Internal Medicine

P1: SBT

0521779407-05 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 20:49

208 Autosomal Dominant PRD Autosomal Recessive PRD

■hepatic involvement – pain management issues are the same as with

renal pain. Aspiration/sclerosis if there are a few dominant cysts.

follow-up

Routine

■annual assessment of renal function, blood pressure control and

imaging

■as needed to manage complications

complications and prognosis

■50% of patients progress to end-stage renal disease.

■risk factors for progression – male sex, gross hematuria, hypertension

before age 35, total renal volume >500 mL on imaging, women with

>3 pregnancies and 24-h urine protein >500 mg

Autosomal Recessive Polycystic Renal Disease...............

WILLIAM M. BENNETT, MD

history & physical

■Parents are unaffected; there may be disease in a sibling, usually

presents in childhood with hypertension, renal dysfunction, failure

to thrive

■associated liver fibrosis, more prominent in older children

■no specific physical findings

tests

■urinalysis

■serum electrolytes

■uric acid

■BUN

■creatinine

■creatinine clearance as baseline to measure progression

■diagnosis is made by renal imaging: ultrasound, CT, MRI

■best imaging modality unsettled, probably ultrasound especially in

young children

differential diagnosis

■renal dysplasias

■obstructive uropathy

■ADPKD early onset