Fatal Postpartum Hemorrhage in a Patient with Niemann-Pick Disease Type B.

In conclusion, physicians should keep in mind that patients with a history of splenectomy and/or uncontrollable hemorrhage must be carefully evaluated for rare diseases like lysosomal storage diseases and that NPD B can cause mortality because of postpartum bleeding. Adult intensivists should be familiar with adult presentations of rare metabolic or genetic diseases as more and more children with metabolic or genetic diseases will survive to adulthood and will be admitted to and unfortunately will even die in the adult ICU.