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   Articles with "familial chylomicronemia" as a keyword



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Disappearance of recurrent pancreatitis after splenectomy in familial chylomicronemia syndrome.

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recommendations! Published in 2018 at "Atherosclerosis"

DOI: 10.1016/j.atherosclerosis.2018.06.870

Abstract: BACKGROUND AND AIMS Recurrent pancreatitis is a severe complication of familial chylomicronemia syndrome (FCS) mainly secondary to lipoprotein lipase deficiency. The mechanism and interindividual variability of pancreatitis in FCS are not fully understood, but abnormalities… read more here.

Keywords: pancreatitis; familial chylomicronemia; disappearance; recurrent pancreatitis ... See more keywords
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Estimating health state utilities associated with a rare disease: familial chylomicronemia syndrome (FCS)

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recommendations! Published in 2020 at "Journal of Medical Economics"

DOI: 10.1080/13696998.2020.1776719

Abstract: Abstract Aims: Familial chylomicronemia syndrome (FCS) is a rare genetic disorder with no currently approved therapies. Treatments are in development, and cost-utility analyses will be needed to examine their value. These models will require health… read more here.

Keywords: health state; state utilities; familial chylomicronemia; utility ... See more keywords
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Building a better understanding of the burden of disease in familial chylomicronemia syndrome

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recommendations! Published in 2017 at "Expert Review of Clinical Pharmacology"

DOI: 10.1080/17512433.2017.1251839

Abstract: Familial chylomicronemia syndrome (FCS), also referred to as type 1 hyperlipoproteinemia or lipoprotein lipase deficiency, is a rare genetic lipid disorder characterized by severe elevations in cir... read more here.

Keywords: building better; familial chylomicronemia; better understanding; chylomicronemia syndrome ... See more keywords
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Advances in diagnosis and potential therapeutic options for familial chylomicronemia syndrome

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recommendations! Published in 2018 at "Expert Opinion on Orphan Drugs"

DOI: 10.1080/21678707.2018.1419863

Abstract: ABSTRACT Introduction: Familial chylomicronemia syndrome (FCS) is a rare disorder in which there is a lack of chylomicron clearance from the plasma leading to severe hypertriglyceridemia. This is classically due to deficiency in lipoprotein lipase… read more here.

Keywords: advances diagnosis; familial chylomicronemia; fcs; chylomicronemia syndrome ... See more keywords
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Efficacy of therapeutic plasma exchange in reducing the incidence of recurrent pancreatitis related to familial chylomicronemia

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recommendations! Published in 2019 at "Transfusion"

DOI: 10.1111/trf.15532

Abstract: Familial chylomicronemia syndrome (FCS) is caused by a genetic defect in triglyceride (TG) metabolism that leads to severe hypertriglyceridemia, which in turn is associated with multiple morbidities and may cause severe pancreatitis that is both… read more here.

Keywords: pancreatitis; familial chylomicronemia; plasma exchange; recurrent ... See more keywords

OR21-3 Familial Chylomicronemia Syndrome: Distinguishing the Rare Among the Common in Adults for Appropriate Management

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recommendations! Published in 2019 at "Journal of the Endocrine Society"

DOI: 10.1210/js.2019-or21-3

Abstract: Abstract Background: Hypertriglyceridemia (HTG) is common, but familial chylomicronemia syndrome (FCS) is a very rare cause of severe HTG, associated with pancreatitis, which can be fatal. It is due to impaired lipoprotein lipase (LPL) function,… read more here.

Keywords: pancreatitis; lpl; htg; familial chylomicronemia ... See more keywords