Semaglutide as a potential therapeutic alternative for HNF1B-MODY: a case study

Maturity-onset diabetes of the young (MODY) is a grouping of monogenic disorders. It is characterized by dominantly inherited, non-insulin-dependent diabetes. MODY is relatively rare, encompassing up to 3.5% in those diagnosed under 30 years of age. Specific types are most commonly treated with sulf...

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Main Authors: Angham Almutair, Beshaier Almulhem
Format: Article
Language:English
Published: Frontiers Media S.A. 2024-03-01
Series:Frontiers in Endocrinology
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fendo.2024.1294264/full
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author Angham Almutair
Angham Almutair
Angham Almutair
Beshaier Almulhem
Beshaier Almulhem
Beshaier Almulhem
author_facet Angham Almutair
Angham Almutair
Angham Almutair
Beshaier Almulhem
Beshaier Almulhem
Beshaier Almulhem
author_sort Angham Almutair
collection DOAJ
description Maturity-onset diabetes of the young (MODY) is a grouping of monogenic disorders. It is characterized by dominantly inherited, non-insulin-dependent diabetes. MODY is relatively rare, encompassing up to 3.5% in those diagnosed under 30 years of age. Specific types are most commonly treated with sulfonylurea, particularly those identified as HNF4A-MODY and HNF1A-MODY. HNF1B-MODY is another type that is most frequently managed with insulin therapy but lacks a defined precision treatment. We present an 18-year-old, non-obese female patient diagnosed with HNF1B-MODY. She displays complete gene deletion, a renal cyst, and hypomagnesemia. Her treatment plan includes both long- and short-acting insulin, though she frequently encountered hypoglycemia and hyperglycemia. Semaglutide, a GLP-1RA, was administered weekly over 4 months. The patient’s glucose level was continuously tracked using Dexcom’s Continuous Glucose Monitoring system. The data suggested a notable improvement in her condition: time-in-range (TIR) increased from 70% to 88%, with some days achieving 100%, and the frequency of hypoglycemic episodes, indicated by time-below-range values, fell from 5% to 1%. The time-above-range values also dropped from 25% to 10%, and her HbA1c levels declined from 7% to 5.6%. During the semaglutide therapy, we were able to discontinue her insulin treatment. Additionally, her body mass index (BMI) was reduced from 24.1 to 20.1 kg/m2. However, the semaglutide treatment was halted after 4 months due to side effects such as nausea, vomiting, and reduced appetite. Other contributing factors included exam stress and a COVID-19 infection, which forced a switch back to insulin. Her last recorded HbA1c level under exclusive insulin therapy rose to 7.1%, and her BMI increased to 24.9 kg/m2. In conclusion, semaglutide could potentially replace insulin to improve glucose variability, TIR, and HbA1c in patients with HNF1B-MODY. However, more extensive studies are required to confirm its long-term safety and efficacy.
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spelling doaj.art-12340feba5dd4c81bef5d38e65bc7abd2024-03-08T04:45:29ZengFrontiers Media S.A.Frontiers in Endocrinology1664-23922024-03-011510.3389/fendo.2024.12942641294264Semaglutide as a potential therapeutic alternative for HNF1B-MODY: a case studyAngham Almutair0Angham Almutair1Angham Almutair2Beshaier Almulhem3Beshaier Almulhem4Beshaier Almulhem5Pediatric Department, King Abdullah Specialized Children’s Hospital, King Abdulaziz Medical City, Ministry of National Guard Health Affairs, Riyadh, Saudi ArabiaCollege of Medicine, King Saud bin Abdul-Aziz University for Health Sciences, Ministry of National Guard Health Affairs, Riyadh, Saudi ArabiaKing Abdullah International Medical Research Center, Ministry of National Guard Health Affairs, Riyadh, Saudi ArabiaPediatric Department, King Abdullah Specialized Children’s Hospital, King Abdulaziz Medical City, Ministry of National Guard Health Affairs, Riyadh, Saudi ArabiaCollege of Medicine, King Saud bin Abdul-Aziz University for Health Sciences, Ministry of National Guard Health Affairs, Riyadh, Saudi ArabiaCollege of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, Saudi ArabiaMaturity-onset diabetes of the young (MODY) is a grouping of monogenic disorders. It is characterized by dominantly inherited, non-insulin-dependent diabetes. MODY is relatively rare, encompassing up to 3.5% in those diagnosed under 30 years of age. Specific types are most commonly treated with sulfonylurea, particularly those identified as HNF4A-MODY and HNF1A-MODY. HNF1B-MODY is another type that is most frequently managed with insulin therapy but lacks a defined precision treatment. We present an 18-year-old, non-obese female patient diagnosed with HNF1B-MODY. She displays complete gene deletion, a renal cyst, and hypomagnesemia. Her treatment plan includes both long- and short-acting insulin, though she frequently encountered hypoglycemia and hyperglycemia. Semaglutide, a GLP-1RA, was administered weekly over 4 months. The patient’s glucose level was continuously tracked using Dexcom’s Continuous Glucose Monitoring system. The data suggested a notable improvement in her condition: time-in-range (TIR) increased from 70% to 88%, with some days achieving 100%, and the frequency of hypoglycemic episodes, indicated by time-below-range values, fell from 5% to 1%. The time-above-range values also dropped from 25% to 10%, and her HbA1c levels declined from 7% to 5.6%. During the semaglutide therapy, we were able to discontinue her insulin treatment. Additionally, her body mass index (BMI) was reduced from 24.1 to 20.1 kg/m2. However, the semaglutide treatment was halted after 4 months due to side effects such as nausea, vomiting, and reduced appetite. Other contributing factors included exam stress and a COVID-19 infection, which forced a switch back to insulin. Her last recorded HbA1c level under exclusive insulin therapy rose to 7.1%, and her BMI increased to 24.9 kg/m2. In conclusion, semaglutide could potentially replace insulin to improve glucose variability, TIR, and HbA1c in patients with HNF1B-MODY. However, more extensive studies are required to confirm its long-term safety and efficacy.https://www.frontiersin.org/articles/10.3389/fendo.2024.1294264/fullHNF1B-MODYsemaglutidesuccessfulMODYGLP-1RA
spellingShingle Angham Almutair
Angham Almutair
Angham Almutair
Beshaier Almulhem
Beshaier Almulhem
Beshaier Almulhem
Semaglutide as a potential therapeutic alternative for HNF1B-MODY: a case study
Frontiers in Endocrinology
HNF1B-MODY
semaglutide
successful
MODY
GLP-1RA
title Semaglutide as a potential therapeutic alternative for HNF1B-MODY: a case study
title_full Semaglutide as a potential therapeutic alternative for HNF1B-MODY: a case study
title_fullStr Semaglutide as a potential therapeutic alternative for HNF1B-MODY: a case study
title_full_unstemmed Semaglutide as a potential therapeutic alternative for HNF1B-MODY: a case study
title_short Semaglutide as a potential therapeutic alternative for HNF1B-MODY: a case study
title_sort semaglutide as a potential therapeutic alternative for hnf1b mody a case study
topic HNF1B-MODY
semaglutide
successful
MODY
GLP-1RA
url https://www.frontiersin.org/articles/10.3389/fendo.2024.1294264/full
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