sickle cell esrd

Anemia is commonly observed in patients with end-stage renal disease (ESRD) on maintenance hemodialysis (HD) and can be quite severe, particularly when there is an additional comorbidity. With the use of erythropoietin-stimulating agents (ESAs), anemia is effectively treated, but the complete normalization of hemoglobin is not recommended since these agents increase the risk of thrombosis. With improvements in the therapy of sickle cell disease (SCD), patients now survive longer and may more frequently reach end-stage renal disease and require renal replacement therapy...

BACKGROUND: Hemoglobinopathies, among which thalassemic syndromes (transfusion-dependent and non-transfusion dependent thalassemias) and sickle cell disease (SCD), are the most widespread monogenic diseases worldwide. Hemoglobinopathies are endemic and spread-out all-over Italy, as result of internal and external migration flows. Nowadays, the increase therapeutic options associated to the general aging of patients with hemoglobinopathies related to the improvement in clinical management, contribute to the abnormalities in kidney function going from blood and urine test alterations to chronic kidney disease and end stage renal disease...

BACKGROUND: Sickle cell disease is the most common inherited blood disorder in humans and constitutes a major public health burden. It is a multisystemic condition with long-term renal complications. Early detection of sickle cell nephropathy and initiation of appropriate interventions are associated with improved survival and quality of life. This study aimed to compare the cystatin C-derived estimated glomerular filtration rate (GFR) of the study groups and also, to correlate the clinical features of chronic kidney disease (CKD) with decreased GFR in children with sickle cell anaemia (SCA)...

Late presentations of liver failure were previously rare in clinical practice given the high mortality of sickle cell disease (SCD) and shorter life expectancy compared to the general population. With advancements in therapeutics for SCD, patients with SCD have increased lifespans, and with them, patients are placed at increased risk for differing patterns of chronic and end-organ failures. We describe a case of an elderly patient who had multiple chronic complications from her years of SCD, including end-stage renal disease (ESRD) on hemodialysis, acquired hemochromatosis, cirrhosis, and pulmonary hypertension...

BACKGROUND: Sickle cell disease is an inherited red blood cell disorder that affects approximately 100,000 people in the USA and 25 million people worldwide. Vaso-occlusion and chronic hemolysis lead to dysfunction of vital organ systems, with the kidneys being among the most commonly affected organs. SUMMARY: Early renal manifestations include medullary ischemia with the loss of urine-concentrating ability and hyperfiltration. This can be followed by progressive damage characterized by persistent albuminuria and a decline in the estimated glomerular filtration rate...

BACKGROUND: Sickle cell disease (SCD), one of the commonest severe monogenic disorders, is caused by the inheritance of two abnormal haemoglobin (beta-globin) genes. SCD can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Kidney disease is a frequent and potentially severe complication in people with SCD. Chronic kidney disease (CKD) is defined as abnormalities of kidney structure or function present for more than three months. Sickle cell nephropathy refers to the spectrum of kidney complications in SCD...

BACKGROUND: Literature on 30-day readmission in adults with sickle cell disease (SCD) is limited. This study examined the overall and age-stratified rates, risk factors, and healthcare resource utilization associated with 30-day readmission in this population. METHODS: Using the Nationwide Readmissions Database, a retrospective cohort study was conducted to identify adult patients (aged ≥ 18) with SCD in 2016. Patients were stratified by age and followed for 30 days to assess readmission following an index discharge...

Background: Children and young adults with sickle cell disease (SCD) develop kidney disease early in childhood with some patients progressing to require dialysis and kidney transplantation. The prevalence and outcomes of children with end stage kidney disease (ESKD) due to SCD is not well described. This study aimed to assess the burden and outcomes of ESKD in children and young adults with SCD in a large national database. Methods: Utilizing the United States Renal Data System (USRDS) we retrospectively examined ESKD outcomes in children and young adults with SCD from 1998 - 2019...

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We report the first population-based period life table, the expected lifetime survival for Medicare and Medicaid beneficiaries with Sickle Cell Disease (SCD), and the disparities in survival by insurance types in the United States. We constructed a retrospective cohort of individuals with diagnosed SCD receiving Common Care (any real-world patterns of care except transplant) based on nationwide Medicare and Medicaid claims data (2008-2016), covering beneficiaries in all 50 states. We analyzed lifetime survival probabilities using Kaplan-Meier curves and projected life expectancies at various ages for all and stratified by sex and insurance types...

Chronic kidney disease (CKD) has a significant impact on sickle cell disease (SCD) morbidity and mortality. Early identification of individuals at highest risk of developing CKD may allow therapeutic intervention to prevent worse outcomes. This study aimed to evaluate the prevalence and risk factors for reduced estimated glomerular filtration rate (eGFR) among adults with SCD in Brazil. Participants in the REDS-III multicenter SCD cohort with more severe genotypes aged ≥ 18 years with at least two serum creatinine values were analyzed...

