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Alport Syndrome

Alport Syndrome is a genetic disorder characterized by the scarring of the small filtering units in kidneys called glomeruli. As glomeruli are damaged, blood and proteins will leak into urine through them, resulting in proteinuria and hematuria.

Symptoms

As the mutation in Alport Syndrome can affect type IV collagen proteins which are important to the normal structure and function of inner eyes and ears. Eye and ear problems are also pretty common in the patients with Alport Syndrome. If you are suspected to have Alport Syndrome, look for these symptoms:

alport syndrome basics

* Hematuria

* Proteinuria

* High blood pressure

* Swelling

* Cataracta and even vision loss

* Hearing problems and even hearing loss

Alport Syndrome symptoms are more severe in boys than girls. As boys grow, a myriad of signs will occur including protein in urine and hematuira. These symptoms are usually present by the time the boys are teenagers. These symptoms do not appear in girls until much later in their life. However, occasionaly, the teen-aged girls with Alport Syndrome also appear these symptoms. Deafness can occur in about 80% boys with Alport Syndrome, but it rarely occurs in girls.

Prognosis

Alport Syndrome can cause progressive kidney damage. As the kidney structure is scarred, it will lead to Renal Failure. All boys with Alport Syndrome will develop Renal Failure during their teen-age or young the adult years, but it may occur by age 30 to 40. The great majority of girls with Alport Syndrome do not develop Kidney Failure. However, as they grow older, the possibility of developing Kidney Failure increases.

Diagnosis

If you are suspected to have Alport Syndrome, the following tests will be ordered by your doctor.

* Urinalysis

* Blood tests

* Renal biopsy

* Audiometry(test ability to hear)

* Skin biopsy

If you have been diagnosed with Alport Syndrome, we're here to help. Call us: +86-311-86954186 or email us: kidneyabc@hotmail.com(Monday through Sunday) to make an appointment.

alport syndrome symptoms

Alport Syndrome is also known as Hereditary Nephritis characterized by inflammation of glomeruli, the tiny capillaries in the kidneys that filter wastes from the blood. Apart from kidney damage, hearing and eyes are also be affected the disease.

Kidneys

Hematuria( presence of blood in urine) is a typical feature of Alport Syndrome. Boys with X-linked Alort Syndrome develop hematuria, and it is always present. Most of girls with X-linked Alport Syndrome also experience hematuria, but it may come and go. Microscopic hematuria is very common in the patients, but gross hematuria is rarely seen. If gross hematuria occurs, the urine will become brown, pink or red. A cold or flu is a common trigging of hematuria. The gross hematuria will subside after several days and it is not harmful.

Symptoms usually occurs earlier in boys with Alport Syndrome than in girls. As boys grow, other symptoms of Kidney Disease will appear, including proteinuria and high blood pressure. These symptoms usually begin to show in the teen-aged boys. Girls with Alport Syndrome usually do not have protein in urine and high blood pressure until much later in life. However, occasionally, it occurs earlier in their life.

Eyes

As the abnormal gene in Alport Syndrome affect type IV collagen proteins. Because the protein contribute to the normal functioning of the eyes and ears, many patients may experience eye problem and even hearing loss. About 15% men with Alport Syndrome have abnormality in the shape of anterior lenticonus. They may develop cataracts and even vision loss.

Ears

Hearing problem is commonly seen among the patients with Alport Syndrome and it is more common in boys than in girls. Deafness can occur in about 80% cases of in boys, often by the time they are teenagers. Girls with Alport Syndrome may also develop deafness, but less frequently than boys, and usually in later life. Luckily, hearing acids are helpful for them.

