Pulse Brain · Growing Health Evidence Index
Peer-reviewed

Development and validation of clinical criteria to identify familial chylomicronemia syndrome (FCS) in North America

Robert A. Hegele, Zahid Ahmad, Ambika P. Ashraf, Andrew Baldassarra, Alan S. Brown, Alan Chait, Steven D. Freedman, Brenda Kohn, Michael Miller, Nivedita Patni, Daniel Soffer, Jian Wang, Michael S. Broder, Eunice Chang, Irina Yermilov, Cynthia Campos, Sarah N. Gibbs

Journal of clinical lipidology · 2024

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Summary

BACKGROUND: Familial chylomicronemia syndrome (FCS) is an ultrarare inherited disorder. Genetic testing is not always feasible or conclusive. European clinicians developed a "FCS score" to differentiate between FCS and multifactorial chylomicronemia syndrome (MCS), a more common condition with overlapping features. A diagnostic score has not been developed for use in the North American (NA) context. OBJECTIVE: To develop and validate a diagnostic score for NA patients based on signs, symptoms and biochemical traits of FCS. METHODS: Using the RAND/UCLA modified Delphi process, we convened 10 US/Canadian physicians with experience recognizing and treating FCS and 1 adult patient with FCS. The panel developed and rated 296 scenarios describing patients with FCS. Linear regression analyses use

Source type
Peer-reviewed study
DOI
10.1016/j.jacl.2024.09.008
Catalogue ID
SNmojj1ioh-a5lvme
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