bluuebunny/crossref_metadata_2025_split huggingface dataset:
Late diagnosis and atypical brain imaging of Aicardi–Goutières syndrome: are we failing to diagnose Aicardi–Goutières syndrome‐2?
Authors: Svingen Leah , Goheen Mitchell , Godfrey Rena , Wahl Colleen , Baker Eva H , Gahl William A , Malicdan May Christine V , Toro Camilo 
Published: August 2017

Aicardi–Goutières syndrome (AGS) is a rare disorder with in utero or postnatal onset of encephalopathy and progressive neurological deterioration. The seven genetic subtypes of AGS are associated with abnormal type I interferon‐mediated innate immune response. Most patients with AGS present with progressive microcephaly, spasticity, and cognitive impairment. Some, especially those with type 2 (AGS2), manifest milder phenotypes, reduced childhood mortality, and relative preservation of physical and cognitive abilities. In this report, we describe two siblings (sister and brother) diagnosed with AGS2 in their second decade, who exhibited static encephalopathy since 1 year of age with spastic quadriplegia and anarthria but preserved intellect. Both were homozygous for the common pathogenic RNASEH2B allele (c.529G>A, p.Ala177Thr). Rather than manifesting calcifications and leukoencephalopathy, both had increased iron signal in the basal ganglia. Our report broadens the clinical and imaging spectrum of AGS2 and emphasizes the importance of including AGS2 in the differential diagnosis of idiopathic spastic cerebral palsy.What this paper adds

We identified two siblings (sister and brother) with atypical Aicardi–Goutières syndrome type 2 due to RNASEH2B mutation. Manifestations included spastic quadriplegia and anarthria but preserved intellect and increased iron signal in the basal ganglia.

RNASEH2B‐related Aicardi–Goutières syndrome type 2 can have present with a variable phenotype, including idiopathic spastic cerebral palsy.
Proportion of daily capillary blood glucose readings required in the target range for target glycaemic control: shift of focus from target range to proportion in range
Authors: Sivasubramaniyam S. , Amiel S. A. , Choudhary P. 
Published: August 2017

AbstractAimMost guidelines provide people with Type 1 diabetes with pre‐ and post‐meal capillary blood glucose (CBG) targets to achieve optimal glycaemic control. We evaluated the proportion of daily CBG readings between 4 and 10 mmol/l in people achieving different HbA1c levels.MethodWe analysed CBG data from routine pump/meter downloads from 201 adults treated with continuous subcutaneous insulin infusion (CSII) at a single hospital clinic. Exclusion criteria were CSII < 6 months, < 3 CBG/day, pregnancy, haemoglobinopathy and continuous sensor use. People were categorized into three groups based on HbA1c: < 58 mmol/mol, < 7.5% (n = 58); 58–74 mmol/mol, 7.5–8.9% (n = 107); and ≥ 75 mmol/mol, ≥ 9.0% (n = 36).ResultsParticipants had a mean age of 43 ± 13 years and mean HBA1c of 64 mmol/mol (8.0 ± 1.1%). 47% of people started CSII for raised HbA1c, 25% due to hypoglycaemia and the rest during pregnancy. Downloads contained a mean of 22 ± 6.8 days of data per participant. CBG frequency was similar between the three groups (5.6 ± 2.0, 5.6 ± 1.9 and 5.4 ± 1.2 CBG/day; P = 0.468). The proportion of CBG readings between 4 and 10 mmol/l (72–180 mg/dl) was 57.3 ± 25.4%, 50.6 ± 11.1% and 39.9 ± 16.5% (P < 0.0001); < 4 mmol was 13.8%, 8.8% and 4.4% (P < 0.0001) and > 10 mmol/l was 28.9 ± 16.5%, 40.6 ± 12.1% and 55.6 ± 17.9% (P < 0.0001) in the three groups respectively.ConclusionsParticipants achieving HBA1c < 58 mmol/mol (< 7.5%) had ~ 60% of CBG readings in range (4–10 mmol/l), with up to 30% of readings > 10 mmol/l. This target of achieving 60% or more readings within target, and being permissive with up to 30% readings > 10 mmol/l may be a novel target for people with diabetes, and may reduce anxiety associated with readings out of range.
Cross‐infectivity of oil palm by Phytophthora spp. isolated from perennial crops in Malaysia
Authors: Latifah M. , Zainal Abidin M.A. , Kamaruzaman S. , Nusaibah S. A. 
Published: August 2017

