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sickle cell esrd

Romain Arrestier, Caroline Dudreuilh, Philippe Remy, Ghada Boulahia, Bouteina Bentaarit, Claire Leibler, Amir Adedjouma, Tomek Kofman, Marie Matignon, Dil Sahali, Roger Dufresne, Jean-Francois Deux, Charlotte Colin, Philippe Grimbert, Philippe Lang, Pablo Bartolucci, Bernard Maitre, Jeanne Tran Van Nhieu, Vincent Audard
Calciphylaxis is a small vessel vasculopathy, characterized by medial wall calcification that develops in a few patients with chronic renal failure. The prognosis of skin calciphylaxis has improved considerably since the introduction of sodium thiosulfate (STS), but it remains unclear whether this therapy is effective against organ lesions related to calciphylaxis. Pulmonary calciphylaxis is a usually fatal medical condition that may occur in association with skin involvement in patients with end-stage renal disease...
February 2016: Medicine (Baltimore)
Alicja Czopek, Rebecca Moorhouse, David J Webb, Neeraj Dhaun
Our growing understanding of the role of the endothelin (ET) system in renal physiology and pathophysiology is from emerging studies of renal disease in animal models and humans. ET receptor antagonists reduce blood pressure and proteinuria in chronic kidney disease and cause regression of renal injury in animals. However, the therapeutic potential of ET receptor antagonism has not been fully explored and clinical studies have been largely limited to patients with diabetic nephropathy. There remains a need for more work in nondiabetic chronic kidney disease, end-stage renal disease (patients requiring maintenance dialysis and those with a functioning kidney transplant), ischemia reperfusion injury, and sickle cell disease...
March 1, 2016: American Journal of Physiology. Regulatory, Integrative and Comparative Physiology
Mei Lin Z Bissonnette, Kammi J Henriksen, Kristie Delaney, Nicole Stankus, Anthony Chang
Sickle cell nephropathy is a common complication in patients with sickle cell hemoglobinopathies. In these disorders, polymerization of mutated hemoglobin S results in deformation of red blood cells, which can cause endothelial cell injury in the kidney that may lead to thrombus formation when severe or manifest by multilayering of the basement membranes (glomerular and/or peritubular capillaries) in milder forms of injury. As the injury progresses, the subsequent ischemia, tubular dysfunction, and glomerular scarring can result in CKD or ESRD...
May 2016: Journal of the American Society of Nephrology: JASN
Ilham Youssry, Samuel Makar, Rania Fawzy, Manal Wilson, Ghada AbdAllah, Eman Fathy, Happy Sawires
BACKGROUND: Given the burden and poor outcome of end-stage renal disease in sickle cell disease (SCD), early markers of sickle cell nephropathy (SN) are desirable. Disordered angiogenesis underlies many complications of SCD. We aimed to determine the relationship between serum FMS-like tyrosine kinase-1 (sFLT-1) and other biomarkers of renal damage for the early diagnosis of SN. METHODS: Forty-seven SCD patients and 49 healthy controls were enrolled. Microalbuminuria was determined in patient urine samples...
December 2015: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Santosh L Saraf, Xu Zhang, Binal Shah, Tamir Kanias, Krishnamurthy P Gudehithlu, Rick Kittles, Roberto F Machado, Jose A L Arruda, Mark T Gladwin, Ashok K Singh, Victor R Gordeuk
Intravascular hemolysis and hemoglobinuria are associated with sickle cell nephropathy. ApoL1 is involved in cell-free hemoglobin scavenging through association with haptoglobin-related protein. APOL1 G1/G2 variants are the strongest genetic predictors of kidney disease in the general African-American population. A single report associated APOL1 G1/G2 with sickle cell nephropathy. In 221 patients with sickle cell disease at the University of Illinois at Chicago, we replicated the finding of an association of APOL1 G1/G2 with proteinuria, specifically with urine albumin concentration (β=1...
October 2015: Haematologica
Suzanne M Boyle, Benjamin Jacobs, Farzana A Sayani, Brenda Hoffman
While patients with sickle cell disease currently constitute a very small minority of the US dialysis population (0.1%), there is anticipated growth of this group as the life expectancy of those with sickle cell disease (SCD) increases. SCD patients suffer a high burden of morbidity, which is enhanced by the presence of end-stage renal disease (ESRD). In this review, we discuss the pathophysiology of SCD and the basic tenets of its management with focus on the dialysis patient with SCD. Anemia in dialysis patients with SCD is a unique challenge...
