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Background: Biallelic variants in POLR1C are associated with POLR3-related leukodystrophy (POLR3-HLD), or 4H leukodystrophy (Hypomyelination, Hypodontia, Hypogonadotropic Hypogonadism), and Treacher Collins syndrome (TCS). The clinical spectrum of POLR3-HLD caused by variants in this gene has not been described. Methods: A cross-sectional observational study involving 25 centers worldwide was conducted between 2016 and 2018. The clinical, radiologic and molecular features of 23 unreported and previously reported cases of POLR3-HLD caused by POLR1C variants were reviewed. Results: Most participants presented between birth and age 6 years with motor difficulties. Neurological deterioration was seen during childhood, suggesting a more severe phenotype than previously described. The dental, ocular and endocrine features often seen in POLR3-HLD were not invariably present. Five patients (22%) had a combination of hypomyelinating leukodystrophy and abnormal craniofacial development, including one individual with clear TCS features. Several cases did not exhibit all the typical radiologic characteristics of POLR3-HLD. A total of 29 different pathogenic variants in POLR1C were identified, including 13 new disease-causing variants. Conclusions: Based on the largest cohort of patients to date, these results suggest novel characteristics of POLR1C-related disorder, with a spectrum of clinical involvement characterized by hypomyelinating leukodystrophy with or without abnormal craniofacial development reminiscent of TCS.
Background: Continuous electroencephalographic (cEEG) monitoring is essential to diagnosing non-convulsive seizures (NCS), reported to occur in 7-46% of at-risk critically ill patients. However, cEEG is labour-intensive, and given scarcity of resources at most centres cEEG is feasible in only selected patients. We aim to evaluate the clinical utility of cEEG at our centre in order to optimize further cEEG allocation among critically ill patients. Methods: Using a clinical database, we identified critically ill children who underwent cEEG monitoring in 2016, 2017 and 2018. We abstracted underlying diagnoses, indication for cEEG monitoring, cEEG findings, and associated changes in management. Results: Over this three year period, 928 cEEGs were performed. Among the 100 studies analyzed to date, primary indications for monitoring were characterization of events of unclear etiology (32%), diagnosis of NCS (30%), and monitoring of therapy for seizures (17%). Seizures were captured in 31% of patients (22% subclinical only, 5% electroclinical only, 4% both), which resulted in a treatment change in 90% of cases. Non-epileptic events were captured in 26% of patients. Conclusions: cEEG yielded clinically meaningful information in 57% of cases, frequently resulting in management changes. Subgroup analyses by cEEG indication and ICU location will be presented.
To propose a new classification of inner-ear anomalies that is more clinically oriented and surgically relevant: the SMS (Sawai Man Singh) classification of cochleovestibular malformations.
A retrospective multicentric study was conducted of 436 cochlear implantations carried out in 3 Indian tertiary care institutes. Patients with anomalous anatomy were included and classified, as per the new SMS classification, into cochleovestibular malformation types I, II, III and IV, based on cochlear morphology, modiolus and lamina cribrosa.
There were 19, 23, 8 and 4 patients with cochleovestibular malformation types I, II, III and IV, respectively. Two-year post-operative Meaningful Auditory Integration Scale scores were statistically analysed.
This new classification for inner-ear anomalies is a simpler, more practical, outcome-oriented classification that can be used to better plan the surgery. These merits make it a more uniform classification for recording results.
Obstructive sleep apnoea is a common chronic sleep disorder characterised by collapse of the upper airway during sleep. The nasal airway forms a significant part of the upper airway and any obstruction is thought to have an impact on obstructive sleep apnoea. A systematic review was performed to determine the role of rhinological surgical interventions in the management of obstructive sleep apnoea.
A systematic review of current literature was undertaken; studies were included if they involved comparison of a non-surgical and/or non-rhinological surgical intervention with a rhinological surgical intervention for treatment of obstructive sleep apnoea.
Sixteen studies met the selection criteria. The pooled data suggest that there are reductions in the apnoea/hypopnea index and respiratory disturbance index following nasal surgery. However, the current body of studies is too heterogeneous for statistically significant meta-analysis to be conducted.
Nasal surgery may have limited benefit for a subset of patients based on current evidence.
