关键词: Atypical hemolytic uremic syndrome Complement biology. Graft failure Histocompatibility Kidney transplantation

Mesh : Adult Antibodies, Monoclonal, Humanized / therapeutic use Atypical Hemolytic Uremic Syndrome / diagnosis genetics immunology therapy Biopsy Complement Activation / drug effects genetics Complement Factor I / genetics Complement Inactivating Agents / therapeutic use DNA Mutational Analysis Genetic Predisposition to Disease Humans Kidney / drug effects immunology pathology surgery Kidney Transplantation / adverse effects Male Mutation Phenotype Recurrence Reoperation Treatment Failure

来  源:   DOI:10.1111/j.1600-6143.2009.02764.x   PDF(Sci-hub)

Abstract:
Atypical hemolytic uremic syndrome (aHUS) has gained increased visibility over several years as an important cause of renal failure. Unfortunately, diagnosis is often difficult because individual courses can be highly variable depending the causative genetic mutations. Here we present the case of a patient with a failed renal allograft and acute failure of a second allograft who was ultimately diagnosed with aHUS. Interestingly, he developed early de novo donor specific antibodies (DSA) after the second renal transplant in context of likely recurrent aHUS. Terminal complement inhibition with eculizumab resulted in prompt improvement of renal allograft function.
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