Profound neurological injury in a patient with atypical hemolytic uremic syndrome

Salem, G; Flynn, Joseph M.; Cataland, Spero R.

doi:10.1007/s00277-012-1615-y

Cited by 21 publications

(9 citation statements)

References 4 publications

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“…Remissions were achieved in patients with and without identified complement regulatory deficiencies. Subsequent case reports about eculizumab in native kidneys confirmed these observations [63][64][65][66][67][68][69][70][71][72][73][74][75].…”

Section: Eculizumab --First Experiences Case Reports and Retrospectisupporting

confidence: 53%

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Pharmacological treatment of atypical hemolytic-uremic syndrome

Vester¹,

Mache

2013

Expert Opinion on Orphan Drugs

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Section: Eculizumab --First Experiences Case Reports and Retrospectisupporting

confidence: 53%

“…Apart from hematological and renal manifestations of aHUS, positive effects of eculizumab have also been described in other aHUS-related symptoms, including neurological, ocular, cardiac and vascular manifestations [38,63,66,69,71,72,86].…”

Section: Eculizumab --First Experiences Case Reports and Retrospectimentioning

confidence: 99%

Pharmacological treatment of atypical hemolytic-uremic syndrome

Vester¹,

Mache

2013

Expert Opinion on Orphan Drugs

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“…However, eculizumab was impressively effective in two children with lifethreatening aHUS and acute distal ischemia (digital gangrene) [55] and skin necrosis with intestinal perforation [121], and two adults with ulcerative skin lesions [122]. That eculizumab may be efficacious to rescue central nervous system involvement is suggested by nine case reports, including four in children [87,90,110,[123][124][125][126][127]. Eculizumab also appeared lifesaving in four children with myocardial involvement [83,90,92,110].…”

Section: Education Information Cardmentioning

confidence: 97%

An international consensus approach to the management of atypical hemolytic uremic syndrome in children

et al. 2015

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Atypical hemolytic uremic syndrome (aHUS) emerged during the last decade as a disease largely of complement dysregulation. This advance facilitated the development of novel, rational treatment options targeting terminal complement activation, e.g., using an anti-C5 antibody (eculizumab). We review treatment and patient management issues related to this therapeutic approach. We present consensus clinical practice recommendations generated by HUS International, an international expert group of clinicians and basic scientists with a focused interest in HUS. We aim to address the following questions of high relevance to daily clinical practice: Which complement investigations should be done and when? What is the importance of anti-factor H antibody detection? Who should be treated with eculizumab? Is plasma exchange therapy still needed? When should eculizumab therapy be initiated? How and when should complement blockade be monitored? Can the approved treatment schedule be modified? What approach should be taken to kidney and/or combined liver-kidney transplantation? How should we limit the risk of meningococcal infection under complement blockade therapy? A pressing question today regards the treatment duration. We discuss the need for prospective studies to establish evidence-based criteria for the continuation or cessation of anticomplement therapy in patients with and without identified complement mutations.

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“…Previously, neurological abnormalities have been used to differentiate TTP from other TMAs, because the involvement of the nervous system was reported in up to 79% of patients with TTP ( 82 83 84 85 86 ). However, various degrees of neurological injury have also been observed in ADAMTS13 nondeficient aHUS patients ( 3 87 88 89 ). Therefore, the clinical utility of neurological symptoms is somewhat limited.…”

Section: Diagnosismentioning

confidence: 99%

Clinical Practice Guidelines for the Management of Atypical Hemolytic Uremic Syndrome in Korea

Cheong

Yoon

et al. 2016

J Korean Med Sci

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Atypical hemolytic uremic syndrome (aHUS) is a rare syndrome characterized by micro-angiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. The major pathogenesis of aHUS involves dysregulation of the complement system. Eculizumab, which blocks complement C5 activation, has recently been proven as an effective agent. Delayed diagnosis and treatment of aHUS can cause death or end-stage renal disease. Therefore, a diagnosis that differentiates aHUS from other forms of thrombotic microangiopathy is very important for appropriate management. These guidelines aim to offer recommendations for the diagnosis and treatment of patients with aHUS in Korea. The guidelines have largely been adopted from the current guidelines due to the lack of evidence concerning the Korean population.

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Profound neurological injury in a patient with atypical hemolytic uremic syndrome

Cited by 21 publications

References 4 publications

Pharmacological treatment of atypical hemolytic-uremic syndrome

Pharmacological treatment of atypical hemolytic-uremic syndrome

An international consensus approach to the management of atypical hemolytic uremic syndrome in children

Clinical Practice Guidelines for the Management of Atypical Hemolytic Uremic Syndrome in Korea

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