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Getasew Kassaw,1 Rezika Mohammed,1 Getahun Mengistu Tessema,1 Tesfaye Yesuf,1 Ayenew Molla Lakew,2 Gebrekidan Ewnetu Tarekegn2 1Department of Internal Medicine, School of Medicine, College of Medicine and Health Science, University of Gondar, Gondar, Ethiopia; 2Department of Epidemiology and Biostatistics, Institute of Public Health, College of Medicine and Health Science, University of Gondar, Gondar, EthiopiaCorrespondence: Gebrekidan Ewnetu Tarekegn, Department of Epidemiology and Biostatistics, Institute of Public Health, College of Medicine and Health Sciences, University of Gondar, P.O. Box. 196, Gondar, Ethiopia, Tel +251 930781289, Email ewnetuwendale@gmail.comBackground: Severe community-acquired pneumonia is a common life-threatening infection with a high rate of unfavorable outcome. This study aimed to assess the outcomes and predictors of hospitalized severe community-acquired pneumonia patients at University of Gondar comprehensive specialized hospital.Methods: A prospective follow-up study was conducted at University of Gondar comprehensive specialized hospital from May 1 to September 31, 2021. The data was collected by reviewing patients’ charts and interviewing the patients themselves. Descriptive statistics, binary and multivariable logistic regression analysis were performed accordingly. Variables with p-value < 0.2 on binary logistic regression were analyzed using multivariable logistic regression and variables with p< 0.05 were considered to have significant association.Results: A total of 239 admitted patients with severe community-acquired pneumonia were enrolled in the study. An unfavorable outcome was observed in 105 (44%) patients; 24.27% was in-hospital all-cause mortality, 12.5% was nonresolution, 5.8% was complicated cases, and 1.26% were gone against medical care for poor prognosis. After analyzing multivariable logistic regression, confusion (OR= 4.84; 95%CI: 1.47– 15.88), anemia (OR= 2.36; 95%CI: 1.01– 5.52), leukopenia (OR=4.38; 95%CI: 1.26– 15.25), leukocytosis (OR=3.15; 95%CI: 1.23– 7.96), elevated creatinine (OR=5.67; 95%CI: 1.72– 18.65), intubation (OR=7.27; 95%CI: 1.58– 33.37) and antibiotic revision during treatment for a different reason (OR=0.02; 95%CI: 0.01– 0.07) were variables significantly associated with unfavorable outcome.Conclusion: Unfavorable outcome was high among hospitalized severe community acquired pneumonia patients, and confusion, elevated creatinine, anemia, leukopenia, leukocytosis, intubation during admission, and antibiotic revision during the course were independent predictors associated significantly with the unfavorable outcome. It is important to consider the development of a treatment protocol for the hospital and to further research incorporating the microbiologic profile of the patients.Keywords: pneumonia, unfavorable outcome, community-acquired pneumonia, Ethiopia

Abstract Background Pegunigalsidase alfa is a novel, PEGylated α-galactosidase-A enzyme-replacement therapy approved in the EU and US to treat patients with Fabry disease (FD). Objective/methods BRIDGE is a phase 3 open-label, switch-over study designed to assess safety and efficacy of 12 months of pegunigalsidase alfa (1 mg/kg every 2 weeks) treatment in adults with FD who had been previously treated with agalsidase alfa (0.2 mg/kg every 2 weeks) for ≥ 2 years. Results Twenty-seven patients were screened; 22 met eligibility criteria; and 20 (13 men, 7 women) completed the study. Pegunigalsidase alfa was well-tolerated, with 97% of treatment-emergent adverse events (TEAEs) being of mild or moderate severity. The incidence of treatment-related TEAEs was low, with 2 (9%) discontinuations due to TEAEs. Five patients (23%) reported infusion-related reactions. Overall mean (SD; n = 22) baseline estimated glomerular filtration rate (eGFR) was 82.5 (23.4) mL/min/1.73 m2 and plasma lyso-Gb3 level was 38.3 (41.2) nmol/L (men: 49.7 [45.8] nmol/L; women: 13.8 [6.1] nmol/L). Before switching to pegunigalsidase alfa, mean (standard error [SE]) annualized eGFR slope was − 5.90 (1.34) mL/min/1.73 m2/year; 12 months post-switch, the mean eGFR slope was − 1.19 (1.77) mL/min/1.73 m2/year; and mean plasma lyso-Gb3 reduced by 31%. Seven (35%) out of 20 patients were positive for pegunigalsidase alfa antidrug antibodies (ADAs) at ≥ 1 study timepoint, two of whom had pre-existing ADAs at baseline. Mean (SE) changes in eGFR slope for ADA-positive and ADA-negative patients were + 5.47 (3.03) and + 4.29 (3.15) mL/min/1.73 m2/year, respectively, suggesting no negative impact of anti-pegunigalsidase alfa ADAs on eGFR slope. Conclusion Pegunigalsidase alfa may offer a safe and effective treatment option for patients with FD, including those previously treated with agalsidase alfa. TRN: NCT03018730. Date of registration: January 2017.

Abstract Background Acid Sphingomyelinase Deficiency (ASMD) is a rare autosomal recessive disorder caused by mutations in the SMPD1 gene. This rarity contributes to misdiagnosis, delayed diagnosis and barriers to good care. There are no published national or international consensus guidelines for the diagnosis and management of patients with ASMD. For these reasons, we have developed clinical guidelines that defines standard of care for ASMD patients. Methods The information contained in these guidelines was obtained through a systematic literature review and the experiences of the authors in their care of patients with ASMD. We adopted the Appraisal of Guidelines for Research and Evaluation (AGREE II) system as method of choice for the guideline development process. Results The clinical spectrum of ASMD, although a continuum, varies substantially with subtypes ranging from a fatal infantile neurovisceral disorder to an adult-onset chronic visceral disease. We produced 39 conclusive statements and scored them according to level of evidence, strengths of recommendations and expert opinions. In addition, these guidelines have identified knowledge gaps that must be filled by future research. Conclusion These guidelines can inform care providers, care funders, patients and their carers about best clinical practice and leads to a step change in the quality of care for patients with ASMD with or without enzyme replacement therapy (ERT).

