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01-07-2019 | Liver Transplantation | Brief Report

Escherichia coli-associated hemolytic uremic syndrome and severe chronic hepatocellular cholestasis: complication or side effect of eculizumab?

Authors: Mathilde Mauras, Justine Bacchetta, Anita Duncan, Marie-Pierre Lavocat, Barbara Rohmer, Etienne Javouhey, Sophie Collardeau-Frachon, Anne-Laure Sellier-Leclerc

Published in: Pediatric Nephrology | Issue 7/2019

Abstract

Background

Liver lesions of hemolytic uremic syndrome due to Shiga-toxin-producing Escherichia coli (STEC-HUS) are uncommon.

Case-diagnosis/treatment

We report three observations of severe STEC-HUS with delayed hepatic involvement. They presented with multiple organ failure and received eculizumab; 15 days after the onset of STEC-HUS, cholestasis appeared and cytolysis worsened. Abdominal ultrasonography showed vesicular sludge. Liver biopsy performed 3 to 6 months after the STEC-HUS found cholangiolar proliferation and inflammatory portal fibrosis. Despite renal recovery, cholestasis persisted and worsened in two cases, leading to biliary cirrhosis and subsequent liver transplantation. Pathological examination of one native liver found thrombotic microangiopathy.

Conclusions

Even though the pathological examination performed on one native liver demonstrated areas of thrombotic microangiopathy, we cannot completely rule out that eculizumab may have worsened the liver lesions. Before the efficacy of eculizumab in STEC-HUS is formally demonstrated, physicians should stay cautious in its use.

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Fakhouri F, Zuber J, Frémeaux-Bacchi V, Loirat C (2017) Haemolytic uraemic syndrome. Lancet 390:681–696. https://doi.org/10.1016/S0140-6736(17)30062-4 CrossRefPubMed

de Buys Roessingh AS, de Lagausie P, Baudoin V, Loirat C, Aigrain Y (2007) Gastrointestinal complications of post-diarrheal hemolytic uremic syndrome. Eur J Pediatr Surg 17:328–334. https://doi.org/10.1055/s-2007-965013 CrossRefPubMed

Lapeyraque A-L, Malina M, Fremeaux-Bacchi V, Boppel T, Kirschfink M, Oualha M, Proulx F, Clermont MJ, Le Deist F, Niaudet P, Schaefer F (2011) Eculizumab in severe Shiga-toxin-associated HUS. N Engl J Med 364:2561–2563. https://doi.org/10.1056/NEJMc1100859 CrossRefPubMed

Brady TM, Pruette C, Loeffler LF, Weidemann D, Strouse JJ, Gavriilaki E, Brodsky RA (2016) Typical Hus: evidence of acute phase complement activation from a daycare outbreak. J Clin Exp Nephrol 1:11CrossRefPubMedPubMedCentral

Urushihara N, Ariki N, Oyama T, Chouda Y, Yagi T, Inoue T, Tomiyama Y, Nishiuchi R, Oda M, Tanaka N (2001) Secondary sclerosing cholangitis and portal hypertension after O157 enterocolitis: extremely rare complications of hemolytic uremic syndrome. J Pediatr Surg 36:1838–1840. https://doi.org/10.1053/jpsu.2001.28858 CrossRefPubMed

Matthies J, Hünseler C, Ehren R, Volland R, Körber F, Hoppe B, Weber LT, Habbig S (2016) Extrarenal manifestations in Shigatoxin-associated haemolytic uremic syndrome. Klin Padiatr 228:181–188. https://doi.org/10.1055/s-0042-108444 CrossRefPubMed

Riley MR, Lee KK (2004) Escherichia coli O157:H7-associated hemolytic uremic syndrome and acute hepatocellular cholestasis: a case report. J Pediatr Gastroenterol Nutr 38:352–354CrossRefPubMed

