Health education to promote knowledge about sickle cell disease and newborn screening in pregnant women: a community-based pilot study using the healthy beginning initiative.

BACKGROUND: Pregnancy presents a critical period for any maternal and child health intervention that may impact the health of the newborn. With low antenatal care attendance by pregnant women in health facilities in Nigeria, community-based programs could enable increased reach for health education about sickle cell disease (SCD) and newborn screening (NBS) among pregnant women. This pilot study aimed to assess the effect of education on the knowledge about SCD and NBS among pregnant women using the Healthy Beginning Initiative, a community-based framework. METHODS: A pre-post study design was used to evaluate knowledge of SCD and NBS in a convenience sample of 89 consenting pregnant women from three communities. Participants were given surveys prior to and following completion of a health education session. McNemar's test was used to compare the proportion of participants with correct responses. The level of significance was taken as p < 0.05. RESULTS: Compared to pre-test values, post-test values showed that participants understood that SCD is hereditary (93.3% vs. 69.7%), both parents must have at least one gene for someone to have SCD (98.9% vs. 77.5) and blood test is the right way to know if one has SCD (98.8% vs. 78.7%). Also, a large proportion of participants (post-test ~ 89.9%; compared to pre-test ~ 23.6%) understood that the chance of conceiving a child with SCD was 25% for a couple with the sickle cell trait (SCT). Knowledge of the possibility of diagnosing SCD shortly after birth was highly increased in the post test phase of the study when compared to the pre-test phase (93.3% vs. 43.9%, respectively). Concerning the overall knowledge scores, those with high level of knowledge significantly increase from 12.6% pretest to 87.4% posttest (p = 0.015). CONCLUSION: The health education intervention was associated with significant improvement on almost all measures of SCD knowledge. Focused health education for pregnant women using community structures can improve knowledge of SCD and NBS.

Effect of dolutegravir-based drug combinations on the level of medication adherence and viral load among adolescents living with HIV in resource-limited setting: a pre-post design.

OBJECTIVES: The use of antiretroviral drugs among HIV-infected adolescents has been challenged with poor medication adherence, leading to an unsuppressed viral load and ultimately to drug resistance. Recently, dolutegravir has been approved for use in adolescents with HIV, but the evidence on medication adherence and viral load suppression is limited. The study determined the effect of dolutegravir-based drug regimens on the level of medication adherence and viral load among treatment-experienced adolescents. METHODS: A longitudinal pre-post design study was done among adolescents living with HIV at a paediatrics clinic in Nigeria. Assessment of medication adherence and viral load testing was done before and at six months of transitioning to dolutegravir-based regimens. McNemar-chi-square was used to determine the effectiveness of the drug on adherence and viral load suppression. Multivariate logistic regression analysis was performed to determine the predictors of drug adherence and viral suppression. RESULTS: The mean age of the participants was 14.7 years (SD=3.2), and 53.3 % were males. The mean duration of ART use was 10.3 years (SD=3.2). Six months after transitioning to dolutegravir, there was a significant decrease in viral load (Z=-7.947, p<0.001) and an increase in medication adherence score (Z=-7.554, p<0.001), among the treatment-experienced adolescents. Viral suppression was 13 times higher among respondents with good medication adherence as compared to those with poor medication adherence (AOR=13.24, CI=3.25-53.90). CONCLUSIONS: Dolutegravir is effective in improving medication adherence and viral suppression among Adolescents living with HIV. Thus, the need to transition eligible adolescents to dolutegravir to sustain better treatment outcomes.

Effect of nutrition education on dietary diversity among HIV Patients in Southeast, Nigeria.

Background: Integrating nutrition interventions which include nutrition education in HIV/AIDS care program may help people living with HIV/AIDS (PLWHA) make better decisions regarding their nutrition to improve their immune system. Objective: To determine the effect of nutrition education on dietary diversity among HIV/AIDS patients in Southeast, Nigeria. Methods: A quasi-experimental study was conducted among 370 HIV patients divided into two groups of 185 each for the study and control groups. The nutrition education program was delivered to the study group. In both groups, their practice of dietary diversity was ascertained pre- and post-intervention. The Chi-square test and McNemar were used in the analysis. Results: Practice of dietary diversity was low among 79(42.7) and 69(37.3) respondents in the study and control groups respectively (x2 =1.126, p=0.289). Three months after the intervention, the proportion of respondents with low dietary diversity significantly decreased from 42.7% to 22.7% in the study group while the control group had no appreciable reduction (x2=7.532, p=0.006). Conclusion: Nutrition education plays a positive role in the dietary diversity of PLWHA. This suggests that nutrition education should be a key component in the care of PLWHA for a better nutritional outcome.

