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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 hae- moglobin 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. Keywords: Sickle cell anaemia; disease severity; folate status; children; Enugu.

Background: Asthma prevalence is rising worldwide. Amongst the various factors influencing asthma control, the magnesium level of the affected individuals seems to make a major contribution to achieving asthma control through i ts anti - inflammatory and bronchodilation effects.This study therefore was aimed at determining the magnesium level of stable asthmatic children and its relationship, if any, with their levels of asthma control.Methods: A cross-sectional study where the serum magnesium levels of 30 stable asthmatic children attending Asthma clinic and those of 30 children without asthma attending Children Out-patient clinic (CHOP) both at the University of Nigeria Teaching Hospital (UNTH), Enugu, were assessed using atomic absorption spectrophotometer. The levels of asthma control of children with asthma were assessed using asthma therapyassessment questionnaire (ATAQ). Mean serum magnesium levels of the different asthma control levels were compared using the analysis of variance (ANOVA).Results: The mean ages of children with asthma and those without asthma were 10.4 ± 2.98 years and 10.5 ±3.00 years respectively. The mean serum magnesium level of stable children with asthma (1.13 ±1.04 mmol/L) was found to be significantly lower than those of the non-asthmatic controls (1.46 ±1.01 mmol/L), p= 0.004.A significant association was also found between the serum magnesium levels and the levels of asthma control (p = 0.015).Conclusion: This study shows that the serum magnesium level of stable asthmatic children is significantly lower than that of children without asthma and there is a significant association between the mean serum magnesium levels and their levels of asthma control. Keywords: Serum magnesium, asthma control, stable asthma

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.

Introduction: Glanzmann’s Thrombasthenia (GT) is a rare autosomal recessive bleeding disorder due to defective platelet membrane glycoprotein GP IIb/IIIa (integrin αIIbβ3). 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 diag- nosis 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 consan- guinity is rarely practised. Our health facilities require adequate diagnostic and treatment facilities for rare diseases like GT. Keywords: Glanzmann’s thrombasthenia; Menorrhagia; Nigerian girl.

Background: HIV infection significantly increases depression risk in the caregivers of HIV-infected children. Our study is aimed at determining the depression prevalence and severity in caregivers of children who are infected with HIV. Materials and Methods: This was a hospital-based, cross-sectional, and comparative study. Study participants who met the inclusion criteria were enrolled consecutively to achieve the calculated sample size. Their sociodemographic variables were obtained, and the Zung Self-rating Depression Scale was used to assess the prevalence and severity of depression in the caregivers. Results: There were 352 study participants which comprised 176 caregivers of children infected with HIV, who served as cases and 176 caregivers of HIV-negative children, who served as controls. One hundred and fifty-five (88.1%) of the cases were the biologic caregivers, whereas the biologic caregivers comprised of 133 (75.6%) for the controls. Furthermore, 43 (24.4%) and 133 (75.6%) of the cases were males and females, respectively, whereas 47 (26.7%) and 129 (73.3%) of the controls were, respectively, males and females. The prevalence of depression among the cases and controls was 43.2% and 6.8%, respectively (P < 0.001; odds ratio [OR]: 10.39). Among the cases with depression, 73 (47.1%) were the biological caregivers, whereas 3 (14.3%) were the nonbiological caregivers. This was statistically significant (P = 0.009; OR = 5.341). The severity of depression among the cases was mild depression (52.6%), moderate depression (30.3%), and severe depression (17.1%). Conclusions: There is a high prevalence of depression among the caregivers of children infected with HIV and the biologic caregivers of these children tend to have a higher depression burden than their nonbiologic counterparts.

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.

Background Sickle cell anaemia (SCA) is characterized by attendant ischemia-reperfusion injury especially to the heart. Methods The aim of this work is to compare the right ventricular function of children with SCA in steady state (subjects) with those with haemoglobin AA genotype (controls), using echocardiography. It is a cross-sectional study, which echocardiographic measurements to assess right ventricular function among children with SCA and their controls. Results The mean trans annular plane systolic excursion (TAPSE) in subjects, 28.24 ± 5.23 (Z score: 0.258 ± 1.10) was higher than that in control, 25.82 ± 3.59 (Z score: - 0.263 ± 0.80), and the difference in mean was statistically significant, (t = 2.703, p = 0.008). Significantly higher proportion of subjects with sickle cell anaemia had right ventricular dysfunction (Abnormal TAPSE), 25 (50.0%) when compared with those in control, 11 (22.0%), {χ2 = 8.5, p = 0.0035}. A higher proportion of subjects with sickle cell anaemia (25.5%) had Pulmonary hypertension (RVP) when compared with control (2.0%) and the difference in proportions was found to be statistically significant, (χ2 = 11.668, p = 0.001). The prevalence of right ventricular diastolic dysfunction in subjects was 9.8% while control was 0%. Conclusion Children with sickle cell anaemia present with right ventricular dysfunction. Prevalence of right ventricular systolic and diastolic dysfunction were higher in subjects. More of the subjects in this study (25.5%) had pulmonary hypertension.

