ABSTRACT
Objective: The current study was carried out to compare pulmonary function tests [PFTs] in pediatric Kuwaiti sickle cell disease [SCD] patients to age-matched normal controls and to investigate the association of PFTs with selected clinical and laboratory parameters
Subjects and Methods: There were 38 patients with SCD and 36 controls in the study. The patients were recruited from the Pediatric Hematology Clinics of Mubarak AI-Kabeer and AI-Amiri Hospitals, Kuwait, and were studied in steady state. The controls were healthy, non-sickle cell siblings of the patients. Forced expiratory volume in 1 s [FEV], forced vital capacity [FVC], total lung capacity, and other PFT parameters were obtained using a constant-volume, variable-pressure, body plethysmograph. Hemoglobin, fetal hemoglobin, serum bilirubin, and lactate dehydrogenase were determined using standard methods
Results: The mean ages of the patients and controls were 10.5+/- 3.2 and 10.5 +/-3.5 years, respectively. The FEV/o predicted of 84.1 +/- 15.4% among the patients was significantly lower than the 92.1 =/- 11.8% in the controls [p = 0.003]. The FVC% predicted was also significantly lower [p = 0.022] in the patients than in the controls, although the values were generally within the normal range. There was no association of FEV] with pain phenotype, acute chest syndrome [ACS], or blood transfusions. Also, there was nosignificant correlation with reticulocytes, bilirubin, or lac-tate dehydrogenase
Conclusions: In this study, changes in PFT, especially FEV1; developed early in the SCD patients. There was no demonstrable association with frequent vaso-occlusive crisis, ACS, and other variables. Hence, there is a need for follow-up studies with serial PFTs to identify vulnerable patients, who might need intervention to prevent early mortality