January 2016: Seminars in Dialysis
Ahmed M Alkhunaizi, Adil A Al-Khatti
Proteinuria is a complication of sickle cell nephropathy that can progress to renal insufficiency and end-stage renal disease. The magnitude of proteinuria among patients with sickle cell disease (SCD) has been reported with variable prevalence. The aim of this study was to determine the prevalence of proteinuria in a large number of patients with SCD in Eastern Saudi Arabia. The urinalyses of 940 non-diabetic patients with SCD were tested for the presence of proteinuria. The glomerular filtration rate (e-GFR) of all patients was estimated using the Cockcroft- Gault equation...
September 2014: Saudi Journal of Kidney Diseases and Transplantation
Kenneth I Ataga, Vimal K Derebail, David R Archer
Sickle cell disease (SCD) produces many structural and functional abnormalities in the kidney, including glomerular abnormalities. Albuminuria is the most common manifestation of glomerular damage, with a prevalence between 26 and 68% in adult patients. The pathophysiology of albuminuria in SCD is likely multifactorial, with contributions from hyperfiltration, glomerular hypertension, ischemia-reperfusion injury, oxidative stress, decreased nitric oxide (NO) bioavailability, and endothelial dysfunction. Although its natural history in SCD remains inadequately defined, albuminuria is associated with increased echocardiography-derived tricuspid regurgitant jet velocity, systemic blood pressure, and hypertension, as well as history of stroke, suggesting a shared vasculopathic pathophysiology...
September 2014: American Journal of Hematology
Claire C Sharpe, Swee Lay Thein
Renal disease is one of the most frequent and severe complications experienced by patients with sickle cell disease; its prevalence is likely to increase as the patient population ages. We recommend regular monitoring for early signs of renal involvement and a low threshold for the use of hydroxyurea as preventative measures for end-stage renal disease. Once renal complications are detected, a careful assessment of the patient is required to rule out other causes of renal disease. Proteinuria and hypertension should be managed aggressively and the patient referred to a specialist nephrology center when progressive decline in renal function is noted...
June 12, 2014: Blood
Abdullah Alhwiesh
Sickle cell disease (SCD) is a major health problem in many countries. Sickle cell nephropathy (SCN) is now a well-characterized entity with specific manifestations, risk factors and prognosis. The presence of sickled erythrocytes in the renal medullary vessels is the hallmark of the disease with a variety of renal complications. Renal manifestations of SCD include renal ischemia, microinfarcts, renal papillary necrosis and renal tubular abnormalities with variable clinical presentations. Proximal tubule dysfunction generally impairs urinary concentration, while more distal tubule dysfunction may impair potassium excretion, leading to hyperkalemia...
March 2014: Saudi Journal of Kidney Diseases and Transplantation
U H Okafor, C Wachukwu, P Emem-Chioma, F S Wokoma
Sickle cell nephropathy (SCN) is a common complication of sickle cell disease (SCD). It has variable presentation, ranging from hyposthenuria to end-stage renal disease (ESRD). Management of ESRD in SCD patients is froth with multiple challenges which has potential to impact negatively the outcome of the patient. Kidney transplant is the preferred renal replacement therapy in these patients. The objective of this case study is to report kidney transplant in a Nigerian young man with sickle cell nephropathy and to highlight the outcome and the challenges to kidney transplant in this patient...
2012: Case Reports in Nephrology
Maud Cazenave, Bérengère Koehl, Dominique Nochy, Pierre-Louis Tharaux, Vincent Audard
Sickle cell disease (SCD), the most common hemoglobinopathy, is an increasing cause of chronic kidney disease. In the last decade, we have witnessed a better understanding in the characterization of clinical manifestations and pathogenesis of sickle cell nephropathy. The spectrum of renal diseases during SCD includes various renal manifestations such as impairment of urinary concentrating ability, defect in urine acidification, renal papillary necrosis and proteinuria related to glomerular injury leading to progressive end-stage renal disease...
February 2014: Néphrologie & Thérapeutique
Louis-Philippe Laurin, Patrick H Nachman, Payal C Desai, Kenneth I Ataga, Vimal K Derebail
BACKGROUND: Albuminuria is an early manifestation of sickle cell nephropathy. Prior small case series suggests benefit of hydroxyurea in reducing albuminuria, with a similar trend noted in pediatric studies. We aimed to comprehensively evaluate hydroxyurea use and prevalence of albuminuria in adult sickle cell patients. METHODS: We performed a cross-sectional study of 149 adult patients followed between 2000 and 2011 in a comprehensive sickle cell clinic. All patients were assessed for albuminuria either by direct measurement or by urinary chemical strip (dipstick) testing...
June 2014: Nephrology, Dialysis, Transplantation
Ann C McClellan, Jean-Christophe Luthi, Janet R Lynch, J Michael Soucie, Roshni Kulkarni, Antonio Guasch, Edwin D Huff, David Gilbertson, William M McClellan, Michael R DeBaun
Adequate pre-dialysis care reduces mortality among end-stage renal disease (ESRD) patients. We tested the hypothesis that individuals with ESRD due to sickle cell disease (SCD-ESRD) receiving pre-ESRD care have lower mortality compared to individuals without pre-ESRD care. We examined the association between mortality and pre-ESRD care in incident SCD-ESRD patients who started haemodialysis between 1 June, 2005 and 31 May, 2009 using data provided by the Centers for Medicare and Medicaid Services (CMS)...
