End-stage renal disease in patients with Fabry disease.
نویسندگان
چکیده
Anderson-Fabry disease (AFD) is a rare cause of end-stage renal disease (ESRD). Renal pathology in AFD is notable for diffuse deposition of glycosphingolipid in the renal glomeruli, tubules, and vasculature. Light microscopic findings include a “foamy” appearance of the glomeruli with diffuse swelling and vacuolization of visceral podocytes. Electron microscopic findings show podocytes and mesangial cells filled with lysosomal electron dense granules arranged in a lamellar, myelin pattern. Toluidine blue stain brings out the lipid deposits in these areas. ESRD is a common complication of AFD (see Alroy et al. and Branton et al. in this issue). Current information regarding ESRD in patients with AFD derives primarily from two studies that used data from the United States Renal Data System (USRDS) and the European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) registry. In this article, we review the prevalence of ESRD due to AFD, the characteristics of Fabry patients at initiation of renal replacement therapy (RRT), and the outcomes associated with dialysis and transplantation.
منابع مشابه
Dentomaxillofacial Radiographic Changes in a Group of Iranian Patients with End Stage Renal Disease Undergoing Hemodialysis
Introduction: This study aims to evaluate the dentomaxillofacial radiographic changes in end stage renal disease (ESRD) patients who were on hemodialysis. Methods: Parathyroid hormone (PTH), calcium, phosphorus and alkaline phosphatase (ALP) measurements, as well as Panoramic and periapical radiographs were obtained from seventy four patients with a history of end stage renal disease (ESRD). Re...
متن کاملAvoidance of Dialysis in an End-Stage Renal Disease Patient Status-post Off-pump Coronary Artery Bypass Grafting
Certain benefits are clearly associated with the use of off-pump coronary artery bypass grafting (CABG) as compared with the on-pump CABG. The superiority is more evident in patients with multiple co morbidities including renal failure.We reviewed the medical records of a 67-year-old male with a past medical history that was significant for multiple cardiovascular diseases and new-onset end-sta...
متن کاملPrevalence of cutaneous manifestations in end stage renal disease patients under hemodialysis in Imam Khomeini Hospital, Tehran in 2003
Background and aim: New modalities in the treatment of end-stage renal disease (ESRD) patients have increased their life expectancy and quality of life. A wide range of cutaneous manifestations are seen in ESRD patients. This study was conducted to determine the prevalence of cutaneous manifestations in ESRD patients on maintenance hemodialysis in the dialysis department of Imam Khomeini ...
متن کاملLactulose efficacy in reduction of nitrogen products, blood potassium and fluid overload in patients with end-stage renal failure.
Abstract Introduction. Chronic kidney disease (CKD) is a major public health problem that often goes unrecognized until late-stage disease. Patients with chronic kidney disease (CKD) face with uremic toxins and hyperkalemia. Also fluid overload in CKD patients is associated with rapid decline in kidney function. Lactulose is a hyper osmotic agent and as a prebiotic could reduce serum urea and p...
متن کاملPrevalence of infectious diseases in patients with end stage renal disease in Gorgan City in Iran
Background: The mortality rate of sepsis and pneumonia is higher in end-stage renal disease (ESRD) patients than in the general population. Bacterial infections are the most common cause of hospitalization in dialysis patients and the most common source of bacteremia is vascular access in these patients. The aim of this study was to determine the prevalence of infectious causes of hospitalizati...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Journal of the American Society of Nephrology : JASN
دوره 13 Suppl 2 شماره
صفحات -
تاریخ انتشار 2002