If you have been diagnosed with Alport Syndrome, we're here to help. Call us: +86-311-86954186 OR email us: kidneyabc@hotmail.com(Monday through Sunday) to make an appointment.

alport syndrome treatment

Alport Syndrome is a genetic kidney disorder. Although there is no cure for it, an effective and timely treatment can prevent the development of kidney failure. The treatment options for Alport Syndrome include:

Medication

Angiotensin-converting enzyme(ACE) inhibitors are medications for high blood pressure. They have been shown to slow the progression of Alport Syndrome with a low occurrence of major side effects. Also, angiotensin-converting enzyme(ACE) inhibitors have benefits in reducing proteinuria and slowing progression of Alport Syndrome. ACE inhibitors and/or ARBs should be given to patients with Alport Syndrome who have proteinuria with or without high blood pressure.

Dialysis and kidney transplant

Conventionally, there is no treatment which can prevent Alport Syndrome from developing End Stage Renal Disease( ESRD). When kidneys fail to work completely or almost completely, dialysis or kidney transplant has to be required. Dialysis can relieve the symptoms of Alport Syndrome, but has no use in improving renal function at all. As Alport Syndrome is a familial disease, related kidney donors must be carefully evaluated for this disease. Also, the potential risks and complications after transplant can not be preventable.

Micro-Chinese Medicine Osmotherapy

Micro-Chinese Medicine Osmotherapy is a natural therapy developing on the basis of traditional Chinese medicines. It is an external therapy, in which the medicines can be penetrated into kidney lesions directly through the skin in kidney area. The medicines can prevent the scarring of glomeruli and stimulate the regeneration of impaired kidney tissues and cells.

Medicated bath

Skin is a powerful protective and excretory system of human body. A part of wastes is filtered from body by perspiring, such as salts, extra fluids, urea. In Alport Syndrome, when kidneys are damaged, dangerous levels of toxins and wastes will build up in body. Medicated bath can work in excreting the wastes. Moreover, some nutrients and medicine ingredients will be absorbed by skin and then made use of by body. Medicated bath has remarkable effects in relieving symptoms, such as swelling, skin itching, etc.

Blood purification

Blood purification is used to remove wastes and toxins in body. It is used in the later stage of Alport Syndrome. Compared with dialysis alone which only can clear micromolecular toxins, it can give a complete removal of toxins and wastes from body including micromolecular, middle-molecular and macromolecular toxins and harmful substances.

If you have been diagnosed with Alport Syndrome, we're here to help. Call us: +86-311-86954186 OR email us: kidneyabc@hotmail.com(Monday through Sunday) to make an appointment.

alport syndrome diagnosis

When you are suspected to have Alport Syndrome, your doctor will order the following procedures and tests to get a confirmed diagnosis.

Physical examination

Initially, you may not show obvious physical presentation. However, with time, a myriad of symptoms will appear, mainly including high blood pressure, edema, proteinuria, hematuria. Moreover, various extrarenal features may also be observed, as follows:

Hearing loss occurs commonly in males with Alport Syndrome, accounting for 80% cases and it usually occurs by the time of teenagers. It less commonly occurs in females than in males and late in life. The risk of developing hearing loss by age 40 years is approximately 90% in males and 10% in females with X-linked Alport Syndrome. Also, eye problems and even vision loss can be frequently seen in the patients with Alport Syndrome. So hearing and vision evaluation and testing is essential in diagnosis of Alport Syndrome.

Family history

As Alport Syndrome is a genetic disorder, detailed family history and possibly urinalyses on first-and second-degree can help diagnosis of the disease.

Renal biopsy

Alport Syndrome can cause unique changes in the walls of tiny blood vessels of the glomeruli. The changes can be detected by performing electron microscopy on the kidney biopsy material. Also, kidney biopsy also can test the the presence and absence of the type IV collagen alpha-3, alpha-4, alpha-5 chains. This information can help get a confirmed diagnosis of Alport Syndrome.

Skin biopsy

If you are suspected to have X-linked Alport Syndrome, skin biopsy will be ordered by your doctor. As type IV collagen alpha-5 chain is normally present in the skin, a skin biopsy can test the presence or absence of this collagen chain.

Genetic testing

After the above tests have been finished, the diagnosis still remains doubtful. Genetic testing may be performed. However, for its high cost, th time consuming, low detection rate of COL4A5, etc, genetic testing is not widely used yet.

