HPE HPE 85 – Spring 2017 - Page 60

Practical therapeutics with a positive family history, clear imaging criteria based upon the number of cysts per kidney in an age-dependent fashion have been formulated and validated. 3,4 Renal ultrasound is generally sufficient to make the diagnosis in these patients. In addition to the number of cysts detected, attention should be paid to the enlargement of the kidneys and ubiquitous localisation of the cysts throughout the parenchyma that would be expected in ADPKD (Figure 1). Furthermore, extra-renal symptoms can help to distinguish ADPKD from other cystic kidney disease entities such as nephronophthisis or simple kidney cysts. 5 Therapy Until the TEMPO 3:4 trial, only supportive measures were available to influence the course of disease in ADPKD patients. These include salt restriction (<5–7g per day) and a sufficient fluid intake of above 3l per day. Furthermore, women taking hormone preparations (such as oral contraceptives) should be prescribed with formulations containing no (or low) oestrogen content. 1,5 Oestrogens drive the growth of the liver, which can be a significant problem especially in young female patients. Nephrotoxic substances – for example NSAIDs – should be avoided and a healthy lifestyle (for example, Mediterranean diet, physical activity and refraining from smoking) should be adopted to lower the risk of cardiovascular disease resulting from chronic kidney disease and early onset of arterial hypertension. 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