Pediatric Lipid Clinic Children's of Alabama · UAB Heersink School of Medicine
A guide for families

Understanding your child's cholesterol or triglyceride diagnosis

High cholesterol and high triglycerides look different in childhood than in adulthood — and they're treatable. This page explains four conditions we manage often: FCS, homozygous FH, heterozygous FH, and general pediatric dyslipidemia, in plain language.

Find your child's pattern Contact the clinic
Orientation

Where your child's labs may fit

These four conditions sit differently on two questions your care team will already be asking: is it mainly cholesterol or mainly triglycerides, and is it driven by an inherited gene change or by broader factors like diet and weight. This is a map for orientation, not a diagnosis — your labs and, when needed, genetic testing tell us where your child actually falls.

Mainly Triglycerides  ←→  Mainly LDL Cholesterol
Familial Chylomicronemia Syndrome (FCS)
Rare · single-gene · severe triglycerides
Homozygous Familial Hypercholesterolemia (HoFH)
Rare · two gene copies · severe LDL
General Dyslipidemia
Common · often diet/weight-related
Heterozygous Familial Hypercholesterolemia (HeFH)
1 in ~250 · one gene copy · elevated LDL
Less clearly inherited Strongly inherited (one or both parents)

If your child's triglycerides are very high (often well over 1,000 mg/dL) with no clear cause, FCS is on our radar. If LDL cholesterol is extremely high from a young age and shows up in both parents' family history, we think about HoFH. A single parent with early heart disease and elevated LDL points toward HeFH. Everything else — often tied to weight, diet, or mild inherited tendencies — falls under general pediatric dyslipidemia.

Four conditions, explained

What each diagnosis means

Each card covers what it is, what we watch for, and how it's typically managed in children.

Triglyceride disorder · ultra-rare FCS — Familial Chylomicronemia Syndrome

The body can't clear fat from the blood

<1 in 1,000,000estimated prevalence
LPL pathwaygenes involved
>1,000 mg/dLtypical triglycerides
  • Caused by changes in genes that help break down fat in the blood (most often LPL, also APOC2, APOA5, GPIHBP1, LMF1)
  • Main risk is recurrent, sometimes severe pancreatitis (abdominal pain, vomiting)
  • May cause belly fullness, skin bumps (eruptive xanthomas), or a milky appearance to blood drawn for labs
  • Managed with a very low-fat diet, close dietitian support, and newer injectable therapies that target the underlying pathway
Most children with FCS lead full lives once the diet and monitoring plan is in place — the goal is preventing pancreatitis, not restriction for its own sake.
Cholesterol disorder · rare HoFH — Homozygous Familial Hypercholesterolemia

Both gene copies carry a change

1 in ~300,000estimated prevalence
LDLR, APOB, PCSK9genes involved
Often >400 mg/dLuntreated LDL-C
  • Inherited from both parents, each carrying one copy of a gene change (often LDLR, sometimes APOB, PCSK9, or LDLRAP1)
  • LDL cholesterol is very high from birth, raising the risk of early heart disease if untreated
  • May cause visible cholesterol deposits in skin or around the eyes
  • Managed aggressively: statins, ezetimibe, PCSK9 inhibitors, and sometimes lomitapide, evinacumab, or LDL apheresis (a filtering procedure)
Early, aggressive treatment substantially changes the long-term outlook — this is why we monitor closely from diagnosis.
Cholesterol disorder · common HeFH — Heterozygous Familial Hypercholesterolemia

One gene copy carries a change

1 in ~250estimated prevalence
LDLR, APOB, PCSK9genes involved
~160–400 mg/dLtypical LDL-C
  • Inherited from one parent — often a parent or grandparent with early heart disease
  • LDL cholesterol is elevated from childhood but usually without symptoms
  • Found through routine screening (ages 9–11) or because of family history
  • Managed with heart-healthy diet first, then statins typically starting around age 8–10 if levels stay high, plus screening for siblings and parents
This is the most common inherited lipid condition we see — most children do very well with steady, lifelong management.
Mixed pattern · most common General Pediatric Dyslipidemia

Cholesterol or triglycerides outside the target range

~1 in 5kids with some abnormal lipid value
Multiple factorsdiet, weight, activity, genetics
Often mild–moderateseverity
  • Usually related to a mix of diet, weight, physical activity, and mild inherited tendencies — not a single gene change
  • Often discovered through universal screening (recommended once between ages 9–11, and again between 17–21)
  • Sometimes linked to insulin resistance, thyroid issues, or other medical conditions we screen for
  • Managed primarily with nutrition, activity, and family-based lifestyle changes; medication only when levels stay high
This category responds especially well to lifestyle changes the whole family can do together.
For FCS & severe hypertriglyceridemia

Daily fat-gram diet calculator

This is specific to FCS and similar severe hypertriglyceridemia management — it is not a general weight-management calculator, and the percentages are deliberately much lower than typical adult fat-intake guidance, per the NLA's 2025 FCS dietary guideline.

Use alongside your dietitian, not instead of one Exact targets should be individualized by a registered dietitian based on growth, triglyceride response, and tolerance. This calculator gives a reasonable starting range from published guidelines, not a prescription.

