Preferred Label : hyperinsulinemic hypoglycemia, familial, 6;

Définition CISMeF : Hyperinsulinism-hyperammonemia syndrome (HIHA) is a frequent form of diazoxide-sensitive diffuse hyperinsulinism, characterized by an excessive/ uncontrolled insulin secretion (inappropriate for the level of glycemia), asymptomatic hyperammonemia and recurrent episodes of profound hypoglycemia induced by fasting and protein rich meals, requiring rapid and intensive treatment to prevent neurological sequelae. Epilepsy and cognitive deficit that are unrelated to hypoglycemia may also occur.;

MeSH synonym : hyperinsulinism-hyperammonemia syndrome;

Détails


Consulter ci-dessous une sélection des principales ressources :

Vous pouvez consulter :

Hyperinsulinism-hyperammonemia syndrome (HIHA) is a frequent form of diazoxide-sensitive diffuse hyperinsulinism, characterized by an excessive/ uncontrolled insulin secretion (inappropriate for the level of glycemia), asymptomatic hyperammonemia and recurrent episodes of profound hypoglycemia induced by fasting and protein rich meals, requiring rapid and intensive treatment to prevent neurological sequelae. Epilepsy and cognitive deficit that are unrelated to hypoglycemia may also occur.

http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=FR&Expert=35878
France
French
hyperinsulinism
hyperammonemia
hyperinsulinemic hypoglycemia, familial, 6
hypoglycemia

---
Nous contacter.
27/07/2025


[Accueil] [Haut de page]

© CHU de Rouen. Toute utilisation partielle ou totale de ce document doit mentionner la source.