Bloodless management of significantly elevated transcranial Doppler velocity value in a Jehovah's witness child with sickle cell disease: A tertiary centre experience-A case report.

BACKGROUND: Effective management of complications in sickle cell disease (SCD), such as stroke prevention, often necessitates the use of blood transfusions. However, individuals who adhere to the religious tenets of Jehovah's Witnesses strictly abstain from accepting blood transfusions, thereby presenting a formidable challenge in clinical decision-making. CASE REPORT: This is a case of a 3 year old child Jehovah's Witness who was found to have significantly elevated transcranial Doppler (TCD) velocity values between 193 and 203 cm/s, following routine screening. This was an otherwise clinically stable child, whose mother was diligently ensuring he had adequate medical care. Ideally, a prophylactic exchange blood transfusion program would have been commenced immediately but was not done due to due to the lack of consent from the caregiver. Patient was initially on hydroxyurea at 15 mg/kg and self medicating on omega 3 supplements and astymin syrup. Further elevation of TCD velocity upto 242 cm/s after a repeat testing, necessitated graduated increase of the dosage of hydroxyurea to 35 mg/kg to optimize its therapeutic effect, and discontinuation of omega 3 fatty acids and replacement of astymin with folic acid, vitamin C and B complex. Following these adjustments, the TCD dropped to below 190 cm/s reducing the risk of stroke in the child. CONCLUSION: This case report demonstrates the successful implementation of a bloodless management strategy for stroke prevention in a Jehovah's Witness child with SCD. This study contributes to the existing literature by providing valuable insights and practical guidance for healthcare providers facing similar ethical and medical dilemmas.

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.

Neutrophil-Lymphocyte, Platelet-Neutrophil, and Platelet-Lymphocyte Ratios as Indicators of Sickle Cell Anaemia Severity.

Background: Sickle cell anaemia (SCA) imposes a substantial healthcare burden, affecting millions of people worldwide. Understanding the determinants influencing SCA severity is crucial for enhanced disease management and optimized patient outcomes. This study aimed to investigate the relationship between Neutrophil-Lymphocyte Ratio (NLR), Platelet-Neutrophil Ratio (PNR), Platelet-Lymphocyte Ratio (PLR), and SCA severity. Methods: A cohort of 45 children diagnosed with SCA and undergoing treatment at Chukwuemeka Odumegwu Ojukwu University Teaching Hospital, Awka, was included in this study. Demographic and clinical data, along with laboratory measurements of the aforementioned ratios, were collected. The severity of SCA was assessed using numerical scoring. Results: The analysis revealed that PNR and PLR emerged as significant predictors of SCA severity, irrespective of the level of adiposity. In contrast, NLR demonstrated no predictive value in relation to SCA severity. Conclusion: The findings challenge the conventional notion that neutrophils alone play a central role in the pathogenesis of sickle cell crises. These results contribute to a deeper understanding of the disease and provide insights into possible alternative mechanisms underlying SCA severity. Further research is warranted to explore the intricate interplay between platelets, neutrophils, lymphocytes, and other biological factors within the context of SCA. Ultimately, this knowledge may pave the way for targeted interventions and improved management strategies for individuals living with SCA.

Prevalence of venous thromboembolism and its associations in a large racially homogenous population of sickle cell disease patients.