Calculate today's fat-gram target

You'll need your child's estimated daily calorie needs — this is best set by your dietitian using growth-based calculations, not a generic online estimate.

Runs locally in your browser — nothing is sent or saved.

    Track today's intake

    Daily fat-gram log

    Log what your child eats through the day and watch the running total against your target. Type in the fat grams from the food label or your fat-counter book — for whole fruits and vegetables (almost always 0g fat) you can use the quick-add buttons below.

    This log does not save between visits It resets if you close or refresh the page, so it's meant for tracking through a single day — print it for your records or for clinic visits using the print button below. Use the exact target your dietitian gave your child (their examples have ranged from 10–20 g/day depending on the plan).

    Quick add (whole fruits & vegetables, ~0 g fat):

    MealFoodFat (g)
    Nothing logged yet today.
    0 g of 15 g target
    15 g remaining
    For referring providers & care team

    FH / HoFH triage reference

    A working calculator and reference workflows for classifying suspected pediatric FH and HoFH, screening for secondary causes, and routing genetic testing and cascade screening.

    Draft clinical reference — not an independently validated device The logic below is built from published criteria (Cuchel et al. HoFH consensus; National Lipid Association 2026 pediatric FH guidance; AAP/StatPearls secondary-cause screening) but has not itself been validated against outcomes data. Review the logic and sourcing against your own clinic's protocols before using it to guide patient care, and treat its output as a starting point for clinical judgment, not a replacement for it.

    FH probability & referral urgency calculator

    Enter the proband's data. This estimates a diagnostic category and referral urgency — it does not submit anywhere or store data.

    Ideally a repeat, confirmed value — LDL-C fluctuates in childhood.
    Runs locally in your browser — nothing is sent or saved.

      Secondary-cause screen

      Standard work-up before attributing an elevated lipid panel to a primary genetic cause.

      • TSH — rules out hypothyroidism, a common secondary cause of elevated LDL-C and triglycerides
      • Fasting glucose / HbA1c — screens for diabetes-driven dyslipidemia
      • Urinalysis ± urine albumin:creatinine — screens for nephrotic syndrome
      • Liver function tests — screens for hepatic/cholestatic causes
      • Medication & substance history — steroids, retinoids, atypical antipsychotics, oral estrogens, alcohol
      • Pregnancy — consider in adolescent females with new hypertriglyceridemia

      Sources used in this draft

      • Cuchel M, et al. Homozygous familial hypercholesterolemia consensus statement — clinical diagnostic criteria for HoFH.
      • National Lipid Association. Update on familial hypercholesterolemia: expert clinical consensus (2026) — pediatric LDL-C diagnostic cutoffs.
      • ERKNet/ESPN consensus on lipoprotein apheresis in pediatric HoFH — apheresis LDL-C thresholds.
      • AAP clinical guidance and StatPearls pediatric dyslipidemia review — secondary-cause work-up.
      • LIPIGEN-FH-PED, EAS, and Simon Broome pediatric criteria comparisons — diagnostic sensitivity in children.
      • Ashraf, A.P., et al. Case studies in pediatric lipid disorders and their management. The Journal of Clinical Endocrinology & Metabolism, 2021. 106(12): p. 3605-3620.
      • de Ferranti, Sarah D., et al. Circulation 139.13 (2019): e603-e634.
      Beyond this site

      External resources

      These open other organizations' websites in a new tab — they're not produced by this clinic.

      Patient advocacy & community

      Support, education, and connection with other families managing inherited cholesterol conditions.

      Family Heart Foundationfamilyheart.org ↗

      Healthy living

      General heart-healthy nutrition and lifestyle guidance for the whole family.

      AHA Healthy Livingheart.org ↗

      This points to AHA's general Healthy Living hub — let us know if you had a more specific AHA page in mind and we'll swap it in.

      Patient advocacy, community & recipes

      FCS-specific support network with patient stories, webinars, and healthy recipes.

      FCS Foundationlivingwithfcs.org ↗

      FCS cookbook

      Nineteen low-fat, sugar-free recipes created for people living with FCS, with fat and calorie counts per serving.

      Everyday Balance — FCS Cookbookspotlightfcs.com · PDF ↗

      Meant to inspire, not serve as a full dietary guide — remind families to check individual recipes against their own fat-gram target.

      General lipid education

      Patient education from the Foundation of the National Lipid Association, including a low-fat cookbook and LDL-C recipes.

      Learn Your Lipidslearnyourlipids.com ↗
      Common questions

      Frequently asked questions

      No. Diet can affect the numbers, but the inherited forms (FCS, HoFH, HeFH) are caused by gene changes present from birth. Nutrition is part of the treatment, not the cause.
      It depends on the condition. Inherited forms like HoFH and HeFH are usually lifelong and lifelong management is expected. General dyslipidemia often improves substantially with lifestyle changes and may not need long-term medication.
      Often yes, especially for the inherited conditions. We routinely offer screening for siblings and parents once a genetic or strongly familial pattern is confirmed.
      In almost all cases, yes — activity is encouraged and is part of treatment. We'll flag the rare exceptions specific to your child's condition.