Longitudinal Decline in Lung Volume in a Population of Children with Sickle Cell Disease

MacLean, Joanna E.; Atenafu, Eshetu G.; Kirby‐Allen, Melanie; MacLusky, Ian; Stephens, Derek; Grasemann, Hartmut; Subbarao, Padmaja

doi:10.1164/rccm.200708-1219oc

Cited by 82 publications

(82 citation statements)

References 43 publications

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Section: Discussionsupporting

confidence: 81%

“…Prior studies have suggested changes in lung function measurements are an early marker of sickle cell lung disease [1,12,27]. Many of these studies showed that unlike a normal nonsmoking individual, where pulmonary function is expected to increase during childhood, in SCD there was a progressive decline in pulmonary function [1,6,12,27].…”

Section: Discussionmentioning

confidence: 99%

“…Many of these studies showed that unlike a normal nonsmoking individual, where pulmonary function is expected to increase during childhood, in SCD there was a progressive decline in pulmonary function [1,6,12,27]. Our study affirmed this data and showed that average FEV1% of predicted trended below normal with the lowest levels observed during the early adolescent period [1,2,6,12]. This was seen even in the 70 patients where a clearly documented smoking history or exposure was absent and may be a reflection of chronic subclinical inflammation resulting in chronic lung injury.…”

Section: Discussionmentioning

confidence: 99%

“…Sickle cell disease (SCD) is a group of genetic disorders identified primarily by homozygosity of hemoglobin S (HbSS) [1][2][3]. SCD affects approximately 70,000-100,000 people within the United States [3].…”

Section: Introductionmentioning

confidence: 99%

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Progression and Prognostic Indicators of Bronchial Disease in Children with Sickle Cell Disease

Williams

Nussbaum

Yoonessi

et al. 2014

Lung

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Section: Discussionsupporting

confidence: 81%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Progression and Prognostic Indicators of Bronchial Disease in Children with Sickle Cell Disease

Williams

Nussbaum

Yoonessi

et al. 2014

Lung

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“…[19][20][21][22] The bronchodilator response and patterns of cytokine expression in SCD share features with asthma, and suggest that wheezing and obstruction may be manifestations of SCD or of concurrent comorbid states. 23 Comprehensive assessment of lung disease typically includes spirometric pulmonary function testing that may not be feasible in younger children.…”

Section: Lungmentioning

confidence: 99%

Primary Prophylaxis in Sickle Cell Disease: Is It Feasible? Is It Effective?

Thompson

2011

Hematology

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Consideration of primary prophylaxis to prevent complications of sickle cell disease (SCD) requires a clear understanding of the earliest manifestations of SCD-related organ injury, a reliable clinical or laboratory tool to detect organ dysfunction, and evidence that an intervention instituted in the presymptomatic state can mitigate disease progression. This review examines the pathophysiology of SCD in organs that may be potential targets for intervention, our current capacity to evaluate early SCD manifestations, results of clinical trials, and opportunities for future interventions.

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Asthma management: Reinventing the wheel in sickle cell disease

Morris¹

2009

American J Hematol

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Asthma is a common comorbidity in sickle cell disease (SCD) with a reported prevalence of 30-70%. The high frequency of asthma in this population cannot be attributed to genetic predisposition alone, and likely reflects in part, the contribution of overlapping mechanisms shared between these otherwise distinct disorders. There is accumulating evidence that dysregulated arginine metabolism and in particular, elevated arginase activity contributes to pulmonary complications in SCD. Derangements of arginine metabolism are also emerging as newly appreciated mechanism in both asthma and pulmonary hypertension independent of SCD. Patients with SCD may potentially be at risk for an asthma-like condition triggered or worsened by hemolysis-driven release of erythrocyte arginase and low nitric oxide bioavailability, in addition to classic familial asthma. Mechanisms that contributed to asthma are complex and multifactorial, influenced by genetic polymorphisms as well as environmental and infectious triggers. Given the association of asthma with inflammation, oxidative stress and hypoxemia, factors known to contribute to a vasculopathy in SCD, and the consequences of these factors on sickle erythrocytes, comorbid asthma would likely contribute to a vicious cycle of sickling and subsequent complications of SCD. Indeed a growing body of evidence documents what should come as no surprise: Asthma in SCD is associated with acute chest syndrome, stroke, pulmonary hypertension, and early mortality, and should therefore be aggressively managed based on established National Institutes of Health Guidelines for asthma management. Barriers to appropriate asthma management in SCD are discussed as well as strategies to overcome these obstacles in order to provide optimal care. Am. J. Hematol. 84:234-241, 2009. V

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Longitudinal Decline in Lung Volume in a Population of Children with Sickle Cell Disease

Abstract: Lung volumes in children with SCD decline with age. The pattern of decline begins in childhood, and supports a predominately restrictive defect.

Cited by 82 publications

References 43 publications

Progression and Prognostic Indicators of Bronchial Disease in Children with Sickle Cell Disease

Progression and Prognostic Indicators of Bronchial Disease in Children with Sickle Cell Disease

Primary Prophylaxis in Sickle Cell Disease: Is It Feasible? Is It Effective?

Asthma management: Reinventing the wheel in sickle cell disease

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