Clinical Utilities of Electrocardiography in the Diagnosis of Myocardial Ischemia in Children With Sickle Cell Anemia: Correlation With Serum Cardiac Troponin I.

BACKGROUND: Sickle cell anemia (SCA) is associated with recurrent vaso-occlusive crisis (VOC) and the risk of myocardial ischemia (MI). This study investigated the utility of electrocardiography (ECG) and cardiac troponin I (cTnI) in diagnosing MI during VOC. MATERIALS AND METHODS: Children with SCA 5 to 15 years of age in VOC (patients) and age-matched and sex-matched steady-state controls were studied. Their ECG and cTnI levels were measured at contact and after 4 to 6 weeks. RESULTS: One hundred eighty-six children (93 patients and 93 controls) were studied. The mean (SD) ages of the patients and controls were 8.8 (3.2) and 9.0 (3.1) years, respectively. The mean MI score was significantly higher for the patients, 1.7 (1.2), than the controls, 1.3 (1.0), P=0.002. A significantly higher proportion of the patients, 18 (19.4%), also had significant ischemia compared with the controls, 8 (8.6%), P=0.016. The median (interquartile range) serum cTnI level was significantly higher in the patients than the controls, P=0.006. All 7 of the patients with elevated cTnI had VOC. No significant correlation was found between MI score and cTnI in both groups. CONCLUSIONS: cTnI is elevated and ECG features of MI worsen during VOC. Longitudinal studies to investigate their evolvement over time are advocated.

Juvenile Dermatomyositis in an 11 Year Old Nigerian-Boy: A Case Report and Review of Literature.

We report the case of an 11-year-old boy with proximal myopathy, heliotrope, and Gottron papule-like rashes. Serum chemistry revealed muscle enzyme elevations, whereas muscle biopsy histology showed necrosis and inflammation, which were in keeping with juvenile dermatomyositis. Plain radiographic examination of the thigh 3 weeks after commencing treatment with prednisolone was normal. The aim of this presentation is to highlight the diagnostic challenges posed by this rare condition in a resource-limited setting and to underscore the need for prompt diagnosis and appropriate management. We hope that this report will assist physicians practicing in similar settings to make a prompt and accurate diagnosis when confronted with the same disease.

Plasma and Cerebrospinal Fluid Beta-Endorphin Levels Show a Strong Association in Children with Cerebral Malaria.

BACKGROUND: Beta (ß)-endorphins are endogenous neuropeptides found in the plasma and cerebrospinal fluid (CSF) of humans but there have been reports of the relationship between the plasma and CSF ß-endorphin levels in different clinical conditions. However, the relationship between ß-endorphin levels in the plasma and CSF of children with cerebral malaria (CM) has not been reported. AIM: To determine the relationship between ß-endorphin levels in the CSF and plasma of children with CM. SETTINGS AND DESIGN: This cross-sectional study involved 40 children, aged between 6 months and 14 years, admitted with a diagnosis of CM at the Obafemi Awolowo University Teaching Hospitals Complex (OAUTHC), Ile-Ife, Nigeria. MATERIALS AND METHODS: One milliliter (mL) of venous blood and 1mL of CSF obtained from each subject at admission were used to determine the ß-endorphin levels using enzyme-linked immunosorbent assay (ELISA) method. STATISTICAL ANALYSIS: Bivariate linear regression was used to determine the association between plasma and CSF ß-endorphin levels using the correlation coefficient (r), coefficient of determination (R 2), and P values. RESULTS: The plasma ß-endorphin levels significantly positively correlated with CSF ß-endorphin (r = 0.568, P = 0.001) such that for every unit rise in plasma ß-endorphin, CSF ß-endorphin rose by 0.252 pmol/L (confidence interval: 0.132-0.371 pmol/L). CONCLUSION: The finding of positive correlation between plasma and CSF ß-endorphin levels in this study suggests a possible direct link between plasma and CSF in CM, probably from the disruption of the blood-brain barrier that has been reported in CM.

Foetal Haemoglobin and Disease Severity in Nigerian Children with Sickle Cell Anaemia.

