Monitoring

Early detection and treatment of complications of autosomal-dominant PKD (ADPKD) are likely to improve quality of life and life expectancy in patients affected by ADPKD. Particular attention should be paid to cardiovascular complications, which are the most common cause of morbidity and mortality in ADPKD.

Patients with hypertension should be followed up every 6 to 12 months to ensure BP is controlled to target of <130/80 mmHg. BP monitor should be checked against office BP monitor annually. Patients with early or mild disease without hypertension are followed up every 1 to 3 years: instructions regarding BP monitoring are provided; a healthy diet is recommended, including advice on avoidance of excessive salt or protein; cardiovascular risk factors such as hyperlipidemia are corrected; and lifestyle modifications, if appropriate, such as smoking cessation, are discussed. Close monitoring of renal function is recommended, as antihypertensive doses may need to be adjusted if renal function declines.

Frequency of follow-up of patients should be adjusted to their comorbidities. If metabolically active nephrolithiasis (i.e., actively forming stones) is present, these patients need to be followed up every 6 to 12 months to monitor their metabolic activity. CT imaging of the kidneys may be indicated.

The patient should be referred to a nephrologist if renal function is impaired to monitor closely the rate of renal function decline, adjust diet and antihypertensive medications, prepare for renal replacement therapy, and manage metabolic abnormalities associated with chronic kidney disease progression. Erythropoiesis-stimulating agents should be started to maintain target hemoglobin of 10 to 11 g/dL.

Pain control issues may require more frequent visits and adequate analgesia should be provided. Given the high prevalence of depression, and its significant impact on morbidity and mortality, screening should be considered.

Early referral to a nephrologist is likely to lead to an improvement in GFR [114] and longer duration of regular nephrology care in non-ESRD period is associated with decreased hospitalization and better long-term survival once commenced on dialysis. [115] [116]

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