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Polycystic kidney disease (PKD) may be autosomal dominant or autosomal recessive, depending on the genetic pattern. The complications are similar in many respects, but the time of onset, the severity and the associated risks are different.
The complications of autosomal dominant PKD include:
Most patients with AKPKD complain of pain at some point, and it is often the presenting symptom. Possible causes of acute pain may be a hemorrhage into a cyst or renal calculi with or without obstruction.Chronic pain occurse more often due to cyst infection, urinary tract infections or cyst growth causing stretching of the renal capsule.
Fever may present in some patients due to acute infection (acute pyelonephritis) or retroperitoneal rupture of a cyst.
While hypertension complicates ADPKD in approximately half the cases at initial presentation, kidney function is usually clinically normal at this stage. However, once renal insufficiency and failure sets in, almost all patients develop severe hypertension. This may be marked by symptoms such as dizziness, vomiting, dyspnea, diplopia and headache. The risks of high blood pressure include:
Early occurrence of renal insufficiency may also occur due to:
The frequency of urinary tract infections is increased because of several factors, such as:
Complications of frequent UTIs include permanent fibrosis and further deterioration of renal function.
Renal calculi occur in 2 of every 10 people with ADPKD. The increased frequency of urate and oxalate stones may be due to reduced ammonia secretion in urine, increased protein intake, low urinary pH or low levels of urinary citrate. These can cause symptoms such as:
Renal failure occurs when there is no longer sufficient intact renal tissue to support normal function. People with ADPKD go into renal failure around the age of 70 years, on average. End-stage renal disease is more common in males with ADPKD, for unknown causes.
The reasons for renal failure include:
An individual may notice the following symptoms:
Liver cysts are extremely common in ADPKD, but are generally asymptomatic.
Pancreatic cysts may also occur in AKPKD, and may result in pancreatitis.
Heart valve abnormalities are found in up to a fourth of people with ADPKD, including mitral valve prolapse, aortic root insufficiency and aortic dissection. Symptoms of these may range from none to severely limited exercise tolerance with aortic root dilatation, to chest pain and back pain with dissection of the thoracic aorta.
Colonic diverticula may lead to colicky pain in the abdomen, characteristically left-sided, with bloating, or alterations in the passage of feces. At this stage the patient usually has end-stage renal disease. However, in most patients the condition is asymptomatic, though it may be complicated by hemorrhage into or infection of the diverticula.
Aneurysms, most often in the brain, but also in the aorta or the coronary vessels. A brain aneurysm may occur in only 4% of young patients, but up to a tenth of older patients. It may present with severe headaches, often with forceful vomiting and Cerebrovascular bleeding following aneurysm rupture, which usually happens by age 50 in up to 75% of such individuals. The risk of rupture is high if the following factors are present:
In the recessive form, polycystic kidney disease is far more severe and renal failure occurs early. The complications may be present at birth, and include:
Severe respiratory failure, due to pulmonary hypoplasia. This is because of abnormal pressure on the lungs by the enlarged kidneys, during intrauterine life. Simultaneously, pressure continuing after birth makes lung inflation difficult. 30% of these infants die within a year because of pulmonary complications, which include:
Failure to thrive, which may be due to several factors such as:
Renal failure in childhood, usually before the age of 20, which requires renal replacement therapy or a kidney transplant. Liver cirrhosis as a result of the development of multiple liver cysts, leading to:
Some patients may also be affected by hypertension, which further aggravates renal damage and frequent urinary tract infections.