To determine the prevalence of venous thromboembolism (VTE) among adult sickle cell disease (SCD) patients in Nigeria. METHODS: This was a multicentre retrospective study in which the medical records of adult SCD patients were reviewed. Information on demographics, steady-state haemogram, clinical phenotypes, duration of follow-up, history of VTE including risk factors and management was collected. RESULTS: Of the 509 SCD patients with a median (IQR) duration of follow-up of 2 years, 10 (2.0%) had VTE (9 DVT and 1 PE). Their median (IQR) age was 27 (22.8-30.3) years. Identifiable risk factors for VTE included positive family history (2, 20%) surgery, splenectomy, paraplegia and cancer (1, 10% each). No risk factor was identifiable in four persons. VTE had no significant association with age and gender. VTE was significantly associated with the following events: acute chest syndrome [p = .002, odds ratio (OR) 8, 95% CI 2.2-28.9], osteonecrosis [p = .012, OR 5.24, 95% CI, 1.45-18.91] and vaso-occlusive crisis [p = .035]. Also significantly associated with VTE were pulmonary hypertension [p = .001, OR 23.3, 95%CI 5.18-105.06] and stroke [p = .032, OR 9.35, 95%CI 0.87-53.25]. CONCLUSION: The prevalence of VTE among SCD patients in Nigeria is low. It is significantly associated with vaso-occlusive crisis, pulmonary hypertension and stroke.

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.

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.

Hyperhaemolytic Syndrome in Sickle Cell Disease: Clearing the Cobwebs.

Sickle cell disease (SCD) presents with a dynamic background of haemolysis and deepening anaemia. This increases the demand for transfusion if any additional strain on haemopoiesis is encountered due to any other physiological or pathological causes. Patients with cerebrovascular accidents are placed on chronic blood transfusion; those with acute sequestration and acute chest syndrome are likewise managed with blood transfusion. These patients are prone to develop complications of blood transfusion including alloimmunization and hyperhaemolytic syndrome (HHS). This term is used to describe haemolysis of both transfused and "own" red cells occurring during or post-transfusion in sickle cell patients. Hyperhaemolysis results in worsening post-transfusion haemoglobin due attendant haemolysis of both transfused and autologous red cells. The mechanism underlying this rare and usually fatal complication of SCD has been thought to be secondary to changes in the red cell membrane with associated immunological reactions against exposed cell membrane phospholipids. The predisposition to HHS in sickle cell is also varied and the search for a prediction pattern or value has been evasive. This review discusses the pathogenesis, risk factors and treatment of HHS, elaborating on what is known of this rare condition.

The Perception of People Living with Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome toward Human Immunodeficiency Virus Infection: A Single-Center Experience.

BACKGROUND: The prevention and control of human immunodeficiency virus (HIV) infection depend on the prevention of new infections as well as treating currently infected individuals. Adequate knowledge of HIV infection among person living with HIV/acquired immunodeficiency syndrome (AIDS) (PLWHA) may be an important tool in reducing spread of the virus. OBJECTIVE: The objective of the study was to evaluate knowledge and attitude of PLWHA on HIV infection. METHODOLOGY: This was a cross-sectional study conducted at the Chukwuemeka Odumegwu Ojukwu Teaching Hospital. Knowledge of infection, spread, control, and effect was sought from HIV-positive respondents using a structured questionnaire. Information about their attitude and beliefs was also obtained. Collected data were analyzed using the Statistical Package for Social Sciences for Windows, Version 21.0. RESULTS: A total of 70 HIV-positive patients, including 23 (32.9%) males and 47 (67.1%) females with a mean age of 37.7 years were participated. The overall knowledge on HIV transmission, clinical effects, complications, and controls was good in 15.7%, average in 72.9%, and poor in 11.4%. Knowledge of means of transmission was appropriate in majority of them. Majority of 66 (94.3%) patients showed a positive attitude to life. CONCLUSION: Most of the HIV-positive patients had average knowledge on HIV, and majority had a positive attitude to life.

Correlates of neutrophil/lymphocyte, platelet/lymphocyte, and platelet/neutrophil ratios of neonates of women with hypertensive disease of pregnancy with neonatal birth outcomes.

