Longitudinal assessment of lung function in children with sickle cell disease

Lunt, Alan; McGhee, Emily; Sylvester, Karl; Rafferty, Gerrard F.; Dick, Moira; Rees, David C.; Height, Sue; Thein, Swee Lay; Greenough, Anne

doi:10.1002/ppul.23367

Cited by 47 publications

(46 citation statements)

References 39 publications

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“…Longitudinal studies have shown that there is a decline in lung volume and pulmonary function over the years in children with SCD; however, the pathophysiology of respiratory disorders in childhood has not been fully elucidated 9 35 This decline was reported in a cohort study by Lunt et al, 36 who demonstrated that a history of ACS episodes was the only independent factor associated with reduced lung volumes. Some biological markers, such as leukocytosis, are known to be associated with SCD severity, but their relationship with pulmonary function has only recently been the subject of investigation 37 .…”

Section: Discussionmentioning

confidence: 99%

Pulmonary function in children and adolescents with sickle cell disease: have we paid proper attention to this problem?

et al. 2016

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Objective:To evaluate pulmonary function and functional capacity in children and adolescents with sickle cell disease. Methods:This was a cross-sectional study involving 70 children and adolescents (8-15 years of age) with sickle cell disease who underwent pulmonary function tests (spirometry) and functional capacity testing (six-minute walk test). The results of the pulmonary function tests were compared with variables related to the severity of sickle cell disease and history of asthma and of acute chest syndrome. Results:Of the 64 patients who underwent spirometry, 15 (23.4%) showed abnormal results: restrictive lung disease, in 8 (12.5%); and obstructive lung disease, in 7 (10.9%). Of the 69 patients who underwent the six-minute walk test, 18 (26.1%) showed abnormal results regarding the six-minute walk distance as a percentage of the predicted value for age, and there was a ≥ 3% decrease in SpO2 in 36 patients (52.2%). Abnormal pulmonary function was not significantly associated with any of the other variables studied, except for hypoxemia and restrictive lung disease. Conclusions:In this sample of children and adolescents with sickle cell disease, there was a significant prevalence of abnormal pulmonary function. The high prevalence of respiratory disorders suggests the need for a closer look at the lung function of this population, in childhood and thereafter.

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Section: Discussionmentioning

confidence: 99%

Pulmonary function in children and adolescents with sickle cell disease: have we paid proper attention to this problem?

et al. 2016

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“…The lung is often affected in sickle cell disease (SCD), acutely with acute chest syndrome (ACS) (1) and chronically with dyspnea and reduced exercise capacity (2,3), loss of lung function (4,5), and pulmonary hypertension (6). Studies have sought to define pulmonary function abnormalities in children and adolescents with SCD (4, 7-13) using pulmonary function evaluations conducted when children and adolescents were at their baseline status, with tests most often obtained in clinical laboratories during routine care.…”

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

Cohen

Strunk

Rodeghier³

et al. 2016

Annals ATS

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“…The estimated prevalence of obstructive lung disease in SCD children ranges from 8% to 57%, independent of a diagnosis of asthma (Table ) . Young children appear to have an obstructive pattern of lung disease while restrictive and diffusion defects are more commonly seen in older children and adults . Further, in the study by Maclean et al lung function in children with SCD was noted to decline over time at a rate of 3% decline in forced expiratory volume in 1 sec (FEV1) per year, a decline more rapid than in children with asthma .…”

Section: Sickle Cell Disease Acute Chest Syndrome and Asthmamentioning

confidence: 99%

Airway inflammation in sickle cell disease—A translational perspective

Manwani

Rastogi

2018

Pediatric Pulmonology

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Asthma and sickle cell disease (SCD) are common chronic conditions in children of African ancestry that are characterized by cough, wheeze, and obstructive patterns on pulmonary function. Pulmonary function testing in children with SCD has estimated a prevalence of obstructive lung disease ranging from 13% to 57%, and airway hyper-responsiveness of up to 77%, independent of a diagnosis of asthma. Asthma co-existing with SCD is associated with increased risk of acute chest syndrome (ACS), respiratory symptoms, pain episodes, and death. However, there are inherent differences in the pathophysiology of SCD and asthma. While classic allergic asthma in the general population is associated with a T-helper 2 cell (Th-2 cells) pattern of cell inflammation, increased IgE levels and often positive allergy testing, inflammation in SCD is associated with different inflammatory pathways, involving neutrophilic and monocytic pathways, which have been explored to a limited extent in mouse models and with a dearth of human studies. The current review summarizes the existent literature on sickle cell related airway inflammation and its cross roads with allergic asthma-related inflammation, and discusses the importance of further elucidating and understanding these common and divergent inflammatory pathways in human studies to facilitate development of targeted therapy for children with SCD and pulmonary morbidity.

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Longitudinal assessment of lung function in children with sickle cell disease

Abstract: Lung function deteriorated with increasing age in SCD children and the rate of decline was greater in younger children in whom ACS episodes were more common. Pediatr Pulmonol. 2016;51:717-723. © 2015 Wiley Periodicals, Inc.

Cited by 47 publications

References 39 publications

Pulmonary function in children and adolescents with sickle cell disease: have we paid proper attention to this problem?

Pulmonary function in children and adolescents with sickle cell disease: have we paid proper attention to this problem?

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

Airway inflammation in sickle cell disease—A translational perspective

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