Fabry disease (FD) is an X-linked deficiency of alpha-galactosidase-A, leading to lysosomal storage of sphingolipids in multiple organs. Myocardial accumulation contributes to arrhythmia and sudden death, the most common cause of FD mortality. Therefore, there is a need for risk stratification and prediction to target device therapy. Implantable loop recorders (ILRs) allow for continual rhythm monitoring for up to 3 years. Here, we performed a retrospective study to evaluate current ILR utilisation in FD and quantify the burden of arrhythmia that was detected, which resulted in a modification of therapy. This was a snapshot assessment of 915 patients with FD across three specialist centres in England during the period between 1 January 2000 and 1 September 2022. In total, 22 (2.4%) patients underwent clinically indicated ILR implantation. The mean implantation age was 50 years and 13 (59%) patients were female. Following implantation, nine (41%) patients underwent arrhythmia detection, requiring intervention (six on ILR and three post-ILR battery depletion). Three patients experienced sustained atrial high-rate episodes and were started on anticoagulation. Three had non-sustained tachyarrhythmia and were started on beta blockers. Post-ILR battery depletion, one suffered complete heart block and two had sustained ventricular tachycardia, all requiring device therapy. Those with arrhythmia had a shorter PR interval on electrocardiography. This study demonstrates that ILR implantation in FD uncovers a high burden of arrhythmia. ILRs are likely to be underutilised in this pro-arrhythmic cohort, perhaps restricted to those with advanced FD cardiomyopathy. Following battery depletion in three patients as mentioned above, greater vigilance and arrhythmia surveillance are advised for those experiencing major arrhythmic events post-ILR monitoring. Further work is required to establish who would benefit most from implantation.

Introduction Fabry disease (FD) is an X-linked lysosomal storage disorder caused by enzyme deficiency, leading to glycosphingolipid accumulation. Cardiac accumulation triggers local tissue injury, electrical instability and arrhythmia. Bradyarrhythmia and atrial fibrillation (AF) incidence are reported in up to 16% and 13%, respectively.Objective We conducted a systematic review evaluating AF burden and bradycardia requiring permanent pacemaker (PPM) implantation and report any predictive risk factors identified.Methods We conducted a literature search on studies in adults with FD published from inception to July 2019. Study outcomes included AF or bradycardia requiring therapy. Databases included Embase, Medline, PubMed, Web of Science, CINAHL and Cochrane. The Risk of Bias Agreement tool for Non-Randomised Studies (RoBANS) was utilised to assess bias across key areas.Results 11 studies were included, eight providing data on AF incidence or PPM implantation. Weighted estimate of event rates for AF were 12.2% and 10% for PPM. Age was associated with AF (OR 1.05–1.20 per 1-year increase in age) and a risk factor for PPM implantation (composite OR 1.03). Left ventricular hypertrophy (LVH) was associated with AF and PPM implantation.Conclusion Evidence supporting AF and bradycardia requiring pacemaker implantation is limited to single-centre studies. Incidence is variable and choice of diagnostic modality plays a role in detection rate. Predictors for AF (age, LVH and atrial dilatation) and PPM (age, LVH and PR/QRS interval) were identified but strength of association was low. Incidence of AF and PPM implantation in FD are variably reported with arrhythmia burden likely much higher than previously thought.PROSPERO database CRD42019132045.

Abstract Background Niemann-Pick Disease Type C (NPC) is an autosomal recessive rare disease characterised by progressive neurovisceral manifestations. The collection of on-going large-scale NPC clinical data may generate better understandings of the natural history of the disease. Here we report NPC patient data from the International Niemann-Pick Disease Registry (INPDR). Method The INPDR is a web-based, patient-led independent registry for the collection of prospective and retrospective clinical data from Niemann-Pick Disease patients. Baseline data from NPC patients enrolled into the INPDR from September 2014 to December 2019 was extracted to analyse the demographic, genetic and clinical features of the disease. Results A total of 203 NPC patients from six European countries were included in this study. The mean age (SD) at diagnosis was 11.2 years (14.2). Among enrolled patients, 168 had known neurological manifestations: 43 (24.2%) had early-infantile onset, 47 (26.4%) had late-infantile onset, 41 (23.0%) had juvenile onset, and 37 (20.8%) had adult onset. 10 (5.6%) patients had the neonatal rapidly fatal systemic form. Among the 97 patients with identified NPC1 variants, the most common variant was the c. 3182T > C variant responsible for the p.lle1061Thr protein change, reported in 35.1% (N = 34) of patients. The frequencies of hepatomegaly and neonatal jaundice were greatest in patients with early-infantile and late-infantile neurological onset. Splenomegaly was the most commonly reported observation, including 80% of adult-onset patients. The most commonly reported neurological manifestations were cognitive impairment (78.5%), dysarthria (75.9%), ataxia (75.9%), vertical supranuclear gaze palsy (70.9%) and dysphagia (69.6%). A 6-domain composite disability scale was used to calculate the overall disability score for each neurological form. Across all with neurological onset, the majority of patients showed moderate to severe impairments in all domains, except for ‘swallowing’ and ‘seizure’. The age at diagnosis and death increased with increased age of neurological symptom onset. Miglustat use was recorded in 62.4% of patients and the most common symptomatic therapies used by patients were antiepileptics (32.9%), antidepressants (11.8%) and antacids (9.4%). Conclusion The proportion of participants at each age of neurological onset was relatively equal across the cohort. Neurological manifestations, such as ataxia, dysphagia, and dysarthria, were frequently observed across all age categories.

Abstract Background Fabry disease (FD) is a treatable X-linked condition leading to progressive cardiac disease, arrhythmia and premature death. We aimed to increase awareness of the arrhythmogenicity of Fabry cardiomyopathy, by comparing device usage in patients with Fabry cardiomyopathy and sarcomeric HCM. All Fabry patients with an implantable cardioverter defibrillator (ICD) implanted in the UK over a 17 year period were included. A comparator group of HCM patients, with primary prevention ICD implantation, were captured from a regional registry database. Results Indications for ICD in FD varied with 72% implanted for primary prevention based on multiple potential risk factors. In FD and HCM primary prevention devices, arrhythmia occurred more frequently in FD over shorter follow-up (HR 4.2, p

