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

  • Mathilde Mauras
  • Justine Bacchetta
  • Anita Duncan
  • Marie-Pierre Lavocat
  • Barbara Rohmer
  • Etienne Javouhey
  • Sophie Collardeau-Frachon
  • Anne-Laure Sellier-LeclercEmail author
Brief Report



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


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.


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.


HUS Pediatrics Eculizumab Cholestatis Liver transplantation 



The authors would like to thank Pr Chantal Loirat for her expertise and skillful help during the management of the cases, as well as Dr Véronique Frémeaux-Bacchi for genetic explorations.

Compliance with ethical standards

This retrospective series of cases was approved by the local IRB (Comité d’éthique des Hospices Civils de Lyon, session May 3, 2017).

Conflict of interest

Justine Bacchetta and Anne-Laure Sellier-Leclerc received travel grants and honoraria from Alexion (2013, 2014).

Supplementary material

467_2019_4234_MOESM1_ESM.pptx (596 kb)
Chart 1 Chart: iconography of case report. Patient 1: a. Partially opacified cholangiography due to porous gallbladder walls with contrast product leaks. b. Simultaneous ultrasound found: an obstruction of the extrahepatic bile ducts by a mixture of sludge and microlithiasis, moderate dilation of the upstream bile ducts and inflammatory thickening of the gallbladder walls, seat of an infundibular macrolithiasis. c. Histology of hepatectomy with a diffuse greenish cholestatic aspect of the hepatic parenchyma. d. Electron microscopy of histology HESPortal area ×200: portal tract enlargement and fibrosis with acute inflammation, cholangitis and ductular proliferation. Patient 2: e. Homogeneous liver parenchyma. No intra or extrahepatic dilation of the bile ducts. Vesicular sludge associated with hydrocholecyst. f. Electron microscopy of histology HESPortal area ×200: mild chronic inflammation concerning a few portal tracts. Patient 3: g. BiliMRI: Hepatosplenomegaly. Moniliform dilation of the biliary tract suggestive of cholangitis with periportal hyper signal. Obstacle free vesicular distention. h. HES Centrolobular Area ×200: no lesions suggestive of thrombotic microangiopathy. i. Masson’s trichrome stain ×40: portal bridges. (PPTX 595 kb)


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Copyright information

© IPNA 2019

Authors and Affiliations

  • Mathilde Mauras
    • 1
  • Justine Bacchetta
    • 2
    • 3
  • Anita Duncan
    • 2
  • Marie-Pierre Lavocat
    • 1
  • Barbara Rohmer
    • 4
  • Etienne Javouhey
    • 3
    • 5
  • Sophie Collardeau-Frachon
    • 3
    • 6
  • Anne-Laure Sellier-Leclerc
    • 2
    Email author
  1. 1.Service de Pédiatrie, Hôpital de Saint EtienneSaint-EtienneFrance
  2. 2.Centre de Référence des Maladies Rénales RaresHôpital Femme Mère EnfantBronFrance
  3. 3.Faculté de Médecine Lyon EstUniversité Lyon 1LyonFrance
  4. 4.Service de Gastro Entérologie PédiatriqueHFMEBronFrance
  5. 5.Service de Réanimation PédiatriqueHFMEBronFrance
  6. 6.Service d’AnatomopathologieHFMEBronFrance

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