Abstract
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary form of kidney disease. Clinical data on this multisystem disorder are scarce from developing countries. We conducted a prospective observational study of the clinical profile of ADPKD patients at a single center over a period of six years. A total of 208 patients were studied. Majority were male (60.6%) and the mean age was 45.8 ± 14.5 years. About 61.5% had early stage (Stages 1-3) of chronic kidney disease (CKD) and 38.5% had advanced CKD (Stages 4 and 5). Clinical features observed included pain abdomen (46.2%), nocturia (65.9%), hematuria (21.6%), nephrolithiasis (38.9%), urinary tract infection (UTI) (38.9%), hypertension (69.5%), and raised serum creatinine (54.3%). The prevalence of nocturia, hypertension, and renal dysfunction showed a significant increase with age (P = 0.001). Extrarenal manifestations were polycystic liver disease in 77 patients (37%), cysts in pancreas in two (1%), and stroke in three (1.5%) (hemorrhage in 2 and infarct in 1). There was significantly higher prevalence of hypertension (P = 0.027) and nephrolithiasis (P = 0.044) in males compared to females. Ninety-two patients (44.2%) had a positive family history for ADPKD. Fifteen (7.2%) had kidney failure at the diagnosis of ADPKD, were hospitalized, and underwent emergency dialysis. A total of 20 patients (9.6%) developed end-stage kidney disease during the study period. The age at diagnosis was higher, and there was a high prevalence of hypertension, nocturia, abdominal pain, nephrolithiasis, UTI, and renal dysfunction in Indian ADPKD patients.
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