We report the synthesis and optical properties of pure ZnS and Ag doped ZnS nanostructures. ZnS(Ag) was synthesized by using the hydrothermal technique and later annealed at different temperatures under vacuum conditions. It was observed that the photoluminescence (PL) emission from the ZnS(Ag) nanostructures can be easily tuned from the blue (445 nm) to green (530 nm) region of visible light by varying the annealing temperature. This tunability has been attributed to the introduction of excess sulfur vacancy states, which is evident from the PL excitation spectra. This observed change in the PL emission wavelength can be highly beneficial in the imaging screens where ZnS is regularly used and can be easily interfaced with the silicon photodiodes showing maximum sensitivity at 550 nm.
Mental health and wellbeing, including addressing impacts of historical trauma and substance use among young people, has been identified as a key priority by Indigenous communities and leaders across Canada and globally. Yet, research to understand mental health among young Indigenous people who have used drugs is limited.
To examine longitudinal risk and strengths-based factors associated with psychological distress among young Indigenous people who use drugs.
The Cedar Project is an ongoing cohort study involving young Indigenous people who use drugs in Vancouver, Prince George, and Chase, British Columbia, Canada. This study included participants who completed the Symptom Checklist-90-Revised, returned for follow-up between 2010 and 2012, and completed the Childhood Trauma Questionnaire. Adjusted linear mixed-effects models estimated effects of study variables on changes in area T-scores of psychological distress.
Of 202 eligible participants, 53% were women and the mean age was 28 years. Among men, childhood maltreatment (emotional abuse, physical abuse, sexual abuse, physical neglect), any drug use, blackouts from drinking, and sex work were associated with increased distress. Among women, childhood maltreatment (emotional abuse, physical abuse, physical neglect), blackouts from drinking, and sexual assault were associated with increased distress, while having attempted to quit using drugs was associated with reduced distress. Marginal associations were observed between speaking their traditional language and living by traditional culture with lower distress among men.
Culturally safe mental wellness interventions are urgently needed to address childhood trauma and harmful coping strategies that exacerbate distress among young Indigenous people who use drugs.
Background: The role of extent of surgical resection (EOR) on clinical outcomes in patients with low-grade glioma requires further examination. Methods: We systematically searched MEDLINE, Embase, and the Cochrane Library for studies published between January 1, 1990 and January 5, 2018 on predefined patient outcomes regarding different EOR of low-grade glioma. Results: Our literature search yielded 60 studies including 13,289 patients. Pooled estimates of overall survival showed an increase from 3.79 years (95% CI, 2.37–5.22) in the biopsy group to 6.68 years (95% CI, 4.19–9.16) in STR to 10.65 years (95% CI, 6.78–14.52) in GTR. When compared to STR, GTR prolonged progression-free survival by 2.08 years (95% CI, 0.26–3.89; P=0.025). Pooled estimates of seizure control showed an improvement from 47.8% (95% CI, 26.7–69.6) with biopsy to 54.2% (95% CI, 48.7–59.6) with STR to 81.0% (95% CI, 74.6–86.2) with GTR. Compared to STR, GTR delayed malignant transformation (RR, 0.43; 95% CI, 0.20–0.93; P=0.032), without increasing postoperative mortality (RR, 0.38; 95% CI, 0.07–1.97; P=0.250) or morbidity (RR, 1.22; 95% CI, 0.65–2.28; P=0.540). Conclusions: Among patients with low grade gliomas, higher degrees of safe EOR, were associated with longer overall and progression-free survival, better seizure control, and delayed malignant transformation, without increased mortality or morbidity.
The study primarily aimed to calculate the orientation of the cochlea pre-operatively, using high-resolution computed tomography of the temporal bone, and predict the ease of electrode insertion.
Pre-operatively, high-resolution computed tomography scans were conducted on children scheduled for cochlear implantation, and two angles, α and β, were calculated. The values of α and β were then correlated with intra-operative difficulty in insertion of the electrode array.
Ninety-six children were included in the study. Of the seven patients who had an α angle of less than 50 degrees, the surgeon experienced difficulties in electrode insertion. However, there were four patients with an α angle of more than 50 degrees for whom the surgeon also experienced difficulties in electrode insertion. In all these patients, the β angle was more than 20 degrees.
