We hereby present a case of an atypical hemolytic uremic syndrome (aHUS) precipitated by coronavirus disease 2019 (COVID-19). A 26-year-old male was diagnosed with COVID-19 and acute kidney injury. His kidney biopsy was suggestive of thrombotic microangiopathy. Five sessions of plasmapheresis were done but were discontinued in view of nonrecovery of kidney function. He was then referred for a kidney transplant. On genetic analysis, he was found to have mutations in the complement system (CFHR1 and CFHR3), which suggested this was a case of aHUS precipitated by COVID-19. In view of the high risk of recurrence of the primary disease in live-related kidney donor transplantation, he was advised for simultaneous liver and kidney transplants.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9364994PMC
http://dx.doi.org/10.4103/ijn.ijn_196_21DOI Listing

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Article Synopsis
  • Hemolytic uremic syndrome (HUS) is a serious condition that can happen after an infection or due to problems in the body's immune system, and there are two types: typical and atypical.
  • This text discusses a 9-year-old boy who had a rare form of aHUS caused by specific genetic issues, which made his body produce autoantibodies that affected his kidneys and brain.
  • The study found that many kids with aHUS needed kidney treatment and that early treatment with special therapy may help prevent kidney failure, even if the patient has infections like STEC.
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Atypical hemolytic uremic syndrome (aHUS) is a rare disease caused by uncontrolled complement activation due to complement dysregulation. It is often triggered by precipitating events such as infections, inflammation, pregnancy, or medications. Dengue, an endemic viral infection in Southeast Asia, can activate the complement pathway, thereby triggering aHUS in genetically susceptible individuals.

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Hot Spot of Complement Factor I Rare Variant p.Ile357Met in Patients With Hemolytic Uremic Syndrome.

Am J Kidney Dis

August 2024

Department of Nephrology, Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Paris, France; Paris University, Paris, France.

Atypical hemolytic uremic syndrome (aHUS) is a rare kidney disease due to a dysregulation of the complement alternative pathway. Complement factor I (CFI) negatively regulates the alternative pathway and CFI gene rare variants have been associated to aHUS with a low disease penetrance. We report 10 unrelated cases of HUS associated to a rare CFI variant, p.

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We hereby present a case of an atypical hemolytic uremic syndrome (aHUS) precipitated by coronavirus disease 2019 (COVID-19). A 26-year-old male was diagnosed with COVID-19 and acute kidney injury. His kidney biopsy was suggestive of thrombotic microangiopathy.

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Article Synopsis
  • The study investigates the link between IgA nephropathy (IgAN) and atypical hemolytic uremic syndrome (aHUS), highlighting a poor renal prognosis for patients with both conditions.* -
  • Six adult patients with IgAN and aHUS were analyzed, all progressing to end-stage kidney disease, with findings indicating low serum C3 levels and genetic predisposition for aHUS.* -
  • The research suggests that the activation of the alternative complement pathway may contribute significantly to the poor outcomes in these patients, proposing that underlying genetic or other factors may explain the pathophysiology of the connection between aHUS and IgAN.*
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