Cerebral artery conditional blood velocity in sickle cell disease: a multicentre study and evidence for active treatment.

OBJECTIVE: To obtain multicentre data on the prevalence of normal, high or conditional (intermediate) blood velocity in the cerebral arteries among children with sickle cell disease (SCD) in Nigeria. DESIGN: A prospective observational study in five tertiary healthcare institutions. By transcranial Doppler (TCD) ultrasonography, cerebral artery peak systolic blood velocity (PSV) was determined in 193 children with SCD and time averaged mean of the maximum blood velocity (TAMMV) in a different cohort of 115 children. This design was to make the findings relevant to hospitals with TCD equipment that measure either PSV or TAMMV. SETTING: Nigeria. PARTICIPANTS: 308 children (126 girls, 182 boys; age 2-16 years). MAIN OUTCOME MEASURES: Percentage of children with SCD who have normal, high or intermediate (often termed conditional) PSV or TAMMV. RESULTS: In the cohort of 193 children, PSV was normal in 150 (77.7%), high in 7 (3.6%) and conditional in 36 (18.7%). In the cohort of 115 children, TAMMV was normal in 96 (84%), high in 7 (6%) and conditional in 12 (10%). There were no significant differences in gender or age distribution between the PSV and TAMMV cohorts. Altogether, cerebral artery blood velocity was normal in 246/308 children (80%), high in 14 (4.5%) and conditional in 48 (15.5%). CONCLUSION: Since conditional blood velocity in cerebral arteries can progress to high values and predispose to stroke, the proportion of children with SCD who are affected (15.5%) raises the question of whether regular monitoring and proactive intervention ought to be the standard of care.

Assessment of depression in children and adolescents with sickle cell anemia in a low-resource setting: a comparative study.

Children with sickle cell anemia (SCA) usually face psychological complications especially depression. Assessment of depression in resource-limited settings may help identify the extent to which the children with SCA in such settings may need its introduction as part of routine care. This study aimed to assess depression in children and adolescents with SCA in a low-resource setting. This cross-sectional observational study involved 84 children and adolescents with SCA aged 7-17 years who were selected using a systematic random sampling technique. Their controls were 84 age- and sex-matched individuals with AA hemoglobin genotype. A structured questionnaire was used to collect socio-demographic data while depression was assessed with the Children's Depression Inventory. The prevalence of depression was non-significantly higher in subjects compared to the controls (8.3% vs. 2.4%) (Fisher's χ2 = 1.88, p = 0.171). Though not statistically significant, the subjects had 3.7 times higher odds of having depression compared to the controls (OR = 3.7; 95% CI 0.75-18.50; p = 0.107). Of the 5 depression subscales, the subjects had a significantly higher difference in the negative mood (p = 0.042). Despite the comparable prevalence of depression with their normal controls, children and adolescents with SCA had a higher negative mood and higher odds of having depression than normal individuals. Thus, there is a need for the introduction of depression assessment as a complement to routine care of these children with SCA in resource-poor settings.

Effect of nutrition education on nutritional status and serum zinc level of HIV and AIDS clients: implication for care in resource-limited settings.

