The treatment of atypical hemolytic uremic syndrome with eculizumab in pediatric patients: a systematic review

Souza, Raquel Medeiros de; Correa, Bernardo Henrique Mendes; Melo, Paulo Henrique Moreira; Pousa, Pedro Antunes; Mendonça, Tamires Sara Campos; Rodrigues, Lucas Gustavo Castelar; Silva, Ana Cristina Simões e

doi:10.1007/s00467-022-05683-2

Cited by 5 publications

(2 citation statements)

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“…A systematic review that considered 15 studies involving 940 pediatric patients with aHUS treated with eculizumab confirms that the treatment resulted in a satisfactory response, with improvements in kidney function and hematological parameters for most patients. However, most studies were observational and had small sample sizes 80 …”

Section: Two Prototypical Complement‐mediated Kidney Diseasesmentioning

confidence: 99%

“…However, most studies were observational and had small sample sizes. 80 Eculizumab has been shown to induce remission of acute episodes of aHUS when administered early after the onset of the disease, 16,81,82 but can also successfully be used as a prophylactic treatment to prevent post-transplantation aHUS recurrence in individuals who are at a moderate to high risk of recurrence. [83][84][85][86] Specifically, individuals with pathogenic variants in C3, CFB, and CFH or those who have the CFH/CFHR1 hybrid allele are considered to be at high risk for disease recurrence, whereas those carrying CFH antibodies, pathogenic variants in CFI, variants of uncertain significance, and/or no identified pathogenic variants are considered at moderate risk for disease recurrence.…”

Section: Therapy and Monitoringmentioning

confidence: 99%

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Kidney diseases

Daina

Cortinovis

2022

Immunological Reviews

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The complement system, a central part of the innate immunity that serves as a first line of defense against foreign and altered host cells, is an extremely effective cell-killing and inflammation-provoking pathway. However, complement activation is a double-edged sword because uncontrolled stimulation can be highly detrimental to host tissues. [1][2][3] In order to avoid self-damage, a plethora of inhibitory mechanisms are known to prevent overwhelming activation at all stages of the complement cascade. The alternative pathway (AP) of complement is particularly significant for survival against invading pathogens and can be triggered by several other conditions, such as trauma, surgery, or pregnancy. Inappropriate AP activation may be

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Section: Two Prototypical Complement‐mediated Kidney Diseasesmentioning

confidence: 99%