Abstract Background The Gaucher Investigative Therapy Evaluation is a national clinical cohort of 250 patients aged 5–87 years with Gaucher disease in the United Kingdom—an ultra-rare genetic disorder. To inform clinical decision-making and improve pathophysiological understanding, we characterized the course of Gaucher disease and explored the influence of costly innovative medication and other interventions. Retrospective and prospective clinical, laboratory and radiological information including molecular analysis of the GBA1 gene and comprising > 2500 variables were collected systematically into a relational database with banking of collated biological samples in a central bioresource. Data for deep phenotyping and life-quality evaluation, including skeletal, visceral, haematological and neurological manifestations were recorded for a median of 17.3 years; the skeletal and neurological manifestations are the main focus of this study. Results At baseline, 223 of the 250 patients were classified as type 1 Gaucher disease. Skeletal manifestations occurred in most patients in the cohort (131 of 201 specifically reported bone pain). Symptomatic osteonecrosis and fragility fractures occurred respectively in 76 and 37 of all 250 patients and the first osseous events occurred significantly earlier in those with neuronopathic disease. Intensive phenotyping in a subgroup of 40 patients originally considered to have only systemic features, revealed neurological involvement in 18: two had Parkinson disease and 16 had clinical signs compatible with neuronopathic Gaucher disease—indicating a greater than expected prevalence of neurological features. Analysis of longitudinal real-world data enabled Gaucher disease to be stratified with respect to advanced therapies and splenectomy. Splenectomy was associated with an increased hazard of fragility fractures, in addition to osteonecrosis and orthopaedic surgery; there were marked gender differences in fracture risk over time since splenectomy. Skeletal disease was a heavy burden of illness, especially where access to specific therapy was delayed and in patients requiring orthopaedic surgery. Conclusion Gaucher disease has been explored using real-world data obtained in an era of therapeutic transformation. Introduction of advanced therapies and repeated longitudinal measures enabled this heterogeneous condition to be stratified into obvious clinical endotypes. The study reveals diverse and changing phenotypic manifestations with systemic, skeletal and neurological disease as inter-related sources of disability.

Abstract Introduction There is relatively little published on the effects of COVID-19 on respiratory physiology, particularly breathing patterns. We sought to determine if there were lasting detrimental effect following hospital discharge and if these related to the severity of COVID-19. Methods We reviewed lung function and breathing patterns in COVID-19 survivors > 3 months after discharge, comparing patients who had been admitted to the intensive therapy unit (ITU) (n = 47) to those who just received ward treatments (n = 45). Lung function included spirometry and gas transfer and breathing patterns were measured with structured light plethysmography. Continuous data were compared with an independent t-test or Mann Whitney-U test (depending on distribution) and nominal data were compared using a Fisher’s exact test (for 2 categories in 2 groups) or a chi-squared test (for > 2 categories in 2 groups). A p-value of

Abstract Background We present baseline characteristics and follow-up data of a Managed Access Agreement (MAA), including patients with mucopolysaccharidosis IVA (MPS IVA) receiving elosulfase alfa enzyme replacement therapy (ERT) in England on a conditional basis. Patients enrolled in the MAA programme are reviewed on an annual basis. Therapy can be continued if patients are compliant, able to tolerate infusions, and meet four out of five pre-defined clinical and patient-reported outcomes (PRO) criteria. Baseline and follow-up clinical and PRO data are presented for all participants who completed ≥ 1 year of assessments in the MAA. Results The analysis included data from 55 patients, including 26 patients previously enrolled in clinical trials and 29 who started ERT after enrolling in the MAA. In patients with both baseline and follow-up data, mean 6-min walk test distance increased from 217 m at baseline to 244 m after a mean follow-up of 4.9 years. Improvement or stabilisation was seen regardless of age at treatment initiation or duration of treatment. Mean forced vital capacity and forced expiratory volume in 1 s were 0.87 L and 0.78 L, respectively at baseline and 1.05 L and 0.88 L after a mean follow-up of 5.5 years. PRO data showed overall improvements over time in Mobility, Self-care, and Caregiver assistance scores of the MPS-Health Assessment Questionnaire, relatively stable quality of life, and some improvements in pain scores. Conclusions The MAA data confirm the effects of elosulfase alfa on clinical and PRO results observed in the clinical trials and provide real-world evidence for long-term stabilisation in these measures, suggesting a positive impact on the natural history of MPS IVA.

Abstract We report six adult patients with Tyrosinaemia type 1 (HT‐1) who presented with recurrent porphyria‐like neurological crises after discontinuation/interruption of 2‐(2‐nitro‐4‐trifluoro‐methylbenzyol)‐1,3 cyclohexanedione (NTBC) treatment. The crises were life‐threatening for some of the patients, with respiratory muscle paralysis requiring ventilatory support, hemodynamic disturbance due to autonomic changes requiring resuscitation, acute progressive ascending motor neuropathy causing profound impairment, recurrent seizures, and neuropathic pain. Our patients’ porphyria‐like presentations were variably misdiagnosed, with delay to diagnosis resulting in more severe recurrent attacks. We report the first series of neurological crises in adult patients with HT‐1. These crises, which may be fatal, can be prevented and treated effectively with neurologist/physician awareness and patient education.

Abstract Alström Syndrome (ALMS) is an ultra-rare multisystem genetic disorder caused by autosomal recessive variants in the ALMS1 gene, which is located on chromosome 2p13. ALMS is a multisystem, progressive disease characterised by visual disturbance, hearing impairment, cardiomyopathy, childhood obesity, extreme insulin resistance, accelerated non-alcoholic fatty liver disease (NAFLD), renal dysfunction, respiratory disease, endocrine and urologic disorders. Clinical symptoms first appear in infancy with great variability in age of onset and severity. ALMS has an estimated incidence of 1 case per 1,000,000 live births and ethnically or geographically isolated populations have a higher-than-average frequency. The rarity and complexity of the syndrome and the lack of expertise can lead to delayed diagnosis, misdiagnosis and inadequate care. Multidisciplinary and multiprofessional teams of experts are essential for the management of patients with ALMS, as early diagnosis and intervention can slow the progression of multi-organ dysfunctions and improve patient quality of life. These guidelines are intended to define standard of care for patients suspected or diagnosed with ALMS of any age. All information contained in this document has originated from a systematic review of the literature and the experiences of the authors in their care of patients with ALMS. The Appraisal of Guidelines for Research & Evaluation (AGREE II) system was adopted for the development of the guidelines and for defining the related levels of evidence and strengths of recommendations. These guidelines are addressed to: a) specialist centres, other hospital-based medical teams and staffs involved with the care of ALMS patients, b) family physicians and other primary caregivers and c) patients and their families.

