The deficiency of fat-soluble vitamins in cholestasis is a prevalent issue even in specialized centers, according to a review by researchers in the US and Canada that assessed these vitamins and their supplementation in cholestasis. 

“Early and frequent monitoring are essential followed by aggressive supplementation and ongoing monitoring,” Binita M. Kamath, MBBChir, MRCP, MTR, from the Division of Gastroenterology, Hepatology, and Nutrition at the Hospital for Sick Children in Canada and the coauthors of the study said.

Chronic cholestasis is an important symptom of Alagille syndrome (ALGS), a rare genetic disease caused by defects in the Notch signaling pathway and characterized by intrahepatic bile duct paucity.

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Fat-soluble vitamin deficiency often occurs within the first months after birth in babies with neonatal cholestasis and can persist even with multivitamin supplements, according to the review.

Read more about the symptoms of ALGS

“A one size fits all algorithm does not work,” wrote the authors of the review who said that there are factors such as the cost of vitamin supplementations to families and the availability of different fat-soluble vitamin preparations at different centers.

“Although local resources will impact supplementation strategies, we recommend the use of multivitamin preparations designed for cholestasis, if available,” the authors wrote. They emphasized the need for advocacy to establish coverage for nutritional supplements based on the fact that the cost of these supplements poses a significant burden to families.

Cholestasis is a hepatic disease that occurs as a result of the reduction or stoppage of bile flow from the liver. This is associated with impaired fat digestion and can lead to deficiencies of fat-soluble vitamins such as vitamins A, D, E, and K. In ALGS, cholestasis is the result of intrahepatic bile duct paucity.

The review was published in the journal Clinics in Liver Disease.


Kamath BM, Alonso EM, Heubi JE, et al. Fat soluble vitamin assessment and supplementation in cholestasis. Clin Liver Dis. 2022;3:537-553. doi:10.1016/j.cld.2022.03.011