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Polycystic Kidney Disease

Polycystic kidney disease (PKD) is an inherited disorder that causes fluid-filled cysts to form in the kidneys, leading to impaired kidney function and potential kidney failure. Symptoms can include abdominal pain, blood in urine, frequent urination, back pain, and more.

Polycystic kidney disease (PKD) is an inherited kidney disorder. It causes fluid-filled cysts to form in the kidneys. PKD may impair kidney function and eventually cause kidney failure.

PKD is the fourth leading cause of kidney failure. People with PKD may also develop cysts in the liver and other complications.

Many people live with PKD for years without experiencing symptoms associated with the disease. Cysts typically grow 0.5 inches or larger before a person starts noticing symptoms. Initial symptoms associated with PKD can include:

  • pain or tenderness in the abdomen
  • blood in the urine
  • frequent urination
  • pain in the sides
  • urinary tract infection (UTI)
  • kidney stones
  • pain or heaviness in the back
  • skin that bruises easily
  • pale skin color
  • fatigue
  • joint pain
  • nail abnormalities
  • Children with autosomal recessive PKD may have symptoms that include:

  • high blood pressure
  • UTI
  • frequent urination
  • Symptoms in children may resemble other disorders. It's important to get medical attention for a child experiencing any of the symptoms listed above.

    PKD is generally inherited. Less commonly, it develops in people who have other serious kidney problems. There are three types of PKD.

    Autosomal dominant PKD

    Autosomal dominant (ADPKD) is sometimes called adult PKD. According to the National Kidney Foundation, it accounts for about 90 percent of cases. Someone who has a parent with PKD has a 50 percent chance of developing this condition.

    Symptoms usually develop later in life, between the ages of 30 and 40. However, some people begin to experience symptoms in childhood.

    Autosomal recessive PKD

    Autosomal recessive PKD (ARPKD) is much less common than ADPKD. It's also inherited, but both parents must carry the gene for the disease.

    People who are carriers of ARPKD won't have symptoms if they have only one gene. If they inherit two genes, one from each parent, they'll have ARPKD.

    There are four types of ARPKD:

  • Perinatal form is present at birth.
  • Neonatal form occurs within the first month of life.
  • Infantile form occurs when the child is 3 to 12 months old.
  • Juvenile form occurs after the child is 1 year old.
  • Acquired cystic kidney disease

    Acquired cystic kidney disease (ACKD) isn't inherited. It usually occurs later in life.

    ACKD usually develops in people who already have other kidney problems. It's more common in people who have kidney failure or are on dialysis.

    Because ADPKD and ARPKD are inherited, your doctor will review your family history. They may initially order a complete blood count to look for anemia or signs of infection and a urinalysis to look for blood, bacteria, or protein in your urine.

    To diagnose all three types of PKD, your doctor may use imaging tests to look for cysts of the kidney, liver, and other organs. Imaging tests used to diagnose PKD include:

  • Abdominal ultrasound. This noninvasive test uses sound waves to look at your kidneys for cysts.
  • Abdominal CT scan. This test can detect smaller cysts in the kidneys.
  • Abdominal MRI scan. This MRI uses strong magnets to image your body to visualize kidney structure and look for cysts.
  • Intravenous pyelogram. This test uses a dye to make your blood vessels show up more clearly on an X-ray.
  • In addition to the symptoms generally experienced with PKD, there may be complications as cysts on the kidneys grow larger.

    These complications may include:

  • weakened areas in the walls of arteries, known as aortic or brain aneurysms
  • cysts on and in the liver
  • cysts in the pancreas and testicles
  • diverticula, or pouches or pockets in the wall of the colon
  • cataracts or blindness
  • liver disease
  • mitral valve prolapse
  • anemia, or insufficient red blood cells
  • bleeding or bursting of cysts
  • high blood pressure
  • liver failure
  • kidney stones
  • heart disease
  • The goal of PKD treatment is to manage symptoms and avoid complications. Controlling high blood pressure is the most important part of treatment.

