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.

Adiponectin and Disease Severity in Sickle Cell Anemia Patients Attending a Tertiary Health Institution in Nnewi, Southeast Nigeria.

Background: Hemoglobin polymerization in sickle cell anemia (SCA) leads to abnormally rigid and adhesive erythrocytes that obstruct blood vessels, leading to poor tissue perfusion, hence provoking inflammation and damage of surrounding tissues. Adiponectin, a protein hormone, presumptively has anti-inflammatory characteristics, hence may be an important therapeutic target in SCA. Aim: The aim of the study was to evaluate the status of adiponectin and its correlation with disease severity in SCA. Patients and Methods: A total of 84 subjects were recruited for the study comprising 34 homozygous sickle cell (HbSS) subjects (25 in the steady state and nine in the resolving crisis state) and 50 controls (25 heterozygous sickle cell [HbAS] and 25 hemoglobin phenotype AA subjects). The hemoglobin phenotype, adiponectin levels, and full blood counts were evaluated. Anthropometric measurements were also conducted. Results: A significant difference was observed in the mean body mass index between the different hemoglobin phenotype groups and also between the SCA in crisis resolution patients and the control group (p < 0.05). There was no significant difference in the median serum levels of adiponectin in the different hemoglobin phenotype groups and between SCA patients in the steady state compared with those in the crisis resolution state. Also, there was no correlation between disease severity and adiponectin in SCA patients in the steady state (p = 0.87). Conclusion: Our study seems to suggest that in our data set of sickle cell anemia patients in the steady state, adiponectin does not constitute part of the endocrinopathy that affects these patients.

Utilization of Pneumococcal Vaccine and Penicillin Prophylaxis in Sickle Cell Disease in Three African Countries: Assessment among Healthcare Providers in SickleInAfrica.

Sickle cell disease is a genetic disease with a predisposition to infections caused by encapsulated organisms, especially Streptococcus pneumoniae. Pneumococcal vaccines and prophylactic penicillin have reduced the rate of this infection and mortality in sickle cell disease. However, implementation of these interventions is limited in Africa. The objectives of the study were to assess health care providers' behaviors with the implementation of pneumococcal vaccination and penicillin prophylaxis and to identify barriers to their use. A 25-item online questionnaire was administered through SickleinAfrica: a network of researchers, and healthcare providers, in Ghana, Nigeria, and Tanzania, working to improve health outcomes of sickle cell disease in Africa. Data was collected and managed using the Research Electronic Data Capture (REDCap), tools and data analysis was done using STATA version 13 and R statistical software. Eighty-two medical practitioners responded to the questionnaire. Only 54.0 and 48.7% of respondents indicated the availability of published guidelines on sickle cell disease management and pneumococcal vaccine use, respectively, at their facilities. The majority (54.0%) perceived that the vaccines are effective but over 20.0% were uncertain of their usefulness. All respondents from Ghana and Tanzania affirmed the availability of guidelines for penicillin prophylaxis in contrast to 44.1% in Nigeria. Eighty-five percent of respondents affirmed the need for penicillin prophylaxis but 15.0% had a contrary opinion for reasons including the rarity of isolation of Streptococcus pneumoniae in African studies, and therefore, the uncertainty of its benefit. Lack of published guidelines on the management of sickle cell disease and doubts about the necessity of prophylactic measures are potential barriers to the implementation of effective interventions.

comparative study of carbohydrate antigen 19-9 in sickle cell disease subjects and controls in Nnamdi Azikiwe University Teaching Hospital, Nnewi, Nigeria.

