Hypocomplementaemia due to a genetic deficiency of beta 1H globulin.

  title={Hypocomplementaemia due to a genetic deficiency of beta 1H globulin.},
  author={Ronald A Thompson and Michael H. Winterborn},
  journal={Clinical and experimental immunology},
  volume={46 1},
An 8-month-old Asian boy who presented with the haemolytic uraemic syndrome was found to have a low haemolytic complement, and in particular a very low C3 level, with a normal C4 level. These abnormalities persisted after recovery and were not associated with the presence of circulating C3 nephritic factor. A clinically healthy 3-year-old brother was found to have an identical complement profile, which indicated increased alternative pathway activation. Both brothers had normal levels of the… CONTINUE READING