Atypical hemolytic uremic syndrome (aHUS), a rare variant of thrombotic microangiopathy, is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. The condition is associated with poor clinical outcomes with high morbidity and mortality. Atypical HUS predominantly affects the kidneys but has the potential to cause multi-organ system dysfunction. This uncommon disorder is caused by a genetic abnormality in the complement alternative pathway resulting in over-activation of the complement system and formation of microvascular thrombi. Abnormalities of the complement pathway may be in the form of mutations in key complement genes or autoantibodies against specific complement factors. We discuss the pathophysiology, clinical manifestations, diagnosis, complications, and management of aHUS. We also review the efficacy and safety of the novel therapeutic agent, eculizumab, in aHUS, pregnancy-associated aHUS, and aHUS in renal transplant patients.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1111/1744-9987.12763 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Physical Performance and Activity in Older Prostate Cancer Survivors in Comparison with Population-based Matched Controls.
Eur Urol Open Sci
January 2025
The Research Center for Age-Related Functional Decline and Disease, Innlandet Hospital Trust, Ottestad, Norway.
Article Synopsis
- The study aimed to assess the long-term physical performance and activity levels of older men who have survived radical prostate cancer compared to matched controls.
- The research included 109 men aged 70 and older who had received robotic surgery or radiation therapy, and their results were compared to 327 men from the general population.
- Overall, survivors showed similar physical performance and activity levels as controls, with only minor differences in grip strength and balance that might not be clinically significant, indicating that cancer treatment likely did not adversely affect their functional status.
Subtypes of brain change in aging and their associations with cognition and Alzheimer's disease biomarkers.
Neurobiol Aging
December 2024
Center for Lifespan Changes in Brain and Cognition, Department of Psychology, University of Oslo, Oslo 0373, Norway.
Structural brain changes underlie cognitive changes and interindividual variability in cognition in older age. By using structural MRI data-driven clustering, we aimed to identify subgroups of cognitively unimpaired older adults based on brain change patterns and assess how changes in cortical thickness, surface area, and subcortical volume relate to cognitive change. We tested (1) which brain structural changes predict cognitive change (2) whether these are associated with core cerebrospinal fluid (CSF) Alzheimer's disease biomarkers, and (3) the degree of overlap between clusters derived from different structural modalities in 1899 cognitively healthy older adults followed up to 16 years.
View Article and Find Full Text PDFA Case of Thrombotic Microangiopathy Secondary to Hypertensive Emergency: Presentation, Management, and Distinguishing Features.
Cureus
November 2024
Department of Medicine, Mercyhealth Graduate Medical Education (GME) Consortium, Rockford, USA.
Thrombotic microangiopathies (TMA) are a group of conditions that present with varying degrees of microthrombi, thrombocytopenia, microangiopathic hemolytic anemia, renal dysfunction, and neurological impairment. Etiologies can be primary, such as thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), and atypical hemolytic uremic syndrome (aHUS), or secondary, such as due to systemic infections, malignancies, immune-mediated conditions, and hypertensive emergencies. In hypertensive emergencies, this presentation can occur from mechanical stress placed on red blood cells as they pass through narrowed arteries due to edema and microangiopathic changes within the vessels themselves.
View Article and Find Full Text PDFLongitudinal cholesterol trends across socioeconomic groups in Norway: the influence of lipid-lowering drugs in the population-based Tromsø Study 1994-2016.
BMJ Open
December 2024
Health Services Research Unit, Akershus University Hospital, Lorenskog, Norway.
Objectives: There is limited evidence regarding the impact of lipid-lowering drugs (LLDs) on the socioeconomic gradient in a longitudinal perspective. The study investigates the longitudinal socioeconomic gradient in total cholesterol levels and whether this is affected by the use of LLDs.
Design: Population-based cohort study.
Cholecystectomy-induced thrombotic microangiopathy (TMA) in a postpartum patient successfully treated with eculizumab: a case report.
J Med Case Rep
December 2024
Department of Internal Medicine, University of California Irvine Medical Center, 333 City Blvd West, Suite 500, Orange, CA, 92868, USA.
Background: Thrombotic microangiopathy (TMA) is a rare, life-threatening disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end-organ damage. Atypical hemolytic uremic syndrome (aHUS) is even less common, comprising less than 10% of hemolytic uremic syndrome (HUS) cases. aHUS in postpartum is associated with poor maternal outcomes, with the majority of cases resulting in end-stage renal disease.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!