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Haemolytic uraemic syndrome
Fadi Fakhouri1, Julien Zuber2, Véronique Frémeaux-Bacchi3
1Department of Nephrology, Centre Hospitalier Universitaire, and INSERM UMR S1064, Nantes, France.
Lancet (London, England)
|March 1, 2017
Summary
Haemolytic uraemic syndrome (HUS) is a kidney-damaging condition with multiple causes. While some HUS forms now have targeted treatments, others like Shiga toxin-induced HUS lack specific therapies.
Area of Science:
- Nephrology
- Hematology
- Pathophysiology
Background:
- Thrombotic microangiopathy characterized by thrombocytopenia, hemolytic anemia, and acute kidney injury.
- Encompasses diverse disorders including Shiga toxin-induced, pneumococcus-induced, complement-related, and cobalamin C defect-related HUS.
- Also includes HUS secondary to infections, drugs, cancer, and systemic diseases.
Purpose of the Study:
- To review the pathophysiology and diagnostic advancements in various forms of hemolytic uremic syndrome.
- To highlight the availability of mechanism-based treatment for specific HUS subtypes.
- To identify HUS types lacking specific therapeutic options.
Main Methods:
- Review of experimental, genetic, and clinical studies over the past two decades.
- Analysis of pathophysiological mechanisms underlying different HUS forms.
- Evaluation of current diagnostic and therapeutic strategies.
Main Results:
- Significant progress in understanding HUS pathophysiology and improving diagnostics.
- Development of specific, mechanism-based treatment for atypical HUS due to complement dysregulation.
- Absence of specific treatments for several HUS types, notably Shiga toxin-induced HUS.
Conclusions:
- Advances in research have improved HUS management and diagnosis.
- Targeted therapies are available for certain HUS forms, offering improved outcomes.
- Further research is crucial to develop treatments for HUS types currently lacking specific therapeutic options.
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