IgG-immunoadsorptions and eculizumab combination in STEC-hemolytic and uremic syndrome pediatric patients with neurological involvement

Karmali MA, Petric M, Lim C, Fleming PC, Arbus GS, Lior H (1985) The association between idiopathic hemolytic uremic syndrome and infection by verotoxin-producing Escherichia coli. J Infect Dis 151:775–782

Article  PubMed  CAS  Google Scholar 

Tarr PI, Gordon CA, Chandler WL (2005) Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome. Lancet 365:1073–1086

PubMed  CAS  Google Scholar 

Siegler RL (1995) The hemolytic uremic syndrome. Pediatr Clin North Am 42:1505–1529

Article  PubMed  CAS  Google Scholar 

Sheth KJ, Swick HM, Haworth N (1986) Neurological involvement in hemolytic-uremic syndrome. Ann Neurol 19:90–93

Article  PubMed  CAS  Google Scholar 

Oakes RS, Siegler RL, McReynolds MA, Pysher T, Pavia AT (2006) Predictors of fatality in postdiarrheal hemolytic uremic syndrome. Pediatrics 117:1656–1662

Article  PubMed  Google Scholar 

Rosales A, Hofer J, Zimmerhackl LB, Jungraithmayr TC, Riedl M, Giner T, Strasak A, Orth-Höller D, Würzner R, Karch H, German-Austrian HUS Study Group (2012) Need for long-term follow-up in enterohemorrhagic Escherichia coli-associated hemolytic uremic syndrome due to late-emerging sequelae. Clin Infect Dis 54:1413–1421

Article  PubMed  Google Scholar 

Spinale JM, Ruebner RL, Copelovitch L, Kaplan BS (2013) Long-term outcomes of Shiga toxin hemolytic uremic syndrome. Pediatr Nephrol 28:2097–2105

Article  PubMed  Google Scholar 

Monet-Didailler C, Godron-Dubrasquet A, Madden I, Delmas Y, Llanas B, Harambat J (2019) Long-term outcome of diarrhea-associated hemolytic uremic syndrome is poorly related to markers of kidney injury at 1-year follow-up in a population-based cohort. Pediatr Nephrol 34:657–662

Article  PubMed  Google Scholar 

Legendre CM, Licht C, Muus P, Greenbaum LA, Babu S, Bedrosian C, Bingham C, Cohen DJ, Delmas Y, Douglas K, Eitner F, Feldkamp T, Fouque D, Furman RR, Gaber O, Herthelius M, Hourmant M, Karpman D, Lebranchu Y, Mariat C, Menne J, Moulin B, Nürnberger J, Ogawa M, Remuzzi G, Richard T, Sberro-Soussan R, Severino B, Sheerin NS, Trivelli A, Zimmerhackl LB, Goodship T, Loirat C (2013) Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med 368:2169–2181

Article  PubMed  CAS  Google Scholar 

Licht C, Greenbaum LA, Muus P, Babu S, Bedrosian CL, Cohen DJ, Delmas Y, Douglas K, Furman RR, Gaber OA, Goodship T, Herthelius M, Hourmant M, Legendre CM, Remuzzi G, Sheerin N, Trivelli A, Loirat C (2015) Efficacy and safety of eculizumab in atypical hemolytic uremic syndrome from 2-year extensions of phase 2 studies. Kidney Int 87:1061–1073

Article  PubMed  PubMed Central  CAS  Google Scholar 

Fakhouri F, Hourmant M, Campistol JM, Cataland SR, Espinosa M, Gaber AO, Menne J, Minetti EE, Provôt F, Rondeau E, Ruggenenti P, Weekers LE, Ogawa M, Bedrosian CL, Legendre CM (2016) Terminal complement inhibitor eculizumab in adult patients with atypical hemolytic uremic syndrome: a single-arm, open-label trial. Am J Kidney Dis 68:84–93

Article  PubMed  CAS  Google Scholar 

Greenbaum LA, Fila M, Ardissino G, Al-Akash SI, Evans J, Henning P, Lieberman KV, Maringhini S, Pape L, Rees L, van de Kar NC, Vande Walle J, Ogawa M, Bedrosian CL, Licht C (2016) Eculizumab is a safe and effective treatment in pediatric patients with atypical hemolytic uremic syndrome. Kidney Int 89:701–711

Article  PubMed  CAS  Google Scholar 

Monnens L, Molenaar J, Lambert PH, Proesmans W, van Munster P (1980) The complement system in hemolytic-uremic syndrome in childhood. Clin Nephrol 13:168–171

PubMed  CAS  Google Scholar 

Thurman JM, Marians R, Emlen W, Wood S, Smith C, Akana H, Holers VM, Lesser M, Kline M, Hoffman C, Christen E, Trachtman H (2009) Alternative pathway of complement in children with diarrhea-associated hemolytic uremic syndrome. Clin J Am Soc Nephrol 4:1920–1924

Article  PubMed  PubMed Central  CAS  Google Scholar 

Orth D, Khan AB, Naim A, Grif K, Brockmeyer J, Karch H, Joannidis M, Clark SJ, Day AJ, Fidanzi S, Stoiber H, Dierich MP, Zimmerhackl LB, Würzner R (2009) Shiga toxin activates complement and binds factor H: evidence for an active role of complement in hemolytic uremic syndrome. J Immunol 182:6394–6400

