Congenital hyperinsulinism: report of 4 clinical cases and management challenges in a developing country

Authors

  • Miguel Ángel De los Santos La Torre
  • Carlos Manuel Del Águila Villar
  • Oswaldo Nuñez Almache
  • Eliana Manuela Chávez Tejada
  • Oscar Antonio Espinoza Robles
  • Paola Marianella Pinto Ibárcena
  • Martha Rosario Calagua Quispe

DOI:

https://doi.org/10.61651/rped.2018v70n2p17-22

Keywords:

Congenital Hyperinsulinism, Hypoglycemia

Abstract

Congenital hyperinsulinism (CHI) is a clinical heterogeneous disorder with great variability regarding its clinical phenotype. Two well defined histopathological forms exist: focal and diffuse lesion, both with different genetic basis but they shared a similar outcome if not treated accordingly, may cause severe hypoglycemia with significant neurological impairment. At Instituto Nacional de Salud del Niño we assessed 4 early onset cases of CHI (three males, one female) reporting presenting clinical characteristics, biomarkers of hyperinsulinism also the genetic analysis, work up and treatment. We addressed the existing challenges in our country concerning management of this complex metabolic disorder and a brief discussion of the perspectives on future developments.

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Published

2018-12-31

How to Cite

1.
De los Santos La Torre M Ángel, Del Águila Villar CM, Nuñez Almache O, Chávez Tejada EM, Espinoza Robles OA, Pinto Ibárcena PM, et al. Congenital hyperinsulinism: report of 4 clinical cases and management challenges in a developing country. Rev Peru Pediatr [Internet]. 2018 Dec. 31 [cited 2024 Nov. 21];70(2):17-22. Available from: https://pediatria.pe/index.php/pedperu/article/view/294

Issue

Vol. 70 Num. 2 (2018)

Section

CLINICAL CASE

Categories

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