Original ArticlesPrevalence and reversibility of lower airway obstruction in children with sickle cell disease☆
Section snippets
Methods
The patient population consisted of African American (n = 35; 22 male) and Hispanic (n = 28; 12 male) children and adolescents, aged 5 to 18 years, with homozygous HbSS SCD receiving care at the Sickle Cell Clinic at Babies & Children’s Hospital of New York. Pulmonary function testing was part of their routine medical care and was performed in the pediatric pulmonary function laboratory at Babies & Children’s Hospital. Clinical information regarding episodes of VOC and of ACS, as well as
Results
Pulmonary function test results from 63 patients, representing 73% of patients with HbSS SCD in the 5- to 18-year-old group, were analyzed (Table I).
Normal pattern of lung function was found in 36 patients (57%), obstructive in 22 patients (35%), and restrictive in only 5 patients (8%). These patterns were virtually the same whether they were determined by lung volumes plus spirometry or by spirometry alone. There was no difference in the patterns of lung function between African American and
Discussion
We performed a detailed analysis of the patterns of lung function in a cross-section of children with HbSS SCD. We found that LAO, and not restrictive disease, is the most common abnormality of lung function in children and adolescents with HbSS SCD. These findings, which are in accordance with those of our previous study,9 provide further evidence that airway hyperreactivity may be implicated in the pathogenesis of LAO.
The determination of LAO in adult patients is often based on the
References (29)
- et al.
Pulmonary function abnormalities in childhood sickle disease
J Pediatr
(1993) - et al.
Airway hyperreactivity in children with sickle cell disease
J Pediatr
(1997) - et al.
Ventilatory functions of normal children and young adults—Mexican-American, white, and black. I. Spirometry
J Pediatr
(1979) - et al.
Sickle cell chronic lung disease: prior morbidity and the risk of pulmonary failure
Medicine
(1988) - et al.
Pulmonary function studies in sickle cell disease
J Appl Physiol
(1970) - et al.
An assessment of lung volumes and gas transfer in sickle anaemia
Thorax
(1971) - et al.
Lung function in sickle cell hemoglobinopathy patients compared with healthy subjects
J Natl Med Assoc
(1988) - et al.
Cardiorespiratory adjustments in chronic sickle cell anemia
Bull Eur Physiol Respir
(1983) - et al.
Lung function in children with sickle cell anemia
Am Rev Respir Dis
(1979) - et al.
Pulmonary function in children with sickle cell disease [abstract]
Am J Respir Crit Care Med
(1994)
Lung function in infants with sickle cell disease
Pediatr Pulmonol
Airway obstruction in children and young adults with sickle cell disease [abstract]
Am J Respir Crit Care Med
Standards of pulmonary function in children
Am Rev Respir Dis
Reference values for residual volume, functional residual capacity and total lung capacity
Eur Respir J
Cited by (149)
Reduced Lung Diffusion Capacity Caused by Low Alveolar Volume and Restrictive Disease Are Common in Sickle Cell Disease
2022, Archivos de BronconeumologiaSickle Cell Disease: Monitoring, Current Treatment, and Therapeutics Under Development
2019, Hematology/Oncology Clinics of North AmericaEnd points for sickle cell disease clinical trials: Renal and cardiopulmonary, cure, and low-resource settings
2019, Blood AdvancesCitation Excerpt :C-reactive protein, sPLA2, thrombospondin-1, and pentraxin 3 are possible biomarkers for predicting occurrence of ACS. Asthma is a comorbid factor in SCD.96-103 Studies confirm that asthma predisposes to complications such as vaso-occlusive crises (VOCs),82,104-107 ACS,108,109 and stroke109 and is associated with increased mortality.110,111
Essentials of Pulmonology
2019, A Practice of Anesthesia for Infants and ChildrenHow I treat hypoxia in adults with hemoglobinopathies and hemolytic disorders
2018, BloodCitation Excerpt :It is with this framework in mind that a clinician should approach the diagnosis and treatment of chronic hypoxia in SCD. Up to 70% of pediatric patients with SCD have airway hyperreactivity (AHR).18-23 Data suggest that wheezing and obstructive lung disease may occur independently of asthma and are a marker of disease severity or a high output state.24,25
- ☆
Reprint requests: Anastassios C. Koumbourlis, MD, Division of Pediatric Pulmonology, Babies and Children’s Hospital of New York, BHS-7, 3959 Broadway, New York, NY 10032.