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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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
Introduction: Pediatric patients with Sickle Cell Anemia (SCA) are at risk for developing albuminuria. Hyperfiltration precedes the development of albuminuria in patients with diabetes but the natural history of hyperfiltration on the progression to albuminuria has not been studied in children with SCA.Methods: We have enrolled 185 participants with HbSS or SB0 thalassemia in a prospective pediatric cohort study evaluating progression to chronic kidney disease; the mean current age of participants in this cohort is 14 years. We have abstracted 817 urine microalbumin creatinine measurements and 891 estimations of GFR (eGFR)...
Source: Blood - Category: Hematology Authors: Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Organ Damage and Clinical Complications in Sickle Cell Disease Source Type: research
This study examined influenza vaccination in patients with SCD as a potential marker of quality of care delivery. The study population included black individuals aged 1 to =1 diagnosis of stroke, TIA or epilepsy/recurrent seizures); cardio pulmonary complications (>=1 diagnosis of chronic pulmonary heart disease, pulmonary hypertension, malaise and fatigue, edema, chest pain or hypoxemia); kidney disease (>=1 diagnosis of chronic kidney disease/renal failure, proteinuria), avascular necrosis or ulcer of lower limbs (adapted from Afenyi-Annan, 2008, Candrilli. 2011, Elmariah. 2014). A total of 1544 patients with SCD w...
Source: Blood - Category: Hematology Authors: Tags: 903. Outcomes Research-Non-Malignant Hematology: Poster II Source Type: research
Background: Sickle-cell disease (SCD) has been identified as a major public health problem by the World Health Organization (WHO) and is one of the most common hematological genetic diseases. In United States, there are approximately 100,000 admissions per year attributed to SCD. Our objective is to provide nationally representative rates for unplanned 30-day readmissions in SCD patients as well as identify reasons for, characteristics and predictors of these readmissions.Methods: We performed retrospective analysis using the 2013 Nationwide Readmissions Database (NRD) of the Healthcare Cost and Utilization Project (HCUP) ...
Source: Blood - Category: Hematology Authors: Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Poster I Source Type: research
CONCLUSIONS: In our cohort, determination of proteinurea evaluated by dipstick (16%) grossly underestimated CKD prevalence (35%) determined by eGFR and AL/CRE ratios. Subjects with HbSC had 3-fold less prevalence of CKD comparing to the patients with HbSS. ORM/CRE levels correlated with the stages of CKD stages (OR 2.72). Further longitudinal study is needed to determine whether ORM/CRE ratio can be used as a prognostic marker of renal disease development.ACKNOWLEDGMENTS: This work was supported by NIH Research Grants 1P50HL118006, 1R01HL125005 and 5G12MD007597. AT was supported by ASH MMSAP summer program. The content is ...
Source: Blood - Category: Hematology Authors: Tags: 113. Hemoglobinopathies, Excluding Thalassemia-Basic and Translational Science: Poster II Source Type: research
Conclusion:Rapid decline in eGFR is common in SCD. Use of ACE-I/ARB and history of stroke are associated with rapid decline in renal function. Rapid decline in eGFR is associated with an increased risk of death in SCD. Long-term studies are required to determine if therapies that may reduce loss of kidney function may decrease mortality in SCD.DisclosuresAtaga: Pfizer: Research Funding; Bioverativ: Honoraria, Membership on an entity's Board of Directors or advisory committees; Novartis Pharmaceuticals: Honoraria; Modus Therapeutics: Honoraria; Global Blood Therapeutics: Honoraria, Membership on an entity's Board of Directo...
Source: Blood - Category: Hematology Authors: Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster I Source Type: research
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