n engl j med 371;24 nejm.org december 11, 2014 2331 5. Pfeffer MA, Brenner BM, McMurray JJ. Aliskiren in type 2 diabetes and cardiorenal end points. N Engl J Med 2013;368:1065-6. 6. Luciano RL, Dahl NK. Extra-renal manifestations of autosomal dominant polycystic kidney disease (ADPKD): considerations for routine screening and management. Nephrol Dial Transplant 2014;29:247-54. 7. Chapman AB, To...

BACKGROUND AND OBJECTIVES Plasma copeptin, a marker of arginine vasopressin, is elevated in patients with autosomal dominant polycystic kidney disease and predicts disease progression. It is unknown whether elevated copeptin levels result from decreased kidney clearance or as compensation for impaired concentrating capacity. Data from patients with autosomal dominant polycystic kidney disease a...

autosomal dominant polycystic kidney disease (adpkd) is the most common form of inherited kidney disease that results in renal failure. pkd currently has no causative therapy. however, some treatment options are available, ranging from symptomatic therapy to delaying the onset of end-stage renal failure. early diagnosis of adult polycystic kidney disease is vital in order to prevent its complic...

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease and is responsible for 8-10% of patients with end-stage renal failure. The major extrarenal complications of ADPKD are cardiovascular abnormalities. Interrupted aortic arch (IAA) is a lethal congenital cardiac abnormality seen with a frequency of 3/1000000 births and is defined as a segment of the ...

Normal Anatomy of the Urinary Prune-Belly Syndrome/ 289 Tract/ 255 Megacystis-Microcolon-intestinal Bilateral Renal Agenesis/ 259 Hypoperistalsis Syndrome/ 291 Infantile Polycystic Kidney Congenital Mesoblastic Disease/ 266 Nephroma/ 292 Adult Polycystic Kidney Disease/ 268 Wilms’ Tumor/ 293 Multicystic Kidney Disease/ 270 Normal Anatomy of the Adrenal Ureteropelvic junction Glands/ 295 Obstruc...

Autosomal dominant polycystic kidney disease is a multisystem disease involving many organs. An association with other diseases such as tuberous sclerosis, von Hippel-Lindau disease and Marfan syndrome have been previously described. We describe a 35 year old female with achondroplasia who developed polycystic kidney disease involving both kidneys and progressing to end-stage renal disease. To ...

A captive 10-year-old female northern pintail (Anas acuta) with a history of unilateral lameness was diagnosed at necropsy with polycystic disease most severely affecting the right kidney. The lameness was attributed to pressure on the sacral nerve plexus caused by the unusually large cyst arising from the right kidney. Polycystic kidney disease previously has been considered an incidental find...

Unilateral and segmental localised polycystic disease is a rare type of cystic disease of the kidney. It takes the form of a segmental cystic abnormality in one kidney morphologically identical to the autosomal dominant adult form of polycystic kidney disease. The clinical, radiological, and pathological appearances of a case are described. The differential diagnosis and a possible pathogenic m...

BACKGROUND The prevalence of kidney disease (KD) due to inherited genetic conditions in Ireland is unknown. The aim of this study was to characterise an adult kidney disease population in Ireland and to identify familial clusters of kidney disease within the population. METHODS This was a multicenter cross-sectional study of patients with kidney disease in the Republic of Ireland, from Januar...

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 ...