scholarly journals Atypical hemolytic uremic syndrome: A report of two cases

2016 ◽  
Vol 9 (1) ◽  
pp. 75
Author(s):  
Md. Habibur Rahman ◽  
Morsheda Akhter ◽  
Syed Symul Haque
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Kidney Injury ◽  
Course Of Illness ◽  
Atypical Hus ◽  
Excellent Outcome ◽  
Uremic Syndrome ◽  
Stage Renal Disease ◽  
End Stage ◽  
Insight Into

Hemolytic uremic syndrome (HUS) is one of the important cause of acute kidney injury in children. There is excellent outcome in patients with typical HUS but atypical HUS is associated with high mortality, risk of recurrence and may lead to end stage renal disease. We report two cases of 5 and 6 year old child having clinical & laboratory characteristics of atypical HUS. These children had a fulminant course of illness with complications involving various systems. The report provides an insight into the etio pathogenesis, diagnoses and treatment of this condition.

Atypical hemolytic uremic syndrome & treatment: case study

2021 ◽  
pp. 1-7
Author(s):  
Azhar Ali Khan ◽  
Muhammad Usman Ahraf ◽  
Usman Javaid
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Kidney Injury ◽  
Daily Basis ◽  
Fresh Frozen Plasma ◽  
Atypical Hus ◽  
Fresh Frozen ◽  
Uremic Syndrome ◽  
Stage Renal Disease ◽  
End Stage

Abstract Atypical Hemolytic Uremic Syndrome (aHUS) is considered as an uncommon pathology that usually affects young adults and causes acute kidney injury which can further lead to End Stage Renal Disease (ESRD). Atypical HUS usually occurs due to impairment in alternate pathway of complement system. aHUS are usually sporadic and less than 20% cases are familial. About 50% aHUS cases show no clear initiating factors. Mortality rate has been reduced in atypical HUS over the years due to progress in intensive care and dialysis facility. First treatment option should be PE, with exchange of 1.5 plasma volumes (60-75 ml/kg) per session, under the cover of fresh frozen plasma (FFP). PE needs to be performed on daily basis while waiting for the results of platelet count, LDH and Hb levels to be normal and renal functions showing improvement. Here we present a case in 15-year-old boy who was healthy previously. Continuous...

scholarly journals Acute Progression of Adult-Onset Atypical Hemolytic-Uremic Syndrome due to CFH Mutation: A Case Report

2013 ◽  
Vol 2013 ◽  
pp. 1-4
Author(s):  
Bartlomiej Posnik ◽  
Dorota Sikorska ◽  
Krzysztof Hoppe ◽  
Krzysztof Schwermer ◽  
Krzysztof Pawlaczyk ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Late Onset ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Genetic Mutation ◽  
Current Data ◽  
Factor H ◽  
Rapid Progression ◽  
Uremic Syndrome ◽  
Stage Renal Disease ◽  
End Stage

Atypical hemolytic-uremic syndrome (aHUS), unlike typical HUS, is not due to bacteria but rather to an idiopathic or genetic cause that promotes dysregulation of the alternative complement pathway. It leads to hemolytic anemia, thrombocytopenia, and renal impairment. Although aHUS secondary to a genetic mutation is relatively rare, when occurring due to a mutation in Factor H (CFH), it usually presents with younger onset and has a more severe course, which in the majority ends with end-stage renal failure. Paradoxically to most available data, our case features acute aHUS due to a CFH mutation with late onset (38-year-old) and rapid progression to end-stage renal disease. Due to current data indicating a high risk of graft failure in such patients, the diagnosis of aHUS secondary to a genetic cause has disqualified our patient from a living (family) donor renal transplantation and left her with no other option but to begin permanent renal replacement therapy.

scholarly journals Atypical Hemolytic Uremic Syndrome Successfully Treated with Eculizumab

2020 ◽  
Vol 95 (2) ◽  
pp. 124-128
Author(s):  
Jung Hyun Kim ◽  
Won Kyung Han ◽  
Yu Bum Choi ◽  
Hyung Jong Kim ◽  
Jisu Oh ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
End Stage Renal Disease ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Delayed Diagnosis ◽  
Membrane Attack Complex ◽  
Acute Renal Injury ◽  
Humanized Monoclonal Antibody ◽  
Uremic Syndrome ◽  
Stage Renal Disease ◽  
End Stage

Atypical hemolytic uremic syndrome (aHUS) is a rare syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal injury, which results from uncontrolled complement activation. Delayed diagnosis and treatment of aHUS may result in end-stage renal disease (ESRD) and an associated dependence on dialysis. In extreme cases, it may cause death due to multi-organ failure. Eculizumab, a humanized monoclonal antibody against C5, inhibits the formation of the terminal membrane attack complex and is used to treat aHUS. Here, we report a 46-year-old male patient who suffered from aHUS relapse, despite prior treatment with repeated plasma exchange and hemodialysis. Eculizumab therapy improved his hematologic findings without use of hemodialysis.

