Diseases of bile duct in children

Table 6 Medications for cholestasis and pruritus in Alagille syndrome[109,114,123]

Medications	Actions	Dose (kg/d)	Adverse effects
Ursodeoxycholic acid	Choleretic, stimulates bile flow	10-20 mg	Vomiting, diarrhea, and abdominal pain
Cholestyramine	Bile acid-binding resins	0.25-0.5 g	Vomiting, diarrhea, and poor palatability
Rifampicin	Pregnane X receptor agonist increases the metabolism of pruritogenic substances	5-10 mg	Hepatitis, thrombocytopenia, hemolytic anemia, and red discoloration of bodily fluid (sweat, tears)
Phenobarbital	Choleretic enhancement of glucuronyl transferase activity	5-10 mg	Central nervous system depression and vomiting
Naltrexone	Opioid receptor antagonist	0.25-0.5 mg	Opioid withdrawal-like reactions, abdominal pain, and irritability
Maralixibat	Ileal bile salt transporter	380 μg	Diarrhea, abdominal pain, and rash
Ondansetron	Serotonin (5-HT3) receptor antagonist	0.1-0.2 mg	Dizziness, constipation
Hydroxyzine	Antihistamine	2 mg	Rash, drowsiness, and dry mouth
Diphenhydramine	Antihistamine	5 mg/kg/d	Drowsiness, constipation, and nausea