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Complement Factor H and Complement Factor H-Related Protein 5 Mutations Associated with Atypical Hemolytic Uremic Syndrome in a Systemic Lupus Erythematosus Patient: Efficacy of Eculizumab

Sibel ERSAN et al · Turkish Society of Nephrology · 2019

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Author / responsible party
Sibel ERSAN et al
Publisher
Turkish Society of Nephrology
Year
2019
ISSN
2667-4440
ISSN
2667-4440
Language
English
Serial publication

"ONE HUNDRED AND THIRTY-FIVE HOURS UNDER THE RUIN : A CASE WITH TRAUMATIC RHABDOMYOLYSIS IN GÖLCÜK EARTHQUAKE"

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Atypical hemolytic uremic syndrome (aHUS) is a disorder characterized by a propensity to thrombotic microangiopathy (TMA) due to defective regulation of the alternative complement pathway. Mutations in genes encoding complement factor H (CFH) and complement factor H-related proteins (CFHR) can be related with aHUS and identified in patients with rheumatologic diseases. Atypical HUS typically presents as acute kidney injury (AKI) associated with microangiopathic hemolytic anemia (MAHA) and thrombocytopenia. We describe a SLE patient with severe AKI due to aHUS related CFH and CFHR5 mutations. The patient was refractory to immunosuppressives and plasmapheresis but responded well to eculizumab.

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APA 7

al, S. E. E. (2019). Complement Factor H and Complement Factor H-Related Protein 5 Mutations Associated with Atypical Hemolytic Uremic Syndrome in a Systemic Lupus Erythematosus Patient: Efficacy of Eculizumab. https://turkjnephrol.org/index.php/pub/article/view/1244

MLA

al, Sibel ERSAN et. "Complement Factor H and Complement Factor H-Related Protein 5 Mutations Associated with Atypical Hemolytic Uremic Syndrome in a Systemic Lupus Erythematosus Patient: Efficacy of Eculizumab." 2019. https://turkjnephrol.org/index.php/pub/article/view/1244.

Chicago

al, Sibel ERSAN et. 2019. "Complement Factor H and Complement Factor H-Related Protein 5 Mutations Associated with Atypical Hemolytic Uremic Syndrome in a Systemic Lupus Erythematosus Patient: Efficacy of Eculizumab.". https://turkjnephrol.org/index.php/pub/article/view/1244.

Harvard

al, S. E. E. 2019, Complement Factor H and Complement Factor H-Related Protein 5 Mutations Associated with Atypical Hemolytic Uremic Syndrome in a Systemic Lupus Erythematosus Patient: Efficacy of Eculizumab, Turkish Society of Nephrology, available at: https://turkjnephrol.org/index.php/pub/article/view/1244 [Accessed 8 Aug. 2026].

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Title
Complement Factor H and Complement Factor H-Related Protein 5 Mutations Associated with Atypical Hemolytic Uremic Syndrome in a Systemic Lupus Erythematosus Patient: Efficacy of Eculizumab
Author / contributors
Sibel ERSAN et al
Publisher
Turkish Society of Nephrology
Publication year
2019
ISSN
2667-4440
ISSN
2667-4440
Language
English

Subjects

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SLE; aHUS; Eculizumab
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