Sickle Cell Nephropathy in the Pediatric Population

Background: Compared to the past, patients with sickle cell disease (SCD) currently live longer due to improvements in diagnosis and comprehensive care. Due to these advances, long-term chronic complications pose a greater challenge in the management of patients with SCD. In particular, sickle cell nephropathy (SCN) is associated with significant morbidity and mortality across all age groups. Furthermore, SCN is an understudied condition with relatively few symptoms and therefore requires close surveillance. In this review, we sought to explore the epidemiology, natural history, and treatment options for SCN with an emphasis on the pediatric population.Summary: SCN invariably begins in childhood with evidence of structural changes detected as early as infancy. These indolent changes can progress undetected to advanced chronic kidney disease by late adolescence or early adulthood. The risk factors for progression are not well defined, but significant albuminuria (which is also the most common presentation in childhood) is a key factor in progression. One of the main challenges in understanding SCN in children is the poor correlation between estimated and measured glomerular filtration rates. Another challenge is the lack of large-scale longitudinal studies that track the clinical outcomes of pediatric patients over time. Several studies aim to identify early biomarkers of SCN in children, as albuminuria presents only following significant chronic damage. The utility of angiote...
Source: Blood Purification - Category: Hematology Source Type: research

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Publication date: Available online 21 February 2019Source: American Journal of Kidney DiseasesAuthor(s): Vimal K. Derebail, Emily J. Ciccone, Qingning Zhou, R. Rosina Kilgore, Jianwen Cai, Kenneth I. AtagaRationale &ObjectiveProgression of chronic kidney disease (CKD) in sickle cell disease (SCD) and its risk factors remain poorly defined. We identified characteristics associated with CKD as well as decline in estimated glomerular filtration rate (eGFR) and presence of proteinuria over time in adults with SCD.Study DesignRetrospective observational study.Setting &ParticipantsPatients with SCD 18 years or older in a...
Source: American Journal of Kidney Diseases - Category: Urology & Nephrology Source Type: research
Conclusion: SCT was not associated with increased risk of renal dysfunction among young adults in Nigeria. Further studies are needed to clarify the controversy, especially in Nigeria, with a relatively higher prevalence of SCT.
Source: Nigerian Journal of Clinical Practice - Category: Rural Health Authors: Source Type: research
Conclusion: SCT is associated with an increased risk for CAD in African American males with CKD. The excess risk in males with SCT appears to follow the same pattern as risk in males with SCD. Larger studies are needed to confirm these findings.Am J Nephrol 2019;49:93 –102
Source: American Journal of Nephrology - Category: Neurology Source Type: research
Abstract BACKGROUND: Sickle cell trait (SCT) is common among African Americans and has been historically considered to be benign. Recently, SCT has been associated with an increased risk for chronic kidney disease (CKD) and cardiovascular disease in the general population. Our understanding of SCT has been extrapolated largely from data of patients with sickle cell disease (SCD). Notably, in SCD, the outcomes differ by sex. The effect of SCT on cardiovascular risk in the African American CKD population is unknown, and the interaction between SCT and sex on cardiovascular risk has not been investigated. M...
Source: American Journal of Nephrology - Category: Urology & Nephrology Authors: Tags: Am J Nephrol Source Type: research
ConclusionThe present study has revealed a high prevalence of CKD amongst patients with SCA in this region. Various clinical and laboratory predictors of eGFR were also identified. Monitoring and detection of early stages of these groups of patients may allow for interventions which may delay progression into advance stages and ESRD.
Source: Mediterranean Journal of Hematology and Infectious Diseases - Category: Hematology Source Type: research
Authors: Pecker LH, Naik RP Abstract Sickle cell trait (SCT) is unique among the carrier states that are identified during newborn screening. Unlike other heterozygous states for rare recessive diseases, SCT is exceedingly prevalent throughout regions of the world, making sickle cell disease one of the most common monogenetic diseases worldwide. Because of this high frequency, reproductive counseling is of paramount importance. In addition, unlike other carrier states, SCT seems to be a risk factor for several clinical complications, such as extreme exertional injury, chronic kidney disease, and venous thromboembol...
Source: Hematology ASH Education Program - Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research
Sickle cell trait (SCT) is unique among the carrier states that are identified during newborn screening. Unlike other heterozygous states for rare recessive diseases, SCT is exceedingly prevalent throughout regions of the world, making sickle cell disease one of the most common monogenetic diseases worldwide. Because of this high frequency, reproductive counseling is of paramount importance. In addition, unlike other carrier states, SCT seems to be a risk factor for several clinical complications, such as extreme exertional injury, chronic kidney disease, and venous thromboembolism. Increasing knowledge about these clinica...
Source: Blood - Category: Hematology Authors: Tags: Red Cells, Iron, and Erythropoiesis, Review Articles Source Type: research
In this study, we have performed an exploratory study to identify urinary biomarkers, which may be predictive of kidney damage in children with SCD.Methods: Consecutive patients with SCD seen at a pediatric chronic care clinic at Kamuzu Central Hospital in Lilongwe, Malawi, between January and May 2015 were evaluated. Patient urine samples were analyzed for metabolites using NMR-based methodologies. Spot urine measurements for albumin/creatinine ratio (UACR) were required to ascertain the degree of albuminuria (normal albuminuria [UACR
Source: Blood - Category: Hematology Authors: Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster III Source Type: research
In this study, all patients who were hospitalized between the years 2000 to 2015 were analyzed and mortality was studied in SCD patients. Mortality during hospital stay was studied with respect to hospital size, rural vs. urban location and the most common medical comorbidities. Data pertaining to demographic factors of gender, geographic location and hospital size (measure of expertise and facilities available) were extracted. Access to healthcare was assessed using insurance coverage as a surrogate and its effect on mortality was evaluated. The effect of common medical comorbid conditions such as atrial fibrillation (Afi...
Source: Blood - Category: Hematology Authors: Tags: 903. Outcomes Research-Non-Malignant Hematology: Poster III Source Type: research
BackgroundSickle cell disease (SCD) is a monogenetic disorder, with 300,000 to 400,000 infants born annually with the disease around the world (Kato et al, Nat Rev Dis Primers 2018). SCD is a progressively debilitating and life-threatening disease characterized by hemolytic anemia, painful vaso-occlusive events (VOEs) and persistent vasculopathy that result in significant morbidity, poor quality of life and early mortality. There is significant heterogeneity in clinical manifestations of SCD, given that vaso-occlusion and hemolysis-associated endothelial dysfunction may manifest in any tissue or organ. Severely affected pa...
Source: Blood - Category: Hematology Authors: Tags: 903. Outcomes Research-Non-Malignant Hematology: Poster III Source Type: research
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