Tag Archives: MARS

Living on MARS

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As anyone who follows this blog knows, I really like good acronyms.  MARS which stands for molecular adsorbents recirculating system is another good one.  Data regarding the use of MARS for acute liver failure (ALF) due to Wilson disease in children has been recently reported (JPGN 2014; 58: 160-64, editorial 140-41).

Background: MARS is a form of dialysis to remove albumin-bound toxins via a specialized membrane. MARS has been studied as a potential bridge to liver transplantation or as a support to try to avoid liver transplantation in some cases.

The present study with only four children is not terribly informative.  However, both the article and the editorial provide references on small randomized controlled trials which concur with the conclusions of the authors that “biological and clinical improvement is demonstrated in the MARS treatment group compared with the standard medical treatment group.”  Yet, recent large multicenter randomized controlled trials are inconclusive with regard to whether MARS improves survival.

Bottomline (from editorial): “MARS does not prevent transplantation, and survival outcome post transplantation is unclear.  There is no robust evidence to justify the financial implications of this intervention in a clinical setting. The present role of MARS remains within the research setting.”

Severe Pruritus with Alagille Syndrome

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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.

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