New Insights into the Role of Lipoprotein(a) as Predictor of Early Onset of Cardiovascular Disease in Pediatric Familial

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LETTER TO THE EDITOR

New Insights into the Role of Lipoprotein(a) as Predictor of Early Onset of Cardiovascular Disease in Pediatric Familial Hypercholesterolemia (FH) Paola Sabrina Buonuomo1   · Gerarda Mastrogiorgio1 · Michaela Carletti2 · Ippolita Rana1 · Marina Macchiaiolo1 · Michaela Veronika Gonfiantini · Davide Vecchio1 · Ottavia Porzio2 · Andrea Bartuli1 Received: 11 May 2020 / Accepted: 6 June 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020

To the Editor, Lipoprotein(a) [Lp(a)] is a marker that predicts and stratify risk in atherosclerotic cardiovascular disease (ASCVD) in adults with familial hypercholesterolemia (FH) in combination with low-density lipoprotein cholesterol (LDL-C) level [1, 2], but its role in children is debated [3, 4]. The aim of our study was to examine Lp(a) values in a homogeneous cohort of pediatric FH patients and to relate to anamnestic and laboratory data. We performed a retrospective review of 83 pediatric patients affected by FH, strictly diagnosed by SimoneBroome criteria and/or confirmed by detection of genetic FH-related mutations/deletions. We collected data on LDL-C and Lp(a) levels and familial history of ASCVD. A total of 83 children (49 females, 34 males, median age at diagnosis 8.6 years) from 76 families were included in the analysis; Lp(a) was high in 24/83 patients (29%). In 23/24 patients with Lp(a) levels over the normal range, we recognized a family history of ASCVD. Anyway, 21 patients with normal Lp(a) had a family history of ASCVD too. In addition, we did not find a significant association between Lp(a) and LDL-C in this cohort. Zawacki et al. [3] suggested that children with FH in addition to high Lp(a) may be at higher risk for ASCVD than their LDL-C alone would suggest. Similarly, Qayum et al. [4] reported that Lp(a) in a FH pediatric population was associated with a positive family history of ASCVD but they

* Paola Sabrina Buonuomo [email protected] 1



Rare Diseases and Medical Genetics, Bambino Gesù Children’s Hospital Rome, Rome, Italy



Laboratory of Chemistry, Bambino Gesù Children’s Hospital Rome, Rome, Italy

2

did not find early adverse changes in the vasculature that could be related to high Lp(a). Our findings are consistent with previous studies reporting that high Lp(a) in children may be associated with a family history of ASCVD. Indeed, the hypothesis that it may play a role in stratifying cardiovascular risk better than LDL-C alone as an independent variable remain unclear. To the best of our knowledge, it should be considered as covariable in addition to clinical/imaging findings and more studies are needed to better define its role in pediatric age.

Funding  This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

Compliance with Ethical Standards  Conflict of interest  No author has any conflict of interest. The measurement of Lp(a) level is included in local standard of care protocols. Ethical Approval  All procedures performed in st