Background: People living with HIV or AIDS in resource-limited settings are faced with challenges in their nutrition and micronutrient levels. This study aimed to determine the effect of nutrition education on the nutrition status and serum zinc level of HIV and AIDS clients, and its implication for HIV care in resource-limited settings.Methods: This intervention study was conducted among HIV clients at the University of Nigeria Teaching Hospital and Enugu State University Teaching Hospital, which served as the intervention and control groups respectively. A nutrition education programme was delivered to the intervention group. In both groups, before and after the intervention, the body mass index of participants was calculated using their height (m2) and weight (kg) while their serum zinc levels were analysed using spectrophotometry. Chi-square and McNemar chi-square tests were used in the analysis.Results: A total of 185 respondents was studied in each group, with the majority in the 30-39 and 40-49 age groups, respectively (65.4% of the study group and 64.4% of the control group). Larger proportions of respondents in both groups were females (72.4% vs 75.1% respectively). Three months after the intervention, the proportion of respondents with normal serum zinc levels improved significantly from 20.5% to 51.9% in the intervention group (χ2McNemar, p < 0.001), whereas the control group had no appreciable improvement (16.8% vs 22.7%) (χ2McNemar, p < 0.117). Furthermore, the post-intervention serum zinc level difference between the intervention and control groups was statistically significant (χ2 = 33.699; p < 0.001). No statistically significant difference existed in the nutritional status between the groups (χ2 = 3.469; p = 0.325).Conclusion: Nutrition education had a positive effect on the serum zinc levels of HIV clients. Integrating nutrition education programmes as a key component of HIV care in resource-limited settings will help improve the serum zinc level of people living with HIV, which ultimately improves their immune status and life expectancy.

Clinical depression in children and adolescents with sickle cell anaemia: influencing factors in a resource-limited setting.

BACKGROUND: Sickle cell anaemia (SCA) is the commonest monogenic haematologic disorder resulting from the inheritance of homozygous mutant haemoglobin genes from both parents. Some factors have been identified as important in explaining the variability in depression in sickle cell anaemia (SCA). Information on this is limited in a resource-limited setting like Nigeria. This study aims to determine factors which influence depression in children and adolescents with sickle cell anaemia in a resource-limited setting. METHODS: Systematic random sampling technique was used in this cross-sectional study to select children and adolescents aged 7-17 years at the weekly sickle cell clinic of the University of Nigeria Teaching hospital (UNTH) Enugu, Nigeria. Pretested, structured questionnaire was used to collect sociodemographic and disease severity data while depression was assessed using the Children's Depression Inventory. RESULTS: Age and educational level had significant positive linear relationships with depression (r = 0.253, p = 0.02; r = 0.225, p = 0.04 respectively) while gender (χ2 = 0.531, p = 0.466), socioeconomic status (χ2 = 0.451, p = 0.798) and disease severity (χ2 = 0.422, p = 0.810) had no relationship with depression in children and adolescents with SCA. CONCLUSION: Depression in children and adolescents with SCA increased with increasing age and educational level. Psychological evaluation should be integrated into routine assessment of children with SCA during their follow up visits as they get older and progress in class.

Relationship between disease severity and folate status of children with sickle cell anaemia in Enugu, South East Nigeria.

BACKGROUND: Repeated crises in children with sickle cell anaemia (SCA), which is a manifestation of disease severity, results in depletion of their minimal tissue folate stores, with higher likelihood of folate deficiency. The study aimed to determine the relationship between disease severity and the folate status of children with SCA attending University of Nigeria Teaching Hospital (UNTH), Enugu. METHODS: This was a hospital based, cross-sectional study conducted between September 2018 and March 2019. One hundred participants were recruited, consisting of 50 children having sickle cell crisis and 50 age and gender matched haemoglobin AA genotype controls. Relevant information was documented using a pretested questionnaire. Sickle cell severity score was determined using frequency of crisis, admissions and transfusions in the preceding one year, degree of liver and splenic enlargement, life-time cummulative frequency of specific complications of SCA, leucocyte count and haematocrit. RESULTS: Folate deficiency was observed in eight percent of the subjects and none of the controls. The difference was not significant (Fisher's exact = 4.167, p=0.117). The odds of being folate deficient was 8.5 times more likely during anaemic crisis than in vaso-occlusive crisis, though not significant (95% C.I 0.05 - 89.750, p = 0.075). The mean SCA severity score was 8.06 ± 3.64, signifying a moderate SCA severity in the study population. There was a no relationship between folate status and severity of SCA (Fisher's exact = 0.054, p = 0.949). CONCLUSION: Folate status in children with SCA is not affected by their disease severity. Therefore, there may be no need for additional folate supplementation with increasing severity of sickle cell anaemia.

Community based screening for sickle haemoglobin among pregnant women in Benue State, Nigeria: I-Care-to-Know, a Healthy Beginning Initiative.

