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

PO, per os; IV, intravenous