Familial chylomicronemia syndrome (FCS) is an ultra-rare genetic disorder characterized by the
abnormal build-up of chylomicrons the largest type of lipoprotein which transport dietary fat
from the gut to the rest of the body. Patients with FCS often experience severe symptoms the
most feared of which is acute potentially life-threatening pancreatitis. This resource is
intended to raise awareness of FCS among all members of the healthcare team who come into
contact with patients with FCS with the aim of earlier diagnosis and management thus
preventing some of the more devastating physical neurological and cognitive symptoms of the
disorder. Table of Contents: -Terminology etiology and pathophysiology -Diagnosis
-Complications -Management and prevention -Research directions