Plasma inducible degrader of the LDLR, soluble low-density lipoprotein receptor, and proprotein convertase subtilisin/kexin type 9 levels as potential biomarkers of familial hypercholesterolemia in children

Familial hypercholesterolemia (FH) in children is under-detected. Plasma biomarkers associated with low-density lipoprotein receptor (LDLR) function could help identifying FH children. We aim to assess the clinical value of inducible degrader of the LDLR (IDOL), soluble LDLR (sLDLR), and proprotein...

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Veröffentlicht in:Journal of clinical lipidology 2018-01, Vol.12 (1), p.211-218
Hauptverfasser: Girona, Josefa, Rodríguez-Borjabad, Cèlia, Ibarretxe, Daiana, Heras, Mercedes, Amigo, Nuria, Feliu, Albert, Masana, Luis, Plana, Nuria, Amigó, E., Andrés, P., Barrio, M., Bilbao, J.Á., Bosch, M., Cabedo, J.L., Calvo, J., Campillo, C., Castejón, E., Castillejo, G., Castro, M., Cliville, R., De Gotardo, E., Domènech, V., Domínguez, D., Escolà, M., Fernández, M., García, J., Girona, R., Guijarro, E., Gutiérrez, M.A., Iglesias, D., Salsas, J.M., Luque, V., Machado, P., Maixé, J., Mallafré, M., Martin, R., Jiménez, M., Monne, R., Morillo, S., Naranjo, À., Pérez, C., Planelles, M., Querol, C., Rabadà, M.J., Remedi, A., Riquelme, C., Rodríguez, N., Rosell, L., Salvado, M., Salvador, O., Santos, A., Sanz, N., Segura, S., Subirana, G., Tarrades, P., Vendrell, M., Vilella, M., Zabala, E.
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Sprache:eng
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Zusammenfassung:Familial hypercholesterolemia (FH) in children is under-detected. Plasma biomarkers associated with low-density lipoprotein receptor (LDLR) function could help identifying FH children. We aim to assess the clinical value of inducible degrader of the LDLR (IDOL), soluble LDLR (sLDLR), and proprotein convertase subtilisin/kexin type 9 (PCSK9) plasma concentrations in children with FH compared with control children (CCh). This was a cross-sectional study performed in a Lipid Unit from a University hospital. The participants were 177 children distributed into FH (n = 77) and CCh (n = 100). Main outcomes were changes in IDOL, sLDLR, and PCSK9 plasma concentrations between children groups; secondary outcomes were the association between IDOL, sLDLR, and PCSK9 and lipid profile determined by 2-dimensional nuclear magnetic resonance. The IDOL levels were higher in FH compared with CCh (P = .007). The PCSK9 levels were elevated in FH (P 
ISSN:1933-2874
1876-4789
DOI:10.1016/j.jacl.2017.10.003