Pulmonary function indices in children with sickle cell anemia in Enugu,south-east Nigeria

Objectives:

To determine the pulmonary function indices of children with sickle cell anemia (SCA) attending the pediatric sickle cell clinic at the University of Nigeria Teaching Hospital, Enugu, south-east Nigeria and to compare these indices with the results obtained from other regions.

Methods:

A case control study of lung function in children with SCA aged 6-20 years. The study was carried out in the University of Nigeria/University of Nigeria Teaching Hospital, Enugu State, Nigeria between October 2014 and January 2015. Measurements of the peak expiratory flow rate, forced vital capacity (FVC), and forced expiratory volume in one second (FEV1) were evaluated.

Results:

A total of 80 subjects were recruited into the study, comprising 40 homozygous HbSS (hemoglobin SS) patients and an equal number of controls. Children with SCA had statistically lower values of FEV1 (1.6±0.52), FVC (1.76±0.95), and peak expiratory flow rate (PEFR) (309.00±82.64) when compared with normal hemoglobin genotype FEV1 (12.01±0.53), FVC (2.12±0.54), and PEFR (364.10±87.85). The mean FVC, FEV1/FVC, and PEFR were also higher in the male control group compared with the HbSS male group, but these differences were not statistically significant. Female controls had significantly larger FEV1, FVC, and PEFR values compared with the HbSS females.

Conclusion:

The lung function indices were significantly lower in children and adolescents with SCA compared with the matched controls with a hemoglobin genotype AA.Sickle cell anemia (SCA) is a genetic hematological 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 <5 years worldwide or 6.4% in Africa.2 The prevalence of SCA in Nigeria ranges from 0.4-3%.3 Approximately 85% of sickle cell disorders and >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 results from obstruction of the microvasculature by the sickle cells and red blood cell hemolysis, which causes multi-systemic manifestation. The lungs are affected in a variety of ways by these pulmonary insults, and recurrence overtime may leave the lungs with chronic interstitial, parenchymal, or vascular damage that compromises pulmonary function.5,6 It has been documented that the prevalence of hypoxemia among SCA children was 13%.4 This prevalence was attributable to the chronic anemic state, micro vascular occlusion of the circulation by sickle 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 This is defined as bone and joint pain or multiple sites of pain needing analgesics or hospitalization.8 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 sickle cell disease (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 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 the total lung capacity (TLC) of 50% has been reported in advanced forms. Acute chest syndrome refers to a spectrum of pulmonary pathology having in common, chest pain, fever, dyspnea with abnormal clinical, and radiologic chest signs as well as leucocytosis.10,11 It is the most common cause of death in children with sickle cell anemia over 10 years of age.12 The etiology of ACS is not clear, lung and bone infarction, infection, and acute pulmonary sequestration, among other possible causes have been proposed.10 In children with sickle anemia in steady state, the major abnormality in pulmonary function is a restrictive pathology, characterized by a slight decrease in total lung capacity, with attendant ventilation perfusion mismatch.10 This can cause a defect in diffusion capacity for carbon monoxide.10 These abnormalities worsen with age and are associated with increases in pulmonary-artery pressures.11 Whereas some studies have documented impaired lung function in SCA (hemoglobin SS) patients,8-10 previous studies8-10 reported what appears to be contrasting findings when the lung function in children with SCA and those of healthy controls with normal hemoglobin genotype were compared. It is therefore necessary that ventilatory function studies be undertaken in this parts 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 SCA on the pulmonary function indices in patients attending the pediatric sickle cell clinic at the University of Nigeria Teaching Hospital (UNTH) Enugu, south-east Nigeria and compare it with matched controls and other studies. Many studies have described and assessed the pattern of pulmonary function in SCD from childhood to adulthood, but much is not known on this topic in South Eastern Nigeria. Most of the original studies are from western Nigeria.13,14 This study could therefore corroborate or refute regional or ethnic differences in lung function in children with SCD. The study hypothesis seeks to answer the following questions? Do children with SCA attending UNTH Enugu present with any alteration in lung function? If they do, is there any gender and age difference? Are these lung volume findings similar to that obtained from other region?     

