Atypical Hemolytic Uremic Syndrome: A Case Series of 5 Patients

2016 ◽  
Vol 67 (5) ◽  
pp. A113
Author(s):  
Lucio Manenti ◽  
Giovanni Maria Rossi ◽  
Isabella Pisani ◽  
Micaela Gentile ◽  
Francesco Fontana ◽  
...  

2021 ◽  
Vol 0 (0) ◽  
pp. 0
Author(s):  
Sibabratta Patnaik ◽  
MukeshKumar Jain ◽  
NikunjKishore Rout ◽  
AmitRanjan Rup ◽  
ChinmayKumar Behera ◽  
...  

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Christine J. Kurian ◽  
Zachary French ◽  
Patrick Kukulich ◽  
Matthew Lankiewicz ◽  
Sushil Ghimire ◽  
...  

Abstract Background Atypical hemolytic uremic syndrome is an exceedingly rare thrombotic microangiopathy caused by accelerated activation of the alternative complement pathway. Case presentation Here, we report two cases of patients presenting with suspected atypical hemolytic uremic syndrome precipitated by coronavirus disease 2019 infection. The first patient, a 25-year-old Hispanic male, had one prior episode of thrombotic microangiopathy presumed to be atypical hemolytic uremic syndrome precipitated by influenza A, and re-presented with thrombocytopenia, microangiopathic hemolytic anemia, nonoliguric renal failure, and normal ADAMTS13 activity, with confirmed coronavirus disease 2019 positivity. The second patient, a 31-year-old Caucasian female, had no personal history of thrombotic microangiopathy, though reported a family history of suspected atypical hemolytic uremic syndrome. She presented with similar laboratory derangements, oliguric renal failure requiring hemodialysis, and confirmed coronavirus disease 2019 positivity. Both patients were treated with eculizumab with complete resolution of their hematologic and renal complications. Conclusion To our knowledge, this represents the largest case series of atypical hemolytic uremic syndrome precipitated by coronavirus disease 2019 in adults.


Author(s):  
Monika Vitkauskaitė ◽  
Artūras Vinikovas ◽  
Marius Miglinas ◽  
Laurynas Rimševičius ◽  
Agnė Čerkauskaitė ◽  
...  

Our case series showed that eculizumab is efficacious and safe in treating atypical hemolytic-uremic syndrome, as well as it has positive effects on quality of life. Further extensive studies are required to develop unified treatment guidelines.


2016 ◽  
Vol 18 (6) ◽  
pp. 413-420 ◽  
Author(s):  
Selçuk Yüksel ◽  
Havva Evrengül ◽  
Z. Birsin Özçakar ◽  
Tülay Becerir ◽  
Nagihan Yalçın ◽  
...  

2020 ◽  
Author(s):  
Lilian Monteiro Pereira Palma ◽  
Renato George Eick ◽  
Gustavo Coelho Dantas ◽  
Michele Káren dos Santos Tino ◽  
Maria Izabel de Holanda

Abstract Background Atypical hemolytic uremic syndrome (aHUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia and kidney injury caused by a dysregulation of the alternative complement pathway. Methods We conducted a multicenter nonregistry study aimed at collecting clinical, laboratory and genetic information of patients with aHUS in Brazil. Demographic data, genetic findings, treatments and outcomes are presented. Results Thirty-four patients were included, 62% were female and 67% were Caucasian. Half of the patients had the first manifestation of aHUS before the age of 18 years (pediatric group). Among the 17 patients who had the first manifestation after the age of 18 years (adult group), 6 were kidney transplant patients. Overall, 22 patients (65%) received plasma exchange/plasma infusion (PE/PI) and 31 patients (91%) received eculizumab. Eculizumab was started later in the adult group compared with the pediatric group. Two patients stopped dialysis after PE/PI and 19 patients stopped dialysis after eculizumab despite a late start. A pathogenic/likely pathogenic variant was found in 24.3% of patients. A coexisting condition or trigger was present in 59% of patients (infections, pregnancy, hypertension, autoimmune disease and transplant), especially in the adult group. There was a 30% relapse rate after stopping eculizumab, irrespective of genetic status. Conclusion This is the largest case series of aHUS in Brazil involving a wide range of patients for which eculizumab was the main treatment. Although eculizumab was started later than advised in the guidelines, most patients were able to stop dialysis at variable intervals. Discontinuation of eculizumab was associated with a 30% relapse of aHUS.


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