Calculation of cochlear orientation and its angle with the surgical axis (α and β) can aid the planning of surgery, particularly with regard to the cochleostomy site and preservation of residual hearing.
Agriculture in the Central Himalayan Region depends on the availability of suitable germplasm as well as natural conditions. Due to extreme weather conditions, food and nutrition security is a major issue for communities inhabiting these remote and inaccessible areas. Millets are common crops grown in these areas. Foxtail millet (Setaria italica (L.) P. Beauv) is an important crop and forms a considerable part of the diet in this region. The aim of the present study was to explore, collect, conserve and evaluate the untapped genetic diversity of foxtail millet at the molecular level and discover variability in their nutritional traits. A total of 30 accessions having unique traits of agronomic importance were collected and molecular profiling was performed. A total of 63 alleles were generated with an average of 2.52 alleles per locus and average expected heterozygosity of 0.37 ± 0.231. Significant genetic variability was revealed through the genetic differentiation (Fst) and gene flow (Nm) values. Structure-based analysis divided whole germplasm into three sub-groups. Rich variability was found in nutritional traits such as dietary fibre in husked grains, carbohydrate, protein, lysine and thiamine content. The collected germplasm may be useful for developing nutritionally rich and agronomically beneficial varieties of foxtail millet and also designing strategies for utilization of unexploited genetic diversity for food and nutrition security in this and other similar agro-ecological regions.
During tonsillectomy, one of the key concerns is establishing adequate haemostasis. Intra-operative haemorrhage from the upper pole of the tonsil fossae can be a challenge to deal with because of an inability to clearly visualise bleeding points. Laryngeal mirrors are readily available in an ENT operating theatre and routinely used for indirect visualisation of the post-nasal space during adenoidectomy.
This paper describes a technique, utilising a laryngeal mirror placed within the tonsil fossa (after having removed the palatine tonsil), to visualise the upper pole and any bleeding points.
Results and conclusion:
Laryngeal mirrors can be used with case to inspect the tonsil fossa post-tonsillectomy for bleeding points which may not be directly visible, so that these can be cauterised.
The interaction of laser radiation with low density aerogels is of crucial importance in areas such as the creation of coherent radiation sources in the X-ray range, simulation of astrophysical as well as nuclear fusion phenomena in laboratory and fundamental studies of the properties of soft condensed matter under dynamic pressure in the Mbar range. In the present paper, the experimental results on the X-ray emission of laser induced plasma in TEOS based, MTMS based aerogels and solid quartz targets, are reported. The aerogels were produced by the sol-gel processing of tetraethoxysilane (TEOS) and methyltrimethoxysilane (MTMS) followed by supercritical drying. Silica alcogels were produced using 0.001 M oxalic acid (C2H4O4), 0.5 M ammonium hydroxide (NH4OH) catalysts. Different densities of aerogels varying from 0.02 to 0.06 g/cm3 have been obtained using different molar ratios of TEOS, MTMS, ethanol and catalysts. The laser, with intensity up to 2 x 1014 W/m2, interaction with TEOS and MTMS based aerogels have been conducted using 30J/500 ps Nd : glass laser system. The resulting soft (0.8 – 1.56 Kev) and hard (>4 Kev) X-ray emissions have been measured using semiconductor photodiodes. It has been observed that the soft X-ray yield increases by a factor of two for the silica aerogel targets compared to the X-ray emission from the solid quartz target, whereas the hard X-ray yield reduces. The enhanced soft X-ray yield in silica aerogel targets is attributed to the large volume heating.
The Global Muon Detector Network (GMDN) is composed by four ground cosmic ray detectors distributed around the Earth: Nagoya (Japan), Hobart (Australia), Sao Martinho da Serra (Brazil) and Kuwait city (Kuwait). The network has operated since March 2006. It has been upgraded a few times, increasing its detection area. Each detector is sensitive to muons produced by the interactions of ~50 GeV Galactic Cosmic Rays (GCR) with the Earth′s atmosphere. At these energies, GCR are known to be affected by interplanetary disturbances in the vicinity of the earth. Of special interest are the interplanetary counterparts of coronal mass ejections (ICMEs) and their driven shocks because they are known to be the main origins of geomagnetic storms. It has been observed that these ICMEs produce changes in the cosmic ray gradient, which can be measured by GMDN observations. In terms of applications for space weather, some attempts have been made to use GMDN for forecasting ICME arrival at the earth with lead times of the order of few hours. Scientific space weather studies benefit the most from the GMDN network. As an example, studies have been able to determine ICME orientation at the earth using cosmic ray gradient. Such determinations are of crucial importance for southward interplanetary magnetic field estimates, as well as ICME rotation.