BACKGROUND: Foetal haemoglobin (HbF) is a major modifying factor influencing sickle cell disease (SCD) severity. Despite this, HbF estimation is not routinely done in Nigeria. The relationship between HbF and SCD severity among affected children is also poorly studied. METHODS: In this descriptive cross-sectional study, we determined the relationship between steady state HbF levels and disease severity of Nigerian children aged 1 - 15 years with homozygous SCD. For each child, the socio-demographic characteristics and SCD clinical severity were determined. The latter was assessed based on the frequency of significant painful episodes, blood transfusion, and hospitalisation in the preceding 12 months; lifetime cumulative incidence of SCD-related complications; the degree of splenic and hepatic enlargement; current haematocrit and leucocyte count. Foetal haemoglobin levels were quantified with high-performance liquid chromatography. RESULTS: The mean HbF level of the 105 children with SCA was 9.9 ± 6.0%. Male had significantly lower mean HbF levels than females, 8.0 ± 5.6% vs. 12.2 ± 5.8% (p < 0.001). None of the children had severe disease. However, the 32 children with moderate disease had significantly lower mean foetal haemoglobin levels than the 73 with mild disease (7.7 ± 5.6% vs 10.8 ± 6.0% respectively). The mean HbF level was also significantly lower in children who had a history of acute chest syndrome and stroke compared to those without these complications, p = 0.002 and 0.010 respectively. CONCLUSION: Children with SCA who had a moderate disease and those with a history of life-threatening complications such as stroke and acute chest syndrome had significantly low HbF levels. Therefore, it is recommended that facilities for early quantification of foetal haemoglobin and HbF inducement were made available to reduce the morbidity and mortality among these children.

Cerebrospinal fluid and plasma ß-endorphin levels in children with cerebral malaria.

OBJECTIVES: Cerebral malaria (CM) is the most lethal form of malaria, yet its pathogenesis is not fully understood. Cytoadherence, sequestration, alterations in cytokine expression, inflammation, and microvascular obstruction are all hypothesized to be important in the aetio-pathogenesis of coma which characterizes cerebral malaria and the death which sometimes result. Beta (ß)-endorphin has been postulated to be involved in the pathogenetic processes of inflammation and cytokine expression, although the exact role is unknown. The aim of this study was to determine the levels of ß-endorphin in cerebrospinal fluid (CSF) and plasma of children with CM and compare the levels of ß-endorphin in the plasma of children with CM with that of apparently healthy age- and sex-matched controls at Ile-Ife, Nigeria. MATERIALS AND METHODS: Additional to the standard investigation for CM, CSF and venous blood samples were obtained from the subjects for the determination of ß-endorphin levels. RESULTS: Forty children with CM were studied along with forty age- and sex-matched controls. The mean CSF ß-endorphin (± SD) level for the children with CM was 1.8 ± 0.9 pmol/L. The mean plasma ß-endorphin levels at admission (3.1 ± 2.0 pmol/L) and discharge (4.1 ± 3.3 pmol/L) were higher in children with CM than in the control subjects (2.7 ± 0.7 pmol/L). However, only the mean plasma ß-endorphin levels at discharge was significantly higher than that of controls (p = .012). CONCLUSION: Children with CM had higher mean plasma ß-endorphin levels compared to the controls and there was increased production of ß-endorphins in children with CM during the course of the illness.

Early neonatal bilirubin, hematocrit, and glucose-6-phosphate dehydrogenase status.

OBJECTIVE: To document the patterns of bilirubin and hematocrit values among glucose-6-phosphate dehydrogenase (G6PD)-deficient and G6PD-normal Nigerian neonates in the first week of life, in the absence of exposure to known icterogenic agents. METHODS: The G6PD status of consecutive term and near-term neonates was determined, and their bilirubin levels and hematocrits were monitored during the first week of life. Infants were stratified into G6PD deficient, intermediate, and normal on the basis of the modified Beutler's fluorescent spot test. Means of total serum bilirubin (TSB) and hematocrits of the 3 groups of infants were compared. RESULTS: The 644 neonates studied comprised 353 (54.8%) boys and 291 (45.2%) girls and 540 (83.9%) term and 104 (16.1%) near-term infants. They consisted of 129 (20.0%) G6PD-deficient, 69 (10.7%) G6PD-intermediate, and 446 (69.3%) G6PD-normal neonates. The G6PD-deficient and G6PD-intermediate infants had higher mean TSB than their G6PD-normal counterparts at birth and throughout the first week of life (P < .001). Mean peak TSB levels were 14.1 (9.48), 10.2 (3.8), and 6.9 (3.3) mg/dL for G6PD-deficient, G6PD-intermediate, and G6PD-normal neonates, respectively. Peak TSB was attained on approximately day 4 in all 3 groups, and trends in TSB were similar. Mean hematocrits at birth were similar in the 3 G6PD groups. However, G6PD-deficient and -intermediate infants had higher declines in hematocrit, bilirubin levels, and need for phototherapy than G6PD-normal infants (P < .001). CONCLUSIONS: The G6PD-deficient and G6PD-intermediate neonates had a higher risk of neonatal hyperbilirubinemia and would therefore need greater monitoring in the first week of life, even without exposure to known icterogenic agents.

