To send content items to your account,
please confirm that you agree to abide by our usage policies.
If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account.
Find out more about sending content to .
To send content items to your Kindle, first ensure email@example.com
is added to your Approved Personal Document E-mail List under your Personal Document Settings
on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part
of your Kindle email address below.
Find out more about sending to your Kindle.
Note you can select to send to either the @free.kindle.com or @kindle.com variations.
‘@free.kindle.com’ emails are free but can only be sent to your device when it is connected to wi-fi.
‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.
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.
Background: Pediatric craniocerebral gunshot wounds occur in the context of both accidental and intentional trauma. Unique physiologic factors merited research into prognostic factors and treatment priorities in the pediatric population. Methods: A systematic search of MEDLINE, EMBASE, Web of Science, LILACS, Cochrane Registered Trials and Systematic Reviews, ISRCTN, and ClinicalTrials.gov was conducted. Selection criteria included all studies published in any language since 2000 which described intracranial isolated gunshot wounds in a civilian individual or population of pediatric age. Post-mortem and epidemiological studies were excluded. Screening was conducted through Covidence. Results: Initial database search revealed 349 unique studies for abstract and title screening. Fifty studies were selected for full text screening. Nine studies were included in the final review. Study quality was assessed with the Newcastle-Ottawa Scale. Case series noted bullet migration, pituitary deficiency, neurovascular and neuropsychological concerns. Three single-center retrospective studies of 71, 30, and 48 pediatric patients suggested multiple negative prognostic signs on initial presentation. Early aggressive surgical treatment was recommended by some authors. Conclusions: This systematic review analyzed the best current understanding of evidence for prognostic factors and treatment considerations of intracranial gunshot wounds in the pediatric neurotrauma context. Areas for future research with larger multi-center studies were highlighted.
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.
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 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.
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.
Bagasse and rice husk are two important agro-industrial by-products that are used as fuel in the sugar and rice mill industries, thus producing large quantities of bagasse ash (BA; 0·05 of bagasse) and rice husk ash (0·20 of rice husk) as waste material. Applying BA and rice husk ash (RHA) to agricultural land improves yield, nutrient uptake and chemical fertility of soil, particularly with special reference to available phosphorus (P) and potassium (K). The present field experiment was conducted for 3 years to evaluate the P fertilizer value of these agro-industrial waste materials in a wheat–rice system (WRS). The experiment was laid out in a split-plot design with RHA and BA applied at 10 t/ha and including a no-amendment control as the main plot treatments and three levels of fertilizer P (0, 13 and 26 kg P/ha; designated P0, P13 and P26, respectively) as sub-plot treatments to wheat in WRS. Application of fertilizer P increased the wheat grain yield up to P26 in the un-amended control treatment. However, a significant response of wheat to fertilizer P was also observed up to P13 in the presence of BA and RHA, thereby saving 50% of fertilizer P. Both RHA and BA increased wheat productivity by 12 and 16%, respectively, over the un-amended control. The subsequent rice crop also produced 14% higher paddy yield when the two ashes were applied along with P13 to the previous wheat crop. The increases in grain yield were accompanied by significant increases in the uptake of P and K, and P content (Olsen P) in the soil. The application of recommended P (P26) in un-amended plots resulted in a negative P balance of 9·3 kg P/ha/year. On the other hand, the application of BA alone and RHA along with P13 resulted in neutral/slightly positive P balance. A strong linear relationship (R2 = 0·98) was observed between P balance and Olsen-P build up in the soil. It may be concluded that application of BA and RHA has the potential to increase system productivity and reduce the cost of inputs in terms of reduced application of fertilizer P to wheat and rice.
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)
A novel reduced-order model for time-varying nonlinear flows arising from a resolvent decomposition based on the time-mean flow is proposed. The inputs required for the model are the mean-flow field and a small set of velocity time-series data obtained at isolated measurement points, which are used to fix relevant frequencies, amplitudes and phases of a limited number of resolvent modes that, together with the mean flow, constitute the reduced-order model. The technique is applied to derive a model for the unsteady three-dimensional flow in a lid-driven cavity at a Reynolds number of 1200 that is based on the two-dimensional mean flow, three resolvent modes selected at the most active spanwise wavenumber, and either one or two velocity probe signals. The least-squares full-field error of the reconstructed velocity obtained using the model and two point velocity probes is of the order of 5 % of the lid velocity, and the dynamical behaviour of the reconstructed flow is qualitatively similar to that of the complete flow.
