Renal outcome with eculizumab in two diarrhea-associated hemolytic–uremic syndrome cases with severe neurologic involvement

Ekinci, Zelal; Bek, Kenan; Aytaç, Mehmet Baha; Karadenizli, Aynur; Hancer, Veysel Sabri

doi:10.1016/j.hkjn.2014.07.001

Cited by 9 publications

(4 citation statements)

References 6 publications

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“…We identified eight case series and case reports that described 21 total patients with STEC‐HUS treated with eculizumab . It included the case series study published by Lapeyraque et al who first systematically described the use of eculizumab in three pediatric cases of severe STEC‐HUS with neurological involvement, demonstrating rapid neurologic improvement within 24 h of first dose, and the normalization of disease markers including platelet count and LDH .…”

Section: Resultsmentioning

confidence: 99%

Use of complement monoclonal antibody eculizumab in Shiga toxin producing Escherichia coli associated hemolytic uremic syndrome: A review of current evidence

Mahat

Matar

Rotz

2019

Pediatric Blood & Cancer

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Complement activation plays an important role in the pathogenesis of atypical hemolytic uremic syndrome. Eculizumab is a monoclonal antibody that blocks complement activity and has been approved for use in the treatment of atypical hemolytic uremic syndrome (HUS). Less well appreciated is the role of complement in Shiga toxin-induced HUS (Shiga toxin producing Escherichia coli[STEC]-HUS). To a limited extent, eculizumab has been used off label in patients with severe STEC-HUS with neurological involvement. Through a systematic search of available databases, we identified 16 reports describing the use of eculizumab in STEC-HUS (eight case reports/series, seven retrospective studies, and one prospective cohort study). All studies described its use in severe STEC-HUS with neurological or multiorgan dysfunction; none were randomized or blinded. Four studies used the control groups. Although the overall quality of evidence is low, some published studies showed positive clinical improvement after treatment with eculizumab in severe STEC-HUS with progressive neurological involvement. K E Y W O R D Scomplement, E. coli, eculizumab, Shiga toxin, STEC-HUS

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Section: Resultsmentioning

confidence: 99%

Use of complement monoclonal antibody eculizumab in Shiga toxin producing Escherichia coli associated hemolytic uremic syndrome: A review of current evidence

Mahat

Matar

Rotz

2019

Pediatric Blood & Cancer

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“…All patients had renal failure and neurological involvement which had not responded to plasma exchange. Due to the cost of the drug, its use is limited to only the most severe cases and therefore treatment is often delayed, [19]. Within a paediatric population, early treatment of HUS with eculizimab in patients with neurological complications led to better outcomes, compared to delayed therapy initiation in patients with rapidly progressive HUS, [20].…”

Section: Discussionmentioning

confidence: 99%

Haemolytic uraemic syndrome - a rare case report of bloody diarrhoea in adults

et al. 2019

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Background Haemolytic uraemic syndrome is a rarely seen in adults often leading to critical illness. This case highlights how difficult it can be to establish a diagnosis and treat when a patient presents with bloody diarrhoea. Case presentation A 17-year-old Iraqi man presented to the emergency department with abdominal pain and bloody diarrhoea. He was initially treated as acute appendicitis, undergoing an appendectomy but following a recurrence in his symptoms a colonoscopy was performed. A diagnosis of shiga toxin-producing Escherichia coli leading to HUS was suspected following histology obtained at colonoscopy and this was confirmed on antibody testing. Despite intravenous fluids and supportive therapy the patient’s symptoms and condition deteriorated. He developed seizures and acute renal failure requiring intubation and plasma exchange in the intensive care setting. He eventually required treatment with ecluzimab therapy; a monoclonal antibody and subsequently made a full recovery. Conclusions Haemolytic uraemic syndrome is a triad of progressive renal failure, thrombocytopenia and haemolytic anaemia which is a condition rarely seen in adults. It is usually associated with an E. coli infection and supportive therapy remains the mainstay of treatment.

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“…The potential benefit of eculizumab in STEC-HUS has not been established to date and remains a matter of debate and ongoing research. Based on the dramatic effectiveness of eculizumab in atypical HUS, and on evidence showing complement activation during STEC-HUS, complement blockade has been attempted and reported in some cases and small series [8][9][10][11]. In a first report including 3 young patients with severe STEC-HUS (i.e., neurological involvement), administration of eculizumab was rapidly followed by a dramatic resolution of symptoms, suggesting potential benefit of therapeutic complement blockade [8].…”

Section: Case Reports In Nephrology and Dialysismentioning

confidence: 99%

“…Despite the higher risk of death associated with severe CNS involvement in STEC-HUS, the appropriate treatment remains ill-defined. Experimental data showed that Shiga toxin directly activates complement system and case series suggested that complement inhibition with eculizumab may be beneficial in patients with STEC-HUS complicated by CNS involvement [7][8][9][10][11]. However, such benefit was not confirmed among patients treated during the 2011 outbreak of E. coli O104:H4 and whether eculizumab should be used in patients with STEC-HUS and severe CNS symptoms still remains debated [12].…”

Section: Introductionmentioning

confidence: 99%

Severe Neurological Involvement in an Adult with Shiga Toxin-Producing Escherichia coli-Hemolytic Uremic Syndrome Treated with Eculizumab

Vanesse

Georgery

Duprez

et al. 2023

Case Rep Nephrol Dial

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A 68-year-old man with a medical history of hypertension was admitted to the emergency department for diffuse abdominal pain preceded by bloody diarrhea. Upon admission, neurological examination was normal, but he suddenly developed a left-sided hemiparesis. After a normal brain computed tomography, intravenous thrombolysis was administered for a suspicion of ischemic stroke. In the first laboratory investigations, hemoglobin was 16.9 g/dL, platelets 121 × 109/L (150–450), and serum creatinine 1.17 mg/dL. By the second hospital day, the platelet level dropped to 79 × 109/L, with haptoglobin at 0.12 g/L, 3% schistocytes, and normal ADAMTS13 activity (57%). Serum creatinine increased to 1.84 mg/dL with oliguria. The suspicion of thrombotic microangiopathy was supported by the identification of Shiga toxin genes stx1 and stx2 on a rectal swab and the isolation of an eaeA-negative Shiga toxin-producing E. coli O113:H4. The patient presented a generalized tonic-clonic seizure, and orotracheal intubation was required for decreased consciousness. Plasma exchange therapy was started, and eculizumab was given 6 days after symptoms onset. Brain magnetic resonance imaging (MRI) on day 13 showed symmetric hyperintensities within basal ganglia that disappeared on a second MRI on day 37. At 2-month follow-up, the patient had made a complete neurological and renal recovery and eculizumab therapy was stopped.

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Renal outcome with eculizumab in two diarrhea-associated hemolytic–uremic syndrome cases with severe neurologic involvement

Cited by 9 publications

References 6 publications

Use of complement monoclonal antibody eculizumab in Shiga toxin producing Escherichia coli associated hemolytic uremic syndrome: A review of current evidence

Use of complement monoclonal antibody eculizumab in Shiga toxin producing Escherichia coli associated hemolytic uremic syndrome: A review of current evidence

Haemolytic uraemic syndrome - a rare case report of bloody diarrhoea in adults

Severe Neurological Involvement in an Adult with Shiga Toxin-Producing Escherichia coli-Hemolytic Uremic Syndrome Treated with Eculizumab

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