Mathilde Mauras1, Justine Bacchetta2,3, Anita Duncan2, Marie-Pierre Lavocat1, Barbara Rohmer4, Etienne Javouhey3,5, Sophie Collardeau-Frachon3,6, Anne-Laure Sellier-Leclerc7. 1. Service de Pédiatrie, Hôpital de Saint Etienne, Saint-Etienne, France. 2. Centre de Référence des Maladies Rénales Rares, Hôpital Femme Mère Enfant, 59 Boulevard Pinel, 69677, Bron, France. 3. Faculté de Médecine Lyon Est, Université Lyon 1, Lyon, France. 4. Service de Gastro Entérologie Pédiatrique, HFME, Bron, France. 5. Service de Réanimation Pédiatrique, HFME, Bron, France. 6. Service d'Anatomopathologie, HFME, Bron, France. 7. Centre de Référence des Maladies Rénales Rares, Hôpital Femme Mère Enfant, 59 Boulevard Pinel, 69677, Bron, France. anne-laure.sellier-leclerc@chu-lyon.fr.
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.
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.
Authors: Anne-Laure Lapeyraque; Michal Malina; Véronique Fremeaux-Bacchi; Tobias Boppel; Michael Kirschfink; Mehdi Oualha; François Proulx; Marie-José Clermont; Françoise Le Deist; Patrick Niaudet; Franz Schaefer Journal: N Engl J Med Date: 2011-05-25 Impact factor: 91.245
Authors: N Urushihara; N Ariki; T Oyama; Y Chouda; T Yagi; T Inoue; Y Tomiyama; R Nishiuchi; M Oda; N Tanaka Journal: J Pediatr Surg Date: 2001-12 Impact factor: 2.545
Authors: J Matthies; C Hünseler; R Ehren; R Volland; F Körber; B Hoppe; L T Weber; S Habbig Journal: Klin Padiatr Date: 2016-06-13 Impact factor: 1.349