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.

Congenital cerebral malaria: a masquerader in a neonate.

BACKGROUND: Congenital malaria, which is caused by vertical transmission of malaria parasites, is a potentially fatal condition. Despite Africa's high malaria burden, congenital malaria is not routinely screened for, and thus may go undiagnosed. Malaria, if not treated promptly, can quickly progress to severe forms and result in death. Severe congenital malaria is believed to be uncommon in neonates due to maternal antibodies, fetal haemoglobin, and the placenta's sieving effect. The majority of reported cases were classified as having severe anaemia. Following a thorough review of the literature, only one case of congenital cerebral malaria (CCM) has been reported, and it was misdiagnosed. CASE PRESENTATION: A 5-day-old Nigerian neonate born to an apparently healthy mother initially displayed characteristics consistent with neonatal sepsis and severe neonatal hyperbilirubinaemia. He quickly developed characteristics consistent with meningitis. Surprisingly, the peripheral blood film revealed evidence of malaria parasites, which was immediately confirmed by Giemsa-stained thick and thin blood film microscopy for malaria. The patient was diagnosed with congenital cerebral malaria. The medication was modified to parenteral artesunate followed by oral artemisinin combination therapy. The neonate recovered fully and had no neurological deficits on follow up. CONCLUSION: Because CCM and infant meningitis have similar clinical presentations, CCM could be misdiagnosed and lead to death if there isn't a high index of suspicion.

Compendium of Oral Pathologies in Children Presenting to A Tertiary Hospital in Nigeria

Background: Oral diseases are one of the most common non-communicable diseases. They are also the most neglected particularly in children. Objective: To determine the pattern and trend of oral diseases among children who presented in the dental clinic of a Nigerian Tertiary hospital over 58 months. Methodology: A retrospective study that assessed for oral diseases among children using the dental health records. Results: 1104 cases presented at the dental clinic, comprising 546 males and 558 females with a ratio of 1:1.02. The mean age of the subjects was 10.6 years ± 4.2 with an age range of 1 month to 17 years. Close to ninety-eight percent of cases reviewed were symptomatic. Across all age groups, dental caries and its sequalae was observed in 62.2% of cases and this was independent of age group and year of presentation (p<0.0001). Conclusion: An increasing trend of oral diseases was observed annually with the leading diagnosis been dental caries and its sequalae