Dyspnea, pulmonary function and exercise capacity in adult Saudi patients with sickle cell disease.

OBJECTIVE: To examine pulmonary function, dyspnea and exercise capacity in adult Saudi sickle cell disease (SCD) patients. METHODS: The patients were recruited from the hematology clinic at King Khalid University Hospital in Riyadh from January to December 2005. The study involved 39 patients with stable SCD (20 women and 19 men), with a mean age of 22.7+/-7.1 years, hemoglobin level of 95.5+/-14.6 g/L and hemoglobin F level of 13.7+/-8.6%. Patients underwent pulmonary function tests (PFT) (forced expiratory volume in first second [FEV1], forced vital capacity [FVC], and diffusion capacity of carbon monoxide [DLco] data are presented as a percentage of the normal prediction), a 6-minute walk test (6MWT) and echocardiography. Dyspnea was assessed using the Borg score. The 6MWT data were compared to body mass index-matched healthy controls. RESULTS: Forty-one percent of SCD patients had mild dyspnea at rest, and this increased to 61% at the end of the 6MWT. Pulmonary function tests were abnormal in 51% (36% of patients had a restrictive pattern, 10% had isolated decrease in DLco, and 5% had a mixed restrictive-obstructive pattern). The 6MWD was shorter in SCD patients compared to the controls (368+/-67 versus 407+/-47m, p=0.005). No hematological variables correlated with outcome variables. CONCLUSION: Chronic pulmonary complications in adult Saudi SCD patients are relatively mild but common. Pulmonary function in these patients differs from that published for African-origin SCD patients. This difference may reflect a different natural history of SCD in the 2 populations.

Echocardiographic abnormalities in adolescent and adult Saudi patients with sickle cell disease.

OBJECTIVE: Cardiovascular complications in sickle cell disease (SCD) have been well documented but cardiac involvement in Saudi patients with SCD is not known. We sought to identify cardiac abnormalities by echocardiography in adolescent and adult Saudi patients with stable SCD. METHODS: Sixty-five consecutive patients with SCD followed at King Khalid University Hospital, Riyadh, were prospectively studied from January 2005 to December 2005. All patients underwent echocardiographic examination to determine chamber dimensions, left ventricular function, valvular anomalies and pulmonary artery pressure. Data were compared to normal age and gender-matched controls. Hematological data were also collected from the patients and correlated with the echocardiographic results. RESULTS: Twenty-eight males and 37 females were evaluated. The mean age of the group was 24.5+/-9.2 (range 14-44) years. The most common abnormality found was pulmonary hypertension (PH) present in 25 (38%) patients. The majority of these patients had mild PH and only 6 (9%) patients had pulmonary artery systolic pressure (PASP) more than 40 mm Hg. Older age, lower level of fetal hemoglobin and high serum ferritin were associated with increased PASP. Other abnormalities present included dilated left atrium in 17 (26%) patients, dilated right atrium 13 (20%), dilated left ventricle 10 (15%), valvular anomalies 21 (32%) and reduced ejection fraction in 4 (6%) patients. CONCLUSION: Cardiac abnormalities are found in a significant proportion of Saudi patients with SCD. Pulmonary hypertension is the most common finding while other abnormalities are less frequent.