Patient Experience With Efanesoctocog Alfa for Severe Hemophilia A: Results From the XTEND-1 Phase 3 Clinical Study Exit Interviews.

Purpose: Hemophilia A is a rare bleeding disorder that leads to recurrent hemarthrosis, which can ultimately result in reduced mobility and poor quality of life. Qualitative exit interviews provide insights into patient perspectives and support the interpretation of quantitative trial data, such as patient-reported outcome measures. In the Phase 3 XTEND-1 study (NCT04161495) of efanesoctocog alfa in participants with severe hemophilia A, exit interviews were conducted to understand pre- and post-study experiences with pain and physical functioning and to evaluate participants' treatment experiences.

Comprehensive genomic filtering algorithm to expose the cause of skewed X chromosome inactivation. The proof of concept in female haemophilia expression.

Background: Exploring the expression of X linked disorders like haemophilia A (HA) in females involves understanding the balance achieved through X chromosome inactivation (XCI). Skewed XCI (SXCI) may be involved in symptomatic HA carriers. We aimed to develop an approach for dissecting the specific cause of SXCI and verify its value in HA.

Evidence-based management of adult traumatic brain injury with raised intracranial pressure in intensive critical care unit at resource-limited settings: a literature review.

Background: In underdeveloped countries, there is a greater incidence of mortality and morbidity arising from trauma, with traumatic brain injury (TBI) accounting for 50% of all trauma-related deaths. The occurrence of elevated intracranial pressure (ICP), which is a common pathophysiological phenomenon in cases of TBI, acts as a contributing factor to unfavorable outcomes. The aim of this systematic review is to analyze the existing literature regarding the management of adult TBI with raised ICP in an intensive critical care unit, despite limited resources.

The effect of preoperative anemia on perioperative outcomes among patients undergoing emergency surgery: A multicenter prospective cohort study.

Introduction: Preoperative anemia is a common finding among surgical patients. It is associated with an increased risk of perioperative morbidity and mortality. Outcomes among emergency surgical patients are not established.

The hidden joint in children with haemophilia on prophylaxis.

Unlabelled: Prophylaxis is the gold standard treatment for children with haemophilia (CWH). MRI studies revealed joint damage, even with this treatment; this suggests the presence of subclinical bleeding. In the case of children with haemophilia, it is relevant to detect early signs of joint damage, as this allows the medical team to provide the appropriate treatment and follow-up, in order to avoid arthropathy development and its consequences.

Haemophilia B, severe childhood obesity and other extra-haematological features associated with similar 4Mb-deletions on Xq27: Clinical findings, molecular insights and literature update.

Introduction: Haemophilia B (HB) is associated with pathogenic variants in F9. Hemizygous deletions encompassing the entire F9 and proximate genes may express extra-haematological clinical phenotypes.

Aim: To analyse the genotype/phenotype correlations in two unrelated boys with severe early childhood obesity (SCO), global developmental delay (GDD) and similar bleeding phenotype associated with comparable Xq27 deletions spanning the entire F9 and proximate genes, and characterise the pathogenic events estimating the most likely mutational mechanism involved.

How mild is mild haemophilia?

Introduction: People with mild haemophilia (PWMH) experience sporadic bleeds and are less likely to receive an early diagnosis, appropriate treatment and medical care. Arthropathy is a key determinant of health-related quality of life (QoL), producing pain, limitations in mobility and daily activities. The aim of this study is to evaluate the incidence, risk factors and QoL associated with arthropathy in PWMH.

Determinants of postoperative emergence delirium in patient undergoing general anesthesia in Dilla University referral hospital. A case-control study.

Background: Delirium is affecting of concentration, decreases the ability to forward-thinking, attention, sustainability, change, and decreases orientation to the environment. Delirium has a serious impact on the overall outcome of the patient. Post-operative emergence delirium (POED) increases hospital mortality by 5% and post-discharge hospitalization by 33%, compared to those without postoperative delirium.

Evaluation of Essential and Emergency Surgery Provide in Primary Hospitals of Gedeo Zone and Sidama Region, South, Ethiopia, 2020.

Background: Surgical services at level referral hospitals were an essential part of overall health care. The surgical uhservice was approximated to account for 11% of the worldwide load of disease, with a large percentage of that burden being uncovered in resource-constraint settings. Even though the surgery service is significant and growing across all economic sectors, the majority of resource-limited countries have been unable to provide essential surgical services.

Determinants of neonatal mortality in the neonatal intensive care unit of Dilla University Referral Hospital, Southern Ethiopia; 2019-2020; A matched, case-control study.

Background: Neonatal mortality rate (NMR) refers to the number of deaths occurring from birth to 28 days of life per-1000 Live Births (LB). The global NMR declined from 37 deaths per- 1,000 LB in 1990 to 18 in 2017, whereas it was 27 deaths per 1000 LB in the Sub-Saharan region. Ethiopia plans to reduce the NMR from 28 deaths to 11 deaths per 1,000 LB by 2020 and to end all preventable child deaths by 2035.

Effect of anesthesia choice on hemodynamic stability and fetomaternal outcome of the preeclamptic patient undergoing cesarean section.

Background: The main aim of this study is to compare hemodynamic stability and feto-maternal outcome between general and spinal anesthesia in pre-eclampsia patients undergoing C/S.

Methods: A prospective cohort study was used with a calculated sample size of 266. Comparison of numerical variables between study groups was done using unpaired student t-test and Manny Whitney U test for symmetric and asymmetric data respectively.

The impact of novel coronavirus disease (COVID-19) on emergency and essential surgical care in Gedeo and Sidama zone hospitals: An institutional-based multicenter cross-sectional study.

