Dietary management in ALD is not a treatment that halts the disease. It matters for normal growth and good nutritional status.
This page summarises Dietary Management in Adrenoleukodystrophy, the first dietary guide for ALD patients published in Korea. Authorship is credited at the foot of the page.
1. What diet can and cannot do
Reducing dietary intake of very long chain fatty acids alone cannot lower body VLCFA levels. The body synthesises them as well.
For that reason, a moderate restriction of foods high in saturated fat is generally preferred to a strict low-fat regimen.
There is a second point. Lorenzo’s oil combined with a low-fat diet does bring plasma C26:0 to normal or near-normal values in most patients. But there are no controlled trials showing improved outcome. The 2022 international consensus recommendations state this explicitly (recommendation 36).
A number improving and a disease improving are not the same thing.
Diet still matters, for a different reason. Strict restriction and Lorenzo’s oil can bring gastrointestinal discomfort and a fall in overall intake, leading to undernutrition. Understanding the principles helps avoid that.
2. Aims of dietary management
The guide sets out three.
- Maintain normal growth and good nutritional status
- Eat permitted foods across the range
- Reduce VLCFA intake, and reduce what the body makes
The commonest problem is emphasising the third at the expense of the first.
3. Assessing nutritional status
Adults — via ideal body weight and percentage of ideal weight.
- Ideal body weight: height(m) × height(m) × 22 for men, × 21 for women
- Percentage: current weight ÷ ideal weight × 100
| Percentage | Assessment |
|---|---|
| ≤ 90% | Underweight |
| 91–110% | Normal |
| 111–119% | Overweight |
| ≥ 120% | Obese |
Children and adolescents — assessed against standard growth charts for the same age and sex. The guide includes a table of standard weight for height as an appendix.
4. What to reduce
Very long chain fatty acids
Fatty acids are classified by chain length as short, medium, long or very long. The very long chain fatty acids include behenic acid (C22:0), lignoceric acid (C24:0) and cerotic acid (C26:0). The one that accumulates in ALD is C26:0.
They occur in fruit and vegetable skins and seeds, wholegrains, nuts, vegetable oils and shortening — mostly alongside foods high in saturated fat.
Saturated fat
Fats solid or semi-solid at room temperature, including palmitic acid (C16:0) and stearic acid (C18:0).
In ALD, synthesis of very long chain fatty acids is normal; the problem is that they cannot be oxidised and so accumulate. Saturated fat intake is therefore restricted alongside VLCFA. The guide suggests keeping it below 3% of energy intake.
Foods high in saturated fat include animal fats such as short ribs, pork belly and bacon, and dairy fat in butter, cream, milk and ice cream.
Total fat
The guide suggests cooking to a low-fat pattern of roughly 10–20% of total energy, allowing for essential fatty acid requirements.
Cutting fat too far leaves essential fatty acids short and impairs absorption of fat-soluble vitamins. Reducing is not the same as eliminating.
5. Permitted, caution and restricted foods
As classified in the guide.
| Group | Permitted | Caution | Restricted |
|---|---|---|---|
| Grains | Rice, white bread, potato, noodles, rice cake, flour, acorn jelly, sweet potato, some cereals | English muffin, rye bread, bagel | Biscuits and breads made with butter or margarine, crisps, pizza, doughnuts, corn, crackers, chips, oatmeal, pitta bread |
| Meat and fish | Lean meat with fat removed, chicken breast, egg white, cod, flounder, crab, clam, sea bass, conch, rockfish, abalone, sea cucumber, shellfish, pollock, prawn, tofu, lima and green peas, cottage cheese | — | Fried or battered meat and fish, offal, ham, sausage, fish cake, egg yolk, oily fish, tinned fish |
| Vegetables | Vegetables with skin and seeds removed, sweet pumpkin, oyster mushroom, seaweed, laver | (small amounts) mushroom, carrot, aubergine, turnip, cucumber, celery, lettuce, onion, broccoli, spinach | Fried or battered vegetables, cabbage, squash, kale, young garlic, hijiki, kelp, green laver, wild greens, dried greens, enoki, curled mallow, pepper leaves, cauliflower, deodeok, gulfweed |
| Dairy | Skimmed milk powder, skimmed milk, fat-free yoghurt | — | Milk, soy milk, cheese, whole milk powder, ice cream, chocolate milk |
| Oils | Lorenzo’s oil, GTO/GTE oil | — | All cooking oils, sesame, mayonnaise, margarine, butter |
| Fruit | Tomato, apple, peach, pear, watermelon, melon, juices, fruit with skin and seeds removed | Plum, cherry, mango, pineapple | Banana, fruit eaten with skin or seeds, raspberry, apricot, dried fruit, concentrated orange juice |
| Other | Jam, jelly, sweets, honey, sugar, soft drinks, syrup, maple syrup, ketchup, Worcestershire sauce | Mustard | Cocoa powder |
Points on preparation and choice
- Choose white rice over mixed grains, white bread over wholemeal. Peel potatoes and sweet potatoes
- Choose lean cuts, and blanch to remove fat
- Use egg white
- Exclude processed meats and tinned fish
- Remove skin and seeds from vegetables and fruit
- Choose low-fat or fat-free dairy
- Exclude sesame salt in cooking
- Use sugar, syrup, honey, sweets and jelly to make up energy
6. Lorenzo’s oil
Composition
A mixture of glycerol trioleate (GTO) and glycerol trierucate (GTE) in a 4 : 1 ratio. GTO is 90% oleic acid; GTE is over 90% erucic acid.
