Outcome of Emergency Blood Transfusion in Children Seen at a Tertiary Children's Hospital in Freetown: A Descriptive Cross-sectional Study.

OBJECTIVE: This study sought to describe the prevalence and clinical outcome after emergency blood transfusion among children presenting with severe anemia in a tertiary children's hospital in Sierra Leone. METHOD: This was a retrospective study of 395 children who received emergency blood transfusion at the Ola During Children's Hospital in Freetown. Association between mortality and sociodemographic and clinical factors was assessed using χ2 test. Statistical significance was set at P < 0.05. RESULTS: Three hundred ninety-five of 4719 children (8.4%) admitted to the emergency room of the Ola During Children's Hospital received emergency blood transfusion within 24 hours of presentation. Twenty-five (6.3%) were excluded because of incomplete data. The median age of the subjects was 24 months (interquartile range, 14-48). There were 191 boys (51.6%) and 179 girls (48.4%), giving a male/female ratio of 1.06:1. The most common indication for emergency transfusion was severe malaria anemia (67.8%). The mean hemoglobin concentration before transfusion was 4.8 g/dL (±1.4 g/dL). A total of 339 children (91.6%) were discharged home, 8 (2.2%) were discharged against medical advice, and 23 children (6.2%) died. The median length of stay on admission was 3 days (interquartile range, 2-5). Children with severe sepsis (P < 0.001) and those with pretransfusion hemoglobin concentration less than 5% (P = 0.047) were significantly more likely to die after transfusion compared with the other categories of patients. In a multivariate regression analysis, none of the factors were independently associated with clinical outcome after blood transfusion in children who received emergency blood transfusion. CONCLUSION: The study suggests that emergency blood transfusion in children with severe anemia may improve in-hospital survival.

An Assessment of Medication Errors Among Pediatric Patients in Three Hospitals in Freetown Sierra Leone: Findings and Implications for a Low-Income Country.

Background: Pediatric patients are prone to medicine-related problems like medication errors (MEs), which can potentially cause harm. Yet, this has not been studied in this population in Sierra Leone. Therefore, this study investigated the prevalence and nature of MEs, including potential drug-drug interactions (pDDIs), in pediatric patients. Methods: The study was conducted in three hospitals among pediatric patients in Freetown and consisted of two phases. Phase one was a cross-sectional retrospective review of prescriptions for completeness and accuracy based on the global accuracy score against standard prescription writing guidelines. Phase two was a point prevalence inpatient chart review of MEs categorized into prescription, administration, and dispensing errors and pDDIs. Data was analyzed using frequency, percentages, median, and interquartile range. Kruskal-Wallis H and Mann-Whitney U-tests were used to compare the prescription accuracy between the hospitals, with p<0.05 considered statistically significant. Results: Three hundred and sixty-six (366) pediatric prescriptions and 132 inpatient charts were reviewed in phases one and two of the study, respectively. In phase one, while no prescription attained the global accuracy score (GAS) gold standard of 100%, 106 (29.0%) achieved the 80-100% mark. The patient 63 (17.2%), treatment 228 (62.3%), and prescriber 33 (9.0%) identifiers achieved an overall GAS range of 80-100%. Although the total GAS was not statistically significant (p=0.065), the date (p=0.041), patient (p=<0.001), treatment (p=0.022), and prescriber (p=<0.001) identifiers were statistically significant across the different hospitals. For phase two, the prevalence of MEs was 74 (56.1%), while that of pDDIs was 54 (40.9%). There was a statistically positive correlation between the occurrence of pDDI and number of medicines prescribed (r=0.211, P=0.015). Conclusion: A Low GAS indicates poor compliance with prescription writing guidelines and high prescription errors. Medication errors were observed at each phase of the medication use cycle, while clinically significant pDDIs were also reported. Thus, there is a need for training on prescription writing guidelines and medication errors.

Designing a leadership and management training curriculum for undergraduate health professions students: Lessons from the University of Sierra Leone.