Background: COVID-19 was initially detected in China's Wuhan, the capital of Hubei Province, in December 2019, and has since spread throughout the world, including Ethiopia. Long-term epidemics will overwhelm the capacity of hospitals and the health system as a whole, with dire consequences for the developing world's damaged health systems. Focusing on COVID-19-related activities while continuing to provide essential services such as emergency and essential surgical care is critical not only to maintaining public trust in the health system but also to reducing morbidity and mortality from other illnesses.

Evidence-Based Guideline on Management of Postoperative Delirium in Older People for Low Resource Setting: Systematic Review Article.

Background: Postoperative delirium is the highest prevalence and life-threatening complication following geriatric surgery. The overall incidence rate varies from 5% to 52% of hospitalized surgical patients based on the type of surgery that often began in the postanesthesia care unit and continues up to 5 days post-surgery. Postoperative delirium manifests as a hypoactive, hyperactive and mixed subtype.

A multicentre real-world data study to assess the bleeding rate and management of patients with haemophilia A and factor VIII inhibitors in Argentina.

The development of inhibitors against factor VIII (FVIII) concentrates is a severe complication of treatment for patients with haemophilia. We investigated annualized bleeding rates (ABRs) in patients in Argentina with haemophilia A with inhibitors and analysed potential differences between treatment strategies. This multicentre, retrospective, real-world data, cohort design study comprised ambulatory paediatric and adult patients with congenital haemophilia A and FVIII inhibitors treated according to standard clinical practice, with 12-months follow-up.

Knowledge, attitude, and practice of healthcare workers toward COVID-19 and its prevention in Ethiopia: A multicenter study.

Background: An emerging respiratory disease abbreviated as coronavirus disease 2019 was first reported in December 2019 in Wuhan city of China. The virus is zoonotic and tends to be transmitted between animals to humans and humans to humans. The major route of transmission of coronavirus disease 2019 is droplet and close contact.

Comparison of thiopentone with lidocaine spray vs propofol for laryngeal mask airway insertion at tikur anbessa specialized hospital. A prospective cohort study.

Background: Insertion of laryngeal mask airway (LMA) requires an adequate depth of anesthesia. Optimal insertion conditions and hemodynamic stability during LMA insertion are mainly influenced by the choice of the intravenous induction agent. Propofol was recommended as a standard induction agent for LMA insertion.

Clinical and ultrasound evaluation of patients with haemophilia on prophylaxis.

Introduction: Primary prophylaxis is the current gold standard in haemophilia care for the prevention of bleeding and ensuing joint damage. Early detection of joint bleeding, whether symptomatic or subclinical, preferably during childhood, helps prevent joint deterioration and subsequent disability. The aim of this study is to evaluate the level of agreement between the Haemophilia Joint Health Score and the Haemophilia Early Arthropathy Detection with Ultrasound tools in children with severe haemophilia on primary and secondary prophylaxis.

Magnitude and Associated Factors for Attitude and Practice Toward COVID-19 and Its Prevention Among the Residents of Gedeo Zone, Southern Ethiopia: A Community-Based Cross-Sectional Study.

Background: COVID-19 first broke out in China, Hubei province on December 29, 2019, since then it took hundreds of thousand lives worldwide. Although all countries are taking varying degrees of measures to curb the dissemination, the virus is still spreading rapidly in all parts of the world. Adherence of people for COVID-19 precaution measures is one of the most important factors which determine the effectiveness of curbing the spread.

Evidence-Based Guideline for Adult Sedation, Pain Assessment, and Analgesia in a Low Resource Setting Intensive Care Unit: Review Article.

Background: Agitation and anxiety occur frequently in ICU and affect about 30-80% of patients in ICU present with delirium worldwide, and it is associated with adverse clinical outcomes. This review aimed to systematically review articles and finally draw an evidence-based guideline for an area with limited resources.

Methods: The review was reported based on preferred reporting items for systemic and meta-analysis (PRISMA) protocol.

Comparing Analgesic Effect of Intravenous Fentanyl, Femoral Nerve Block and Fascia Iliaca Block During Spinal Anesthesia Positioning in Elective Adult Patients Undergoing Femoral Fracture Surgery: a Randomized Controlled Trial.

Background: Femoral fracture is the most painful bone injury and performing spinal anesthesia is extremely challenging due to very poor positioning unless we have a very good mode of analgesia. Intravenous strong opioids are commonly used but to date nerve blocks are also being utilized. The reliability and effectiveness of the aforementioned methods are not conclusive to practice worldwide.

Molecular insights into the mechanism of nonrecurrent F8 structural variants: Full breakpoint characterization and bioinformatics of DNA elements implicated in the upmost severe phenotype in hemophilia A.

Hemophilia A (HA) provides excellent models to analyze genotype-phenotype relationships and mutational mechanisms. NhF8ld's breakpoints were characterized using case-specific DNA-tags, direct- or inverse-polymerase chain reaction amplification, and Sanger sequencing. DNA-break's stimulators (n = 46), interspersed repeats, non-B-DNA, and secondary structures were analyzed around breakpoints versus null hypotheses (E-values) based on computer simulations and base-frequency probabilities.

Inverse PCR to perform long-distance haplotyping: main applications to improve preimplantation genetic diagnosis in hemophilia.

Among other applications of long-distance haplotype phasing in clinical genetics, determination of linked DNA markers as surrogate for problematic structural variants (e.g., repeat-mediated rearrangements) is essential to perform diagnosis from low-quality DNA samples.

Platelet Rich Plasma for Chronic Synovitis Treatment in Patients with Haemophilia.

Introduction: Haemophilic synovitis is caused by chronic accumulation of blood in the joint. Conservative treatment is insufficient to solve this pathology. Platelet-rich plasma (PRP) has a high concentration of growth factors (GFs) that play a key role in regulation and stimulation of healing processes.