Autosomal dominant polycystic kidney disease with diffuse proliferative glomerulonephritis - an unusual association: a case report and review of the literature

<p>Abstract</p> <p>Introduction</p> <p>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.</p> <p>Case presentation</p> <p>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 treatment and the drug most commonly used to treat nephrotic syndrome empirically.</p> <p>Conclusion</p> <p>Despite the reluctance of physicians to carry out a renal biopsy on patients with autosomal dominant polycystic kidney disease, our case supports the idea that renal biopsy is needed in patients with polycystic kidney disease with nephrotic-range proteinuria to make an accurate diagnosis. It also illustrates the importance of open renal biopsy in planning appropriate treatment for patients with autosomal dominant polycystic kidney disease with nephrotic-range proteinuria. The treatment for various histological subtypes leading to nephrotic syndrome is different, and in this modern era we should practice evidence-based medicine and should avoid empirical therapy with its associated adverse effects.</p

Use of the iPhone for Cobb angle measurement in scoliosis

Purpose: The Cobb technique is the universally accepted method for measuring the severity of spinal deformities. Traditionally, Cobb angles have been measured using protractor and pencil on hardcopy radiographic films. The new generation of mobile phones make accurate angle measurement possible using an integrated accelerometer, providing a potentially useful clinical tool for assessing Cobb angles. The purpose of this study was to compare Cobb angle measurements performed using an Apple iPhone and traditional protractor in a series of twenty Adolescent Idiopathic Scoliosis patients. Methods: Seven observers measured major Cobb angles on twenty pre-operative postero-anterior radiographs of Adolescent Idiopathic Scoliosis patients with both a standard protractor and using an Apple iPhone. Five of the observers repeated the measurements at least a week after the original measurements. Results: The mean absolute difference between pairs of iPhone/protractor measurements was 2.1°, with a small (1°) bias toward lower Cobb angles with the iPhone. 95% confidence intervals for intra-observer variability were ±3.3° for the protractor and ±3.9° for the iPhone. 95% confidence intervals for inter-observer variability were ±8.3° for the iPhone and ±7.1° for the protractor. Both of these confidence intervals were within the range of previously published Cobb measurement studies. Conclusions: We conclude that the iPhone is an equivalent Cobb measurement tool to the manual protractor, and measurement times are about 15% less. The widespread availability of inclinometer-equipped mobile phones and the ability to store measurements in later versions of the angle measurement software may make these new technologies attractive for clinical measurement applications