Atypical Hemolytic‐Uremic Syndrome: An Update on Pathophysiology, Diagnosis, and Treatment

Raina, Rupesh; Krishnappa, Vinod; Blaha, Taryn; Kann, T; Hein, William; Burke, Linda; Bagga, Arvind

doi:10.1111/1744-9987.12763

Cited by 126 publications

(209 citation statements)

References 92 publications

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“…At present, laboratory analysis methods for testing genetic mutations potentially causing aHUS are able to show mutations in only up to 40-60% of aHUS cases, leaving the possibility of false negative cases. Therefore, a negative test result for mutations does not rule out a true aHUS (16). Rarely aHUS has been induced after pregnancy and parturition.…”

Section: Discussionmentioning

confidence: 99%

Eculizumab Treatment for Postpartum HELLP Syndrome and aHUS—Case Report

2020

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Preeclampsia is a pregnancy-specific disorder affecting ca 3% of all pregnant women. Preeclampsia is the source of severe pregnancy complications. Later life consequences for mother and infant include increased risk of cardiovascular disease. Preeclampsia is caused by the dysfunction of the endothelium with subsequent activation of complement and coagulation systems. HELLP syndrome is considered to be an extreme complication of preeclampsia but it can also present independently. Diagnostic symptoms in HELLP syndrome are Hemolysis, Elevated Liver enzymes, and Low Platelets. Similar phenotype is present in thrombotic microangiopathies (TMAs) and HELLP syndrome is considered part of the TMA spectrum. Here, we present a case of severe preeclampsia and HELLP syndrome, which exacerbated rapidly and eventually led to need of intensive care, plasma exchange, and hemodialysis. The patient showed signs of hemolysis, disturbance in the coagulation, and organ damage in liver and kidneys. After comprehensive laboratory testing and supportive care, the symptoms did not subside and treatment with complement C5 inhibitor eculizumab was started. Thereafter, the patient started to recover. The patient had pregnancy-induced aHUS. Earlier initiation of eculizumab treatment may potentially shorten and mitigate the disease and hypothetically decrease future health risks of preeclamptic women.

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Section: Discussionmentioning

confidence: 99%

Eculizumab Treatment for Postpartum HELLP Syndrome and aHUS—Case Report

2020

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“…It is thought that until TTP is excluded plasma exchange should be started [5]. In larger laboratories the ADAMST13 takes a few hours to run; however in smaller hospitals such as ours the study takes a couple of days.…”

Section: Diagnosis and Treatment In Critical Care Settingmentioning

confidence: 98%

Treating Patients with Atypical Familial Hemolytic Uremic Syndrome (aHUS) In the Critical Care Setting: A Case and Discussion

2019

JCRC

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Hemolytic Uremic Syndrome (HUS) is usually recognized as the diarrheal associated (D+) and less as atypical/non diarrheal (aHUS). AHUSis caused by over activation of the alternative pathway of the complement system. It is a rare genetic disorder that is not often seen. Here we present a case of a patient with aHUS, who came to our ICU, discuss the pathophysiology, and how to treat and monitor a patient with aHUS with plasmapheresis and eculizumab in the critical care setting

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“…The diagnosis of a-UHS requires the following findings: 1) microangiopathic hemolytic anemia (hemoglobin < 10 g/dl) with direct negative Coombs test, elevation of lactate dehydrogenase (DHL), decrease of serum haptoglobin with peripheral blood schistocytes, 2) thrombocytopenia (<150,000 cell/mm 3 ), 3) acute kidney injury [101]. Patients require evaluation of the complement pathway, at least with serum levels of C3, factor H, I and B in addition to autoantibodies against factor H [102].…”

Section: Atypical Hemolytic Uremic Syndrome (A-hus)mentioning

confidence: 99%

Phenocopies: Mimics of Inborn Errors of Immunity

Alonso-Bello¹,

Espinosa‐Padilla²,

Temix-Delfin³

et al. 2020

OALib

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A phenocopy is defined as a clinical non-inherited phenotype in an individual, with environmental induction, which is identical to the genetically determined phenotype of another. Until February 2017, the IUIS (International Union of Immunological Societies) reported in its classification 354 innate immunity errors and a final group (classification table IX) with conditions that are not part of the innate alterations and are called phenocopies. These are classified into two types, the associated with somatic mutations and those associated with auto-antibodies. The phenotypes that occur by any of the mechanisms mentioned are complex and varied. It is necessary to know the clinical manifestations of the pathologies classified in this group to enrich the possible differential diagnoses in individuals with suspected immunodeficiency.

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Atypical Hemolytic‐Uremic Syndrome: An Update on Pathophysiology, Diagnosis, and Treatment

Cited by 126 publications

References 92 publications

Eculizumab Treatment for Postpartum HELLP Syndrome and aHUS—Case Report

Eculizumab Treatment for Postpartum HELLP Syndrome and aHUS—Case Report

Treating Patients with Atypical Familial Hemolytic Uremic Syndrome (aHUS) In the Critical Care Setting: A Case and Discussion

Phenocopies: Mimics of Inborn Errors of Immunity

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