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High Lp(a)

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hudgins
hudgins's picture
High Lp(a)

I have a 10 yo M, southasian/Indian who was sent to me for borderline LDL of 128. He is obese, BMI 96%.
Father has "high cholesterol," and in his 20-30's had stents placed as per mother. His parents lived to 90-100's. No other FHx known.

Repeated the child's profile fasting TC 154, LDL 104, HDL 45, TG 73. I sent a lipo(a) and its 333 (normal<75).
I don't have the parent's lipid profiles yet.

Lifestyle changes are obviously necessary because of the obesity.

The very high lipo(a) and father's hx makes me worried though. Thoughts?

Thanks for answering all these (maybe easy?) questions.

AT

--
Aaron Turkish, M.D.
Assistant Professor of Clinical Pediatrics
New York Presbyterian Queens
Weill-Cornell Medical College
Pediatric Gastroenterology, Hepatology & Nutrition

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hudgins
hudgins's picture
High Lp(a)

Aaron -
 
Thanks for sharing this interesting case.  The family history and child's findings are obviously of concern.  As you know, Lp(a) is a genetically altered form of LDL-cholesterol.  In adults, it has been linked to premature CVD and premature CVD-related events. Because it has homology to plasminogen, it may also contribute to stroke. Niacin is the only generally effective intervention for lowering lipoprotein (a). However, because of its unpleasant side effects, niacin is difficult for most children to tolerate.  If niacin is used, it would be important to monitor LFTs and look for evidence of insulin resistance/hyperglycemia.  His BMI of 96% is, at best, problematic and may be a contraindication, given the association of niacin and insulin resistance.  To my knowledge, there are no long-term, prospective studies proving the benefits of lowering lipoprotein (a) in children.
 
It is important to mitigate all other possible risk factors including maintaining a normal blood pressure, avoiding smoking, 60 minutes of moderate to vigorous physical activity a day, and maintaining a prudent diet.  As a potential added benefit, you could also consider lipid-lowering therapy for his LDL 104 mg/dL.
 
As you may know, Lp (a) anti-sense oligonucleotide therapy is currently in development.  If proven safe and effective, this or other potentially emerging therapies might be helpful in the future. Therefore, mitigating his risk short term would hopefully “buy some time” until more effective therapies become available.  Finally, if it is available, noninvasive vascular imaging such serial cIMT might be informative, given the father's history of CVD-related events at such an early age.
 
As always, I am interested in hearing/learning from others.
 
Regards – Don (Wilson)

hudgins
hudgins's picture
High Lp(a)

My clinical approach, similar/based on the logic Don describes, is to focus on lifestyle. I wouldn’t treat with niacin, nor would I add statin unless the LDL rises at least above 130. But I would worry. And I’d screen relatives for Lp(a).  Interested to hear what others think. Sarah
 
Sarah de Ferranti, MD MPH
Director, Preventive Cardiology Clinic, Boston Children's Hospital
300 Longwood Avenue Boston, MA 02115 tel: 617 355-0955  fax: 617 730-0600  page: 617 355-6363 #1165  sarah.deferranti@cardio.chboston.org

hudgins
hudgins's picture
High Lp(a)

Agree that the FHx and the Lp(a) are very concerning. I do not use niacin either. I would definitely screen the family and consider treating the LDL if > 130
We don't use cIMT clinically, but do have it as a research tool.  Are other pediatric centers getting cIMT data on these type of patients if its available?
 
Sarah B. Clauss, MD
Department of Cardiology
Children's National Heart Institute
Children's National Health System
111 Michigan Ave, NW
Washington, DC 20010
202-476-2020

hudgins
hudgins's picture
High Lp(a)

We had a family referred to me with an eight year old with elevated Lp(a) diagnosed by our hematology group because she presented with a retinal venous clot.  As part of the workup for hypercoagulability, they performed  Lp(a) which was elevated.  They started her on niacin and baby aspirin and sent her to me.  I probably made the mistake of testing her three siblings and parents for lipids and Lp(a).  Lipids were all normal as was the case in the patient but everyone had elevated Lp(a). This is a family from Africa and they were heading back.  Niacin is not well tolerated usually but the patient was taking 500 mg + BASA daily and had no problems.   The father, who is a physician involved in public health, was not aware of higher incidence of Lp(a) in his country or for that matter, was not at all familiar with Lp(a).  We discussed the options of niacin and because the older one was tolerating it well, he thought he might try it as it is OTC.  They have left the country but I will be in email contact with them in the future.  Another adolescent patient I have has elevated Lp(a) also discovered after a thrombosis in his arm.  His sister tested positive also but we did not put her on either a statin or niacin.  Other fasting lipids were normal and the brother was being anti-coagulated by hematology.
Again, we do not routinely do cIMT on patients but am interested in imaging.  Best,  Irwin
 
Irwin Benuck, MD, PhD
Division Head, Community Based General Pediatrics
Professor of Pediatrics, Northwestern University Feinberg School of Medicine
T-312.227.6852 l F 312 227.9418 l ibenuck@luriechildrens.org l luriechildrens.org
225 East Chicago Avenue, Box 16, Chicago, Illinois 60611-2605

hudgins
hudgins's picture
High Lp(a)

Interesting that peds neuro folks consider elevated Lp(a) a risk factor for cva’a or other clotting disorders, but not considered a risk factor for same in adults as far as I know.
 
Bill

hudgins
hudgins's picture
High Lp(a)

That is true, Mendelian Randomization studies do not show a relationship.  Also, there is no data that lowering Lp(a) alters outcomes

 

Sam Gidding

hudgins
hudgins's picture
High Lp(a)

Great LDL-C response to ? lifestyle so far, but would also try to follow Lp(a) cholesterol, which might be relatively resistant to lowering. If he were to develop an atherogenic profile, with his lowered obesity theshold due to South Asian descent, he possibly could be considered to have a high risk equivalent condition and a case for LDL lowering based on the adult FATS data, but not now with a non-HDL below target. Might also consider a full baseline coagulation work-up and ECHO for aortic stenosis before he is 18y at which time there may be treatment options like Lp(a) antisense.      Piers   

 

Cecephus58
Cecephus58's picture
High Lp(a)

Thank you for this interesting case.  My partner and I have experienced a recent influx of at least 5 patients who have been referred after stroke, each has an Lp(a) that is 3-5 times higher than normal  (normal <75nmol). Most on them came to us already treated with aspirin or in one case warfarin (after 3 stroke events).  Each has tolerated niacin when given prior to bedtime. We don't have enough follow-up to determine response to therapy.

Constance Cephus, PhD, CPNP

Preventive Cardiology

Texas Children's Hospital

Houston, Tx



by Dr. Radut.