Eculizumab in the treatment of Shiga toxin haemolytic uraemic syndrome

التفاصيل البيبلوغرافية
العنوان: Eculizumab in the treatment of Shiga toxin haemolytic uraemic syndrome
المؤلفون: Patrick R. Walsh, Sally Johnson
المصدر: Pediatric Nephrology (Berlin, Germany)
بيانات النشر: Springer Science and Business Media LLC, 2018.
سنة النشر: 2018
مصطلحات موضوعية: Nephrology, medicine.medical_specialty, Complement Pathway, Alternative, Complement, 030232 urology & nephrology, Review, Disease, 030204 cardiovascular system & hematology, Antibodies, Monoclonal, Humanized, urologic and male genital diseases, Pathogenesis, 03 medical and health sciences, 0302 clinical medicine, Renal Dialysis, hemic and lymphatic diseases, Internal medicine, Haemolytic uraemic syndrome, medicine, Humans, Child, Shiga-Toxigenic Escherichia coli, STEC-HUS, biology, business.industry, Acute kidney injury, Shiga toxin, Complement System Proteins, Eculizumab, medicine.disease, female genital diseases and pregnancy complications, Complement system, Complement Inactivating Agents, Treatment Outcome, Hemolytic-Uremic Syndrome, Pediatrics, Perinatology and Child Health, Immunology, biology.protein, Haemolytic-uraemic syndrome, Erythrocyte Transfusion, business, medicine.drug
الوصف: Haemolytic uraemic syndrome (HUS) remains a leading cause of paediatric acute kidney injury (AKI). Haemolytic uraemic syndrome is characterised by the triad of microangiopathic haemolytic anaemia, thrombocytopenia and AKI. In ~ 90% of cases, HUS is a consequence of infection with Shiga toxin-producing E. coli (STEC), most commonly serotype O157:H7. Acute mortality from STEC-HUS is now less than 5%; however, there is significant long-term renal morbidity in one third of survivors. Currently, no specific treatment exists for STEC-HUS. There is growing interest in the role of complement in the pathogenesis of STEC-HUS due to the discovery of inherited and acquired dysregulation of the alternative complement system in the closely related disorder, atypical HUS (aHUS). The treatment of aHUS has been revolutionised by the introduction of the anti-C5 monoclonal antibody, eculizumab. However, the role of complement and anti-complement therapy in STEC-HUS remains unclear. Herein, we review the current evidence of the role of complement in STEC-HUS focusing on the use of eculizumab in this disease.
تدمد: 1432-198X
0931-041X
الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::cb2f9c8def4d9afb4310d67d3f7197a6Test
https://doi.org/10.1007/s00467-018-4025-0Test
حقوق: OPEN
رقم الانضمام: edsair.doi.dedup.....cb2f9c8def4d9afb4310d67d3f7197a6
قاعدة البيانات: OpenAIRE