Background: Burst-suppression is an electroencephalographic pattern observed during coma and reflects severe encephalopathy. We investigated the reactivity of burst-suppression to photic stimulation in children with acquired brain injury. Methods: Intensive care unit electroencephalographic monitoring recordings containing burst-suppression were obtained from 5 comatose children with acquired brain injury of various etiologies. Intermittent photic stimulation was performed at 1 Hz for 1 minute to assess reactivity. We quantified reactivity by measuring the change in the burst ratio (fraction of time in burst) following photic stimulation. Results: Photic stimulation evoked bursts in all patients, resulting in a transient increase in the burst ratio, while the mean heart rate remained unchanged. The regression slope of the change in burst ratio, referred to as the standardized burst ratio reactivity, correlated with subjects’ Glasgow Coma Scale scores. Conclusions: Reactivity of the burst-suppression pattern to photic stimulation occurs across diverse coma etiologies. Standardized burst ratio reactivity appears to reflect coma severity. Measurement of burst ratio reactivity may represent a simple bedside tool to monitor coma severity in critically ill children.
The effect of streamwise-varying steady transpiration on turbulent pipe flow is examined using direct numerical simulation at fixed friction Reynolds number
. The streamwise momentum equation reveals three physical mechanisms caused by transpiration acting in the flow: modification of Reynolds shear stress, steady streaming and generation of non-zero mean streamwise gradients. The influence of these mechanisms has been examined by means of a parameter sweep involving transpiration amplitude and wavelength. The observed trends have permitted identification of wall transpiration configurations able to reduce or increase the overall flow rate
, respectively. Energetics associated with these modifications are presented. A novel resolvent formulation has been developed to investigate the dynamics of pipe flows with a constant cross-section but with time-mean spatial periodicity induced by changes in boundary conditions. This formulation, based on a triple decomposition, paves the way for understanding turbulence in such flows using only the mean velocity profile. Resolvent analysis based on the time-mean flow and dynamic mode decomposition based on simulation data snapshots have both been used to obtain a description of the reorganization of the flow structures caused by the transpiration. We show that the pipe flows dynamics are dominated by a critical-layer mechanism and the waviness induced in the flow structures plays a role on the streamwise momentum balance by generating additional terms.
To investigate the assessment and management of paediatric snoring and obstructive sleep apnoea in UK otolaryngology departments.
A telephone questionnaire survey of UK otolaryngology departments was conducted over a 16-week period.
The response rate was 61 per cent (85 out of 139 trusts). Use of pre-operative pulse oximetry was reported by 84 per cent of respondents, mainly to diagnose obstructive sleep apnoea (73 per cent) or stratify post-operative risk (46 per cent). Thirty-one per cent of respondents reported using post-operative pulse oximetry. Twenty-five per cent of respondents have a dedicated management protocol for paediatric obstructive sleep apnoea and snoring. Thirty-four per cent require prior clinical commissioning group approval before performing surgery. Fifty-eight per cent of respondents reported following up their obstructive sleep apnoea patients after surgery. The mean follow-up period (±standard deviation) was 6.8 ± 1.2 weeks.
There is variation in the assessment and management of paediatric snoring and obstructive sleep apnoea across the UK, particularly in the use of pre- and post-operative pulse oximetry monitoring, and further guidelines regarding this are necessary.
In search of a suitable resource conservation technology under pigeonpea (Cajanus cajan L.)–wheat (Triticum aestivum L.) system in the Indo-Gangetic Plains, the effects of conservation agriculture (CA) on crop productivity and water-use efficiency (WUE) were evaluated during a 3-year study. The treatments were: conventional tillage (CT), zero tillage (ZT) with planting on permanent narrow beds (PNB), PNB with residue (PNB + R), ZT with planting on permanent broad beds (PBB) and PBB + R. The PBB + R plots had higher pigeonpea grain yield than the CT plots in all 3 years. However, wheat grain yields under all plots were similar in all years except for PBB + R plots in the second year, which had higher wheat yield than CT plots. The contrast analysis showed that pigeonpea grain yield of CA plots was significantly higher than CT plots in the first year. However, both pigeonpea and wheat grain yields during the last 2 years under CA and CT plots were similar. The PBB + R plots had higher system WUE than the CT plots in the second and third years. Plots under CA had significantly higher WUE and significantly lower water use than CT plots in these years. The PBB + R plots had higher WUE than PNB + R and PNB plots. Also, the PBB plots had higher WUE than PNB in the second and third years, despite similar water use. The interactions of bed width and residue management for all parameters in the second and third years were not significant. Those positive impacts under PBB + R plots over CT plots were perceived to be due to no tillage and significantly higher amount of estimated residue retention. Thus, both PBB and PBB + R technologies would be very useful under a pigeonpea–wheat cropping system in this region.
To assess the efficacy of endoscopic-guided botulinum toxin injection into the cricopharyngeus muscle and evaluate the duration of its effects.
A 3-year prospective study of 12 patients undergoing injection of botulinum toxin was conducted, with a telephone survey to assess dysphagia pre-operatively, and at 1, 3 and 6 months post-treatment, using the MD Anderson Dysphagia Inventory.
