Results 91 to 100 of about 10,576 (237)

Developing a congenital hyperinsulinism prioritized research agenda: a patient-driven international collaborative research network

Frontiers in Endocrinology
IntroductionCongenital Hyperinsulinism (HI) is a rare disease that causes severe and recurrent hypoglycemia due to dysregulated insulin secretion. HI is the most frequent cause of severe, persistent hypoglycemia in newborns and children.
Tai L. S. Pasquini, Indraneel Banerjee, Henrik Thybo Christesen, Louise S. Conwell, Antonia Dastamani, Diva D. De Leon, Sarah E. Flanagan, David Gillis, Jennifer M. Kalish, Katherine Lord, Mahlet Mesfin, Jennifer Schmitt, Senthil Senniappan, Charles A. Stanley, Paul S. Thornton, David Zangen, Julie Raskin, the Congenital Hyperinsulinism Collaborative Research Network Members   +17 more
doaj   +1 more source

Opening closed inward rectifier potassium channel doors

British Journal of Pharmacology, Volume 183, Issue 10, Page 2197-2218, May 2026.
Inwardly rectifying potassium (KIR) channels are essential regulators of membrane potential in excitable and non‐excitable tissues. Although KIR channels exhibit a biophysical preference for potassium influx due to voltage‐dependent block of outward current by polyamines and Mg2+, under physiological conditions, they predominantly mediate K+ efflux ...
Anna Stary‐Weinzinger, Fabian Kaiser, Marcel A. G. van der Heyden, Saïd Bendahhou   +3 more
wiley   +1 more source

The difficult management of persistent, non‐focal congenital hyperinsulinism: A retrospective review from a single, tertiary center

Pediatric Diabetes, 2020
Congenital hyperinsulinism (CHI) is a rare, heterogeneous disease with transient or persistent hypoglycemia. Histologically, focal, diffuse, and atypical forms of CHI exist, and at least 11 disease‐causing genes have been identified.
Amalie Greve Rasmussen, M. Melikian, E. Globa, S. Detlefsen, L. Rasmussen, H. Petersen, K. Brusgaard, A. H. Rasmussen, M. Mortensen, H. Christesen   +9 more
semanticscholar   +1 more source

Transjugular Leadless Pacemaker Implantation in an Adolescent With Central Hypoventilation Syndrome: A Case Report

Pacing and Clinical Electrophysiology, Volume 49, Issue 5, Page 606-609, May 2026.
ABSTRACT Background Permanent pacing in pediatric patients is complicated by small body size, vascular access limitations, and the need for durable long‐term management. Leadless pacemakers offer an emerging alternative that minimizes infection and lead‐related complications, yet vascular access remains a key challenge in young patients.
Marzia Giaccardi, Gulio Porcedda, Zaccaria Ricci, Hendrik Bonnemeier, Benito Baldauf   +4 more
wiley   +1 more source

Congenital hyperinsulinism and mosaic abnormalities of the ploidy.

, 2006
BACKGROUND: Congenital hyperinsulinism and Beckwith-Wiedemann syndrome both lead to beta islet hyperplasia and neonatal hypoglycaemia. They may be related to complex genetic/epigenetic abnormalities of the imprinted 11p15 region.
Rahier, J., Nihoul-Fékété, C., Hubert, L, Brunelle, F., Bellanné-Chantelot, C, Henquin, J. C., de Lonlay, P, Giurgea, I, De Lonlay, P., Rahier, Jacques, Robert, J. J., Vekemans, M, Groos, M. S., Dunne, M. J., Fournet, J-C, Touati, G, Jaubert, F., Dunne, M J, Junien, C., Vekemans, M., Jaubert, F, Robert, J-J, Bellanné-Chantelot, C., Sempoux, Christine, Sempoux, C., Sanlaville, D, Touati, G., Groos, M-S, Henquin, Jean-Claude, Sanlaville, D., Giurgea, Irina, Fournet, J. C., Brunelle, F, Nihoul-Fékété, C, Hubert, L.   +34 more
core   +1 more source

The molecular mechanisms, diagnosis and management of congenital hyperinsulinism

Indian Journal of Endocrinology and Metabolism, 2013
Congenital hyperinsulinism (CHI) is the result of unregulated insulin secretion from the pancreatic β-cells leading to severe hypoglycaemia. In these patients it is important to make an accurate diagnosis and initiate the appropriate management so as to ...
Senthil Senniappan, Ved Bhushan Arya, Khalid Hussain   +2 more
doaj   +1 more source

Contrasting Effects of Chronic Glucokinase Activation and Inhibition on Pancreatic Beta‐Cell Function

The FASEB Journal, Volume 40, Issue 7, 15 April 2026.
In the non‐diabetic beta‐cell (top left) activation of glucokinase (bottom left) leads to inhibition of mitochondrial metabolism and thereby impairs insulin secretion. The effect is similar to that of chronic hyperglycaemia as seen in the diabetic beta‐cell (top right).
Matthew Lloyd, Natascia Vedovato, Samuel J. Mullan, Alice Elphick, Aerin J. Dumenil, Chantal Rufer, Alexandra C. Title, Burcak Yesildag, Elizabeth Haythorne, Frances M. Ashcroft   +9 more
wiley   +1 more source

From the cytosol to the inner membrane: biogenesis of the mitochondrial carrier family

Protein Science, Volume 35, Issue 4, April 2026.
Abstract Mitochondrial carrier proteins are essential for cellular physiology as they are active in a wide range of metabolic pathways including production of cellular energy, amino acid synthesis, redox balance and ion homeostasis. The double membrane of mitochondria provides a tightly gated environment through which carrier proteins facilitate the ...
Catherine S. Palmer, Phoebe J. Leeming, Diana Stojanovski   +2 more
wiley   +1 more source

Cardiometabolic Risk Assessment in a Cohort of Children and Adolescents Diagnosed with Hyperinsulinemia

Diseases
Background: Individuals with hyperinsulinemia may initially not meet any diagnostic criteria for metabolic syndrome, though displaying a higher risk of cardiovascular complications combined with obesity, diabetes, and hypertension.
Giorgio Sodero, Donato Rigante, Lucia Celeste Pane, Linda Sessa, Ludovica Quarta, Marcello Candelli, Clelia Cipolla   +6 more
doaj   +1 more source

Avaliação do hiperinsulinismo em amostra de crianças pré-puberes Hyperinsulinism assessment in a sample of prepubescent children

Jornal de Pediatria, 2010
OBJETIVOS: Determinar a relação entre o índice de massa corporal (IMC), o homeostasis model assessment - insulin resistance (HOMA-IR) e a insulinemia. MÉTODOS: Realizou-se um estudo observacional prospectivo transversal com 132 crianças pré-púberes em ...
Stephanye Felicye Armecy Mieldazis, Ligia Ajaime Azzalis, Virginia Berlanga Campos Junqueira, Fabíola Isabel Suano Souza, Roseli Oselka Sacardo Sarni, Fernando Luiz Affonso Fonseca   +5 more
doaj   +1 more source