1 PULMONARY FUNCTION INDICES OF CHILDREN WITH SICKLE CELL ANAEMIA IN ENUGU, SOUTH-EAST NIGERIA Abstract Background The impact of sickle cell anaemia on the pulmonary function indices of children cannot be downplayed. This is because the disorder causes obstruction of the …….and red blood cell haemolysis which impairs lung function Objectives: This is to determine the impact of sickle cell anaemia on the pulmonary function indices of patients attending the paediatric sickle cell clinic at the University of Nigeria Teaching Hospital (UNTH) Enugu, south-east Nigeria. Methods: A cross sectional study of lung function of children with sickle cell anaemia aged 6-20 years in the city of Enugu. The study was compiled, analyzed and proofread between October 2014 and January 2015. Measurements of the peak expiratory flow rate, Forced Vital Capacity (FVC) and the Forced Expiratory Volume in 1 second (FEV1) were taken using a single mini Wright peak flow meter and an automated single breath vitalograph respectively. Results: A total of 80 subjects were recruited into the study, comprising 40 HbSS patients and an equal number of controls. Children with sickle cell anemia had lower values of FEV1(1.6±0.52), FVC (1.76±0.95) and PEFR(309.00±82.64) when compared to those with normal Haemoglobin genotype FEV1(12.01±0.53), FVC(2.12±0.54) and PEFR(364.10±87.85) and is statistically significant. The mean FVC, FEV1/FVC and PEFR were also higher in the male controls compared to the HbSS males but these differences were not statistically significant (p>0.05). Female controls had significantly larger FEV1, FVC and PEFR compared to the SS females (p<0.05). Conclusion: The lung function indices were significantly lower in children and adolescents with sickle cell anaemia compared to matched controls with haemoglobin genotype HbAA. 2 Key words: Children; lung function; sickle cell anemia; Nigeria Introduction Sickle cell anaemia (SCA) is a genetic haematological disorder characterized by red blood cells that assume an abnormal, rigid, sickle shape.1 This hereditary disorder contributes the equivalent of 3.4% mortality in children aged under -5 worldwide or 6.4% in Africa.2 The prevalence of SCA in Nigeria ranges from 0.4% to 3%. 3 About 85% of sickle cell disorders and over 70% of all affected births occur in Africa. 4 It is worth noting that at least 5.2% of the world population carry a significant trait. The clinical consequence of SCA, result from obstruction of the microvasculature by the sickled cells and red blood cell haemolysis which causes multi-systemic manifestation. The lungs are affected in a variety of ways by these pulmonary insults which if recurrent overtime, may leave the lungs with chronic interstitial, parenchymal or vascular damage which may compromise pulmonary function.5,6 It has been documented that the prevalence of hypoxemia among SCA children is 13%.4 This prevalence is attributable to the chronic anaemic state, micro vascular occlusion of the circulation by sickled hemoglobin and constant pertubation of the endothelial membrane and consequent elaboration of endothelial molecules which are commonly seen among SCA children especially those with various types of vaso-occlusive episodes.7 Acute and chronic pulmonary complications occur frequently in patients with SCA, and contribute to morbidity and mortality later in life. Although the pathogenesis of chronic pulmonary disease in SCD has not been clearly defined, recurrent microvascular obstruction resulting in the development of pulmonary hypertension, endothelial dysfunction, and parenchymal fibrosis are probably the primary mechanisms.6 3 There is increasing evidence that repeated episodes of acute chest syndrome (ACS) may cause permanent damage to the pulmonary parenchyma and vasculature. Repeated attacks of ACS are a major risk factor for