Abstract
Background and Aims
The identification of possible risk factors for the progression of Autosomal Dominant Polycystic Kidney Disease (ADPKD) is an emerging field especially after the introduction of the first disease-specific treatment. The present study aims to explore the associations between epidemiological, clinical and imagining data in a large cohort of ADPKD patients.
Method
This study was from a single outpatient clinic following patients with ADPKD. Patients were included in the study if they had a recent Magnetic Resonance Imaging (MRI) for measurement of Total Kidney Volume (TKV), a validated biomarker for disease progression. For all patients, the Mayo Clinic Imagining Category (MCIC) and the respective prediction for End Stage Renal Disease (ESRD) were calculated. Patients eligible for tolvaptan treatment (MCIC 1C, 1D, 1E, age < 55 years old and estimated-glomerular filtration rate (e-GFR) ≥ 25 ml/min) were identified. Characteristics including individual medical history, clinical and laboratory data were examined for possible associations with renal and imagining parameters using linear regression models.
Results
A total of 158 patients were included. Based on measurements of height-adjusted TKV (ht-TKV) and age, 5% of the patients were classified as 1A, 20% as 1B, 34% as 1C, 25% as 1D and 16% as 1E, MCIC. In multivariable analysis, patient’s age (p = 0.01), male sex (p < 0.001), parent’s age at which ESRD was reached (adjusted for patient age) (p < 0.001) and proteinuria (p = 0.04) were associated with ht-TKV. Parent’s age at ESRD differed significantly between the MCICs of the offspring (mean±(SD)), 70.83 (12.90) in 1A, 63.79 (11.39) in 1B, 57.32 (10.42) in 1C, 51.42 (9.18) in 1D and 47.94 (5.73) years old in 1E, (p < 0.001). Similarly, there were significant differences in the presence and the age of hypertension onset (p =0.004 and p = 0.003, respectively). In 104 patients (50 females, 54 males) who were eligible for tolvaptan treatment age at ADPKD diagnosis, age at hypertension onset and parent’s age reaching at ESRD were all significantly lower (p < 0.001 for all) when compared to non-eligible patients. Finally, factors associated with the prediction score of ESRD (e-GFR 10/ml/min) were hypertension, uric acid and the age at ESRD of the affected parent (p = 0.001, 0.02 and 0.01, respectively).
Conclusion
The age at which an affected parent had reached ESRD, as heritability estimator, was significantly associated with a worst phenotype, prognosis and tolvaptan indication. Early diagnosis of the disease, hypertension and its early onset, proteinuria and male sex are also possible risk factors for the progression of ADPKD.
High-risk human papillomavirus infection and associated factors in the anal canal of HIV-positive patients in Medellín, 2017–2018