Summary Background Nutrition plays a pivotal role in brain development throughout life. Sub-optimal intellectual ability and poor school performance are said to be among the long term effects of malnutrition. The aim of this study was to determine the association between nutritional status of the participants, their intelligence quotient (IQ) and academic performance. Methods Children aged 6–12 years who met the inclusion criteria were recruited from the public and private primary schools in the local government area using a proportionate multistage sampling technique. Weight and height were measured using standard protocols and interpreted as normal or abnormal using the World Health Organization AnthroPlus®. IQ was assessed using the Raven's Standard Progressive Matrices and was grouped into optimal and suboptimal. Academic performance was assessed using the past records of class assessment, and was classified into high, average and low academic performance. A semi-structured questionnaire was used to obtain data such as–age, gender, socioeconomic indices and family size of the study participants. Results The prevalence of underweight, thinness (wasting), stunting, overweight and obesity were 2.0%, 3.6%, 2.1%, 6.7%, and 4.2%, respectively. Indices of over-nutrition were significantly associated with optimal IQ and good academic performance. There was a trend in the association between wasting and suboptimal intelligence [AOR (95%CI) = 1.5 (1.0–3.0), p = 0.06]. Conclusion Acute and chronic under-nutrition did not adversely affect the IQ and academic performance of the study population. The relationship between over-nutrition, IQ and academic performance disappeared when socio-economic status was controlled for.

Introduction:intellectual capacity measured as intelligence quotient (IQ) is one of the determinants of school performance of children. It influences academic achievement, future personal health, social well-being and therefore, is of public health significance. The objectives of the study was to determine the intelligence quotient (IQ) and academic performance of primary school children in Enugu-East LGA. Methods:children who met the inclusion criteria were recruited from both public and private primary schools in the Local Government Area (LGA) using a proportionate multistage sampling technique. Academic performance was classified into high, average and low academic using past records of class assessment. Intelligence Quotient was assessed using the Raven´s Standard Progressive Matrices (RSPM) and was grouped into optimal and suboptimal. A semi-structured questionnaire was used to obtain data such as-age, gender, socio-economic indices and family size of the study participants. Analysis was done with Statistical Package for Social Sciences (IBM-SPSS). Results:a total of 1,122 pupils aged 6 to 12 years were recruited. Optimal IQ and high academic performance was found in 54.0% and 58.8% of the study participants. Being from upper social class, in private school, and family size less than 4 were the significant determinants of high IQ and good academic performance (p4 children) and improve the socio-economic status of families are needed environmental measures to improve intelligence and academic performance.

Intoduction: 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.Keywords: Burkitt lymphoma, generalized lymphadenopathy, Nigerian child.

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.

Background: Thrombocytopaenia is known to complicate many neonatal illnesses and contributes significantly to morbidity and mortality. Early diagnosis and treatment are necessary to avoid complications. There are a few studies on the prevalence of neonatal thrombocytopaenia in South Eastern Nigeria. This study aimed to determine the prevalence and clinical correlates of neonatal thrombocytopaenia in South Eastern Nigeria.Methods: The prospective study was carried out over a period of eight months (December 2015 to July 2016). One hundred and fifty nine neonates admitted consecutively into the Special Care Baby Unit of the Federal Medical Centre, Owerri were recruited and evaluated for thrombocytopaenia on admission and after forty-eight hours in the study. Blood platelet was analysed using Sysmex KX-21N automatic platelet analyser and manual counting. Thrombocytopenia was defined as blood platelet less than 100×109/L.Results: The subjects included 89 (56%) males and 70 (44%) females. The prevalence of neonatal thrombocytopaenia was 6.3% on admission and 8.3% after forty-eight hours. Bleeding from orifices (p=0.012), prolonged pregnancy (p=0.047) and petechiae (p=0.020) were clinical correlates significantly associated with thrombocytopaenia. No specific clinical diagnosis was significantly associated with thrombocytopaenia, though the odds of a newborn having thrombocytopaenia was higher in certain conditions indicating increased risk.Conclusions: The prevalence of thrombocytopaenia in this study suggests that platelet count should be done for neonates admitted into special care baby units in resource-poor settings. This should be more in neonates with bleeding from orifices, prolonged pregnancy and petechiae.

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.

Background Cardiac complications contribute significantly to morbidity and mortality in children with HIV/AIDS. These rates have been under-reported in sub-Saharan African children. Methods This was an observational, cross-sectional Doppler echocardiographic study of ventricular systolic function, performed at a tertiary clinic on children with HIV/AIDS. Results Left ventricular systolic dysfunction was present in 27.0% of the children with HIV infection and 81.2% of those with AIDS. The mean fractional shortening in the AIDS group (31.6 ± 9.5%) was significantly lower than in the HIV-infected group (35.3 ± 10.5%, p = 0.001). A significant correlation was found with CD4(+) cell count and age, and these were the best predictors of left ventricular systolic dysfunction in the stepwise multiple regression analysis (r = 0.396, p = 0.038; r = -0.212, p = 0.025, respectively). Conclusion Left ventricular systolic dysfunction is common in Nigerian children with HIV/AIDS.

