Eculizumab for Atypical Hemolytic–Uremic Syndrome

Nürnberger, Jens; Philipp, Thomas; Witzke, Oliver; Saez, Anabelle Opazo; Vester, Udo; Baba, Hideo A.; Kribben, Andreas; Zimmerhackl, Lothar Bernd; Janecke, Andreas R.; Nagel, Mato; Kirschfink, Michael

doi:10.1056/nejmc0808527

Cited by 342 publications

(230 citation statements)

References 4 publications

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“…We here describe the effect of eculizumab in the treatment of an adolescent with aHUS. In contrast to the patient reported by Nü rnberger et al (22), the effect of eculizumab was only transient and aHUS relapses occurred early after recovery of complement hemolytic activity.…”

Section: H Emolytic Uremic Syndrome (Hus) Is a Clinical Triad Ofcontrasting

confidence: 52%

“…Beneficial effects of eculizumab treatment have already been demonstrated in patients with paroxysmal nocturnal hemoglobinuria (20). Recently, eculizumab was reported to be effective also in aHUS (21,22). Remissions of aHUS were achieved in a case of congenital aHUS and in an adult with posttransplantation recurrence (21,22).…”

Section: H Emolytic Uremic Syndrome (Hus) Is a Clinical Triad Ofmentioning

confidence: 85%

“…Recently, eculizumab was reported to be effective also in aHUS (21,22). Remissions of aHUS were achieved in a case of congenital aHUS and in an adult with posttransplantation recurrence (21,22). In the adult patient, a single dose of eculizumab was effective to maintain a remission for 8 mo (22).…”

Section: H Emolytic Uremic Syndrome (Hus) Is a Clinical Triad Ofmentioning

confidence: 92%

“…In the adult patient with posttransplantation aHUS recurrence, a single dose of eculizumab induced long-term remission; however, an additional effect of tacrolimus withdrawal cannot be excluded (22). Both patients showed a complete renal recovery (21,22). In the patient reported here, eculizumab was administered as a rescue therapy after 12 wk.…”

Section: Discussionmentioning

confidence: 99%

“…Remissions of aHUS were achieved in a case of congenital aHUS and in an adult with posttransplantation recurrence (21,22). In the adult patient, a single dose of eculizumab was effective to maintain a remission for 8 mo (22). We here describe the effect of eculizumab in the treatment of an adolescent with aHUS.…”

Section: H Emolytic Uremic Syndrome (Hus) Is a Clinical Triad Ofmentioning

confidence: 96%

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Complement Inhibitor Eculizumab in Atypical Hemolytic Uremic Syndrome

Mache

Acham-Roschitz

Frémeaux‐Bacchi

et al. 2009

Clinical Journal of the American Society of Nephrology

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144

103

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Background and objectives: Atypical hemolytic uremic syndrome (aHUS) is associated with a congenital or acquired dysregulation of the complement alternative pathway that leads to continuous complement activation on host cells causing inflammation and damage. Eculizumab, a humanized mAb against complement protein C5, inhibits activation of the terminal complement pathway.Design, setting, participants, & measurements: We report an adolescent with relapsing unclassified aHUS. On admission, a high plasma creatinine level indicated a poor prognosis, and hemodialysis had to be started. Plasma exchanges were initially effective against the microangiopathic hemolytic activity and allowed a temporary improvement of renal function with termination of hemodialysis after 7 wk. Subsequently, plasma exchanges (three times per week) failed to prevent ongoing aHUS activity and progressive renal failure. After 12 wk, aHUS treatment was switched to eculizumab.Results: Eculizumab was effective in terminating the microangiopathic hemolytic process in two aHUS relapses; however, after normalization of complement activity, aHUS recurred and ultimately led to anuric end-stage renal failure.Conclusions: In this patient, complement inhibition by eculizumab temporarily terminated the microangiopathic hemolytic activity. Nevertheless, renal damage as a result of preceding and subsequent aHUS activity resulted in end-stage renal failure; therefore, therapeutic success may depend on early administration of eculizumab. The optimal duration of treatment may be variable and remains to be determined.

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Section: H Emolytic Uremic Syndrome (Hus) Is a Clinical Triad Ofcontrasting

confidence: 52%

Section: H Emolytic Uremic Syndrome (Hus) Is a Clinical Triad Ofmentioning

confidence: 85%

Section: H Emolytic Uremic Syndrome (Hus) Is a Clinical Triad Ofmentioning

confidence: 92%

Section: Discussionmentioning

confidence: 99%

Section: H Emolytic Uremic Syndrome (Hus) Is a Clinical Triad Ofmentioning

confidence: 96%

See 3 more Smart Citations

Complement Inhibitor Eculizumab in Atypical Hemolytic Uremic Syndrome

Mache

Acham-Roschitz

Frémeaux‐Bacchi

et al. 2009

Clinical Journal of the American Society of Nephrology

Self Cite

144

103

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Clinical Evaluation of 3 Families With Basal Laminar Drusen Caused by Novel Mutations in the Complement Factor H Gene

Ven¹,

Boon²,

Fauser³

et al. 2012

Arch Ophthalmol

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Elucidation of the clinical BLD phenotype will facilitate identification of individuals predisposed to developing disease-related comorbidity, such as membranoproliferative glomerulonephritis type II. Moreover, with upcoming treatment modalities targeting specific components of the complement system, early identification of patients with BLD and detection of the genetic defect become increasingly important.

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Thrombotic Microangiopathy: Biology, Diagnosis, and Management

Dalia

Saba

2014

Hemostasis and Thrombosis

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Eculizumab for Atypical Hemolytic–Uremic Syndrome

Cited by 342 publications

References 4 publications

Complement Inhibitor Eculizumab in Atypical Hemolytic Uremic Syndrome

Complement Inhibitor Eculizumab in Atypical Hemolytic Uremic Syndrome

Clinical Evaluation of 3 Families With Basal Laminar Drusen Caused by Novel Mutations in the Complement Factor H Gene

Thrombotic Microangiopathy: Biology, Diagnosis, and Management

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