Treatments administered to patients positive or negative for anti-FH autoantibodies at disease onset
Treatments
Anti-FH positive, n = 12
Anti-FH negative, n = 30
P-value (Fisher’exact test)
Dialysis
6 (50%)
15 (50%)
1.00
Plasmapheresis
5 (42%)
11 (37%)
1.00
Plasmapheresis followed by eculizumab
3 (25%)
3 (10%)
0.33
Eculizumab only
2 (17%)
1 (3%)
0.19
Supportive care only
1 (8%)
10 (33%)
0.13
Immunosupression
8 (67%)
9 (30%)
< 0.05
Corticosteroids
6 (50%)
9 (30%)
0.29
Mycophenolate, cyclophosphamide
4 (33%)
0 (0%)
< 0.005
Values in this table represent number of patients and the percentage of the total
Declarations
Acknowledgments
We would like to thank the patients and their families for their participation in the study, as well as the physicians and the institutions for their contribution to the acquisition of patients data. We thank James Castaneda, Bespoke English, for his diligent proofreading of this manuscript.
Author contributions
CDS: Conceptualization, Investigation, Visualization, Writing—original draft, Writing—review & editing. JT and SC: Investigation, Formal analysis. LA and PAC: Investigation, Resources, Writing—review & editing. FJM: Investigation, Resources. MFA: Investigation, Formal analysis, Writing—review & editing. ASL: Conceptualization, Investigation, Visualization, Resources, Writing—review & editing. All authors read and approved the submitted version.
Conflicts of interest
The authors declare that they have no conflicts of interest.
Ethical approval
The study was approved by the Institutional Review Board (or Ethics Committee) of Academia Nacional de Medicina, Buenos Aires (28/22/CEIANM, dated 7/09/2022), and it complies with the Declaration of Helsinki.
Consent to participate
Informed consent to participate in the study was obtained from parents/legal guardians.
Consent to publication
Not applicable.
Availability of data and materials
The datasets for this manuscript are not publicly available due to privacy and ethical concerns. Requests for accessing the datasets should be directed to Célia Dos Santos (cdossantosimex@gmail.com).
Funding
This study was supported by Fundación Baron, CONICET, Agencia I+D+I – FONCYT and Academia Nacional de Medicina de Buenos Aires providing fellowship funding for graduate/PhD students and funding for material and reagents. This study was partially funded by educational grants (2015–2017) from Alexion Argentina and grant for clinical support from Laboratorios Raffo (2018–2022). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
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