BACKGROUND Nigeria has the highest burden of paediatric HIV in the world. HIV infection may result in significant life stressors, on both the infected children and their caregivers. METHODS This included a hospital-based cross-sectional and comparative study. Subjects and controls who met the inclusion criteria were enrolled consecutively. Their socio-demographic variables were obtained and GHQ-28 was used to assess their psychosocial status. RESULTS A total of 154 caregivers of HIV-infected children (subjects) and 154 caregivers of HIV-negative children (controls) were enrolled into this study. In total, 42 (27.3%) and 112 (72.7%) of the subjects were males and females, respectively, compared with 37 (24.0%) and 117 (76.0%) of the controls, respectively. The prevalence of psychosocial disorders among the subjects and controls was 39 and 2.6%, respectively (p

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

Introduction: Several factors including the parental literacy, illness, socioeconomic status, poor sanitation and hygienic practices affect the physical growth of children. The aim of this study was to determine the socio-demographic determinants of malnutrition among primary school aged children in Enugu, Nigeria. Methods: A cross-sectional descriptive study involving primary school children in Enugu was carried out over a 3 month period. Subjects were selected using multistage sampling technique. Weight and height were measured using a digital scale and a wooden stadiometer, respectively. Body Mass Index (BMI), weight-for-age (WAZ), Height-for-age (HAZ) and BMI-for-age z scores were then derived using the new WHO reference standards. Results: 348 children (40.4%) were recruited from 5 public schools while 512 (59.6%) were recruited from 9 private schools. The mean age of the study participants was 9.2 ± 1.8 years. 7 (0.8%) children were stunted, 26 (3.3%) wasted and 28 (3.3%) underweight. Of all the study participants, overweight and obesity were observed in 73 (8.5%) and 35 (4.1%) children, respectively. Children of lower socioeconomic class were more stunted, underweight and wasted, while overweight and obesity were more prevalent among children from the upper socioeconomic class. Conclusion: Factors such as age and sex, parental education and socioeconomic class had a significant impact on nutritional status. Overweight and obesity were more prevalent among the children from the upper socioeconomic class, attending private schools, while stunting and wasting were more in children of the lower class attending public schools. Keywords: Socio-demographic, malnutrition, school-age, children, Enugu

Background: Sickle cell anaemia is one of the most common inherited disorders globally. Some affected children have retardation of physical growth which is also seen in those with zinc deficiency. Objective: To assess the relationship between zinc levels and anthropometric indices of SCA children.Methods: A crosssectional, case-control study on young females aged 6-18 years at the UNTH, Enugu. Relevant clinical data as well as 24 hour dietary recall were collected. Weights and heights were measured using standard protocols and BMI calculated. Serum zinc was determined using Atomic Absorption Spectrophotometer. Data was analyzed using SPSS version 15.0 while the level of statistical significance was set at P < 0.05.Results: Eighty-one subjects with HbSS and 81 matched controls with HbAA were studied. Mean weights of 34.58 ± 12.76kg found in patients were significantly lower than 40.19 ± 13.37kg in controls. Also mean BMI of 16.27 ± 2.76kg/m2 in patients were significantly lower than 18.40 ± 2.96kg/m2 in controls (P = 0.01). Mean heights of patients were lower than that of the controls though not significantly so (P > 0.05). Mean serum zinc levels of 58.01 ± 10.58μg/d1 in patients were significantly lower than 68.37 ± 8.6μg/ dl in controls (P = 0.01). Positive correlation was found between serum zinc and BMI of the studied children. Serum zinc has a significant relationship with weight, height and BMI.Conclusion: Reduced serum zinc in SCA children was associated with low anthropometric indices. Estimation of serum zinc is also recommended in SCA children with low anthropometric indices.Keywords: Anthropometric indices, female children, serum zinc, sickle cell anemia

School health services (SHS) have widespread impact on the health of a large number of children with implications on access to primary health care especially in developing countries. The aim of this study was to assess health services in primary schools in Enugu East Nigeria. Thirty-three head teachers of primary schools in Enugu east Nigeria and officials of Ministry of Education were interviewed using a questionnaire adapted from school health evaluation scale. Four private schools had health personnel. Only six private schools had a health room. Two public schools had a functional first aid box. There were no health records available in any of the schools. School lunch was given by only one private school. Of a maximum of 45, public and private schools had a mean score of 10.3 and 12.7 respectively on the school health evaluation scale (P=0.01). Three schools only attained the minimum acceptable score of 19. Health services are at a minimal level in primary schools in Enugu East Nigeria. A state school health policy should be developed through inter-sectoral collaboration of the relevant stakeholders to use the platform provided by schools to ensure access to primary health care and also act as bridge for more formal medical care for school children.