Article  PubMed  CAS  Google Scholar 

Ståhl AL, Sartz L, Karpman D (2011) Complement activation on platelet-leukocyte complexes and microparticles in enterohemorrhagic Escherichia coli-induced hemolytic uremic syndrome. Blood 117:5503–5513

Article  PubMed  Google Scholar 

Morigi M, Galbusera M, Gastoldi S, Locatelli M, Buelli S, Pezzotta A, Pagani C, Noris M, Gobbi M, Stravalaci M, Rottoli D, Tedesco F, Remuzzi G, Zoja C (2011) Alternative pathway activation of complement by Shiga toxin promotes exuberant C3a formation that triggers microvascular thrombosis. J Immunol 187:172–180

Article  PubMed  CAS  Google Scholar 

Ahlenstiel-Grunow T, Hachmeister S, Bange FC et al (2016) Systemic complement activation and complement gene analysis in enterohaemorrhagic Escherichia coli-associated paediatric haemolytic uraemic syndrome. Nephrol Dial Transplant 31:1114–1121

Article  PubMed  CAS  Google Scholar 

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 1:11

Article  PubMed  PubMed Central  Google Scholar 

Karnisova L, Hradsky O, Blahova K et al (2018) Complement activation is associated with more severe course of diarrhea-associated hemolytic uremic syndrome, a preliminary study. Eur J Pediatr 177:1837–1844

Article  PubMed  Google Scholar 

Ferraris JR, Ferraris V, Acquier AB et al (2015) Activation of the alternative pathway of complement during the acute phase of typical haemolytic uraemic syndrome. Clin Exp Immunol 181:118–125

Article  PubMed  PubMed Central  CAS  Google Scholar 

Netti GS, Santangelo L, Paulucci L et al (2020) Low C3 serum levels predict severe forms of STEC-HUS with neurologic involvement. Front Med (Lausanne) 7:357

Article  PubMed  Google Scholar 

Lapeyraque AL, Malina M, Fremeaux-Bacchi V et al (2011) Eculizumab in severe Shiga-toxin-associated HUS. N Engl J Med 364:2561–2563

Article  PubMed  CAS  Google Scholar 

Kielstein JT, Beutel G, Fleig S, Steinhoff J, Meyer TN, Hafer C, Kuhlmann U, Bramstedt J, Panzer U, Vischedyk M, Busch V, Ries W, Mitzner S, Mees S, Stracke S, Nürnberger J, Gerke P, Wiesner M, Sucke B, Abu-Tair M, Kribben A, Klause N, Schindler R, Merkel F, Schnatter S, Dorresteijn EM, Samuelsson O, Brunkhorst R, Collaborators of the DGfN STEC-HUS registry (2012) Best supportive care and therapeutic plasma exchange with or without eculizumab in Shiga-toxin-producing E. coli O104:H4 induced haemolytic-uraemic syndrome: an analysis of the German STEC-HUS registry. Nephrol Dial Transplant 27:3807–3815

Article  PubMed  CAS  Google Scholar 

Menne J, Nitschke M, Stingele R, Abu-Tair M, Beneke J, Bramstedt J, Bremer JP, Brunkhorst R, Busch V, Dengler R, Deuschl G, Fellermann K, Fickenscher H, Gerigk C, Goettsche A, Greeve J, Hafer C, Hagenmüller F, Haller H, Herget-Rosenthal S, Hertenstein B, Hofmann C, Lang M, Kielstein JT, Klostermeier UC, Knobloch J, Kuehbacher M, Kunzendorf U, Lehnert H, Manns MP, Menne TF, Meyer TN, Michael C, Münte T, Neumann-Grutzeck C, Nuernberger J, Pavenstaedt H, Ramazan L, Renders L, Repenthin J, Ries W, Rohr A, Rump LC, Samuelsson O, Sayk F, Schmidt BM, Schnatter S, Schöcklmann H, Schreiber S, von Seydewitz CU, Steinhoff J, Stracke S, Suerbaum S, van de Loo A, Vischedyk M, Weissenborn K, Wellhöner P, Wiesner M, Zeissig S, Büning J, Schiffer M, Kuehbacher T, EHEC-HUS consortium (2012) Validation of treatment strategies for enterohaemorrhagic Escherichia coli O104:H4 induced haemolytic uraemic syndrome: case-control study. BMJ 345:e4565

Article  PubMed  PubMed Central  Google Scholar 

Walsh PR, Johnson S (2019) Eculizumab in the treatment of Shiga toxin haemolytic uraemic syndrome. Pediatr Nephrol 34:1485–1492

Article  PubMed  Google Scholar 

Loos S, Aulbert W, Hoppe B et al (2017) Intermediate follow-up of pediatric patients with hemolytic uremic syndrome during the 2011 outbreak caused by E. coli O104:H4. Clin Infect Dis 64:1637–1643

Article  PubMed  Google Scholar 

Loos S, Ahlenstiel T, Kranz B et al (2012) An outbreak of Shiga toxin-producing Escherichia coli O104:H4 hemolytic uremic syndrome in Germany: presentation and short-term outcome in children. Clin Infect Dis 55:753–759

Article  PubMed  CAS  Google Scholar 

Delmas Y, Vendrely B, Clouzeau B et al (2014) Outbreak of Escherichia coli O104:H4 haemolytic uraemic syndrome in France: outcome with eculizumab. Nephrol Dial Transplant 29:565–572

Article  PubMed  CAS 

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