Odevixibat, sold under the trade name Bylvay, is a medication for the treatment of progressive familial intrahepatic cholestasis (PFIC). It is taken by mouth.The most common side effects include diarrhea, abdominal pain, hemorrhagic diarrhea, soft feces, and hepatomegaly (enlarged liver).Odevixibat is a reversible, potent, selective inhibitor of the ileal bile acid transporter (IBAT).Odevixibat was approved for medical use in the United States and in the European Union in July 2021. Medical uses
In the United States, odevixibat is indicated for the treatment of pruritus in people three months of age and older with progressive familial intrahepatic cholestasis (PFIC). In the European Union it is indicated in people six months of age and older. Mechanism of action
Odevixibat is a reversible inhibitor of the ileal sodium/bile acid transporter which is the transporter responsible for reabsorption of the majority of bile acids in the distal ileum. The reduced absorption of the bile acids in the distal ileum compounds and leads to a decrease in stimulation of FXR, decreasing the inhibition of bile acid synthesis. Pharmacokinetics
Odevixibat is majorly protein-bound in-vitro. A dose of Odevixibat that is 7.2 mg reaches a Cmax concentration of 0.47 ng/mL with an AUC (0-24h) of 2.19 h*ng/mL. Adult and pediatric patients given the therapeautic dose of Odevixibat did not display plasma concentrations of the drug. Odevixibat is eliminated majorly unchanged. Odevixibat has an average half-life of 2.36 hours.
Children with SMS are often given psychiatric diagnoses such as autism, attention deficit/hyperactivity disorder (ADHD), obsessive-compulsive disorder (OCD), attention deficit disorder (ADD) and/or mood disorders. Treatment
Treatment for Smith–Magenis syndrome relies on managing its symptoms. Children with SMS often require several forms of support, including physical therapy, behaviour therapy, occupational therapy and speech therapy. Support is often required throughout an affected persons lifetime. Medication is often used to address some symptoms. Melatonin supplements and trazodone are commonly used to regulate sleep disturbances. In combination with exogenous melatonin, blockade of endogenous melatonin production during the day by the adrenergic antagonist acebutolol can increase concentration, improve sleep and sleep timing and aid in improvement of behaviour. Other medications (such as risperidone) are sometimes used to regulate violent behavior. Eponym
The eponym Smith–Magenis refers to two scientists who described the condition in 1986, namely, Ann C. M. Smith, a genetic counselor at the National Institutes of Health, and R. Ellen Magenis, a pediatrician, medical geneticist and cytogeneticist at the Oregon Health Sciences University. See also
Serine hydroxymethyltransferase
Charcot-Marie-Tooth disease
Potocki-Lupski syndrome
Prader-Willi syndrome

References

This article incorporates public domain text from The U.S. National Library of Medicine


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