scholarly journals Pregnancy-triggered atypical hemolytic uremic syndrome (aHUS): a Global aHUS Registry analysis

2021 ◽  
Author(s):  
Fadi Fakhouri ◽  
Marie Scully ◽  
Gianluigi Ardissino ◽  
Imad Al-Dakkak ◽  
Benjamin Miller ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Clinical Characteristics ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Factor H ◽  
Complement Factor ◽  
Childbearing Age ◽  
History Of ◽  
Uremic Syndrome ◽  
Stage Renal Disease ◽  
End Stage

Abstract Background Atypical hemolytic uremic syndrome (aHUS) is a rare disease in which uncontrolled terminal complement activation leads to systemic thrombotic microangiopathy (TMA). Pregnancy can trigger aHUS and, without complement inhibition, many women with pregnancy-triggered aHUS (p-aHUS) progress to end-stage renal disease (ESRD) with a high risk of morbidity. Owing to relatively small patient numbers, published characterizations of p-aHUS have been limited, thus the Global aHUS Registry (NCT01522183, April 2012) provides a unique opportunity to analyze data from a large single cohort of women with p-aHUS. Methods The demographics and clinical characteristics of women with p-aHUS (n = 51) were compared with those of women of childbearing age with aHUS and no identified trigger (non-p-aHUS, n = 397). Outcome evaluations, including renal survival according to time to ESRD, were compared for patients with and without eculizumab treatment (a complement C5 inhibitor) in both aHUS groups. Results Baseline demographics and clinical characteristics were broadly similar in both groups. The proportion of women with p-aHUS and non-p-aHUS with pathogenic variant(s) in complement genes and/or anti-complement factor H antibodies was similar (45% and 43%, respectively), as was the proportion with a family history of aHUS (12% and 13%, respectively). Eculizumab treatment led to significantly improved renal outcomes in women with aHUS, regardless of whether aHUS was triggered by pregnancy or not: adjusted hazard ratio for time to ESRD was 0.06 (p = 0.006) in the p-aHUS group and 0.20 (p < 0.0001) in the non-p-aHUS group. Conclusion Findings from this study support the characterization of p-aHUS as a complement-mediated TMA. Graphic abstract

scholarly journals Preemptive Use of Eculizumab for Living-Donor Kidney Transplantation in a Child with Atypical Hemolytic Uremic Syndrome

2016 ◽  
Vol 30 (1&2) ◽  
pp. 22 ◽  
Author(s):  
Oleh Akchurin ◽  
Samriti Dogra ◽  
Frederick Kaskel ◽  
Dominique Jan ◽  
Stuart Greenstein ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Hemolytic Uremic Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Factor H ◽  
Complement Factor ◽  
Pediatric Kidney Transplantation ◽  
Uremic Syndrome ◽  
Few Data ◽  
Stage Renal Disease ◽  
End Stage

Eculizumab is an anti-complement C5 monoclonal antibody that has recently been reported as an effective therapy for atypical hemolytic uremic syndrome. However, few data are available on the preemptive use of this medication in pediatric kidney transplantation. This report describes a successful preemptive use of eculizumab in combination with living unrelated kidney transplanta- tion in a 10-year-old child with end-stage renal disease secondary to atypical hemolytic uremic syndrome who has a complement factor H mutation that has not been previously reported. Further observations and clinical trials are required to address the challenges and areas of uncertainty related to preemptive eculizumab therapy for kidney transplantation in children and adults with atypical hemolytic uremic syndrome. 

ATYPICAL HEMOLYTIC UREMIC SYNDROME IN CHILDREN: PRACTICAL ASPECTS OF DIFFERENTIAL DIAGNOSTICS AND TREATMENT

2021 ◽  
Vol 100 (4) ◽  
pp. 64-73
Author(s):  
S.V. Baiko ◽  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Alternative Pathway ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Differential Diagnostics ◽  
E Coli ◽  
Uremic Syndrome ◽  
Stage Renal Disease ◽  
End Stage ◽  
The Complement System ◽  
Terminal Complement

Atypical hemolytic uremic syndrome (aHUS) is a rare progressive form of systemic thrombotic microangiopathy (TMA) that develops as a result of uncontrolled activation of the alternative pathway of the complement system. In the case of late diagnosis and inadequate treatment, aHUS has an unfavorable outcome with death rates as high as 25% during the acute phase and up to 50% of cases progressing to end-stage renal disease. Clinically, aHUS is very similar to disseminated intravascular coagulation, other TMAs: HUS associated with enterohemorrhagic E. coli, thrombotic thrombocytopenic purpura, etc. The article presents the sequence and scope of studies for the differential diagnosis of aHUS. Eculizumab and its biosimilars, blocking the terminal complement complex, have changed the future of patients with aHUS, so timely diagnosis and early treatment are crucial in the outcome of the disease.

scholarly journals Hemolytic Uremic Syndrome Caused by Mycoplasma Pneumoniae Infection in Children: A Case Report and Literature Review