Leadership by health professionals is key in any health system, but health leadership training programmes are varied in their conceptualisation, learning objectives, and design. This paper describes an undergraduate leadership and management module for health students at the University of Sierra Leone and provides lessons from the design process. Our methods included an initial scoping review and qualitative study, followed by a co-design process of 10 workshops and 17 consultation meetings. The result was a curriculum with learning outcomes emphasising leadership identity, proactiveness, management of people and of change, and the formation of peer relationships. Learning methods included group teaching, team quality improvement projects, mentoring, and reflective practice. Lessons from the design process included the importance of support from university leadership and extensive consultation. Virtual workshops enabled broader participation but limited relationship building. Integrating doctoral research into the process facilitated inclusion of evidence and theory but risked reducing ownership by faculty. The importance of interprofessionalism and management skills in leadership training emerged during the process, illustrating the effectiveness of a co-design approach. Our programme is broadly aligned with other health leadership frameworks and is distinctive due to its undergraduate focus, offering insights for leadership training design in other settings.

Antibiotic use and consumption in Freetown, Sierra Leone: A baseline report of prescription stewardship in outpatient clinics of three tertiary hospitals.

Objective: As there are no country-representative data on bacterial sensitivities to guide antimicrobial stewardship (AMS) interventions, an AMS programme was established in the outpatient clinics of three tertiary hospitals in Freetown, Sierra Leone. Methods: The study employed a cross-sectional design to collect antibiotic prescribing data from 370 pregnant women and lactating mothers, 314 children and 229 regular patients in the outpatient clinics of the Princess Christian Maternity Hospital (PCMH), Ola During Children's Hospital and Connaught Hospital (CH), respectively, in April 2022. All data were analysed using Stata Version 16. Results: Of 913 patients, most were female (n=635, 69.5%), treated at PCMH (n=370, 40.5%) and had a bacterial infection (n=661, 72.4%). The indication for prescribing antibiotics was inappropriate in 252 (27.6%) patients. Of the 1236 prescriptions, 393 (31.8%) were made at CH. The duration of antibiotic use was not stated in 230 (18.6%) prescriptions. Overall antibiotic consumption was 55.3 defined daily doses per 1000 outpatient-days. Conclusion: Gaps in antibiotic prescriptions were identified in the outpatient clinics of three national referral hospitals in Sierra Leone. In order to combat antimicrobial resistance, AMS interventions are needed to reduce the prescription of antibiotics for inappropriate indications or without specified duration.

Making every death count: institutional mortality accuracy at Ola During Children's Hospital, Sierra Leone.

INTRODUCTION: health care data accuracy feeds the development of sound healthcare policy and the prioritisation of interventions in scarce resource environments. We designed a retrospective study at the sole paediatric government hospital in Sierra Leone to examine mortality statistics, specifically: the accuracy of mortality data collected in 2017; and the quality of cause of death (CoD) reporting for 2017. METHODS: the retrospective audit included all available mortality statistics collected at the hospital during the 2017 calendar year. For the purpose of calculating a mortality rate, admission data was additionally gathered. Four different hospital entities were identified that collected mortality data (the Monitoring and Evaluation (M&E) office; the nurse ledgers; the office of births and deaths; and the mortuary). Data from each hospital entity were used for the comparative analysis. RESULTS: striking differences were found in the rate of hospital mortality reported by different entities. The M&E office (responsible for providing data to the ministry of health and sanitation) reported a hospital mortality rate of 2.94% in 2017. Mortuary and nursing admissions records showed a hospital mortality rate of 18.7%. Discrepancies and issues of quality in CoD reporting between hospital entities were identified. CONCLUSION: significant variations were found in the generation of official hospital mortality data. Mortality data informs health service prioritisation, resource distribution, outcome measures and epidemiological surveillance. Resources to support quality improvement initiatives are needed in the creation of an in-hospital system that reports accurate data with a process for real-time institutional data feedback.

Acute hemorrhagic edema of infancy: report of 4 cases and review of the current literature.

Acute hemorrhagic edema of infancy (AHEI) is a cutaneous leukocytoclastic vasculitis that usually occurs in children younger than 2 years of age. It is a rare disease characterized by mild fever, a violent onset of hemorrhagic skin lesions, and edema usually followed by a spontaneous and complete recovery. Although the etiology is unknown, AHEI often follows infections, drug treatment, or vaccination. In the present report, the authors describe 4 cases of AHEI and review the relevant literature.

Vagus nerve stimulation improves severely impaired heart rate variability in a patient with Lennox-Gastaut-Syndrome.

Vagus nerve stimulation (VNS) is a new therapeutic option for refractory epilepsy. We report a patient with Lennox-Gastaut-Syndrome (LGS) and a severe impairment of heart rate variability (HRV), we could demonstrate in our patient that HRV was improved by VNS.