In low- and middle-income countries, mental health training often includes sending few generalist clinicians to specialist-led programs for several weeks. Our objective is to develop and test a video-assisted training model addressing the shortcomings of traditional programs that affect scalability: failing to train all clinicians, disrupting clinical services, and depending on specialists.
We implemented the program -video lectures and on-site skills training- for all clinicians at a rural Nepali hospital. We used Wilcoxon signed-rank tests to evaluate pre- and post-test change in knowledge (diagnostic criteria, differential diagnosis, and appropriate treatment). We used a series of ‘Yes’ or ‘No’ questions to assess attitudes about mental illness, and utilized exact McNemar's test to analyze the proportions of participants who held a specific belief before and after the training. We assessed acceptability and feasibility through key informant interviews and structured feedback.
For each topic except depression, there was a statistically significant increase (Δ) in median scores on knowledge questionnaires: Acute Stress Reaction (Δ = 20, p = 0.03), Depression (Δ = 11, p = 0.12), Grief (Δ = 40, p < 0.01), Psychosis (Δ = 22, p = 0.01), and post-traumatic stress disorder (Δ = 20, p = 0.01). The training received high ratings; key informants shared examples and views about the training's positive impact and complementary nature of the program's components.
Video lectures and on-site skills training can address the limitations of a conventional training model while being acceptable, feasible, and impactful toward improving knowledge and attitudes of the participants.
To investigate the feasibility of a national audit of epistaxis management led and delivered by a multi-region trainee collaborative using a web-based interface to capture patient data.
Six trainee collaboratives across England nominated one site each and worked together to carry out this pilot. An encrypted data capture tool was adapted and installed within the infrastructure of a university secure server. Site-lead feedback was assessed through questionnaires.
Sixty-three patients with epistaxis were admitted over a two-week period. Site leads reported an average of 5 minutes to complete questionnaires and described the tool as easy to use. Data quality was high, with little missing data. Site-lead feedback showed high satisfaction ratings for the project (mean, 4.83 out of 5).
This pilot showed that trainee collaboratives can work together to deliver an audit using an encrypted data capture tool cost-effectively, whilst maintaining the highest levels of data quality.
Odour emissions are a normal part of broiler production but they potentially threaten the sustainable development of the broiler industry. There are currently no effective methods to reduce odour emissions that are practical or affordable for use in commercial farms. Diets can be formulated to more closely meet the bird's nutritional requirements to avoid overfeeding and to reduce the excretion of undigested components. This will decrease the amount of substrates that the microbes metabolise to odorous compounds. Diet can affect gut microflora, faecal microflora, litter moisture content, pH and water activity, all of which may affect the emission of odorants. This review details the role of diets on odour emission from broiler production. In the first part of this review, key odorants from broiler production, their origin, and measurement techniques have been discussed. This is followed by the role of feed ingredients, enzymes, feed additives, minerals, dietary protein levels, necrotic enteritis and litter conditions on odour emissions. It has been reported that nutritional strategies such as partial replacement of soybean meal with meat meal in the diet, use of a low sulphur diet, low protein diet, Bacillus subtilis based probiotic and saponin may reduce emissions. Additionally, drying the litter results in lower emission of odorants including the ones that contain sulphur and prevention of necrotic enteritis and wet litter condition may also reduce odour emission from broiler production.
To describe our experience and provide guidelines for maximum safe balloon sizes according to age in children undergoing balloon dilatation.
A retrospective review was conducted of children undergoing balloon dilatation for subglottic stenosis in a paediatric tertiary unit between May 2006 and February 2016.
A total of 166 patients underwent balloon dilatation. Mean ( ± standard deviation) patient age was 4.5 ± 3.99 years. The median balloon size was 8 mm, the median balloon inflation pressure was 10 atm, and the mean balloon inflation time was 65.1 ± 18.6 seconds. No significant unexpected events occurred. The Pearson correlation co-efficient for the relationship between patient age and balloon size was 0.85 (p = 0.001), suggesting a strongly positive correlation.
