Sickle cell disease and the kidney

2020 ◽  
pp. 5032-5034
Author(s):  
Claire C. Sharpe
Keyword(s):  
Chronic Kidney Disease ◽  
Sickle Cell Disease ◽  
Kidney Disease ◽  
Sickle Cell ◽  
Clinical Symptoms ◽  
Specific Treatment ◽  
Cell Disease ◽  
Sickle Cell Nephropathy

About 60% of patients with sickle cell disease have sickle cell nephropathy. Clinical symptoms reflect medullary compromise, with polyuria, troublesome nocturia, enuresis, and dehydration being typical early manifestations. Haematuria, nonvisible and visible, is common. The prevalence of albuminuria rises with age, and those in whom this progresses rapidly are at greatest risk of developing endstage kidney disease, which eventually affects 10 to 15% of patients with sickle cell nephropathy. Management of chronic kidney disease due to sickle cell nephropathy is along standard lines: no specific treatment has been shown to prevent the condition or retard its progression.

scholarly journals Clinical predictors of poor outcomes in patients with sickle cell disease and COVID-19 infection

2021 ◽  
Vol 5 (1) ◽  
pp. 207-215
Author(s):  
Caterina P. Minniti ◽  
Ahmar U. Zaidi ◽  
Mehdi Nouraie ◽  
Deepa Manwani ◽  
Gary D. Crouch ◽  
...  
Keyword(s):  
Chronic Kidney Disease ◽  
Lactate Dehydrogenase ◽  
Sickle Cell Disease ◽  
Kidney Disease ◽  
Sickle Cell ◽  
Case Fatality ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Risk Of Death ◽  
D Dimer

Abstract We aimed to identify predictors of outcomes and survival in patients living in 4 major metropolitan areas who had sickle cell disease (SCD) and COVID-19 to inform best approaches to prevention and care. Data were collected at baseline and during the clinical course in SCD patients diagnosed with COVID-19 in four COVID-19 epicenters. Patients were followed up posthospital discharge for up to 3 months. Of sixty-six SCD patients with COVID-19, fifty patients (75%) required hospitalization, and seven died (10.6%). Patients with preexisting kidney disease (chronic kidney disease) were more likely to be hospitalized. The most common presenting symptom was vaso-occlusive pain. Acute chest syndrome occurred in 30 (60%) of the 50 hospitalized patients and in all who died. Older age and histories of pulmonary hypertension, congestive heart failure, chronic kidney disease, and stroke were more prevalent in patients who died, as were higher creatinine, lactate dehydrogenase, and D-dimer levels. Anticoagulation use while inpatient was twice less common in patients who died. All deaths occurred in individuals not taking hydroxyurea or any other SCD-modifying therapy. Patients with SCD and COVID-19 exhibited a broad range of disease severity. We cannot definitively state that the overall mortality is higher in patients with SCD, although our case fatality rate was ∼10% compared with ∼3% in the general population, despite a median age of 34 years. Individuals with SCD aged >50 years, with preexisting cardiopulmonary, renal disease, and/or stroke not receiving hydroxyurea, who present with high serum creatinine, lactate dehydrogenase, and D-dimer levels, are at higher risk of death, irrespective of genotype or sex.

scholarly journals Elevated Plasma Soluble Urokinase-Type Plasminogen Activator Receptor (suPAR) in Sickle Cell Disease - a Marker of Chronic Kidney Disease

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 968-968
Author(s):  
Nowah Kokou Apeadoufia Afangbedji ◽  
James G. Taylor ◽  
Sergei Nekhai ◽  
Marina Jerebtsova
Keyword(s):  
Chronic Kidney Disease ◽  
Sickle Cell Disease ◽  
Kidney Disease ◽  
Renal Disease ◽  
Plasminogen Activator ◽  
Sickle Cell ◽  
Cell Disease ◽  
Type Plasminogen Activator ◽  
Urokinase Type Plasminogen Activator ◽  
Plasminogen Activator Receptor

