Your browser doesn't support javascript.
loading
Congenital Hyperinsulinism: An Historical Perspective.
Thornton, Paul S; Stanley, Charles A; De Leon, Diva D.
Afiliação
  • Thornton PS; Congenital Hyperinsulinism Center, Cook Children's Medical Center, Fort Worth, Texas, USA.
  • Stanley CA; Congenital Hyperinsulinism Center, Division of Endocrinology and Diabetes, Department of Pediatrics, The Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
  • De Leon DD; Congenital Hyperinsulinism Center, Division of Endocrinology and Diabetes, Department of Pediatrics, The Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Horm Res Paediatr ; 95(6): 631-637, 2022.
Article em En | MEDLINE | ID: mdl-36446321
Congenital hyperinsulinism is the most common cause of persistent hypoglycemia in neonates, infants, and children. Since the first case descriptions in the 1950s, the field has advanced significantly. It was the development of the insulin radioimmunoassay by Yalow and Berson a decade later that made it possible to demonstrate that this form of persistent hypoglycemia was caused by insulin, and a few years later, Drash described the successful treatment of children with hyperinsulinism with the antihypertensive diazoxide, which until today remains the only approved treatment for hyperinsulinism. In the mid 1970s, Baker and Stanley described that hyperinsulinism can be recognized by inappropriate responses of metabolic fuels and hormones during the course of a provocative fasting challenge. Later, advances in molecular genetics led to the discovery of the different genetic subtypes of hyperinsulinism. One of the most impactful discoveries in the field was the recognition of the focal form of hyperinsulinism and the development of 18F-DOPA PET for the localization of focal lesions before surgery which has resulted in the possibility of cure for children with focal disease. However, treatment options for children with nonfocal diazoxide-unresponsive hyperinsulinism have continued to be limited. New drug development programs for hyperinsulinism promise to change this in the next few years. Unfortunately, despite all these advances, children with hyperinsulinism around the world continue to experience neurological sequelae at high rates, highlighting the importance of early diagnosis and effective treatment.
Assuntos
Palavras-chave

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Hiperinsulinismo Congênito Tipo de estudo: Screening_studies Limite: Child / Humans / Infant / Newborn Idioma: En Ano de publicação: 2022 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Hiperinsulinismo Congênito Tipo de estudo: Screening_studies Limite: Child / Humans / Infant / Newborn Idioma: En Ano de publicação: 2022 Tipo de documento: Article