Abstract Background Alström syndrome (ALMS) is a rare ciliopathy characterised by early onset insulin resistance, obesity, and dyslipidaemia and is a model for diseases that have huge social, health and economic impact. Cardiomyopathy develops in the majority, with high rates of morbidity and mortality, the definitive features of which are coarse replacement fibrosis and diffuse myocardial fibrosis (DIF). The pathogenesis of heart failure is thought to involve fibroblast accumulation and expansion of the extracellular matrix with excess protein deposition, leading to distorted organ architecture and impaired contractile function. Consecutive adults with genetically proven ALMS attending the National Centre for Rare Disease in Birmingham, England were studied. All patients underwent serial CMR, echocardiography and venous blood sampling, with computed tomography coronary angiography (CTCA) performed to assess severity of CAD. The aims of this study were: 1) to evaluate changes over time in DIF by cardiovascular magnetic resonance tissue characterization in ALMS; 2) to examine whether changes in DIF are associated with alteration in systolic or diastolic function; and 3) to evaluate the frequency and severity of coronary artery disease as a confounder for progression of ischaemic versus non-ischaemic fibrosis. Results In total, 30/32 adults (63% male; 67% White British) participated. The median age at first scan was 21.3 years (interquartile range: 19.0–32.6) and participants were followed for a maximum of 67 months. Only 4 patients had significant coronary artery stenosis on post-mortem, invasive coronary angiography or CTCA. Mid short axis myocardial T1 times, myocardial extracellular volume, and left ventricular mass increased significantly over time, by an average of 21.8 ms (95% CI 17.4–26.1; p

Abstract A 77‐year‐old gentleman, normally fit and well, was admitted with acute confusion. On admission, Glasgow Coma Scale (GCS) was 14/15, vital signs were within the normal limits and bilateral crepitation at the lung base. Head CT scan was normal. CXR showed some air space opacification. Investigations revealed hyponatraemia, raised CRP, and positive for COVID‐19. Treated with antibiotics and intravenous saline, sodium returned to normal. Delirium remained unchanged 4 weeks post‐incidence. Neurological manifestations were documented in patients with COVID‐19; however no report has shown delirium as a primary manifestation. This case illustrates acute confusion may be the only presenting symptom of COVID‐19 without overt lung disease.

Abstract Background Fabry disease (FD) is a genetic disorder caused by a deficiency in the enzyme alpha-galactosidase A, leading to an accumulation of glycosphingolipids in tissues across the body. Cardiac disease is the leading cause of morbidity and mortality. Advanced disease, characterised by extensive left ventricular hypertrophy, ventricular dysfunction and fibrosis, is known to be associated with an increase in arrhythmia. Data identifying risk factors for arrhythmia are limited, and no Fabry-specific risk stratification tool is available to select those who may benefit from initiation of medical or device therapy (implantable cardiac defibrillators). Current monitoring strategies have a limited diagnostic yield, and implantable loop recorders (ILRs) have the potential to change treatment and clinical outcomes. Aim The aim of this study is to determine whether ILRs can (1) improve arrhythmia detection in FD and (2) identify risk predictors of arrhythmia. Methods A prospective, 5-year, open-label, international, multi-centre randomised controlled trial of a minimum of 164 participants with genetically or enzymatically confirmed FD (or both) who have evidence of cardiac disease will be recruited from five centres: Queen Elizabeth Hospital, Birmingham, UK; Salford Royal Hospital, Salford, UK; Royal Free Hospital, London, UK; Addenbrookes Hospital, Cambridge, UK; and Westmead Hospital, Sydney, Australia. Participants will be block-randomised (1:1) to two study arms for cardiac monitoring (i) control arm: standard of care with annual 24 h or 5-day Holter monitor or (ii) treatment arm: continuous cardiac monitoring with ILR implantation plus standard of care. Participants will undergo multiple investigations—blood/urine biomarkers, 12-lead and advanced electrocardiogram (ECG) recording, echocardiography and cardiovascular magnetic resonance (CMR) imaging—at baseline and 6–12 monthly follow-up visits. The primary endpoint is identification of arrhythmia requiring initiation or alteration in therapy. Secondary outcome measures include characterising the risk factors associated with arrhythmia and outcome data in the form of imaging, ECG and blood biomarkers. Discussion This is the first study evaluating arrhythmia burden and the use of ILR across the spectrum of risk profiles in Fabry cardiomyopathy. This will enable detailed characterisation of arrhythmic risk predictors in FD and ultimately support formulation of Fabry-specific guidance in this high-risk population. Trial registration ClinicalTrials.gov (NCT03305250). Registered on 9 October 2017.

Fabry disease (FD) is a rare X-linked lysosomal storage disorder manifesting as progressive multi-organ accumulation of sphingolipids due to deficiency in the enzyme α-Galactosidase A. Sphingolipid accumulation can take place in all cardiac cell types which manifests as left ventricular hypertrophy, microvascular ischaemia, conduction abnormalities, arrhythmia, heart failure, and valvular disease. The use of advanced cardiovascular imaging techniques have enabled clinicians to stage and prognosticate the disease and guide therapy. Transthoracic echocardiography (TTE) and cardiac magnetic resonance imaging (CMR) in conjunction are the hallmark imaging modalities to allow for this assessment. Traditionally, the assessment of cardiac involvement in FD was based on the assessment of maximal wall thickness (MWT) and the development of left ventricular hypertrophy (LVH). It is now understood that sphingolipid accumulation takes place before the development of LVH. Advances in techniques within TTE and CMR, particularly that of strain assessment and T1/T2 mapping, have meant that Fabry cardiomyopathy (FCM) can be diagnosed earlier in the disease process. This potentially provides a window for initiation of enzyme replacement therapy (ERT) at a stage where it is likely to have the most beneficial effect in reducing the high mortality associated with FCM. This review outlines the advances in multimodality imaging in staging and prognosticating FCM, as well as the applications of cardiac imaging in assessing symptoms and complications of FCM.

Abstract Background Alström syndrome (ALMS) is a very rare autosomal recessive monogenic disorder caused by a mutation in the ALMS1 gene and characterised by childhood onset obesity, dyslipidaemia, advanced non-alcoholic fatty liver disease, diabetes and extreme insulin resistance. There is evidence of multi-organ fibrosis in ALMS and severity of the disease often leads to organ failure with associated morbidities, resulting in reduced life expectancy. There are no specific treatments for this disease, and current management consists of only symptomatic therapies. PBI-4050 is a new molecular entity with demonstrated anti-inflammatory and anti-fibrotic activities in preclinical models, including animal models of human diseases characterized by progressive fibrosis in the kidney, heart, liver and lungs. Moreover, completed Phase 2 studies in type 2 diabetes mellitus with metabolic syndrome and idiopathic pulmonary fibrosis further support the anti-inflammatory and anti-fibrotic activity of PBI-4050. Together, these data suggest that PBI-4050 has the potential to treat the pathological inflammatory and fibrotic features of ALMS. The aim of this study is to evaluate the safety and anti-inflammatory & anti-fibrotic activities of PBI-4050 in subjects with ALMS. Methods This is a Phase 2, single-centre, single-arm, open-label trial. A total of 18 patients with ALMS will be enrolled to receive PBI-4050 at a total daily oral dose of 800 mg for an initial 24 weeks with continuation for an additional 36 or 48 weeks. Standard assessments of safety include adverse events, clinical laboratory tests, vital signs, physical examination and electrocardiograms. Efficacy assessments include adipose tissue biopsy, hyperinsulinaemic-euglycaemic glucose clamp, adipose tissue microdialysis, liver transient elastography, liver and cardiac magnetic resonance imaging, and laboratory blood tests. Discussion This is the first clinical study of PBI-4050 in subjects with ALMS. Given the rarity and complexity of the disease, a single-centre, single-arm, open-label design has been chosen to maximise subject exposure and increase the likelihood of achieving our study endpoints. The results will provide valuable safety and preliminary evidence of the effects of PBI-4050 in ALMS, a rare heterogeneous disease associated with progressive fibrosis and premature mortality. Trial registration The trial is registered on ClinicalTrials.gov (Identifier; NCT02739217, February 2016) and European Union Drug Regulating Authorities Clinical Trials (EudraCT Number 2015–001625-16, Sept 2015).