    Some treatment options may include:

  • pain medication, except ibuprofen (Advil), which isn't recommended since it may worsen kidney disease
  • blood pressure medication
  • antibiotics to treat UTIs
  • a low-sodium diet
  • diuretics to help remove excess fluid from the body
  • surgery to drain cysts and help relieve discomfort
  • In 2018, the Food and Drug Administration approved a drug called tolvaptan (brand name Jynarque) as a treatment for ADPKD. It's used to slow the progression of kidney decline.

    One of the serious potential side effects of tolvaptan is severe liver damage, so your doctor will regularly monitor the health of your liver and kidneys while on this medication.

    With advanced PKD that causes renal failure, dialysis and kidney transplant may be necessary. One or both of the kidneys may need to be removed.

    A diagnosis of PKD may mean changes and considerations for you and your family. You may experience a range of emotions when you receive a PKD diagnosis and as you adjust to living with the condition.

    Reaching out to a support network of family and friends can be helpful.

    You may also wish to reach out to a dietitian. They can recommend dietary steps to help you keep blood pressure low and reduce the work required of the kidneys, which must filter and balance electrolytes and sodium levels.

    There are several organizations that provide support and information for people living with PKD:

    You can also talk to your nephrologist or local dialysis clinic to find support groups in your area. You don't have to be on dialysis in order to access these resources.

    If you aren't ready or don't have the time to attend a support group, each of these organizations have online resources and forums available.

    Reproductive support

    Because PKD can be an inherited condition, your doctor may recommend seeing a genetic counselor. They can help you figure out a map of your family's medical history with respect to PKD.

    Genetic counseling may be an option that can help you weigh important decisions, such as the likelihood your child could have PKD.

    Kidney failure and transplant options

    One of the most serious complications of PKD is kidney failure. This is when the kidneys are no longer able to:

  • filter waste products
  • maintain fluid balance
  • maintain blood pressure
  • When this occurs, your doctor will discuss options with you that may include a kidney transplant or dialysis treatments to act as artificial kidneys.

    If your doctor does place you on a kidney transplant list, there are several factors that determine your placement. These include your overall health, expected survival, and time you have been on dialysis.

    It's also possible that a friend or relative could donate a kidney to you. Because people can live with only one kidney with relatively few complications, this can be an option for families who have a willing donor.

    The decision to undergo a kidney transplant or donate a kidney to a person with kidney disease can be a difficult one. Speaking to your nephrologist can help you weigh your options. You can also ask what medications and treatments can help you live as well as possible in the meantime.

    According to the University of Iowa, the average kidney transplant will allow kidney function from 10 to 12 years.

    For most people, PKD slowly gets worse over time. It's estimated that 50 percent of people with PKD will experience kidney failure by age 60, according to the National Kidney Foundation.

    This number increases to 60 percent by age 70. Because the kidneys are such important organs, their failure may start to affect other organs, such as the liver.

    Proper medical care can help you manage PKD symptoms for years. If you don't have other medical conditions, you may be a good candidate for a kidney transplant.

    Also, you may consider speaking with a genetic counselor if you have a family history of PKD and are planning to have children.


    Dr. Haqqani: Controlling Blood Pressure Can Reduce Risk Of Chronic Kidney Disease

    Dr. Omar P. Haqqani

    The National Kidney Foundation (NKF) reports that about 15% of Americans, or 37 million people, have chronic kidney disease (CKD). NKF further states that hypertension (high blood pressure) is second only to diabetes in the cause of kidney failure. About 20% of those with hypertension may have CKD.

    According to the Centers for Disease Control and Prevention (CDC), 90% of those with CKD don't know they have it. It also affects 38% of those ages 65 and over, 12% of those ages 45-64, and 6% of individuals ages 18-44.

    Recently, the U.S. Food and Drug Administration (FDA) highlighted the correlation between hypertension and chronic kidney disease on its website, urging Americans to control their blood pressure in order to reduce their chances for CKD.

