P1: SBT
0521779407-05 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 20:49
Autosomal Dominant PRD 207■creatinine clearance as a baseline to measure progression
■diagnosis is made by renal imaging: ultrasound, CT, MRI
■false positives rare
■false negative common through third decade (15–20%) with ultra-
sound
■CT and MRI more sensitivedifferential diagnosis
■simple cysts (tend to be single and unilateral)
■von Hippel Lindau syndrome – diagnosis by extrarenal manifesta-
tions; cerebellar tumors, pheochromocytoma
■tuberous sclerosis – diagnosis by extrarenal manifestations; ade-
noma sebaceummanagement
■establish baseline renal function and imaging (MRI or CT best) for
longitudinal follow-up
■introduce patient and family to Polycystic Kidney Research Founda-
tion (1.800.PKD.CURE)
■genetic counseling imperative for autosomal dominant disorder
■screen patients with family history of cerebral aneurysms or neuro-
logical symptoms with MRA or CTspecific therapy
■none available
■blood pressure management: goal is normal pressure for age. Some
theoretical considerations favor ACE inhibitors, angiotensin recep-
tor blockers. Few comparative studies available.
■nephrolithiasis: usually uric acid or calcium oxalate – manage as
usual
■lower urinary tract infection: treat as usual patient
■upper urinary tract infection – treat with antibiotics that are known to
penetrate cysts: trimethoprim-sulfamethoxazole, fluoroquinolones,
chloramphenicol; avoid instrumentation of the urinary tract
■pain & discomfort – non-narcotic analgesics preferably acetamino-
phen, avoid aspirin and NSAIDs (except for short courses with good
hydration), opiates as needed, rule-out obstruction and infection; if
pain becomes chronic consider multidisciplinary pain clinic, antide-
pressants, cyst aspiration/sclerosis, laparoscopic cyst decompres-
sion or open cyst reduction. Absolute contraindications for surgical
approaches are advanced renal insufficiency and inability to tolerate
general anesthesia.