ArticlePediatric nephrology (Berlin, Germany)2017
The clinical and laboratory features of Chinese Han anti-factor H autoantibody-associated hemolytic uremic syndrome.
Article in Pediatric nephrology (Berlin, Germany), 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers, 1 of them a synthesis that pooled it.
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Who cites it
12 citing papers in PubMed, 1 synthesis or guideline pooled it, 18 citations in OpenAlex.
- Variants in complement genes are uncommon in patients with anti-factor H autoantibody-associated atypical hemolytic uremic syndrome.Pediatric nephrology (Berlin, Germany) · 2023Pooled it
- IPNA clinical practice recommendations for the management of aHUS and secondary TMA in children.Pediatric nephrology (Berlin, Germany) · 2026Article
- Article
- Managing anti-factor H antibody-associated hemolytic uremic syndrome: time for consensus.Pediatric nephrology (Berlin, Germany) · 2024Article
- Clinical features of children with anti-CFH autoantibody-associated hemolytic uremic syndrome: a report of 8 cases.Renal failure · 2022Article
- Anti-factor H antibody and its role in atypical hemolytic uremic syndrome.Frontiers in immunology · 2022Article
- Patient-specific iPSC-derived endothelial cells reveal aberrant p38 MAPK signaling in atypical hemolytic uremic syndrome.Stem cell reports · 2021Article
- Early relapse rate determines further relapse risk: results of a 5-year follow-up study on pediatric CFH-Ab HUS.Pediatric nephrology (Berlin, Germany) · 2021Observational
- Anti-Factor H Antibodies in Egyptian Children with Hemolytic Uremic Syndrome.International journal of nephrology · 2021Article
- Immunological features and functional analysis of anti-CFH autoantibodies in patients with atypical hemolytic uremic syndrome.Pediatric nephrology (Berlin, Germany) · 2019Article
- Clinical and Immunological Profile of Anti-factor H Antibody Associated Atypical Hemolytic Uremic Syndrome: A Nationwide Database.Frontiers in immunology · 2019Article
- Immunological Characteristics of Chinese Adult Patients with Anti-Complement Factor H Autoantibodies-Associated Complement-Mediated Thrombotic Microangiopathy.Kidney diseases (Basel, Switzerland)Article
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Authors and funding
12 authors at 6 institutions in 1 country.
Funding
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Abstract
backgroundAnti-complement factor H (CFH) autoantibody-associated hemolytic uremic syndrome (HUS) is a severe sub-type of HUS.
methodsWe assessed the clinical and renal pathological features, circulating complement levels, and genetic background of Chinese pediatric patients with this sub-type of HUS. Thirty-three consecutive patients with acute kidney injury who tested positive for serum anti-CFH autoantibodies were enrolled in this study.
resultsAll of the eight patients who underwent renal biopsies presented with changes typical of thrombotic microangiopathy, especially changes in chronic characteristics. Compared to patients in remission and normal control subjects, patients with acute disease had significantly lower plasma CFH levels and significantly higher plasma complement 3a (C3a), C5a, and terminal complement complex (SC5b-9) levels. The CFH-anti-CFH immunoglobin G (IgG) circulating immunocomplex (CFH-CIC) titers were more closely correlated with CFH plasma levels than anti-CFH IgG levels. Of the 22 patients, four (18%) were homozygous for CFHR3-1Δ and ten were heterozygous for CFHR1 or CFHR3 deletions. Most patients responded well to a combination of plasma and immunosuppressive therapies, with a remission rate of 87%. At the end of the follow-up, nine patients reached the combined end-points, including two with end-stage renal disease and seven with relapses.
conclusionPlasma C3a, C5a, and SC5b-9 levels predicted disease activity in anti-CFH autoantibody-associated HUS patients enrolled in this study. These patients responded well to plasma therapy combined with immunosuppression.
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