Rural-urban disparities in maternal immunization knowledge and childhood health-seeking behavior in Nigeria: a mixed method study

Background

Immunization and appropriate health-seeking behavior are effective strategies to reduce child deaths.

Objectives

To compare maternal knowledge about immunization, use of growth chart and childhood health-seeking behavior in rural and urban areas.

Methods

A cross-sectional comparative study done in Lagos, Nigeria. Questionnaire survey and focus group discussions were done. 300 respondents were selected by multi-stage sampling while discussants were purposively selected.

Results

Awareness of immunization was high but knowledge of vaccine preventable diseases (VPDs) was poor in both areas. Urban women utilized preventive services more; growth monitoring (p<0.001) and immunization (p<0.001) while higher proportions of rural women utilized nutritional counseling (p=0.005) and treatment of illness (p<0.001). Growth chart utilization was better in the urban areas (p<0.001). Increasing maternal education increased use of growth chart in both areas. Both groups of women use multiple treatment sources for children (more in urban), determined by cost, time, perceived severity of illness and type of ailment (urban) and peculiarity of illness (rural). There is a preference for orthodox care in the rural area.

Conclusions

Knowledge of VPDs was poor and multiple treatment sources were common among rural and urban women. Education is vital to improve immunization knowledge and health-seeking behavior in both areas.     

Solution and Colour Stability of the Bark Extract of Bridelia ferruginea Benth

A solvent system consisting of water: propylene glycol: ethanol (2:2:1) was found suitable for diluting the ethanol bark extract and for reconstituting the freeze-dried extract of Bridelia ferruginea Benth for preparation of a gargle formulation. Such solutions remained clear when subjected to high storage temperatures and eight freeze-thaw cycles. The wine colour of Bridelia bark extract solution darkened on storage. Darkening was accelerated by heat while light had minimal effect. Inclusion of certain antioxidants—sodium metabisulphite, ascorbic acid and citric acid, reduced the colour change. Heating the freeze-dried extract at 115°C for 4 h and at 130°C for at least 1 h arrested the colour change. When lime juice was added to the solution of the heated powder, the colour of the resulting solution became unstable implicating a pH effect on colour stability. The colour of bottle and use of carton for storage had no appreciable effect on colour stability.     

Renal vein thrombosis in the neonate: a case report and review of the literature

We report a case of bilateral renal vein thrombosis in a neonate, and reviewed available literature. The neonate was a macrosomic male born to a mother with glycosuria in pregnancy. There was delay in commencing breasttfeeding for up to 36 hours due to lack of lactation by themother. Clinical and laboratory examination showed enlarged palpable kidneys and azotemia. Diagnosis of bilateral renal vein thrombosis was confirmed by renal ultrasonography. The child is being managed conservatively. Measures aimed at prevention of the disease because of its poor outcome were highlighted.     

Sensorineural hearing loss in Nigerian children with sickle cell disease

AIM: To study the prevalence and pattern of sensorineural hearing loss (SNHL) in Nigerian children with sickle cell disease (SCD). MATERIALS: Fifty-two children with SCD were assessed. Otologic examination and audiometric tests were performed. There were 36 males and 16 females. Their age ranged from 6 to 19 years. RESULT: Seven children had hearing impairment. After hemoglobin categorization, all the SCD patients were found to belong to the homozygous (HbSS) group. There were no HbSC nor other variants detected. Prevalence of SNHL was 13.4% in these patients but the general population prevalence rate found in a control group was 6.2%. CONCLUSION: SCD has many organ manifestations and complication among which is sensorineural hearing loss. Regular audiologic assessment, counselling and rehabilitation of these patients with hearing aids are recommended.     

