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

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

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

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

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.

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

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

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

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

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

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

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.

Addition of multimodal therapy to standard management of steady state sickle cell disease.

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.