What are the symptoms of Alport Syndrome?

Alport Syndrome always affects the kidneys. Many people with Alport Syndrome also have hearing problems and abnormalities of the eyes, because the type IV collagen proteins are important to the normal structure and function of the inner ear and the eye.

Kidneys: The central feature of the disease is the presence of blood in the urine (hematuria). Boys with X-linked Alport Syndrome develop hematuria in infancy, and it is always present. The great majority of girls with X-linked Alport Syndrome also have hematuria, but it may come and go The hematuria of Alport Syndrome is usually microscopic, meaning it can only be detected with a microscope or a urine dipstick. Sometimes children with Alport Syndrome have brown, pink or red urine ( gross hematuria) for several days, brought on by a cold or the flu. This gross hematuria will go away on its own and while it may be frightening, it is not harmful.

As boys with Alport Syndrome grow, they begin to show other signs of kidney disease, including protein in the urine and high blood pressure. These symptoms are often present by the time the boys are teen-agers. Girls with Alport Syndrome usually do not have protein in the urine and high blood pressure until much later in life, but occasionally these symptoms appear in teen-aged girls with Alport Syndrome.

Ears:Deafness is another important feature of Alport Syndrome. About 80% of boys with Alport Syndrome will develop deafness at some point in their lives, often by the time they are teen-agers. The deafness affects both ears. Fortunately, hearing aids are usually very effective in these people. Girls with Alport Syndrome may also develop deafness, but less frequently than boys, and usually later in life. Kidney transplantation does not improve the deafness of Alport Syndrome.

Eyes:About 15% of men with Alport Syndrome have an abnormality in the shape of the lens called anterior lenticonus. People with anterior lenticonus may have some problems with their vision, and may develop cataracts.

How to Take care Patients with Diabetic Nephropathy?

Diabetic Nephropathy is the disease that Diabetes leads to different levels of kidney damage. In 10 years, most patients with Diabetic Nephropathy will develop to Chronic Kidney Failure and Uremia. After having Diabetic Nephropathy, the glomerular capillary basement membrane will grow thick and then appear glomerular sclerosis. The clinical symptoms are proteinuria (protein in the urine), swelling, high blood pressure, anemia, abnormal kidney function and retinal disease. For patients with Diabetic Nephropathy, from excreting proteinuria to dying of Uremia, the death interval is about 10 years. For the patients with protein more than 3g, they are usually dying within 6 years. Uremia is a serious and high concurrent rate complication in the urinary system for patients with Diabetes. So Diabetic Nephropathy is one of the most common complications for Diabetes. Patients usually develop to End Stage Kidney Failure because of Diabetic Nephropathy, which is the direct inducement resulting to a high death rate for patients with diabetes.

In Yumei Kidney Disease Hospital, there are many experts with rich clinical experience for Diabetic Nephropathy treatment. The treatment using integration of Western Medicine treatment and Traditional Chinese Medicine treatment is very effective. As to how to take care of patients with Diabetic Nephropathy, the experts have much reasonable suggestions. The suggestions are:
1. Control and mix the diet reasonably.
Patients should eat less food with high sugar, such as potato, sweet potato, banana, rice, wheat flour. All these food contains large amounts of starch, after digestion, it is easy to transform to carbohydrate, which can increase kidney burden.
Patients can eat a little food with low protein, such as milk without fat, egg white, fish and shrimp and such protein food, accompanying with vegetables containing much cellulose, which cannot only ensure that protein intake will not increase kidney burden, but also help patients recuperate the more healthy physique.
2. Doing exercise and prevent disease
Patients with diabetes can consume and control blood sugar through doing exercise. According to patients’ physique and disease condition, they can do exercise with the help of Patients can medical sports equipment or walking and running to strengthen the function of muscle tissue resolving glucose, which can eliminate excessive fat and improve lipid metabolism.
3. Activities that is suitable for patients with Diabetic Nephropathy.
For patients with Diabetic Nephropathy and Diabetes, they should choose the slow, regular-frequency, persistent and malleable activity. Patients should choose the low-intensity and low-toughness activity.
4. The characteristic treatment in Yumei Kidney Disease Hospital.
(1)Use the Traditional Chinese Medicine treatment to differentiate the syndrome and symptoms. Integration of Traditional Chinese Medicine and Western Medicine Treatment, and systemic treatment.
(2)Use medicine through multiple pathways to accelerate medicine factor absorb and improve immune balance.
(3) Classical prescription together with external treatment can tonify the kidneys, clear away the lung-heat, promote blood circulation, keep balance of Yin and Yang, repair the damaged cells and recover kidney function.
(4)The theory of Traditional Chinese medicine is strengthening genuine Qi to eliminate pathogenic factors, strengthening spleen and tonifying lung.