Antioxidant activity in HIV and malaria co-infected subjects in Anambra State,southeastern Nigeria

ObjectiveTo determine the antioxidant status of HIV and malaria co-infected participants.MethodsBlood samples collected from the 193 randomly recruited participants were used for HIV screening, Plasmodium falciparum antigen screening, malaria parasite density count, CD4⁺ T cell count, glutathione reductase, glutathione peroxidase and total antioxidant status measurement. Standard laboratory methods were used for the analysis.ResultsThe results showed that glutathione reductase, glutathione peroxidase, total antioxidant status and CD4⁺ T cell count were significantly lowered in symptomatic HIV participants with and without malaria co-infection (P<0.01) in each case compared with control participants. Also, glutathione reductase, glutathione peroxidise, total antioxidant status and CD4⁺ T cell count were significantly lowered in asymptomatic HIV participants with and without malaria co-infection (P<0.05) in each case, compared with control participants without malaria. Similarly, these antioxidants were significantly lowered in control participants with malaria infection (P<0.05) compared with control participants without malaria. The malaria parasite density in symptomatic HIV infected participants was negatively associated with glutathione reductase (r = ?0.906, P<0.01), glutathione peroxidase (r = ?0.719, P<0.01) and total antioxidant status (r = ?0.824, P<0.01).ConclusionsThe antioxidant activity was affected in HIV infected participants with malaria co-infection. Malaria co-infection in HIV seems to exert additional burden on antioxidants. This calls for concern in malaria endemic areas with increasing prevalence of HIV infection.     

Covalent binding and glutathione depletion in the rat following niridazole (ambilhar) pretreatment

In vivo and in vitro studies with rats have shown that (¹⁴C) niridazole (Ambilhar) binds covalently to tissue proteins, but not to nucleic acids. In the in vitro experiments, binding required the presence of NADPH in the incubation medium, suggesting the production of an active metabolite via a cytochrome P-450-mediated reaction. Niridazole also caused significant dose-dependent decreases in liver and kidney glutathione levels, even though it had no apparent effect on blood glutathione. Alteration of tissue glutathione availability by pretreatment with chloracetamide or cysteine respectively either increased or decreased the NADPH-dependent covalent binding. Pretreatment with phenobarbital, 3-methylcholanthrene or cobaltous chloride, which change the rate of metabolism of (¹⁴C) niridazole, similarly altered the extent of protein binding. It is shown that the decrease in tissue glutathione concentration is not due to an effect of the drug on the activities of either glucose-6-phosphate dehydrogenase or glutathione-S-transferases. However, there is a significant reduction in glutathione reductase activity in all the tissues studied. The possible relationships between the results obtained and the cytotoxic effects of niridazole have been discussed.     

The Epidemiology of Dependence in Older People in Nigeria: Prevalence, Determinants, Informal Care, and Health Service Utilization. A 10/66 Dementia Research Group Cross-Sectional Survey

OBJECTIVES: To describe the prevalence and determinants of dependence in older Nigerians and associations with informal care and health service utilization. DESIGN: A single‐phase cross‐sectional catchment area survey. SETTING: Dunukofia, a rural community in southeastern Nigeria. PARTICIPANTS: One thousand two hundred thirty‐eight adults aged 65 and older, for whom full data were available on 914. MEASUREMENTS: The full 10/66 Dementia Research Group survey protocol was applied, including ascertainment of depression, cognitive impairment, physical impairments, and self‐reported diagnoses. The interviewer rated dependence as not needing care, needing some care, or needing much care. The prevalence of dependence and the independent contribution of underlying health conditions were estimated. Sources of income, care arrangements, caregiver strain, and health service use are described according to level of dependence. RESULTS: The prevalence of dependence was 24.3% (95% confidence interval=22.1–26.5%), with a concentration in participants aged 80 and older. Only 1% of participants received a pension, and fewer than 7% had paid work. Those who were dependent were less likely than others to receive income from their family. Cognitive impairment, physical impairments, stroke, and depression were each independently associated with dependence. Depression made the largest contribution. Dependence was strongly associated with health service use (particularly private doctor and traditional healer services) and with high levels of out‐of‐pocket expenditure. CONCLUSION: In Nigeria, dependence is an important outcome given rapid demographic aging and increases in chronic disease prevalence in all developing regions. Enhancing the social protection of dependent older adults should be a policy priority. Cognitive and mental disorders are important contributors to disability and dependence; more attention should be given to their prevention, detection, and treatment.