Acute Myocarditis and Eculizumab Caused Severe Cholestasis in a 17-Month-Old Child Who Has Hemolytic Uremic Syndrome Associated with Shiga Toxin-Producing Escherichia coli

Abstract

Cardiovascular involvement is uncommon in pediatric patients with hemolytic uremic syndrome associated with Shiga toxin-producing Escherichia coli (STEC-HUS). In this case report we presented a case of 17-month-old toddler who had a sporadic type of STEC-HUS complicated by acute myocarditis. The patient was successfully treated by a single dose of eculizumab after six doses of therapeutic plasma exchange (TPE) were inefficient to prevent the cardiac complication. Hepatotoxicity was observed after a single dose of eculizumab. Hepatic and cholestatic enzyme levels slowly returned to normal within 6 months. To the best of our knowledge, this is the first case of myocarditis/cardiomyopathy treated with eculizumab in STEC-HUS. This case illustrates the need for vigilance regarding myocardial involvement and eculizumab-induced hepatotoxicity in STEC-HUS.

Keywords

hemolytic uremic syndrome - myocarditis - eculizumab - therapeutic plasma exchange

Authors' Contributions

All authors participated in creating content for the manuscript, editing, and provided final approval for submission. No undisclosed authors contributed to the manuscript.

Ethical Approval

Permission was granted by the parents and patient to publish the case report.

Publikationsverlauf

Eingereicht: 14. Februar 2020

Angenommen: 02. Mai 2020

Artikel online veröffentlicht:
05. August 2020

• References

• 1 Canpolat N. Hemolytic uremic syndrome. Turk Pediatri Ars 2015; 50 (02) 73-82
• 2 Khalid M, Andreoli S. Extrarenal manifestations of the hemolytic uremic syndrome associated with Shiga toxin-producing Escherichia coli (STEC HUS). Pediatr Nephrol 2019; 34 (12) 2495-2507
• 3 Thayu M, Chandler WL, Jelacic S, Gordon CA, Rosenthal GL, Tarr PI. Cardiac ischemia during hemolytic uremic syndrome. Pediatr Nephrol 2003; 18 (03) 286-289
• 4 F Andersen R, V Bjerre J, V Povlsen J, Veien M, Kamperis K, Rittig S. HUS-induced cardiac and circulatory failure is reversible using cardiopulmonary bypass as rescue. Pediatr Nephrol 2017; 32 (11) 2155-2158
• 5 Palanca Arias D, López Ramón M, Jiménez Montañés L. Biomarkers detect involvement of acute myocardial injury in a paediatric haemolytic-uraemic syndrome patient. Cardiol Young 2016; 26 (05) 983-986
• 6 Abu-Arafeh I, Gray E, Youngson G, Auchterlonie I, Russell G. Myocarditis and haemolytic uraemic syndrome. Arch Dis Child 1995; 72 (01) 46-47
• 7 Ray CG, Portman JN, Stamm SJ, Hickman RO. Hemolytic-uremic syndrome and myocarditis. Association with coxsackievirus B infection. Am J Dis Child 1971; 122 (05) 418-420
• 8 Buelli S, Zoja C, Remuzzi G, Morigi M. Complement activation contributes to the pathophysiology of shiga toxin-associated hemolytic uremic syndrome. Microorganisms 2019; 7 (01) E15
• 9 Loos S, Oh J, Kemper MJ. Eculizumab in STEC-HUS: need for a proper randomized controlled trial. Pediatr Nephrol 2018; 33 (08) 1277-1281
• 10 Mahat U, Matar RB, Rotz SJ. Use of complement monoclonal antibody eculizumab in Shiga toxin producing Escherichia coli associated hemolytic uremic syndrome: a review of current evidence. Pediatr Blood Cancer 2019; 66 (11) e27913
• 11 Percheron L, Gramada R, Tellier S. et al. Eculizumab treatment in severe pediatric STEC-HUS: a multicenter retrospective study. Pediatr Nephrol 2018; 33 (08) 1385-1394
• 12 Hayes W, Tschumi S, Ling SC, Feber J, Kirschfink M, Licht C. Eculizumab hepatotoxicity in pediatric aHUS. Pediatr Nephrol 2015; 30 (05) 775-781
• 13 Oruc A, Ayar Y, Vuruskan BA. et al. Hepatotoxicity associated with eculizumab in a patient with atypical hemolytic uremic syndrome. Nefrologia 2018; 38 (04) 448-450
• 14 Yesilbas O, Sevketoglu E, Petmezci MT. et al. Infant onset severe complement-mediated hemolytic uremic syndrome complicated by secondary sclerosing cholangitis. J Clin Apher 2018; 33 (05) 619-623
• 15 Mauras M, Bacchetta J, Duncan A. et al. Escherichia coli-associated hemolytic uremic syndrome and severe chronic hepatocellular cholestasis: complication or side effect of eculizumab?. Pediatr Nephrol 2019; 34 (07) 1289-1293