Clinical Study on Autosomal Dominant Polycystic Kidney Disease among North Tunisians - Abstract
Background: Autosomal Dominant Polycystic kidney disease (ADPKD) is the most frequent renal hereditary disease, most commonly revealed in adulthood. It is characterized by the development of multiple cysts in the kidney and many other extra-renal manifestations. We aim to determine the epidemiological, clinical, therapeutic and prognostic factors of ADPKD progression to end stage renal disease (ESRD) among our patients. Methods: In a retrospective multicentric study, we reviewed the records of 569 patients with ADPKD, hospitalized in a nephrology department or followed at the outpatient department of university and regional hospitals covering the north and the center of the country, during the period (1969-2016). Epidemioclinical, paraclinical, therapeutic and evolutive data were collected based on medical observations. Prognostic factors of renal survival were analyzed by multivariate analysis using the comparison of the survival rates by the log rank test. Results: The mean age of patients was 48,54 ± 13,68 years, 14% were young adults (<40 years). There were 272 female patients and 297 male patients (sex-ratio M/F = 1.09). Thirty eight percent of patients were from the northest. A family history of ADPKD was found in 43.7 % of cases. Renal symptoms were dominated by lombalgia, renal failure, hypertension and hematuria in respectively 51.9%, 48.2%, 29.1% and 24.6%. The median serum creatinine level was 459 µmol/l (range : 47-2454), hypertension had preceded the onset of ADPKD in 28.8% of cases. Extra-renal manifestations consisted in liver cysts (43.5%), cardiac involvement (31.9%), cerebral aneurysms (12.9%) and gastro-intestinal involvement (9.4%). Urologic complications were observed in 54.6% of cases. ESRD occured in 43.1% after a mean follow-up of 47 months (range : 0-384). Risk factors of poor renal prognosis were: age >40 years (P=0.009), hematuria (P= 0.034), hemoglobine >14 g/dl (p=0.0013), high uricemia level (P=0.001) and leucocyturia (P=0.02). Age >40 years and leucocyturia were independant factors associated with poor renal survival. Death occured in 59 cases (10.3%) mostly caused by infectious complications (44.1%). Conclusion: In our study, ADPKD was tardily diagnosed for most cases. We emphasize the importance of the family screening which will enable early detection and management of the complications associated with the ADPKD.