Abstract Background: Sickle cell nephropathy (SCN) is one of the most common complications of SCD, leading in most cases to chronic kidney disease (CKD) and end-stage renal disease (ESRD). Despite the high prevalence of CKD in sickle cell disease (SCD) patients, there remains a poor understanding of the pathophysiological mechanism of SCN and a lack of biomarkers for early detection of SCD-associated CKD. Soluble urokinase-type plasminogen activator receptor (suPAR) is an emerging biomarker of CKD. suPAR is a member of the fibrinolytic system, which is dysregulated in SCD patients. Objective: To evaluate suPAR as a biomarker of SCD-associated nephropathy and identify plasma proteases responsible for its increase in SCD. Methods: The study was approved by Howard University review board (IRB) and all subjects provided written inform consent prior to the sample collection. Whole blood and urine samples were collected from 77 SCD patients and 10 healthy individuals, and plasma was isolated. Levels of creatinine and cystatin C in plasma and albumin and creatinine in urine were measured by ELISA. eGFR was calculated using CKD-EPI creatinine-cystatin equation, and CKD stages were assigned. Plasma suPAR was measured by ELISA and was correlated with CKD stages. The activities of candidates uPAR proteases: Neutrophile elastase (NE), urokinase-type plasminogen activator (uPA) and plasmin in plasma samples from SCD patients were measured and compared to healthy participants. Results: The average age of SCD patients was 42.5 years (range 18-67 years). Most patients had HbSS genotype (67.5%),19.5% of patients were HbSC (hemoglobin C sickle cell compound heterozygous), and 13% had HbS β-thalassemia. More than half (53.2 %) were females. We observed an increased level of plasma suPAR (>3ng/ml) in more than 60% of SCA patients without renal disease, representing a risk factor for CKD progression. Plasma suPAR levels further increased in the patients with CKD and positively correlated with stages of CKD (r=0.419, R2=0.1696). Analysis of plasma proteases that cleaved uPAR producing soluble peptides (suPAR) demonstrated increased urokinase-type plasminogen activator (uPA) activity without significant changes in neutrophile elastase. Conclusion: This study validated plasma suPAR as a potential marker of CKD in SCD patients and identified plasma uPA as a uPAR protease that may increase circulating suPAR in SCD. Future longitudinal analysis of suPAR levels in patients with SCA is needed. Acknowledgments: We thank Drs. Namita Kumari and Xiaomei Niu for their help in samples identification. This work was supported by NIH Research Grants 1R01HL125005-06A1, 5U54MD007597, 1P30AI117970-06,1UM1AI26617, and 1SC1HL150685. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

scholarly journals Elevated Pulse Pressure is Associated with Hemolysis, Proteinuria and Chronic Kidney Disease in Sickle Cell Disease

PLoS ONE ◽  
2014 ◽  
Vol 9 (12) ◽  
pp. e114309 ◽  
Author(s):  
Enrico M. Novelli ◽  
Mariana Hildesheim ◽  
Caterina Rosano ◽  
Rebecca Vanderpool ◽  
Marc Simon ◽  
...  
Keyword(s):  
Chronic Kidney Disease ◽  
Sickle Cell Disease ◽  
Kidney Disease ◽  
Sickle Cell ◽  
Pulse Pressure ◽  
Cell Disease

Prevalence and Progression of Chronic Kidney Disease in Adult Patients With Sickle Cell Disease

2014 ◽  
Vol 62 (5) ◽  
pp. 804-807 ◽  
Author(s):  
Elvira O. Gosmanova ◽  
Sahar Zaidi ◽  
Jim Y. Wan ◽  
Patricia E. Adams-Graves
Keyword(s):  
Chronic Kidney Disease ◽  
Sickle Cell Disease ◽  
Kidney Disease ◽  
Sickle Cell ◽  
Adult Patients ◽  
Cell Disease

scholarly journals Outcome and Challenges of Kidney Transplant in Patients with Sickle Cell Disease

2013 ◽  
Vol 2013 ◽  
pp. 1-8 ◽  
Author(s):  
U. H. Okafor ◽  
E. Aneke
Keyword(s):  
Sickle Cell Disease ◽  
Kidney Disease ◽  
Kidney Transplant ◽  
Sickle Cell ◽  
Cell Disease ◽  
End Stage Kidney Disease ◽  
Transplant Kidney ◽  
Short And Long Term ◽  
Sickle Cell Nephropathy ◽  
End Stage

Sickle cell nephropathy is a common presentation in patients with sickle cell disease. End-stage kidney disease is the most severe presentation of sickle cell nephropathy in terms of morbidity and mortality. Sickle cell disease patients with end-stage kidney disease are amenable to renal replacement therapy including kidney transplant. Kidney transplant in these patients has been associated with variable outcome with recent studies reporting short- and long-term outcomes comparable to that of patients with HbAA. Sickle cell disease patients are predisposed to various haematological, cardiorespiratory, and immunological challenges. These challenges have the potential to limit, delay, or prevent kidney transplant in patients with sickle cell disease. There are few reports on the outcome and challenges of kidney transplant in this group of patients. The aim of this review is to highlight the outcome and challenges of kidney transplant in patients with sickle cell disease.

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