the development of sickle cell chronic lung disease. Studies of lung function in SCD have also demonstrated a restrictive defect 8,9 while a reduction in total lung capacity (TLC) of 50% has been reported in advanced forms. During steady-state sickle-cell disease, the major abnormality in pulmonary function is a restrictive ventilatory impairment, characterized by a mild reduction in total lung capacity, and reduced diffusion capacity for carbon monoxide 10 . These abnormalities worsen with age and are associated with increases in pulmonary-artery pressures11 Whereas some studies have documented impaired lung function in sickle cell anaemia (HbSS) patients.8-11 Other workers, however, reported what appears to be contrasting findings when they compared lung function in children with sickle cell anaemia and those of healthy controls with normal haemoglobin genotype.8-11 It is therefore necessary that ventilatory function studies be undertaken in this part of the world to see if there is any difference with known values in other part of the world. In this study we determine the impact of sickle cell anaemia on the pulmonary function indices of patients attending the paediatric sickle cell clinic at the University of Nigeria Teaching Hospital (UNTH) Enugu, south-east Nigeria and compare it with matched control and other findings elsewhere. Subjects and methods Study area This study was carried out in UNTH Ituku-Ozalla Enugu, south-east Nigeria. The hospital is a referral centre for various health facilities in Enugu state and her environs. Enugu has a population of 3.5 million people according to the National population census.12 Study population 4 These were patients with haemoglobin genotype HbSS attending the paediatric sickle cell clinic at UNTH, who fulfilled the criteria for inclusion into the study. The controls comprised healthy children with haemoglobin genotype HbAA matched for age, sex and height randomly selected from primary and post primary schools within Enugu metropolis. All the controls also fulfilled the criteria for inclusion into the study. Ethical Consideration and Consent Ethical clearance for the study was obtained from the Research and Ethical Committee of the UNTH and the Post Primary Schools Management Board, as well as from the Headmasters/mistresses and principals of the selected primary and secondary schools whose pupils/students were expected to participate in the study. Informed written consent was obtained from the parents or guardians as appropriate prior to the recruitment of their children or wards into the study. Verbal consent was also obtained from each of the study participants. Study design A cross sectional study of lung function was conducted in Enugu metropolis. The study was compiled, analyzed and proofread between October 2014 and January 2015. Children with sickle cell anaemia in steady state aged between 6-20 years who are willing to participate fully with a consent obtained from the patient and parents/ guardian as applicable are included in the study while children with presence of spinal deformity or any acute or chronic cardio-respiratory disease which may affect the lung function indices sought were excluded from the study. For the controls, the selection process was random, by multistage simple ballot. All the post-primary schools selected were government institutions which have a fair representation of students from all social strata. All the schools selected were visited by the researchers. In each of the schools, two streams of each class were randomly selected by simple ballot. In each of the selected streams the students or pupils as appropriate were first stratified according to age, sex, and height. 