Abstract Background Benign Lymphoepithelial Lesion (BLEL) is a rare disease observed in the adult population. Despite the growing numbers of people suffering from BLEL, the etiology and mechanisms underlying its pathogenesis remain unknown. Methods In the present study, we used gene and cytokines expression profiling, western blot and immunohistochemistry to get further insight into the cellular and molecular mechanisms involved in the pathogenesis of BLEL of the lacrimal gland. Results The results showed that Macrophage Migration Inhibitory Factor (MIF) was the most highly expressed cytokine in BLEL, and its expression positively correlated with the expression of Th2 and Th17 cells cytokines. MIF was found to regulate biological functions and pathways involved in BLEL pathogenesis, such as proliferation, resistance to apoptosis, MAPK and PI3K/Akt pathways. We also found that MIF promotes fibrosis in BLEL by inducing BLEL fibroblast differentiation into myofibroblasts as well as the synthesis and the deposit of extracellular matrix in BLEL tissues. Conclusions Our findings demonstrate the contribution of MIF to the pathogenesis of BLEL of the lacrimal gland and suggested MIF as a promising therapeutic target for its treatment.

Abstract Niemann-Pick Type C (NPC) is a progressive and life limiting autosomal recessive disorder caused by mutations in either the NPC1 or NPC2 gene. Mutations in these genes are associated with abnormal endosomal-lysosomal trafficking, resulting in the accumulation of multiple tissue specific lipids in the lysosomes. The clinical spectrum of NPC disease ranges from a neonatal rapidly progressive fatal disorder to an adult-onset chronic neurodegenerative disease. The age of onset of the first (beyond 3 months of life) neurological symptom may predict the severity of the disease and determines life expectancy. NPC has an estimated incidence of ~ 1: 100,000 and the rarity of the disease translate into misdiagnosis, delayed diagnosis and barriers to good care. For these reasons, we have developed clinical guidelines that define standard of care for NPC patients, foster shared care arrangements between expert centres and family physicians, and empower patients. The information contained in these guidelines was obtained through a systematic review of the literature and the experiences of the authors in their care of patients with NPC. We adopted the Appraisal of Guidelines for Research & Evaluation (AGREE II) system as method of choice for the guideline development process. We made a series of conclusive statements and scored them according to level of evidence, strengths of recommendations and expert opinions. These guidelines can inform care providers, care funders, patients and their carers of best practice of care for patients with NPC. In addition, these guidelines have identified gaps in the knowledge that must be filled by future research. It is anticipated that the implementation of these guidelines will lead to a step change in the quality of care for patients with NPC irrespective of their geographical location.

Abstract Background Late-onset Pompe disease is a rare genetic neuromuscular disorder caused by lysosomal acid alpha-glucosidase (GAA) deficiency that ultimately results in mobility loss and respiratory failure. Current enzyme replacement therapy with recombinant human (rh)GAA has demonstrated efficacy in subjects with late-onset Pompe disease. However, long-term effects of rhGAA on pulmonary function have not been observed, likely related to inefficient delivery of rhGAA to skeletal muscle lysosomes and associated deficits in the central nervous system. To address this limitation, reveglucosidase alfa, a novel insulin-like growth factor 2 (IGF2)-tagged GAA analogue with improved lysosomal uptake, was developed. This study evaluated the pharmacokinetics, safety, and exploratory efficacy of reveglucosidase alfa in 22 subjects with late-onset Pompe disease who were previously untreated with rhGAA. Results Reveglucosidase alfa plasma concentrations increased linearly with dose, and the elimination half-life was

The study was aimed at mapping the dairy value chain, assessing constraints and opportunities in the sector, and identifying factors affecting channel choices of producers in Harar and Dire Dawa milkshed areas. Data were collected from 93 producers, six collectors, seven wholesalers, seven retailers, and ten consumers. Both descriptive and econometric analysis were employed. The study revealed that the channel choices available to producers include selling to collectors, wholesalers, retailers, and directly to consumers. The multinomial model output indicated that being in rural areas, breed type, separate milking place, and supply of hay negatively determined the choice to sell to wholesalers, retailers, and consumers. In contrast, education status and milk storage duration positively determined producers’ choice not to sell to collectors. The major recommendations include provision of training, disseminating dairy technologies, encouraging value chain actors to add values; and enhancing collective actions of producers.

The ethanol extract of the leaves of Moringa stenopetala led to the isolation of three glycosides. The compounds were identified as rutin, 4-(4'-O-acetyl--L-rhamnosyloxy)- benzylisothiocyanate and 4-(4'-O-acetyl--L-rhamnosyloxy)-benzaldehyde. The last two compounds are reported for the first time from M. stenopetala. Structure determination was accomplished by means of spectroscopic and chemical studies.

