Pattern of Lung Function Is Not Associated with Prior or Future Morbidity in Children with Sickle Cell Anemia

Cohen, Robyn T.; Strunk, Robert C.; Rodeghier, Mark; Rosen, Carol L.; Kirkham, Fenella J.; Kirkby, Jane; DeBaun, Michael R.

doi:10.1513/annalsats.201510-706oc

Cited by 44 publications

(43 citation statements)

References 42 publications

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“…Low FEV1/FVC is a marker of airway obstruction that affects about one in five children with SCD 13,45 . Reduction in lung growth, 46 higher hemolysis 13 and/or anemia, 47 and increased pulmonary capillary blood volume 48 may contribute to airway obstruction in SCD children.…”

Section: Discussionmentioning

confidence: 99%

Tricuspid regurgitation velocity and other biomarkers of mortality in children, adolescents and young adults with sickle cell disease in the United States: The PUSH study

et al. 2020

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In the US, mortality in sickle cell disease (SCD) increases after age 18‐20 years. Biomarkers of mortality risk can identify patients who need intensive follow‐up and early or novel interventions. We prospectively enrolled 510 SCD patients aged 3‐20 years into an observational study in 2006‐2010 and followed 497 patients for a median of 88 months (range 1‐105). We hypothesized that elevated pulmonary artery systolic pressure as reflected in tricuspid regurgitation velocity (TRV) would be associated with mortality. Estimated survival to 18 years was 99% and to 25 years, 94%. Causes of death were known in seven of 10 patients: stroke in four (hemorrhagic two, infarctive one, unspecified one), multiorgan failure one, parvovirus B19 infection one, sudden death one. Baseline TRV ≥2.7 m/second (>2 SD above the mean in age‐matched and gender‐matched non‐SCD controls) was observed in 20.0% of patients who died vs 4.6% of those who survived (P = .012 by the log rank test for equality of survival). The baseline variable most strongly associated with an elevated TRV was a high hemolytic rate. Additional biomarkers associated with mortality were ferritin ≥2000 μg/L (observed in 60% of patients who died vs 7.8% of survivors, P < .001), forced expiratory volume in 1 minute to forced vital capacity ratio (FEV1/FVC) <0.80 (71.4% of patients who died vs 18.8% of survivors, P < .001), and neutrophil count ≥10x109/L (30.0% of patients who died vs 7.9% of survivors, P = .018). In SCD children, adolescents and young adults, steady‐state elevations of TRV, ferritin and neutrophils and a low FEV1/FVC ratio may be biomarkers associated with increased risk of death.

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Section: Discussionmentioning

confidence: 99%

Tricuspid regurgitation velocity and other biomarkers of mortality in children, adolescents and young adults with sickle cell disease in the United States: The PUSH study

et al. 2020

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“…Studies are now beginning to explore the impact of pulmonary complications on lung function for adults and children with SCA . While most children with SCA have lung function within normal limits, lung growth is significantly reduced compared to age and race‐matched controls and adulthood lung restrictive defects are commonly reported . However, few studies have prospectively evaluated the longitudinal trajectory of lung function in children with SCA.…”

Section: Introductionmentioning

confidence: 99%

Age is a predictor of a small decrease in lung function in children with sickle cell anemia

Willen

Cohen

Rodeghier³

et al. 2018

American J Hematol

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The longitudinal pattern of lung function in children with sickle cell anemia (SCA) has shown a decrease in FEV % predicted, a risk factor for death in adults with SCA, but predictors for this decline are poorly characterized. In a prospective longitudinal multi-center cohort of children with SCA, we tested the hypotheses that: (1) FEV % predicted declines over time; and (2) SCA-specific characteristics and therapy predict this decline. At three clinical centers, children with SCA (HbSS or HbSβ thalassemia), unselected for respiratory disease, were enrolled in the Sleep and Asthma Cohort (SAC) study. Study-certified pulmonary function technicians performed spirometry and lung volumes. Each assessment was reviewed centrally. Predicted values were determined for TLC, FEV , FVC, and FEV /FVC ratio. A total of 197 participants, mean age 11.0 years at first testing (range 4-19.3 years), had a minimum of three spirometry measurements, over an average of 4.4 years (range 1.1-6.5 years) from baseline to endpoint. In a multivariable model, FEV % predicted declines by 0.3% for every additional year of age (95% CI -0.56 to -0.05, P = .020). Sex, asthma history, hemoglobin, reticulocyte count, white blood cell count, incidence rate of severe acute pain and acute chest syndrome episodes, and hydroxyurea therapy were not associated with a decline in FEV % predicted. In a large, rigorously evaluated, prospective cohort of an unselected group of children with SCA, FEV % predicted declines minimally over an average of 4 years, and none of the examined disease features predict the decline.

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“…We thank Patel and colleagues for their interest in our recently published article about lung function patterns in children with sickle cell anemia (1). In our multicenter, prospective study, we demonstrated that the majority of children with sickle cell anemia had normal lung function.…”

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confidence: 56%

Reply: Decline of Lung Function in Children with Sickle Cell Disease Is Not Associated with Restrictive Defects

Cohen

Rodeghier²,

DeBaun

2017

Annals ATS

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Pattern of Lung Function Is Not Associated with Prior or Future Morbidity in Children with Sickle Cell Anemia

Cited by 44 publications

References 42 publications

Tricuspid regurgitation velocity and other biomarkers of mortality in children, adolescents and young adults with sickle cell disease in the United States: The PUSH study

Tricuspid regurgitation velocity and other biomarkers of mortality in children, adolescents and young adults with sickle cell disease in the United States: The PUSH study

Age is a predictor of a small decrease in lung function in children with sickle cell anemia

Reply: Decline of Lung Function in Children with Sickle Cell Disease Is Not Associated with Restrictive Defects

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