5 Pilot Study A pilot investigation was undertaken in a group of 10 sickle cell (HbSS) patients, and an equal number of healthy controls with genotype HbAA who were subsequently excluded from the main study. Pulmonary function tests Measurements of the peak expiratory flow rate were taken using a single mini Wright peak flow meter [Airmed, Clement Clarke International Limited Harlow, England W19516]. The instrument was calibrated from the factory to a maximum PEFR of 800 litres per minute (l/min). Measurements of the Forced Vital Capacity (FVC) and the Forced Expiratory Volume in 1 second (FEV1) were taken using an automated single breath vitalograph [Spirovit-SP-1, SCHILLER-AG, AH gasse 68, post fach, 6340 Barr, Switzerland]. Before the commencement of the study, the researchers were trained on the use of all equipment for the present study by the Chief respiratory technician attached to the respiratory laboratory at UNTH. On completion of the training, the researchers and the Chief respiratory technician independently evaluated FVC, FEV1 and PEFR on seven subjects randomly selected from the children’s’ outpatient clinic. The results obtained by the two observers were subjected to statistical analysis using the student t-test. There was no statistically significant difference between the results obtained by the two observers (p>0.05). The procedure involved taking in as deep a breath as possible, and then applying their lips firmly around the mouth piece to avoid any air leaks. Thereafter, the subject then breathed out as quickly and as forcibly as possible into the peak flow meter. Recordings were made without nasal clips. 6 After each subject had become familiar with the technique the procedure was repeated three times, and the best of these was recorded. For the spirometry, the same procedure was repeated. After a rest period of about 5 minutes, the subjects after taking as deep a breath as possible applied their lips tightly to the mouth piece of the spirometer to avoid any air leaks. They were instructed to blow into the mouth piece as rapidly and completely as possible until they were told to stop. Weight in kilograms scale [Deteco scales Inc. Brooklyn, New York, USA] sensitivity 0.5kg, standing height in centimeters (cm) [stadiometer CMS weighing equipment of 17 Campdem Road, London, NW1]. Were also taken The social classes of subjects were determined using the mean of father’s occupation and mother’s education. Socio-economic index scores were awarded to each child using the method recommended by Oyedeji to assign family socio-economic class in their work done in a similar setting. Data Analysis All data were coded, entered, and then analyzed using the Statistical Package for Social Sciences program (SPSS), version 17. Results were presented in cross tabulation and tables. A value less than 0.05 were accepted as significant for each statistical test Results A total of 80 subjects were recruited into the study, comprising 40 HbSS patients and an equal number of controls (healthy children of similar age, sex and height). The distribution of the study population according to age and sex is illustrated in Table I. The distribution of the HbSS patients and controls according to socio-economic groups is shown in Table II. 7 Table III showed that children with sickle cell anemia had lower values of FEV1 (1.6±0.52), FVC (1.76±0.95) and PEFR (309.00±82.64) when compared to those with normal Haemoglobin genotype FEV1 (12.01±0.53), FVC(2.12±0.54) and PEFR (364.10±87.85) and is statistically significant The mean FVC, FEV1/FVC and PEFR were also higher in the male controls compared to the HbSS males but these differences were not statistically significant (p>0.05). Female controls had significantly larger FEV1, FVC and PEFR compared to the SS females (p<0.05). Table IV The FEV1 increased consistently with age in both the HbSS patients and controls. The controls had significantly higher FEV1 in all the age groups compared to the HbSS