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Hypoglycemia in Prader-Willi syndrome.
Rena A Harrington1, David A Weinstein, Jennifer L Miller
1Pediatric Endocrinology, University of Florida, Gainesville, Florida.
American Journal of Medical Genetics. Part A
|January 25, 2014
Summary
Infants with Prader-Willi syndrome (PWS) may be prone to hypoglycemia from birth. Early detection and treatment of hypoglycemia in PWS patients could improve neurocognitive outcomes.
Area of Science:
- Pediatrics
- Endocrinology
- Genetics
Background:
- Prader-Willi syndrome (PWS) is a complex genetic disorder.
- Mouse models suggest hypoglycemia in PWS, but human data is limited.
- Adrenal and growth hormone deficiencies in PWS increase hypoglycemia risk.
Purpose of the Study:
- To investigate the incidence and characteristics of hypoglycemia in human infants with Prader-Willi syndrome.
- To determine if hypoglycemia is a significant issue in early life for children with PWS.
Main Methods:
- Retrospective review of medical records for 95 patients with PWS aged 2 months to 5 years.
- Analysis of hypoglycemia occurrence, severity, timing, and associated factors.
- Definition of repeated hypoglycemia based on blood glucose (BG) levels and age.
Main Results:
- Hypoglycemia was recorded in 12.6% (12/95) of patients with PWS.
- Six patients had BG levels <40 mg/dl; seven experienced their first episode within the first day of life.
- Repeated hypoglycemia occurred in 83% of those with hypoglycemia; only two had documented adrenal insufficiency.
Conclusions:
- Infants with Prader-Willi syndrome may have a predisposition to hypoglycemia from birth.
- Further research is needed to confirm these findings and identify the causes of hypoglycemia.
- Early detection and management of hypoglycemia in PWS could potentially enhance neurocognitive development.
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