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Pediatric Nephrology

Published in:

01-05-2012 | Original Article

Molecular Adsorbents Recirculating System dialysis in children with cholestatic pruritus

Authors: Betti Schaefer, Franz Schaefer, Dieter Wittmer, Guido Engelmann, Daniel Wenning, Claus Peter Schmitt

Published in: Pediatric Nephrology | Issue 5/2012

Abstract

Background

Cholestatic pruritus may severely compromise quality of life. The Molecular Adsorbents Recirculating System (MARS) allows removal of pruritogenic substances without exposure to foreign proteins. Pediatric data, however, are scant.

Methods

We retrospectively analyzed the efficacy of MARS in three boys with severe cholestatic pruritus. They received a total of 135 MARS sessions during 8, 4, and 13 months prior to liver transplantation. Total serum bilirubin and bile acids were monitored, and pruritus was assessed by a numerical rating scale (NRS 0 = no pruritus, 10 = maximal pruritus).

Results

MARS sessions were initially performed three times weekly at a mean duration of 6.3 ± 1.4 h. Sessions could be reduced to once weekly and once every other week in two patients. Pre-MARS plasma bile acid concentrations averaged 207 ± 67 μmol/l. They declined to 67 ± 9%, 48 ± 3%, 38 ± 14%, and 37 ± 5% of baseline within 2, 4, 6 and 8 h of therapy, respectively (all p < 0.05). The average interdialytic increase of plasma bile acids was 34 ± 33 μmol/l per day. Mean NRS score decreased from 6.5 ± 2.3 to 3.3 ± 2.9 (p < 0.01). Skin lesions from itching disappeared. All MARS treatments were well tolerated.

Conclusion

MARS dialysis substantially reduces cholestatic pruritus in children refractory to pharmacological treatment.

Younossi ZM, Boparai N, Price LL, Kiwi ML, McCormick M, Guyatt G (2001) Health-related quality of life in chronic liver disease: the impact of type and severity of disease. Am J Gastroenterol 96:2199–2205PubMedCrossRef

Jones EA, Bergasa NV (1996) Why do cholestatic patients itch? Gut 38:644–645PubMedCrossRef

Régina A, Demeule M, Laplante A, Jodoin J, Dagenais C, Berthelet F, Moghrabi A, Béliveau R (2001) Multidrug resistance in brain tumors: roles of the blood-brain barrier. Cancer Metastasis Rev 20:13–25PubMedCrossRef

Dagenais C, Ducharme J, Pollack GM (2001) Uptake and efflux of the peptidic delta-opioid receptor agonist. Neurosci Lett 301:155–158PubMedCrossRef

Banks WA, Kastin AJ (1990) Peptide transport systems for opiates across the blood-brain barrier. Am J Physiol 259:1–10

Hofmann AF (2002) Cholestatic liver disease: pathophysiology and therapeutic options. Liver 22:14–19PubMedCrossRef

Franco J (1999) Pruritus. Curr Treat Options Gastroenterol 2:451–456PubMedCrossRef

Glasova H, Beuers U (2002) Extrahepatic manifestations of cholestasis. J Gastroenterol Hepatol 17:938–948PubMedCrossRef

Bergasa NV (2005) The pruritus of cholestasis. J Hepatol 43:1078–1088PubMed

10.

Hoek FJ, Grijm R, Sanders GT, Tytgat GN, Wilmink JM (1982) Removal of bile acids from the blood by hemodialysis with a polyacrylonitril membrane: treatment of pruritus of cholestatic disease. Digestion 23:135–140PubMedCrossRef

11.

Omokawa S, Yamashita M, Malchesky PS, Koo AP, Matsushita M, Nosè Y (1990) Therapeutic plasmapheresis for cholestatic liver diseases: study of 9 cases. Prog Clin Biol Res 337:233–236PubMed

12.

Geerdink P, Snel P, van Berge Henegouwen GP, Huybregts A, Tangerman A, Kunst VA, van Tongeren JH (1978) Treatment of intractable pruritus in patients with cholestatic jaundice by plasma exchange and plasma perfusion. Neth J Med 21:239–244PubMed

13.