What it does
It does not halt neurological progression. It competitively inhibits the synthesis of very long chain fatty acids, lowering their concentration in blood.
How it is taken, as described in the guide
Whether to take it, and at what dose, is a decision for the treating clinicians. What follows is as described in the guide.
- It also offsets the energy shortfall created by a low-fat diet
- Intake of at least 20% of total energy, or 2–3 mL/kg body weight, is described
- It is heat-labile and is not used in cooking
- Taken in small amounts at intervals at room temperature — three times with meals and once before bed
- Where enteral feeding is used, mixed into the formula and given slowly
Essential fatty acids
The GTO component contains about 5% linoleic acid, supplying only around 1.25% of energy. Safflower, fish and perilla oils are therefore taken with meals to make up essential fatty acids.
Fatty acid composition of oils, from the guide:
| Oil | Saturated (%) | Linoleic (%) | Linolenic (%) | Monounsaturated (%) |
|---|---|---|---|---|
| Canola | 6 | 22 | 10 | 62 |
| Perilla | 7 | 33 | 49 | 11 |
| Safflower | 10 | 77 | trace | 13 |
| Sunflower | 11 | 69 | 0 | 20 |
| Corn | 13 | 61 | 0 | 25 |
| Olive | 14 | 8 | 1 | 77 |
| Soybean | 15 | 54 | 7 | 24 |
| Sesame | 15 | 45 | — | 40 |
| Peanut | 18 | 33 | 0 | 49 |
| Cod liver | 23 | 1 | 1 | 47 |
| Palm | 45 | 12 | 1 | 37 |
| Coconut | 92 | 2 | 0 | 6 |
7. Common problems
Constipation
More fibre and fluid. Fruit and vegetables for fibre, water or other drinks for fluid, and as much activity as is manageable.
Weight loss and faltering growth
A low-fat diet reduces energy intake, and weight or growth may suffer. Regular weight checks are needed. Eating fully within the permitted range, small frequent meals when hungry, and keeping a record of intake are suggested.
Fat-soluble vitamins, calcium and phosphorus
These may fall short as meat intake decreases; adequate intake needs maintaining.
Gastrointestinal discomfort
Nausea, vomiting and diarrhoea can occur with Lorenzo’s oil. Dividing it into more frequent portions, taking it closer to mealtimes, or mixing it into food are suggested.
Difficulty swallowing
Dysphagia diets progress purée → blended → minced → regular. A purée is thick enough not to run off a spoon.
Thickening agents can be used to reduce the risk of aspiration. Add the thickener to water or a liquid product, stir, and check the consistency two to three minutes after it has fully dissolved.
Inadequate intake
Where intake, digestion or absorption is limited, oral nutritional supplements can be used. Choosing a product low in fat is a consideration.
8. Recipes
The guide contains 31 recipes developed on low-fat principles, each with ingredients and quantities, method, nutritional composition and preparation notes.
The approach is “restricted, but not far from what the family already eats” — a diet a child will not eat is of no use.
- Achieving the texture of fried food without frying — using the oven and a dry pan
- Cooking without oil — simmering and reducing in stock, blanching to remove fat
- Dairy substitutes — fat-free and skimmed products
- Egg whites only
- Everyday dishes — cutlets, hamburger steak, fish cake, bossam, spaghetti, porridge, rolls
- Snacks and desserts too, because making up energy matters
Full recipes are in the guide itself.
Sources
This page is based on the following.
Dietary Management in Adrenoleukodystrophy
Dietary content researched and written by — Eunju Lee, dietitian With the support of — Songmi Lee and Jinsu Kim, Nutrition Team, Severance Hospital Reviewed with preface by — Hoon-Chul Kang, MD, PhD, Division of Pediatric Neurology, Severance Children’s Hospital, Yonsei University College of Medicine
The first dietary and cooking guide for patients with adrenoleukodystrophy published in Korea.
Statements about the evidence for Lorenzo’s oil follow the 2022 international consensus recommendations (Neurology, recommendation 36).
Last reviewed: 7 August 2026
This page provides general information and does not replace medical or dietetic care for an individual patient. Dietary plans should be made with your treating clinicians and dietitian.