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Burden of diagnostic radiation exposure in children with sickle cell disease.

ABSTRACT: Children with sickle cell disease (SCD) are repeatedly exposed to diagnostic radiation. We identified 938 children with SCD who had 9,246 radiographic tests. Mean number of tests/patient was 9.9 (95% CI: 8.9-10.9) over 8,817 patient-years. Mean rate was 1.5 tests/year (95% CI: 1.3-1.6). On average, a child with SCD will have 26.7 (95% CI: 24.1-29.3) radiographic tests by 18 years of age, and 5% will have ?100 tests. Six percent have ?3 CT scans, which may be associated with an increased risk of cancer. Strong consideration should be given to limiting the exposure of children with SCD to radiation.

SUBMITTER: Vetter CL

PROVIDER: S-EPMC4008662 | biostudies-literature | 2014 Jul

REPOSITORIES: biostudies-literature

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Burden of diagnostic radiation exposure in children with sickle cell disease.

Vetter Courtney L CL Buchanan George R GR Quinn Charles T CT

Pediatric blood & cancer 20140122 7

Children with sickle cell disease (SCD) are repeatedly exposed to diagnostic radiation. We identified 938 children with SCD who had 9,246 radiographic tests. Mean number of tests/patient was 9.9 (95% CI: 8.9-10.9) over 8,817 patient-years. Mean rate was 1.5 tests/year (95% CI: 1.3-1.6). On average, a child with SCD will have 26.7 (95% CI: 24.1-29.3) radiographic tests by 18 years of age, and 5% will have ≥100 tests. Six percent have ≥3 CT scans, which may be associated with an increased risk of ...[more]

PMID: 24453133

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Project description: Not available

| S-EPMC9426816 | biostudies-literature

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Proteomic discovery in sickle cell disease: Elevated neurogranin levels in children with sickle cell disease.

Project description:PurposeSickle cell disease (SCD) is an inherited hemoglobinopathy that causes stroke and silent cerebral infarct (SCI). Our aim was to identify markers of brain injury in SCD.Experimental designPlasma proteomes were analyzed using a sequential separation approach of hemoglobin (Hb) and top abundant plasma protein depletion, followed by reverse phase separation of intact proteins, trypsin digestion, and tandem mass spectrometry. We compared plasma proteomes of children with SCD with and without SCI in the Silent Cerebral Infarct Multi-Center Clinical Trial (SIT Trial) to age-matched, healthy non-SCD controls.ResultsFrom the SCD group, 1172 proteins were identified. Twenty-five percent (289/1172) were solely in the SCI group. Twenty-five proteins with enriched expression in the human brain were identified in the SCD group. Neurogranin (NRGN) was the most abundant brain-enriched protein in plasma of children with SCD. Using a NRGN sandwich immunoassay and SIT Trial samples, median NRGN levels were higher at study entry in children with SCD (0.28 ng/mL, N = 100) compared to control participants (0.12 ng/mL, N = 25, p < 0.0004).Conclusions and clinical relevanceNRGN levels are elevated in children with SCD. NRGN and other brain-enriched plasma proteins identified in plasma of children with SCD may provide biochemical evidence of neurological injury.

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Project description:Sickle cell disease (SCD) is caused by a pathogenic hemoglobin (Hb) mutation, yet patients can have dramatically variable clinical manifestations. Here we address the genetic basis of this clinical heterogeneity. Using a systems genetics approach, we performed whole blood gene expression analysis and eQTL analysis on different clinical phenotypes in SCD patients. We generated whole blood gene expression profiles for 311 West-African children recruited from the National Sickle Cell Disease Centre in Cotonou, Benin which included 250 patients with varying degrees of SCD severities and 61 age-matched controls. SCD is caused by a point-mutation in the beta-hemoglobin gene that changes the normal HbAA protein into, most often, an abnormal HbSS or HbSC protein. The SCD patients recruited in the study either had HbSS or HbSC phenotypes and were categorized into different 3 clinical states based on follow-up status (Rahimy, MC, et al. Effect of a comprehensive clinical care program on disease course in severely ill children with sickle cell anemia in sub-Saharan African setting. Bood 102, 834-838. 2002). When patients are refered to the clinic, they are enrolled when they are in steady-state condition, and are labeled as entry (E). Patients followed at the SCD clinic are labeled as FU. Control patients were recruited and are labeled as C. Patients were also assigned a severity score (Sebastiani, P. et al. A network model to predict the risk of death in sickle cell disease. Blood 110, 2727-2735, 2007). Hemoglobin protein status (Hb phenotype) was confirmed for each patient using standard electrophoretic techniques. We generated genotypes for 263 of these individuals and performed principal component analysis (PCA) which identified 2 signigicant genotypic principal components (gPC1 and gPC2). Using the gene expression and genotyping data, we performed an eSNP analysis. . Gene expression data presented in this study.

2014-07-01 | E-GEOD-35007 | biostudies-arrayexpress

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