A recent study reviews the King’s College experience for managing pruritus associated with cholestasis in patients with Alagille syndrome (AGS) (JPGN 2013; 57: 149-54).
This retrospective study examined 62 patients (1995-2010). 82% (n=51) had pruritus. Most common treatments:
- Ursodeoxycholic acid in 40 patients. 1st line Rx in 31. Efficacy was rated as good in 20% and some efficacy in 67.5%.
- Rifampicin in 39 patients. 1st line Rx in 8. Efficacy was rated as very good/good in 49% and some efficacy in 46%.
- Cholestyramine in 18 patients. 1st line Rx in 9. Efficacy was rated as very good in 17% and some efficacy in 67%.
- Naltrexone in 14 patients. Efficacy was rated as good in 43% and some efficacy in 36%.
- Alimemazine in 13 patients
- Nonsedating antihistamines in 7 patients
- Ondansetron in 5 patients
- Phenobarbital in 1 patient.
Despite these medications, pruritus was controlled by medication in 41% (n=21). 16 patients were referred for liver transplantation and 11 of these patients have been transplanted. These 11 patients make up 55% of those who had permanent resolution of their pruritus.
The authors proposed an algorithm for treatment:
- 1st line: ursodeoxycholic acid 10-20 mg/kg/day divided in 2 doses or cholestyramine 240 mg/kg/day divided into 3 doses
- 2nd line: (if needed) Add/substitute rifampicin 5-10 mg/kg/day divided into 2 doses (max 600 mg/day)
- 3rd line: (if needed) Add/substitute naltrexone 0.25-0.5 mg/kg/day (max 50 mg/day)
- 4th line: (if needed) Add/substitute ondansetron max 8 mg/day divided into 2 doses per day (or phenobarbital 5-10 mg/kg/day divided into 2 doses.
- If none of these are helpful, options could include MARS (molecular adsorbent recirculation system), partial external biliary diversion, or liver transplantation.
Related blog entries:
- Itchy and Scratchy | gutsandgrowth
- Learning a lot from ChiLDREN (part 1) | gutsandgrowth
- Alagille and liver transplantation | gutsandgrowth This post has a number of useful website links as well as a number of other references.