Gastric rupture and necrosis in Prader-Willi syndrome.
Hyperphagia and obesity are common features in individuals with Prader-Willi syndrome (PWS). Demographic and cause-of-death data from individuals with PWS were obtained through a national support organization. Four reports of unexpected mortality due to gastric rupture and necrosis were found in 152 reported deaths, accounting for 3% of the causes of mortality. Four additional individuals were suspected to have gastric rupture. Vomiting and abdominal pain, although rare in PWS, were frequent findings in this cohort. The physician should consider an emergent evaluation for gastric rupture and necrosis in individuals with PWS who present with vomiting and abdominal pain.
Department of Pediatrics, Division of Medical Genetics, University of Utah, Salt Lake City, UT 84132, USA. david.stevenson@hsc.utah.edu
David A
DA
Stevenson
Janalee
J
Heinemann
Moris
M
Angulo
Merlin G
MG
Butler
Jim
J
Loker
Norma
N
Rupe
Patrick
P
Kendell
Suzanne B
SB
Cassidy
Ann
A
Scheimann
D015746:Abdominal Pain / Q000209:etiology; D000293:Adolescent; D000328:Adult; D002423:Cause of Death; D002648:Child; D005260:Female; D013271:Gastric Dilatation / Q000150:complications; D006801:Humans; D008297:Male; D008875:Middle Aged; D009336:Necrosis / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology; D011218:Prader-Willi Syndrome / Q000150:complications*; D013275:Stomach Rupture / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology; D014839:Vomiting / Q000209:etiology