In-hospital psychoeducation for family caregivers of Nigerian children with cancer (The RESCUE Study).

OBJECTIVES: High levels of caregiver burden (CB) are experienced by informal caregivers of pediatric patients with cancer. There is increasing evidence highlighting the extent of CB across sub-Saharan African countries, although there remains lack of interventions that target improvements in their experience. This study aimed to determine the impact of a structured psychoeducation program on caregivers' outcomes relating to preparedness to provide care, burden of caregiving, and quality of life (QoL). METHODS: This quasi-experimental (pre-and-posttest) design, involved family caregivers of children on admission for cancer treatment in 4 Nigerian tertiary hospitals. Eligible participants received 2 structured, psychoeducational training sessions delivered by a multidisciplinary oncology team, focusing on the management of patients' condition, spiritual care, self-care, and support. RESULTS: Subjects were mainly female (79.5%) and mostly mothers to children undergoing cancer treatment (74.7%). Commonest cancer type was acute lymphoblastic leukemia (23.9%) with evidence of metastatic disease found in 9.6% of children. Significant improvements were observed between pre- and posttest for unmet needs (z = -9.3; p < 0.001), preparedness for caregiving in palliative care (PCPC) (z = -7.0; p < 0.001), and overall QoL (z = -7.3; p < 0.001). A significant reduction in CB was also reported (z = -8.7; p < 0.001). SIGNIFICANCE OF RESULTS: This psychoeducational intervention (PEI) resulted in significant improvements in unmet needs, CB and significant improvements in PCPC. However, a reduction in QoL of the family caregivers was also observed. Findings from this study should encourage the use of well-crafted PEIs, delivered within hospital settings to promote improvements in outcomes for informal caregivers of hospitalized children suffering from cancer, in an African context. Further intervention development is required to better understand intervention components influencing changes in outcomes, while exploring feasibility testing and adaptation to similar settings in Nigeria and within Africa.

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.

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.

Impact of zinc on sexual maturation of female sickle cell anemia (SCA) children in Enugu, Southeast Nigeria.

Adolescence is an important developmental period of childhood. Good health and adequate nutrition consisting major food constituents and trace elements like zinc are fundamental for optimal sexual maturation. To determine the relationship between zinc levels and pattern of breast and pubic hair development, as well as menarcheal age of female SCA children aged 6-18 years and their matched controls with hemoglobin genotype AA. Cross sectional, case-control study. Information on biodata, age at menarche, medical and drug history as well as 24-hour dietary recall was documented using interviewer administered questionnaire. Sexual maturation was assessed using Tanner staging and zinc levels determined using Atomic absorption spectrophotometer. Eighty-one subjects were compared with 81 controls. There was significant delay in the mean age of attainment of various Tanner stages of breast and pubic hair in the subjects. Mean age of 14.81 ± 1.07 years at menarche in subjects was significantly higher than 12.62 ± 1.18 years in controls (p = 0.001). Serum zinc of 58.01 ± 10.58 µg/dl in subjects was significantly lower than 68.37 ± 8.67 µg/dl in controls (p = 0.001). Serum zinc levels were found to have a significant positive relationship with stages of sexual maturation and mean age at menarche. Reduced serum zinc in children with SCA was associated with delayed sexual maturation.

Neurocognitive Function of School-aged HIV-infected Children in Enugu, Nigeria.

BACKGROUND: Evidence has shown neurocognitive problems often exist among human immunodeficiency virus (HIV)-infected children. There are limited data for children in Nigeria. METHODS: This was a cross-sectional study of 100 school-aged perinatally HIV-infected children seen in the paediatric HIV clinic and age/sex-matched controls from the general paediatric clinic. Neuro-cognitive functioning was assessed using the Raven's progressive matrices (RPM) that has been adapted for the Nigerian population. RESULTS: The mean RPM score of subjects was 22.97 ± 11.35 compared with 32.93 ± 15.71 among controls (p < 0.001). Twenty-two percent of subjects in the HIV-infected group vs. 56% of controls were in the above-average intelligence group on the RPM. Thirty-four percent had average scores, while 22% were in the below-average scoring range. Neuro-cognitive functioning of the subjects was significantly affected by immunologic staging and socio-economic status. CONCLUSIONS: Neurocognitive functioning of the HIV-infected children was significantly lower than those of their un-infected counterparts. Neurodevelopmental evaluation should be part of standard care in HIV-infected children in Nigerian setting.

Preliminary Evaluation of a Point-of-Care Testing Device (SickleSCAN™) in Screening for Sickle Cell Disease.