BACKGROUND: Haemoglobin genotype screening at prenatal care offers women an opportunity to be aware of their genotype, receive education on sickle cell disease (SCD) and may increase maternal demand for SCD newborn screening. In developed countries, most pregnant women who access prenatal care and deliver at the hospital receive haemoglobin genotype screening. In settings with low prenatal care attendance and low hospital deliveries, community-based screening may provide similar opportunity for pregnant women. We assessed the feasibility and acceptability of integrating haemoglobin genotype screening into an existing community-based HIV program. METHODS: Onsite community-based integrated testing for HIV, hepatitis B virus and haemoglobin electrophoresis, were conducted for pregnant women and their male partners. Community Health Advisors implementing the NIH and PEPFAR-supported Healthy Beginning Initiative (HBI) program provided education on SCD, collected blood sample for haemoglobin electrophoresis and provided test results to participants enrolled into the HBI program. We concurrently conducted a cross-sectional study using a pretested, semi-structured, interviewer administered questionnaire to collect demographic data and assess awareness of individual haemoglobin "genotype" among HBI pregnant women participants. RESULTS: In this study, 99.9% (10,167/10,168) of pregnant women who received education on SCD accepted and completed the survey, had blood drawn for haemoglobin electrophoresis and received their results. A majority of participating pregnant women (97.0%) were not aware of their haemoglobin "genotype". Among the participants who were incorrect about their haemoglobin "genotype", 41.1% (23/56) of women who reported their haemoglobin "genotype" as AA were actually AS. The odds of haemoglobin "genotype" awareness was higher among participants who were in younger age group, completed tertiary education, had less number of pregnancies, and attended antenatal care. Overall prevalence of sickle cell trait (AS) was 18.7%. CONCLUSIONS: It is feasible to integrate haemoglobin "genotype" testing into an existing community-based maternal-child program. Most pregnant women who were unaware of their haemoglobin "genotype" accepted and had haemoglobin genotype testing, and received their test results. Increasing parental awareness of their own haemoglobin "genotype" could increase their likelihood of accepting newborn screening for SCD.

Assessment of breastfeeding techniques in Enugu, South-East Nigeria.

Background: Good breastfeeding technique is important in ensuring adequate milk delivery and preventing breastfeeding problems. Exclusive breastfeeding rate is quite low, and requisite skills regarding proper positioning and attachment of an infant while breastfeeding appears lacking among mothers in Nigeria. This study was undertaken to assess breastfeeding techniques of mothers attending the well-child clinics of two tertiary hospitals in southeast Nigeria. Materials and Methods: This cross-sectional descriptive study of 396 mother and child pairs who attend the well child clinics of two tertiary hospitals in Enugu (Enugu state University Teaching Hospital and University of Nigeria Teaching Hospital) between September 2018 and February 2019. An interviewer administered, well-structured pro forma was used to collect data while mothers were observed closely as they breastfed and scored using the World Health Organization criteria. Data were analyzed using SPSS version 22. Results: Most of the mothers studied (357; 90.2%) attended ante-natal care, and 365 (92.2%) of the deliveries were assisted by a health worker. Only 194 (49%) of mothers practiced good breastfeeding techniques. Maternal age (20-30 years) (P < 0.001, odds ratio [OR] 0.464), attendance to antenatal clinic (P < 0.001; OR 8.336), health education and demonstration on breastfeeding techniques before and after delivery (P = 0.001) and maternal level of education (χ2 = 13.173, P = 0.001) but not parity (P = 0.386; OR 1.192) were significantly associated with good breastfeeding techniques. Conclusion: There are suboptimal breastfeeding techniques among mothers. Increased awareness creation and regular demonstration of breastfeeding techniques are needed.

