Nephritis is a general term for inflammation of the kidneys. There are several hereditary forms of nephritis. Alport's syndrome (AS) is one of these but is unique because of the additional symptom of hearing loss in the presence of kidney failure. The disorder causes chronic glomerulonephritis with destruction of the glomeruli. The eventual result is end stage renal disease (ESRD) requiring dialysis (or transplantation) develops between adolescence and age 40.

AS affects the basement membranes which are the supporting layers of epithelial and endothelial tissues that make up much of the body's surfaces. The cause is a mutation in a gene for collagen. The disorder is uncommon, affecting about 2 out of 10,000 people.

Nephritis is a general term for inflammation of the kidneys. There are several hereditary forms of nephritis. Alport's syndrome (AS) is one of these but is unique because of the additional symptom of hearing loss in the presence of kidney failure. The disorder causes chronic glomerulonephritis with destruction of the glomeruli. The eventual result is end stage renal disease (ESRD) requiring dialysis (or transplantation) develops between adolescence and age 40.

The cause of AS is a mutation in a gene for collagen. The disorder is uncommon, affecting about 2 out of 10,000 people. Treatment requires medication, and often, kidney transplant.

Get the Facts... With your MediFocus Guide

The Medifocus Guide on Alport's Syndrome provides answers to the following important questions and medical issues:

• What are the most common symptoms of Alport's Syndrome?
  
• Are there any recognized risk factors for developing Alport's Syndrome?
  
• What kinds of medical tests are used to establish the diagnosis of Alport's Syndrome?
  
• What is the current standard of care for the treatment of Alport's Syndrome?
  
• What treatment options are available for the management of Alport's Syndrome?
  
• Are there any promising new developments or potential breakthroughs in treatment?
  
• Who are the most notable medical authorities who specialize in Alport's Syndrome?
  
• Where are the leading hospitals and centers of research for Alport's Syndrome?
  
• What are the most important questions to ask my doctor about Alport's Syndrome?
  

What Your Doctor Reads:

This MediFocus Guide contains an extensive listing of citations and abstracts of recent journal articles that have been published about this condition in trustworthy medical journals. This is the same type of information that is available to physicians and other health care professionals. A partial selection of journal articles that are abstracted in this MediFocus Guide includes:

• Alport syndrome and diffuse leiomyomatosis. Clinical aspects, pathology, molecular biology and extracellular matrix studies. A synthesis.
  Nephrologie. 2000
• Animal models of Alport syndrome: advancing the prospects for effective human gene therapy.
  Experimental Nephrology. 2000
• Alport syndrome. An inherited disorder of renal, ocular, and cochlear basement membranes.
  Medicine. 1999
• Alport syndrome. A review of the ocular manifestations.
  Ophthalmic Genetics. 1997
• Can Alport syndrome be treated by gene therapy?.
  Kidney International. 1997
• Alport's syndrome.
  Journal of Medical Genetics. 1997
• Alport syndrome.
  Kidney International - Supplement. 1997
• Alport syndrome.
  Kidney International. 1996
• Ocular clues to the nature of disease causing end-stage renal failure.
  Nephrology, Dialysis, Transplantation. 2000
• Long-term effects of cyclosporine A in Alport's syndrome.
  Kidney International. 1999

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