Benign familial haematuria (BFH) is an inherited renal disorder characterised by the presence of haematuria and the absence of progressive renal dysfiinction. Analysis of renal biopsies from affected individuals has shown that typically the only abnormality is widespread thinning of the glomerular basement membrane (GEM) visible on electron microscopy (EM). Thinning of the GBM has also been observed as the sole abnormality in patients with nonfamilial (isolated) haematuria, resulting in the diagnostic term thin basement membrane disease (TBMD). It is possible that some patients with benign familial haematuria have normal basement membranes, while others have thin basement membranes. The predominance of TBMD in patients with BFH has led some authors to use the terms interchangeably, but there are also some patients with thin basement membrane who do not have a benign prognosis. Thus the precise relationship between an abnormally thin GBM, isolated haematuria and BFH remains unclear.
| Identifer | oai:union.ndltd.org:bl.uk/oai:ethos.bl.uk:497918 |
| Date | January 2008 |
| Creators | Makanjuola, Akinwale David |
| Publisher | King's College London (University of London) |
| Source Sets | Ethos UK |
| Detected Language | English |
| Type | Electronic Thesis or Dissertation |
Page generated in 0.139 seconds