BACKGROUND: Sickle cell disease is characterized by chronic complications that affect almost all body organs. Pancreatic disease is rare in SCD. CA 19-9 is a non-specific surrogate marker for pancreatic disease especially carcinoma. CA 19-9 levels have not been evaluated in SCD patients in our environment. OBJECTIVES: The study aimed to compare the levels of CA 19-9 in homozygous sickle cell disease subjects in steady state with those of (Hb AS) and normal healthy subjects (Hb AA). METHOD: Seventy nine subjects including 39 Hb SS, 19 Hb AS and 21 Hb AA subjects were recruited in a cross-sectional study in Nnamdi Azikiwe University and Teaching Hospital. Haemoglobin genotype and CA 19-9 estimation were done using Hb electrophoresis and enzyme linked immunosorbent assay respectively. Data was analyzed with IBM SPSS 21. P value was set at 0.05. RESULT: The mean CA 19-9 (U/ml) level in Hb SS, Hb AS and Hb AA were 13.6 ± 7.6, 15.3 ± 9.9, and 20.0 ± 15.9 respectively. [Reference value <37U/ml] CA 19-9 was significantly lower in Hb SS compared to Hb AA subjects (p = 0.035). CONCLUSION: Low levels of CA 19-9 in Hb SS may suggest reduced pancreatic disorders in SCA.

Knowledge and attitude of donating and using cord blood for transfusion among patients attending Nnamdi Azikiwe University Teaching Hospital, Nnewi, South East Nigeria.

BACKGROUND: Allogeneic blood for transfusion is in short supply in most parts of the developing world. Cord blood for transfusion can be a significant source of blood supply to our health institutions. AIMS: This study aims to investigate the knowledge and attitude to the donation and use of umbilical cord blood (UCB) for transfusion among the patients receiving services in a tertiary health institution in South-East Nigeria. MATERIALS AND METHODS: This was a cross-sectional study; an anonymous structured questionnaire was used. A total of 549 consenting patients randomly selected from the antenatal, postnatal, sickle cell clinics, and wards were the subjects. Statistical analysis of the data was done using SPSS version 20.0. RESULTS: The mean age of the participants was 31.9 ± 9.5 years. The majority were females (77.2%), married (86.4%). About 26.2% of the respondents were willing to accept UCB for transfusion to them or their child. Following counseling, the acceptance rate increased to 71.5%. Most of the respondents (80.0%) were willing to donate the UCB of their baby; or be tested for HIV (93.3%), if necessary. Educational level was significantly associated with knowledge of UCB. After logistic regression, occupation, and gender were significantly associated with acceptance of UCB for transfusion. Up to 52% belonged to low income family background, approximately 150 US dollars monthly family income (50,000 naira). CONCLUSION: The knowledge and acceptance of UCB for transfusion are low in our environment. However, proper counseling and public enlightenment could change this attitude.

Blood transfusion safety; current status and challenges in Nigeria.

The attainment of blood transfusion safety in Nigeria (and probably the rest of Sub-Saharan Africa) remains an uphill task due to a number of factors, ranging from shortage of blood, poor implementation of blood transfusion guidelines, infrastructural deficits to high prevalence of transfusion-transmissible infections (TTIs), particularly hepatitis and human immune deficiency viruses. We reviewed available data on blood transfusion practices and safety in Nigeria using the PubMed, PubMed Central, Google Scholar, and African Index Medicus search engines, through a combination of word and phrases relevant to the subject. The World Health Organization has been in the forefront of efforts to establish safe, available, and affordable blood transfusion services in most parts of Africa through encouraging adequate blood donor recruitment, donor blood testing, and collection as well developing strategies for the rational use of blood. Even though modest improvement has been recorded, particularly with regards to donor blood screening for common TTIs, considerable efforts are needed in the form of robust public enlightenment campaigns (on blood donation) and continuous system improvement to drive the current transfusion practices in the country toward safety and self-sustenance.

Normal pregnancy and coagulation profile: from the first through the third trimester.

