The Chylomicronemia Syndrome Is Most Often Multifactorial: A Narrative Review of Causes and Treatment.
Ann Intern Med
; 170(9): 626-634, 2019 05 07.
Article
em En
| MEDLINE
| ID: mdl-31035285
ABSTRACT
The chylomicronemia syndrome occurs when triglyceride levels are severely elevated (usually >16.95 mmol/L [1500 mg/dL]) and is characterized by such clinical features as abdominal pain, acute pancreatitis, eruptive xanthomas, and lipemia retinalis. It may result from 1 of 3 conditions the presence of secondary forms of hypertriglyceridemia concurrent with genetic causes of hypertriglyceridemia, termed multifactorial chylomicronemia syndrome (MFCS); a deficiency in the enzyme lipoprotein lipase and some associated proteins, termed familial chylomicronemia syndrome (FCS); or familial partial lipodystrophy. Most chylomicronemia syndrome cases are the result of MFCS; FCS is very rare. In all these conditions, triglyceride-rich lipoproteins accumulate because of impaired plasma clearance. This review describes the 3 major causes of the chylomicronemia syndrome; their consequences; and the approaches to treatment, which differ considerably by group.
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1
Base de dados:
MEDLINE
Assunto principal:
Hiperlipoproteinemia Tipo I
Idioma:
En
Ano de publicação:
2019
Tipo de documento:
Article