Demographic and Clinical Characteristics of Patients with Autosomal Dominant Polycystic Kidney Disease: A Multicenter Experience

Abstract

Aim: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. It accounts for 5–10% of patients with end-stage renal disease (ESRD). The aim of this multicenter study was to investigate the demographic and clinical characteristics of patients with ADPKD. Methods: 1,139 patients with ADPKD who were followed up at 12 different centers were recruited for this study. The investigated demographic and clinical characteristics were gender, age, smoking history, educational status, the existence of hypertension, hematuria, urinary tract infection, urinary tract stones and renal replacement therapy. Patients were considered as hypertensive if they were taking antihypertensive medications or if they had blood pressure (BP) of 140/90 mm Hg or greater. If the patients were currently on antihypertensive drugs, the classes of these agents were noted. Results: 548 male and 591 female patients were included and the mean age at initial diagnosis was 37.1 ± 16.3 years. 20.3% were current smokers whereas 15% were ex-smokers. The mean systolic and diastolic BPs were 136.1 ± 29.8 and 84.9 ± 17.8 mm Hg, respectively. 63.7% used antihypertensive drugs and 73.1% of those used renin-angiotensin system blockers. 11.8% had ESRD, of which 75.8% were treated with hemodialysis. Conclusion: This study showed that hypertension is the most common (72.6%) clinical finding in ADPKD patients in Turkey and renin-angiotensin system blockers are widely used.

Vascular endothelial growth factor as an angiogenesis biomarker for the progression of autosomal dominant polycystic kidney disease

“…Hypertension, described as the most common clinical manifestation of ADPKD (Paul and Vanden Heuvel, 2014), was observed in 79% of the patients, which was similar to the percentage (71%) reported by Taylor et al (2005). However, hypertension showed no relevance in the regression analysis, although it has been previously considered as the most common clinical manifestation of ADPKD (Kazancioglu et al, 2011), and has been related to disease progression (Ozkok et al, 2013).…”

Total Kidney Volume in Autosomal Dominant Polycystic Kidney Disease: A Biomarker of Disease Progression and Therapeutic Efficacy

“…15,49,54,55 Hematuria usually occurs several years or decades before a decline in kidney function is detectable. 54,56 The prevalence of nephrolithiasis in patients with ADPKD is 8% to 36%, [57][58][59] approximately twice that observed in the general population. There are a number of potential factors that, alone or in combination, make stone formation more likely among patients with ADPKD.…”

“…These include cyst-associated structural abnormalities and kidney tubular stasis, as well as the comorbid metabolic disorders. [57][58][59] Nephrolithiasis has been shown to be associated with enlarged kidneys. 15,59 Hypertension occurs in 50% of patients aged 20 to 34 years with ADPKD with normal kidney function and increases to w100% in patients with ESRD; it is associated with faster progression to ESRD and is an important risk factor for cardiovascular morbidity and mortality in those patients.…”

Polycystic kidney disease gene in the Lewis polycystic kidney rat is mapped to chromosome 10q21–q26

“…However, this disease is a result of mutations in at least eight genes which are different from the PKD genes. 23 ARPKD is an infantile disease, 24 ADPKD has an adult onset, 25 while nephronophthisis may be inherited as a dominant or recessive trait. 23 These diseases lead to development of extrarenal cysts, high systolic blood pressure, anemia, and eventually renal failure.…”