Abstract
Background: Pulmonary complications account for significant morbidity and mortality in sickle cell disease (SCD) patients. Abnormal pulmonary functions (PFTs) in these patients include airway obstruction, restriction, abnormal diffusing capacity, and hypoxemia. This study was carried out to assess PFTs among SCD patients, and the effects of different clinical and laboratory variables on PFTs.
Methods: Forty five steady state SCD patients, previously diagnosed and followed up at the Hematology Outpatient Clinic of New Children Hospital; Cairo University, were included. Twenty seven were homozygous for HbSS and 18 sickle β thalassemia. Their mean age was 15.1 years with a range of 5-33years. Clinical, laboratory and PFTs were performed.
Results: Abnormalities of PFTs were found in 57.8% of our patients (n=26, 23 having restrictive and 3 obstructive patterns). According to severity of affection, 44.4%, 8.9% and 4.4% had mild, moderate and moderate to severe abnormalities respectively. Patients' age was the only clinical variable that showed a statistically significant difference between SCD patients with normal and abnormal PFTs (p=0.02) and between those with normal and restrictive patterns (p =0.05). Frequency of vaso-occlusive crisis and serum ferritin level showed a statistically significant relation with severity of pulmonary affection (p=0.01 and 0.01 respectively). No statistically significant difference in parameters of PFTs of patients on hydroxyurea (HU) and those not.
Conclusion: Pulmonary function abnormalities, mostly mild and restrictive, were found among our SCD patients. These changes may be more prominent with increasing age. Severity of pulmonary affection was related to frequency of VOC.
- © 2011 ERS