If you have been diagnosed with Alport Syndrome, we're here to help. Call us: +86-311-86954186 OR email us: kidneyabc@hotmail.com(Monday through Sunday) to make an appointment.

alport syndrome knowledge

Alport Syndrome is an inherited kidney disease caused by genetic mutations that affects the type of IV collagen family of proteins. It can be inherited to children in different modes.

Type IV collagen is a family of six proteins, known as known as alpha-1,alpha-2,alpha-3,alpha-4,alpha-5 alpha-6. When alpha-3, alpha-4, or alpha-5 type IV collagen chains is affected, Alport Syndrome will occur.

Alport Syndrome has three genetic types, in which, X-linked Alport Syndrome is the most common form, accounting for at least 80% cases. X-linked Alport Syndrome is caused by mutations in the gene that produces the alpha-5 chain of type IV collagen, which is located on the X chromosome.

X and Y is sex chromosome. Men have only one X chromosome and women have two X chromosome. Men pass their X chromosome to their doctors, but not to their sons. Therefore, if a man has X-linked Alport Syndrome, all of his daughters will get the disease, but his sons will not be affected by the disease. As for a woman with X-linked Alport Syndrome, every child ( girl and boy) has 50% chance of getting the disease.

In 15% all cases, the patients have autosomal recessive Alport Syndrome. Alpha-3 and alpha-4 type IV collagen chains are located on chromosome 2. Everyone has two copies of chromosome 2, so everyone has two copies of the alpha-3 and alpha-4 chain genes. Autosomal recessive Alport Syndrome occurs when genetic mutation occurs in both copies of alpha-3 or alpha-4. It can affect girls as severely as boys.

As a person with autosomal recessive Alport Syndrome only passes one of the mutant copies of the alpha 3 or 4 chain gene to a child. Therefore, the risk of developing the disease is very low. However, every child carries a mutated copy of the abnormal gene.

In 5% all cases, the patients have autosomal dominant Alport Syndrome. These people have one mutant copy of the alpha 3 or alpha 4 chain gene. Every child has 50% chance of inheriting the mutant copy of the gene.

If you have been diagnosed with Alport Syndrome, we're here to help. Call us: +86-311-86954186 OR email us: kidneyabc@hotmail.com(Monday through Sunday) to make an appointment.

alport syndrome healthcare

Alport Syndrome can run in the family and influence generation after generation. Along with effective treatments, proper health care also pays a vital role in slowing the disease progression and improving its prognosis.

Diet

* Limit the consumption of saturated fat. As saturated fat is loaded in cholesterol, too much consumption can increase its level, which can increase the risk of cardiovascular diseases. It is wise for you to reduce the animal oil intake and some vegetable oil mainly including coconut oil, cottonseed oil and cacao oil.

* In Alport Syndrome, swelling and high blood pressure can be caused by retention of sodium and fluids in body. Therefore, you are recommended to limit the intake of sodium and fluids. If you are suffering from swelling, you should reduce your sodium intake to 1 grams each day.

* Proteins can produce urea which can put extra strain on your kidneys. If you are diagnosed with proteinuria, limiting protein intake to 1g/kg per body weight each day is a wise choice. Meanwhile, you should choose high-quality protein foods, such as fish, egg white, etc.

Apart from the above dietary principles, you should also reduce the intake of foods rich in phosphorus and potassium. It is helpful in reducing the incidence of complications.

Exercise

Proper exercise can improve immunity and reduce the risk of infection as well as improve blood circulation and increase the excretion of wastes from body. You should choose proper exercise based on your overall condition and degree of kidney damage. Walking, bicycling, swimming, jogging and other aerobic exercises are good choices for you. However, if you have serious swelling, you should stay in bed until it relieves. Therefore, before starting any exercise program, you should consult your doctor to make an individualized exercise regime.

If you have been diagnosed with Alport Syndrome, we're here to help. Call us: +86-311-86954186 OR Email us: kidneyabc@hotmail.com(Monday through Sunday) to make an appointment.

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