[A teenager with Prader-Willi syndrome with loss of appetite].
Клучни зборови
Апстракт
BACKGROUND
Prader-Willi syndrome is characterised by hyperphagia and binge eating, without regurgitation.
METHODS
We present a 16-year-old girl with Prader-Willi syndrome exhibiting loss of appetite, stomach ache and regurgitation. Gastro-enteritis was suspected. However, she rapidly developed severe septic shock. During emergency surgery, a fully necrotic and ruptured stomach was seen. Despite respiratory, haemodynamic and surgical efforts, the patient died of necrotic intestinal bleeding.
CONCLUSIONS
Binge eating or deviant gastric homeostasis could account for the relatively high incidence of gastric necrosis in patients with Prader-Willi syndrome. Loss of appetite and regurgitation in patients with this syndrome should be considered as warning signs of a possible life-threatening disorder.