Valproic acid-induced pancreatitis: 16 new cases and a review of the literature.

BACKGROUND: Acute pancreatitis is rarely seen in children, and, in contrast to cases in adults, it is often drug induced. One possible medication is the antiepileptic drug valproic acid (VPA), which is commonly prescribed for generalized and focal epilepsy, migraine, neuropathic pain, and bipolar disorder. The common side effects associated with VPA are typically benign, but less common but more serious adverse effects may occur. These include hepatotoxicity, hyperammonemic encephalopathy, coagulation disorders, and pancreatitis. Since 1979, a few cases of pancreatitis induced by VPA have been published in the medical literature. METHODS: We mailed a questionnaire to all members of the "German Section of the International League against Epilepsy," asking about VPA-induced side effects. We also reviewed the medical literature for VPA-induced pancreatitis. RESULTS: Fifty-three publications (90 patients) published from 1979 to 2005 were found. Our survey in Germany, however, yielded 16 cases of pancreatitis from 1994 to 2003 whose original files we could study in detail. None of these patients had been published previously. CONCLUSIONS: The difference between 90 patients reported worldwide from 1979 to 2005 and the 16 new documented cases from only Germany over 10 years corroborates that the occurrence of this severe side effect is under reported.

Reversible hepatotoxicity, pancreatitis, coagulation disorder and simultaneous bone marrow suppression with valproate in a 2-year-old girl.

Valproic acid (VPA) is considered to be a drug of first choice for the therapy of generalized and focal epilepsies, including special epileptic syndromes like the WEST-syndrome. The drug is usually well tolerated; rare serious complications may occur in some patients, including haemorrhagic pancreatitis, coagulapathies, bone marrow suppression, VPA-induced hepatotoxicity and encephalopathy. We report a case of combined appearance of several severe VPA-associated side effects in a two- and a half-year-old girl with lissencephaly.

Oral rapid loading of valproic acid--an alternative to the usual saturation scheme?

Valproic acid (VPA) is considered to be a drug of first choice for the therapy of generalized and focal epilepsies. Due to its broad field of application and its good compatibility, VPA is one of the most frequently prescribed antiepileptic drugs (AED) worldwide. Previous studies have examined the safety and tolerability of rapid intravenous-loaded VPA in the treatment of epilepsy and status epilepticus, but rapid oral loading has not been evaluated in paediatrics systematically in the past. The standard titration scheme takes 10-14 days, some physicians prefer a slower titration of up to 4 weeks. At many institutes, especially children are treated as inpatients until the desired dosage is reached. This causes high costs to the health system and is very inconvenient for the families affected. We have developed a new loading scheme to achieve a therapeutic serum level on the third day of treatment, in order to minimize the time between the beginning of the therapy and reaching the therapeutic serum level. This is the first attempt at doing this with VPA for children with epilepsy.

Capillary microscopy and hemorheology in children during antiepileptic monotherapy with carbamazepine and valproate.

The interactions of epilepsy and antiepileptic therapy an one hand and cardiovascular system on the other hand are multiple and complex. Antiepileptic drugs (AEDs) cause alterations of serum lipids and of the fatty acid composition of the membranes. Homocystein, known to induce vascular endothelial damage was found to be elevated in patients on valproate (VPA) and carbamazepine (CBZ) therapy. Marked coronary artherosclerosis and myocardial infarction may already occur in children treated with CBZ. Community based studies corroborated a higher incidence of myocardial infarction, peripheral vascular diseases hypercholesterinemia, left ventricle hypertrophy and stroke in patients with epilepsy. In this context, we wanted to elevate changes of microcirculation related to AEDs commonly prescribed such as VPA and CBZ. Capillary microscopy is a non-invasive technique for measuring the velocity of red blood cells and for determining nutritional blood flow in the capillaries of the skin. It can easily be performed in children. The aim of this study was to look for possible effects an antiepileptic monotherapy with carbamazepine or valproate has on the peripheral microcirculation in epileptic children. We were able to examine 14 children with CBZ and 24 children with VPA, recruited in our neuropediatric Unit. The results were compared to normative values, determined in former analyses of 207 healthy children. We found significant differences in capillary density, tortuous index of the capillaries, capillary diameter and flow rate of erythrocytes for both antiepileptic drugs. Additionally, there were changes in plasma viscosity and the aggregation of erythrocytes. These microcapillary effects could be of special interest in the relationship of a long-term antiepileptic therapy and the development of vascular diseases. We suggest that the influence of AEDs on microcirculation should also be considered in further studies on cardiovascular changes in patients with antiepileptic long-term medication.

