scholarly journals Hyperuricemia and abnormal nocturnal dipping impact glomerular filtration rate in patients with sickle cell anemia

2021 ◽  
Author(s):  
Jeffrey D. Lebensburger ◽  
Inmaculada Aban ◽  
Lee M. Hilliard ◽  
Daniel I. Feig
Keyword(s):  
Glomerular Filtration Rate ◽  
Glomerular Filtration ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Filtration Rate ◽  
Nocturnal Dipping

Elevated Glomerular Filtration Rate (GFR) in Young Patients with Sickle Cell Anemia.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3745-3745
Author(s):  
Sherri A. Zimmerman ◽  
Jacqueline S. Davis ◽  
Nicole A. Mortier ◽  
Russell E. Ware
Keyword(s):  
Renal Function ◽  
Glomerular Filtration Rate ◽  
Young Children ◽  
Glomerular Filtration ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Filtration Rate ◽  
Plasma Clearance ◽  
Glomerular Hyperfiltration ◽  
Schwartz Equation

Abstract Nephropathy is a well-recognized complication of sickle cell anemia (SCA) that is associated with considerable morbidity and mortality. Sickle nephropathy begins early in life, with glomerular damage characterized by hyperfiltration and glomerulomegaly, as well as tubular damage characterized by hyposthenuria. School-aged children can develop proteinuria and one-third of patients will eventually develop chronic renal failure as adults. Among the earliest markers of sickle nephropathy is glomerular hyperfiltration, typically measured as an elevated glomerular filtration rate (GFR). To date, however, no formal measurements of GFR have been published in young children with SCA, and its feasibility and interpretation in this age group have not been demonstrated. As part of a prospective, single-institution, IRB-approved open-label protocol using hydroxyurea in toddlers with SCA, the pre-treatment GFR was measured using plasma clearance of 99-Tc DTPA. The goal of this procedure was to determine the onset of hyperfiltration among young children with SCA, to identify risk factors associated with its onset, and to investigate the potential benefit of hydroxyurea in improving or preserving renal function. After intravenous injection of the DTPA radiotracer, 3–5 mL aliquots of venous blood were removed at 1 and 3 hours post-injection and analyzed for plasma radioactivity. Because DTPA is filtered at the glomerulus without substantial metabolism, secretion, or reabsorption, the plasma clearance allows an accurate and precise GFR measurement. The GFR was also estimated using the Schwartz equation, where GFR = height (cm) x k/serum creatinine, with k=0.55 for children between ages 1 and 12 years. A total of 13 children with HbSS (3 females, 10 males) were enrolled in this study, none of whom had laboratory evidence of renal disease at the time of evaluation. One child could not complete the DTPA study due to inadequate venous access. For the remaining 12 children, baseline GFR measurements were performed at age 3.0 ± 0.8 years (range 1.7 to 4.4 years) without complications. The average GFR measurement (mean ± SD) by DTPA clearance was 140.3 ± 20 mL/min/1.73m2, median 133 mL/min/1.73m2, range 117.9 to 172.7 mL/min/1.73m2 (normal 100 ± 20 mL/min/1.73m2). The baseline DTPA GFR measurement was elevated above 150 mL/min/1.73m2 in 5 of the 12 children, including 4 of 7 over age 3 years, although there was no signfiicant correlation between GFR and age or fetal hemoglobin. GFR estimates by the Schwartz equation were modestly correlated with the DTPA GFR measurements (R2 = 0.32, p = 0.055) but were typically slightly higher than the corresponding DTPA measurements. Three children who completed 24 months of hydroxyurea therapy had post-treatment DTPA clearance studies that revealed stable GFR measurements (average increase = 5.6 mL/min/1.73m2). These results illustrate that GFR measurement by DTPA clearance can be performed without difficulty in young children with SCA, requiring only peripheral intravenous access. Glomerular hyperfiltration as a manifestation of renal damage begins early in life for children with SCA, with elevated GFR values observed in the toddler age range. The Schwartz equation provides an estimate of GFR but probably cannot be used in lieu of the DTPA clearance study. Treatment with hydroxyurea may preserve renal function by abrogating further GFR hyperfiltration.

scholarly journals High bias and low precision for estimated versus measured glomerular filtration rate in pediatric sickle cell anemia

Haematologica ◽  
2020 ◽  
Vol 106 (1) ◽  
pp. 295-298
Author(s):  
Jeffrey D. Lebensburger ◽  
Jeffrey Gossett ◽  
Rima Zahr ◽  
Winfred C. Wang ◽  
Kenneth I. Ataga ◽  
...  
Keyword(s):  
Glomerular Filtration Rate ◽  
Glomerular Filtration ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Filtration Rate ◽  
Measured Glomerular Filtration Rate ◽  
High Bias

scholarly journals Sex differences in the trajectory of glomerular filtration rate in pediatric and murine sickle cell anemia

2020 ◽  
Vol 4 (2) ◽  
pp. 263-265
Author(s):  
Malgorzata Kasztan ◽  
Inmaculada Aban ◽  
Suraj P. Hande ◽  
David M. Pollock ◽  
Jeffrey D. Lebensburger
Keyword(s):  
Sex Differences ◽  
Glomerular Filtration Rate ◽  
Glomerular Filtration ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Filtration Rate

Important clinical and laboratory correlates of glomerular filtration rate in sickle cell anemia

2015 ◽  
Vol 18 (5) ◽  
pp. 633 ◽  
Author(s):  
AJ Madu ◽  
A Ubesie ◽  
S Ocheni ◽  
J Chinawa ◽  
KA Madu ◽  
...  
Keyword(s):  
Glomerular Filtration Rate ◽  
Glomerular Filtration ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Filtration Rate

