We report on 2 siblings with autosomal-recessive polycystic kidney disease, diagnosed at the ages of 14 and 18 years, respectively. Clinical findings and differential diagnosis, especially for autosomal-dominant polycystic kidney disease, are given. The consequences for genetic counselling are discussed.
Microaneurysms of renal and visceral arteries are characteristic signs of periarteritis nodosa. Normally they are not found in Wegener's disease, where glomerulonephritis is commonly observed. We report on a patient with vasculitis of the upper and lower respiratory tract, focal glomerulonephritis, prostatic and pulmonary granulomas and anti-cytoplasm antibodies corresponding to Wegener's disease. The most striking findings in angiography were multiple small aneurysms of the peripheral branches of the renal artery. At necroscopy these angiographic findings were histologically proven as necrosis of the arterial wall with destruction of the elastic lamina, causing local vascular ectasia. These renal vascular changes are a characteristic sign of periarteritis nodosa. The combination of clinical, laboratory, radiologic and histologic findings in our patient can be explained as an overlap-syndrome of Wegener's disease and periarteritis nodosa. We assume that the combination of pathologic findings in our patient correspond to a rare atypical renal manifestation of Wegener's disease.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.