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Serum cystatin C levels in children with sickle cell disease
Ofelia Alvarez1, Gaston Zilleruelo, Dale Wright
1Division of Pediatric Hematology, University of Miami, 1611 NW 12th Avenue, ACC West, Rm 514, Miami, FL 33136, USA. oalvarez2@med.miami.edu
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Serum cystatin C effectively assesses kidney function in children with sickle cell disease (SCD). It correlates with albuminuria levels, offering a reliable marker for glomerular filtration rate (GFR) in pediatric SCD patients.
Area of Science:
- Pediatric Nephrology
- Hematology
Background:
- Sickle cell disease (SCD) is associated with progressive kidney damage starting in childhood.
- Assessing glomerular filtration rate (GFR) is crucial for managing SCD-related renal complications.
Purpose of the Study:
- To evaluate serum cystatin C as a marker for GFR in pediatric SCD patients.
- To compare the efficacy of serum cystatin C against serum creatinine and creatinine clearance (CrCl) for assessing renal function in this population.
Main Methods:
- A cohort of twenty children (ages 9-21) with SCD, with and without albuminuria, was studied.
- Serum cystatin C, serum creatinine, and CrCl levels were measured.
- GFR was estimated using serum cystatin C and calculated via CrCl.
Main Results:
- Serum cystatin C levels varied significantly across subgroups with different levels of albuminuria (proteinuria, microalbuminuria, no albuminuria).
- GFR derived from serum cystatin C showed abnormal values in the proteinuric group, unlike serum creatinine and CrCl.
- Serum creatinine and CrCl did not significantly reflect the varying degrees of albuminuria.
Conclusions:
- Serum cystatin C demonstrates a strong correlation with albuminuria levels in children with SCD.
- Serum cystatin C appears to be a reliable and sensitive indicator for monitoring renal function in pediatric SCD patients.
Abstract:
Patients with sickle cell disease (SCD) may develop kidney dysfunction from childhood. The purpose of this study was to examine the value of serum cystatin C as a marker for glomerular filtration rate (GFR) in children with SCD, as compared to serum creatinine and creatinine clearance (CrCl). Twenty children (ages 9-21, ten males) with SCD with and without albuminuria were studied. The mean serum cystatin for the whole group was 0.89 mg/l (0.5-1.7 mg/l). Mean serum cystatin C was significantly different among the children with proteinuria (n=4), microalbuminuria (n=5), and without albuminuria (n=11) (1.25 mg/l, 0.84 mg/l, and 0.78 mg/l, respectively). The mean GFR derived from serum cystatin was significantly different among these subgroups, becoming abnormal in the proteinuric cohort (63 ml/min per 1.73 m2), in contrast to 94 for the microalbuminuric, and 103 for the normal subgroups. Serum creatinine (mean: 0.58 mg/dl, range: 0.3-1.1) did not change significantly with the level of albuminuria. Mean CrCl remained normal to increased within the subgroups, (133 ml/min per 1.73 m2 for those with proteinuria, 144 for those with microalbuminuria, and 163 for the normal subgroup). We conclude that serum cystatin C correlates with the level of albuminuria and may be a reliable method to measure renal function in SCD.
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