TY - JOUR
T1 - Lipoprotein(a) levels in children with homozygous familial hypercholesterolaemia
T2 - A cross-sectional study
AU - de Boer, Lotte M.
AU - Reijman, M. Doortje
AU - Hutten, Barbara A.
AU - Wiegman, Albert
N1 - Funding Information: Conflict of interest: AW reports research support from pharmaceutical trials of lipid modification agents from Amgen, Regeneron, and Novartis, and is a member of the safety board at Amryt. BAH and AW received a research grant from Silence Therapeutics that partially funded this work. LMdB and MDR have no conflicts of interest. Publisher Copyright: © 2023
PY - 2023/5/1
Y1 - 2023/5/1
N2 - Homozygous familial hypercholesterolaemia (HoFH) is a life-threatening disorder characterized by extremely elevated low-density lipoprotein cholesterol (LDL-C) levels. Untreated, severe atherosclerotic cardiovascular disease (ASCVD), including aortic valve stenosis (AVS), may already occur in childhood. Another important genetic risk factor for ASCVD and AVS is elevated lipoprotein(a) [Lp(a)], which is highly prevalent in the general paediatric population. However, data on Lp(a) in children with HoFH are scarce. Therefore, we performed a cross-sectional study to evaluate Lp(a) levels in children with HoFH and compared them to children with heterozygous FH (HeFH) and unaffected children. Adjusted least-square mean (95% CI) Lp(a) levels in HoFH (n=29), HeFH (n=101) and unaffected children (n=102) were 18.7 (12.0-29.1), 15.3 (11.8-19.8) and 10.5 (8.3-13.2) mg/dL, respectively (p-for-trend=0.007). Lp(a) levels in children with HoFH were higher than in children with HeFH and in unaffected children. Given the very high ASCVD risk with HoFH, identifying other risk factors such as elevated Lp(a) in these children is important. Therefore, Lp(a) levels should be measured at least once in all children with HoFH.
AB - Homozygous familial hypercholesterolaemia (HoFH) is a life-threatening disorder characterized by extremely elevated low-density lipoprotein cholesterol (LDL-C) levels. Untreated, severe atherosclerotic cardiovascular disease (ASCVD), including aortic valve stenosis (AVS), may already occur in childhood. Another important genetic risk factor for ASCVD and AVS is elevated lipoprotein(a) [Lp(a)], which is highly prevalent in the general paediatric population. However, data on Lp(a) in children with HoFH are scarce. Therefore, we performed a cross-sectional study to evaluate Lp(a) levels in children with HoFH and compared them to children with heterozygous FH (HeFH) and unaffected children. Adjusted least-square mean (95% CI) Lp(a) levels in HoFH (n=29), HeFH (n=101) and unaffected children (n=102) were 18.7 (12.0-29.1), 15.3 (11.8-19.8) and 10.5 (8.3-13.2) mg/dL, respectively (p-for-trend=0.007). Lp(a) levels in children with HoFH were higher than in children with HeFH and in unaffected children. Given the very high ASCVD risk with HoFH, identifying other risk factors such as elevated Lp(a) in these children is important. Therefore, Lp(a) levels should be measured at least once in all children with HoFH.
KW - Children
KW - FH
KW - Familial hypercholesterolaemia
KW - HoFH
KW - Lipoprotein(a)
KW - Lp(a)
UR - http://www.scopus.com/inward/record.url?scp=85153097821&partnerID=8YFLogxK
U2 - https://doi.org/10.1016/j.jacl.2023.03.010
DO - https://doi.org/10.1016/j.jacl.2023.03.010
M3 - Article
C2 - 37087364
SN - 1933-2874
VL - 17
SP - 415
EP - 419
JO - Journal of clinical lipidology
JF - Journal of clinical lipidology
IS - 3
ER -