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Pulmonary Dysfunction Among Adolescents and Adults with Sickle Cell Anemia in Nigeria
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A2058 - Pulmonary Dysfunction Among Adolescents and Adults with Sickle Cell Anemia in Nigeria
Author Block: O. B. Ozoh1, O. O. Kalejaiye1, Y. O. Adelabu2, D. A. Nmadu3, O. E. Eromosele2, M. O. Kehinde1; 1Dept of Medicine, Univ of Lagos, Lagos, Nigeria, 2Dept of Medicine, Lagos University Teaching Hospital, Lagos, Nigeria, 3Department of Medicine, Lagos University Teaching Hospital, Lagos, Nigeria.
RATIONALE: Pulmonary complications contribute to excess mortality in sickle cell anemia (SSA) and may manifest as pulmonary dysfunction. Pattern of dysfunction and its associated factors may suggest potential approaches to limit the sequel of chronic lung disease. We aim to describe spirometry abnormalities in SSA and explore its relationship with clinical and laboratory parameters.
METHODS: A cross sectional study among non-smoking SSA patients attending the Lagos University Teaching Hospital. We obtained relevant medical history and presence of respiratory symptoms using the Medical Research Council questionnaire. We measured oxygen saturation (SPO2), performed spirometry and obtained blood samples for complete blood counts and level of hemoglobin F (HbF).
RESULTS: Two hundred and forty-five participants performed good quality spirometry tests. Age ranged from 14 to 62 years (mean±SD =26.7±7.9), 50.2% female, 91.8% SS genotype, 8.1% SC genotype and 72.7% had at least one crisis in the preceding year. There was a history of acute chest syndrome in 13.9%, 1.2% stroke, 15.1% priapism, 20.1% leg ulcer, 7.9% avascular necrosis of femoral head and 1.2% asthma. Only 19.1% took hydroxyurea and 15.1% for one year or longer. At least one respiratory symptom was reported by 42.6% of participants (21.6% shortness of breath, 21.2% exercise induced symptoms, 5.3% sleep related symptoms, 13.5% symptoms related to exposure to dust or smoke, 8.6% cough and 9% phlegm). Mean SPO2 was 95.1%±3.2. Complete blood count for 188 participants showed a median hemoglobin concentration of 7.8g/dl (IQR 6.8-8.9), white cell count 9400x109 cells/L (7400-11575), neutrophils 5250x109 cells/L (3900-6900), lymphocytes 3400x109 cells/L (2600-4300) and platelets 271500 (193000-355250). Median HbF levels for 196 participants was 4.8 (2.6-8.4). Mean FVC was 3.1L± 0.7, FVC% predicted 87.8±16.1, FEV1 2.5L±0.6, FEV1% predicted 80.8±14.5 and FEV1/FVC 0.8±0.08. Based on standard criteria 52.2% of participants had abnormal spirometry (30.2% probable restriction, 20.4% obstruction and 1.6% mixed defect). To determine the factors that influence abnormal pulmonary function we included 187 participants with complete blood counts and hemoglobin F levels of which 48.4% had abnormal spirometry (23.4% probable restriction, 22.8% obstruction, 2.2% mixed). There was no significant association between abnormal spirometry pattern and any clinical or hematologic parameter in univariate and multivariate regression analysis.
CONCLUSION: Pulmonary function abnormalities are common in SSA and obstructive and restrictive abnormalities occur frequently. While restriction may signify sickle cell chronic lung disease, obstruction appears curious and warrant further research including the evaluation of treatment options used in the management of airway diseases.
Author Block: O. B. Ozoh1, O. O. Kalejaiye1, Y. O. Adelabu2, D. A. Nmadu3, O. E. Eromosele2, M. O. Kehinde1; 1Dept of Medicine, Univ of Lagos, Lagos, Nigeria, 2Dept of Medicine, Lagos University Teaching Hospital, Lagos, Nigeria, 3Department of Medicine, Lagos University Teaching Hospital, Lagos, Nigeria.
RATIONALE: Pulmonary complications contribute to excess mortality in sickle cell anemia (SSA) and may manifest as pulmonary dysfunction. Pattern of dysfunction and its associated factors may suggest potential approaches to limit the sequel of chronic lung disease. We aim to describe spirometry abnormalities in SSA and explore its relationship with clinical and laboratory parameters.
METHODS: A cross sectional study among non-smoking SSA patients attending the Lagos University Teaching Hospital. We obtained relevant medical history and presence of respiratory symptoms using the Medical Research Council questionnaire. We measured oxygen saturation (SPO2), performed spirometry and obtained blood samples for complete blood counts and level of hemoglobin F (HbF).
RESULTS: Two hundred and forty-five participants performed good quality spirometry tests. Age ranged from 14 to 62 years (mean±SD =26.7±7.9), 50.2% female, 91.8% SS genotype, 8.1% SC genotype and 72.7% had at least one crisis in the preceding year. There was a history of acute chest syndrome in 13.9%, 1.2% stroke, 15.1% priapism, 20.1% leg ulcer, 7.9% avascular necrosis of femoral head and 1.2% asthma. Only 19.1% took hydroxyurea and 15.1% for one year or longer. At least one respiratory symptom was reported by 42.6% of participants (21.6% shortness of breath, 21.2% exercise induced symptoms, 5.3% sleep related symptoms, 13.5% symptoms related to exposure to dust or smoke, 8.6% cough and 9% phlegm). Mean SPO2 was 95.1%±3.2. Complete blood count for 188 participants showed a median hemoglobin concentration of 7.8g/dl (IQR 6.8-8.9), white cell count 9400x109 cells/L (7400-11575), neutrophils 5250x109 cells/L (3900-6900), lymphocytes 3400x109 cells/L (2600-4300) and platelets 271500 (193000-355250). Median HbF levels for 196 participants was 4.8 (2.6-8.4). Mean FVC was 3.1L± 0.7, FVC% predicted 87.8±16.1, FEV1 2.5L±0.6, FEV1% predicted 80.8±14.5 and FEV1/FVC 0.8±0.08. Based on standard criteria 52.2% of participants had abnormal spirometry (30.2% probable restriction, 20.4% obstruction and 1.6% mixed defect). To determine the factors that influence abnormal pulmonary function we included 187 participants with complete blood counts and hemoglobin F levels of which 48.4% had abnormal spirometry (23.4% probable restriction, 22.8% obstruction, 2.2% mixed). There was no significant association between abnormal spirometry pattern and any clinical or hematologic parameter in univariate and multivariate regression analysis.
CONCLUSION: Pulmonary function abnormalities are common in SSA and obstructive and restrictive abnormalities occur frequently. While restriction may signify sickle cell chronic lung disease, obstruction appears curious and warrant further research including the evaluation of treatment options used in the management of airway diseases.
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