Characterization of diabetes following pancreatic surgery in patients with congenital hyperinsulinism
Wissenschaftlicher Artikel
Authors
Welters, Alena
Meißner, Thomas
Grulich-Henn, Jürgen
Fröhlich-Reiterer, Elke
Warncke, Katharina
Faculties
Medizinische FakultätInstitutions
Institut für Epidemiologie und Medizinische BiometrieExternal cooperations
Charité - Universitätsmedizin BerlinHeinrich-Heine-Universität Düsseldorf
Universitätsklinikum Heidelberg
Medizinische Universität Graz
Technische Universität München
Published in
Orphanet Journal of Rare Diseases ; 13 (2018). - Art.-Nr. 230. - ISSN 1750-1172
Link to original publication
https://dx.doi.org/10.1186/s13023-018-0970-8Peer review
ja
Document version
publishedVersion
Abstract
BACKGROUND: Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycaemia in infancy that leads to unfavourable neurological outcome if not treated adequately. In patients with severe diffuse CHI it remains under discussion whether pancreatic surgery should be performed or intensive medical treatment with the acceptance of recurrent episodes of mild hypoglycaemia is justified. Near-total pancreatectomy is associated with high rates of insulin-dependent diabetes mellitus and exocrine pancreatic insufficiency. Little is known about the management and long-term glycaemic control of CHI patients with diabetes after pancreatic surgery. We searched the German/Austrian DPV database and compared the course of 42 CHI patients with diabetes to that of patients with type 1 diabetes mellitus (T1DM). Study groups were compared at diabetes onset and after a follow-up period of 6.1 [3.3-9.7] (median [interquartile range]) years.
RESULTS: The majority of CHI patients with diabetes were treated with insulin (85.2% [70.9-99.5] at diabetes onset, and 90.5% [81.2-99.7] at follow-up). However, compared to patients with T1DM, significantly more patients in the CHI group with diabetes were treated with conventional insulin therapy (47.8% vs. 24.4%, p = 0.03 at diabetes onset, and 21.1% vs. 6.4% at follow-up, p = 0.003), and only a small number of CHI patients were treated with insulin pumps. Daily insulin dose was significantly lower in CHI patients with diabetes than in patients with T1DM, both at diabetes onset (0.3 [0.2-0.5] vs. 0.6 IE/kg/d [0.4-0.8], p = 0.003) and follow-up (0.8 [0.4-1.0] vs. 0.9 [0.7-1.0] IE/kg/d, p = 0.02), while daily carbohydrate intake was comparable in both groups. Within the first treatment year, HbA1c levels were significantly lower in CHI patients with diabetes (6.2% [5.5-7.9] vs. 7.2% [6.5-8.2], p = 0.003), but increased to a level comparable to that of T1DM patients at follow-up. Interestingly, in CHI patients, the risk of severe hypoglycaemia tends to be higher only at diabetes onset (14.8% vs. 5.8%, p = 0.1).
CONCLUSIONS: In surgically treated CHI patients insulin treatment needs to be intensified in order to achieve good glycaemic control. Our data furthermore emphasize the need for improved medical treatment options for patients with diazoxide- and/or octreotide-unresponsive CHI.
Funding information
Deutsche Diabetes Gesellschaft (DDG)
European Foundation for the Study of Diabetes (EFSD)
Deutsches Zentrum für Diabetesforschung (DZD)
Robert Koch-Institut (RKI)
European Foundation for the Study of Diabetes (EFSD)
Deutsches Zentrum für Diabetesforschung (DZD)
Robert Koch-Institut (RKI)
EU Project
INNODIA / Translational approaches to disease modifying therapy of type 1 diabetes: an innovative approach towards understanding and arresting type 1 diabetes – Sofia ref.: 115797 / EC / H2020 / 115797
Subject Headings
Hyperinsulinämie [GND]Pankreatektomie [GND]
Diabetes mellitus [GND]
Congenital hyperinsulinism [MeSH]
Diabetes mellitus [MeSH]
Pancreatectomy [MeSH]
Keywords
DPV initiativeDewey Decimal Group
DDC 610 / Medicine & healthMetadata
Show full item recordCitation example
Welters, Alena et al. (2020): Characterization of diabetes following pancreatic surgery in patients with congenital hyperinsulinism. Open Access Repositorium der Universität Ulm. http://dx.doi.org/10.18725/OPARU-30059