Unexplained hypoglycemia during continuous nocturnal gastric drip-feeding in a patient with glycogen storage disease type ia: is it a dumping-like syndrome?
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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.
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.
Abstract:
A 5 years old boy affected with Glycogen Storage Disease type Ia (GSD-Ia) with previous optimal metabolic control developed severe erratic hypoglycemic episodes during continuous nocturnal gastric drip-feeding (CNGDF) administered by nasogastric tube. The episodes of hypoglycemia were not related to pump failure or human errors or wrong position of the tube in the gastrointestinal tract. Hyperinsulinism was also considered in this patient but it was excluded mainly because hypoglycemia was only nocturnal. Moreover, hypoglycemic episodes disappeared when CNGDF was stopped and he was fed with normal meals. The fact that hypoglycemia resolved after stopping CNGDF when nocturnal meals were introduced led us to hypothesize that CNGDF rich with simple carbohydrates might have been the cause of a sort of dumping-like syndrome. Dumping syndrome (DS) develops when a large amount of carbohydrate reaches the small intestine due to rapid gastric emptying (Tack et al. 2009; Hejazi et al. 2010). We suppose that CNGDF induced a disturbance of gastric motility with a gastric accumulation of fluids at a certain time of the night followed by a rapid voiding of the stomach leading to DS.
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