publication . Article . 2008

Congenital hyperinsulinism - clinical review and case report

Bandeira, A.; Cardoso, C.; Sizenando, J.; Proença, E.; Martins, E.;
Open Access Portuguese
  • Published: 01 Sep 2008
  • Publisher: Nascer e Crescer
  • Country: Portugal
Abstract
O hiperinsulinismo congénito constitui a causa mais frequente de hipoglicemia persistente no recém-nascido e lactente e engloba um grupo heterógeneo de defeitos genéticos que afectam o metabolismo da secreção de insulina. Os autores apresentam três casos clínicos de hiperinsulinismo congénito. Os três casos ilustram a diversidade na apresentação clínica, desde a iritabilidade, a um quadro clínico de sepsis ou convulsões neonatais. A necessidade de grandes aportes de glicose para corrigir e manter a normoglicemia pode levantar a suspeita do diagnóstico, assim como a resposta clínica ao glucagon. Num dos casos foi realizado cateterismo pancreático por imagem suspe...
Subjects
free text keywords: Hiperinsulinismo congénito, diazóxido, hipoglicemia, Congenital hyperinsulinism, diazoxide, hypoglycaemia
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