RésuméContexte: Une bonne technique d'allaitement est importante pour assurer une livraison adéquate du lait et prévenir les problèmes d'allaitement. Exclusive le taux d'allaitement est assez faible, et les compétences requises en ce qui concerne le positionnement et l'attachement appropriés d'un nourrisson pendant l'allaitement semblent manqué chez les mères au Nigéria. Cette étude a été entreprise pour évaluer les techniques d'allaitement des mères qui fréquentent les cliniques hôpitaux tertiaires dans le sud-est du Nigeria. Matériaux et méthodes: Cette étude descriptive transversale de 396 couples de mères et d'enfants assister aux cliniques pour enfants de deux hôpitaux tertiaires à Enugu (Hôpital universitaire d'Enseignement de l'Université d'Enugu et Université du Nigeria Enseignement hôpital) entre septembre 2018 et février 2019. Un intervieweur administré, bien structuré pro forma a été utilisé pour recueillir des données les mères ont été observées de près au fur et à mesure qu'elles allaitaient et scorelaient selon les critères de l'Organisation mondiale de la santé. Les données ont été analysées à l'aide du SPSS version 22. Résultats: La plupart des mères étudiées (357; 90,2 %) soins prénatals, et 365 (92,2 %) des livraisons ont été assistées par un travailleur de la santé. Seulement 194 (49%) des mères pratiquaient de bonnes techniques d'allaitement. Âge maternel (20­30 ans) (P 0,001, rapport de cotes [OR] 0.464), présence à la clinique prénatale (P 0,001; OU 8.336), éducation à la santé et démonstration sur les techniques d'allaitement avant et après (P = 0,001) et le niveau d'éducation maternel (2 = 13,173, P = 0,001) mais pas la parité (P = 0,386; OR 1.192) ont été significativement associés avec de bonnes techniques d'allaitement. Conclusion: Il existe des techniques d'allaitement sous-optimales chez les mères. Création accrue de sensibilisation et une démonstration régulière des techniques d'allaitement sont nécessaires.

Pilot assessment of omega-3 fatty acids and potassium thiocyanate in sickle cell anemia patients with conditional peak systolic cerebral artery blood velocity.

OBJECTIVE: The purpose of this pilot study was to explore the effect of omega-3 fatty acids and potassium thiocyanate on conditional peak systolic cerebral artery blood velocity in children with sickle cell anemia (SCA). METHODS: Transcranial doppler ultrasonography (TCD) was done on 232 SCA children, and 21 found with conditional peak systolic blood velocity (PSV) of 200-249 cm/s in internal carotid, middle or anterior cerebral arteries. These were randomized to receive omega-3 fatty acids and potassium thiocyanate with standard treatment of SCA (test group, N = 14), or standard treatment only (control group, N = 7). After 3 months of treatment, PSV was measured again. RESULTS: Right middle cerebral artery PSV was significantly reduced in the test relative to the control groups (p = 0.04). PSV returned to normal in 79% of the test versus 43% of the control group; and increased to abnormal in one member of the control group, but none of the test group. CONCLUSIONS: The pilot data suggest that in SCA, omega-3 fatty acids and potassium thiocyanate might reduce conditional blood velocity to normal, or prevent progression to abnormal values. A larger, randomized, clinical trial is required to further address the current gap in management of conditional TCD blood velocity.

Glanzmann's thrombasthenia: a rare bleeding disorder in a Nigerian girl.

INTRODUCTION: Glanzmann's Thrombasthenia (GT) is a rare autosomal recessive bleeding disorder due to defective platelet membrane glycoprotein GP IIb/IIIa (integrin αIIbß3). The prevalence is estimated at 1:1,000,000 and it is commonly seen in areas where consanguinity is high. CASE PRESENTATION: The authors report a 12 year old Nigerian girl of Igbo ethnic group, born of non-consanguineous parents, who presented with prolonged heavy menstrual bleeding which started at menarche 3 months earlier, weakness and dizziness. She had a past history of recurrent episodes of prolonged epistaxis, gastrointestinal bleeding and gum bleeding during early childhood. On examination, she was severely pale with a haemic murmur and vaginal bleeding. The initial diagnosis was menorrhagia secondary to bleeding diathesis possibly von Willebrand's Disease. She was on supportive treatment with fresh whole blood, fresh frozen plasma and platelets until diagnosis of GT was made in the USA. Currently, she is on 3 monthly intramuscular Depo-provera with remarkable improvement. CONCLUSION: To the best of our knowledge, this is the first documented report of GT in our environment where consanguinity is rarely practised. Our health facilities require adequate diagnostic and treatment facilities for rare diseases like GT.

