Unexpected death due to refractory metabolic acidosis and massive hemolysis in a young infant with Prader-Willi

To our knowledge, massive acute hemolysis has not yet reported among the catastrophic events related to the demise of patients with PWS. We believe that this phenomenon might have played a decisive role in the terminal course of our patient, even though the causative mechanism remains unclear. While further studies are needed in order to better clarify this issue, we suggest a close surveillance of these patients during acute diseases such as gastroenteritis and upper respiratory tract infections or when anticonvulsant treatment must be provided