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Lp(a) and Pediatric Population
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2011 Dec;128 Suppl 5:S213-56. doi: 10.1542/peds.2009-2107C. Epub 2011 Nov 14.

 

Expert panel on integrated guidelines for cardiovascular health and risk reduction in children and adolescents: summary report.

  

 

In terms of other lipid measurements, (1) most but not all studies have found that measurement of apolipoprotein B and apolipoprotein A-1 for universal screening provides no additional advantage over measuring non-HDL cholesterol, LDL cholesterol, and HDL cholesterol levels, (2) measurement of lipoprotein(a) is useful in the assessment of children with both hemorrhagic and ischemic stroke, (3) in offspring of a parent with premature CVD and no other identifiable risk factors, elevations of apolipoprotein B, apolipoprotein A-1, and lipoprotein(a) have been noted, and (4) measurement of lipoprotein subclasses and their sizes by advanced lipoprotein testing has not been found to have sufficient clinical utility in children at this time (grade B).

 

2015 Sep-Oct;9(5 Suppl):S57-66. doi: 10.1016/j.jacl.2015.07.006. Epub 2015 Jul 21.

Click title below to see full article.

Lipoprotein(a): Its relevance to the pediatric population.

Abstract

 

Lipoprotein(a) (Lp(a)) is a highly atherogenic and heterogeneous lipoprotein that is inherited in an autosomal codominant trait. A unique aspect of this lipoprotein is that it is fully expressed by the first or second year of life in children, a pattern that is distinctly different from other lipoproteins, which typically only reach adult levels after adolescence. Despite decades of research, Lp(a) metabolism is still poorly understood but what is abundantly clear is that it is an independent risk factor for atherosclerotic cardiovascular disease (ASCVD). The Expert Panel on Integrated Guidelines for Cardiovascular Health and Risk Reduction in Children and Adolescents does not recommend measuring Lp(a) levels as part of routine screening except in youth with an ischemic or hemorrhagic stroke or youth with a parental history of ASCVD not explained by classical risk factors. One of the reasons that both the pediatric and adult guidelines fail to include this lipoprotein as part of routine lipid screening is the absence of data to show that lowering Lp(a) will reduce current or future ASCVD risk independently of low-density lipoprotein cholesterol (LDL-C) lowering. The cholesterol carried by Lp(a) is included in the low-density lipoprotein cholesterol measurement, but a separate test is used to measure the lipoprotein mass and/or cholesterol carried only by Lp(a). Because levels seem to be largely under genetic control, studies of lifestyle modification have been inconclusive although one study in obese children showed a decrease in the Lp(a) level comparable with the favorable effect on other lipids. The most compelling data regarding the importance of Lp(a) in the pediatric population are the increased risk associated with arterial ischemic stroke, a risk that is comparable with that associated with antiphospholipid antibodies or protein C deficiency. Although no specific pharmaceutical treatments are recommended to lower Lp(a) levels in youth, it is vitally important to educate youth and their parents about the excessive risk associated with this lipoprotein and the need to avoid the acquisition of other lifestyle-related risk factors such as smoking, excess weight, and physical inactivity to preserve more ideal cardiovascular health in adulthood.

2014 May 20;63(19):1982-9. doi: 10.1016/j.jacc.2014.01.063. Epub 2014 Mar 13.


Lipoprotein(a) levels in familial hypercholesterolemia: an important predictor of cardiovascular disease independent of the type of LDL receptor mutation.


Abstract

OBJECTIVES:


The aim of this study was to determine the relationship between lipoprotein(a) [Lp(a)] and cardiovascular disease (CVD) in a large cohort of patients with heterozygous familial hypercholesterolemia (FH).

BACKGROUND:


Lp(a) is considered a cardiovascular risk factor. Nevertheless, the role of Lp(a) as a predictor of CVD in patients with FH has been a controversial issue.

METHODS:


A cross-sectional analysis of 1,960 patients with FH and 957 non-FH relatives recruited for SAFEHEART (Spanish Familial Hypercholesterolemia Cohort Study), a long-term observational cohort study of a molecularly well-defined FH study group, was performed. Lp(a) concentrations were measured in plasma using an immunoturbidimetric method.

