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AuthorGrulich-Henn, Jü
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AbstractBACKGROUND: 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.dc.description.abstract
PublisherUniversität Ulmdc.publisher
LicenseCC BY 4.0 Internationaldc.rights
Link to license text
KeywordDPV initiativedc.subject
Dewey Decimal GroupDDC 610 / Medicine & healthdc.subject.ddc
MeSHCongenital hyperinsulinismdc.subject.mesh
MeSHDiabetes mellitusdc.subject.mesh
TitleCharacterization of diabetes following pancreatic surgery in patients with congenital hyperinsulinismdc.title
Resource typeWissenschaftlicher Artikeldc.type
GNDDiabetes mellitusdc.subject.gnd
FacultyMedizinische Fakultätuulm.affiliationGeneral
InstitutionInstitut für Epidemiologie und Medizinische Biometrieuulm.affiliationSpecific
Peer reviewjauulm.peerReview
DCMI TypeTextuulm.typeDCMI
In cooperation withCharité - Universitätsmedizin Berlinuulm.cooperation
In cooperation withHeinrich-Heine-Universität Düsseldorfuulm.cooperation
In cooperation withUniversitätsklinikum Heidelberguulm.cooperation
In cooperation withMedizinische Universität Grazuulm.cooperation
In cooperation withTechnische Universität Münchenuulm.cooperation
In cooperation withOtto-von-Guericke-Universität Magdeburguulm.cooperation
In cooperation withAKK Altonaer Kinderkrankenhausuulm.cooperation
In cooperation withKinderkrankenhaus Auf der Bultuulm.cooperation
DOI of original publication10.1186/s13023-018-0970-8dc.relation1.doi
Source - Title of sourceOrphanet Journal of Rare Diseasessource.title
Source - Place of publicationBMC Springer Naturesource.publisher
Source - Volume13source.volume
Source - Year2018source.year
Source - Article number230source.articleNumber
Source - ISSN1750-1172source.identifier.issn
EU projectINNODIA / 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 / 115797uulm.projectEU
FundingDeutsche Diabetes Gesellschaft (DDG)uulm.funding
FundingEuropean Foundation for the Study of Diabetes (EFSD)uulm.funding
FundingDeutsches Zentrum für Diabetesforschung (DZD)uulm.funding
FundingRobert Koch-Institut (RKI)uulm.funding
University Bibliographyjauulm.unibibliographie

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