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Lung function in infants with sickle cell disease
A C Koumbourlis1, A Hurlet-Jensen, M R Bye
1Department of Pediatrics, College of Physicians and Surgeons of Columbia University, New York, New York, USA.
Insights
Sickle cell disease can cause abnormal lung function, specifically lower airway obstruction and hyperinflation, even in early infancy. These early lung changes may be linked to airway reactivity but require further study.
Area of Science:
- Pediatric Pulmonology
- Hematology
- Neonatology
Background:
- Sickle cell disease (SCD) is a genetic blood disorder with potential systemic complications.
- Pulmonary complications in SCD are well-documented in older individuals but less understood in infants.
- Early detection of lung dysfunction in infants with SCD is crucial for timely intervention.
Purpose of the Study:
- To investigate the presence and nature of pulmonary function abnormalities in infants with sickle cell disease.
- To determine if abnormal lung function develops early in life in this population.
Main Methods:
- Pulmonary function testing was conducted on 20 infants (3-30 months) with SCD.
- Techniques included passive occlusion for respiratory system compliance and resistance, nitrogen washout for functional residual capacity, and thoracoabdominal compression for tidal flow-volume loops.
- Patients were categorized into Hb SS (Group I) and other hemoglobinopathies (Group II).
Main Results:
- Both groups exhibited elevated functional residual capacity (FRC) and decreased maximum expiratory flows at FRC, indicating lower airway obstruction (LAO) and hyperinflation.
- Patients with Hb SS showed a trend towards more acute chest syndrome (ACS) and vasoocclusive crisis (VOC).
- Restrictive lung disease was uncommon, observed in only three patients.
Conclusions:
- Infants with sickle cell disease, particularly those with Hb SS, may present with abnormal lung function, predominantly LAO and hyperinflation, in early infancy.
- Airway reactivity is a potential factor in the pathogenesis of these early pulmonary changes.
- The precise relationship between these early lung abnormalities and clinical SCD events like VOC or ACS remains to be elucidated.
Abstract:
We performed pulmonary function testing in 20 infants (11 male and 9 female; ages 3-30 months) with sickle cell disease to assess whether abnormal lung function develops early in life. Respiratory system compliance (Crs) and resistance (Rrs) were measured by the passive occlusion technique, functional residual capacity (FRC) was measured by the nitrogen washout technique, and tidal flow-volume loops and partial expiratory flow-volume curves were obtained by the thoracoabdominal compression technique to detect airway obstruction. Patients with Hb SS (Group I, n = 12) had significantly lower hemoglobin levels and a higher (but not significant) incidence of acute chest syndrome (ACS), vasoocclusive crisis (VOC), splenic sequestration, transfusions, and history of intermittent bronchospasm compared to with patients with hemoglobinopathies Hb SC, Hb Sbt and Hb SF (Group II; n = 8). Both groups had elevated FRC, decreased maximum expiratory flows at FRC (V'max,FRC), and decreased time needed to reach peak expiratory flow (tme/tE), suggesting lower airway obstruction (LAO) and hyperinflation. Restrictive disease was found in only three patients of Group I. Our findings suggest that in sickle cell disease (especially among patients with Hb SS), abnormal lung function (predominantly LAO) may be present in early infancy. Airway reactivity may play a role in the pathogenesis, but the relation to VOC or ACS remains unclear.