Sickle cell disease affects about 150,000 births annually in Nigeria. Early diagnosis is hampered by factors such as centralized and urban localization of laboratories, high cost of diagnostic equipment and inadequate skilled manpower to operate them. The need for a low-cost, portable, easy-to-use diagnostic test for sickle cell disease is critical, especially in resource-poor countries. In this study, we evaluated the performance characteristics of a novel point-of-care testing device (SickleSCAN™), and its acceptability and feasibility, as a possible screening tool for sickle cell disease. In the first phase, we assessed the performance characteristics of SickleSCAN™ by evaluating 57 subjects comprising both children and adults attending a primary health center, for Hb SS (ßS/ßS; HBB: c.20A>T), Hb SC (ßS/ßC; HBB: c.19G>A) and Hb AS (ßA/ßS) using SickleSCAN™, cellulose acetate electrophoresis (CAE) and high performance liquid chromatography (HPLC). Performance characteristics such as diagnostic sensitivity and specificity were compared to HPLC as a standard method. We subsequently undertook a second phase wherein the acceptability and feasibility of the device for sickle cell disease screening, was evaluated using semi-structured and structured questionnaires among 197 healthcare personnel and 221 subjects, respectively. Sickle cell disease was carried by 3.4% of the subjects. The diagnostic sensitivity, specificity and test efficiency of SickleSCAN™ for sickle cell disease (Hb SS and Hb SC), were 100.0, 98.2 and 98.2%, respectively. Findings from this study showed SickleSCAN™ to be a viable screening tool that can easily be applied in community-based screening for early diagnosis of sickle cell disease with little expertise and low cost.

Hematological Indices at Birth of Infants of HIV-Positive Mothers Participating in a Prevention of Mother-to-Child Transmission Program.

BACKGROUND: The mother-to-child transmission of HIV, which accounts for 90% of infections in children, has been reduced markedly through the use of antiretroviral drugs by pregnant women and their newborns. Changes to the World Health Organization guidelines support further extension of the prevention of mother-to-child transmission programs with increased risk of toxicity on the fetuses. AIM: To determine the hematological indices at birth of infants exposed in utero to maternal antiretroviral drugs. METHOD: A comparative analytical study of 126 neonates whose blood samples were analyzed to determine their hematological indices. RESULT: The hemoglobin, hematocrit, the total white blood cell (WBC) count and absolute neutrophil count (ANC) were significantly lower in infants of HIV-positive mothers. The total WBC and ANC were also significantly lower in the highly active antiretroviral therapy. HAART group and those exposed to maternal drugs for <1 year. CONCLUSION: There are significant changes in the hematological indices of infants of HIV-positive mothers at birth.

Microbiological profile of chronic suppurative otits media among HIV infected children in South Eastern Nigeria.

Background: Immune compromised HIV/AIDS infected children have consistently shown a higher prevalence of chronic suppurative otitis media than their immune competent counterparts.This study aimed to compare the microbial isolates from ear discharges in HIV infected and non infected children. Method: This was a Hospital based prospective cohort study designed to determine the relative prevalence of the common organisms present in chronically discharging ears of HIV infected children and their antimicrobial sensitivities and compare that of age and sex matched non HIV infected children. Fifty HIV infected children being followed up at the paediatric HIV clinic of a tertiary health facility, had ear swabs obtained for culture and antimicrobial sensitivity.The same was done for another cohort of fifty age and sex matched non HIV infected children. Results: The mean age of the study population and controls were 6years 11months (SD 0.96) and 7years 11 months (SD 1.00) respectively. Male to female ratio for each group was 1:1.Ear discharge was bilateral in 27(54%) HIV infected children and in 35(70%) of the controls. The most prevalent bacterial isolate in both groups was Pseudomonas aeruginosa though it was more prevalent in HIV infected children(P=0.005). Fungal isolates were commoner in non HIV infected children(P=0.001).Ninety percent sensitivity to the 4 Quinolones was recorded by isolates in HIV infected children while sensitivity to the Aminoglycosides was greater in non HIV infected children Conclusion: Pseudomonas aeruginosa is the predominant organism isolated from HIV infected children with chronic otitis media.Fungal isolates are less frequently encountered in ear discharges of HIV infected children. The 4 Quinolones are the drugs of choice in treatment of Chronic suppurative otitis media in HIV infected children.

Obesity in Preschool-Aged Children with Sickle Cell Anemia: Emerging Nutritional Challenge in a Resource Limited Setting.

