Abstract:
:We present a rare case of IgA nephropathy in a patient who developed atypical hemolytic uremic syndrome (aHUS) associated with a complement factor H (CFH) gene mutation, and who was successfully treated with eculizmab. A 76-year-old man was admitted as the patients had thrombotic microangiopathies findings. The patient was treated with plasma exchange, hemodialysis and methylprednisolone. A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 level was not decreased. Light microscopy findings were consistent with hemolytic uremic syndrome and immunofluorescence analysis revealed IgA and C3 were detected. Genetic analysis revealed that mutation of p.Arg1215Gln in CFH was identified. The diagnosis of aHUS was confirmed and eculizmab therapy was currently effective for 5 months.
journal_name
Nephronjournal_title
Nephronauthors
Nakamura H,Anayama M,Makino M,Makino Y,Tamura K,Nagasawa Mdoi
10.1159/000485194subject
Has Abstractpub_date
2018-01-01 00:00:00pages
324-327issue
4eissn
1660-8151issn
2235-3186pii
000485194journal_volume
138pub_type
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