Background: Obesity and overweight are emerging major health problems in developing countries in the background of undernutrition. These have been linked to a substantial increase in mortality and morbidity.Objectives: This cross-sectional survey was aimed at determining the prevalence of underweight, overweight, and obesity using body mass index (BMI) in primary school pupils in Abakaliki metropolis of Ebonyi State, south-east Nigeria.Method: Eight hundred and four participants aged 6–12 years, from four public and four private primary schools had their weights and heights measured using standard methods. BMI value was calculated for each subject and compared with BMI for age and sex from World Health Organisation (WHO 2007) reference standard. Socioeconomic status was determined using method proposed by Oyedeji.Results: Out of 804 subjects, 426 (53.0%) were from public schools, whereas 378 (47%) were from private schools (P ≤ 0.01). Four hundred and fifteen (51.6%) were males and 389 (48.4%) were female (P = 0.88). The prevalence of underweight, overweight, and obesity using BMI were 4.5% and 1.2%, 0% in public schools and 1.1%, 5.0%, and 3.0% in private schools, (P < 0.001). The socioeconomic class significantly affected the prevalence of underweight, overweight, and obesity as more subjects with overweight and obesity belonged to upper social class, whereas more underweight subjects belonged to lower social class.Conclusion: Overweight and obesity are emerging in a background of undernutrition, showing ‘‘double burden’’ of nutritional disorder.Keywords: Body mass index, obesity, overweight, primary school children, socioeconomic class, underweight

Background: Poor growth and nutritional status are common features of sickle cell anemia (SCA) in children. The rising trend of obesity in children in developing countries has been reported despite a huge burden of undernutrition in these settings. In SCA, overweight/obesity is being increasingly reported. Aims: To evaluate the nutritional status and its determinants in children with SCA and to compare the same with hemoglobin AA (HbAA) controls of similar age, gender, and socioeconomic status. Methods: The study was a cross-sectional analytical study involving 175 subjects and controls aged 1–18 years who met the inclusion criteria. Weight and height were measured and body mass index (BMI) was calculated. Z scores were computed for the anthropometric measurements using the World Health Organization (WHO) standard reference. Hemoglobin concentration was determined using HemoCue Hb201+ Analyzer. Results: Subjects had significantly lower Z- scores for weight, height, and BMI compared with controls. Stunting, wasting, and overweight/obesity were observed in 10.9%, 24.6%, and 5.1% of subjects compared with 2.3%, 5.7%, and 9.7% respectively in controls. Wasting, stunting and overweight/obesity in SCA were significantly associated with age while overweight/obesity was significantly associated with upper social class (P = 0.001). Conclusions: Poor growth and nutritional status are still prevalent while overweight and obesity are emerging comorbidities among children with SCA in our environment. Regular nutritional assessment of children with SCA should be encouraged while those at risk of under/over-nutrition should receive adequate nutritional rehabilitation to prevent possible complications.

Aim To determine the normative values of stretched penile length in apparently healthy term Igbo newborn males in South eastern Nigeria and its relationship with gestational age, birth weight and birth length. Methods Eight hundred and eleven apparently healthy term male neonates within the first 72 h of life were studied. Enrolled subjects were consecutively delivered babies of Igbo extraction from three hospitals. The stretched penile length was measured using Schonfeld method, and the smoothed centiles (3 rd to 97th percentile values) for stretched penile length by gestational age were determined. Results The mean stretched penile length of term newborns was 3.46 ± 0.44 cm. The lower and upper limits (±2.5 SD) were 2.36-4.56 cm. Stretched penile length correlated positively with birth length and birth weight (r = 0.343, p = 0.001; r = 0.229, p = 0.001). Conclusion This study has created the first smoothed percentile values for stretched penile length by gestational age for Igbo babies.

Background: Reports show that children with sickle‑cell anemia (SCA) have a tendency for nocturnal enuresis when compared with their counterparts with normal hemoglobin. Although nocturnal enuresis in SCA has been attributed to several factors including tubular and even bladder dysfunction, its relationship with hyposthenuria has been questioned in some studies.Aim: The study aims to determine the relationship of hyposthenuria with nocturnal enuresis seen in school‑aged children with SCA.Subjects and Methods: A cross‑sectional study of seventy school‑aged children with SCA, who met the study criteria and seventy age‑ and gender‑matched controls was conducted at the Sickle‑cell Clinic, University of Nigeria Teaching Hospital in Enugu, Southeast Nigeria. The diagnosis of enuresis among the subjects and controls was based on the Diagnostic and Statistical Manual of Mental Disorders‑IV criteria while urine specific gravity (USG) was determined on dipstick urinalysis. The frequencies of categorical variables were compared using Chi‑square test or Fisher exact test as appropriate and the means of continuous variables with Student’s t‑test. The level of statistical significance was taken as P < 0.05.Results: The prevalence of hyposthenuria was 4.5% and 8.3% among enuretic and nonenuretic subjects respectively, 6.7% and 10.9% among enuretic and nonenuretic controls and 4.5% and 6.7% among enuretic subjects and controls, respectively. The differences were not statistically significant. The mean ± standard deviation USG was significantly higher in the subjects than in the controls (1.02 ± 0.01 vs. 1.01 ± 0.01, P = 0.013) and enuretic subjects than enuretic controls (1.02 ± 0.01 vs. 1.01 ± 0.01, P = 0.007). The prevalence of nocturnal enuresis was significantly higher in male subjects compared to female subjects (odds ratio [OR] [95% confidence interval (95% CI)] =8.14 (2.12, 31.24), χ2 = 12.21, P < 0.001) and male controls (χ2 = 5.57, P = 0.018). Enuretic subjects had a significantly higher prevalence of parental history of childhood enuresis (OR [95% CI] =10.39 [2.45, 44.05], P < 0.002) than the enuretic controls. The relationship between the enuretic subjects and controls with respect to age of attainment of urinary control, family size, socioeconomic class, and sibling history of enuresis were not statistically significant.Conclusions: Nocturnal enuresis in children with SCA may not be related to hyposthenuria. However, male gender and parental history of childhood enuresis are significant risk factors.Key words: Hyposthenuria, nocturnal enuresis, sickle‑cell anemia