Persistent hematuria among children with sickle cell anemia in steady state

ABSTRACT Introduction: Persistent hematuria is a chronic complication of sickle cell anemia (SCA) which can progress to chronic kidney disease. The practice of early detection of persistent hematuria in children with SCA in steady state is important for timely intervention. Objective: To determine the prevalence of persistent hematuria among children with sickle cell anemia in steady state and compare the result with that of a group of HbAA controls. The outcome will possibly strengthen the health policy on the need for regular screening for persistent hematuria in children with SCA. Methods: Children with sickle cell anemia, aged 2-18 years in steady state, were recruited consecutively from the sickle cell clinic at the University of Nigeria teaching Hospital Enugu. The controls were similarly recruited from the children's outpatient clinic. To determine persistent hematuria, dipstick urinalysis and microscopy were performed for both subjects and controls at enrollment and repeated after four weeks. Results: Out of the 122 children with SCA studied, 5 (4.1%) had persistent hematuria. None (0%) of the 122 age- and gender-matched HbAA controls had persistent hematuria. This difference in prevalence of persistence between HbSS patients and HbAA controls was statistically significant (p= 0.02). Conclusion: Persistent hematuria still occurs significantly more among children with SCA, even among those in steady state. Routine urinalysis at follow-up visits in children with SCA is strongly recommended, as this will aid early detection and prompt management to prevent progression to chronic kidney disease.

Knowledge and awareness of individual sickle cell genotype among adolescents in a unity school in Southeast, Nigeria: a cross-sectional study.

OBJECTIVE: Our study sought to assesses the knowledge and awareness of individual sickle cell genotype among adolescents. METHODS: Participants were recruited from a large school in Southeast Nigeria where adult prevalence of sickle cell trait is 25%. Data was collected through a 50-item survey with previously pretested questions that assessed awareness of individual genotype, general knowledge of sickle cell disease, perception of sickle cell trait and sickle cell anaemia (SCA). Additionally, self-reported genotype was compared to the school's admission records to determine accuracy of self-reported genotype. The knowledge scores were summed on a binary basis with one point assigned for a correct answer while zero was given for an incorrect response. RESULTS: Four hundred and nine (409) students were approached and enrolled in the study. A vast majority (94%) of the respondents reported being aware of their genotype and two-thirds had the awareness during school admission. However, in specific knowledge of sickle cell, majority (89.7%) of the participants miscalculated the probability of having a child with SCA in married carrier couple and 71.9% misidentified the proportion of Nigerians with sickle cell trait. Assessing level of knowledge of sickle cell, only very few of the adolescents (7.3%) were found with the expected high knowledge scores of 7-8. CONCLUSION: Although a significant proportion of respondents were aware of their genotype, most were unaware of the implications of sickle cell trait and thought that people with sickle cell trait also have symptoms of sickle cell disease. Also, only a few of the respondents have the expected level of sickle cell knowledge. A focused educational intervention among this age group is crucial as they embark on making reproductive health decisions.

Pre-Transition Readiness in Adolescents and Young Adults with Four Chronic Medical Conditions in South East Nigeria - An African Perspective to Adolescent Transition.

INTRODUCTION: When a child reaches a certain age, he or she moves over to the adult physician. For this to maximally benefit the child, there has to be a process of equipping the child with skills required for taking on more responsibilities. Transitioning involves a process in which the adolescent with chronic illness is prepared ahead of time to enable them to eventually transfer to adult care with good outcomes. In high-income countries with well-organized health financing, the transitioning process begins as early as 12 years. In Africa, this process is not as organized and most hospitals would write a referral letter once the child turns 18 and transfer to adult clinic. In four of our chronic disease clinics (asthma, HIV, sickle cell anaemia and chronic kidney diseases) patients up to 24 years old are still attending the paediatric clinics. Understanding transition readiness among African adolescents remains a gap. Our findings will form a basis for informed practices for adolescent clinics in African countries. METHODS: This was a descriptive cross-sectional study of pre-transition readiness in adolescents and young adults with chronic illnesses attending four outpatient specialist clinics in a tertiary hospital in Enugu Nigeria. This was done using the validated STARx Questionnaire. Total scores were computed and scores nearer the upper limit of 90 were acceptable, while mean subdomain scores of 4 and above were considered as optimal level of transition readiness. Demographic and clinical data were also collected. Acceptability to move on to adult-oriented care was documented using binary response (yes/no). Cross tabulations were done, and likelihood ratios obtained for predictors of acceptability of transition. Significant value was set at p-value of ≤0.05. RESULTS: A total of 142 adolescents and young adults aged 12 to 24 years were studied. There were 38.0% (54), 24.6% (35), 22.5% (32) and 14.8% (21) from HIV, sickle cell anaemia, asthma and nephrology clinics, respectively. Their mean age was 15.6 years ± 2.4, and 48.6% (69) were male. The mean total transition readiness score was 56±14 and this was not nearer the higher spectrum of total scores obtainable. Highest mean scores (3.7) occurred in the knowledge subdomain while least mean score (2) was noted in the use of medication reminders. The males had highest scores in the knowledge subdomain while the females were better informed about medication adherence and were more inquisitive about their chronic illness. Only about 37% (53) of the adolescents and young adults welcomed the idea of moving on to adult-care clinics. Children who had less frequent emergency hospital visits and better treatment outcome accepted the idea of transfer to adult care. Irrespective of the age all participants had suboptimal subdomain scores. High scores did not influence the participants' choice to embrace transfer to adult care. CONCLUSION: There is suboptimal transition readiness irrespective of the age. The older age groups were less willing to transfer to adult care. Better disease knowledge and better communication skills did not positively influence acceptability of transfer to adult care.

