Department of Internal Medicine and Hematology, Semmelweis University, Budapest, Hungary
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Understanding the Basis for Blocking MCPggaac Haplotype Complement Activity after Atypical Hemolytic Uremic Syndrome Incidence in Three Countries of Southeastern Europe
Author(s): Velibor Turudic*
We present a series of cases involving individuals with the homozygous MCPggaac haplotype, a genetic configuration associated with an
increased likelihood and severity of atypical hemolytic uremic syndrome (aHUS), particularly when combined with other high-risk aHUS mutations.
Complement blockade therapy was administered at a median age of 92 months (with an interquartile range of 36 to 252 months). Prior to initiating
CBT (Eculizumab), patients experienced a median of two disease relapses. These relapses transpired within an average span of 22.16 months
(median of 17.5, ranging from a minimum of 8 months to a maximum of 48 months) following the initial subsequent disease onset (observed in 6 out
of 8 patients). Treatment encompassed plasmapheresis/intravenous plasma exchange (PI/PEX), occasionally supplemented by renal replacement
therapy (RRT). Upon the imple.. Read More»
DOI:
10.37421/2472-1026.2023.8.213
Journal of Forensic Medicine received 165 citations as per Google Scholar report