Background:A number of factors influence sexual maturation in adolescents, including chronic illnesses like HIV. Marshall and Tanner devised a method of classifying the adolescent based on the level of sexual maturation into five stages. This study compared the Tanner staging of HIV-infected and uninfected girls.Methods:This was a cross-sectional study of 100 HIV-infected girls aged 8–18 years and 100 uninfected counterparts matched for age and social class. Using standard photographs as a guide, stages of sexual maturation were determined according to the method proposed by Marshall and Tanner. Data analysis was done with SPSS version 20. p-values Results:The study participants were aged 8–17 years. Fifty-five subjects compared to 39 controls were still in pre-pubertal breast developmental stages while 45 subjects (45%) compared to 61 controls (61%) have commenced breast development (p=0.024). Similarly, 52 subjects compared to 31 controls were in the pre-pubertal pubic hair developmental stages, while 48 subjects (48%) compared to 69 controls (69%) had commenced pubic hair development (p=0.003).Conclusions:Perinatal HIV infection affected the onset of pubic hair and breast development but did not significantly affect the attainment of sexual maturation.

Background: Childhood cancer is a leading cause of child mortality in developed countries as well as a recognized contributor to malnutrition and death in developing countries, in particular those of low socioeconomic status. It is estimated that about 50% of children with cancer in developing countries are malnourished while in developed countries, malnutrition among cancer patients is related to the type and the extent of tumor. Anthropometry is the most common and easy way for assessing the nutritional status of children. Objectives: This study aimed at assessing the nutritional status of children with cancer at the University of Nigeria Teaching Hospital, Enugu, compared with non cancer controls, by using anthropometric parameters. It also attempted to determine the best anthropometric parameter to detect malnutrition in cancer patients. Methods: Over a 36 month period, a case controlled study of children aged 3 months to 18 years with or without cancer was conducted to determine their nutritional status. The children comprised 104 patients with cancer (cases) and 104 controls seen at children’s outpatient clinic with minor illnesses. Their anthropometric measurements were taken within 24 hours of admission and at point of contact for the controls. Result: The prevalence of malnutrition was higher in cancer patients as the anthropometric parameters were significantly lower among them than the controls (WFA: t=0.019, HFA: t=0.019). Prevalence of malnutrition was higher in the low socioeconomic group. Skin fold thickness was observed to be a more sensitive indicator than weight related anthropometry in assessing the nutritional status of children with cancer. Conclusion: Children with cancer on admission were significantly more malnourished than those without cancer and will require nutritional support to reduce the morbidity and mortality arising from such illness.

In a previous retrospective study, it was observed that the greater the amounts of the omega-3 fatty acids eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) in the blood, the lesser the number of complications of sickle cell disease (SCD) and the higher the steady state haemoglobin level. SCD causes ischaemia-reperfusion injury and inflammation; which can be ameliorated by a metabolite of DHA that down-regulates expression of pro-inflammatory genes. The objectives of this prospective pilot study were to evaluate the effects of DHA and EPA supplements in SCD, and test the hypothesis that these effects are mediated partly by reducing inflammation. Oral DHA and EPA supplements were given to 16 SCD patients for 6 months. We then compared pre- and post-supplementation values of number of crisis, steady state Hb, plasma unconjugated bilirubin and three indices of inflammation: plasma interleukin-6, blood neutrophil and platelet counts. There was a significant reduction in the plasma level of unconjugated bilirubin, and the number of sickle cell crisis; but not in the markers of inflammation. The pilot data suggest that DHA and EPA supplements reduce the number of crisis and steady state haemolysis in SCD; but provide no evidence that these effects are mediated by reducing inflammation.

