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Table 2 Frequencies of autoantibodies against complement proteins in patients affected by BSS

From: Immunological features of patients affected by Barraquer-Simons syndrome

 C3NeFanti-C3anti-FBanti-Panti-FIanti-FHC3 (mg/dl)
BSS1PositivePositiveNegativeNegativeNegativeNegative3.0
BSS2PositivePositiveNegativeNegativeNegativeNegative11.9
BSS3PositivePositivePositivePositiveNegativeNegative31.7
BSS4PositivePositiveNegativeNegativeNegativeNegative7.9
BSS5PositiveNegativeNegativeNegativeNegativeNegative14.8
BSS6NegativeNegativeNegativeNegativeNegativeNegative88.5
BSS7PositiveNegativePositivePositiveNegativeNegative38.7
BSS8NegativeNegativeNegativeNegativeNegativeNegative121.0
BSS9PositiveNegativePositivePositiveNegativeNegative20.6
BSS10PositiveNegativePositivePositiveNegativeNegative50.2
BSS11NegativeNegativeNegativeNegativeNegativeNegative127.0
BSS12PositiveNegativeNegativePositiveNegativeNegative8.9
BSS13NegativeNegativeNegativeNegativeNegativeNegative113.0
 9/13 (69.2%)4/13 (30.8%)4/13 (30.8%)5/13 (38.5%)0/13 (0%)0/13 (0%)Range: 75–135 mg/dl
  1. Abbreviations: C3NeF C3 nephritic factor, FB factor B, P properdin, FI factor I, FH factor H