Factors affecting the progression of renal disease in autosomal-dominant polycystic kidney disease

Patricia A. Gabow, Ann M. Johnson, William D. Kaehny, William J. Kimberling, Dennis C. Lezotte, Irene T. Duley, Richard H. Jones

Research output: Contribution to journalArticle

390 Citations (Scopus)

Abstract

Autosomal-dominant polycystic kidney disease results in renal failure at a varying age from childhood to old age. We postulated that factors other than the culprit gene alone contribute to the course of progression of the renal failure. We studied 580 subjects with autosomal-dominant polycystic kidney disease and 194 unaffected family members. We calculated survival curves to end-stage renal failure or death and developed a linear model for testing the effects of single or multiple variables on the progression of renal failure as estimated from the reciprocal of serum creatinine. Fifty-two subjects died and 94 reached end-stage renal failure during the period of observation, yielding functional survivals of 71% at age 50 years, 53% at 58 years and 23% at 70 years. The following variables were independently associated with worse mean renal function at a given age (P value less than 0.01): the PKD1 gene, younger age at diagnosis, male gender, hypertension, increased left ventricular mass, hepatic cysts in women, three or more pregnancies, gross hematuria, urinary tract infections in men and renal size expressed as renal volume. The following were not associated significantly with the course of renal function: gender of affected parent, mitral valve prolapse, intracranial aneurysms, any pregnancy, hepatic cysts in men and urinary tract infections in women. The identification of unalterable maleficent factors such as the PKD1 gene and male gender permit more informed counseling while the identification of alterable factors such as hypertension, number of pregnancies and recurrent urinary tract infections provides the clinician with the opportunity to modify these factors and improve the management of patients with autosomal-dominant polycystic kidney disease.

Original languageEnglish (US)
Pages (from-to)1311-1319
Number of pages9
JournalKidney International
Volume41
Issue number5
DOIs
StatePublished - May 1992

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Autosomal Dominant Polycystic Kidney
Disease Progression
Urinary Tract Infections
Renal Insufficiency
Kidney
Pregnancy
Chronic Kidney Failure
Cysts
Genes
Hypertension
Mitral Valve Prolapse
Survival
Liver
Intracranial Aneurysm
Hematuria
Counseling
Linear Models
Creatinine
Observation
Serum

ASJC Scopus subject areas

  • Nephrology

Cite this

Gabow, P. A., Johnson, A. M., Kaehny, W. D., Kimberling, W. J., Lezotte, D. C., Duley, I. T., & Jones, R. H. (1992). Factors affecting the progression of renal disease in autosomal-dominant polycystic kidney disease. Kidney International, 41(5), 1311-1319. https://doi.org/10.1038/ki.1992.195

Factors affecting the progression of renal disease in autosomal-dominant polycystic kidney disease. / Gabow, Patricia A.; Johnson, Ann M.; Kaehny, William D.; Kimberling, William J.; Lezotte, Dennis C.; Duley, Irene T.; Jones, Richard H.

In: Kidney International, Vol. 41, No. 5, 05.1992, p. 1311-1319.

Research output: Contribution to journalArticle

Gabow, PA, Johnson, AM, Kaehny, WD, Kimberling, WJ, Lezotte, DC, Duley, IT & Jones, RH 1992, 'Factors affecting the progression of renal disease in autosomal-dominant polycystic kidney disease', Kidney International, vol. 41, no. 5, pp. 1311-1319. https://doi.org/10.1038/ki.1992.195
Gabow PA, Johnson AM, Kaehny WD, Kimberling WJ, Lezotte DC, Duley IT et al. Factors affecting the progression of renal disease in autosomal-dominant polycystic kidney disease. Kidney International. 1992 May;41(5):1311-1319. https://doi.org/10.1038/ki.1992.195
Gabow, Patricia A. ; Johnson, Ann M. ; Kaehny, William D. ; Kimberling, William J. ; Lezotte, Dennis C. ; Duley, Irene T. ; Jones, Richard H. / Factors affecting the progression of renal disease in autosomal-dominant polycystic kidney disease. In: Kidney International. 1992 ; Vol. 41, No. 5. pp. 1311-1319.
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