Attitude to organ donation among healthcare workers in Nigeria.

INTRODUCTION: As transplantation services are scaled up in Nigeria so will the need for organ donation. Crucial to the success of organ donation is the attitude of healthcare workers (HCW); this was determined in the present study. METHODS: HCW participating in three workshops were requested to complete a pretested questionnaire structured to elicit their attitude to organ donation. Predictors of willingness to donate were also determined. RESULTS: Of the 205 questionnaires distributed, 182 (88.8%) were returned; 10 were excluded for incomplete responses. The mean age of the respondents was 39.9 (8.9) yr. The majority were females (76.7%), Christians (87.8%), and worked in tertiary hospitals (77.3%). Medical doctors made up 55% of the respondents. Of the 172 respondents, 102 (59.3%) reported willingness to donate an organ. The majority of Muslims respondents willing to donate would prefer living donation. Being a medical doctor (odds ratio of 2.64 [1.17-5.94]) was the strongest predictor of willingness to donate an organ. The most common reasons for unwillingness to donate were "fear of complications" (44.9%) and "mistrust of the health sector" (20.6%). CONCLUSION: The majority of the HCW are favorable to organ donation. Being a medical doctor is highly predictive of willingness to donate an organ.

School performance of Nigerian adolescents with epilepsy.

PURPOSE: The study assessed the school performance of Nigerian adolescents with epilepsy compared with healthy controls and examined the variables correlating with their academic difficulties. METHODS: The school grades of adolescents with epilepsy aged 12 to 18 years (n = 73) over the past academic year were compared with the grades of their classmates of the same age and gender. Risk factors possibly associated with school performance, such as adolescent variables (age, gender, perceived stigma, attitude toward epilepsy, and psychopathology), seizure variables (age at onset of illness, years of illness, types of seizures, and frequency of seizures per month), drug variables [types of antiepileptic drugs (AEDs), number of AEDs and side effects of AEDs], and family variables (family's socioeconomic status, family functioning, caretakers' psychopathology, and caretakers' perceived stigma) were assessed. RESULTS: The mean school grades of adolescents with epilepsy are significantly lower than are those of their healthy controls (p < 0.001) in all the subjects. The variables that significantly predict poor school performance in adolescents with epilepsy include psychopathology in the caretaker (p < 0.001), adolescents' perceived poor family functioning (p = 0.002), adolescents' attitude toward the illness (p = 0.001), adolescents' felt stigma (p = 0.002), externalizing symptoms in the adolescents (p = 0.004), and duration of illness (p = 0.024). CONCLUSIONS: The determinants of poor school performance in adolescents with epilepsy in Nigeria are multivariate, with psychosocial factors most important. These should be noted for early identification and screening of those children at greatest risk for academic failure and the greatest need for appropriate educational remediation services.

Impact of psychiatric morbidity on parent-rated quality of life in Nigerian adolescents with epilepsy.

Despite the prevalence of anxiety and depressive disorders in children and adolescents with epilepsy, their impact on the quality of life has not been sufficiently studied. Adolescents with epilepsy (n=90) aged 12 to 18 were assessed for anxiety and depressive disorders with the Diagnostic Interview Schedule for Children, Version IV (DISC-IV), and their quality of life was assessed with the parent-rated Impact of Childhood Illness Scale (ICIS). Sociodemographic and illness variables were also obtained. Predictors of poor quality of life in adolescents with epilepsy include anxiety disorders, depressive disorders, frequency of seizures, and side effects of antiepileptic drugs. Depressive and anxiety disorders impacted on both the adolescents and the family. Programs designed to improve the overall quality of life of these adolescents should include the evaluation and treatment of possible comorbid anxiety and depressive disorders and involve the family.