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Polycystic kidney disease
Polycystic kidney disease (PKD) is an inherited disorder characterized by the development of fluid-filled cysts in the kidneys, which can lead to kidney damage and various complications such as high blood pressure, kidney failure, and pain. There are two types of PKD: autosomal dominant (ADPKD), which is more common, and autosomal recessive (ARPKD). Management focuses on controlling symptoms and preventing complications, as there is no cure for the cysts themselves.
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Polycystic kidney disease
- 1. POLYCYSTIC KIDNEY DISEASE PRESENTED BY-NEHA BHARTI NURSING TUTOR DPT:- MEDICAL SURGICAL NURSING SMVDCoN, KAKRYAL
- 2. • Polycystic kidneydisease (PKD) is an inherited disorder in which clusters of cysts develop primarily within kidneys, causing kidneys to enlarge and lose function over time. • Cysts are noncancerous round sacs containing fluid. The cysts vary in size, and they can grow very large. Having many cysts or large cysts can damage kidneys
- 3. DEFINITION • Polycystic kidneydisease (PKD) is an inherited disease that affects the kidneys. Sacs of fluid (called cysts) grow in the kidneys. If too many cysts grow or if they get too big, the kidneys become damaged. • The cysts may also cause pain or may get infected.
- 4. • In PKD,cysts, or fluid-filled pouches, are found primarily in the kidney but they can also affect other organs, including the liver, pancreas, spleen and ovary. • Out pouching may occur in the walls of the large intestine and in the walls of blood vessels in the brain, where they may cause aneurysms. • They may also be found in the abdominal wall, causing hernias. In addition, valves of the heart may be involved, becoming floppy and resulting in a heart murmur in some patients.
- 5. CAUSES • Abnormal genescause polycystic kidney disease, which means that in most cases, the disease runs in families. • Rarely, a genetic mutation occurs on its own (spontaneous), so that neither parent has a copy of the mutated gene.
- 6. TYPES POLYCYSTIC KIDNEY DISEASE •There are two major forms of polycystic kidney disease: AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY DISEASE
- 7. 1. AUTOSOMAL DOMINANTPOLYCYSTIC KIDNEY DISEASE •The adult type of PKD (also called autosomal dominant PKD or ADPKD) is passed from parent to child by an autosomal dominant type of inheritance. •This means that only one copy of the abnormal gene is needed to cause the disease. Therefore, if one parent has the disease, each child has a 50-50 chance of developing the disease.
- 8. • ADPKD isa common disorder, occurring in approximately 1 in every 400 to 1000 people. Only about one-half of people with ADPKD will be diagnosed during the persons lifetime because the disease is often without symptoms.
- 9. 2. AUTOSOMAL RECESSIVE POLYCYSTICKIDNEY DISEASE •The less common form of PKD (also called autosomal recessive PKD or ARPKD) is passed by an autosomal recessive pattern of inheritance. •This means that both parents must carry the abnormal gene, and both must pass the gene to the child in order for the Child to develop the disease.
- 10. • In thissituation, every child has a 25 percent chance of developing the disease in a family that is at risk. The estimated incidence is 1 in 10,000 to 20,000 people.
- 11. COMPLICATIONS HIGH BLOOD PRESSURE •Elevatedblood pressure is a common complication of polycystic kidney disease. •Untreated, high blood pressure can cause further damage to kidneys. LOSS OF KIDNEY FUNCTION •Progressive loss of kidney function is one of the most serious complications of polycystic kidney disease. Nearly half of those with the disease have kidney failure by age 60. •PKD can interfere with the ability of your kidneys to keep wastes from building to toxic levels, a condition called uremia. As the disease worsens, end-stage kidney (renal) failure may result.
- 12. CONTD… PREGNANCY COMPLICATIONS •Pregnancy issuccessful for most women with polycystic kidney disease. In some cases, however, women may develop a life- threatening disorder called preeclampsia. •Those most at risk have high blood pressure before they become pregnant. GROWTH OF CYSTS IN THE LIVER •The likelihood of developing liver cysts for someone with polycystic kidney disease increases with age. •While both men and women develop cysts, women often develop larger cysts. Female hormones might contribute to cyst development.
- 13. DEVELOPMENTOF AN ANEURYSM INTHE BRAIN •A balloon like bulge in a blood vessel (aneurysm) in brain can cause bleeding (haemorrhage) if it ruptures.
- 14. Chronic pain. • Painis a common symptom for people with polycystic kidney disease. It often occurs in your side or back. • The pain can also be associated with a urinary tract infection, a kidney stone or a malignancy. Colon problems. • Weaknesses and pouches or sacs in the wall of the colon may develop in people with polycystic kidney disease.
- 15. CLINICAL MANIFESTATIONS ABDOMINAL PAINOR TENDERNESS AN INCREASE IN THE SIZE OF THE ABDOMEN
- 16. BLOOD IN THEURINE FLANK PAIN ON ONE OR BOTH SIDES
- 17. Additional symptoms thatmay be associated with this disease include the following: •Drowsiness •High blood pressure •Painful menstruation
- 18. DIAGNOSTIC EVALUATIONS Ultrasound. • Duringan ultrasound, a wand like device called a transducer is placed on body. It emits sound waves that are reflected back to the transducer — like sonar. A computer translates the reflected sound waves into images of kidneys. CT scan. • As patient lie on a movable table and guided into a big, doughnut- shaped device that projects thin X-ray beams through patient body. Doctor is able to see cross-sectionalimages of kidneys. MRI scan. • As patient lie inside a large cylinder, magnetic fields and radio waves generate cross-sectionalviews of kidneys.
- 19. MANAGEMENT • No treatmentis available for the cysts caused by PKD. If the cysts are causing symptoms, these symptoms can be treated so patient will be more comfortable. • The goal of treatment is to control symptoms and prevent complications. Treating high blood pressure can have a dual benefit in people with polycystic kidney disease because it can slow the decline in kidney function and minimize the risk that a cerebral aneurysm will rupture.
- 20. High blood pressure. •Controlling high blood pressure can delay the progression of the disease and slow further kidney damage. Combining a low- sodium, low-fat diet that's moderate in protein and calorie content with not smoking, increasing exercise and reducing stress may help control high blood pressure. • However, medications are usually needed to control high blood pressure. • Medications called angiotensin-converting enzyme (ACE) inhibitors or angiotensin II receptor blockers (ARBs) are often used to control high blood pressure.
- 21. Pain. • Patient mightbe able to control the pain of polycystic kidney disease with over-the-counter medications containing acetaminophen.
- 22. • Any urinarytract infection should be treated promptly with appropriate antibiotics. Cysts that are painful, infected, bleeding or causing an obstruction may need to be drained. • Surgical removal of one or both kidneys may be required. • Treatment of end-stage kidney disease may include kidney dialysis or kidney transplantation.
- 23. Aneurysms. • If patienthave polycystic kidney disease and a family history of ruptured brain (intracranial) aneurysms, doctor may recommend regular screening for intracranial aneurysms. • If an aneurysm is discovered, surgical clipping of the aneurysm to reduce the risk of bleeding may be an option, depending on its size. • Nonsurgical treatment of small aneurysms may involve controlling high blood pressure and high blood cholesterol, as well as quitting smoking.
- 24.