RESULTS:


Patients with FH, especially those with CVD, had higher Lp(a) plasma levels compared with their unaffected relatives (p < 0.001). A significant difference in Lp(a) levels was observed when the most frequent null and defective mutations in LDLR mutations were analyzed (p < 0.0016). On multivariate analysis, Lp(a) was an independent predictor of cardiovascular disease. Patients carrying null mutations and Lp(a) levels >50 mg/dl showed the highest cardiovascular risk compared with patients carrying the same mutations and Lp(a) levels <50 mg/dl.

CONCLUSIONS:


Lp(a) is an independent predictor of CVD in men and women with FH. The risk of CVD is higher in those patients with an Lp(a) level >50 mg/dl and carrying a receptor-negative mutation in the LDLR gene compared with other less severe mutations.

 

2007 Aug;37(2):121-6.


Risk factors for pediatric stroke: consequences for therapy and quality of life.

Abstract


A retrospective population-based study was conducted to calculate incidence of pediatric stroke, detect risk factors, determine long term outcome and to optimize therapy. Patients (age: >1 month and <19 years) with stroke in Vorarlberg, Austria, from 1984-2005 were investigated. Outcome was evaluated in terms of neurological deficits, neuropsychological measures, and quality of life. Consequences on therapy were established according to current guidelines. Twenty-two children, median age 6 years (range, 0.9-14) years, 16 ischemic and 6 hemorrhagic stroke, correspond to an incidence of 1.96 and 0.74/100.000 child-years, respectively. No child died; one had a recurrent stroke. Twenty children (12 boys) were included after a median time of 3.7 years (range, 0.4-18). Risk factors were vasculopathy (17/20; 85%), lipometabolic disorders (17/20; 85%), and prothrombotic abnormality (10/20; 50%). Three children had no risk factor; four children had a reduced quality of life. The study led to therapeutic consequences in 13 of 20 children (65%): aspirin (5/20; 25%), folic acid in (3/20; 15%), and rehabilitative therapy (9/20; 45%). Most children with pediatric stroke have more than one risk factor, mainly vasculopathies or elevation of lipoprotein(a). Overall outcome is determined solely by neurological deficits and is positively influenced by good quality of life.

 


ELEVATED LIPOPROTEIN(A) AND ISCHEMIC STROKES- A CASE REPORT
Objectives

Elevated Lipoprotein (a) (Lp(a)) is an independent risk factor for Cerebrovascular Disease (CVD) by promoting atherosclerosis, inflammation and impaired fibrinolysis. Children with elevated Lp(a), compared to normal children, suffer a four fold increase in the risk of a cerebral thrombotic event (CTE) and following this there is a ten fold increase in the risk of a repeat event within the first year. Lipoprotein-apheresis (LA) is presently the only therapy with demonstrated success in significantly lowering Lp(a) and CVD risk.  

Methods

An eleven year old boy presented with a five day history of headaches, dizziness, lethargy, altered mental status, visual field and memory deficits. MRI of the brain revealed infarcted areas in the parietal, temporal, cerebellar, thalamic and bilateral frontal lobe while CT angiogram revealed occlusion of the Posterior Cerebral Artery. Past medical history, hematologic, rheumatologic and oncologic lab workup were non-significant. The only identifiable risk factor was an elevated Lp(a).His neurological symptoms deteriorated and CT angiogram revealed evolving basilar artery thrombus. Thrombectomy allowed near complete recanalization of the basilar artery. Due to his Lp(a) level and continued CVD, Biweekly LA was initiated.The patient tolerated his procedures and following six LA treatments demonstrated significant improvement in neurological function.


Results

Parameters Measured Pre LA Post LA
Lp(a)- C (Normal<3mg/dL) 33 10
Lp(a)- Mass (Normal<30mg/dL) 148 28
Lp(a)- P (Normal<75nmol/L,) 273 Undetected Levels
LDL-C (Normal<100mg/dL) 55 9
Apo B (Normal<60mg/dL) 40 <5
Apo A1 (Normal>131mg/dL) 111 106
Fibrinogen (Normal<390mg/dL) 449 238


Conclusions

LA can be used safely for pediatric patients with CTE due to elevated Lp(a).

 
Co-authors
P. Moriarty 1, N. Sehar 1, L. Denney 1, F. Perez-Marques 2, A. Panchal 2, P. Luna 2
1University Of Kansas Medical Center, Clinical Pharmacology, Kansas City, USA
2University Of Kansas Medical Center, Pediatrics, Kansas City, USA


 

 

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