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Persistent hyperinsulinaemic hypoglycaemia of infancy (nesidioblastosis): a report from Kuwait

D G Ramadan1, M H Badawi, M Zaki

  • 1Department of Paediatrics of Sabah, Hospital, Kuwait.

Annals of Tropical Paediatrics
|December 22, 1999
PubMed

Insights

Persistent hyperinsulinaemic hypoglycaemia (PHHI) in Bedouin children presents with seizures and severe hypoglycemia. Early diagnosis and treatment are crucial for neurodevelopmental outcomes.

Area of Science:

  • Pediatrics
  • Endocrinology
  • Genetics

Background:

  • Persistent hyperinsulinaemic hypoglycaemia (PHHI) is a rare but serious condition.
  • The Bedouin community in Kuwait exhibits a high incidence of PHHI, suggesting a genetic component.
  • PHHI can lead to severe neurological complications if not managed promptly.

Purpose of the Study:

  • To describe the clinical presentation, management, and outcomes of PHHI in Bedouin children.
  • To highlight the high incidence and potential genetic inheritance patterns of PHHI in an inbred community.
  • To emphasize the importance of early diagnosis and intervention for PHHI.

Main Methods:

  • Retrospective case series of nine Bedouin children with PHHI over 13 years.
  • Review of clinical data including presentation, treatment modalities (diazoxide, octreotide, pancreatectomy), and neurodevelopmental outcomes.
  • Analysis of family history to identify inheritance patterns.

Main Results:

  • Nine Bedouin children diagnosed with PHHI, with a high incidence of 1:20,000 in this community.
  • Seizures and severe recurrent hypoglycemia were common presentations, often in the neonatal period.
  • Treatment outcomes varied: diazoxide achieved remission in one, octreotide was successful in another, and four underwent pancreatectomy.
  • Two children had normal neurodevelopment, four experienced mental retardation, and three died.
  • Nesidioblastosis was observed in all surgically treated patients (diffuse in two, localized in two).

Conclusions:

  • PHHI in this Bedouin population has a high incidence and is potentially linked to autosomal recessive inheritance.
  • Prompt diagnosis and management, including medical and surgical options, are vital to prevent severe neurological damage.
  • Genetic counseling is recommended for families with affected siblings due to the possibility of inherited forms of PHHI.

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