    Hypertension and kidney function

    One of the functions of the kidneys is to eliminate waste, extra fluids and acids from the body. Additionally, the kidneys maintain the proper balance for certain nutrients and minerals, including sodium, potassium, calcium and phosphorus.

    The National Institute of Diabetes and Digestive and Kidney Diseases states that the correct balance of these minerals helps to ensure the efficient operation of muscles, nerves and certain tissues found within the body. Among other kidney functions is the creation of hormones that make red blood cells and help keep bones strong.

    The kidneys also create hormones that regulate blood pressure. Because of that, hypertension is both a cause and an effect of an imbalance in kidney function. While high blood pressure can cause kidneys to operate improperly, if another condition causes an imbalance in the kidneys, high blood pressure can be the result.

    Risk factors for kidney disease and hypertension

    Some of the risk factors for kidney disease and high blood pressure are common to both. While those with hypertension are considered more likely candidates for chronic kidney disease, conditions that contribute to both include obesity, diabetes and older age. For chronic kidney disease, its presence in family medical history, inherited kidney disorders and previous kidney damage may raise the probability.

    Some issues that increase blood pressure may have a negative impact on other parts of the body, including the overall cardiovascular system and the kidneys. They include lack of exercise, smoking, vaping or excessive alcohol use. The intake of too much salt or a deficient potassium level can also raise blood pressure. Stress, sleep apnea and pregnancy have the potential to create hypertension.

    Controlling, managing, preventing high blood pressure also help prevent CKD

    FDA has listed approved hypertension-controlling medication and lifestyle changes that reduce blood pressure and, as a result, lower CKD probability. Two approved types of medications are designed to keep blood vessels from narrowing to increase pressure. One is known as the "ACE" (angiotensin-converting enzyme) inhibitor and the other is "ARB," which stands for Angiotensin II receptor blockers.

    Calcium channel blockers help blood vessels relax while beta blockers allow the heart to beat more slowly, thereby decreasing the amount of blood being forced through the blood vessels at one time.  

    Certain lifestyle practices have proven useful in both management and prevention of high blood pressure. They include changing medications to equally effective ones that do not have hypertension as a side effect. Also, implementing weight management and obesity reduction to impact metabolic syndrome is very helpful.

    Switching to a different diet may also help, and a physician may recommend diet and nutritional counseling for both salt restriction and to impact dyslipidemia (cholesterol) as part of a metabolic syndrome.

    Because smoking, lack of exercise and stress are causes of hypertension, controlling them may require a physician's advice, depending on present health conditions, weight and other factors. An exercise regimen suited to the individual can be formulated.

    Additionally, recommendations for a smoking cessation program may also be in order if a person has not had success in stopping tobacco use. Counseling may also be recommended in reducing stress. This may include assistance in identifying stressors in a patient's life and strategies to deal with them.

    The recommendations for prevention of high blood pressure are also helpful in CKD prevention. Also important is early detection for high-risk individuals. Those more likely to contract chronic kidney disease should be tested periodically.

    To learn more about a variety of health conditions, management and treatment, log on to vascularhealthclinics.Org.

    If you have questions about your cardiovascular health, including heart, blood pressure, stroke lifestyle and other issues, we want to answer them. Please submit your questions to Dr. Haqqani by e-mail at questions@vascularhealthclinics.Org .

    Omar P. Haqqani is the Chief of Vascular and Endovascular Surgery at Vascular Health Clinics in Midland.


    High Blood Pressure And Kidney Disease

    High blood pressure (hypertension) is a leading cause of kidney disease and kidney failure (end-stage renal disease). Kidney disease can also cause a type of high blood pressure called renal hypertension.

    Hypertension can cause damage to the blood vessels and filters in the kidney, making removal of waste from the body difficult. Once a person is diagnosed with end-stage renal disease, dialysis—a blood-cleansing process—or kidney transplantation is necessary.

    What Are the Symptoms of Kidney Disease?

    The symptoms of kidney disease include:

  • High/worsening blood pressure
  • Decrease in amount of urine or difficulty urinating
  • Edema (fluid retention), especially in the lower legs
  • A need to urinate more often, especially at night
  • How Is Kidney Disease Diagnosed?