Assessment of sexual maturation of children continues to have great relevance in monitoring their overall development. The interplay between innate disease characteristics and environmental modifiers such as socioeconomic status is not clearly understood among children with sickle cell anaemia (SCA).To evaluate impact of socioeconomic status on sexual development of children living with sickle cell anaemia.A cross-sectional case-control study evaluating sexual maturation of male patients with SCA (subjects) compared with non-SCAs (controls) was done. Tanner staging and testicular volume (TV) assessment were used for evaluating sexual maturation among both groups matched for age and socioeconomic status. Pattern of sexual maturation was related to socioeconomic status.Subjects demonstrated delay in onset and completion of sexual maturation compared to controls. Testicular volumes of subjects were smaller than controls but when related to Tanner staging showed no significant difference between both groups. Onset of puberty was earlier in children from higher socioeconomic classes though this was very modest and not statistically significant. Similarly children of higher classes showed slightly larger TVs compared with those from lower classes.SCA patients continue to demonstrate delay in sexual maturation compared with controls with normal haemoglobin genotype. Higher socioeconomic status showed a slight improvement on sexual maturation. This should provide a strong basis for advocacy to improve the welfare of families of children living with SCA as a worthwhile measure to improve their development and overall outlook. A larger study of a prospective nature is highly indicated.

Background Sickle-cell anaemia (SCA) is the most common inherited haemoglobinopathy affecting the Negroid race. Renal complications such as enuresis can occur during childhood. Reports show that children and adolescents with SCA may be at a higher risk of nocturnal enuresis than their counterparts with normal haemoglobin genotype. Aims The study aims to determine the prevalence of nocturnal enuresis and possible risk factors among school-aged children with SCA in a South-east Nigerian city. Methods A hospital-based and cross-sectional descriptive study of 70 school-aged children with SCA who met the study criteria, and 70 age- and sex-matched controls with normal haemoglobin genotype was conducted in the Paediatric Sickle-cell Anaemia Clinic of the University of Nigeria Teaching Hospital (UNTH) Enugu. Data was subjected to multivariate analysis using logistic regression model with nocturnal enuresis as the dependent variable and the possible risk factors as the independent variables. Results The prevalence of nocturnal enuresis among the Subjects and the Controls was 31.4 and 21.4 % respectively (p = 0.180). It was significantly higher among male Subjects (48.7 %) than among male Controls (23.1 %) [OR (95 % CI) =8.14 (2.12–31.24), p

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. Saudi Med J 2015; Vol. 36 (8): 928-934 doi: 10.15537/smj.2015.8.11525

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.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 p0.05.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).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.

Intra‑thecal chemotherapy is a recognized therapy for hematological malignancies such as acute lymphoblastic leukemia (ALL). Despite the advantage of these drugs in treating or preventing central nervous system disease, they are not without complications. The authors describe a 12‑year‑old girl with ALL, who developed progressive myelopathy following intra‑thecal administration of cytosine arabinoside. Initial presentation was urine and fecal retention that progressed to paraplegia, and finally encephalopathy. magnetic resonance imaging of the neuroaxis showed T2‑weighted foci of increased signal intensity within the substance of the cervical cord indicative of myelopathy. Physicians should be wary of this rare complication of intra‑thecal chemotherapy.Key words: Cytosine arabinoside, intra‑thecal, myelopathy

Background: Non-communicable diseases are increasing worldwide due to rapidly changing lifestyles and socio-economic status. It is contributing significantly to the global burden of diseases. Objective: To determine the pattern of non-communicable diseases in children admitted into the Paediatrics ward in a tertiary health centre in Enugu. Materials and Methods: A review of admissions into the Paediatrics ward of the University of Nigeria Teaching Hospital Enugu, between January 1999 and December 2008 was done using the registry of admission and discharge. Results: The age range of patients admitted during the period was 2 months to 18 years (mean 5.27 ± 5.42 years). There were 1173 (59.6%) males and 796 (40.4%) females. Disorders of the haematological system accounted for 514 (23.3%) of the non-communicable diseases among the admissions, malignancies accounted for 424 (19.2%) among the admissions, whereas the renal, central nervous, and cardiovascular systems were involved in 282 (12.8%), 274 (12.4%), and 241 (10.9%) patients, respectively. There were 274 (12.4%) deaths and 1667 (75.5%) discharges while 38 (1.7%) were discharged against medical advice. Data on 221(10.2%) of the patients were reported missing. Malignancies contributed to 75 (27.3%) of the deaths, haematological disorders accounted for 44 (16%) whereas renal disorders and nutritional disorders contributed to 43 (15.7%) and 41 (15%) of the deaths, respectively. Conclusion: Non-communicable diseases affect children in our environment and contribute to morbidity and mortality in children. Strategies to prevent these diseases should be encouraged in order to avert the challenges of double burden of the diseases in children.