(5) The treatment together with natural treatment can improve the effect, no poisoning, no side-effects, and also reduce the recovering time to regulate systemically, which can effectively recover the kidney function and prevent recurring.

Minimal Change Nephrosis

　　
　　Minimal Change Nephrosis (MCN) is also called Lipoid Nephrosis. It is characterized with the basically normal glomeruli under light microscope; and with glomerular epithelial podocytes enation, fusion and vanish, with no visible mesangial cell proliferation, the matrix broadening and immunoglobulin deposition under the electron microscope. The clinical manifestation of Minimal Change Nephrosis are often presenting as Nephritic Syndrome, such as: large volume of proteinuria, hypoalbuminemia, edema, hyperlipidemia and so on. Minimal Change Nephrosis is one of the most common glomerular disease in Children. MCN is sensitive to the glucocorticoids treatment with tendency of easy relieving and easy relapsing. For this disease the general prognosis is good, less of the patients (<5%) may progress to End Stage Kidney Failure.
　　The cause of Minimal Change Nephrosis.
　　Minimal Change Nephrosis can be divided into primary and secondary MCN. The cause of primary MCN is unknown. The cause of secondary MCN includes:
　　Drugs: non-steroidal anti-inflammatory drugs (NASIDs), antibiotic (rifampicin, ampicillin, cefixime and so no), Interferon, gold and lithium compound, Methimazole, and Enalapril and so on.
　　Infection: HIV, Guillain-Barre Syndrome, Syphilis, Parasite (such as blood fluke) and so on.
　　Tumor: Hodgkin’s Disease, Non-Hodgkin’s Disease, solid tumor, Acidophilic Cell- Lymphoma (Kumara Disease).
　　Allergy: food, pollen, dust, insect biting and so on.
　　Familial Minimal Change Nephrosis: at present, only reported that less families have the Familial Minimal Change Nephrosis, and the pathological gene is unclear.
　　The Clinical Manifestation.
　　1. Edema.
　　The obvious edema is often the first manifestation of MCN. The most children patients present the facial edema, and the adult patients present obvious edema in lower limb.
　　2. Proteinuria
　　Patients present large volume of proteinuria, and they are often high-selectivity proteinuria, mainly are albumin. The urine protein will be more than 10g/d.
　　3. Hypoalbuminemia
　　The plasma albumin is often significantly declined. Some individuals patients (commonly to see in children) will be less than 10g/d. Hypoalbuminemia is closely related with amounts of protein lost.
　　4. Hyperlipidemia
　　Patients with MCN may appear lipid metabolic disorder, plasma cholesterol and triacylglycerol obvious increasing, serum showing chyle color. The patients with hyperlipidemia usually have lipid urine.
　　5. Blood Pressure Change
　　The patients with obvious hypoalbuminemia and effective circulating blood volume decrease may appear orthostatic hypotension, pulselessness and so on. Meanwhile, some patients may appear temporary hypertension.
　　6. Hematuria (blood in the urine)
　　About 20% of the patients may appear microscopic hematuria.
　　7. Kidney Function Change.
　　Most of the patients have a normal kidney function. Less of them may accompany with Prerenal Azotemia due to obvious edema. Some individual patients may appear serious Acute Kidney Failure without obvious inducements. Such disease is called Idiopathic Acute Kidney Failure.
　　Diagnosis and Differentiation
　　Minimal Change Nephrosis (MCN) is the pathological diagnosis. MCN often has the typical clinical manifestation of Nephrotic Syndrome, that is: large volume of proteinuria (≥3.5g/d), Hypoproteinemia (≤30g/L), severe edema and hyperlipidemia. The pathological feature is: under the light microscope, the glomeruli are basically normal; fatty degeneration can be seen in the epithelial cells of proximal convoluted tubule; the immune pathological test is negative; under electronic microscope, there are broad masses glomerular epithelial foot processes disappeared, and there is no electron dense deposition in the glomeruli are the main diagnosis basis .
　　For MCN patients, especially for adult patients, they must carefully detect and exclude the factors for secondary MCN, such as drugs, tumor, infection and allergy, which can only diagnose primary MCN.
　　To clarify the diagnosis of MCN must rely on the pathological diagnosis of renal biopsy. It is not difficult to make the diagnosis according to the clinical symptoms and laboratory examination (including renal pathology) result. For children Nephrotic Syndrome patients, they may not consider to do renal biopsy at first. They may receive the glucocorticoid treatment regarding as MCN. If they are steroid-resistant and steroid-dependent, they need to do renal biopsy. For adult Nephrotic Syndrome patients, MCN is not their main pathological type, when they can’t make the diagnosis of MCN according to the clinical symptoms , it is necessary for them to do the renal biopsy.
　　Treatments
　　1. General Treatment
