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

• Published in: American journal of medical genetics. Part A Vol. 158A; no. 11; pp. 2866 - 2869
• Main Authors: McCandless, Shawn E., Powell, Karen Potter, Sandberg, Ulrika
• Format: Journal Article
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 01.11.2012; Wiley-Liss; Wiley Subscription Services, Inc
• Subjects: Adolescent; Biological and medical sciences; Child; Child, Preschool; Complex syndromes; Feeding Behavior; Hazardous Substances; Humans; Medical genetics; Medical sciences; Metabolic diseases; Obesity; poisoning; Prader-Willi Syndrome - epidemiology; Prader–Willi syndrome; Prevalence; Risk; Surveys and Questionnaires; toxic ingestion; Endocrinopathy; Human; Diseases of the osteoarticular system; Risk factor; Prader—Willi syndrome; Risk; Poisoning; Complex syndrome; Child; Prader Labhart Willi syndrome; toxic ingestion; Genetic disease
• DOI: 10.1002/ajmg.a.34315
• ISSN: 1552-4825; 1552-4833

Individuals with Prader–Willi syndrome (PWS) have several common findings that may predispose to ingestion of potentially dangerous items. This study examined whether individuals with PWS have an increased prevalence of toxic ingestions. A survey regarding history of ingestions in PWS individuals and sibling controls was designed, piloted, and distributed on‐line. The subjects were individuals with PWS (N = 129). The subjects' non‐PWS siblings served as controls (N = 134). Participants who completed the anonymous online survey were either the parents or the primary caretaker of individuals with PWS. Responses were submitted by 141 participants, providing information about 130 PWS subjects (M/F: 66:64) and 134 sibling controls. Subjects and controls ranged in age from 2 to 18 years at the time of the survey. Eleven participants did not answer the questions regarding ingestions. History of toxic ingestion was more prevalent in PWS subjects (20% vs. 2% of controls). Several features of PWS, including history of searching for food and eating unusual objects, along with decreased cognitive ability, appeared to associate with increased prevalence of toxic ingestion in PWS individuals. PWS children appear to have an ∼12‐fold increased risk of ingesting toxins compared to the general population. Geneticists should include this information in counseling and in recommendations to primary care providers. Also, poison control centers need to be aware of this association and of the physiological and behavioral aspects of PWS that may complicate the diagnosis and management of a toxic ingestion. © 2012 Wiley Periodicals, Inc.