0631 SER Kidney book D
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0631 SER Kidney book D 0631 SER Kidney book D Document Transcript

  • 48 THE KIDNEY 12. Cysts in the kidney and polycystic kidney disease A G U I D E F O R PAT I E N T S Cysts in the kidney and polycystic kidney disease Cysts in the kidneys Cysts are fluid-filled structures within or on the edge of the kidney. They are nearly always a coincidental finding on ultrasound, but occasionally they may cause pain and tenderness. Rarely, they may bleed, become infected or if very large, may cause pressure on the surrounding organs such as the large intestine. If a cyst is detected an expert ultrasound review should be made to determine the shape, contents and wall structure of the cyst which might indicate it is not a simple cyst but something more serious such as an inherited cystic disorder of the kidneys or, rarely, a cystic tumour of the kidney. In patients over 50 years of age, 50% have one or more simple cysts, which usually do not cause symptoms or long-term problems. Polycystic kidney disease Autosomal dominant polycystic kidney disease (ADPKD) ADPKD is an inherited cystic disorder of the kidney in which multiple cysts form in the kidneys and progressively increase in size, compressing adjacent kidney tissue. When severe, this may ultimately lead to end stage kidney failure. Normal Kidney Who is at risk of developing ADPKD? In this condition, half of the children born to people with ADPKD will carry the gene, some of whom will eventually develop kidney failure. Recent discoveries now allow us to identify in advance who carries the gene, however no way has yet been devised to predict who will develop end-stage kidney failure. For those offspring who do develop kidney failure, it occurs at an average age of 50–60 years, but it may happen much earlier or much later. In about 10% of affected families the disease occurs later and most of this group do not develop kidney failure. For the few that do, dialysis is required at about 70 years of age. Another small group of people develop ADPKD without anyone in their family ever having suffered from the condition.
  • 49 Y In patients over 50 years of age, 50% have one or more simple cysts, which usually do not cause symptoms or long-term problems. Cysts Polycystic kidney disease In this inherited disease, cysts damage the kidneys resulting in blood in the urine and frequent kidney infections. Kidney failure and high blood pressure may also occur.
  • 50 A G U I D E F O R PAT I E N T S Polycystic kidney disease continued... The earliest sign Can ADPKD be diagnosed before birth? of ADPKD is high There is potential for antenatal screening for ADPKD as the location of blood pressure the gene is now known. Absense of a group of recently identified proteins named polycystins, leads to the development of multiple cysts throughout (hypertension), the kidneys. but blood in the How is ADPKD usually diagnosed? Children born to people known to have ADPKD should be screened with urine (haematuria), an ultrasound scan at about 18-20 years of age. If the ultrasound scan is pain over the kidneys normal there is a less than 5% chance of that person developing the disease. Later in life if the ultrasound scan is again normal at 30 years of age, there is (pain in the back a less than 1% chance of that person subsequently developing the disease. just below the ribs) In the event of the offspring of a person with ADPKD not having an ultrasound scan at the usual age, it is particularly important that it be and/or a feeling performed before starting a family in order for prenatal genetic counselling of fullness in the to be undertaken. The potential carrier of the condition should inform their partner of the risks of their offspring being affected. abdomen may occur. What are the features of ADPKD? The earliest sign of ADPKD is high blood pressure (hypertension), but blood in the urine (haematuria), pain over the kidneys (pain in the back just below the ribs) and/or a feeling of fullness in the abdomen may occur. In the event of a cyst becoming infected, the patient may feel generally unwell and experience pain and a high fever. Sometimes, in older people affected by this condition, the first indication they are affected is when they develop signs of uraemia (an excess of urea in the blood) (see also section 17). There are effects of ADPKD, which occur outside the kidneys. These include intracranial aneurysms (abnormalities of arteries in the brain which predispose stroke), abnormalities of the heart valves and diverticulosis of the bowel (abnormal out-pouchings of the large intestine). Evidence of intracranial aneurysms should be sought if there is a family history of ADPKD and stroke or ruptured aneurysm. They occur in about 5% of people with ADPKD, but do not cause problems in the majority. People with ADPKD may also develop cysts in their livers (especially women) and less often in the pancreas, ovaries and lungs.
  • 51 The kidneys may grow to occupy most of the abdomen and occasionally need to be removed if a dialysis patient with ADPKD is to receive a renal For people in whom transplant. Surgery is otherwise not helpful. There is no increased risk of kidney cancer in ADPKD. the diagnosis is What treatment is available for people with ADPKD? confirmed, high blood For people in whom the diagnosis is confirmed, high blood pressure should pressure should be be treated vigorously (see also section 11). This has been shown to slow the rate of deterioration of the function of the kidneys. It is important to treated vigorously. rapidly treat other factors which have the potential to further damage the This has been shown kidneys such as urinary tract infections and kidney stones. to slow the rate of Many medications have the potential to cause harm to the kidneys and these need to be avoided in people with ADPKD. Hence, it is important deterioration of the to always confer with a doctor or kidney specialist before taking tablets or alternative therapies. These measures, though they may not prevent function of the kidneys. the ultimate failure of the kidneys, may at least delay the need for renal It is important to replacement therapy (see also section 19). rapidly treat other factors which have the potential to further damage the kidneys such as urinary tract infections and kidney stones.
  • 52 A G U I D E F O R PAT I E N T S Cysts continued... Autosomal recessive PKD This condition occurs in infants and young children. It is associated with scarring in the liver (hepatic fibrosis) and has a poor prognosis. Many die from renal failure in the first 12 months of life and the survivors may develop liver failure from the age of 5-10 years. Other cystic lesions in the kidney These are defined by their localisation in the kidney, (eg. parapelvic cysts impact on the pelvis of the kidney) or by their cause - for example, hydatid cysts - (relatively rare), tuberculous cavities/cysts and carcinoma where the central neoplasm (tumour) has become necrotic and hence may appear cystic. Medullary cystic disease This (juvenile nephronophthisis) is a rare condition causing renal failure in adolescents, who are often blonde or red-haired. It is associated with short stature, bone disease (renal rickets), and salt wasting from the kidneys. The blood pressure is usually normal even with advanced kidney failure. It is sometimes associated with eye problems (Senior syndrome). Medullary sponge kidney This is relatively common and leads to small, often multiple stones in the kidneys, which may be complicated by urinary infection. It rarely causes high blood pressure or impairment of kidney function and is generally considered to be a relatively harmless condition.