Alport’s Syndrome

Alport’s syndrome is a kidney disease. It is a hereditary disorder. Alport’s syndrome affects kidney function and can also cause hearing and vision disorders. Alport’s syndrome 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. Alport's Syndrome a significant decrease in or disappearance of proteinuria and apparently good tolerance to CsA were observed in all patients.Alport's Syndrome is thought to be due to an autosomal inherited defect of the glomerular basement membrane of the kidneys.

Alport's Syndrome is an inherited (usually X-linked) disorder involving damage to the kidney, blood in the urine, and in some families. A hereditary condition characterized by kidney disease, sensorineural hearing loss, and some difficulties with eye defects. Important examples are diabetes, dermatological conditions and myotonic dystrophy. A very rare genetic disease of the glomeruli that results in glomerular scarring and eventual. he Alport's Syndrome is dislocation of the lens is a well-known component of such conditions as Marfan's syndrome and homocystinuria. Spherophakia can form part of the Weill-Marchesani syndrome and lenticonus, when anterior and bilateral, is almost always indicative of Alport's syndrome.

Alport’s syndrome is a hereditary disorder characterised by progressive nerve deafness, pyelonephritis or glomerulonephritis and occasionally ocular defects. The main characteristics of this very rare hereditary syndrome are progressive renal failure, nerve deafness and abnormities of the lense of the eye. A significant decrease in or disappearance of proteinuria and apparently good tolerance to CsA were observed in all patients.

Symptom of Alports Syndrome

One of the first symptoms of Alport’s syndrome is usually hematuria, or blood in the urine. Tests also may reveal high levels of protein and white blood cells in the urine and waste products such as urea in the blood (called uremia). Due to the way Alport's Syndrome is inherited, the disease tends to be more severe in men than in women. The central feature of the disease is the presence of blood in the urine. Boys with Alport's develop this symptom in infancy, while girls who carry the Alport gene may or may not have it.

Another important symptom of Alport's Syndrome are:

• hearing loss.
• In boys with the disease, hearing loss is usally detectable by 8-10 years of age, and it may be severe enough to require the use of hearing aids.
• Hearing loss in girls tends to be much milder, rarely resulting in the need for hearing aids.

Causes of Alports Syndrome

Alport’s syndrome is caused by a defective gene carried on the X chromosome. Alport’s syndrome is primarily occurs in men. This syndrome can cause some symptoms in childhood, first symptoms usually occur in men in their twenties and thirties. The cause of Alport’s syndrome 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.

Most commonly causes of Alport's Syndrome

• Most people with Alport syndrome develop kidney failure in early adult life - in their late teens or twenties. Some (particularly women) only get the disease in later life.
• The kidney function deteriorates, there may be blood and protein in the urine, and high blood pressure may develop. Women may never get much more than these changes, but some of them go on to get kidney failure over decades.

How is Alport's Syndrome diagnosed?

Alport's syndrome can be diagnosed in several ways. First, if Alport's syndrome is known to run in the family, a simple test for blood in the urine may be enough to be fairly sure of the diagnosis. Second, someone with kidney disease might have a kidney biopsy.

Treatment and Cure of Alports Syndrome

Currently, there is no specific therapy for Alport's Syndrome. Treatment of the kidney disease consists of controlling high blood pressure and restricting dietary phosphorous. Men with Alport's Sydnrome eventually require replacement of kidney function with dialysis or kidney transplantation.

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