Evaluation of Renal Function by Glomerular Filtration Rate (GFR) in Infants with Sickle Cell Anemia.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1672-1672
Author(s):  
Winfred C. Wang ◽  
Renee R. Rees ◽  
Daner Li ◽  
Zora Rogers ◽  
Rathi Iyer ◽  
...  
Keyword(s):  
Renal Function ◽  
Glomerular Filtration Rate ◽  
Glomerular Filtration ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Filtration Rate ◽  
Plasma Clearance ◽  
Renal Damage ◽  
Phase Iii ◽  
Schwartz Equation

Abstract Chronic anemia and intraparenchymal sickling within the kidney lead to intravascular volume expansion and an increased glomerular filtration rate (GFR) in sickle cell anemia (SCA). An elevated GFR is considered to be an early indicator of renal damage in SCA, and the pathophysiologic changes leading to sickle nephropathy and elevated GFR likely begin at a young age. The Pediatric Hydroxyurea Phase III Clinical Trial (BABY HUG), an NHLBI-sponsored multi-center double-blinded placebo-controlled study, compares hydroxyurea versus placebo in infants with SCA, with the primary goal of determining the efficacy of hydroxyurea for the prevention of organ dysfunction in the spleen and kidney. In the Feasibility and Safety Pilot, a primary objective is to assess GFR in infants with SCA between the age of 12 and 18 months by measuring plasma clearance of 99m Tc-DTPA (diethylenetriaminepentaacetic acid) and by estimating GFR using the Schwartz equation. The DTPA GFR was determined following administration of an IV bolus of 25–50 μCi/kg of the radiotracer, with venous blood samples obtained at 1, 2, and 4 hours. GFR was also calculated using the Schwartz equation: 0.55 x body length (cm) ÷ plasma creatinine (mg/dL). For both measurements, a logarithmic transformation was applied to improve linearity between the variables, and to stabilize the variance of the transformed data. To date, 17 infants with SCA (median age 13.2 months) have had GFR measurements, with no complications occurring. The geometric mean of the GFR (± SD) as measured by DTPA plasma clearance was 112 ± 14.6 mL/min/1.73m2 (range 53–178 mL/min/1.73m2). By regression analysis, the GFR was correlated with age, with an increase of approximately 10% per month (univariate p = 0.006, multivariate p = 0.02), and this correlation could not be ascribed to other age-adjusted changes in hemoglobin concentration (p = 0.35), % fetal hemoglobin (HbF, p = 0.67), white blood cell (WBC) count (p = 0.64), or platelet count (p = 0.76). The estimated GFR calculated by the Schwartz equation was not significantly correlated with age (univariate p = 0.12), and adjustments using hemoglobin, %HbF, platelet or WBC counts did not improve the correlation. There was a modest correlation between GFR determined by DTPA and the Schwartz equation (r = 0.44; p = 0.08). These data indicate that (1) GFR measurement using DTPA plasma clearance is feasible in one year-old infants with SCA; (2) renal damage as measured by an elevated DTPA GFR appears to be present early in life and to be increasing with age; (3) preliminary evaluation of the use of the Schwartz formula indicates only a modest level of correlation with results obtained using DTPA measurements; and (4) in the BABY HUG trial, further evaluation of the efficacy of hydroxyurea in preservation of renal function will likely require DTPA GFR measurements rather than GFR estimates using the Schwartz equation.

scholarly journals Rapid decline in estimated glomerular filtration rate in sickle cell anemia: results of a multicenter pooled analysis

Haematologica ◽  
2020 ◽  
pp. 0-0
Author(s):  
Kenneth I. Ataga ◽  
Qingning Zhou ◽  
Vimal K. Derebail ◽  
Santosh L. Saraf ◽  
Jane S. Hankins ◽  
...  
Keyword(s):  
Glomerular Filtration Rate ◽  
Glomerular Filtration ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Filtration Rate ◽  
Estimated Glomerular Filtration Rate ◽  
Pooled Analysis ◽  
Rapid Decline

Not available.

GLOMERULAR FILTRATION RATE IN CHILDREN WITH SICKLE CELL DISEASE

PEDIATRICS ◽  
1950 ◽  
Vol 5 (1) ◽  
pp. 127-129
Author(s):  
PHILIP L. CALCAGNO ◽  
JOHN MCLAVY ◽  
THOMAS KELLEY
Keyword(s):  
Renal Function ◽  
Glomerular Filtration Rate ◽  
Sickle Cell Disease ◽  
Glomerular Filtration ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Filtration Rate ◽  
Sickle Cell Trait ◽  
Cell Disease

Renal function has been studied quantitatively in five patients with sickle cell anemia, all of whom were proved to have reduced glomerular filtration rates. Two patients with sickle cell trait revealed normal glomerular filtration rates. Possible mechanisms responsible for reduced glomerular filtration flows are discussed.

scholarly journals Estimation of glomerular filtration rate using serum cystatin C and creatinine in adults with sickle cell anemia

2017 ◽  
Vol 92 (10) ◽  
pp. E598-E599 ◽  
Author(s):  
Marianne E. M. Yee ◽  
Peter A. Lane ◽  
David R. Archer ◽  
Clinton H. Joiner ◽  
James R. Eckman ◽  
...  
Keyword(s):  
Glomerular Filtration Rate ◽  
Glomerular Filtration ◽  
Sickle Cell ◽  
Cystatin C ◽  
Sickle Cell Anemia ◽  
Filtration Rate ◽  
Serum Cystatin C ◽  
Serum Cystatin
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