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Metabolic Effects of Glucagon Stimulations in Type 1 Diabetes and Healthy Controls.
J Clin Endocrinol Metab
January 2025
Department of Endocrinology and Nephrology, Nordsjællands Hospital, Hillerød, Denmark.
Introduction: Metabolic responses to glucagon beyond the promotion of endogenous glucose production in type 1 diabetes remains poorly explored. Therefore, we aimed to investigate the metabolic responses to glucagon stimulation in type 1 diabetes and explore whether recent exposure to hypoglycemia would impact glucagon sensitivity.
Research Design And Methods: Twenty-nine participants, 19 with type 1 diabetes and ten healthy controls, underwent a hyperinsulinemic-euglycemic clamp with five-stepwise ascending glucagon injections.
Effect of Hypoglycemia on QT Interval in Individuals with and without Type 2 Diabetes: A Randomized Crossover Trial.
J Clin Endocrinol Metab
January 2025
Harvard Medical School, Boston, MA, USA.
Background: Our goal was to determine in healthy individuals and individuals with type 2 diabetes the impact of repeated episodes of hypoglycemia on the corrected QT (QTc) interval and the time course for QTc recovery. Further, since hypoglycemia increases aldosterone and patients with primary aldosteronism have prolonged QTc, we also determined whether mineralocorticoid receptor blockade prevents hypoglycemia-induced QTc alterations.
Methods: Twenty-seven healthy participants completed a double-blinded crossover trial contrasting 3 experimental conditions: 1) euglycemia, 2) hypoglycemia, and 3) hypoglycemia with mineralocorticoid receptor blockade pretreatment.
Congenital Hyperinsulinism and Long QT Syndrome Attributable to a Variant in KCNE1.
Horm Res Paediatr
January 2025
Division of Endocrinology and Diabetes, Congenital Hyperinsulinism Center, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
Introduction: This is a report of a child with congenital hyperinsulinism associated with a loss-of-function variant in KCNE1. KCNE1 encodes a human potassium channel accessory (beta) subunit that modulates potassium channel Kv7.1 (encoded by KCNQ1).
View Article and Find Full Text PDFWieacker-Wolff syndrome with hyperinsulinemic hypoglycemia successfully treated using diazoxide: A case report.
Clin Pediatr Endocrinol
January 2025
Department of Pediatrics, The University of Tokyo, Tokyo, Japan.
Wieacker-Wolff syndrome (WRWF) is an X-linked genetic disorder characterized by neuromusculoskeletal abnormalities caused by loss-of-function variants of the gene. Here, we report the case of a male infant with WRWF manifesting as multiple joint contractures and congenital anomalies at birth. He underwent gastrostomy to treat the gastroesophageal reflux disease, which caused mixed apnea and transient bradycardia.
View Article and Find Full Text PDFhaploinsufficiency in a mother-daughter pair with young-onset diabetes with and without neonatal hypoglycemia.
Clin Pediatr Endocrinol
January 2025
Department of Molecular Endocrinology, National Research Institute for Child Health and Development, Tokyo, Japan.
The ATP-binding cassette transporter subfamily C member 8 (ABCC8) regulates insulin secretion from β-cells. Loss- and gain-of-function variants of have been implicated in neonatal hyperinsulinemic hypoglycemia and young-onset diabetes, respectively. Although some patients with variants have been reported to exhibit both neonatal hypoglycemia and young-onset diabetes, the molecular and clinical characteristics of this atypical phenotype remain unknown.
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