Background: Hematological abnormalities such as anemia, neutropenia, and thrombocytopenia occur in children infected by the human immunodeficiency virus (HIV). These abnormalities are due to myelosuppression caused by the HIV and contribute to the morbidity and mortality of HIV.infected children. Malnutrition is prominent in HIV-infectedchildren due to associated conditions such as oropharyngeal candidiasis, diarrhea, and cytokine production which resultin poor intake, nutrient loss, and increased metabolic rate, respectively.Objectives: To determine the prevalence of hematological abnormalities (using the World Health Organization (WHO) case definitions) and malnutrition in HIV-infected children receiving care at the University of Nigeria Teaching Hospital, Enugu.Materials and Methods: The hematological and anthropometric indices of HIV.infected children between 18 and 59 months were assessed. Their hemoglobin level, neutrophil, and platelet counts were the hematological profiles evaluated using the WHO case definitions in HIV clinical staging. The weight-for-height z-score index was used to assess the nutritional status of subjects using the WHO reference ranges. The t-test, Chi-square, and Pearson correlation coefficient were used for statistical analysis.Results: There were 67 HIV positive children: 34 males and 33 females, aged 18-59 months. The mean hematological levels of subjects were hemoglobin (Hb) 10.4 ± 1.2 g/dl, neutrophil count 3,031 ± 1,039 cells/mm3, platelets count 294 ± 78 ~ 109/L. Two children (3.0%) had anemia (hemoglobin < 8 gm/dl) and were severely immunosuppressed,on highly active antiretroviral therapy treatment and had advanced HIV disease (clinical stage 3). Children who were malnourished were 15 (22.4%).Conclusion: Hematological abnormalities and malnutrition occur in HIV positive children.Key words: Haematological, malnutrition, anaemia, children

Background: Sickle cell anemia (SCA) is a genetic hematological disorder characterized by red blood cells that assume abnormal, rigid, and sickle shape. The musculoskeletal complications of SCA result from vessel occlusion, leading to tissue ischemia and infarction and progressive end organ damage. Aim: The aim of this study was to determine the pattern of musculoskeletal complications among children with SCA admitted at the University of Nigeria Teaching Hospital (UNTH) Ituku Ozalla. Subjects and Methods: This is a retrospective study of cases of musculoskeletal complications among SCA patients admitted in UNTH over a 58 month period. Data were analyzed using the SPSS version 20 and GraphPad Prism 5 softwares. Variables analyzed include musculoskeletal complications, age and gender distribution of subjects. Fisherfs exact was used to test for significant association of categorical variables while Mann Whitney U.test was used to compare the means. A P value of less than 0.05 was accepted as significant.Results: There were 300 registered patients in our sickle cell clinic. A total of 78 patients (52 males and 26 females) were admitted between January2007 and October 2012. The median age of the patients was 10 years (range: 9 months to 17 years). 25 of the 78 patients had musculoskeletal complications, giving a prevalence rate of 32.1%. The most common musculoskeletal complication was acute osteomyelitis, diagnosed in 10/78 (12.8%) of the patients. Conclusion: Acute osteomyelitis is the most common musculoskeletal complications in children with SCA attending UNTH Ituku Ozalla.Keywords: Children, Musculoskeletal complications, Sickle cell anemia

Objectives: This study aimed at determining the factors that influence the age at diagnosis of sickle cell anemia among children in Enugu, Nigeria. Methods: One hundred and fifty seven children with sickle cell anemia were recruited consecutively using a researcher administered questionnaire. Information obtained from patient’s mother included patient’s bio-data, social history, family history, age at confirmation of diagnosis and clinical information. Result: There was a wide variation of the age at diagnosis of sickle cell anemia ranging from 2 months to 14.7 years with a median age of 24 months. Only 10.4% were diagnosed before the age of six months and all were diagnosed after they have presented with symptoms and complications. The age of the mother and having an elder sibling with sickle cell anemia significantly affected the age at which the disease is diagnosed. Conclusion: There is a delay in the diagnosis of sickle cell anemia in Enugu with mother’s age and having an elder sibling with the disease influencing the age at diagnosis.

Background Hypertension is a prevalent cardiovascular disease risk factor among blacks and adolescent hypertension can progress into adulthood. Objective To determine the prevalence of hypertension and prehypertension among secondary school adolescents in Enugu South East Nigeria. Methodology A study of 2694 adolescents aged 10-18 years in Enugu metropolis was carried out. Socio-demographic profile anthropometric and blood pressure readings were obtained. Derived measurements such as Prehypertension, hypertension and BMI were obtained. Results The results showed that the mean systolic blood pressure and diastolic blood pressure for males were 106.66+ 11.80 mmHg and 70.25 + 7.34 mmHg respectively. The mean SBP and DBP for females were 109.83+ 11.66 mmHg and 72.23 + 8.26 mmHg respectively (p

Background: Hepatitis C virus (HCV) and Human Immunodeficiency virus (HIV), are major public health challenges in the developing world especially sub-Saharan Africa. The aim of this study was to determine the prevalence and risk factors of Hepatitis C virus infection among children infected with HIV.Methods: This was a cross-sectional study conducted at the Paediatric HIV Clinic, UNTH, Enugu between July and December 2009. Antibodies to HCV were analyzed by newer generation rapid chromatographic immunoassay method using the Chromatest one step HCV test kit. The data was analysed using the Statistical Package for Social Sciences (SPSS) version 15 statistical software. The chi squared test was used to test for significantassociation of categorical variables. A p-value of

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.