Persistent hematuria among children with sickle cell anemia in steady state.

INTRODUCTION: Persistent hematuria is a chronic complication of sickle cell anemia (SCA) which can progress to chronic kidney disease. The practice of early detection of persistent hematuria in children with SCA in steady state is important for timely intervention. OBJECTIVE: To determine the prevalence of persistent hematuria among children with sickle cell anemia in steady state and compare the result with that of a group of HbAA controls. The outcome will possibly strengthen the health policy on the need for regular screening for persistent hematuria in children with SCA. METHODS: Children with sickle cell anemia, aged 2-18 years in steady state, were recruited consecutively from the sickle cell clinic at the University of Nigeria teaching Hospital Enugu. The controls were similarly recruited from the children's outpatient clinic. To determine persistent hematuria, dipstick urinalysis and microscopy were performed for both subjects and controls at enrollment and repeated after four weeks. RESULTS: Out of the 122 children with SCA studied, 5 (4.1%) had persistent hematuria. None (0%) of the 122 age- and gender-matched HbAA controls had persistent hematuria. This difference in prevalence of persistence between HbSS patients and HbAA controls was statistically significant (p = 0.02). CONCLUSION: Persistent hematuria still occurs significantly more among children with SCA, even among those in steady state. Routine urinalysis at follow-up visits in children with SCA is strongly recommended, as this will aid early detection and prompt management to prevent progression to chronic kidney disease.

Hypertension in Children: Could the Prevalence be on the Increase?

BACKGROUND: In children particularly in the developing world, there is a tendency to downplay the role of primary hypertension in their health. In adults, a number of factors have clearly been associated with the incidence of hypertension. Knowledge of the prevalence of hypertension and its associated factors among children in our environment is important and could inform the need for lifestyle changes and routine blood pressure (BP) checks in children so as to reduce BP-related health risks. AIM: The aim of this study is to document the prevalence of hypertension and its risk factors among children in Enugu, Nigeria. MATERIALS AND METHODS: Children aged 6-17 years attending the outpatient clinic of a tertiary hospital, were enrolled for the study. Their socioeconomic status (SES), weight, height, BP, and dipstick urinalysis were measured using standardized methods. Adherence to Mediterranean diet was assessed using the Mediterranean Diet Quality Index (KIDMED). The prevalence of hypertension and the influence of these factors on their BP were analyzed. RESULTS: Forty-six (9%) of the 491 participants had hypertension. Of these 46 hypertensive children, 72% were females while a significantly higher proportion 57% (P = 0.006), were in the age group 13-17 years. While age, gender, and the presence of protein in urine were significantly associated with hypertension in these children; body mass index, diet, family history of hypertension, and SES were not. CONCLUSION: The prevalence of hypertension in children in this environment is high and appears to be increasing. There is need for routine BP and urinalysis check for all children in our clinics and wards.

Cancer Mortality in the Niger Delta Region of Nigeria: A Case Study of the University of Port Harcourt Teaching Hospital.

