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Pulmonary function in sickle cell disease with or without acute chest syndrome
1Service de Physiologie-Explorations Fonctionnelles, Hôpital Henri Mondor, Créteil, France.
The European Respiratory Journal
|December 24, 1998
Summary
Recurrent acute chest syndrome (ACS) in sickle cell disease patients is linked to obstructive lung dysfunction. Higher respiratory resistance correlates with more ACS episodes and reduced expiratory flow rates.
Area of Science:
- Pulmonary Medicine
- Hematology
- Cardiopulmonary Research
Background:
- Chronic lung dysfunction is a concern in sickle cell disease (SCD).
- Acute chest syndrome (ACS) is a potential risk factor for lung complications in SCD patients.
Purpose of the Study:
- To investigate the relationship between recurrent acute chest syndrome (ACS) and chronic lung dysfunction in sickle cell disease (SCD).
- To assess lung function parameters in SCD patients with and without a history of ACS.
Main Methods:
- Lung function tests were conducted on 49 stable SCD outpatients.
- Patients were divided into two groups: those with a history of 2-4 ACS episodes (ACS+) and those with no ACS history (ACS-).
- Respiratory resistance (Rrs) and diffusion capacity (TL,CO, KCO) were measured using the forced oscillation technique and CO transfer.
Main Results:
- Respiratory resistance (Rrs) significantly increased with the number of ACS episodes (r=0.55, p<0.0001).
- Higher Rrs was associated with lower expiratory flow rates.
- The ACS+ group showed significantly higher transfer factor (TL,CO) and transfer coefficient (KCO) compared to the ACS- group.
Conclusions:
- Obstructive lung dysfunction is prevalent in sickle cell disease.
- Recurrent acute chest syndrome may contribute to specific obstructive lung defects in SCD.
- Increased respiratory resistance in ACS patients was linked to increased diffusion capacity, possibly due to elevated lung blood volume.