BACKGROUND: The burden of childhood obesity is increasing in most developing countries particularly in the urban areas owing to globalization; and children with sickle cell anemia (SCA) in such environment do not live in isolation. The study is aimed at determining the prevalence of obesity in younger children with SCA. METHODS: A consecutive recruitment of 58 children with SCA aged between 1 year and 5 years 4 months (subjects) and their age and sex matched controls selected using multistage sampling technique were carried out. The weight and height of the children were obtained using standard equipment. The WHO Anthro and Anthro-Plus software were used for analyzing the nutritional status of the children based on standard deviation (z-scores). The data were analyzed using SPSS version 19.0 while the level of significance was set at p < 0.05. RESULTS: The mean BMI for the subjects was 15.38 ± 1.93 (range from 11.50-20.80) and 17.10 ± 2.28 (range from 13.4-23.20) in the controls. Overweight (z-score > + 1 ≤ 2) and obesity (z score >+2) were recorded in 10 (17.2%) and 2 (3.4%) of the subjects while in the controls overweight and obesity were observed in 16 (27.6%) and 13 (22.4%), respectively (p = 0.000). CONCLUSIONS: Obesity and overweight is becoming prevalent among children with SCA and so awareness and preventive health education about obesity should be created among caregivers of children with sickle cell anemia in our setting to avert its possible complications.

Bioelectric impedance analysis of body composition of children and adolescents with sickle cell anemia in Enugu, Nigeria.

BACKGROUND: Body composition indices are widely used to evaluate growth and nutrition in children, particularly those with sickle cell anemia (SCA), who are known to have impaired growth, impaired skeletal maturation, and delayed puberty. The current study is aimed at determining the body composition of children with SCA. METHODS: Consecutive selection of SCA children aged 6 to 18 years, who served as subjects and their age and gender matched children with HbAA (controls), selected using multi-stage systematic sampling. It was a cross-sectional descriptive study conducted at the pediatric hematology and oncology clinic. Bioelectric impedance analysis was used to determine the body composition parameters, including weight, body fat percentage (BFP), visceral fat percentage, body mass index (BMI), skeletal muscle percentage, and resting metabolic rate. Data were analyzed using SPSS 16.0 at P < 0.05. RESULTS: One hundred and thirty-two subjects and controls respectively were studied. Subjects had lower body composition parameters compared with controls, with the older male subjects aged 10 to 18 years, having lower body composition indices (weight, height, BMI, and BFP) compared with controls. Visceral fat percentage was low in both subjects and controls. CONCLUSIONS: Children with SCA, particularly the older males, have impaired body composition indices. There is a need to conduct further studies to determine the longitudinal aspects of growth as well as quantitative and qualitative assessment of nutritional intake in children with SCA, which will inform higher alimentation requirements for HbSS children as compared with HbAA individuals.

Determinants of academic performance in children with sickle cell anaemia.

BACKGROUND: Some factors are known to influence the academic performance of children with Sickle Cell Anaemia (SCA). Information on their effects in these children is limited in Nigeria. The factors which influence academic performance of children with SCA in Enugu, Nigeria are determined in this study. METHODS: Consecutive children with SCA aged 5-11 years were recruited at the weekly sickle cell clinic of the University of Nigeria Teaching Hospital (UNTH) Enugu, Nigeria. Their age- and sex- matched normal classmates were recruited as controls. The total number of days of school absence for 2009/2010 academic session was obtained for each pair of pupils from the class attendance register. Academic performance was assessed using the average of the overall scores in the three term examinations of same session. Intelligence ability was determined with Draw-A-Person Quotient (DAPQ) using the Draw-A-Person Test while socio-economic status was determined using the occupational status and educational attainment of each parent. RESULTS: Academic performance of children with SCA showed statistically significant association with their socio-economic status (χ2 = 9.626, p = 0.047), and significant correlation with DAPQ (r = 0.394, p = 0.000) and age (r = -0.412, p = 0.000). However, no significant relationship existed between academic performance and school absence in children with SCA (r = -0.080, p = 0.453). CONCLUSIONS: Academic performance of children with SCA is influenced by their intelligence ability, age and socio-economic status but not negatively affected by their increased school absenteeism.

Academic performance and intelligence scores of primary school-aged children with sickle cell anemia.