Chronic liver disease (CLD) may be a major cause of morbidity and mortality worldwide, as well as a reduction in health-related quality of life. In Ethiopia, however, little is known about the effect of CLD on quality of life. The purpose of this study was to evaluate CLD patients' health-related quality of life and associated factors. A cross-sectional study was carried out on 227 CLD patients who were being followed up on at the University of Gondar specialized hospital between January 2022 and February 2022. To assess health-related quality of life, the chronic disease Questionnaire was used. Epi-Data version 4.6 was used to enter data, and SPSS version 25 was used for all statistical analysis. To identify factors associated with the outcome variable, a linear regression model was used. Patients with CLD had significantly lower mean scores in all domains of health-related quality of life. Hepatitis B virus treatments and a college education or higher have a positive effect on all domains of health-related quality of life. Upper gastrointestinal bleeding, spontaneous bacterial peritonitis (SBP), hepatic encephalopathy (HE), and ascites were a clinical related factors significantly associated with poor health-related quality of life in simple linear regression. In this study setting, all dimensions of health-related quality of life of patients with CLD were compromised. Antiviral treatment for HBV infection, beta-blocker treatment, and the presence of complications such as ascites were all found to be significantly related to health-related quality of life. This necessitates going above and beyond standard treatments to improve patients' quality of life with CLD., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Substance use is associated with high rates of psychiatric symptoms including psychotic symptoms. Despite the severity of the problem, there are intervention gaps in Ethiopia. To combat this, relevant evidence is required to raise the awareness of service providers. This study aimed to assess the prevalence of psychotic symptoms and its associated factors among the youth population who uses psychoactive substances in the Central Gondar Zone, Northwest Ethiopia., Methods: A community-based cross-sectional study was conducted among the youth population in the Central Gondar zone, Northwest Ethiopia, from 01 January to 30 March 2021. A multistage sampling technique was used to recruit the study participants. All data were collected using questionnaires assessing socio-demographic; family-related variables; Depression, Anxiety, and Stress Scale; Multidimensional Scale Perceived Social Support (MSPSS), and Self-Reporting Questionnaire (SRQ-24). The data were analyzed using the STATA 14 statistical program., Results: A total of 372 young people who used psychoactive substances (79.57%, 53.49%, 34.14%, and 16.13% were consuming alcohol, Khat, tobacco/cigarette products, and other substances such as shisha, inhalants, and drugs, respectively) were included in the study. The prevalence of psychotic symptoms was 24.2% with a 95% CI: 20.1, 28.8. Being married [AOR = 1.87 95% CI: 1.06, 3.48], recent loss of loved ones [AOR = 1.97 95% CI: 1.10, 3.18], low perceived social support [AOR = 1.61 95% CI: 1.11, 3.02], and severe psychological distress [AOR = 3.23 95% CI; 1.64, 6.54] were the factors associated with psychotic symptoms among young people with psychoactive substances use at a p -value of < 0.05., Conclusion: Psychotic symptoms related to psychoactive substances among the youth population in Northwest Ethiopia were high. Thus, it is better to give a special attention to the youth population with low social support and existing psychological distress concurrent with psychoactive substance use., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Kassew, Tilahun, Alemayehu, Getnet, Demilew, Tarekegn, Alemu, Yesuf, Oumer, Mehari, Melkam and Nenko.)

Background: In Ethiopia, the youth are more exposed to substances such as alcohol, Khat, and tobacco than other populations. Despite the seriousness of the situation, low- and middle-income nations, particularly Ethiopia, have intervention gaps. Service providers must be made more aware of relevant evidence to combat these problems. This research focused on finding out how common substance abuse is among teenagers and the factors that influence it., Methods: The 2016 Ethiopian Demographic and Health Survey data were used for secondary data analysis. This survey includes all young people aged 15 to 24 years. The total sample size was 10,594 people. Due to the hierarchical nature of the survey data, a multilevel logistic regression model was employed to uncover the individual- and community-level characteristics related to substances., Results: In Ethiopia, the overall current prevalence of occasional or daily substance use 30 days prior to the survey was 46.74%. Of the participants, 36.34, 12.56, and 0.95% were drinking alcohol, chewing Khat, and smoking cigarettes/any tobacco products, respectively. Male sex, 20-24 years of age, exposure to media, having a job, and living in large central and metropolitan regions were the factors associated with the problem., Conclusion: According to the 2016 EDHS, substance use among young people is widespread in Ethiopia. To lower the prevalence of substance use among youth, policymakers must increase the implementation of official rules, such as restricting alcohol, Khat, and tobacco product marketing to minors, prohibiting smoking in public places, and banning mass-media alcohol advertising. Specific interventions targeting at-risk populations, such as youth, are mainly required in prominent central and metropolitan locations., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Kassew, Tarekegn, Alamneh, Kassa, Liyew and Terefe.)

Background: Alcohol consumption during pregnancy is a known contributor to teratogen and causes a range of effects on pregnancy and birth outcomes. This study aimed to investigate the spatial variation and determinants of alcohol consumption among pregnant women in Ethiopia., Methods: A secondary data analysis was conducted using the 2016 Ethiopian Demographic and Health Survey data. A total of 1,135 pregnant women were included in the analysis. ArcGIS version 10.7 software was used to explore the spatial distribution of alcohol consumption, and SaTScan version 9.6 was employed to identify the significant spatial clusters of alcohol consumption. A mixed multi-level logistic regression analysis was employed to identify the determinant factors of alcohol consumption during pregnancy., Results: The result showed that the prevalence of alcohol consumption during pregnancy was 22.49% (with a 95% CI: 18.18 to 26.17). The spatial analysis showed that the spatial distribution of alcohol consumption significantly varied across the country [Global Moran's I value = 0.30 (P<0.001)]. The SaTScan analysis identified two most likely clusters with high rates of alcohol consumption such as northwest Ethiopia (Log-Likelihood Ratio (LLR) = 155.56, p<0.001) and central Ethiopia (LLR = 19.27, p<0.01). Never in union, divorced and/ widowed [Adjusted odds ratio (AOR) = 2.56; 95% CI: 1.07, 10.14], attended primary school [AOR = 0.45; 95% CI: 0.27, 0.95], having two or more lifetime sexual partners [AOR = 2.59; 95% CI: 1.11, 6.18], living in rural [AOR = 1.52; 95% CI: 1.12, 2.93] and higher community media exposure [AOR = 0.54; 95% CI: 0.28, 0.97] were the factors associated with alcohol consumption., Conclusion: Alcohol consumption during pregnancy in Ethiopia was high. The spatial distribution of alcohol consumption was significantly varied across the country. Therefore, public health interventions targeting areas with high alcohol consumption are needed for drinking cessation and to prevent poor pregnancy outcomes related to alcohol use., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2022 Kassew et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Background: Iron deficiency is one of the significant factors of anemia during pregnancy. Iron supplementation is the main method of prevention and control of iron deficiency anemia, and its effectiveness depends on adherence to the iron supplementation., Methods: This study was based on a secondary analysis of 2005, 2011, and 2016 EDHS data. After the data was weighted using sampling weight, 696, 1,282, and 3,096 in 2005, 2011, and 2016 EDHS data, respectively, were used for the final analysis. The data were edited, cleaned, coded, managed, and analyzed using StataCorp version 16 software. A logit-based multivariable decomposition analysis was used to identify variables significantly associated with the change in the adherence level during pregnancy., Results: Adherence levels increased from 1.1% (95% CI; 0.4, 2.7) in 2005 EDHS to 12.4% (95% CI; 10.9, 14.1) in 2016 EDHS. About 30.9% of the overall change in the adherence level to iron supplement use during pregnancy was due to the difference in women's sociodemographic-related variables. After adjusting for these compositional changes, ~69.1% of the change in the adherence level was because of the difference in the coefficients (behavior-related variables). Among the behavioral characteristics, women's age-group, rich wealth index, and secondary and above-secondary educational status of their husbands had a statistically significant effect on the positive change in the adherence level of pregnant mothers., Conclusion: The adherence level to iron supplement use during pregnancy has increased significantly over the last decade in Ethiopia. Both the compositional and behavioral characteristics of women play a major role in the increasing trend of adherence levels., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Delie, Gezie, Gebeyehu, Tarekegn and Muche.)