This study demonstrated that balloon dilatation is a safe procedure for airway stenosis. The results suggest using a balloon diameter that is equal to the outer diameter of the age-appropriate endotracheal tube +1 mm for the larynx and subglottis and +2 mm for the trachea.
Lower and middle income countries (LMICs) are home to >80% of the global population, but mental health researchers and LMIC investigator led publications are concentrated in 10% of LMICs. Increasing research and research outputs, such as in the form of peer reviewed publications, require increased capacity building (CB) opportunities in LMICs. The National Institute of Mental Health (NIMH) initiative, Collaborative Hubs for International Research on Mental Health reaches across five regional ‘hubs’ established in LMICs, to provide training and support for emerging researchers through hub-specific CB activities. This paper describes the range of CB activities, the process of monitoring, and the early outcomes of CB activities conducted by the five research hubs.
The indicators used to describe the nature, the monitoring, and the early outcomes of CB activities were developed collectively by the members of an inter-hub CB workgroup representing all five hubs. These indicators included but were not limited to courses, publications, and grants.
Results for all indicators demonstrate a wide range of feasible CB activities. The five hubs were successful in providing at least one and the majority several courses; 13 CB recipient-led articles were accepted for publication; and nine grant applications were successful.
The hubs were successful in providing CB recipients with a wide range of CB activities. The challenge remains to ensure ongoing CB of mental health researchers in LMICs, and in particular, to sustain the CB efforts of the five hubs after the termination of NIMH funding.
A novel method to estimate unsteady aerodynamic force coefficients from pointwise velocity measurements is presented. As opposed to other existing methodologies, time-resolved full velocity fields are not required. The methodology is based on a resolvent-based reduced-order model which requires the mean flow to obtain physical flow structures and pointwise measurement to calibrate their amplitudes. A computationally affordable time-stepping methodology to obtain resolvent modes in non-trivial flow domains is introduced and compared with previous existing matrix-free and matrix-forming strategies. The technique is applied to the unsteady flow around an inclined square cylinder at low Reynolds number. The potential of the methodology is demonstrated through good agreement between the fluctuating pressure distribution on the cylinder and the temporal evolution of the unsteady lift and drag coefficients predicted by the model and those computed by direct numerical simulation.
The State of Qatar experienced a sandstorm on the night of April 1, 2015, lasting approximately 12 hours, with winds of more than 100 km/h and average particulate matter of approximately 10 μm in diameter. The emergency department (ED) of the main tertiary hospital in Qatar managed 62% of the total emergency calls and those of higher triage order. The peak load of patients during the event manifested approximately 6 hours after the onset. The Major Emergency Command Centre of the hospital ensured the department was maximally organized in terms of disaster management, and established protocols were brought into action. Multiple timely meetings were convened in efforts to effectively execute plans that included rapid emergency medical services handover time, resourcing staff, maximizing bed space, preventing dust entry in the ED, bypassing certain administrative processes, canceling day-surgeries that did not affect inpatient morbidity, and procuring additional respiratory equipment. Patients arrived mainly with exacerbations of asthma and respiratory distress, ophthalmic emergencies, and vehicular trauma; surprisingly, the incidence of pedestrian injuries did not vary. (Disaster Med Public Health Preparedness. 2017;11:227–238)
Crimean-Congo haemorrhagic fever (CCHF) is an emerging zoonotic disease in India which is prevalent in neighbouring countries. CCHF virus (CCHFV) is a widespread tick-borne virus which is endemic in Africa, Asia, Eastern Europe and the Middle East. In the present study, samples of clinically suspected human cases from different areas of northern-western India were tested for the presence of CCHFV by RT–PCR through amplification of nucleocapsid (N) gene of CCHFV. Positive samples were sequenced to reveal the prevailing CCHFV genotype(s) and phylogenetic relatedness. A phylogenetic tree revealed the emergence of diverse strains in the study region showing maximum identity with the Pakistan, Afghanistan and Iran strains, which was different from earlier reported Indian strains. Our findings reveal for the first time the emergence of the Asia 1 group in India; while earlier reported CCHFV strains belong to the Asia 2 group.