Children with sickle cell anemia (SCA) are faced with complications which may interfere with their educational activities including academic performance. Reports on their academic performance are mainly from developed countries and the results have been inconsistent. This study aimed to determine the academic performance of primary school-aged children with SCA in Nigeria and compare findings with a group of controls. Ninety children with SCA aged 5-11 years were consecutively recruited at the SCA clinic of UNTH Enugu and their age- and sex-matched normal classmates were enrolled as controls. Academic performance of the children with SCA was studied using the overall scores achieved in the three term examinations in the preceding academic year (2009/2010), while their intelligence quotient (IQ) was determined using the Draw-A-Person Test. The findings were compared with that of 90 controls. The mean overall academic score of the children with SCA of 62.71 ± 19.43% was similar to 67.47 ± 16.42% in the controls (P = .077). However, a significantly higher number of children with SCA (32.2% vs. 16.7% of the controls; P = .015) scored below 50%, thus, had poor performance. The mean IQ of the subjects (91.41 ±16.61%) was similar to that of the controls (95.56 ±17.31%, P = .103). However, more SCA patients had lower IQ scores than controls though not statistically significant (P = 0.083). The overall academic performance of children with SCA, therefore, compares favorably with that of controls although there is a higher prevalence of poor performance among them.

Folate levels in children with sickle cell anaemia on folic acid supplementation in steady state and crises at a tertiary hospital in Enugu, Nigeria: a prospective, comparative study.

Introduction: Folic acid supplementation is an integral aspect of the management of children with sickle cell anaemia (SCA) especially in Africa. In spite of this, there have been concerns about lower folate levels, especially during crisis. Aim: To determine red cell folate levels of children with sickle cell anaemia in steady state and during crisis and compare with those with haemoglobin AA genotype. Method: This study was prospective, hospital based, and comparative. Fifty children with sickle cell anaemia were recruited during crises and followed up until they met the criteria for attaining steady state. The controls were fifty children matched with those with SCA for age and gender and had haemoglobin AA genotype. Red cell folate estimation was done with the Electrochemiluminescence Immunoassay (ECLIA) method using the automated Roche Cobas e411 equipment. Results: The median (IQR) red cell folate level in children during sickle cell crisis was 265.95 (134.50) ng/ml, which was significantly lower than the median (IQR) of 376.30 (206.85) ng/ml obtained during steady state. Most children with SCA (41 out of 50) had significantly higher folate levels during steady state (T=1081, Z-score= -4.660, p < 0.001). Median level of red cell folate was lower during anaemic crisis compared to vaso-occlusive crisis, though not significantly so (N(50), U = 214.00, Z-score= -1.077, p = 0.305). The median red cell folate level of normal controls was 343.55 (92.90) ng/ml, which was significantly lower than the 376.30 (206.85) ng/ml obtained during steady state (N(50), U= 209.00, Z-score= -7.177, p <0.001). Conclusion: Median red cell folate levels of the study participants were within normal limits, though most children with SCA had significantly higher levels during steady state compared to crisis. Normal controls had significantly lower red cell folate levels than the children with SCA during steady state.

Sexual maturation of HIV-infected and uninfected male children in Abakaliki, South-East, Nigeria: a cross-sectional study.

Introduction: HIV infection, through various mechanisms causes a derangement in sexual maturation. This study compared the Marshal and Tanner staging of HIV-infected and uninfected males. The aim of the study was to determine the sexual maturation in male children infected with HIV on HAART in Abakaliki. Methods: this was a cross-sectional and comparative study involving 80 HIV-infected boys aged 8-17 years and 80 uninfected counterparts matched for age and socio-economic class. Stages of sexual maturation (testicular size and pubic hair) were determined according to the method proposed by Marshall and Tanner. The testicular size was measured using an orchidometer. Data analysis was done with SPSS version 20. Structured questionnaire was used to collect information on socio-demographics. Results: assessment of pubic hair development, showed that 45 (56.2%) of the subjects were in the pre-pubertal stage compared to 27 (33.8%) among the controls, this relationship was statistically significant (p=0.005, OR = 2.5, C.I=1.3-4.8). The mean testicular volume among subjects was found to be 8.29 ± 8.26mls compared to 11.57 ± 8.26mls found in controls. This relationship was also statistically significant. There were significant statistical relationships between duration on HAART and clinical stages of disease with both pubic hair development and testicular volume of subjects and controls. Conclusion: HIV-infected males had significantly delayed onset and progression of sexual maturation. Routine assessment of the sexual maturation of HIV-infected children as well as addressing the modifiable variables influencing sexual maturity is recommended.

Sexual and procreation preferences of adolescents and young adults infected perinatally with HIV in Southeast Nigeria: a hospital-based cross-sectional study.

