Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ONGOING

all patients

1st line – dietary treatment + vitamin/mineral supplementation

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ONGOING
|
all patients
1st line – 

dietary treatment + vitamin/mineral supplementation

The treatment goal for all patients is to maximize caloric intake and prevent the sequelae of neuromuscular deterioration that results from fat-soluble vitamin deficiency, specifically from vitamin E (alpha-tocopherol).[1][2][7]

In addition to vitamin and mineral supplements, the patient should begin a low-fat diet consisting of <20% fat from total calories (5-20 g fat per day). If fat is ingested, medium-chain triglycerides are least toxic. However, limitation of any fats is most favorable. A diet containing essential fatty acids, found in safflower oil, is recommended. No significant complications arise from this intervention.

Vitamin D (cholecalciferol) can be given, but this is not critical because it can also be activated on the skin by ultraviolet rays from endogenously produced metabolites; hence, requirements in adults seem to be satisfied by nondietary sources. However, a supplement can be given to individuals of all ages and incurs no risk of toxicity, thus no need for monitoring.

Iron, folate, or vitamin B12 (cyanocobalamin) supplementation may be necessary to reverse signs of anemia.[29] Folic acid can be given, though evidence for or against supplementation is lacking. It can be taken indefinitely as it is nontoxic.

Primary options

alpha-tocopherol (vitamin E): 100-300 international units/kg/day orally

OR

vitamin A (retinol): 100-400 international units/kg/day orally

OR

phytonadione (vitamin K1): 5-35 mg/week orally

OR

cholecalciferol (vitamin D3): 800-1200 international units/day orally

OR

ferrous sulfate: 50-100 mg orally three times daily

OR

folic acid (vitamin B9): 0.4 to 1 mg orally once daily

OR

cyanocobalamin (vitamin B12): 1000 micrograms subcutaneously/intramuscularly once daily for 1 week, followed by 1000 micrograms once weekly for 1 month, then 1000 micrograms once monthly thereafter

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Choose a patient group to see our recommendations

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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