November 2012: British Journal of Haematology
O T Adedoyin, O O Adesiyun, O A Adegboye, O A Bello, O P Fatoye
AIMS AND OBJECTIVES: To determine and report the occurrence of overt renal presentations in children with sickle cell disease seen over the last 14 years (1995-2009) at the Emergency Paediatric Unit and Paediatric Ward and followed up at the Paediatric Nephrology clinic of University of Ilorin Teaching Hospital, Ilorin (UITH). PATIENTS AND METHODS: The five cases with overt renal presentations seen during the period were reported and analyzed for age, sex, and renal manifestations...
June 2012: Nigerian Postgraduate Medical Journal
Nestor-M Pakasa, Ernest-K Sumaïli
Chronic kidney disease (CKD) is a major global public health problem. But kidney involvement is more common and appears more severe in Africa than in developed countries. The likely causes of end stage renal disease (ESRD) or CKD stage 3 and above in developed countries are diabetes, hypertension and less frequently glomerular diseases. In contrast, in decreasing order in Africa are glomerulopathies, hypertension and diabetes. The reasons for this preponderance of glomerular diseases are not fully known but may be linked to the persistence or reemergence of tropical diseases...
February 2012: Annales de Pathologie
Kathleen M Fox, Jerry Yee, Ze Cong, John M Brooks, Jeffrey Petersen, Lois Lamerato, Shravanthi R Gandra
BACKGROUND: Transfusion patterns are not well characterized in non-dialysis (ND) chronic kidney disease (CKD) patients. This study describes the proportion of patients transfused, units of blood transfused and trigger-hemoglobin (Hb) levels for transfusions in severe anemic, ND-CKD patients in routine practice. METHODS: A retrospective cohort study of electronic medical record data from the Henry Ford Health System identified 374 adult, ND-CKD patients with severe anemia (Hb < 10 g/dL and subsequent use of erythropoiesis-stimulating agents [ESA] therapy, blood transfusions, or a second Hb < 10 g/dL) between January 2004 and June 2008...
2012: BMC Nephrology
M O Odubanjo, C A Okolo, A O Oluwasola, A Arije
There is paucity of information on the magnitude of the burden of renal disease in our environment. Obtaining accurate data is hampered by the poor socioeconomic status of most patients with lack of access to specialized care in tertiary institutions, where most of the data is generated. The incidence of chronic renal failure (CRF) and end-stage renal disease (ESRD) in any specified area is known to be influenced by the prevalence of specific disease entities resulting in CRF. Hypertension, glomerulonephritis (GN), sickle cell disease, quartan malaria nephropathy, urinary tract schistosomiasis and other parasite-related forms of chronic GN are known to contribute significantly to the incidence of CRF in Nigeria...
September 2011: Saudi Journal of Kidney Diseases and Transplantation
Allison E Ashley-Koch, Emmanuel C Okocha, Melanie E Garrett, Karen Soldano, Laura M De Castro, Jude C Jonassaint, Eugene P Orringer, James R Eckman, Marilyn J Telen
Renal failure occurs in 5-18% of sickle cell disease (SCD) patients and is associated with early mortality. At-risk SCD patients cannot be identified prior to the appearance of proteinuria and the pathobiology is not well understood. The myosin, heavy chain 9, non-muscle (MYH9) and apolipoprotein L1 (APOL1) genes have been associated with risk for focal segmental glomerulosclerosis and end-stage renal disease in African Americans. We genotyped 26 single nucleotide polymorphisms (SNPs) in MYH9 and 2 SNPs in APOL1 (representing the G1 and G2 tags) in 521 unrelated adult (18-83 years) SCD patients screened for proteinuria...
November 2011: British Journal of Haematology
Vimal K Derebail, Patrick H Nachman, Nigel S Key, Heather Ansede, Ronald J Falk, Wayne D Rosamond, Abhijit V Kshirsagar
African-American patients with end-stage renal disease have historically lower hemoglobin concentrations and higher requirements of erythropoiesis-stimulating agent (ESA). While disparities in health-care access may partially explain these findings, the role of variant hemoglobin, such as sickle trait, has not been investigated. To clarify this, we evaluated 154 African-American patients receiving in-center hemodialysis with available hemoglobin phenotyping. The primary exposure was any abnormal hemoglobin variant and the primary outcome of higher-dose ESA was defined as a dose of 6500 or more units per treatment...
November 2011: Kidney International
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