Atypical Hemolytic Uremic Syndrome Associated with Complement Factor H Mutation and IgA Nephropathy: A Case Report Successfully Treated with Eculizumab.

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

Nephron

journal_title

Nephron

authors

Nakamura H,Anayama M,Makino M,Makino Y,Tamura K,Nagasawa M

doi

10.1159/000485194

subject

Has Abstract

pub_date

2018-01-01 00:00:00

pages

324-327

issue

4

eissn

1660-8151

issn

2235-3186

pii

000485194

journal_volume

138

pub_type

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