Urinary Orosomucoid is Associated with Progressive Chronic Kidney Disease Stage in Patients with Sickle Cell Anemia

Source: American Journal of Hematology - Category: Hematology Authors: Tags: Correspondence Source Type: research

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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
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
Introduction: Gene variants in the apolipoprotein L-1 (APOL1) gene are strong modifiers for the development of chronic kidney disease in individuals of African descent and are associated with progression of renal disease and albuminuria in cross-sectional studies of individuals with sickle cell anemia (SCA). While the association of APOL1 with albuminuria in older SCA patients is established, it is unclear whether participants with APOL1 G1 (rs73885319/ rs6090145) and G2 (rs71785313) variants (Ashley-Koch Br J Hematol 2011; Kormann Br J Haematol 2017) are more likely to develop albuminuria early in life. We hypothesized th...
Source: Blood - Category: Hematology Authors: Tags: 114. Hemoglobinopathies, Excluding Thalassemia-Clinical: Poster II Source Type: research
AbstractDiabetes mellitus (DM) has reached epidemic proportions across the globe with the largest increases seen in sub-Saharan Africa. Those that are diagnosed are largely poorly controlled. This review summarizes the limitations of the use of glycated haemoglobin (HBA1c) in Africa and current knowledge on the utility of glycated albumin and fructosamine in African patients. The diagnosis and monitoring of DM in African patients may be compromised by associated conditions like sickle cell anaemia, chronic kidney disease and HIV infection. Glycated albumin reflects short term glycaemia and is not affected by many condition...
Source: Indian Journal of Clinical Biochemistry - Category: Biochemistry Source Type: research
by Laura M. Raffield, Jacob C. Ulirsch, Rakhi P. Naik, Samuel Lessard, Robert E. Handsaker, Deepti Jain, Hyun M. Kang, Nathan Pankratz, Paul L. Auer, Erik L. Bao, Joshua D. Smith, Leslie A. Lange, Ethan M. Lange, Yun Li, Timothy A. Thornton, Bessie A. Young, Goncalo R. Abecasis, Cathy C. Laurie, Deborah A. Nickerson, Steven A. McCarroll, Adolfo Correa, James G. Wilson, NHLBI Trans-Omics for Precision Medicine (TOPMed) Consortium, Hematology&Hemostasis, Diabetes, and Structural Variation TOPMed Working Groups , Guillaume Lettre, Vijay G. Sankaran, Alex P. Reiner Co-inheritance of α-thalassemia has a significant p...
Source: PLoS Genetics - Category: Genetics & Stem Cells Authors: Source Type: research
American Journal of Hematology,Volume 93, Issue 4, Page E107-E109, April 2018.
Source: American Journal of Hematology - Category: Hematology Source Type: research
CONCLUSION: The SS form of the disease, recurrent vaso-occlusive crisis, male sex and advanced age were the main precipitating factors in the development of renal function disorders. PMID: 29311011 [PubMed - as supplied by publisher]
Source: Nephrologie and Therapeutique - Category: Urology & Nephrology Authors: Tags: Nephrol Ther Source Type: research
Authors: Gladwin MT Abstract Sickle cell disease (SCD) is an autosomal recessive disease in which homozygosity for a single point mutation in the gene encoding the β-globin chain produces hemoglobin S molecules that polymerize within the erythrocyte during deoxygenation; the result is sustained hemolytic anemia and vaso-occlusive events. As patients live to adulthood, the chronic impact of sustained hemolytic anemia and episodic vaso-occlusive episodes leads to progressive end-organ complications. This scenario culminates in the development of 1 or more major cardiovascular complications of SCD for which there...
Source: Hematology ASH Education Program - Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research
Suicidal erythrocyte death or eryptosis contributes to or even accounts for anemia in a wide variety of clinical conditions, such as iron deficiency, dehydration, hyperphosphatemia, vitamin D excess, chronic kidney disease (CKD), hemolytic-uremic syndrome, diabetes, hepatic failure, malignancy, arteriitis, sepsis, fever, malaria, sickle-cell disease, beta-thalassemia, Hb-C and G6PD-deficiency, Wilsons disease, as well as advanced age. Moreover, eryptosis is triggered by a myriad of xenobiotics and endogenous substances including cytotoxic drugs and uremic toxins. Eryptosis is characterized by cell membrane scrambling with ...
Source: Cellular Physiology and Biochemistry - Category: Cytology Source Type: research
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