Jalan R, Sen S, Steiner C, Kapoor D, Alisa A, Williams R (2003) Extracorporeal liver support with molecular adsorbents recirculating system in patients with severe acute alcoholic hepatitis. J Hepatol 38:24–31PubMedCrossRef

14.

Sorkine P, Ben Abraham R, Szold O, Biderman P, Kidron A, Merchav H, Brill S, Oren R (2001) Role of the molecular adsorbent recycling system (MARS) in the treatment of patients with acute exacerbation of chronic liver failure. Crit Care Med 29:1332–1336PubMedCrossRef

15.

Schmidt LE, Svendsen LB, Sørensen VR, Hansen BA, Larsen FS (2001) Cerebral blood flow velocity increases during a single treatment with the molecular adsorbents recirculating system in patients with acute on chronic liver failure. Liver Transpl 7:709–712PubMedCrossRef

16.

Schmidt LE, Wang LP, Hansen BA, Larsen FS (2003) Systemic hemodynamic effects of treatment with the molecular adsorbents recirculating system in patients with hyperacute liver failure: a prospective controlled trial. Liver Transpl 9:290–297PubMedCrossRef

17.

Mitzner SR, Klammt S, Peszynski P, Hickstein H, Korten G, Stange J, Schmidt R (2001) Improvement of multiple organ functions in hepatorenal syndrome during albumin dialysis with the molecular adsorbent recirculating system. Ther Apher 5:417–422PubMedCrossRef

18.

Kantola T, Mäklin S, Koivusalo AM, Räsänen P, Rissanen A, Roine R, Sintonen H, Höckerstedt K, Isoniemi H (2010) Cost-utility of molecular adsorbent recirculating system treatment in acute liver failure. World J Gastroenterol 16:2227–2234PubMedCrossRef

19.

Parés A, Herrera M, Avilés J, Sanz M, Mas A (2010) Treatment of resistant pruritus from cholestasis with albumin dialysis: combined analysis of patients from three centers. J Hepatol 53:307–312PubMedCrossRef

20.

Bellmann R, Graziadei IW, Feistritzer C, Schwaighofer H, Stellaard F, Sturm E, Wiedermann CJ, Joannidis M (2004) Treatment of refractory cholestatic pruritus after liver transplantation with albumin dialysis. Liver Transpl 10:107–114PubMedCrossRef

21.

Novelli G, Rossi M, Poli L, Predagostini R, Iappelli M, Morabito V, Lai Q, Novelli L, Mennini G, Corradini SG, Berloco PB (2006) Intractable pruritus in patients with hepatitis C virus. Transplant Proc 38:1089–1091PubMedCrossRef

22.

Montero JL, Pozo JC, Barrera P, Fraga E, Costán G, Domínguez JL, Muntané J, Rodriguez-Ariza A, Pleguezuelo M, Rufián S, López-Cillero P, de la Mata M (2006) Treatment of refractory cholestatic pruritus with molecular adsorbent recirculating system (MARS). Transplant Proc 38:2511–2513PubMedCrossRef

23.

Doria C, Mandalá L, Smith J, Vitale CH, Lauro A, Gruttadauria S, Marino IR, Foglieni CS, Magnone M, Scott VL (2003) Effect of molecular adsorbent recirculating system in hepatitis C virus-related intractable pruritus. Liver Transpl 9:437–443PubMedCrossRef

24.

Javouhey E, Ranchin B, Lachaux A, Boillot O, Martin X, Floret D, Cochat P (2009) Long-lasting extracorporeal albumin dialysis in a child with end-stage renal disease and severe cholestasis. Pediatr Transplant 13:235–239PubMedCrossRef

25.

Möller HJ (2009) Standardised rating scales in psychiatry: methodological basis, their possibilities and limitations and descriptions of important rating scales. World J Biol Psychiatry 10:6–26PubMedCrossRef

26.

Yerushalmi B, Sokol RJ, Narkewicz MR, Smith D, Karrer FM (1999) Use of rifampin for severe pruritus in children with chronic cholestasis. J Pediatr Gastroenterol Nutr 29:442–447PubMedCrossRef

27.

Keppler D, Leier I, Jedlitschky G (1997) Transport of glutathione conjugates and glucuronides by the multidrug resistance proteins MRP1 and MRP2. Biol Chem 378:787–791PubMed

28.