population (P<0.05). Table V and figure 1 The FVC increased consistently with age in both the HbSS patients and controls. The controls had higher values of FVC compared with the HbSS patients in all the age groups. These differences in FVC values were only significant in the11-15years of age (P<0.05). (Table VI and figure 2) The PEFR increased consistently with age in both the HbSS patients and controls. The controls had comparable values of PEFR with the HbSS patients up to 10 years of age. The PEFR in the controls was however, generally higher than PEFR in the HbSS patients at all ages but became significantly higher from 11-20years of age (P<0.05). (Table VII and Figure 3) Table VIII illustrates a comparison of the observed mean FEV1 of controls in the present study with values predicted from previous studies at given heights in both males and females. There was no statistically significant difference between FEVI observed in the present study and those predicted by the two previous studies. 8 Discussion The mean FEV1, FVC and PEFR were all significantly larger in the controls compared to the HbSS patients, while comparable values of FEV1/FVC ratio were documented in the two groups. Sylvester et al [8] also noted similar findings in his study. The limitation of using FEVI/FVC ratio in isolation to determine the severity of obstructive lung disorder has been reported. This is because both FEV1 and FVC may decline with the progression of sickle cell disease Koumbourlis [13] et al also noted decreased lung volume parameters in sickle cell anemia children, he noted that in sickle cell disease (especially among patients with Hb SS), abnormal lung function may be present. Airway reactivity had been implicated in the pathogenesis Some authors have attributed the lower values of lung function indices in HbSS patients to the fact that they have a shorter thorax relative to body size as well as a narrower lateral chest diameter compared to controls of same ethnicity.[14] These differences in thoracic and lung volumes are believed to result in a reduction in the ratio of total lung capacity (TLC) to vital capacity in the HbSS patients. When lung volumes were analyzed according to age, the mean FEV1 was significantly larger in the controls compared to the HbSS patients at all ages matched. Mean FVC and mean PEFR were also generally higher in the controls compared to HbSS patients at all ages. These differences however were not significant in all cases. Values of mean FVC and mean PEFR were comparable in the younger HbSS patients and controls (under 11 years of age). Significant differences appeared in mean PEFR with age, resulting in significantly higher levels in controls compared to the HbSS patients from 11 years. 9 When lung function was analyzed according to sex, no significant difference was noticed between both sexes in the HbSS patients. Similarly in the control group, no significant differences were observed between the lung function test values in the males and females. This finding of comparable lung function between the HbSS males and females is similar to those of previous authors.[15] They concluded that gender may not be a very important determinant of pulmonary function in the HbSS patients. Similar to findings in the present study, other workers have also reported marginally but not significantly higher values of lung function tests in healthy males compared to healthy females with HbAA genotype. In the contrary, Oko –ose [16] and his colleagues in their own study found significantly higher mean values of respiratory function tests in females compared to males. The larger values of weight and body surface areas in females explained this gender variations.. Some workers have reported a plateau effect in lung function of normal males and females at a certain age in life which is believed to be variable.