Alagille syndrome medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
There is no known cure for Alagille's Syndrome. Most of the treatments available are aimed at improving the functioning of the heart, and reducing the effects of impaired liver function.Alagille syndrome may be managed through medical therapies that involve improving bile flow and reducing itching. There is controversy over the degree of success of such treatments.
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.