An adult case of atypical hemolytic uremic syndrome presented with posterior reversible encephalopathy syndrome: Successful response to late-onset eculizumab treatment

Abstract

Atypical hemolytic uremic syndrome is a rare and progressive disease caused by uncontrolled alternative complement activation. Dysregulatıon of the complement activation results in thrombotic microangiopathy and multiorgan damage. A 29-yearold woman who was admitted with complaints of vomiting and headache was detected to have acute renal failure with microangiopathic hemolytic anemia (MAHA). After the diagnosis of atypical hemolytic uremic syndrome (aHUS), she was treated with plasma exchange (PE) and hemodialysis (HD). She has experienced hypertensionrelated posterior reversible encephalopathy syndrome (PRES) at the second plasma exchange. She was initiated on eculizumab therapy because of no response to PE on the 34th days. Her renal functions progressively improved with eculizumab treatment. Dependence on dialysis was over by the 4th month. Dialysis free-serum Creatinine level was 2.2 mg/dL [glomerular filtration rate (e-GFR): 30 mL/min/1.73 m2] after 24 months.Neurological involvement (PRES, etc.) is the most common extrarenal complication and a major cause of mortality and morbidity from aHUS. More importantly, we showed that renal recovery may be obtained following late-onset eculizumab treatment in patient with aHUS after a long dependence on hemodialysis.

Massive Cerebral Edema in Hypertensive Emergency with Complete Neurological Recovery: Atypical PRES? A Case Report

“…Hypertensive emergency is a common cause of TMA and genetic complement mutations are often associated in these cases [7]. There have also been reports of PRES manifestations in aHUS [8]. Considering all results in this case of severe hypertensive emergency with TMA and possible PRES, these findings support a possible pathogenic mutation of complement factor C3 as underlying cause.…”

Early postoperative seizures in liver and kidney recipients

“…Кроме этого, у больных после трансплантации почки есть специфические причины ОССП, а именно гипертензивная энцефалопатия, дизэквилибриумсиндром и уремия [43]. Злокачественная гипертензия у пациентов с терминальной почечной недостаточностью, с гемолитико-уремическим синдромом до и после трансплантации почки ассоциированы с развитием СЗОЛ [47,48]. Клиническая картина подобна такролимусиндуцированному СЗОЛ, дополнительно фиксируется высокий уровень артериального давления.…”

Importance of eculizumab treatment in recurrence of atypical hemolytic uremic syndrome during the SARS-CoV-2 pandemic

“…A 29-year-old female patient was being followed up in remission due to aHUS accompanying posterior reversible encephalopathy syndrome (PRES) [ 2 ]. Genetic analysis of complement protein genes was mutated at on the complement factor H (CFH –p.Glu936Asp, p.Arg1192Ile) antigen and CFHR5 genes (p.Arg356His) with associated aHUS.…”