Background: Substance use leads to serious clinical conditions with the potential to cause major health and emotional impairments in individuals. Individuals with substance use typically report significantly poorer QoL than the general population and as low as those with other serious psychiatric disorders. It has a high impact on morbidity, mortality, and productivity, it also compromises the general safety and performance of the users, i.e., affects the quality of life. Therefore, this study aimed to assess quality of life and identify the potential predictors among youths who use substances., Methods: A multicenter cross-sectional study design was applied to assess quality of life and associated factors among substance use youths in the central Gondar zone from January 1 to March 30/ 2021. A total of 373 substance use youths were included in the study. The data were collected using face-to-face interview by structured questionnaires, and entered to Epi-data version 4.6 and exported to STATA version 16, and AMOS software for further statistical analysis. To identify factors associated with health-related quality of life, structural equation modeling was used, and it also used to estimate the relationships among exogenous, mediating, and endogenous variables., Results: Substance used youths had a moderate overall health-related quality of life (mean score = 50.21 and 14.32 standard deviation, p-value < 0.,0001), and poor health-related quality of life in the environmental health domain (mean score of 45.76 with standard deviation of 17.60). Age (β = 0.06, p<0.001), sex (β = 0.30, p<0.001), psychotic symptoms (β = -0.12, p<0.001), employment status (β = 0.06, p = 0.008,), loss of family (β = 0.35, p<0.001), and social support (β = 0.27, p<0.001) were variables significantly associated with health-related quality of life., Conclusion: According to the findings of this study, substance abuse during adolescence is associated with lower health-related quality of life and a higher report of psychopathological symptoms. Given this finding, mental health and health promotion professionals should learn about and emphasize the impact of substance use on youth quality of life., Competing Interests: The authors have declared that no competing interests exist.

Background: Abortion is one of the top five causes of maternal mortality in low and middle-income countries. It is associated with a complication related to pregnancy and childbirth. Despite this, there was limited evidence on the prevalence and associated factors of abortion in East African countries. Therefore, this study aimed to investigate the prevalence and associated factors of abortion among reproductive-aged women in East African countries., Methods: The Demographic and Health Surveys (DHS) data of 12 East African countries was used. A total weighted sample of 431,518 reproductive-age women was included in the analysis. Due to the hierarchical nature of the DHS data, a multilevel binary logistic regression model was applied. Both crude and Adjusted Odds Ratio (AOR) with 95% Confidence Interval (CI) was calculated for potential associated factors of abortion in East Africa. In the final model, variables with a p value < 0.05 were declared as statistically significant factors of abortion., Results: Around 5.96% (95%CI: 4.69, 7.22) of reproductive-aged women in East Africa had a history of abortion. Alcohol use, tobacco or cigarette smoking, being single, poorer wealth index, currently working, traditional family planning methods, and media exposure were associated with a higher risk of abortion. However, higher parity, having optimum birth intervals, and modern contraceptive uses were associated with lower odds of abortion., Conclusions: The prevalence of abortion among reproductive-aged women in East Africa was high. Abortion was affected by various socio-economic and obstetrical factors. Therefore, it is better to consider the high-risk groups during the intervention to prevent the burdens associated with abortion., (© 2022. The Author(s).)

Introduction: Hypertension, often known as increased blood pressure, is a worldwide public health concern. Globally, ~1 billion people have hypertension and 7.1 million die from this disease. It is disproportionately more prevalent in resource-poor nations, with inadequate health systems like Ethiopia. Moreover, information on the burden of disease from hypertension in the specific area, especially in the newly organized Wolkait Tegedie zone, is essential to develop effective prevention and control strategies. Therefore, this study aimed to assess the prevalence of hypertension and associated factors among adult patients evaluated at the outpatient department of the two district hospitals in the Wolkait Tegedie zone, Northwest Ethiopia., Methods: An institution-based cross-sectional study was conducted from September to October 2021. A systematic random sampling technique was used to select a total of 449 participants. The data were collected and then entered using EPI-INFO version 7 and exported to STATA 14 for analysis. Bivariable and multivariable binary logistic regression analyses were performed. Adjusted odds ratio (AOR) with a 95% confidence interval (CI) was used as a measure of association. Variables having a p -value < 0.05 from the multivariable analysis were considered to have a significant association with the outcome., Results: The prevalence of hypertension among adult patients in this study was 44.91% [95% CI: 40.26%, 49.65%], of which 63.92% were new diagnoses. Being >60 years [AOR = 1.81; 95% CI: 1.11, 3.20], having poor physical exercise [AOR = 1.74; 95% CI: 1.01, 3.15], consuming cruddy oil [AOR = 15.22; 95% CI: 3.86, 60.12], having a family history of hypertension [AOR = 13.02; 95% CI: 3.75, 45.16], and having a history of diabetes mellitus (DM) [AOR = 8.05; 95% CI: 1.24, 51.49] had a statistically significant association with having hypertension., Conclusion: There is a relatively high prevalence of hypertension among adult patients in the outpatient department of the two primary hospitals, Northwest Ethiopia. Factors such as being of older age, having poor physical exercise behavior, cruddy oil consumption, and family history of DM and hypertension had a positive significant statistical association with being hypertensive. Community-based screening programs for hypertension should be designed and implemented to prevent this silent killer disease. Health education and promotion that focus on healthy nutrition and physical exercise should be delivered., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Belay, Fekadu Wolde, Molla, Aragie, Adugna, Melese, Tarekegn, Gezahegn and Kibret.)

