Also, males tend to be more affected as Alport syndrome is X-linked in most cases. In IgA nephropathy, episodes of frank hematuria are more common, and a family history is less common.A kidney biopsy is the only way to diagnose thin basement membrane disease. It reveals thinning of the glomerular basement membrane from the normal 300 to 400 nanometers (nm) to 150 to 250 nm. However, a biopsy is rarely done in cases where the patient has isolated microscopic hematuria, normal kidney function, and no proteinuria. The prognosis is excellent in this setting unless the clinical manifestations progress, as occurs in most males and some females with Alport syndrome and many patients with IgA nephropathy. Treatment
Most patients with thin basement membrane disease need only reassurance. Indeed, this disease was previously referred to as "benign familial hematuria" because of its usually benign course. Angiotensin converting enzyme inhibitors have been suggested to reduce the episodes of hematuria, though controlled studies are lacking. Treating co-existing hypercalciuria and hyperuricosuria will also be helpful in reducing hematuria.The molecular basis for thin basement membrane disease has yet to be elucidated fully; however, defects in the gene encoding the a4 chain of type IV collagen have been reported in some families. Prognosis
Overall, most people with thin basement membrane disease have an excellent prognosis. Some reports, however, suggest that a minority might develop hypertension.Thin basement membrane disease may co-exist with other kidney diseases, which may in part be explained by the high prevalence of thin basement membrane disease.
NF2 increases the risk of early death. Schwannomatosis symptoms develop in early childhood and can worsen with time. Typically life expectancy is unaffected in those with schwannomatosis. History
Descriptions of what is believed to be the condition go back as far back as the 1st century. The conditions were formally described by Friedrich Daniel von Recklinghausen in 1882, after whom it was previously named. References
Further reading
Upadhyaya M, Cooper D (29 January 2013). Neurofibromatosis Type 1: Molecular and Cellular Biology. Springer Science & Business Media. ISBN 9783642328640. External links

National Institute of Neurological Disorders and Stroke - information on Neurofibromatosis