Long‐lasting extracorporeal albumin dialysis in a child with end‐stage renal disease and severe cholestasis

Abstract

We report a case of long-lasting MARS therapy as a bridge to liver-kidney transplantation. A 26-month-old girl with congenital tubulointerstitial nephritis and severe liver fibrosis was placed on MARS for an acute-on-chronic liver failure due to sepsis. She underwent two sessions with good tolerance and recovered her previous neurological status. On the basis of pruritus, sleep, and vomiting improvement, repeated MARS sessions were performed to bridge her to combined liver-kidney transplantation. During eight months, 40 sessions were performed with the MARSmini kit and the MARS monitor (Gambro, Lyon, France). The treatment significantly decreased mean pruritus score from 2.2 +/- 0.9 to 0.8 +/- 0.6 night-time awakening and vomiting episodes. Body weight, height, and HC were -3.2, -3.5 and -2.2 SDS before and -1.7, -4.2, -2.0 SDS after eight months on MARS therapy, respectively. The arm circumference/HC ratio increased from 0.28 to 0.31. Mean total bilirubin serum levels were 303 +/- 72 micromol/L before and 214 +/- 42 micromol/L after MARS cycles. Long-lasting MARS dialysis is feasible in children, decreases adverse effects of severe chronic cholestasis, and may help to preserve nutritional status prior to combined liver-kidney transplantation.

Extracorporeal Liver Support Systems in Paediatric Liver Failure

“…In adults with intractable pruritus, in whom LT is the only alternative, MARS has been used as an intermittent therapy with good results (71)(72)(73)(74). Efficacy of MARS in pruritic relief has also been demonstrated in children, with a 60% reduction in bile acids shown post-MARS therapy (75,76). Concerns regarding tolerability and safety in children of this device, however, remain.…”

Treatment of resistant pruritus from cholestasis with albumin dialysis: Combined analysis of patients from three centers

“…In patients who do not respond to medical therapies, different invasive procedures, such as hemodialysis [11], charcoal hemoperfusion [12], and plasmapheresis [13][14][15] have been used with positive, although uncertain results, due to the small number of patients evaluated. More recently, the therapeutic effects of various methods of extracorporeal albumin dialysis [16][17][18][19][20][21][22][23][24][25][26][27][28], mainly the molecular adsorbent recirculating system (MARS), have been published [16][17][18][19][20][21][22][23][24][25][26][27]. However, from these case reports or short series (the highest has included seven patients) [21], it is difficult to ascertain the actual efficacy of the procedure in patients with cholestatic pruritus.…”

Molecular Adsorbents Recirculating System dialysis in children with cholestatic pruritus

“…MARS appears particularly suitable for patients with cholestatic pruritus not yet suffering from severe coagulopathy, as, in contrast to plasmapheresis, removal of protein-bound hepatic toxins is achieved without exposure of the patient to foreign proteins. Several reports in adults and a single case report in a child suggest relief of pruritus by MARS [19][20][21][22][23][24]. However, in face of the high costs and technical burden, MARS is still infrequently applied.…”