Secondary prevention
Consultation for diagnosis with a genetic counsellor or nephrologist with expertise in PKD should be considered to provide counselling, diagnosis, and treatment recommendations.
Diet, smoking cessation, regular exercise, avoiding contact sport, and lipid and blood pressure control are all initial options. Vaccinations recommended include annual influenza and, in those with evidence of chronic kidney disease, pneumococcal vaccination.
Head magnetic resonance angiography should be done in patients with a family history of intracranial aneurysm or subarachnoid haemorrhage at baseline and every 5 to 10 years, or as needed.
Low sodium, restricted protein, and a low-cholesterol diet should be reviewed yearly. Patients should avoid caffeine (minimise daily caffeinated beverage intake and avoid caffeine-containing foods or related drugs, such as theophylline) as it increases levels of cyclic adenosine monophosphate (cAMP) in vitro, which has been shown to exacerbate cyst enlargement.[122]
Patients should be asked about chest pain and, if detected, an ECG, exercise testing, and echocardiogram, as appropriate, should be obtained.
Magnetic resonance imaging and computed tomography can detect small changes in kidney and cyst volumes over short periods of time.
Antenatal genetic diagnosis is usually not done for autosomal-dominant PKD, as this is not a fatal disease and women do not usually consider termination of pregnancy. Few centres offer this service.
Widespread pre-symptomatic screening for intracranial aneurysms is not justified. Screening is indicated if there is a family history of aneurysm or subarachnoid haemorrhage, if there is previous aneurysm rupture, if the patient is going for elective surgery (such as kidney transplant), if the patient is in a high-risk occupation (e.g., pilots, crane operators), and for patients with extreme anxiety despite adequate information.[31][34]
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