Unexplained hypoglycemia during continuous nocturnal gastric drip-feeding in a patient with glycogen storage disease

A Brambilla1, A Pozzoli, F Furlan

  • 1Metabolic Unit, Department of Pediatrics, MBBM Foundation, University of Milano Bicocca, San Gerardo Hospital, Via Pergolesi 33, 20900, Monza (MB), Italy, alebramby@libero.it.

JIMD Reports
|February 23, 2013
PubMed

Insights

Continuous nocturnal gastric drip-feeding (CNGDF) in a child with Glycogen Storage Disease type Ia (GSD-Ia) may trigger dumping-like syndrome. Stopping CNGDF and introducing normal meals resolved nocturnal hypoglycemia episodes.

Area of Science:

  • Pediatric Endocrinology
  • Gastroenterology
  • Metabolic Disorders

Background:

  • Glycogen Storage Disease type Ia (GSD-Ia) requires careful metabolic management.
  • Continuous nocturnal gastric drip-feeding (CNGDF) is a method for nutritional support.
  • Unexplained severe hypoglycemia occurred in a GSD-Ia patient on CNGDF.

Purpose of the Study:

  • To investigate the cause of severe nocturnal hypoglycemia in a GSD-Ia patient on CNGDF.
  • To explore the potential link between CNGDF and dumping-like syndrome.

Main Methods:

  • Case study of a 5-year-old boy with GSD-Ia.
  • Monitoring for hypoglycemia during CNGDF.
  • Comparison of outcomes with and without CNGDF.
  • Exclusion of other causes like pump failure, human error, tube malposition, and hyperinsulinism.

Main Results:

  • Severe, erratic hypoglycemic episodes occurred exclusively during CNGDF.
  • Hypoglycemia resolved completely upon cessation of CNGDF and introduction of normal meals.
  • Nocturnal hypoglycemia was not attributable to hyperinsulinism or technical issues with feeding.

Conclusions:

  • Continuous nocturnal gastric drip-feeding, particularly with simple carbohydrates, may precipitate a dumping-like syndrome in GSD-Ia patients.
  • Disturbed gastric motility and rapid gastric emptying are hypothesized mechanisms.
  • Alternative feeding strategies may be necessary for GSD-Ia patients experiencing such complications.

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