Table 4 Chylomicron Retention Disease Treatment
Early diagnosis without neurological complications: PO | Delayed diagnosis and neurological complications: PO + IV |
|---|---|
Diet | |
Low-fat diet | |
Enriched in essential fatty acids (vegetable oils, fish...) | |
± Enriched in medium-chain triglycerides | |
Liposoluble Vitamins PO | |
Vitamin E (hydrosoluble form): 50 IU/kg/d | |
Vitamin A: 15,000 IU/d (adjust according to plasma levels) | |
Vitamin D: 800-1200 IU/kg/d or 100,000 IU/2 month if < 5 y old, and 600,000 IU/2 month if > 5 y old | |
Vitamin K: 15 mg/week (adjust according to INR and plasma levels) | |
One perfusion/month | |
Fatty acids: intralipid 20% 2 g/kg/month | |
Vitamin E: 4 to 6 mg/kg/month | |
Vitamin A: 500 IU/kg/month | |