tailieunhanh - Báo cáo y học: " Autosomal dominant polycystic kidney disease with diffuse proliferative glomerulonephritis - an unusual association: a case report and review of the literature"

Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài:Autosomal dominant polycystic kidney disease with diffuse proliferative glomerulonephritis - an unusual association: a case report and review of the literature | D Cruz et al. Journal of Medical Case Reports 2010 4 125 http content 4 1 125 jA CASE REPORTS CASE REPORT Open Access Au tosomal dominant polycystic kidney disease with diffuse proliferative glomerulonephritis - an unusual association a case report and review of the literature Sanjay D Cruz 1 Rajdeep Singh2 Harsh Mohan3 Ravinder Kaur4 Ranjana Walker Minz5 Vinay Kapoor1 and Atul Sachdev1 Abstract Introduction Autosomal dominant polycystic kidney disease is an inherited disorder that is characterized by the development and growth of cysts in the kidneys and other organs. Urinary protein excretion is usually less than 1 g 24 hours in autosomal dominant polycystic kidney disease and an association of nephrotic syndrome with this condition is considered rare. There are only anecdotal case reports of autosomal dominant polycystic kidney disease associated with nephrotic syndrome with focal segmental glomerulosclerosis being the most commonly reported histopathological diagnosis. Nephrotic-range proteinuria in the presence of autosomal dominant polycystic kidney disease with or without an accompanying decline in renal function should be investigated by open renal biopsy to exclude coexisting glomerular disease. To the best of our knowledge this is the first case of autosomal dominant polycystic kidney disease with histologically proven diffuse proliferative glomerulonephritis presenting with nephrotic-range proteinuria. No other reports of this could be found in a global electronic search of the literature. Case presentation We report the case of a 35-year-old Indo-Aryan man with autosomal dominant polycystic kidney disease associated with nephrotic syndrome and a concomitant decline in his glomerular filtration rate. Open renal biopsy revealed diffuse proliferative glomerulonephritis. An accurate diagnosis enabled us to manage him conservatively with a successful outcome without the use of corticosteroid which is the standard

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