Prevalence and reversibility of lower airway obstruction in children with sickle cell disease
Objective: To determine the prevalence and reversibility of lower airway obstruction (LAO) in children and adolescents with hemoglobin SS sickle cell disease (HbSS SCD). Study Design: Retrospective evaluation of lung function in a cross-section of 35 African American and 28 Hispanic children and adolescents with HbSS SCD. Lung function was evaluated with maximal respiratory flow-volume curves and body plethysmography. Each patient was assigned to 1 of 3 patterns of lung function (normal, obstructive, or restrictive). Airway hyperresponsiveness was assessed by means of a trial with bronchodilator. Results: Normal pattern was detected in 57% of the patients, LAO in 35%, and restrictive lung disease in 8%. Positive response to bronchodilator was documented in 30% of those with normal pattern of lung function, 78% in those with LAO, and 67% of those with restrictive lung disease. The pattern of lung function was not associated with race or with history of vaso-occlusive crises, acute chest syndrome, reactive airways disease/asthma, or long-term transfusion therapy. Conclusion: Obstructive lung disease possibly precedes the development of restrictive lung disease, and airway reactivity may be part of the pathogenic mechanism. (J Pediatr 2001;138:188-92) 
Airway hyperreactivity in children with sickle cell disease
Progressive restrictive defect with increasing age, obstructive lung disease, and bronchodilator responsiveness have been reported in sickle cell disease (SCD). Because airway hyperreactivity (AHR) can be underestimated when assessed by bronchodilator responsiveness in patients with normal baseline lung function, the aim of this study was to investigate the prevalence of AHR in SCD by cold-air bronchial provocation testing, and to assess whether AHR can be present in symptom-free patients with SCD. Forty patients aged 6 to 19 years (mean, 10.7 years ± 3.5 SD) performed pulmonary function tests. Eighteen were known to have a history of reactive airway disease (RAD group), and 22 had no known history of RAD (non-RAD group). A control group, aged 6 to 17 years (mean, 10.5 ± 3.1 years), consisted of 10 siblings of the non-RAD SCD group. There were no significant differences in age and height among the groups. If the forced expiratory volume in 1 second (FEV 1 ) was greater than 70%, cold air challenge (CACh) was performed; if the FEV 1 was less than 70%, aerosolized bronchodilator therapy was given. A decrease in FEV 1 of more than 10% after CACh or an increase in FEV 1 of 12% or greater after bronchodilator inhalation was considered evidence of AHR. In the RAD group, the total lung capacity was 88.9% ± 14.0% of race-corrected predicted values, the forced vital capacity was 91.2% ± 12.6%, and FEV 1 was 85.3% ± 16.2%. The mean maximal percent fall in FEV 1 after CACh ( n = 13) was 18.5% ± 9.6% and was greater than 10% in 11 of 13 patients. The mean increase in FEV 1 after bronchodilator therapy ( n = 5) was 11.5% ± 8.3%, and it was greater than 12% in 4 of 5 patients. In the non-RAD group the baseline total lung capacity was 101.6% ± 11.7%, forced vital capacity was 95.5% ± 10.2%, and FEV 1 was 93.3% ± 13.2%. The mean maximal percent fall in FEV 1 after CACh ( n = 19) was 14.1% ± 8.8% and was greater than 10% in 13 of 19 patients. The mean increase in FEV 1 after bronchodilator therapy ( n = 3) was 14.7% ± 11.3%, and was 12% or greater in 1 of 3 patients. In the control group the baseline total lung capacity was 105.7% ± 12.1%, forced vital capacity was 96.2% ± 11.1%, and FEV 1 was 92.9% ± 10.3%. The mean maximal percent fall in FEV 1 was 5.0% ± 2.5%, and was greater than 10% in none of 10 patients. The prevalence of AHR in the control group, the RAD group, and the non-RAD group was zero, 83%, and 64%, respectively ( p < 0.0001). The overall prevalence in the SCD group was 73%. We conclude that there is a high prevalence of AHR in children with SCD and that airway hyperreactivity may exist in patients with SCD even in the absence of the clinical symptoms of RAD. AHR may be a significant component of sickle cell lung disease. (J Pediatr 1997;131:278-83 ) 
Plasma zinc status, growth, and maturation in children with sickle cell disease
Objective: The objective of this study was to determine the relation of plasma zinc (Zn) status to growth and maturation in children with SS genotype sickle cell disease. Study design: A cross-sectional study of 104 subjects who were 50% female and ranged in age from 0.4 to 18 years was performed. Measures included plasma Zn concentration (Znp), height, weight, skinfold thicknesses, elbow breadth, upper arm muscle area, and fat-free mass and fat mass by total body electrical conductivity. Skeletal maturation was assessed by hand-wrist x-ray evaluation and sexual maturation by Tanner stage. Results: A total of 44% of the patients had low Znp (<10.7 μmol/L [70 μg/dl]); those with low Znp had significantly lower SD scores for height (p = 0.003), weight (p = 0.003), upper arm muscle area (p = 0.045), fat-free mass (p = 0.025), and elbow breadth (p = 0.017) and greater skeletal maturation delay (p = 0.04). In older children (>9 years) low Znp was associated with decreased Tanner scores for pubic hair (p = 0.001) and breast and genital maturation (p = 0.009). No significant differences were seen in age, sex, or fat stores according to Zn status. Conclusions: Decreased plasma Zn is common in children with SS genotype sickle cell disease and is associated with decreased linear growth, skeletal growth, muscle mass, and sexual and skeletal maturation. (J Pediatr 1998;132;467-71) 
Sickle Cell Disease in Pregnancy: Maternal and Fetal Outcome in Port Harcourt, Nigeria
Background: Medical experts for many years have daunted the occurrence of pregnancy in homozygote sickle cell patients. This is because of associated high risk for mother and fetus.