Oral valproic acid for epilepsy--long-term experience in therapy and side effects.

Valproic acid (VPA) is considered to be a drug of first choice and one of the most frequently-prescribed antiepileptic drugs worldwide for the therapy of generalized and focal epilepsies, including special epileptic. It is a broad-spectrum antiepileptic drug and is usually well tolerated. Rarely, serious complications may occur in some patients, including hemorrhagic pancreatitis, coagulopathies, bone marrow suppression, VPA-induced hepatotoxicity and encephalopathy, but there is still a lack of knowledge about the incidence and occurrence of these special side effects. Additionally, the consequences for VPA therapy and indication are more or less unclear. By literature review and own data this review addresses some of the challenges of VPA therapy and its side effects, which are not unique to epilepsy in childhood.

Valproate-associated reversible encephalopathy in a 3-year-old girl with Pallister-Killian syndrome.

Valproic acid (VPA) is considered to be a drug of first choice for the therapy of generalized and focal epilepsies, including special epileptic syndromes. The drug is usually well tolerated, rare serious complications may occur in some patients, including hemorrhagic pancreatitis, coagulapathies, bone marrow suppression, VPA-induced hepatotoxicity and encephalopathy. We report a case of VPA-associated encephalopathy without hyperammonemia in a 3-year-old girl with Pallister-Killian-Syndrom, combined with a mild hepatopathy and thrombopathy. After withdrawal of VPA, the clinical symptoms and the electroencephalography-alterations vanished rapidly.

Valproate-associated coagulopathies are frequent and variable in children.

PURPOSE: Valproic acid (VPA) is an antiepileptic drug (AED) commonly used for generalized and focal epilepsies. The clinical relevance of coagulopathies, known as side effects of VPA therapy, especially thrombocytopenia, von Willebrand disease, and a decrease of factor XIII, is still unclear. METHODS: In our institute, we noticed a high incidence of clinically relevant coagulation problems related to VPA in eight patients within 1 year only and a further seven children with significant coagulopathy were identified in the context of planned surgery. RESULTS: We provide an overview of these patients and all six VPA-induced coagulopathies. CONCLUSIONS: At this time, it cannot be recommended to control all hemostatic parameters in every patient. Whenever an increased bleeding tendency is observed, or before surgical procedures, a platelet count, thrombelastography, prothrombin time, activated partial thromboplastin time, TT, fibrinogen, von Willebrand factor, and factor XIII should be examined. With 385 VPA-treated patients per year and 15 cases of coagulation disorders in this period, we estimate the incidence of coagulation disorders related to VPA in children to be nearly 4%.

Tumor type M2 pyruvate kinase (TuM2-PK) as a novel plasma tumor marker in melanoma.

Proliferating cells express the pyruvate kinase isoenzyme type M2 (M2-PK). This enzyme exists as an active tetramer and an inactive dimer. The dimeric form is predominantly found in tumor cells and is therefore termed Tumor M2-PK (TuM2-PK). TuM2-PK molecules are released into the peripheral blood and may hereby function as a marker of tumor load in cancer patients. Our study was aimed to investigate TuM2-PK as a potential plasma marker in melanoma patients compared to the well-established serum marker S100beta. We measured the concentration of TuM2-PK in plasma and S100beta in corresponding serum samples from 300 melanoma patients and 53 healthy controls using a sandwich ELISA and an immunoluminometric assay, respectively. Plasma concentrations of TuM2-PK were significantly increased in melanoma patients compared to healthy controls (9.30 U/ml vs. 7.20 U/ml; p = 0.0036) and correlated with tumor load (p < 0.0005) and disease stage (p < 0.0005). Patients with elevated plasma TuM2-PK (cut-off = 15 U/ml) presented a reduced overall (p < 0.000005) and progression-free (p = 0.023) survival. Multivariate analysis revealed plasma TuM2-PK and serum S100beta as independent predictors of overall survival in metastasized patients. Neither plasma TuM2-PK nor serum S100beta showed prognostic relevance for tumor-free patients. Although the sensitivity and specificity to predict disease progression or death was higher for serum S100beta compared to plasma TuM2-PK, the combination of both markers improved the estimation of prognosis compared to that of serum S100beta alone.