Treatment of Shiga-Toxin Hus with Severe Neurologic Features with Eculizumab

Umscheid, Jacob; Nevil, Collin; Vasudeva, Rhythm; Ali, Mohammed Farhan; Agasthya, Nisha

doi:10.1155/2021/8053246

Cited by 2 publications

(2 citation statements)

References 36 publications

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“…‐Small case series and isolated case reports suggest that eculizumab could promptly improve severe neurological symptoms in children with STEC‐HUS 112–116 …”

Section: Complement Blockadementioning

confidence: 99%

Complement‐driven hemolytic uremic syndrome

Léon

LeStang²,

Sberro-Soussan³

et al. 2023

American J Hematol

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Overactivation of the complement alternative pathway drives the pathogenesis of primary atypical hemolytic uremic syndrome (aHUS). Genetically-determined or acquired dysregulation of the complement is frequently identified in patients with aHUS, pregnancy-related hemolytic uremic syndrome (HUS), and severe hypertensionassociated HUS. In contrast, it is still unclear whether self-limited complement activation, which frequently occurs in other forms of HUS, provides key mechanistic clues or results from endothelial damage. Development of novel biomarkers is underway to firmly establish complement-driven pathogenesis. C5 blockade therapy has revolutionized the management of aHUS patients, resulting in a halving of the subpopulation under chronic dialysis over the course of a few years. On the other hand, the efficacy of C5 blockade in secondary forms of HUS, as assessed by small and uncontrolled case series, is less compelling and should be investigated through properly designed prospective clinical trials. The increased risk of meningococcal infection, related to C5 inhibition, must be rigorously addressed with suitable prophylaxis. Treatment duration should be determined based on an individualized benefit/risk assessment.

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“…‐Small case series and isolated case reports suggest that eculizumab could promptly improve severe neurological symptoms in children with STEC‐HUS 112–116 …”

Section: Complement Blockadementioning

confidence: 99%

Complement‐driven hemolytic uremic syndrome

Léon

LeStang²,

Sberro-Soussan³

et al. 2023

American J Hematol

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“…Although the role of the complement protein in STEC-HUS has not been fully understood, eculizumab has been used as an off-label treatment for STEC-HUS patients with severe complications of the nervous system, such as those with neurological or multiple-organ dysfunction. A positive clinical improvement after treatment has been reported, however, the overall quality of evidence is low [ 179 , 180 ]. For example, three children with STEC-HUS and progressive central nervous system involvement reported significant neurologic improvements within 24 h after the first eculizumab infusion.…”

Section: Stec-hus Therapeutic Strategiesmentioning

confidence: 99%

Diagnosis and Treatment for Shiga Toxin-Producing Escherichia coli Associated Hemolytic Uremic Syndrome

Liu

Thaker

Wang

et al. 2022

Toxins

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Shiga toxin-producing Escherichia coli (STEC)-associated hemolytic uremic syndrome (STEC-HUS) is a clinical syndrome involving hemolytic anemia (with fragmented red blood cells), low levels of platelets in the blood (thrombocytopenia), and acute kidney injury (AKI). It is the major infectious cause of AKI in children. In severe cases, neurological complications and even death may occur. Treating STEC-HUS is challenging, as patients often already have organ injuries when they seek medical treatment. Early diagnosis is of great significance for improving prognosis and reducing mortality and sequelae. In this review, we first briefly summarize the diagnostics for STEC-HUS, including history taking, clinical manifestations, fecal and serological detection methods for STEC, and complement activation monitoring. We also summarize preventive and therapeutic strategies for STEC-HUS, such as vaccines, volume expansion, renal replacement therapy (RRT), antibiotics, plasma exchange, antibodies and inhibitors that interfere with receptor binding, and the intracellular trafficking of the Shiga toxin.

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Treatment of Shiga-Toxin Hus with Severe Neurologic Features with Eculizumab

Cited by 2 publications

References 36 publications

Complement‐driven hemolytic uremic syndrome

Complement‐driven hemolytic uremic syndrome

Diagnosis and Treatment for Shiga Toxin-Producing Escherichia coli Associated Hemolytic Uremic Syndrome

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