Elsevier

The Journal of Pediatrics

Volume 138, Issue 2, February 2001, Pages 188-192
The Journal of Pediatrics

Original Articles
Prevalence and reversibility of lower airway obstruction in children with sickle cell disease

https://doi.org/10.1067/mpd.2001.111824Get rights and content

Abstract

Objective: To determine the prevalence and reversibility of lower airway obstruction (LAO) in children and adolescents with hemoglobin SS sickle cell disease (HbSS SCD). Study Design: Retrospective evaluation of lung function in a cross-section of 35 African American and 28 Hispanic children and adolescents with HbSS SCD. Lung function was evaluated with maximal respiratory flow-volume curves and body plethysmography. Each patient was assigned to 1 of 3 patterns of lung function (normal, obstructive, or restrictive). Airway hyperresponsiveness was assessed by means of a trial with bronchodilator. Results: Normal pattern was detected in 57% of the patients, LAO in 35%, and restrictive lung disease in 8%. Positive response to bronchodilator was documented in 30% of those with normal pattern of lung function, 78% in those with LAO, and 67% of those with restrictive lung disease. The pattern of lung function was not associated with race or with history of vaso-occlusive crises, acute chest syndrome, reactive airways disease/asthma, or long-term transfusion therapy. Conclusion: Obstructive lung disease possibly precedes the development of restrictive lung disease, and airway reactivity may be part of the pathogenic mechanism. (J Pediatr 2001;138:188-92)

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

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    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.

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