Fat-soluble vitamin deficiencies and supplementation for patients with cholestasis due to biliary atresia or Alagille syndrome

Fat-soluble vitamin	How to monitor	Standard oral treatment recommendation	Signs and symptoms of deficiency	Recommended supplementation in setting of deficiency
Vitamin A	Measure serum or plasma retinol and RBP Interpretation: Normal range for retinol 19 to 77 mcg/dL Retinol – RBP <0.8 mol/mol defines deficiency when retinol <20 mcg/dL	Liquid vitamin A: <10 kg – 5000 IU/day^* ≥10 kg – 10,000 IU/day	Keratinization of the skin and mucous membranes Ocular effects such as xerophthalmia, night blindness, xerosis, and bitot spots Retinol:RBP molar ratio <0.8 and serum retinol <20 cgg/dL	Liquid vitamin A 5000 IU/day by mouth; recheck levels in 1 month or 25,000 to 50,000 IU/day by mouth for 1 to 4 weeks; recheck levels weekly or 50,000 IU intramuscularly once/month up to 2 months; recheck levels monthly
Vitamin D	Measure serum 25-OH vitamin D Interpretation: Optimal levels >30 ng/mL	Cholecalciferol or ergocalciferol – 2000 to 5000 IU/day (50 to 125 mcg/day)	Rickets, fractures, osteomalacia 25-OH vitamin D <15 ng/mL	Cholecalciferol or ergocalciferol 1200 to 4000 IU/day by mouth; recheck levels in 1 month If child remains deficient, give 4000 to 8000 IU/day; recheck levels in 1 month or Give 1,25-OH₂ vitamin D 0.05 to 0.2 mcg/kg/day; recheck serum 1,25-OH₂ vitamin D levels in 1 month
Vitamin E	Measure serum vitamin E and total lipids Interpretation: Normal vitamin E:total lipid ratio >0.6 mg/g (age <1 year) or >0.8 mg/g (age >1 year)	TPGS – 15 to 25 IU/kg/day (10 to 17 mg/kg/day)	Peripheral neuropathy, ataxia, ophthalmoplegia Vitamin E:total lipid ratio <0.6 mg/g (age <1 year) or <0.8 mg/g (age >1 year) is deficient	If deficient, 50 IU/kg/day of TPGS; recheck levels in 1 month
Vitamin K	Measure PT, INR, and PIVKA-II	Vitamin K1 2 to 5 mg daily	Prolonged PT, increased INR (INR >1.2), elevated PIVKA-II Coagulopathy, bruising, bleeding	If INR >1.5 and ≤1.8, give 5 mg vitamin K1 daily by mouth and/or 2 to 5 mg vitamin K intramuscularly 1 time; recheck PT/INR in 1 to 2 days

The doses in this table are designed for cholestatic/jaundiced infants and children with biliary atresia or Alagille syndrome. If the cholestasis resolves and vitamins are replete, children can be transitioned to standard doses of multivitamins, with routine monitoring of fat-soluble vitamins as outlined above. These doses are consistent with society guidelines^[1].

1,25-OH2 vitamin D: 1,25-dihydroxyvitamin D; 25-OH vitamin D: 25-hydroxyvitamin D; INR: international normalized ratio; IU: international units; PIVKA-II: protein induced by vitamin K absence II; PT: prothrombin time; RBP: retinol-binding protein; TPGS: tocopherol polyethylene glycol 1000 succinate (water-miscible form of vitamin E); vitamin K1: phytonadione.

* For vitamin A, 1 IU = 0.3 micrograms retinol or 0.6 micrograms beta-carotene.

Reference:

Mouzaki M, Bronsky J, Gupte G, et al. Nutrition Support of Children With Chronic Liver Diseases: A Joint Position Paper of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr 2019; 69:498.

Graphic 51569 Version 7.0

خرید پکیج

Fat-soluble vitamin deficiencies and supplementation for patients with cholestasis due to biliary atresia or Alagille syndrome