The role of complement in Streptococcus pneumoniae-associated haemolytic uraemic syndrome

Szilágyi, Ágnes; Kiss, Nóra; Bereczki, Csaba; Tálosi, Gyula; Rácz, Katalin; Túri, Sándor; Györke, Zsuzsa; Simon, Edina; Horváth, Eszter; Kelen, Kata; Reusz, György; Szabó, Attila; Tulassay, Tivadar; Prohászka, Zoltán

doi:10.1093/ndt/gft198

Cited by 71 publications

(64 citation statements)

References 37 publications

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“…The most frequently reported diseases that lead to clinically evident secondary HUS include infections, especially those caused by Streptococcus pneumoniae, 23 and the influenza virus. 24 These infections are considered as causes of HUS not just triggers for the disease, although the distinction between cause and trigger is not evident.…”

Section: Secondary Husmentioning

confidence: 99%

“…We observed that sialic acid in in vitro assays is the main ligand for factor H on red cells and also on platelets and endothelial cells. 59 Pneumococci 23 and influenza A virus, 24 most often seen in secondary HUS associated with infections, possess very active neuraminidases specialized in removing sialic For personal use only. on May 10, 2018. by guest www.bloodjournal.org From acid from self cell surfaces.…”

Section: Complement In Disease Processes and Therapy Of Hus Infectionmentioning

confidence: 99%

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HUS and atypical HUS

Jokiranta

2017

Blood

240

260

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Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy characterized by intravascular hemolysis, thrombocytopenia, and acute kidney failure. HUS is usually categorized as typical, caused by Shiga toxin–producing Escherichia coli (STEC) infection, as atypical HUS (aHUS), usually caused by uncontrolled complement activation, or as secondary HUS with a coexisting disease. In recent years, a general understanding of the pathogenetic mechanisms driving HUS has increased. Typical HUS (ie, STEC-HUS) follows a gastrointestinal infection with STEC, whereas aHUS is associated primarily with mutations or autoantibodies leading to dysregulated complement activation. Among the 30% to 50% of patients with HUS who have no detectable complement defect, some have either impaired diacylglycerol kinase ε (DGKε) activity, cobalamin C deficiency, or plasminogen deficiency. Some have secondary HUS with a coexisting disease or trigger such as autoimmunity, transplantation, cancer, infection, certain cytotoxic drugs, or pregnancy. The common pathogenetic features in STEC-HUS, aHUS, and secondary HUS are simultaneous damage to endothelial cells, intravascular hemolysis, and activation of platelets leading to a procoagulative state, formation of microthrombi, and tissue damage. In this review, the differences and similarities in the pathogenesis of STEC-HUS, aHUS, and secondary HUS are discussed. Common for the pathogenesis seems to be the vicious cycle of complement activation, endothelial cell damage, platelet activation, and thrombosis. This process can be stopped by therapeutic complement inhibition in most patients with aHUS, but usually not those with a DGKε mutation, and some patients with STEC-HUS or secondary HUS. Therefore, understanding the pathogenesis of the different forms of HUS may prove helpful in clinical practice.

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Section: Secondary Husmentioning

confidence: 99%

Section: Complement In Disease Processes and Therapy Of Hus Infectionmentioning

confidence: 99%

HUS and atypical HUS

Jokiranta

2017

Blood

240

260

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“…terápiája [68] Mivel az alternatív komplementaktivációs út szabályozá-sában központi szerepet játszó H-faktor a sziálsavhoz kötődik a sejtmembránon, és átfedés lehet a neuraminidázmediált és a komplementmediált aHUS formák kö-zött [69], indokolt a diagnosztikai vizsgálatok kiterjesztése a komplementrendszer irányába is.…”

Section: A Streptococcus Pneumoniae-hus (Sp-hus)unclassified

Egészségügyi szakmai irányelv – A thromboticus thrombocytopeniás purpura (TTP) és a haemolyticus uraemiás syndroma (HUS) kezeléséről

…¹

2017

Orvosi Hetilap

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“…Although SpHUS does not clearly fit under the rubric of aHUS, new studies may change this perception because complement dysregulation may be occurring in at least some cases of SpHUS (23). SpHUS (9,28) can be defined as acute hemolytic anemia, thrombocytopenia and acute kidney injury in a patient with invasive S. pneumoniae infection.…”

Section: S Pneumoniae Husmentioning

confidence: 99%