دورية أكاديمية
Anti-factor H autoantibody-associated hemolytic uremic syndrome in an Argentine pediatric cohort
| العنوان: | Anti-factor H autoantibody-associated hemolytic uremic syndrome in an Argentine pediatric cohort |
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| المؤلفون: | Célia Dos Santos, Jesica Trinidad, Santiago Castera, Laura Alconcher, Paula Alejandra Coccia, Federico Javier Manni, María Fabiana Alberto, Analía Sánchez-Luceros |
| المصدر: | Exploration of Immunology, Vol 3, Iss 6, Pp 513-524 (2023) |
| بيانات النشر: | Open Exploration Publishing Inc., 2023. |
| سنة النشر: | 2023 |
| المجموعة: | LCC:Immunologic diseases. Allergy |
| مصطلحات موضوعية: | atypical hemolytic uremic syndrome, complement, factor h, autoantibody, children, Immunologic diseases. Allergy, RC581-607 |
| الوصف: | Aim: To describe the clinical characteristics and frequency of anti-factor H (FH) autoantibody-associated atypical hemolytic uremic syndrome (aHUS) in the first cohort of Argentine patients. Methods: The presence of anti-FH autoantibodies in 70 pediatric patients with suspected aHUS was investigated between 2013 and 2022. Clinical and laboratory parameters were collected and compared between patients who were positive and negative for anti-FH antibodies. Results: The 70 patients screened for anti-FH autoantibodies presented clinical features of non-immune microangiopathic hemolytic anemia, thrombocytopenia and renal injury. Positive titers were found in 14 children [mean: 1,938 arbitrary units per mL (AU/mL), range 179–8,500]. Due to missing clinical data, two patients who tested positive for anti-FH and 20 patients who tested negative for anti-FH were excluded from the data analysis. The laboratory features and clinical manifestations of anti-FH-positive aHUS cases (n = 12) were very similar to those of subjects with no autoantibodies detected (n = 36). Treatment administration was heterogeneous among the 12 patients analyzed. Dialysis was performed in six patients in total. Five children received plasmapheresis, while three patients were treated with plasma exchange followed by administration of eculizumab. Two patients received eculizumab only and one showed significant improvement solely through supportive care. Eight patients in total received immunosuppressive therapy. Follow-up of three patients showed a significant decrease of anti-FH autoantibody titers in 2/3 after treatment and during clinical remission. Conclusions: The cohort of 70 pediatric patients in this study demonstrated that the frequency of anti-FH autoantibody-associated aHUS in Argentina is 20%. The implementation of anti-FH testing in the country can potentially contribute to improved treatment and follow-up for patients with autoimmune aHUS. |
| نوع الوثيقة: | article |
| وصف الملف: | electronic resource |
| اللغة: | English |
| تدمد: | 2768-6655 79989977 |
| Relation: | https://www.explorationpub.com/Journals/ei/Article/1003118; https://doaj.org/toc/2768-6655 |
| DOI: | 10.37349/ei.2023.00118 |
| URL الوصول: | https://doaj.org/article/ec4a62bdef09433b952b7998997732d8 |
| رقم الأكسشن: | edsdoj.4a62bdef09433b952b7998997732d8 |
| قاعدة البيانات: | Directory of Open Access Journals |
Full Text Finder | تدمد: | 27686655 79989977 |
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| DOI: | 10.37349/ei.2023.00118 |