McCarthy DW, Mutabagani K, Mahan JD, Caniano DA, Cooney DR (2000) Infarction of the choledochus, liver, gallbladder, and pancreas: a unique complication of the hemolytic uremic syndrome. J Pediatr Surg 35:502–504. https://doi.org/10.1016/S0022-3468(00)90223-9 CrossRefPubMed

Anastaze Stelle K, Cachat F, Perez M-H, Chehade H (2016) Streptococcus pneumoniae-associated hemolytic and uremic syndrome with cholestasis: a case report and brief literature review. Clin Pediatr (Phila) 55:189–191. https://doi.org/10.1177/0009922815580406 CrossRef

10.

Gallo EG, Gianantonio CA (1995) Extrarenal involvement in diarrhoea-associated haemolytic-uraemic syndrome. Pediatr Nephrol 9:117–119CrossRefPubMed

11.

Delmas Y, Vendrely B, Clouzeau B, Bachir H, Bui HN, Lacraz A, Hélou S, Bordes C, Reffet A, Llanas B, Skopinski S, Rolland P, Gruson D, Combe C (2014) Outbreak of Escherichia coli O104:H4 haemolytic uraemic syndrome in France: outcome with eculizumab. Nephrol Dial Transplant 29:565–572. https://doi.org/10.1093/ndt/gft470 CrossRefPubMed

12.

Wijnsma KL, Schijvens AM, Rossen JWA, Kooistra-Smid AMDM, Schreuder MF, van de Kar NCAJ (2017) Unusual severe case of hemolytic uremic syndrome due to Shiga toxin 2d-producing E. coli O80:H2. Pediatr Nephrol 32:1263–1268. https://doi.org/10.1007/s00467-017-3642-3 CrossRefPubMedPubMedCentral

13.

Hayes W, Tschumi S, Ling SC, Feber J, Kirschfink M, Licht C (2015) Eculizumab hepatotoxicity in pediatric aHUS. Pediatr Nephrol 30:775–781. https://doi.org/10.1007/s00467-014-2990-5 CrossRefPubMed

14.

Brodsky A, Mazzocchi O, Sánchez F, Khursigara G, Malhotra S, Volpacchio M (2012) Eculizumab in paroxysmal nocturnal hemoglobinuria with Budd-Chiari syndrome progressing despite anticoagulation. Exp Hematol Oncol 1:26. https://doi.org/10.1186/2162-3619-1-26 CrossRefPubMedPubMedCentral

15.

Hillebrandt S, Wasmuth HE, Weiskirchen R, Hellerbrand C, Keppeler H, Werth A, Schirin-Sokhan R, Wilkens G, Geier A, Lorenzen J, Köhl J, Gressner AM, Matern S, Lammert F (2005) Complement factor 5 is a quantitative trait gene that modifies liver fibrogenesis in mice and humans. Nat Genet 37:835–843. https://doi.org/10.1038/ng1599 CrossRefPubMed

Title: Escherichia coli-associated hemolytic uremic syndrome and severe chronic hepatocellular cholestasis: complication or side effect of eculizumab?
Authors: Mathilde Mauras
Justine Bacchetta
Anita Duncan
Marie-Pierre Lavocat
Barbara Rohmer
Etienne Javouhey
Sophie Collardeau-Frachon
Anne-Laure Sellier-Leclerc
Publication date: 01-07-2019
Publisher: Springer Berlin Heidelberg
Keywords: Liver Transplantation
Escherichia Coli
Hemolytic Uremic Syndrome
Thrombotic Microangiopathy
Liver Transplantation
Liver Transplantation
Liver Transplantation
Liver Transplantation
Cholangitis
Published in: Pediatric Nephrology / Issue 7/2019
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-019-04234-6

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Escherichia coli-associated hemolytic uremic syndrome and severe chronic hepatocellular cholestasis: complication or side effect of eculizumab?

Abstract

Background

Case-diagnosis/treatment

Conclusions

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