Throughout the lifespan, the kidney of a person with sickle cell disease is at increased risk of injury to the glomeruli, tubules, and renal vasculature. This injury manifests as urine concentrating defects, enuresis, albuminuria, acute kidney injury, chronic kidney disease, and end-stage kidney disease. The outcomes for patients who progress to end-stage kidney disease are poor and access to organ transplantation is limited. Therefore, identifying risk factors for progression, intervening with renoprotective agents early in life, and improving access to care are vital for sickle cell patients...

Sickle cell nephropathy is one of the long-term complications of sickle cell disease (SCD). About a quarter of SCD patients who survive up to 40 years of age will require some form of renal replacement therapy in their lifetime. Organ transplantation in SCD patients poses great challenges, particularly in lower middle income countries (LMIC) like Nigeria. This report highlights the management of three SCD patients who successfully underwent renal transplantation. The patients were aged 39, 47, and 58 years, respectively, with similarly previous history of multiple blood transfusions, recurrent vaso-occlusive crises, and had all progressed to end-stage renal disease...

Patients with transfusion-dependent sickle cell disease (SCD) are at risk of iron overload and its complications. Iron overload is a significant risk factor for chronic liver disease in patients who are dependent on hemodialysis secondary to end-stage renal disease (ESRD). Deferasirox is being increasingly used as an iron-chelating agent for the treatment of iron overload in both adults and children. There are limited reports on its use in pediatric patients with ESRD. Here, we discuss the use of deferasirox to treat iron overload in a 15-year-old male with SCD, ESRD from granulomatosis with polyangiitis, and dependent on hemodialysis...

BACKGROUND: Sickle cell trait is typically considered benign. Although evidence remains inconsistent, recent studies suggest that it is associated with several common diseases. We systematically assessed associations of sickle cell trait with reported diseases in a large population-based cohort. METHODS: Study subjects were self-reported Blacks from the UK Biobank (UKB), a United Kingdom population-based cohort of subjects aged 40-69 years at recruitment in the United Kingdom...

A 61-year-old woman was admitted to the hospital for management of a painful vaso-occlusive crisis. She had a history of sickle cell beta-thalassaemia and end-stage renal disease managed with intermittent haemodialysis. While hospitalised, she became lethargic and unresponsive and developed acute chest syndrome. Initial MR scan of brain, cerebrospinal fluid examination and continuous electroencephalogram were unremarkable, but subsequent MR scan of brain identified a right transverse venous sinus thrombosis and extensive supratentorial and infratentorial microhaemorrhages consistent with fat emboli...

BACKGROUND A congenital hemolytic anemia, sickle cell disease can present with various clinical findings. Sickle cell disease is typically a disease of younger people and multiple myeloma typically occurs in older individuals. Multiple myeloma is rare among patients with sickle cell disease. Both multiple myeloma and sickle cell disease can cause various types of organ damage by different mechanisms. CASE REPORT We report a case of a patient who was born with sickle cell disease and presented with multiple myeloma later in life...

RATIONALE & OBJECTIVE: Apolipoprotein L1 ( APOL1 ) high-risk variants are associated with an increased risk for chronic kidney disease (CKD) among African Americans. Less is known regarding the risk for the development of CKD and kidney failure (end-stage kidney disease [ESKD]) among African Americans with only 1 APOL1 risk variant or whether the risk is modified by sickle cell trait. STUDY DESIGN: The Jackson Heart Study is a community-based longitudinal cohort study...

Clinical Background: Renal involvement in sickle cell disease (SCD), called sickle cell nephropathy (SCN), includes several renal manifestations, such as renal acidification defect, distal nephron dysfunction, renal papillary necrosis, and proteinuria related to glomerular injury, leading to end-stage renal disease. Epidemiology: Many patients with SCD have a defect in urinary concentration, a problem caused by the destruction of the renal medulla that initiates in childhood. The presence of proteinuria in SCD is age-related and starts as microalbuminuria in adolescence and progresses to macroalbuminuria...

There are over 12,000 people with sickle cell disease (SCD) in the UK, and 4-12% of patients who develop Sickle Cell Nephropathy (SCN) progress to End Stage Renal Disease (ESRD). Renal transplantation offers the best outcomes for these patients with but their access to transplantation is often limited. Regular automated exchange blood transfusions (EBT) reduce the complications of SCD and may improve outcomes. However, concerns over alloimmunisation limit its widespread implementation. In this retrospective multicenter study, data were collected on 34 SCD patients who received a kidney transplant across 6 London Hospitals between 1997 and 2017...