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Diagnosis and treatment of hyperinsulinaemic hypoglycaemia and its implications for paediatric endocrinology

Huseyin Demirbilek1, Sofia A Rahman2, Gonul Gulal Buyukyilmaz1

  • 1Department of Paediatric Endocrinology, Hacettepe University, Faculty of Medicine, Ankara, Turkey.

International Journal of Pediatric Endocrinology
|September 1, 2017
PubMed

Insights

Hyperinsulinaemic hypoglycaemia (HH) involves inappropriate insulin secretion, causing dangerous low blood sugar in children. Prompt diagnosis and treatment are vital to prevent brain damage.

Area of Science:

  • Pediatric Endocrinology
  • Metabolic Disorders
  • Molecular Genetics

Background:

  • Glucose homeostasis is tightly regulated by insulin, secreted by pancreatic β-cells.
  • Hyperinsulinaemic hypoglycaemia (HH) is a critical condition in neonates and children, characterized by excessive insulin secretion despite low glucose levels.
  • HH can lead to severe hypoketotic hypoglycaemia and irreversible brain damage if not promptly managed.

Purpose of the Study:

  • To provide an updated review of hyperinsulinaemic hypoglycaemia (HH) in children.
  • To cover the diagnosis, molecular genetics, and therapeutic strategies for HH.
  • To highlight recent advances in managing HH and improving patient outcomes.

Main Methods:

  • Review of current literature on glucose homeostasis and hyperinsulinaemic hypoglycaemia.
  • Discussion of genetic mutations underlying congenital HH (e.g., ABCC8, KCNJ11).
  • Examination of diagnostic tools including 18F-DOPA PET-CT and therapeutic options like medical management and surgery.

Main Results:

  • Mutations in 12 key genes are identified as the molecular basis for congenital HH.
  • Advances in molecular genetics and imaging (18F-DOPA PET-CT) have improved diagnostic accuracy.
  • Modern therapeutic approaches, including medical and surgical interventions, have enhanced patient outcomes.

Conclusions:

  • Early diagnosis and intervention are crucial for preventing neurological complications in children with HH.
  • Understanding the molecular genetics of HH guides diagnosis and treatment strategies.
  • Multidisciplinary management, incorporating genetics, imaging, and tailored therapies, is essential for optimal outcomes in HH patients.

Keywords:
ChildrenCongenital hyperinsulinaemiaDiffuseFocalHyperinsulinaemic hypoglycaemiaSirolimus

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