Introduction:
Most kidneys do a good job of cleansing the blood and maintaining the proper balance of the body's fluids and electrolytes. However, kidney arteries may have the potential to narrow or obstruct. This could severely harm this crucial filtration system. It may restrict the kidneys' ability to receive blood. Renovascular disorders affect the arteries that supply the kidneys. These diseases may result in renal failure or high blood pressure.
What Is Glomerular Thrombotic Microangiopathy?
Thrombotic microangiopathies (TMA) are clinical disorders characterized by hemolytic anemia (destruction of red blood cells), low platelets, and organ damage brought on by the development of minute blood clots in capillaries and small arteries. Although practically any organ may be involved, the kidneys are frequently affected. In other cases, smoldering thrombotic microangiopathy might cause kidney injury without significant anemia or low platelets. Schistocytes, or fragmented red blood cells, are visible in the blood when viewed under a microscope. Atypical hemolytic uremic syndrome, a kidney disease that can be severe and result in over 50 percent of people needing dialysis, is one of the causes of thrombotic microangiopathy. The term "TMA" originally stood for "thrombotic thrombocytopenic purpura or hemolytic uremic syndrome," TTP or HUS. It is now understood that TMA can be caused by a wide range of other disorders.
How Does Thrombotic Microangiopathy Affect the Kidneys?
A group of capillaries, also known as glomerulus, which are tiny blood vessels, are present in a healthy kidney as well as the rest of the body. They are covered with a slippery layer of cells called endothelial cells. When the capillaries get injured, the blood flow through the kidney get reduced. The liquid component of blood called plasma, assists in the transport of waste to the kidney from the body to be removed through urine. The blood also contains solid components, such as platelets and red blood cells. Red blood cells transport oxygen from the lungs to the rest of the body, including the kidney cells. Platelets have the responsibility of sealing off any damaged areas in blood vessels to prevent it from leaking. Defects in the blood vessel wall lining may result in the formation of rough patches which are similar to patch holes on a road that can slow down traffic and cause a lot of damage. Red blood cells have the potential to expand and then rupture. Eventually, malformed red blood cells may explode. Clot formation may result from platelet activation. All of these events may cause blood vessels to shut down entirely. Finally the kidney may stop functioning due to poor blood flow, and the body may become deficient of platelets and red blood cells.
What Are the Signs and Symptoms of Glomerular Thrombotic Microangiopathy?
The signs and symptoms of glomerular thrombotic microangiopathy include:
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Decreased urine.
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Blood in the urine.
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Swollen legs.
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High blood pressure.
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Tiredness.
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Vomiting.
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Dizziness.
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Shortness of breath.
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Bruises.
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Gum or nose bleeds.
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Minor cuts bleed a lot.
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Fever.
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Abdominal pain.
Who Is at Risk for Glomerular Thrombotic MIcroangiopathy?
The risk factor for glomerular thrombotic microangiopathy are:
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Aging.
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High blood pressure.
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High cholesterol.
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Lack of exercise.
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Smoking and other tobacco use.
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A family history of early heart disease.
How to Diagnose Glomerular Thrombotic Microangiopathy?
The doctor may review the medical history and do a physical examination. Other test includes:
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Blood Examination - The doctor may test the blood to check the total blood count of red blood cells and platelets and also examine the blood smear under the microscope to check whether any red blood cells are damaged known as schistocytes.
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Duplex Ultrasound - The purpose of this test is to examine the veins and arteries that supply the kidneys and blood flow. The term duplex means the combination of two different types of ultrasounds are used. First type of ultrasound is used to capture images of the kidney for the examination.In the second type is to examine the blood flow.
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Arteriogram - This is an X-ray test used to capture the images for checking the narrowing or blockage of blood vessels. A skinny, flexible tube is inserted into an artery, and a dye (contrast) is placed through it. On an X-ray, this dye helps to show the blood vessels.
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Renography - This test is used to examine the structure and function of the kidney. It is a specific kind of nuclear radiology procedure. This means that a little quantity of a radioactive substance is utilized throughout the test to aid the visualization of the kidneys.
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Magnetic Resonance Angiography (MRA) - This test combines intravenous (IV) contrast dye with magnetic resonance imaging (MRI) technology to examine the blood vessels. On the magnetic resonance image, the contrast dye makes blood arteries appear solid. This makes the blood vessels visible to the healthcare practitioner.
How to Treat Glomerular Thrombotic Microangiopathy ?
Depend on the severity of the underlying condition and avoid further organ damage, it may require immediate treatment,the treatment includes:
Plasma Exchange - Therapeutic plasma exchange is a common form of treatment in which the body's plasma (the water and protein component of blood) is removed and replaced with new donor plasma. Patients typically get a combination of plasma exchange, immunological suppression, and corticosteroids as part of this effective therapy for thrombocytopenic purpura. The steps of plasma exchange include:
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One of the veins receives a big IV (intravenous) catheter (usually in the neck or groin).
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Afterward, a plasma exchange device receives the blood from that catheter via tubing.
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A blood donor's plasma is used to replace the plasma (liquid) portion of the blood after it has been taken.
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The body receives the treated blood through a catheter before reentering it.
This procedure makes it possible to exchange the abnormal Von William factors that cause thrombocytopenic purpura to healthy Von William factors. This procedure is carried out every one to two days for around two weeks, treatments typically last a few hours each. It is being investigated whether the use of the drug Rituximab can stop thrombocytopenic purpura relapses in people who have experienced the condition on multiple occasions. While HUS typically gets better on its own. When the disease is getting worse, patients will need to be hospitalized for monitoring and hydration. Manual blood purification with dialysis may be required if renal function deteriorates too significantly. The toxin is eliminated from the body when the underlying bacterial infection disappears, and the signs and symptoms of HUS start to get better. The best course of action is still not defined for many of the defect TTP or HUS disease patterns. Although there is some dispute right now, they are frequently treated (like TTP) with plasma exchange.
Conclusion:
The damage to the blood vessels in the kidneys will usually repair on its own over time. HUS has a promising outlook. Kidney failure can frequently become severe enough during the disease's active phase to require manual blood purification with dialysis. Fortunately, it normally only lasts a short while. Actually, after a few months, kidney function almost always recovers to normal.
