Polycystic kidney disease natural history
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Aarti Narayan, M.B.B.S [2]
Overview
Natural History, Prognosis, and Complications
Renal Manifestations: Natural History, Prognosis, and Complications
- The earliest detectable functional aberration seen in patients with ADPKD is impaired concentrating capacity with a suboptimal increase in urinary osmolality following water deprivation.[1]
- The second early manifestation of disease is hypertension. Up to 75% of patients with ADPKD on imaging without any renal insufficiency are hypertensive.[2] This is likely to be linked to an increase in the activity of the renin-angiotensin-aldosterone system (RAAS). RAAS overactivity is due to the increase in renin release secondary to renal ischemia brought on by cystic expansion and stretching of renal arterioles. This is further emphasized by the fact that patients with ADPKD generally respond better to ACE inhibitors and angiotensin receptor blockers than patients with essential hypertension.[3]
- Overt clinical signs and symptoms of renal disease usually appear during the fourth or fifth decade.
- Approximately 60% of patients suffer from flank pain often requiring cyst decompression surgery. Expanding cysts can often by complicated by hemorrhage that self-resolves but usually causes significant pain.
- Urinary tract infections occur in up to 70% of patients particularly with gram negative bacteria. Ascending infection can lead to pyelonephritis or cyst infection which are often difficult to differentiate.
- Nephrolithiasis is also very common due to the distorted urinary system. Approximately 35% of patients will have some form or renal stones.
- Hematuria can be seen in ADPKD especially in advanced cases; however, overt proteinuria is usually uncommon in the context of ADPKD, and proteinuria > 1 g/day should prompt the consideration of a second disease process.
- Generally, PKD1 mutants have more severe renal disease with mean age at onset of ESRD around 50 years compared to 75 years in PKD2 mutants.[4] But, regardless of the mutation, 50% of ADPKD patients will reach ESRD by age 60 years.
- Several factors have been linked to a worse renal outcome (renal function for given age) including early age at diagnosis, male gender, uncontrolled hypertension, left ventricular hypertrophy, cystic liver, gross hematuria, larger kidney volume, and urinary tract infections.
Natural History of Extra-renal Manifestations
References
- ↑ Gabow PA, Kaehny WD, Johnson AM, Duley IT, Manco-Johnson M, Lezotte DC; et al. (1989). "The clinical utility of renal concentrating capacity in polycystic kidney disease". Kidney Int. 35 (2): 675–80. PMID 2709672.
- ↑ Gabow PA (1990). "Autosomal dominant polycystic kidney disease--more than a renal disease". Am J Kidney Dis. 16 (5): 403–13. PMID 2239929.
- ↑ Chapman AB, Johnson A, Gabow PA, Schrier RW (1990). "The renin-angiotensin-aldosterone system and autosomal dominant polycystic kidney disease". N Engl J Med. 323 (16): 1091–6. doi:10.1056/NEJM199010183231602. PMID 2215576.
- ↑ Hateboer N, v Dijk MA, Bogdanova N, Coto E, Saggar-Malik AK, San Millan JL; et al. (1999). "Comparison of phenotypes of polycystic kidney disease types 1 and 2. European PKD1-PKD2 Study Group". Lancet. 353 (9147): 103–7. PMID 10023895.