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Bylvay was approved for marketing by the U.S. Food and Drug Administration (FDA) in July 2021, becoming the first targeted drug for the treatment of progressive familial intrahepatic cholestasis (PFIC).
1. Generic name: Odevixibat (Odevixibat)
2. Trade name: BYLVAY
It is used to treat pruritus symptoms in patients with progressive familial intrahepatic cholestasis (PFIC) aged 3 months and above. It may be ineffective in patients with PFIC type 2 if there is a mutation in the ABCB11 gene that causes the bile salt export pump protein (BSEP-3) to be completely missing or non-functional.
1. Capsule: 400mcg
2. Main ingredients: Orbisibat sesquihydrate, excipients include hypromellose, microcrystalline cellulose, etc.
1. Recommended dose: 40mcg/kg/day, take with meal in the morning. If the itching does not improve after 3 months, the dose can be adjusted in increments of 40mcg/kg to a maximum of 120mcg/kg/day (the total daily dose does not exceed 6mg).
2. Examples of dose by weight: 400mcg per day for patients 7.5-12.4kg, 1600mcg per day for patients 35.5-45.4kg.
1. When abnormal liver enzymes occur (increased ALT/AST, elevated bilirubin), the administration needs to be interrupted. After recovery, it can be restarted from 40mcg/kg and gradually increased.
2. If diarrhea persists, the medication needs to be suspended and restarted at the lowest dose after the symptoms are relieved.
3. Fat-soluble vitamin deficiency needs to be supplemented. If it continues to worsen, discontinue the medication.
1. Medication time : Must be taken in the morning with food, not on an empty stomach.
2. Treatment of missed doses : It is not clearly stated. It is recommended to take the next normal dose.
3. Vomiting treatment : If you vomit after taking the medicine, it is not recommended to take another dose until the next normal dose.
4. Other :
(1) The capsules cannot be chewed or crushed. Those with difficulty swallowing can open them and mix them with soft food;
(2) The capsules must be taken 4 hours apart with bile acid-binding resins (such as cholestyramine).
1. Pregnant women: Animal experiments have shown that it may cause fetal heart malformations. There is insufficient human data, so the risks need to be weighed.
2. Lactation period: The drug absorption rate is low and the risk of breastfeeding is small, but the baby's fat-soluble vitamin levels need to be monitored.
3. Children: It is safe and effective for patients aged 3 months to 17 years old, but there is insufficient data for patients < 3 months old.
4. Liver damage: The safety of this product has not been established in patients with cirrhosis or portal hypertension and requires close monitoring.
1. Common (>2%): Abnormal liver enzymes, diarrhea, abdominal pain, vomiting, and fat-soluble vitamin deficiency.
2. Serious risks: Hepatotoxicity (regular monitoring of liver function is required), dehydration (rehydration is required in case of diarrhea).
There are no clear contraindications.
1. Bile acid-binding resin may reduce the efficacy of the drug, so it needs to be taken 4 hours apart.
2. Combined use with itraconazole (P-gp inhibitor) may increase the exposure of orvixibat, but the clinical significance is not significant.
Stored at room temperature 20°C-25°C, short-term storage at 15°C-30°C allowed.