Background : Identifying impaired quality of life is a recognized component of asthma management with no published data in Nigerian children with asthma. The aim of this study was to describe the health-related quality of life of school-aged children with and without asthma seen at the Asthma Clinic of the University of Nigeria Teaching Hospital, Enugu. Methods : Cross-sectional hospital -based study of children aged 7- 17 years (with and without asthma) attending the Paediatric Asthma and General Children Outpatient Clinic of the University of Nigeria Teaching Hospital (UNTH) from parts of the south – east region of Nigeria were consecutively enrolled. Quality of life (QOL) scores were obtained using the Paediatric Quality of Life inventory (PedsQLTM) questionnaire which measures the core dimensions of health: physical functioning, emotional functioning, social functioning and school functioning. Results : There were a total of 180 study participants: (90 with asthma and 90 without asthma). Overall quality of life scores for children with asthma was worse than in those without asthma; 75.5, SD19.3 and 82.7, SD14.5 respectively (MD 7.1, CI = 2.3 to 12.3, p=0.01). Physical function domain was significantly more affected in asthmatics than nonasthmatics; 73.4, SD 23.2 vs. 84.4, SD17.3 respectively (MD 11.1, CI = 5.0 to 17.1, p=0.002). The psychosocial health summary scores in children with and without asthma were 77.6 SD 18.1 vs.81.1 SD15.1 (MD 3.5, CI= -1.4 to 8.4, p= 0.24), with the highest scores obtained in the social functioning domain for both asthma and nonasthma patients; 82.7, SD 20.3 and 87.6, SD 15.7(MD 4.9, p = 0.08) respectively. In both the overall and specific QOL domains, boys had higher scores than girls, irrespective of age or socioeconomic status with an inverse relationship between increasing age and QOL scores (r=-0.2, p=0.07). Conclusions : Children with asthma showed worse QOL and significant impairment in their physical functioning, more noticeable among the female study population. Information obtained from our QOL study forms a basis for a more informed management plan with regards to which age groups are more affected and the specific domains of health in children with asthma that need to be given closer attention to reduce asthma morbidity. The study emphasizes the need for QOL integration in asthma management for a more holistic approach to outcome evaluation of treatment rather than the physical outcomes.

Most people on folic acid to boost erythropoiesis and prophylactic antimicrobials, the standard management of steady state sickle cell disease (SCD), have unacceptable numbers of crises. The objective of this study was to evaluate the effects of adding multimodal therapy with potassium thiocyanate and omega-3 fatty acids to the standard management of steady state SCD. Pre- and post-treatment numbers of crises and other disease indices were compared in 16 HbSS individuals on folic acid and paludrine after 12 months of adding eicosapentaenoic acid 15 mg/kg/day, docosahexaenoic acid 10 mg/kg/day, and potassium thiocyanate 1-2 mL/day, each milliliter of which contained 250 mg of thiocyanate and 100 micrograms of iodine to prevent hypothyroidism: a possible side-effect due to competitive inhibition of the transport of iodide into the thyroid gland by thiocyanate. Median number of crises reduced from 3/yr to 1/yr (P < 0.0001). There was no evidence of impaired thyroid function. Plasma level of tri-iodothyronine improved (P < 0.0001). Steady state full blood count and bilirubin level did not change significantly. The findings suggest that addition of potassium thiocyanate and eicosapentaenoic and docosahexaenoic acids to standard management of steady state SCD reduces the number of crises. This observation needs to be evaluated in larger studies.

Background: Sickle cell anemia is associated with a hypercoagulable state that may lead to alterations in a coagulation profile. Measurements of coagulation factors are known to have some predictive value for clinical outcome.Objectives: To determine the coagulation profile of children with SCA in steady state and crisis and compare it with those with normal HbAA genotype.Materials and Methods: This is a prospective observational study involving 50 children with SCA in steady state, 50 in crises, and 50 with HbAA genotype, carried out from June to October 2009. All the values of coagulation profile were matched for age and sex.Results: The mean Prothrombin time (PT) (12.5 ± 1.2 secs), Activated partial thromboplastin time (aPTT) (41.6 ± 1.0 secs), and Thrombin time (TT) (12.3 ± 1.2 secs) of subjects with SCA in steady state as well as those during crises [PT (12.6 ± 1.8 secs), aPTT (45.6 ± 1.3 secs), TT (12.5 ± 1.7 secs)] were significantly prolonged compared to those of subjects with HbAA genotype. The mean bleeding time (BT) of 3.4 ± 1.0 mins was significantly shorter in children with SCA in steady state compared to those with HbAA genotype (3.7 ± 1.1 mins), (P < 0.038).Conclusion: Coagulation profile of patients with SCA is prolonged both in steady state and during crisis and when compared to those with HbAA genotype, though all values are within normal range.Key words: Children, coagulation profile, sickle cell anemia