The aim of this study is to determine the prevalence and maternal and fetal outcome of pregnant mothers with sickle cell disease at the University of Port Harcourt Teaching Hospital, Nigeria.
Materials and Methods: This was a retrospective descriptive study of medical case files of all booked pregnant mothers who attended the antenatal clinic of the University of Port Harcourt Teaching Hospital, Nigeria from January 2007 to December 2011. The parameters extracted from the folders included: age, level of education, hemoglobin genotype, full blood count, malaria parasite, urine analysis and culture, complications of pregnancy, Apgar scores and birth weight.
Results: A total of 4,650 mothers were booked for antenatal care. Eight hundred and forty (18.1%) of them were HbAS, five (0.1%) were HbAC, nine (0.2%) were HbSS and 1(0.02%) HbSC. Age and gestation at booking were 18–42 years (mean 28.6± 2.1) and 9–34 weeks gestation (mean 16.6±3.3), respectively. Malaria and vaso-occlusive crisis were the commonest complications encountered in pregnancy. Twenty percent of women had induction of labour and 60% were delivered by emergency caesarean section. Twenty percent had postpartum haemorrhage. Forty four percent of women delivered before 37 completed weeks. Birth weight below 2500 g occurred in 50% of singleton pregnancies. Two neonates developed transient complications related to maternal opiate exposure postnatally. There were 2(20%) maternal and fetal losses from toxaemia of pregnancy.
Conclusion: Pregnancy is uncommon among females with sickle cell disease in Port Harcourt, Nigeria. Sickle cell disease remains a severe complicating factor to pregnancy and associated with increased fetal and maternal losses. 
Trace Elements Deficiency in Patients with Homozygous Sickle Cell Disease
Aim: The serum trace elements statuses of sickle cell patients attending at General Hospital Owerri, Nigeria were investigated to determine whether or not the serum levels of these elements were normal.
Materials and Methods: One hundred confirmed sickle cell patients (HbSS) age 5–30 years were selected. One hundred normal subjects (HbAA) age 5–30 years were used as control.
Results: The levels of trace elements were significantly decreased in sickle cell anemia (p<0.05), except copper, when compared with the control.
Conclusion: The result suggests, but not conclusively, that supplementation of sickle cell patients with food and drug containing trace elements might be helpful, particularly if diminished mineral levels predispose patients to crises. 
 Koumbourlis, A.C., Zar, H.J., Hurlet-Jensen, A. and Goldberg, M.R., 2001. Prevalence and reversibility of lower airway obstruction in children with sickle cell disease. The Journal of pediatrics, 138(2), pp.188-192.
 Leong, M.A., Dampier, C., Varlotta, L. and Allen, J.L., 1997. Airway hyperreactivity in children with sickle cell disease. The Journal of pediatrics, 131(2), pp.278-283.
 Leonard, M.B., Zemel, B.S., Kawchak, D.A., Ohene-Frempong, K. and Virginia, V.A., 1998. Plasma zinc status, growth, and maturation in children with sickle cell disease. The Journal of pediatrics, 132(3), pp.467-471.
 Ugboma, H. A. A. and George, I. O. (2015) “Sickle Cell Disease in Pregnancy: Maternal and Fetal Outcome in Port Harcourt, Nigeria”, Journal of Advances in Medicine and Medical Research, 7(1), pp. 40-44. doi: 10.9734/BJMMR/2015/11602.
 Nnodim, J., Samuel, M., Dioka, C. E., Onah, C., Ihim, A. and Atuegbu, C. (2014) “Trace Elements Deficiency in Patients with Homozygous Sickle Cell Disease”, Journal of Advances in Medicine and Medical Research, 4(21), pp. 3878-3883. doi: 10.9734/BJMMR/2014/7489.