INTRODUCTION: children with perinatal human immunodeficiency virus (HIV) infection now survive to adolescence and adulthood and so are confronted with issues related to sexuality and sexual reproductive health. This study is aimed at determining the sexual knowledge and behaviours of these adolescents, determining their procreation intention and the impact of their age, gender and understanding of the mother to child transmission risk on their procreation intention. METHODS: a hospital-based cross-sectional study of adolescents and young adults aged 15-24 years infected with HIV in the perinatal period, accessing tertiary care in Enugu. A pretested questionnaire was used to obtain information about socio-demographic variables, sexual knowledge and behaviour, procreation intention and knowledge of prevention of mother-to-child transmission of HIV. Data analysis was descriptive and a test association using fisher´s chi square was done on the variables. RESULTS: seventy-one adolescents were studied. The majority (95.8%) were less than 20 years of age. Mean age was 17.01 ± 1.80 years with M: F ratio of 1:1.7. Nineteen (26.8%) were sexually active with 15 (78.9%) having single partners. About 80% had their first sexual activities before the age of 18 years. Fifty-six (78.9%) received some form of sex education. Only gender and socio-economic status was significantly related to marriage and procreation intentions of respondents. CONCLUSION: there´s need for sustained/intensive education programs and policy on sexual practices with focus on perinatally infected adolescents who may not be well informed on risk and consequences of their sexual preferences.

The effect of sickle cell anemia on the linear growth of Nigerian children.

OBJECTIVES: Despite the high prevalence of children with sickle cell anaemia (SCA) in West Africa, there is paucity of data on the height velocity and prevalence of growth failure in SCA patients. With advances in clinical care of SCA patients, could there be a spatial and secular trend in the growth pattern of these children? Hence, the compelling needs to embark on this study. The objectives of the study were to determine the prevalence of growth failure among patients with SCA and its correlation with age, gender and age at diagnosis. METHODS: A Prospective longitudinal study of a cohort of sickle cell anaemic paediatric patients from Pediatrics SCA Clinic, University of Nigeria Teaching Hospital, Ituku Ozalla. Patients were enrolled over a period of two years using a non-parametric convenient sampling method. Their heights were measured at baseline, three months, six months and at 12 months intervals and subsequently plotted on a standard WHO growth chart. The height velocities at different monthly intervals were calculated and compared with the WHO standard normal linear growth rates) for children (used as control) to identify those with GF. (i.e. <10th percentile). The main outcome measures were the mean height velocities at different months' intervals calculated and compared using the repeated measurement analysis of variance (ANOVA) and the Wilcoxon signed test. RESULTS: A cohort of 316 children aged 1-18 years with SCA was evaluated with a male preponderance of 161 (57.4%). The mean age and age at diagnosis were 11.04 ± 5.56 and 4.2 ± 1.7 years, respectively. The prevalence of growth failure and short stature was 84.7%. The burden of GF was highest among post-pubertal participants (94.1%). The most important predictor of growth velocity deficit was age (R2=0.045, standard ß coefficient = -0.22, t=-03.51, p=0.001). CONCLUSIONS: The study demonstrated high prevalence of growth failure in children and adolescents with SCA which intensified with advancement in age and older age at diagnosis.

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.

The burden of pediatric malignant solid tumors in a developing country.

OBJECTIVE: This study evaluates the burden of pediatric malignant solid tumors (PMST) in southeastern Nigeria. METHODS: Analysis of 174 cases of PMST managed at the University of Nigeria Teaching Hospital Enugu, from January 2002 to November 2007. RESULTS: PMST comprised 2.8% of pediatric admissions. Tumors encountered were lymphomas 77 (44.3%), Wilms' tumor 35 (20.1%), sarcomas 20 (11.5%), neuroblastoma 15 (8.6%), retinoblastoma 14 (8.0%), teratomas 8 (4.6%) and hepatoma 5 (2.9%). Mean time for diagnosis was 3.3 +/- 2.4 months. Advanced disease occurred in 135 (77.6%), while 39 (22.4%) had early disease. Only 166 (95.4%) commenced planned therapy, with 67 (40.4%) lost to follow-up. Of 99 children available for evaluation, 43 died (11 treatment related and 32 from relapse). Overall, 56 (56.6%) were alive after a mean follow-up of 20.7 +/- 10.5 months. CONCLUSION: PMST are enormous challenge in our setting. Delayed presentation, poor treatment compliance and healthcare funding have to be addressed to improve outcomes.

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.

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.