Median age was 66.2 years. Causes of cricopharyngeal dysphagia included idiopathic cricopharyngeal hypertrophy (67 per cent), previous cerebrovascular accident (17 per cent), cranial nerve palsy (8 per cent) and previous chemoradiotherapy to the neck (8 per cent). There were no complications. Two patients had repeat injections after six months. There was significant improvement in MD Anderson Dysphagia Inventory scores at one and three months versus pre-operative scores (73.1 ± 14.9 vs 46.9 ± 7.6, p = 0.0001, and 65.1 ± 11.5 vs 46.9 ± 7.6, p = 0.0001), but not at six months (51.0 ± 11.0 vs 46.9 ± 7.6, p = 0.14).
Endoscopic-guided injection of botulinum toxin into the cricopharyngeus muscle is a safe and effective method for treating cricopharyngeal muscle dysfunction, lasting up to six months.
To evaluate the effect of body mass index and neck length on endotracheal tube movement during neck extension in thyroidectomy.
A prospective study was conducted of 30 patients undergoing thyroidectomy during an 8-month period. Patient characteristics were recorded and endotracheal tube displacement was determined.
Mean body mass index was 27.8 kg/m2 (range, 17.5–34.7 kg/m2) and mean neck circumference was 43.2 cm (range, 28–56 cm). The mean (± standard deviation) upward displacement of the endotracheal tube during neck extension was 7.17 ± 5.87 mm. Patients with a larger body mass index had a significantly greater amount of tube displacement (R2 = 0.67, p < 0.0001), as did patients with a smaller neck length (R2 = 0.48, p < 0.0001).
Neck extension results in upward displacement of the endotracheal tube. The amount of displacement is significantly higher in patients with a larger body mass index or shorter neck length. This has particular relevance for nerve monitoring in thyroidectomy.
This study aimed to measure changes in disease-specific quality of life in children following tonsillectomy or adenotonsillectomy.
A multicentre prospective cohort study was performed involving seven ENT departments in England. A total of 276 children entered the study over a 2-month period: 107 underwent tonsillectomy and 128 adenotonsillectomy. Forty-one children referred with throat problems initially managed by watchful waiting were also recruited. The follow-up period was 12 months. Outcome measures were the T14, parental impressions of their child's quality of life and the number of days absent from school.
One-year follow-up data were obtained from 150 patients (52 per cent). The mean baseline T14 score in the non-surgical group was significantly lower (T14 = 23) than in the tonsillectomy group (T14 = 31) or the adenotonsillectomy group (T14 = 35; p < 0.001). There was a significant improvement in the T14 scores of responders in all groups at follow up. The effect size was 1.3 standard deviations (SD) for the non-surgical group, 2.1 SD for the tonsillectomy group and 1.9 SD for the adenotonsillectomy group. Between-group differences did not reach statistical significance. A third of children in the non-surgical group underwent surgery during the follow-up period.
Children who underwent surgical intervention achieved a significant improvement in disease-specific quality of life. Less severely affected children were managed conservatively and also improved over 12 months, but 1 in 3 crossed over to surgical intervention.
This study assessed the use of pulse oximetry testing in children with suspected obstructive sleep apnoea in a hospital setting.
A retrospective review of patients who underwent pulse oximetry testing between April 2013 and October 2013 was performed. Primary outcome measures included positive pulse oximetry results, defined as a McGill oximetry score of 2–4.
Thirty-seven test results were usable for analysis: from 21 pre- and 16 post-operative tests. Only four patients had positive test results. There was a significant difference between pre- and post-operative quality of life outcome scores in the surgical group (p < 0.0001).
Pre-operative pulse oximetry should be used as a guide to help triage patients who require specialist paediatric services, such as a paediatric intensive care unit. The use of pulse oximetry, particularly in the post-operative setting, is unlikely to change patient management and can incur unnecessary financial costs to UK National Health Service Hospital Trusts.
Iron (Fe) and zinc (Zn) contents in hexaploid wheat are very low and are further reduced because of the removal of micronutrient-rich bran of wheat grains during milling and processing. Therefore, hexaploid wheat, its wild species and wheat–Aegilops kotschyi substitution lines were evaluated to identify the genome(s) carrying gene(s) for high Fe and Zn concentrations in bran and endosperm fractions of grains. It is reflected from the results that Triticum monococcum (acc. W463) may serve as a promising donor for biofortification of Fe, and Aegilops speltoides (acc. 3804) may serve as a promising donor for biofortification of Zn in the endosperm of cultivated wheat. Further, among the three wheat–Ae. kotschyi substitution lines, the higher concentration of Fe and Zn in endosperm fraction was observed in BC2F4 63-2-13-1. The work on precise transfer of useful gene(s) from 7Uk chromosome of this line is in progress to reduce linkage drag.