Endothelial Function and Vascular Properties in Children with Sickle Cell Disease

Hadeed, Khaled; Hascoët, Sébastien; Castex, Marie‐Pierre; Munzer, Caroline; Acar, Philippe; Dulac, Yves

doi:10.1111/echo.12851

Cited by 14 publications

(10 citation statements)

References 30 publications

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“…Vascular remodeling and intimal thickening are known complications of SCA that appear later in life and correlate with age. 37,38 Therefore, it is unlikely that vascular remodeling is the sole explanation of this degree of ECV elevation in our study, especially in young patients with high ECV.…”

Section: Discussionmentioning

confidence: 68%

Association between diffuse myocardial fibrosis and diastolic dysfunction in sickle cell anemia

Niss¹,

Fleck²,

Makue

et al. 2017

Blood

116

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Sickle cell anemia (SCA)-related cardiomyopathy is characterized by diastolic dysfunction and hyperdynamic features. Diastolic dysfunction portends early mortality in SCA. Diastolic dysfunction is associated with microscopic myocardial fibrosis in SCA mice, but the cause of diastolic dysfunction in humans with SCA is unknown. We used cardiac magnetic resonance measurements of extracellular volume fraction (ECV) to discover and quantify diffuse myocardial fibrosis in 25 individuals with SCA (mean age, 23 ± 13 years) and determine the association between diffuse myocardial fibrosis and diastolic dysfunction. ECV was calculated from pre- and post-gadolinium T1 measurements of blood and myocardium, and diastolic function was assessed by echocardiography. ECV was markedly increased in all participants compared with controls (0.44 ± 0.08 vs 0.26 ± 0.02, < .0001), indicating the presence of diffuse myocardial fibrosis. Seventeen patients (71%) had diastolic abnormalities, and 7 patients (29%) met the definition of diastolic dysfunction. Participants with diastolic dysfunction had higher ECV (0.49 ± 0.07 vs 0.37 ± 0.04, = .01) and N-terminal pro-brain natriuretic peptide (NT-proBNP; 191 ± 261 vs 33 ± 33 pg/mL, = .04) but lower hemoglobin (8.4 ± 0.3 vs 10.9 ± 1.4 g/dL, = .004) compared with participants with normal diastolic function. Participants with the highest ECV values (≥0.40) were more likely to have diastolic dysfunction ( = .003) and increased left atrial volume (57 ± 11 vs 46 ± 12 mL/m, = .04) compared with those with ECV<0.4. ECV correlated with hemoglobin ( = -0.46, = .03) and NT-proBNP ( = 0.62, = .001). In conclusion, diffuse myocardial fibrosis, determined by ECV, is a common and previously underappreciated feature of SCA that is associated with diastolic dysfunction, anemia, and high NT-proBNP. Diffuse myocardial fibrosis is a novel mechanism that appears to underlie diastolic dysfunction in SCA.

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Section: Discussionmentioning

confidence: 68%

Association between diffuse myocardial fibrosis and diastolic dysfunction in sickle cell anemia

Niss¹,

Fleck²,

Makue

et al. 2017

Blood

116

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“…Thus, reduced bioavailability of NO causes variation in vessel diameter adjustment, which potentially exacerbates the vaso-occlusive process [37]. Hadeed et al [40]did not find ED in children with SCD, however, FMD value tended to be lower in SCD than in controls. The authors suggesting that these manifestations may be related to disease severity and duration.…”

Section: Discussionmentioning

confidence: 99%

Sensorineural hearing loss in children with sickle cell anemia and its association with endothelial dysfunction

et al. 2018

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“…In the study by Zawar et al (10), mean age was 23.1565.27 years old (sample size 537), and in the study by Al-Janabi et al (6), mean age was27.068.9 years old (sample size 530). However, Hadeed et al (9) in France studied 30 younger children with SCD (mean age of 12.364.5 years old) and found no significant difference in FMD between the patients with SCD and age-and sex-matched controls, concluding that manifestations of ED may be more evident in life as the disease progresses. However, their finding was at variance with that of an earlier French study (8) in children (mean age 510.463.3 years old; sample size 521), which reported that FMD was significantly lower in children with sickle cell anemia than in controls and that it did not correlate with age (8).…”

Section: Discussionmentioning

confidence: 97%

“…Endothelial dysfunction (ED) is a known feature of SCD, which is present both in crisis and in steady state (7,8). It has been demonstrated in both children and adults with SCD (8,9), and it is more severe in patients with sickle cell anemia than in patients with sickle cell trait (10). Impaired (reduced) sonographic brachial artery flow-mediated dilation (FMD) is a recognized biomarker for ED in SCD (11).…”

Section: Introductionmentioning

confidence: 99%

Association between Endothelial Dysfunction, Biomarkers of Renal Function, and Disease Severity in Sickle Cell Disease

et al. 2020

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Background Endothelial dysfunction (ED), as ascertained by brachial artery flow-mediated dilation (FMD), is a known feature of sickle cell disease (SCD), which is present both in crisis and in steady state. The assessment of FMD was introduced to examine the vasodilator function. Our objective was to establish the relationship between ED determined by FMD, biomarkers of renal dysfunction, and biomarkers of disease severity in SCD subjects asymptomatic of renal disease. Methods We enrolled 44 patients with homozygous SCD in steady state and 33 age-and sex-matched controls between 2013 and 2014 in a tropical tertiary hospital. Ultrasonographic FMD of the right brachial artery, renal arterial Doppler, complete blood count, creatinine, fetal hemoglobin, soluble P-selectin, and cystatin C (Cys-C) levels were determined. Using the median FMD value of the control group, the SCD subjects were further classified into two groups for comparison. Results The median FMD in SCD subjects of 3.44 (IQR, 0.00-7.08) was significantly lower than that of controls, which was 5.35 (IQR, 3.60-6.78; P50.04). There was negative correlation between FMD and Cys-C levels (r520.372; P50.01) along with renal artery resistivity index (RARI; r520.307; P50.04) in SCD subjects. Additionally, Cys-C level was significantly higher in SCD subjects with FMD,5.35. Conclusions Brachial artery FMD was significantly lower in SCD subjects compared with a control group. Cys-C and RARI show a negative correlation with FMD, indicating that renal function is related to ED in SCD.

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Endothelial Function and Vascular Properties in Children with Sickle Cell Disease

Abstract: Children with SCD have no marked endothelial dysfunction or change in arterial stiffness. These manifestations may be related to disease severity and duration. Changes may become evident later in life as the disease progresses.

Cited by 14 publications

References 30 publications

Association between diffuse myocardial fibrosis and diastolic dysfunction in sickle cell anemia

Association between diffuse myocardial fibrosis and diastolic dysfunction in sickle cell anemia

Sensorineural hearing loss in children with sickle cell anemia and its association with endothelial dysfunction

Association between Endothelial Dysfunction, Biomarkers of Renal Function, and Disease Severity in Sickle Cell Disease

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