[17] This plateau effect was noted at 23 years for males and 19 years for females.[17] When PEFR in controls in the present study were compared to previously reported values in healthy subjects, comparable values were observed in both sexes.[18] but significantly higher than values predicted by Onadeko [18] et al at certain heights. In the control females, the PEFR in the present study were comparable with values in a previous study [18] and maintained consistently but marginally higher levels except between 135-147.9cm and 161-173.9cm where values were significantly higher than those predicted by Onadeko et al [13]. One can therefore surmise that PEFR in the Caucasian population approximated those in the present study in both sexes. However at higher heights in the males, Caucasian values seemed to overtake those of the present study. 10 A reduction in lung volume of 1-2% in the sitting position compared to the standing position has been reported by previous authors. This effect is noted to be greater in obese persons. The observation of comparable PEFR values in the present study compared to Caucasian values did not come entirely as a surprise despite previous reports of higher values of lung function parameters in most Caucasian studies compared to studies in Africans.[1921].Reasons adduced for this previous higher values of lung function tests in Caucasians compared to Africans include differences in environmental conditions like nutrition, infections, as well as genetic differences in chest size, shape and possibly lung volumes. Though some workers [22] have reported adverse effects of low socio-economic class on lung function tests, in the present study the socio-economic spread of the SS patients and matched controls were comparable. Any differences in lung function noticed are therefore unlikely to result from differences in social class between the two groups. Conclusions The lung function indices were significantly lower in children and adolescents with sickle cell anaemia compared to sex, age and height matched controls with haemoglobin genotype HbAA. The lung function abnormality noted in HbSS patients appears to be restrictive rather than obstructive in nature. Lung function indices correlate well with age and stature in south eastern Nigeria as has been reported in other regions of the world. Competing interests The authors declare that we have no competing interests. 11 Authors’ contributions Drs. KIA had primary responsibility for protocol development, patient screening, enrolment, outcome assessment, preliminary data analysis, and writing of the manuscript. Dr. OOI and JMC also supervised the design and execution of the study, and performed the final data analyses. Drs. KIA, OOI and JMC AEO, IAN, EIJ, IBC participated in writing of the manuscript. Funding There were no sponsors or grants given to us in this study. We bore all the expenses accrued in this study Acknowledgements We thank the statistician who helped in data entering and analysis. 12 References 1. Yawn, BP; Buchanan, GR; Afenyi-Annan, AN; Ballas, SK; Hassell, KL; James, AH et al. “Management of sickle cell disease: summary of the 2014 evidence- based report by expert panel members,” JAMA 2014; 312: 1033 -1033. 2. Araba AB. A Survey of haematological variable in 600 healthy Nigerians. Niger Med J 1976;6:49-53. 3. Modell M, Dailison D. Bernadette M, Matthew D. Global epidemiology of haemoglobin disorder and derived service indicators. Bull World Health Organ 2008;86:480-7. 4. Chinawa JM, Ubesie AC, Chukwu BF, Ikefuna AN, Emodi IJ. Prevalence of hypoxemia among children with sickle cell anemia during steady state and crises: A cross-sectional study. Niger J Clin Pract 2013;16:91-5 5. Gladwin MT, Castro OL, Minniti CP, Gordeuk VR. Abnormal pulmonary function and associated risk factors in children and adolescents with sickle cell anemia. J Pediatr Hematol Oncol. 