Table 2.

Basic optimized management of adult patients with ADPKD

InterventionGoalMethods to Achieve GoalEvidencea
Intensive  BP control≤110/75 mm Hg in:Early detection is essentialbGrade 1B
1. 18–50-year-oldsBy order of preference:
2. eGFR>60 ml/min per 1.73 m21. ACEI/ARB
3. Particularly:2. α/β or cardioselective β-blocker
 Mayo Clinic class 1 C–E3. Dihydropyridine CCB
 Intracranial aneurysm4. Diuretic
 Valvular heart diseaseDietary Approaches to Stop Hypertension (DASH)-like diet at early stages
≤130/85 mm Hg in:
1. Other adult hypertensives
SodiumModerate restriction (2.3–3 g/d)CounselingGrade 1C
Adjust for extrarenal losses (hot climate, runners, sauna, bowel disease) if appropriateDietitian follow-up
Monitor 24-h urine sodium
HydrationModerately enhanced hydration spread out over 24 h (during the day, at bedtime, and at night if waking up)CounselingGrade 1C
Maintain urine osmality ≤280 mOsm/kgMonitor first morning urine osmality, plasma copeptin if available
Graphic
Protein0.8–1.0 g/kg of ideal  body wtDietitianGrade 1C
Monitor protein intake:
6.25×(urine urea nitrogen in g/d+[0.03×weight in kilogram])
PhosphorusModerate diet  phosphate restriction  (800 mg/d)DieticianGrade 2C
Read food labels and watch for food additives containing phosphates
Use of phosphate binders not different from other advanced CKD when needed
Acid baseMaintain plasma  bicarbonate within  the normal range  (≥22 mEq/L)Increase fruits/vegetables (2–4 cups/d)Grade 2B
Oral sodium bicarbonate if needed
Caloric intakeMaintain normal BMIDietitian follow-upGrade 1C
Moderation in caloric intakeRegular exercise
Lipid controlAim for serum  LDL ≤100 mg/dlDieticianGrade 2B
Regular exercise
Statin if needed (ezetimibe if intolerant to statin)
  • ADPKD, autosomal dominant polycystic kidney disease; ACEI, angiotensin-converting enzyme inhibitors; ARB, angiotensin receptor blockers; CCB, calcium channel blocker; BMI, body mass index.

  • a Grading of levels of evidence is provided in Supplemental Table 1.

  • b Screen children at risk every 3 years starting at age 5 years. Children with hypertension should be referred and managed by experts in pediatric hypertension.