Postpartum atypical hemolytic uremic syndrome: Evaluating thrombotic microangiopathy in the pregnant woman

Acute kidney injury in women during pregnancy and the puerperium is often ascribed to hypertensive complications of pregnancy, especially pre-eclampsia. However, rarer causes, including atypical hemolytic uremic syndrome (aHUS) can be triggered by pregnancy. We present a case of a woman with post-partum acute kidney injury due to aHUS, which was successfully treated with the C5a inhibitor eculizumab. We also present a summary of the evaluation and management of thrombotic microangiopathy in pregnancy.

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

American Journal of Nephrology ◽

10.1159/000492865 ◽

2018 ◽

Vol 48 (3) ◽

pp. 225-233 ◽

Cited By ~ 4

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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Atypical Hemolytic Uremic Syndrome Presenting as Acute Heart Failure—A Rare Presentation: Diagnosis Supported by Skin Biopsy

Journal of Investigative Medicine High Impact Case Reports ◽

10.1177/2324709619842905 ◽

2019 ◽

Vol 7 ◽

pp. 232470961984290

Author(s):

Asim Kichloo ◽

Savneek Singh Chugh ◽

Sanjeev Gupta ◽

Jay Pandav ◽

Praveen Chander

Keyword(s):

Heart Failure ◽

Skin Biopsy ◽

Hemolytic Uremic Syndrome ◽

Thrombotic Microangiopathy ◽

Atypical Hemolytic Uremic Syndrome ◽

Systolic Heart Failure ◽

Kidney Injury ◽

Rare Presentation ◽

History Of ◽

Uremic Syndrome

Atypical hemolytic uremic syndrome (aHUS) is a rare disorder of uncontrolled complement activation that manifests classically as anemia, thrombocytopenia, and renal failure, although extrarenal manifestations are observed in 20% of the patient most of which involving central nervous system, with relatively rare involvement of the heart. In this article, we report the case of a 24-year-old male with no history of heart disease presenting with acute systolic heart failure along with microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Given his presentation of thrombotic microangiopathy (TMA), along with laboratory results significant for low haptoglobin, platelets, hemoglobin, C3, C4, CH50, and normal ADAMTS13 levels, with no diarrhea and negative STEC polymerase chain reaction in stool, aHUS diagnosis was established with strong clinical suspicion, and immediate initiation of treatment was advised. Kidney biopsy to confirm diagnosis of aHUS was inadvisable because of thrombocytopenia, so the skin biopsy of a rash on his arm was done, which came to be consistent with thrombotic microangiopathy. Our case highlights a relatively rare association between aHUS and cardiac involvement, and the use of skin biopsy to support diagnosis of aHUS in patients who cannot undergo renal biopsy because of thrombocytopenia.

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Hemolytic uremic syndrome associated with rhabdomyolysis – Need to look beyond kidneys in hemolytic uremic syndrome

Tropical Doctor ◽

10.1177/00494755211029166 ◽

2021 ◽

pp. 004947552110291

Author(s):

Abhijit Choudhary ◽

Sneha T ◽

Satish Deopujari ◽

Prakash Khetan

Keyword(s):

Acute Kidney Injury ◽

Hemolytic Uremic Syndrome ◽

Thrombotic Microangiopathy ◽

Kidney Injury ◽

Organ Involvement ◽

High Morbidity ◽

Muscle Involvement ◽

Disease Prognosis ◽

Uremic Syndrome ◽

Gastrointestinal System Involvement

Hemolytic uremic syndrome, a part of thrombotic microangiopathy, is an important cause of acute kidney injury in children. Hemolytic uremic syndrome primarily targets kidney but extrarenal organ involvement is observed in 20–40% of patients. Extra-renal organ involvement in hemolytic uremic syndrome has been associated with greater disease severity and higher mortality. We describe a 31/2-year-old boy of hemolytic uremic syndrome with rhabdomyolysis, which is a rare extrarenal manifestation of hemolytic uremic syndrome. Unlike central nervous or gastrointestinal system involvement in hemolytic uremic syndrome which manifests clinically, muscle involvement may not and, if present, may worsen the existing acute kidney injury and may worsen disease prognosis. Considering the high morbidity and mortality in acute phase of hemolytic uremic syndrome, prompt evaluation to know the extent of extrarenal organ involvement at the earliest is important for management and prognosis of these patients.