2014; 36:185-9. 6. Vig R, Machado RF. Pulmonary complications of hemoglobinopathies. Chest 2010; 138 973-983. 7. Ataga KI, Moore CG, Hillery CA, Jones S, Whinna HC, Strayham A. Coagulation activation and inflammation in sickle cell disease-associated pulmonary hypertension. Haematologica 2008;93;20-6. 8. Sylvester KP, Patey RA, Milligan P, Dick M, Rafferty GF, Rees D, Thein SL, Greenough A. Pulmonary function abnormalities in children with sickle cell disease. Thorax 2004; 59:67–70. 9. Arteta M, Campbell A, Nouraie M, Rana S, Onyekwere OC, Ensing G, Sable C, Dham N, Darbari D, Luchtman Jones L, Kato GJ, Gladwin MT, Castro OL, Minniti CP, Gordeuk VR. Abnormal pulmonary function and associated risk factors in children and adolescents with sickle cell anemia. J Pediatr Hematol Oncol. 2014; 36:185-9. 10. Powars DR, “Sickle cell anemia and major organ failure,” Hemoglobin.1990; 14: 573–598. 11. Anthi A, Machado RF, Jison ML “Hemodynamic and functional assessment of patients with sickle cell disease and pulmonary hypertension,” The American Journal of Respiratory and Critical Care Medicine, vol. 175, pp. 1272–1279, 2007. 12. National population commission 2006 provisional census figures. Census news 31:14. 13. Koumbourlis Ac, Hurlet- Jensen A, Bye MR. Lung function in infants with sickle cell disease. . Pediatr Pulmonol. 1997; 24: 277-81. 13 14. .Pianosi P1, D'Souza SJ, Charge TD, Esseltine DE, Coates AL.Pulmonary function abnormalities in childhood sickle cell disease. . J Pediatr. 1993; 122: 366-71. 15. VanderJagt , Trujillo MR, Jalo I, Bode-Thomas F, Glew HR, “Pulmonary function correlates with body composition in Nigerian children and young adult with sickle cell disease,” Journal of Tropical Pediatrics 2007;54: 87–93. 16. Oko-Ose JN., Iyawe 1V, Egbagbe E, Ebomoyi M .Lung Function Tests in SickleCell Patients in Benin City. ISRN Pulmonology 2012; 2012 :1-5. 17. Vichinsky, Gladwin MT. “Pulmonary complications of sickle cell disease,” The New England Journal of Medicine 2008; 359: 2254–2265 18. Onadeko BO, Iyun AO, Sofowora EO, Adamu SO. Peak expiratory flow rate in normal Nigerian children. Afr J Med Sci 1984; 13: 25-32. 19. Ali MA, Vahlia KV. Some observations of peak expiratory flow in normal school children in northern Nigeria. West Afr J Med 1991; 10: 141-9. 20. John LH, John RO, Kathleen BF. Spirometric Reference Values from a Sample of the General U.S. Population. Am J Respir Crit Care Med. 1999; 159:179–187. 21. Jaja SI, Fagbenro AO. Peak expiratory flow rate in Nigerian school children. Afr J Med Med Sci 1995;24:379–84 22. Murray AB, Cook CD. Measurement of peak expiratory flow rates in 220 normal children from 4.5-18 years. J Paediatr 1963; 62: 186-9. 23. Aderele WI, Oduwole O. Peak flow rate in healthy school children. Nig J Paediatr 1983; 10: 45 – 55. 14 Table I: Age and Sex Distribution of HbSS and Controls Age group (Last birthday) HbSS n (%) 6-10 4(20.0) 4(20.0) 3(15.0) 3(15.0) 7(17.5) 7(17.5) 11-15 11(55.0) 12(60.0) 12(60.0) 12(60.0) 23(57.5) 24(60.0) 16-20 5(25.0) 4(20.0) 5(25.0) 5(25.0) 10(25.0) 9(22.5) Total 20(100.0) 20(100.0) 20(100.0) 20(100.0) 𝓧 = 0.155 Male Control n (%) 𝞉f= 2 Female HbSS Control n (%) n (%) p- value = 0.05 Total HbSS n (%) 40(100.0) Control n (%) 40(100.0) 15 Table II: Distribution According to Socio-economic Classes of HbSS and Controls Socioeconomic Class HbSS Control n (%) n(%) I 1(2.5) 1(2.5) II 4(10.0) 6(15.0) Middle III 11(27.5) 10(25.0) Lower IV 22(55.0) 18(45.0) V 2(5.0) 5(12.5) 40(100.0) 40(100.0) upper Total 𝓧 = 2.133 𝞉f= 4 p- value = 0.7 16 Table III: Pearsons correlation coefficient for HbSS and Controls (Total study population ) Variables FEV1.0 (Litres) HbSS FVC (Litres) PEFR (Litres/min) FEV1.0 (Litres) Control FVC (Litres) PEFR (Litres/min) Age (year) 0.81 0.71 0.75 0.80 