Introduction
Diffuse mesangial sclerosis is a rare kidney condition categorized as a type of nephrotic syndrome. This group of diseases damages small blood vessels in the kidneys and causes protein leakage into the urine. DMS is characterized by abnormal thickening of the glomerular basement membrane, which is responsible for filtering waste from the blood. The medical condition has several features, including its causes, symptoms, diagnosis, and treatment options which will be explained in this article.
What Are the Causes of Diffuse Mesangial Sclerosis?
The following are some of the known causes of DMS:
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Genetic mutations: DMS can be caused by genetic mutations in the genes that regulate the production and metabolism of ECM in the mesangial cells. These mutations can be inherited from one or both parents or can occur spontaneously.
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Congenital Anomalies of the Kidneys and Urinary Tract (CAKUT): Some cases of DMS have been linked to abnormalities in the development of the kidneys and urinary tract, such as vesicoureteral reflux (VUR) or obstructive uropathy.
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Infections: Certain viral infections like cytomegalovirus (CMV) have been associated with the development of DMS in some patients.
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Drug Exposure: Exposure to certain drugs, including cyclosporine and interferon-alpha, has been reported to cause DMS in some patients.
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Other Medical Conditions: DMS has also been linked with other medical conditions, such as hypertension, systemic lupus erythematosus (SLE), and diabetes mellitus.
What Are the Symptoms of Diffuse Mesangial Sclerosis?
The most common symptoms associated with diffuse mesangial sclerosis are as under:
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Nephrotic Syndrome: DMS often presents with nephrotic syndrome, which is characterized by swelling (edema), increased protein levels in the urine (proteinuria), reduced protein levels in the blood (hypoalbuminemia), and high levels of cholesterol in the blood (hyperlipidemia).
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Slow Growth and Development: Children with DMS may experience slow growth and development due to the condition's impact on the kidneys' ability to filter waste and excess fluids from the body.
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Failure to Thrive: In some cases, DMS may cause a child to experience failure to thrive, which is characterized by inadequate weight gain and growth.
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High Blood Pressure: DMS can cause high blood pressure (hypertension), which can lead to further complications if left untreated.
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Kidney Failure: In severe cases, DMS can lead to kidney failure, which can cause a range of symptoms, including fatigue, weakness, and shortness of breath.
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Other Symptoms: Other less common symptoms associated with DMS include nausea, vomiting, and decreased urine output.
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The Severity of Diffuse Mesangial Sclerosis: It is essential to note that the severity and presentation of DMS can vary widely between individuals. Some children may experience few or mild symptoms, while others may experience severe complications.
How to Diagnose Diffuse Mesangial Sclerosis?
Diagnosing diffuse mesangial sclerosis is a multi-step process that involves the following:
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Medical History: During the medical history, the doctor will ask about the patient's symptoms, family history, and any underlying medical conditions. The doctor will inquire about the duration and severity of the symptoms, such as proteinuria, edema, high blood pressure, and fatigue. A positive family history of kidney disease increases the risk of DMS.
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Physical Examination: A physical examination will be performed to check for signs of kidney disease, such as high blood pressure, edema, and enlarged kidneys. The overall health of the patient will be assessed and the doctor may look for any other signs of disease.
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Laboratory Tests: Several laboratory tests are ordered to evaluate kidney function and rule out other causes of kidney disease. Urinalysis is performed to examine the presence of protein, blood cells, and other substances that may indicate kidney damage. Blood tests are done to check creatinine and blood urea nitrogen (BUN) levels, which reflect kidney function. Genetic testing may be performed if DMS is suspected due to positive family history.
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Imaging Studies: Imaging studies are done to assess the structure and function of the kidneys. An ultrasound is one of the imaging techniques that produce images of the kidneys and helps detect any abnormalities, such as enlarged kidneys or cysts. CT or MRI scans are other imaging techniques that produce detailed images of the kidneys and can help detect any structural abnormalities, such as scarring or thickening of the glomeruli.
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Biopsy: A kidney biopsy is the definitive diagnostic test for DMS. In this procedure, a tiny sample of kidney tissue is for examination under a microscope. The biopsy can reveal the characteristic findings of diffuse mesangial sclerosis, including thickening and scarring of the glomeruli.
What Is the Treatment of Diffuse Mesangial Sclerosis?
Treatment of diffuse mesangial sclerosis typically involves a combination of medications, supportive care, lifestyle changes, and clinical trials. Here is a breakdown of the treatment options for DMS:
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Medications:
There is no particular medication that can cure DMS, certain drugs slow the spread of disease and manage the symptoms. Medications may include:
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Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) reduce blood pressure and proteinuria.
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Diuretics are used to eliminate extra fluid from the body.
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Immunosuppressants, such as corticosteroids and cyclosporine, reduce inflammation and can lead to decrease progression of the disease.
2. Supportive care:
People with DMS may require supportive care to manage the complications of the disease. Supportive care may include:
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Dialysis or kidney transplant to replace the damaged kidney function.
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Nutritional support to maintain adequate nutrition.
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Blood pressure control to prevent further damage to the kidneys.
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Monitoring of electrolyte levels to ensure proper balance.
3. Lifestyle changes:
Lifestyle changes can also help manage DMS and improve overall health. These changes may include:
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Following a healthy diet that is low in sodium and protein reduces the workload on the kidneys.
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Exercising regularly to improve cardiovascular health and maintain a healthy weight.
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The risk of further kidney damage can be possible by quitting smoking and by reducing alcohol consumption.
4. Clinical trials:
Various ongoing clinical trials are investigating new treatments for DMS. Some people may participate in a clinical trial for DMS who have not responded to standard treatments. Clinical trials can provide access to new medications or treatments that may be more effective than current options.
Conclusion
DMS is an uncommon health condition that impacts the kidneys and is distinguished by the anomalous thickening of the glomerular basement membrane. It belongs to a category of nephrotic syndromes which may harm the tiny blood vessels in the kidneys and lead to protein leakage into the urine. Although there is no definitive cure for DMS, taking measures to manage the symptoms and decelerate the disease's advancement can be helpful. Prompt identification and treatment are critical to enhance the overall prognosis for those affected by DMS.
