Cesarean section under spinal anesthesia in a first-time gravida with Fabry disease

Fabry disease (FD) (also Fabry Syndrome) is an x-linked inherited lysosomal storage disease characterized by reduced or absent activity of the enzyme α-galactosidase A.1 This leads to accumulation of globotriaosylceramide (Gb3) in various organs such as skin, heart, kidneys, brain, eyes, vascular and nervous systems, with corresponding organ dysfunction.2 Initial symptoms are often nonspecific, such that FD is usually diagnosed late and after se vere symptoms such as stroke, heart or kidney failure are present.
Source: International Journal of Obstetric Anesthesia - Category: Anesthesiology Authors: Tags: Correspondence Source Type: research