Background: Substance use is referring to the use of psychoactive substances like chat, cigarettes, alcohol, and others. The use of substances particularly (alcohol, chat, and cigarette) is a major mental health burden in developing countries including Ethiopia among youth. Suicide ideation and an attempt are thinking or trying to kill oneself that facilitates the act of a person intentionally causing his or her death. Suicide is one of the most serious mental health problems and has a great social impact in the world as it is currently the third leading cause of death for youth. Youth is defined as the period of life between childhood and maturity with an age interval of (15-25)., Method: A cross-sectional study design was used to assess the prevalence of suicidal ideation and attempts with substance use among youth in northwest Ethiopia. Multi-stage sampling techniques of stratified with simple random sample ware used. In the first stage, substance users are selected then as the second stage among substance users the burden of suicide behavior is assessed. ASIST, DASS-21, and other tools were used to assess suicidal behavior with substance use and associated factors. Data were edited, purified, and entered into Epi-data version 4.6 before being exported to the statistical package for social sciences version 20 for analysis of bi-variables to see the associations' p-value < 0.2 and multi-variables to identify the associated variables with a p-value of < 0.05 AOR and CI also done., Results: From a total of 372 substance user participants over all prevalence of suicidal ideation and attempt among youth was 54(14.5%) with 95% CI of (11.0,18.0) and 37(9.9%) with 95% CI (7.0, 13.0) respectively. Being female [AOR =2.36;95% CI:(1.19, 4.68)], poor social support [AOR =3.03; 95% CI: (1.11, 8.25)], and anxiety [AOR = 3.82: 95% CI; (1.96, 7.46)]., Conclusion and Recommendations: The prevalence of suicidal ideation and attempt among substance users was 14.5 and 9.9% respectively therefore, immediate interventional actions needed to be administered to decrease the burden of suicide by reducing substance use and other associated factors., (© 2022. The Author(s).)

Background: Early diagnosis and treatment of childhood fever are essential for controlling disease progression and death. However, the Treatment-seeking behaviour of caregivers is still a significant challenge in rural parts of the African region. This study aimed to assess individual and community-level factors associated with treatment-seeking behaviours among caregivers of febrile under-five age children in Ethiopia., Method: The recent Ethiopian Demographic and Health Survey data (EDHS 2016) was used for the study. The survey collected information among 1,354 under-five children who had a fever within two weeks before the survey. The data were extracted, cleaned, and recoded using STATA version 14. Multilevel logistic regressions were used to determine the magnitude and associated factors of treatment-seeking behaviour among caregivers with febrile children in Ethiopia. Four models were built to estimate both fixed and random effects of individual and community-level factors between cluster variations on treatment-seeking behaviour. The Adjusted Odds Ratios with 95% Confidence Intervals (CI) of the best-fitted model were reported at p<0.05., Result: This study revealed that 491 (36.26%) caregivers seek treatment for their febrile children. Living in metropolitan and small peripheral regions, delivery at health institutions, being poorer, middle and richer wealth quintiles, having a child with diarrhoea, cough, short rapid breathing, and wasting were positively associated with treatment-seeking behaviour of caregivers., Conclusion: The caregivers had poor treatment-seeking behaviour for their febrile children in Ethiopia. Health education programmers should emphasise the importance of seeking early treatment, taking action on childhood febrile illness signs., Competing Interests: The authors have declared that no competing interests exist.

Background: The incidence of eclampsia and its adverse maternal outcomes are very high in developing countries, particularly in Subsaharan African Countries. Identifying predictors for adverse maternal outcomes of eclampsia has paramount importance for helping health care providers to optimize their management outcomes. Therefore, this study aimed to assess the incidence of adverse maternal outcomes of eclampsia and its determinant factors., Methods: A retrospective follow-up study design was applied. The data were extracted from patient charts using a structured, pre-tested, questionnaire. Descriptive analyses (frequencies, means, and standard deviation) were calculated, and bi-variable and multivariable logistic regression models were used to testing the association between independent variables and an outcome variable. After the data were coded and entered into Epi-Info Version 7.2 Software, the data were analyzed using STATA Version 14 Statistical Software., Results: The magnitude of eclampsia was 5.36 per 1000 pregnancies (95% CI: 4.72, 6.10). The incidence of adverse maternal outcomes in eclamptic mothers was 53.7% (95% CI: 47.02, 60.24%). After adjusting for covariates maternal age 30-34, AOR 5.4 [95% CI = 1.02, 28.6]; age above 34, AOR 10.5 [95% CI = 1.3, 88.6]; gravidity 2-4, AOR 0.3 [95% CI = 0.1, 0.9]; 10 or more convulsions, AOR 4.6 [95% CI = 1.4, 14.9]; mild pyrexia, AOR 20.4 [95% CI = 3.7, 112.7]; moderate pyrexia, AOR 14.6 [95% CI = 1.7125.1]; platelet count below 50,000 cells/mm 3 , AOR 34.9 [95% CI = 3.6, 336.2]; platelet count between 50,000 and 99,000 cells/mm 3 , AOR 24.5 [95%CI = 5.4111.6]; and stillbirth of the current pregnancy, AOR 23.2 [95%CI = 2.1257.5] were strong predictors of adverse maternal outcomes in eclamptic mothers., Conclusions: The incidence of adverse maternal outcomes of eclampsia was found to be high compared to similar studies discussed in this study. This study recommends early identification of patients with the risk factors (having many convulsions, high body temperature, low platelet count, patient age above 30 years, and 2-4 pregnancies), strengthening the referral system, and advocation of research on the area of adverse maternal outcomes and thereby encourage evidence-based medicine., (© 2021. The Author(s).)

Background: Antenatal depression is the major obstetric problem that led to significant maternal and perinatal morbidity and mortality worldwide, especially in the third world. However, in Ethiopia this prevalence and association were not studied, as result, this study investigated the prevalence and associated factors of antenatal depression among pregnant mothers who had intimate partner violence during pregnancy. Methodology . An institution-based cross-sectional study was done among 409 pregnant mothers who had intimate partner violence during pregnancy from May to July 2019 at Gondar University Hospital. All pregnant mothers who came for ANC follow-up during the study period approached for screening of intimate partner violence during pregnancy using standard and validated screening method and instrument of the WHO multicountry study on women's health and domestic violence to evaluate intimate partner violence, and we use EPDS for the evaluation of antenatal depression validated in Ethiopia with a cut point of 13., Result: Prevalence of depression among pregnant mothers who had any form of intimate partner violence during pregnancy was 35%: physical abuse (AOR = 1.8; 95% CI: 1.19, 3.30), more than one type of abuse (AOR = 10.18; 95% CI: 7.10, 16.18), poor social support (AOR = 5.81; 95% CI: 1.12, 13.12), and pregnant mothers whose partner drunk for the past twelve months (AOR = 7.16; 95% CI: 183, 8.00) were significantly associated with antenatal depression., Conclusion: High prevalence of antenatal depression among pregnant mothers who had intimate partner violence during pregnancy was highly associated with physical abuse, more than one type of abuse, lack of social support, and partner of pregnant mothers who is a drunk. Hence, this is important to create a screening program and prevention strategy of intimate partner violence during pregnancy at the time of antenatal follow-up to prevent and early identify its morbidity and mortality., Competing Interests: The authors declare that they have no competing interests., (Copyright © 2021 Sileshi Ayele et al.)