ABSTRACT
Introduction: Maturity onset diabetes of young (MODY) is a group of monogenic disorders characterized by AD inherited, accounting for approximately 1% to 6% of all pediatric diabetic patients. Sulfonylurea has been successfully used in MODY type 1 and 3;insulin therapy is needed for other types of MODY, including type 5. GLP-1RAs, Liraglutide daily injection, has been successfully used in one report, after which the patient was off insulin therapy. Objective(s): To present a case of MODY Type 5 that showed a great response to weekly treatment of GLP-1RAs (Semagutide). Method(s): A case report, Consent were obtained. Result(s): In our case report, an 18-years old girl known to have MODY type 5 HNFA1b mutation required both long and short-acting insulin (0.6 units/kg/ day). She had a history of high, low glucose readings and frequent hypoglycemia attacks. Therefore, once weekly, GLP-1RA (Semaglutide) was tried for 3 months. The dose was gradually increased from 0.25 mg to a maximum of 0.5 mg subcutaneously weekly. Insulin therapy was weaned, and then off insulin after the 6th dose;glucose readings were monitored via Dexcom CGM, which confirmed a significant improvement (Figure 1). Moreover, there was a disappearance of hypoglycemic attacks, a reduction of insulin doses approximately to zero, an improvement of time in range (TIR) to about 100%, enhanced glucose variability, and a reduction in the serum HbA1c from 6.1% to 5.6%, which was the lowest record since the diagnosis and finally injections number dropped from 28 per week to only one. However, the medication was stopped as the patient could not tolerate its gastric side effects after she was infected with COVID-19. Conclusion(s): Semaglutide is effective and superior to insulin therapy for MODY type 5 and can be considered to replace insulin therapy. In addition, CGM showed an excellent success that helped safely transfer the patient from intensive insulin therapy to the GLP-1RAs trial. Further studies are needed in MODY type 5.