ScientiaCME Updates in the management of familial chylomicronemia syndrome (FCS): best practices and emerging therapies
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Activity Description / Statement of Need:
In this online, self-learning activity:
Familial chylomicronemia syndrome (FCS) is an ultrarare genetic disease characterized by the buildup of chylomicrons – the largest lipoprotein – as a result of loss of function of one of five genes responsible for lipolysis. Its estimated prevalence is one in one million people and affects patients of all ages. The clinical presentation of FCS varies but includes patients with triglyceride (TG) levels over 10 times and up to a hundred-fold times the upper limit of normal. Complications include eruptive xanthomas on the trunk and extremities, lipemia retinalis, recurrent abdominal pain, hepatosplenomegaly, fatigue, cognitive disorientation, organ failure, necrosis, and pancreatitis, the latter of which is associated with a five- to 30-percent mortality rate in patients affected by FCS. Unfortunately, due in part to the rarity of the condition, patients are commonly undiagnosed, even by endocrinologists.
HCPs including: endocrinologists, cardiologists, and primary care physicians; physician assistants, nurse practitioners, and pharmacists who practice in the aforementioned areas of specialty; and those who otherwise have an interest in or commonly care for or clinically encounter patients with FCS.