapies across clinical and nonclinical stages. Zealand Pharma advanced dasiglucagon in January 2026, while RZ358 is in Phase III evaluation.
Congenital hyperinsulinism pipeline development includes 4+ companies and 4+ pipeline drugs in clinical and nonclinical stages, with Rezolute Inc., Hanmi Pharmaceutical and Zealand Pharma among the companies active in the space. In January 2026, Zealand Pharma announced a long-term safety and efficacy extension trial of dasiglucagon administered as a subcutaneous infusion in children with congenital hyperinsulinism who completed either ZP4207-17103 or ZP4207-17109.
The pipeline report describes congenital hyperinsulinism as the most frequent cause of persistent hypoglycemia in infants and children and says delays in diagnosis and initiation of appropriate treatment contribute to a high risk of neurocognitive impairment. It says congenital hyperinsulinism represents a heterogeneous group of disorders characterized by dysregulated insulin secretion by the pancreatic beta cells, and that there are at least nine known monogenic forms as well as several syndromic forms.
Among emerging therapies, RZ358 is a fully human monoclonal antibody developed to treat congenital hyperinsulinism and other conditions characterized by excessive insulin activity. The drug binds to a unique allosteric site on insulin receptors in the liver, fat, and muscle, counteracting the effects of excess insulin binding and activity to correct hypoglycemia, and is currently in Phase III clinical trial evaluation for congenital hyperinsulinism.
The report identifies promising therapies including HM15136, Dasiglucagon, Glucagon, Sandostatine LP, Avexitide, Lyo avexitide, and LIK066. It also says companies are involved in targeted therapeutics development across early-stage, mid-stage, and late-stage programs, as well as inactive pipeline products.
The pipeline assessment covers product type, stage, route of administration, molecule type, target receptor, monotherapy or combination therapy, and mechanism of action, and includes analysis of collaborations, licensing agreements and financing details for future advancement of congenital hyperinsulinism treatment.