Figure 918

Main clinical manifestations of autosomal-dominant polycystic kidney disease (ADPKD). Renal involvement may be totally asymptomatic at early stages. Arterial hypertension is the presenting clinical finding in about 20% of patients. Its frequency increases with age. Flank or abdominal pain is the presenting symptom in another 20%. The differential diagnosis of acute abdominal is detailed in Figure 9-22. Gross hematuria is most often due to bleeding into a cyst, and more rarely to stone. Renal infection, a frequent reason for hospital admission, can involve the upper collecting system, renal parenchyma or renal cyst. Diagnostic data are obtained by ultrasonography, excretory urography and CT: use of CT in cyst infection is described in Figure 9-21. Frequently, stones are radiolu-cent or faintly opaque, because of their uric acid content. The main determinants of progression of renal failure are the genetic form of the disease (see Fig. 9-19) and gender (more rapid progression in males). Hepatobiliary and intracranial manifestations are detailed in Figures 9-23 to 9-26. Pancreatic and arachnoid cysts are most usually asymptomatic. Spinal meningeal diverticula can cause postural headache. ESRD—end-stage renal disease.

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