Alagille syndrome medical therapy

The medical management of Alagille Syndrome is complex and continues to generate controversy. The significant variability of organ involvement requires the managing physician to have an understanding of the breadth and interplay of the variable manifestations. Furthermore, the liver disease in particular requires an appreciation of the natural history and evolution of the profound cholestasis.

Several medications are used to improve bile flow and reduce itching (pruritus): Ursodiol (Actigall),Hydroxyzine (Atarax), Cholestyramine, Rifampicin, and Phenobarbitol have all been used to varying degrees of success.

Many patients with Alagille's Syndrome will also benefit from a high dose of a multivitamin such as ADEK (contining high levels of vitamins A, D, E, and K), as the reduced bile flow makes it difficult to absorb and utilize these vitamins.It is equally important to optimize nutrition to maximize growth and development. Those with splenomegaly need to use spleen guard during activnities. Routine follow up with a pediatrician is necessary.