Autosomal Dominant Polycystic Kidney Disease: An Unknown Disease in Chad

Abstract

Introduction: Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic renal disease. After about ten years of evolution, it leads to a slow and progressive loss of kidney function eventually causing renal insufficiency and kidney failure. Few data exist on this pathology in Africa and Chad. The objective of this study was to analyze the epidemiological, diagnostic and therapeutic aspects of ADPKD. Methodology: This was a multicenter cross-sectional, descriptive and analytical study conducted over a period of 34 months in two hospitals in N’Djamena, Chad. All patients aged 15 years and above, having a family history of polycystic kidney disease, which were hospitalized or who were coming for consultation that met the unified criteria for ultrasonographic diagnoses (Ravine criteria modified by Pei) of ADPKD were included in the study. The clinical, paraclinical, therapeutic and evolutionary criteria for these patients had been studied. Results: There were a total of 26 cases of polycystic kidney disease that had a hospital prevalence of 1.16%. The average age was 42.4 years with extremes ranging from 15-70 years and a sex ratio of 1.3. The family survey had shown that parental consanguinity was present in 34.6% (n=9). The prevalence of hypertension was 53.8% (n=14). The mean serum creatinine was 45 mg/l with extremes ranging from 4.5 to 274.42 mg/l. It was noted that 65.3% (n=17) had chronic kidney disease with a GFR >30 mL/min/1.73m2 . During diagnosis 26.9% (n=7) had end-stage renal disease (ESRD), while 2 patients progressed to ESRD two years after diagnosis. One patient was hospitalized for chronic hemodialysis. No patient had received a kidney transplant and no patient had undergone a molecular biology and genetics study or had been treated with Tolvaptan. Conclusion: In Chad, the hospital prevalence of ADPKD was 1.16%, affecting young adults (average age of 42.4 years) with a male predominance. Management is usually late and remains limited to symptomatic treatment with complications such as chronic renal failure. Abbreviations: ADPKD: Autosomal Dominant Polycystic Kidney Disease, CRF: Chronic Renal Failure, ESRD: End-Stage Renal Disease, CVA: Cerebro Vascular Accident