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Persistent hyperinsulinemic hypoglycemia of infancy: experience with 28 cases

A al-Rabeeah1, A al-Ashwal, A al-Herbish

  • 1Department of Pediatric Surgery, King Faisal Specialist Hospital, Riyadh, Saudi Arabia.

Journal of Pediatric Surgery
|August 1, 1995
PubMed
Summary

Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) in infants often correlates with consanguinity and family history. Early diagnosis and treatment, including pancreatectomy, lead to excellent outcomes for most PHHI patients.

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Area of Science:

  • Pediatrics
  • Endocrinology
  • Genetics

Background:

  • Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is a rare condition causing severe low blood sugar in newborns.
  • Consanguinity and family history are significant factors in PHHI cases.

Purpose of the Study:

  • To analyze clinical presentation, diagnosis, and treatment outcomes of PHHI in a cohort of infants.
  • To highlight the correlation between PHHI, consanguinity, and family history.

Main Methods:

  • Retrospective review of 28 infants diagnosed with PHHI over 10 years.
  • Assessment of clinical presentation, diagnostic criteria (glucose requirement, glucagon response, insulin-to-glucose ratio), and treatment modalities (pancreatectomy, medical therapy).

Main Results:

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  • Jitters and seizures were the primary clinical signs. High insulin-to-glucose ratios were observed in all patients.
  • Near-total pancreatectomy (90%) was performed on 22 infants, with excellent results in most cases.
  • Twelve infants had pre-referral brain injury; no deaths occurred, and only one patient experienced post-surgical malabsorption.

Conclusions:

  • PHHI is strongly associated with consanguinity and family history, underscoring the importance of genetic counseling.
  • Early diagnosis and prompt intervention, including surgical or medical management, are crucial for favorable outcomes in PHHI.
  • Clinical awareness and timely referral are essential to prevent severe neurological complications in infants with PHHI.