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Treatment of young patients with HNF1A mutations (HNF1A-MODY)

Item Type:Article
Title:Treatment of young patients with HNF1A mutations (HNF1A-MODY)
Creators Name:Raile, K. and Schober, E. and Konrad, K. and Thon, A. and Grulich-Henn, J. and Meissner, T. and Woelfle, J. and Scheuing, N. and Holl, R.W.
Abstract:Aim: Children and adolescents with a molecular diagnosis of HNF1A-MODY should be treated with oral sulfonylurea according to current International Society for Pediatric and Adolescent Diabetes (ISPAD) guidelines. Methods: We surveyed the German-Austrian DPV database of 50 043 people and included 114 patients with a confirmed molecular-genetic diagnosis of HNF1A mutation and diabetes onset at below age 18 years. We analysed hypoglycaemic episodes, metabolic control (HbA1c) and other clinical variables according to treatment groups. Results: People with HNF1A-MODY were included and analysed according to treatment with insulin alone (n = 34), sulfonylurea (n = 30), meglitinides (n = 22) or lifestyle (n = 28). In those receiving any drug treatment (n = 86), severe hypoglycaemia did not occur with meglitinide and was highest (at 3.6 events per 100 patient-years) with insulin. HbA1c was highest with insulin treatment (insulin = 58 mmol/mol, 7.5%; sulfonylurea = 55 mmol/mol, 7.2%; meglitinides = 52 mmol/mol, 6.9%; P = 0.008), whereas weight (BMI SD score), serum lipids and blood pressure were not different. Conclusions: Of note, 40% of people with HNF1A-MODY and medical treatment were receiving insulin alone and thus were not being treated in line with up-to-date International Society for Pediatric and Adolescent Diabetes/International Diabetes Federation guidelines, despite insulin treatment being associated with worse metabolic control and the risk of hypoglycaemia. The unlicensed use of oral drugs in patients below age 18 years and adherence by both doctors and patients to the initial insulin treatment might contribute to this finding.
Keywords:Benzamides, Combination Drug Therapy, Hepatocyte Nuclear Factor 1-{alpha}, Hypoglycemia, Hypoglycemic Agents, Insulins, Mutation, Off-Label Use, Oral Administration, Prospective Studies, Sulfonylurea Compounds, Type 2 Diabetes Mellitus
Source:Diabetic Medicine
ISSN:0742-3071
Publisher:Wiley-Blackwell (U.K.)
Volume:32
Number:4
Page Range:526-530
Date:April 2015
Official Publication:https://doi.org/10.1111/dme.12662
PubMed:View item in PubMed

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