Author/Authors :
Espahbodi، Ebrahim نويسنده Department of Anesthesiology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran. AND Department of Anesthesiology, Farabi Hospital, Tehran University of Medical Sciences, Tehran, Iran. Espahbodi, Ebrahim , Yaghooti، Amir Abbas نويسنده Anesthesiology and Critical Care, Tehran University of
Medical Sciences, Tehran, IR Iran , , Ostadalipour، Abbas نويسنده Anesthesiology and Critical Care, Tehran University of
Medical Sciences, Tehran, IR Iran , , Marashi، Shaqayeq نويسنده Department of Anesthesiology, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran. Marashi, Shaqayeq
Abstract :
Niemann-Pick is a lipid storage disease that results from a
lysosomal enzyme deficiency (sphingomyelinase). It has different
presentations, and it may affect various organs such as the central
nervous system, kidney, liver, and spleen. Due to the complexity of the
disease, careful perianesthetic management is necessary in order to
reduce the risks and sequels. As there is little evidence available in
the literature regarding the anesthetic implications of such patients,
in this case report we describe the anesthetic management of a
two-year-old female with Niemann-Pick disease.