    As with high blood pressure, you may not realize that you have kidney disease. Certain laboratory tests can indicate whether your kidneys are eliminating waste products properly. These tests include serum creatinine and blood urea nitrogen; elevated levels of either can indicate kidney damage. Proteinuria, an excess of protein in the urine, is also a sign of kidney disease.

    Who Is At Risk for Kidney Disease Owing to High Blood Pressure?

    Kidney disease caused by high blood pressure affects every group and race. However, certain groups are at higher risk, including:

  • African-Americans
  • Hispanic-Americans
  • Native Americans
  • Natives of Alaska
  • People who have diabetes
  • People with a family history of high blood pressure and kidney disease
  • How Can I Prevent Kidney Disease?

    To prevent kidney damage from high blood pressure:

  • Try to keep your blood pressure controlled.
  • Make sure you get your blood pressure checked on a regular basis.
  • Eat a proper diet.
  • Get moderate exercise, such as walking, 30 minutes daily.
  • Take the medication your doctor prescribes.
  • How Is Kidney Disease Treated?

    For patients who have high blood pressure and kidney disease, the most important treatment is to control their blood pressure through lifestyle changes. Drugs can lower blood pressure and protect the kidneys from further damage, especially in people with diabetes. However, treatments need to be individualized.

    The most important blood pressure medications to treat renal hypertension include:

    What Is Renal Hypertension?

    Although hypertension is a leading cause of kidney disease, there is a type of hypertension that is actually caused by kidney disease. Renal hypertension, also called renovascular hypertension, is elevated blood pressure caused by kidney disease. It can usually be controlled by the same blood pressure drugs. Some people with renal hypertension can be helped by angioplasty, stenting, or surgery on the blood vessels of the kidney.

    What Causes Renal Hypertension?

    Renal hypertension is caused by a narrowing in the arteries that deliver blood to the kidney. One or both kidneys' arteries may be narrowed. This condition is called renal artery stenosis.

    When the kidneys receive low blood flow, they act as if the low flow is owing to dehydration, so they respond by releasing hormones that stimulate the body to retain sodium and water. Blood vessels fill with additional fluid, and blood pressure goes up.

    The narrowing in one or both renal arteries is most often caused by atherosclerosis (hardening of the arteries). This is the same process that leads to many heart attacks and strokes. A less common cause of the narrowing is fibromuscular dysplasia. This is a condition in which the structure of the renal arteries develops abnormally for unclear reasons.

    What Are the Symptoms of Renal Hypertension?

    Renal hypertension usually causes no symptoms. The narrowing in the arteries can't be felt. Unless it's dangerously high, high blood pressure causes no symptoms either. Symptoms of severely elevated blood pressure include:

    The vast majority of people with renal hypertension never experience these (or any) symptoms. High blood pressure is dangerous, partly because there are no symptoms, so organ damage can occur slowly without being recognized.

    What Are the Treatments for Renal Hypertension?

    For most people with renal hypertension due to renal artery narrowing, medications can effectively control blood pressure. However, more than one blood pressure drug is often needed.

    In some people with renal hypertension due to narrowing of the renal artery, even taking three or more medications every day may not adequately control blood pressure. In these situations, a procedure to improve blood flow to the kidneys may help.

    Possible procedures include:

    Angioplasty. A doctor threads a catheter through a large artery in the groin and advances it into the renal artery. A balloon is then inflated for a few moments. This widens the artery and improves blood flow.

    Stenting. During angioplasty, a wire-mesh stent can be expanded inside the renal artery. The stent stays in place. This keeps the artery open after the balloon is removed. Research, however, has not shown that stenting is more effective than medication for most people with renal hypertension. 

    Surgery. A surgeon can bypass the narrowed renal artery by sewing a healthy blood vessel next to it. Surgery is generally considered only when angioplasty and stenting are not possible and is only rarely performed.

    These procedures are similar to those used to improve blood flow in the heart in people with coronary artery disease.






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