Meier PJ, Stieger B (2002) Bile salt transporters. Annu Rev Physiol 64:635–661PubMedCrossRef

29.

Schaefer B, Schaefer F, Engelmann G, Meyburg J, Heckert KH, Zorn M, Schmitt CP (2011) Comparison of Molecular Adsorbents Recirculating System (MARS) dialysis with combined plasma exchange and haemodialysis in children with acute liver failure. Nephrol Dial Transplant. doi:10.1093/ndt/gfr115

30.

Macia M, Avilés J, Navarro J, Morales S, García J (2003) Efficacy of molecular adsorbent recirculating system for the treatment of intractable pruritus in cholestasis. Am J Med 114:62–64PubMedCrossRef

31.

Bellmann R, Feistritzer C, Zoller H, Graziadei IW, Schwaighofer H, Propst A, Wiedermann CJ, Joannidis M (2004) Treatment of intractable pruritus in drug induced cholestasis with albumin dialysis: a report of two cases. ASAIO J 50:387–391PubMed

32.

Vanholder R, Van Laecke S, Glorieux G (2008) What is new in uremic toxicity? Pediatr Nephrol 23:1211–1221PubMedCrossRef

33.

Lloyd-Thomas HG, Sherlock S (1952) Testosterone therapy for the pruritus of obstructive jaundice. Br Med J 2:1289–1291PubMedCrossRef

34.

Auth MK, Kim HS, Beste M, Bonzel KE, Baumann U, Ballauff A, Wallot M, Borchers T, Vester U, Grasemann C, Hauffa B, Hoyer PF, Gerken G, Voit T (2005) Removal of metabolites, cytokines and hepatic growth factors by extracorporeal liver support in children. J Pediatr Gastroenterol Nutr 40:54–59PubMedCrossRef

35.

Riggio O, Mannaioni G, Ridola L, Angeloni S, Merli M, Carlà V, Salvatori FM, Moroni F (2010) Peripheral and splanchnic indole and oxindole levels in cirrhotic patients: a study on the pathophysiology of hepatic encephalopathy. Am J Gastroenterol 105:1374–1381PubMedCrossRef

36.

Silver RM, Heyes MP, Maize JC, Quearry B, Vionnet-Fuasset M, Sternberg EM (1990) Scleroderma, fasciitis, and eosinophilia associated with the ingestion of tryptophan. N Engl J Med 322:874–881PubMedCrossRef

37.

Meijers BK, Verhamme P, Nevens F, Hoylaerts MF, Bammens B, Wilmer A, Arnout J, Vanrenterghem Y, Evenepoel P (2007) Major coagulation disturbances during fractionated plasma separation and adsorption. Am J Transplant 7:2195–2199PubMedCrossRef

38.

Faybik P, Bacher A, Kozek-Langenecker SA, Steltzer H, Krenn CG, Unger S, Hetz H (2006) Molecular adsorbent recirculating system and hemostasis in patients at high risk of bleeding: an observational study. Crit Care 10:24CrossRef

39.

Bachli EB, Schuepbach RA, Maggiorini M, Stocker R, Müllhaupt B, Renner EL (2007) Artificial liver support with the molecular adsorbent recirculating system: activation of coagulation and bleeding complications. Liver Int 27:475–484PubMedCrossRef

40.

Doria C, Mandalà L, Smith JD, Caruana G, Scott VL, Gruttadauria S, Magnone M, Marino IR (2004) Thromboelastography used to assess coagulation during treatment with molecular adsorbent recirculating system. Clin Transplant 18:365–371PubMedCrossRef

Title: Molecular Adsorbents Recirculating System dialysis in children with cholestatic pruritus
Authors: Betti Schaefer
Franz Schaefer
Dieter Wittmer
Guido Engelmann
Daniel Wenning
Claus Peter Schmitt
Publication date: 01-05-2012
Publisher: Springer-Verlag
Published in: Pediatric Nephrology / Issue 5/2012
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-011-2058-8

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Molecular Adsorbents Recirculating System dialysis in children with cholestatic pruritus

Abstract

Background

Methods

Results

Conclusion

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Background

Methods

Results

Conclusion

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