International Guidelines for the Diagnosis and Management of Hyperinsulinism

• Autores: De Leon, Diva D.; Arnoux, Jean Baptiste; Banerjee, Indraneel; Bergadá, Ignacio; Bhatti, Tricia; Conwell, Louise S.; Fu, Junfen; Flanagan, Sarah E.; Gillis, David; Meissner, Thomas; Mohnike, Klaus; Pasquini, Tai L.S.; Shah, Pratik; Stanley, Charles A.; Vella, Adrian; Yorifuji, Tohru; Thornton, Paul S.
• Año de publicación: 2024
• Idioma: inglés
• Tipo de recurso: artículo
• Materia: Guidelines; Hyperinsulinism; Hypoglycemia; Insulin
• Institución: Consejo Nacional de Investigaciones Científicas y Técnicas

Background: Hyperinsulinism (HI) due to dysregulation of pancreatic beta-cell insulin secretion is the most common and most severe cause of persistent hypoglycemia in infants and children. In the 65 years since HI in children was first described, there has been a dramatic advancement in the diagnostic tools available, including new genetic techniques and novel radiologic imaging for focal HI; however, there have been almost no new therapeutic modalities since the development of diazoxide.Summary: Recent advances in neonatal research and genetics have improved our understanding of the pathophysiology of both transient and persistent forms of neonatal hyperinsulinism. Rapid turnaround of genetic test results combined with advanced radiologic imaging can permit identification and localization of surgically-curable focal lesions in a large proportion of children with congenital forms of HI, but are only available in certain centers in "developed" countries. Diazoxide, the only drug currently approved for treating HI, was recently designated as an "essential medicine" by the World Health Organization but has been approved in only 16% of Latin American countries and remains unavailable in many under-developed areas of the world. Novel treatments for HI are emerging, but they await completion of safety and efficacy trials before being considered for clinical use.Key messages: This international consensus statement on diagnosis and management of HI was developed in order to assist specialists, general pediatricians, and neonatologists in early recognition and treatment of HI with the ultimate aim of reducing the prevalence of brain injury caused by hypoglycemia. A previous statement on diagnosis and management of HI in Japan was published in 2017. The current document provides an updated guideline for management of infants and children with HI and includes potential accommodations for less-developed regions of the world where resources may be limited.
Fil: De Leon, Diva D.. University of Pennsylvania; Estados Unidos
Fil: Arnoux, Jean Baptiste. University of Pennsylvania; Estados Unidos
Fil: Banerjee, Indraneel. University of Manchester; Reino Unido
Fil: Bergadá, Ignacio. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Parque Centenario. Centro de Investigaciones Endocrinológicas "Dr. César Bergada". Gobierno de la Ciudad de Buenos Aires. Centro de Investigaciones Endocrinológicas "Dr. César Bergada". Fundación de Endocrinología Infantil. Centro de Investigaciones Endocrinológicas "Dr. César Bergada"; Argentina
Fil: Bhatti, Tricia. University of Pennsylvania; Estados Unidos
Fil: Conwell, Louise S.. University of Queensland; Australia
Fil: Fu, Junfen. The Children’s Hospital of Zhejiang University School of Medicine; China
Fil: Flanagan, Sarah E.. University of Exeter; Reino Unido
Fil: Gillis, David. The Hebrew University of Jerusalem; Israel
Fil: Meissner, Thomas. Heinrich Heine University; Alemania
Fil: Mohnike, Klaus. Otto-von-Guericke University Magdeburg; Alemania
Fil: Pasquini, Tai L.S.. Congenital Hyperinsulinism International; Estados Unidos
Fil: Shah, Pratik. Queen Mary University; Reino Unido
Fil: Stanley, Charles A.. University of Pennsylvania; Estados Unidos
Fil: Vella, Adrian. Mayo Clinic Cancer Center; Estados Unidos
Fil: Yorifuji, Tohru. Osaka City General Hospital; Japón
Fil: Thornton, Paul S.. University Burnett School of Medicine; Estados Unidos