BACKGROUND: Normal pregnancy is a hypercoagulable state; a physiological safety valve aimed at preventing excessive maternal blood loss at delivery. OBJECTIVE: To evaluate the influence of normal pregnancy on blood coagulation and to explore changes in activity from the first through the third trimester. SUBJECT AND METHODS: Sixty (60) apparently healthy pregnant women (20 from each trimester) and 20 healthy non-pregnant age-matched controls were recruited. Each participant had Prothrombin time (PT). Activated partial thromboplastin time (APTT) and platelet count done. Multiple comparisons were made between control values and coagulation profile at different stages of pregnancy using the Bonferroni statistics. Results were expressed as means and standard deviations, p < 0.01 was significant at 95 % CI. Ethical approval for the study was obtained from the Institutional review board. RESULTS: The means of the APTT were significantly lower in the first, second and third trimesters compared with controls (35.59 ± 4.95 seconds, 32.22 ± 5.79 seconds and 29.60 ± 3.66 seconds, respectively, vs. 40.55 ± 5.95 seconds; p = 0.01). Correspondingly, the platelet count was significantly lower in the 3 trimester of pregnancy compared with controls (178.35 ± 41.52 x 10(9)/L vs. 233.86 ± 55.34 x 10(9)/L; p < 0.01) and equally with level in the 2nd trimester (178.35 ± 41.52 x 10(9)/L vs. 232.10 ± 48.67 x 10(9)/L; p < 0.01). CONCLUSION: The APTT and platelet counts are significantly lower in the 3 trimester of normal pregnancy.

Evaluation of serum ferritin levels and blood counts in apparently normal individuals in Nnewi, South-east Nigeria.

INTRODUCTION: Serum ferritin levels as well as blood counts are variously affected in diverse disease conditions, particularly those characterized by inflammation. OBJECTIVE: To evaluate the relationship between serum ferritin levels and blood counts in apparently healthy Nigerian subjects. SUBJECTS AND METHODS: Five (5ml) of venous blood was collected for full blood count, malaria parasite, serum ferritin and C- reactive protein (CRP) estimation from 88 apparently healthy subjects. Those positive for malaria parasite (on blood film examination) or had elevated serum CRP were excluded. Statistical analysis was performed with SPSS version 16; (SPSS Inc., Chicago IL). Associations were tested between parameters using Pearson's correlation, set at a coefficient of p < 0.05. Ethical approval was obtained from the Institutional Review Board and all participants gave informed consent. RESULTS: The median, mean and modal ages of subjects were 23.50 years, 25.25 years and 16.00 years, respectively while the mean packed cell volume (PCV), total white blood cell (TWBC), platelet count and serum ferritin level were 0.37L/L, 7.10 x 10(9)/L, 246.39 x 10(9)/L and 18.35 ng/ml, respectively. Age of subjects varied significantly with serum ferritin, platelet count and TWBC (p > 0.05). The PCV, platelet count and TWBC varied significantly across the different age groups of the study population (p < 0.05), likewise the PCV in males, compared to females (p < 0.05). CONCLUSION: Serum ferritin level in our apparently healthy subjects is lower than previously reported but did not vary significantly with blood counts.

HEMATOLOGICAL INDICES IN AUTOMOTIVE TECHNICIANS IN NNEWI, SOUTH-EAST NIGERIA.

BACKGROUND: Occupational exposure to petroleum products is known to be associated with a number of health risks as well as adverse effects on haematological indices. OBJECTIVE: To evaluate the effect of exposure to petroleum products on haematological parameters in automobile mechanics in Nnewi, south-east Nigeria. SUBJECTS AND METHODS: Fifty (50) automobile technicians and 50 healthy controls participated in the study. Full blood count was performed for each participant using the automated Sysmex haemocytometer (Model PCE 210) while data analysis was done using the SPSS (statistical package for social sciences) version 16 computer soft ware (Chicago, IL, Inc.). Results were expressed as frequencies, means and standard deviations; comparison of haematological parameters was made between subjects and controls and at different durations of exposure using the students' t-test and analysis of variance (ANOVA), respectively. The level of statistical significance was p < 0.05 (at 95% CI). Ethical approval was obtained from the Institutional review board and all participants gave informed consent. RESULTS: There red blood cell count (RBC) and haematocrit were significantly lower while the platelet count was significantly higher in test subjects compared with controls (p values; 0.04, 0.03 and 0.01, respectively). Correspondingly, the mean cell volume (MCV) and mean cell haemoglobin (MCH) were significantly lower in test subjects compared with controls (p = 0.62 and 0.03, respectively). The red cell count, MCV and platelet count significantly decreased with increasing duration of work exposure (p = 0.001, respectively). CONCLUSION: Occupational exposure to petroleum products adversely affects blood counts, with a significant decrease in RBC, MCV and platelet count as duration of exposure increases.