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Cited by 84Cardiac electrophysiology and arrhythmiasPancreatic function and diabetesIon channel regulation and function

KCNQ1 Long QT Syndrome Patients Have Hyperinsulinemia and Symptomatic Hypoglycemia

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Torekov, Signe S., Iepsen, Eva, Christiansen, Michael, Linneberg, Allan, Pedersen, Oluf, Holst, Jens J., Kanters, Jørgen K., Hansen, Torben·2014·Diabetes
DOI 10.2337/db13-1454
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Abstract

Patients with loss-of-function mutations in KCNQ1 have KCNQ1 long QT syndrome (LQTS). KCNQ1 encodes a voltage-gated K(+) channel located in both cardiomyocytes and pancreatic β-cells. Inhibition of KCNQ1 in β-cells increases insulin secretion. Therefore KCNQ1 LQTS patients may exhibit increased insulin secretion. Fourteen patients, from six families, diagnosed with KCNQ1 LQTS were individually matched to two randomly chosen BMI-, age-, and sex-matched control participants and underwent an oral glucose tolerance test (OGTT), a hypoglycemia questionnaire, and continuous glucose monitoring. KCNQ1 mutation carriers showed increased insulin release (area under the curve 45.6 ± 6.3 vs. 26.0 ± 2.8 min ⋅ nmol/L insulin) and β-cell glucose sensitivity and had lower levels of plasma glucose and serum potassium upon oral glucose stimulation and increased hypoglycemic symptoms. Prolonged OGTT in four available patients and matched control subjects revealed hypoglycemia in carriers after 210 min (range 1.4-3.6 vs. 4.1-5.3 mmol/L glucose), and 24-h glucose profiles showed that the patients spent 77 ± 18 min per 24 h in hypoglycemic states (<3.9 mmol/L glucose) with 36 ± 10 min (<2.8 mmol/L glucose) vs. 0 min (<3.9 mmol/L glucose) for the control participants. The phenotype of patients with KCNQ1 LQTS, caused by mutations in KCNQ1, includes, besides long QT, hyperinsulinemia, clinically relevant symptomatic reactive hypoglycemia, and low potassium after an oral glucose challenge, suggesting…

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  • Long QT, hyperinsulinemia, and low potassium after oral glucose challenge are demonstrated in some reactive hypoglycemic patients who have mutations in KCNQ1.

    “Long QT, hyperinsulinemia, and low potassium after an oral glucose challenge are demonstrated in some reactive hypoglycemic patients who have mutations in KCNQ1.[33]”

    From Postprandial Reactive Hypoglycemia (2019) · cites this paper as [33]

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Authors
Torekov, S. S., Iepsen, E., Christiansen, M., Linneberg, A., Pedersen, O., Holst, J. J., Kanters, J. K., & Hansen, T.
Affiliations
  • University of Copenhagen (DK)
  • Statens Serum Institut (DK)
  • Glostrup Hospital (DK)
Corresponding author
Torekov, Signe S.
ORCID
Venue
Diabetes
Publisher
American Diabetes Association
Published
2014-03-13
Volume
63
Issue
4
Pages
1315-1325
ISSN
  • 0012-1797 (ISSN-L)
  • 1939-327X
DOI
10.2337/db13-1454
OpenAlex
W2140441583
Cited by
84
References
48
Citations by year
2026: 7 · 2025: 6 · 2024: 5 · 2023: 8 · 2022: 6 · 2021: 7 · 2020: 10 · 2019: 9 · 2018: 3 · 2017: 5 · 2016: 6 · 2015: 9 · 2014: 3
FWCI
4.08
Type
journal article
Language
en
Open access
Licence
Locations
  • Diabetes (publishedVersion, cc-by-nc-nd)
  • PubMed (publishedVersion)
  • VBN Forskningsportal (Aalborg Universitet) (publishedVersion, other-oa)
  • Research at the University of Copenhagen (University of Copenhagen) (publishedVersion)
  • University of Southern Denmark Research Portal (University of Southern Denmark) (submittedVersion)
Topics
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
  • Health Sciences › Medicine › Surgery › Pancreatic function and diabetes
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Ion channel regulation and function
Keywords
hyperinsulinemia, reactive hypoglycemia, KCNQ1 mutation, beta-cell insulin secretion, beta-cell glucose sensitivity, oral glucose tolerance test, continuous glucose monitoring, hypoglycemic symptoms, serum potassium
MeSH terms
Insulin Secretion, Blood Glucose, Glucose, Glucose Tolerance Test, Humans, Hyperinsulinism, Hypoglycemia, Insulin, Long QT Syndrome, KCNQ1 Potassium Channel

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