　　The patient at the period of large volume of proteinuria, they should mostly keep stay on the bed or do some proper activities around the bed so as to to prevent the deep venous thrombosis. Patients with obvious edema should have a low salt diet.
　　2. Diuretic Treatment
　　For patients with low response of glucocorticoid and refractory edema, they can use Thiazide for diuretics, the common drug is hydrochlorothiazide. If the effect is not so good, they can use together with Potassium- sparing diuretics, such as Spirolactone, Triamterene and so on, If it is still not good, it can change to Loop Diuretics.
　　3. Anticoagulant Therapy
　　Thrombus and embolism are the common complications of Nephrotic Syndrome. If the patient plasma albumin is < 20g/L, they need to give conventional anticoagulant (such as low molecular heparin subcutaneous injection or taking oral warfarin) and platelet aggregation drugs(dipyridamole or taking oral aspirin). If patients are conformed to have the complications of thrombus and embolism, they need to give anticoagulant treatment as soon as possible.
　　4 Acute Kidney Failure Treatment.
　　For less of MCN patients, their pathogeny of Acute Kidney Failure cannot be found, it can be called MCN complications of idiopathic Acute Kidney Failure. If patients with such disease can be treated correctly and in time, most of them can get complete or better recovery.
　　The treatment measures are as the followings:
　　① Loop diuretic: For patients with good response to loop diuretic should use a large dosage of loop diuretic so that it can break the blockage of tubular casts;
　　② Hemodialysis: If patients have no response for diuretic, and need dialysis, they should do dialysis to maintain the life, and to do some proper dehydration after complementing the plasma products in order to reduce the renal interstitial edema;
　　③ The primary disease treatment: For most patients their pathological type is MCN so they should be treated positively;
　　④ Alkalization of urine: Patients can take sodium bicarbonate alkaline by oral to alkalize the urine and reduce the formation of casts.
　　5 Special Treatment
　　(1) Glucocorticoid: Using the Glucocorticoid to treat MCN, most patients have a good effective and a quick respond. But with the increasing of the patient ages, the effect of Glucocorticoid has will be decreased. So the Glucocorticoid has effect in treating MCN but it is very easy to relapse. In order to reduce the relapsing rate, in China, usually we will use large dosage at the beginning and then gradually reduce the dosage and maintain the dosage for long term.
　　(2) Cyclophosphamide (CTX): The cyclophosphamide treatment may use oral taken or intravenous injection method. Such kind of medicine may induce the relapsing kidney disease to get a long time or complete relieving and reduce its relapse. The doctor should pay more attention to the side-effects of CTX and give the treatment these symptoms, such as total number white blood cells reducing, hair loss, gastrointestinal reaction, hemorrhagic cystitis, gonad damage and so on; And try to avoid the large dosage of CTX cause gonad toxicity, which may lead to the serious side-effects of male infertility and tumor.
　　(3) Cyclosporin A (CsA): The CsA treatment is suitable for patients of Hormone-dependence and Hormone-resistance.
　　(4) Other Immunosuppressors: There is the report of effective treatment of Mycophenloate Mofetil and Tacrolimus (FK506) for MCN.
　　6 The characteristic treatment in our hospital
　　In our hospital, for the treatment of MCN, besides using some conventional treatments, in order to improve the patient’s kidney function, adjust their immunity and blood circulation, the doctors will ask the patients receive some characteristic Traditional Chinese Medicine (TCM) Treatments, such as: TCM Physiotherapy and Osmotherapy. This is a series of internal and external treatments of Natural Immune Balance Therapy. It is a kind of natural treatment with no gastrointestinal absorption and metabolism of liver and kidneys, no pains and no trauma. Together with the clinical practice it has composed an integrative treatment, which has achieved the effects of curing kidney disease both internal and external, coordinating and co-compensating each other, consolidating the treatment effect.
　　For each patient, after the physical examination, the expert doctors will make up a treatment schedule for them according to their examination results and their disease condition, 10 days is one treatment course, commonly, the patient will receive 2 courses continuously treatment, for patient in special condition, their treatment may prolong or stop according to their illness condition.
　　Prognosis

　　Compared Minimal Change Nephrosis with other pathological types of Nephrotic Syndrome, it may have a good prognosis. More than 70% of children patients have no kidney damage and abnormal urine test after they entered adulthood; And more than 90% of adults patients may keep a normal kidney function for more than 10 years.