0.79 0.86 Standing Height (cm) 0.87 0.78 0.79 0.89 0.85 0.90 Weight (Kg) 0.85 0.79 0.78 0.85 0.81 0.88 Chest Circumference(cm) 0.72 0.70 0.71 0.67 0.65 0.70 Body Surface Area (m2) 0.88 0.81 0.80 0.87 0.83 0.88 𝓧= 𝞉f= p- value = 0.01 17 Table IV: Relationship between FEV1, FVC, PEFR, Height, Body Surface Area (BSA) and Weight in Males (HbSS and Controls) Correlation(s) Intercept Gradient SD Regression Equation HbSS FEV1 and Height 0.90 - 3.61 0.04 0.51 FEV1= -3.61+ 0.04 Ht Controls FEV1 and Height 0.88 - 3.59 0.04 0.55 FEV1 =-3.59 + 0.04 Ht HbSS FEV1 and BSA 0.93 -1.37 2.34 0.53 FEV1 = -1.37 + 2.34BSA Controls FEV1and BSA 0.89 - 0.84 2.19 0.56 FEV1 =- 0.84 + 2.2 BSA HbSS FEV1 and Wt 0.95 - 0.52 0.06 0.54 FEV1 = - 0.52 + 0.1 Wt Controls FEV1 and Wt 0.89 0.19 0.51 0.56 FEV1 = - 0.19 + 0.51 Wt HbSS FVC and Height. 0.75 - 3.36 0.03 0.50 FVC= -3.36 + 0.03Ht Controls FVC Height 0.86 - 3.61 0.04 0.56 FVC = - 3. 61 + 0.04Ht HbSS FVC and BSA 0.79 - 1. 21 2.35 0.57 FVC = - 1.21 + 2. 35 BSA Controls FVC and BSA 0.86 - 0.78 2.22 0.56 FVC = - 0.78 + 2.22 BSA HbSS FVC and Weight 0.81 - 0. 41 0.07 0.56 FVC = - 0.41 + 0.07 Wt Controls FVC and Weight 0.85 0.29 0.05 0.56 FVC = - 0.29 + 0. 05 Wt HbSS PEFR and Height 0.72 -301.96 4.22 59.68 PEFR=-301.9 + 4.22 Ht 18 Controls PEFR and Height 0.90 -562.45 6.35 90.99 PEFR= -562.5 + 6.35 Ht Table V. Relationship between FEV1, FVC, PEFR, Height, Body surface Area and Weight in Females (HbSS and Controls) Correlation(s) Intercept Gradient SD Registration Equation HbSS FEV1 and Height 0.82 - 3.27 0.33 0.39 FEV1 =- 3. 27 + 0.33 Ht Controls FEV1 and Height 0.92 - 2.84 0.03 0.3 9 FEV1 -2.84 + 0.03 Ht HbSS FEV1 AND BSA 0.83 - 0.83 1.84 0.39 FEV1 = – 0.83 + 1.84 BSA Controls FEV1 AND BSA 0.87 -0.11 1.54 0.37 FEV1 = 0.11 + 1.54 BSA HbSS FEV1 and Wt 0.80 0.20 0.04 0.38 FEV1 = 0.19+0.04 Wt Controls FEV1 and Wt 0.84 0.62 0.03 0.36 FEV1 = 0. 62 + 0. 04 Wt HbSS FVC and Ht 0.85 - 3. 28 0.03 0.40 FVC = - 3.28 + 0. 03 Ht Controls FVC and Ht 0.85 -2.38 0.03 0.36 FVC = - 2.38 + 0.03 Ht HbSS FVC and BSA 0.86 - 0. 74 1.89 0.40 FVC = - 0.74 + 1.89 BSA Controls FVC and BSA 0.82 0.15 1.44 0.34 FVC = 0. 15 + 1. 44 BSA HbSS FVC and Wt. 0.83 0.31 0.04 0.39 FVC = 0.31 +0.04 Wt Controls FVC and Wt. 0.80 0.82 0.03 0.33 FVC = 0.82 + 0.03 Wt 19 HbSS PEFR and Height 0.88 -614.95 6.31 73.68 PEFR= -614 + 6.31 Ht Controls PEFR and Height 0.89 -476.49 5.75 67.27 PEFR = -476.5 + 5.75 Ht Table VI: Comparison of Observed PEFR values for Male Controls in Present Study and values Predicted from Previous Studies Height(cm) 122-134.90 135-149.90 148-160.90 161 – 173.90 Observed PEFR Predicted PEFR (Present study) (Previous Study) Mean SD 273.94 43.67 330.89 402.71 505.82 32.73 24.75 74.80 Mean SD P ++ 265.38 21.67 > 0.05 + 262.71 26.86 > 0.05 ** 240.74 21.11 > 0.05 + 328 .62 20.46 > 0.05 ++ 318 57 16.50 > 0.05 ** 292.54 16.08 < 0.05 + 409.20 24.18 > 0.05 ++ 383 .60 19.52 > 0.05 ** 355.88 19.01 > 0.05 +469 .05 19.01 > 0.05 20 Prediction equation (Previous Studies): +Murray et al22 (Boston); **Onadeko et al18 (Nigeria); ++ Aderele et al23 (Nigeria) ++431.90 15.34 > 0.05 ** 402. 92 14.94 > 0.05 21 Fig. 1: FEV1 in Relation to Age for HbSS and Controls 3.5 3.0 KEY (Litres) 2.0 AGE (SS) FEV1 2.5 1.5 FEV1 (control) AGE (Control) FEV1 (SS) 1.0 .5 6 8 10 12 14 16 18 20 22 Age (Years) Figure 2: FVC in Relation to Age for HbSS and Controls 4.0 3.5 FVC (Litres) 3.0 KEY 2.5 2.0 FVC (SS) 1.5 AGE (SS) FVC (Control) 1.0 AGE (Control) .5 6 8 10 12 Age (Years) 14 16 18 20 22 22 Figure 3: PEFR in Relation to Age for SS and Controls 700 PEFR ( Litres Per min) 600 500 KEY 400 PEFR (SS) AGE (SS) 300 PEFR (Control) AGE (Control) 200 100 6 8 10 12 Age (Years) Tables’ abbreviation Ht = Height (cm) Wt = Weight (kg) BSA = Body Surface Area (m2) SD = Standard Deviation 14 16 18 20 22
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