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Risk for ingestion of toxic substances in children with Prader-Willi syndrome

Shawn E McCandless1, Karen Potter Powell, Ulrika Sandberg

  • 1Case Western Reserve University and University Hospitals Case Medical Center, Cleveland, Ohio, USA. shawn.mccandless@case.edu

American Journal of Medical Genetics. Part A
|September 19, 2012
PubMed

Insights

Individuals with Prader-Willi syndrome (PWS) face a significantly higher risk of ingesting toxins. This increased risk, linked to PWS behaviors, necessitates greater awareness among healthcare providers and poison control centers.

Area of Science:

  • Genetics
  • Pediatrics
  • Toxicology

Background:

  • Prader-Willi syndrome (PWS) is associated with behaviors that may increase the risk of ingesting dangerous items.
  • Limited data exists on the prevalence of toxic ingestions in individuals with PWS compared to controls.

Purpose of the Study:

  • To determine if individuals with PWS have a higher prevalence of toxic ingestions.
  • To identify PWS-related factors associated with toxic ingestion.
  • To inform genetic counseling and clinical management strategies.

Main Methods:

  • An online survey was administered to parents/caretakers of individuals with PWS and their non-PWS siblings.
  • Data collected included a history of toxic ingestions.
  • PWS subjects (N=130) and sibling controls (N=134) aged 2-18 years were analyzed.

Main Results:

  • Toxic ingestion was significantly more prevalent in PWS subjects (20%) compared to controls (2%).
  • PWS-related features such as food searching, pica, and decreased cognitive ability were associated with increased toxic ingestion.
  • PWS children demonstrated an approximately 12-fold increased risk of toxin ingestion.

Conclusions:

  • Individuals with PWS exhibit a substantially elevated risk of toxic ingestions.
  • Geneticists and primary care providers should be informed to guide counseling and preventative measures.
  • Poison control centers require awareness of PWS-associated factors for effective diagnosis and management of ingestions.

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