AIM: The aim of this study is to determine the pattern of cancer mortality (CM) seen in the University of Port Harcourt Teaching Hospital (UPTH) which is a cancer reference center in the Niger Delta Region. METHODOLOGY: This is a 6-year retrospective study of cancer-related deaths in UPTH using patients' admission registers in all the wards and emergency units. Furthermore, the death certificates of cases were reviewed. RESULTS: Three hundred and sixteen cases of cancer-related deaths occurred, involving 174 females and 142 males, in a female-to-male sex ratio of 1.2:1. All age groups were affected, with age group 40-49 years accounting for the majority (20.6%). CM was seen in all the systems, except the central nervous system. Cancers of the gastrointestinal tract and its accessory organs (liver and gall bladder) caused most mortality (27.9%), in a female-to-male ratio of 0.8:1. The single most involved organ in CM is the female breast (20.6%), distantly followed by mortality due to prostate cancers and hematolymphoid cancers which accounted for 9.2% each. Colorectal cancers accounted for 7.3% of cancer deaths and ranked 4th. Cancers of both cervix and stomach each accounted for 5.7% of mortality. The major histologic diagnoses were carcinomas (adenocarcinoma; 36.7%, invasive ductal carcinoma; 20.3%, squamous cell carcinomas; 8.2% and hepatocellular carcinomas; 4.4%). Leukemias and lymphomas accounted for 9.2% of cases, whereas sarcomas accounted for 5.1% of cases. CONCLUSION: Infection-related and noninfection-related cancers cause most mortality in UPTH. The 5th decade was the most commonly affected, while female breast was the single most involved organ. Breast, prostate and hematolymphoid malignancies are common causes of CM with death from breast occurring earliest. Majority of the deceased were educated, working-class urban dwellers. More advocacies on public acceptance of cancer screening and cancer preventive lifestyles as well as governments' improvement on workforce training and treatment infrastructure will improve the current CM profile in Port Harcourt.

HemoTypeSC, a low-cost point-of-care testing device for sickle cell disease: Promises and challenges.

BACKGROUND: Sickle cell disease (SCD) is a neglected burden of growing importance. >312,000 births are affected annually by sickle cell anaemia (SCA). Early interventions such as newborn screening, penicillin prophylaxis and hydroxyurea can substantially reduce the mortality and morbidity associated with SCD. Nevertheless, their implementation in African countries has been mostly limited to pilot projects. Recent development of low-cost point-of-care testing (POCT) devices for sickle haemoglobin (HbS) could greatly facilitate the diagnosis of those affected. METHODS: We conducted the first multi-centre, real-world assessment of a low-cost POCT device, HemoTypeSC, in a low-income country. Between September and November 2017, we screened 1121 babies using both HemoTypeSC and HPLC and confirmed discordant samples by molecular diagnosis. FINDINGS: We found that, in optimal field conditions, the sensitivity and specificity of the test for SCA were 93.4% and 99.9%, respectively. All 14 carriers of haemoglobin C were successfully identified. Our study reveals an overall accuracy of 99.1%, but also highlights the importance of rigorous data collection, staff training and accurate confirmatory testing. It suggests that HPLC results might not be as reliable in a resource-poor setting as usually considered. INTERPRETATION: The use of such a POCT device can be scaled up and routinely used across multiple healthcare centres in sub-Saharan Africa, which would offer great potential for the identification and management of vast numbers of individuals affected by SCD who are currently undiagnosed. FUNDING US: Imperial College London's Wellcome Trust Centre for Global Health Research (grant #WMNP P43370).

Generalized lymphadenopathy: an unusual presentation of burkitt lymphoma in a Nigerian child: a case report.

INTRODUCTION: Burkitt Lymphoma is the fastest growing tumor in human and the commonest of the childhood malignancies. Generalized lymphadenopathy is a common feature of immunodeficiency associated Burkitt lymphoma but an uncommon presentation of the endemic type in Human Immunodeficiency Virus (HIV) negative children. CASE PRESENTATION: The authors report a 6 year old HIV negative boy who presented with generalized lymphadenopathy, cough, weight loss, fever and drenching night sweat and had received native medication as well as treatment in private hospitals. His examination revealed hepatosplenomegaly, bull neck with generalized significant massive lymphadenopathy. Diagnosis was missed initially until a lymphnode biopsy for histology confirmed Burkitt lymphoma. He was managed on combination chemotherapy with complete resolution and now on follow up. CONCLUSION: To the best of our knowledge, this is the first documented report of its kind of endemic Burkitt lymphoma involving lymphnodes generally as the primary site. High index of suspicion and early biopsy are the key in this uncommon presentation.