2021 ◽  
Author(s):  
Sirinthip Kittivisuit ◽  
Prayong Vachvanichsanong ◽  
Thirachit Chotsampancharoen
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Hemolytic Anemia ◽  
Mycoplasma Pneumoniae ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Renal Dialysis ◽  
Microangiopathic Hemolytic Anemia ◽  
Uremic Syndrome ◽  
Red Cell Transfusion ◽  
Stage Renal Disease ◽  
End Stage

We describe the case of a 6-year-old boy with a Mycoplasma pneumoniae (M. pneumoniae) respiratory tract infection associated with thrombotic microangiopathic hemolytic anemia and thrombocytopenia with renal failure which was diagnosed as atypical hemolytic uremic syndrome. Renal biopsy showed features of thrombotic microangiopathy. The patient was treated with azithromycin for the M. pneumoniae infection, and supportive care with red cell transfusion and renal dialysis in the acute period. The microangiopathic hemolytic anemia and thrombocytopenia resolved within 2 months after diagnosis but the renal function damage was irreversible. The patient developed end-stage renal disease and required long term renal replacement therapy.

scholarly journals Atypical hemolytic uremic syndrome secondary to lupus nephritis, responsive to eculizumab

2016 ◽  
Vol 8 (3) ◽  
Author(s):  
Alexander G. Raufi ◽  
Shruti Scott ◽  
Omar Darwish ◽  
Kevin Harley ◽  
Kanwarpal Kahlon ◽  
...  
Keyword(s):  
Lupus Nephritis ◽  
Hemolytic Uremic Syndrome ◽  
Lupus Erythematosus ◽  
Multidisciplinary Approach ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Organ Damage ◽  
Atypical Hus ◽  
First Line Therapy ◽  
Autoimmune Attack ◽  
Uremic Syndrome

Among the spectrum of disease manifestations associated with systemic lupus erythematosus, lupus nephritis is particularly concerning due to the potential for renal failure. This autoimmune attack may not, however, be limited to the kidney and is increasingly being recognized as a trigger for atypical Hemolytic Uremic Syndrome (aHUS). Atypical HUS falls under the spectrum of the thrombotic microangiopathies (TMAs) – a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ damage. Although plasma exchange is considered first-line therapy for thrombotic thrombocytopenic purpura – a TMA classically associated with autoimmune depletion of ADAMTS-13 – aHUS demonstrates less reliable responsiveness to this modality. Instead, use of the late complement inhibitor Eculizumab has emerged as an effective modality for the management of such patients. Diagnosis of aHUS, however, is largely clinically based, relying heavily upon a multidisciplinary approach. Herein we present the case of a patient with atypical HUS successfully treated with Eculizumab in the setting of Class IV-G (A) lupus nephritis and hypocomplementemia.

Eculizumab Modifies Outcomes in Adults with Atypical Hemolytic Uremic Syndrome with Acute Kidney Injury

2018 ◽  
Vol 48 (3) ◽  
pp. 225-233 ◽  
Author(s):  
Mercedes Cao ◽  
Bruna N. Leite ◽  
Tamara Ferreiro ◽  
María Calvo ◽  
Constantino Fernández ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Hemolytic Uremic Syndrome ◽  
Kidney Function ◽  
Case Reports ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Kidney Injury ◽  
Complete Recovery ◽  
Genetic Abnormalities ◽  
Uremic Syndrome ◽  
Meta Analyses

Background: Atypical hemolytic uremic syndrome (aHUS) is a rare disease associated with congenital or acquired genetic abnormalities that result in uncontrolled complement activation, leading to thrombotic microangiopathy and kidney failure. Until recently, the only treatment was plasma exchange or plasma infusion (PE/PI), but 60% of patients died or had permanent kidney damage despite treatment. Eculizumab, a complement inhibitor, has shown promising results in aHUS. However, data are mainly extracted from case reports or studies of heterogeneous cohorts, and no direct comparison with PE/PI is available. Methods: An observational retrospective study of adult, dialysis-dependent aHUS patients with acute kidney injury (AKI) who were treated with either PE/PI alone or with second-line eculizumab in our center. We compared the effect of PE/PI and eculizumab on kidney function, hypertension, proteinuria, hematologic values, relapse, and death. Results: Thirty-one patients were included (females, 18; sporadic aHUS, 29; mean age, 46 ± 20 years). Twenty-six patients were treated with PE/PI alone, and 5 were deemed to be plasma-resistant and received eculizumab after stopping PE/PI. Among patients receiving eculizumab, 80% attained complete recovery of kidney function, 100% stopped dialysis, 20% had decreased proteinuria, and no patient relapsed (vs. 38.5, 50, 15.4, and 11.5%, respectively, of patients receiving only PE/PI). At 1-year of follow-up, no deaths had occurred in either group. Conclusion: Eculizumab shows greater efficacy than PE/PI alone for the treatment of adult aHUS patients with AKI. Prospective studies and meta-analyses are warranted to confirm our findings and set guidelines for treatment, monitoring, and maintenance.

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