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Atypical hemolytic-uremic syndrome as one of the causes of acute kidney injury in pregnant women

Terapevticheskii arkhiv ◽

10.26442/terarkh201890628-34 ◽

2018 ◽

Vol 90 (6) ◽

pp. 28-34 ◽

Cited By ~ 1

Author(s):

N L Kozlovskaya ◽

Yu V Korotchaeva ◽

E M Shifman ◽

L A Bobrova

Keyword(s):

Acute Kidney Injury ◽

Renal Function ◽

Hemolytic Uremic Syndrome ◽

Atypical Hemolytic Uremic Syndrome ◽

Clinical Manifestations ◽

Kidney Injury ◽

Complete Recovery ◽

Multiple Organ ◽

Aim Analysis ◽

Uremic Syndrome

Obstetric atypical hemolytic uremic syndrome (aHUS) is one of the reasons for the development of acute kidney injury (AKI) and can determine the prognosis of both mother and child. Aim. Analysis of clinical manifestations, course and outcomes of obstetric aHUS. Materials and methods. 45 patients with aHUS development during pregnancy or immediately after childbirth were observed between 2011 and 2017, age from 16 to 42 years. Results and discussion. All patients had AKI (serum creatinine 521,5±388,0 µmol/l, oliguria or anuria that required initiation of hemodialysis). 93.3% pts had extrarenal manifestations of TMA with the development of multiple organ failure (MOF). The mean number of damage organs was 3,7±1,2. In all patients, the development of aHUS was preceded by obstetric complications, surgery, infection, etc. In the outcome: 53.4% women showed complete recovery of renal function, 11.1% developed CKD 4-5 stages, 35.5% had dialysis-dependent end-stage renal failure (ESDR). Maternal mortality was 23.9%. Perinatal mortality was 32.6%. The early start of eculizumab treatment (within 1-2 weeks from the onset of aHUS), compared with therapy start after 3 weeks, increased the chances of favorable outcome for mother in 5.33 times, and the chances for normalization of renal function in 48.7 times. Conclusion. Obstetric aHUS is characterized by the development of AKI in 100% of cases. In most patients, the obstetric aHUS occurs with the development of MOF. Timely diagnosis of aHUS and immediate treatment by eculizumab allows not only to save the life of patients, but also completely restore their health.

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Case series of secondary thrombotic microangiopathy in children with multisystem inflammatory syndrome associated with COVID-19

Russian Medical Inquiry ◽

10.32364/2587-6821-2021-5-11-773-777 ◽

2021 ◽

Vol 5 (11) ◽

pp. 773-777

Author(s):

D.V. Novikov ◽

◽

T.S. Sabinina ◽

T.V. Shalbarova ◽

O.L. Chugunova ◽

...

Keyword(s):

Acute Kidney Injury ◽

Hemolytic Uremic Syndrome ◽

Thrombotic Microangiopathy ◽

World Literature ◽

Case Series ◽

Kidney Injury ◽

Number Of Patients ◽

Uremic Syndrome ◽

Coronavirus Infection ◽

Inflammatory Syndrome

Thrombotic microangiopathy (TMA) is one of the main links in the pathogenesis of multisystem inflammatory syndrome in children (MIS-C) associated with a new coronavirus infection caused by SARS-Cov-2 (COVID-19). In the setting of an increasing number of patients with hemolytic uremic syndrome (HUS) during the COVID-19 pandemic, 5 out of 7 cases of HUS (according to the Khimki Regional Hospital) in children revealed the association between acute kidney injury (AKI) and history of COVID-19. Also, there were recorded laboratory markers of systemic inflammation, the relevant criteria for the diagnosis of MIS-C associated with SARS-CoV-2. The article presents a clinical analysis of the case series concerning HUS, during which a comparison with the world literature data was conducted. It was proposed to consider this pathology in children as secondary thrombotic microangiopathy (TMA) in the setting of new coronavirus infection, similar to secondary TMA, developed due to exposure to HIV. During the COVID-19 pandemic, it is extremely important to establish the association between the TMA and the experienced COVID-19 in children for the therapy correction and further follow-up of patients after discharge from the hospital. KEYWORDS: new coronavirus infection, SARS-CoV-2, COVID-19, multisystem inflammatory syndrome, secondary thrombotic microangiopathy, hemolytic-uremic syndrome, acute kidney injury. FOR CITATION: Novikov D.V., Sabinina T.S., Shalbarova T.V. et al. Case series of secondary thrombotic microangiopathy in children with multisystem inflammatory syndrome associated with COVID-19. Russian Medical Inquiry. 2021;5(11):773–777 (in Russ.). DOI: 10.32364/2587-6821-2021-5-11-773-777.

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