BACKGROUND: @entity4050 ( @entity4050 ) autoantibody-associated @entity1381 ( @entity1381 ) is a severe sub-type of @entity1381 . METHODS: We assessed the clinical and renal pathological features, circulating complement levels, and genetic background of Chinese pediatric @entity1 with this sub-type of @entity1381 . Thirty-three consecutive @entity1 with @entity810 who tested positive for serum anti- @entity4050 autoantibodies were enrolled in this study. RESULTS: All of the eight @entity1 who underwent renal biopsies presented with changes typical of @entity9818 , especially changes in chronic characteristics. Compared to @entity1 in remission and normal control subjects, @entity1 with @entity697 had significantly lower plasma @entity4050 levels and significantly higher plasma complement 3a ( @entity4417 ), @entity2242 , and terminal complement complex (SC5b-9) levels. The @entity4050 -anti- @entity4050 immunoglobin G (IgG) circulating immunocomplex ( @entity4050 -CIC) titers were more closely correlated with @entity4050 plasma levels than anti- @entity4050 IgG levels. Of the 22 @entity1 , four (18%) were homozygous for @entity40244 -1   and ten were heterozygous for @entity8173 or @entity40244 deletions. Most @entity1 responded well to a combination of plasma and immunosuppressive therapies, with a remission rate of 87%. At the end of the follow-up, nine @entity1 reached the combined end-points, including two with @entity454 and seven with relapses. CONCLUSION: Plasma @entity4417 , @entity2242 , and SC5b-9 levels predicted disease activity in anti- @entity4050 autoantibody-associated @entity1381 @entity1 enrolled in this study. These @entity1 responded well to plasma therapy combined with immunosuppression.

The clinical and laboratory features of Chinese Han anti- XXXX autoantibody-associated @entity1381 .

@entity1 :: ('9606', 'Species') :: ['patients'] @entity9818 :: ('MESH:D057049', 'Disease') :: ['thrombotic microangiopathy'] @entity8173 :: ('3078', 'Gene') :: ['CFHR1'] @entity697 :: ('MESH:D000208', 'Disease') :: ['acute disease'] @entity4417 :: ('718', 'Gene') :: ['C3a'] @entity2242 :: ('728', 'Gene') :: ['C5a'] @entity810 :: ('MESH:D058186', 'Disease') :: ['acute kidney injury'] @entity1381 :: ('MESH:D006463', 'Disease') :: ['hemolytic uremic syndrome', 'HUS'] @entity4050 :: ('3075', 'Gene') :: ['CFH', 'Anti-complement factor H'] @entity40244 :: ('10878', 'Gene') :: ['CFHR3'] @entity454 :: ('MESH:D007676', 'Disease') :: ['end-stage renal disease']
@entity4050 :: (3075,Gene) :: ['factor H']