BACKGROUND: Studies have indicated neutrophil/lymphocyte ratio (NLR), Platelet/Lymphocyte ratio (PLR), and platelet/neutrophils ratio (PNR) to be inflammatory markers. The correlation of these values in infants of hypertensive mothers has not been investigated. AIM: To investigate the relationship between NLR, PLR, and PNR of neonates of women with hypertensive disease of pregnancy and neonatal APGAR scores and birth weight. METHODOLOGY: Cord blood samples of 200 neonates collected and the blood counts and ratios obtained. RESULTS: NLR in the babies of the hypertensive mothers was 0.865 and 1.42 in the control group (p = 0.0001). PLR was 34.7 in the neonates of the hypertensive mothers and 62.4 in the control group (p = 0.0001). PNR did not differ significantly between the two groups,p = 0.418. Degree of hypertension had a direct relationship with NLR; SBP had a p value of 0.001 while the DBP had p = 0.002. The PLR had an inverse relationship with the degree of hypertension; SBP p value of 0.0001, while DBP was p = 0.0001. No significant association was observed between the ratios and neonatal birth weight (p ≥ 0.05); however, PNR and PLR were found to be significantly associated with the 1st (p = 0.045 and 0.030) and 5th (0.049 and 0.037) minute APGAR scores in the newborns. CONCLUSION: PLR and NLR in neonates of hypertensive mothers are found to be markedly lower than those of controls, the degree of which is affected by the severity of hypertension. Also, lower PLR is associated with lower APGAR scores. Therefore, severity of high blood pressure and lower PLR may be determinants of poor birth outcome.

Impact of parity and gestational age of mothers with hypertension on birth weight, red blood cells and mode of delivery of their babies.

BACKGROUND: Maternal factors are determinants of birth outcome which includes birth weight, haematological indices and mode of delivery of their babies. OBJECTIVES: To determine the impact of parity and gestational age of hypertensive mothers on some neonatal variables. METHODS: A hospital based cross-sectional study of measurement of neonatal variables (birth weight, red blood cells and mode of delivery) among hypertensive mothers and their controls was conducted over a period of six months. Data were analyzed using the Statistical Package for Social Sciences program (SPSS), version 20. RESULTS: There were statistically significant differences in means between the neonates of the hypertensive group and non-hypertensive group for maternal age (t =1.61, p = 0.002), baby weight (t =2.87, p < 0.001), haemoglobin (Hb) (t =4.65, p = 0.010) and packed cell volume (PCV) (t =4.75, p = 0.009), but none for gravidity (t =1.95, p = 0.927)For all subjects, there was poor correlation between gestational age and variables; birth weight, haemoglobin (Hb), packed cell volume (PCV), nucleated red blood cell (nRBC) and parity. Likewise, parity poorly correlated with variables; age, birth weight, Hb, PCV, and nRBC. There was a statistically significant association between mode of delivery and hypertension (χ2 =53.082, p <0.001) but none with having a family history of hypertension (χ2 =1.13, p = 0.287). CONCLUSION: Parity and gestational age of mothers with hypertension have no impact on birth weight and red cells when compared with their non-hypertensive counterparts. However, mothers of babies delivered by elective and emergency caesarean section were about 2-3 times more likely to be hypertensive than those that delivered through spontaneous vertex delivery.

Disclosure amongst adult HIV patients on antiretroviral therapy in Port Harcourt, Nigeria.

HIV transmission is still a public health concern in sub-Saharan Africa; disclosure is an effective tool for its prevention, contact tracing and treatment. We aimed to evaluate the disclosure behaviours of adult HIV-positive patients receiving antiretroviral therapy (ART) in University of Port Harcourt Teaching Hospital, and identify major challenges to disclosure in a bid to develop ways to improve this practice in the environment. Patients receiving ART in this centre were interviewed using an interviewer-administered questionnaire. A total of 250 clients were interviewed over three months. A majority of the patients were tested on account of ill health 143 (57.2%). They commenced ART within 8 ± 15.4 SD months of presentation. The mean period before disclosure was 4.75 ± 12.8 SD months of diagnosis. Thirty-six (14.4%) of the respondents had not disclosed their HIV status; the major barrier to disclosure was stigmatisation in 19 (36%).