SummaryBud rot disease or “Pudricion del cogollo” (PC) of oil palm is a major constraint on production in Colombia and neighbouring countries such as Brazil, Costa Rica, Ecuador, Nicaragua, Panama, Peru and Surinam. To date, there are no documented reports of Phytophthora disease of oil palm in South‐East Asia. This research, therefore, was conducted to determine the pathogenic potential of Phytophthora palmivora and Phytophthora nicotianae on oil palm using both in vitro and nursery inoculation experiments. In vitro inoculation of both P. palmivora and P. nicotianae on immature oil palm leaflets caused discoloration within 2 days of inoculation and incubation at 25 ± 1.5°C, 100% RH. Similarly, in nursery trials, lesions formed on the buds (unopened leaflets) 3 days after inoculation with P. palmivora or P. nicotianae zoospore suspensions. No lesions developed on untreated leaflets in either in vitro or nursery inoculation experiments. Phytophthora spp. were re‐isolated from leaflet lesions and confirmed as the inoculated pathogens.
A comparative study of brachial plexus sonography and magnetic resonance imaging in chronic inflammatory demyelinating neuropathy and multifocal motor neuropathy
Authors: Goedee H. S. , Jongbloed B. A. , van Asseldonk J.‐T. H. , Hendrikse J. , Vrancken A. F. J. E. , Franssen H. , Nikolakopoulos S. , Visser L. H. , van der Pol W. L. , van den Berg L. H. 
Published: August 2017

Background and purposeTo compare the performance of neuroimaging techniques, i.e. high‐resolution ultrasound (HRUS) and magnetic resonance imaging (MRI), when applied to the brachial plexus, as part of the diagnostic work‐up of chronic inflammatory demyelinating neuropathy (CIDP) and multifocal motor neuropathy (MMN).MethodsFifty‐one incident, treatment‐naive patients with CIDP (n = 23) or MMN (n = 28) underwent imaging of the brachial plexus using (i) a standardized MRI protocol to assess enlargement or T2 hyperintensity and (ii) bilateral HRUS to determine the extent of nerve (root) enlargement.ResultsWe found enlargement of the brachial plexus in 19/51 (37%) and T2 hyperintensity in 29/51 (57%) patients with MRI and enlargement in 37/51 (73%) patients with HRUS. Abnormal results were only found in 6/51 (12%) patients with MRI and 12/51 (24%) patients with HRUS. A combination of the two imaging techniques identified 42/51 (83%) patients. We found no association between age, disease duration or Medical Research Council sum‐score and sonographic nerve size, MRI enlargement or presence of T2 hyperintensity.ConclusionsBrachial plexus sonography could complement MRI in the diagnostic work‐up of patients with suspected CIDP and MMN. Our results indicate that combined imaging studies may add value to the current diagnostic consensus criteria for chronic inflammatory neuropathies.
Hydrodynamics and seed dispersal in the lower Amazon
Authors: da Cunha Alan Cavalcanti , Mustin Karen , dos Santos Eldo Silva , dos Santos Éwerton Wânderson Gonçalves , Guedes Marcelino Carneiro , Cunha Helenilza Ferreira Albuquerque , Rosman Paulo Cesar Colonna , Sternberg Leonel da Silveira Lobo 
Published: August 2017

Abstract

The transport of seeds by water, i.e. hydrochory, is a key mechanism of long‐distance dispersal constrained by the attributes of the seed and hydrodynamics. In the freshwater Amazon estuary, river hydrodynamics are influenced by seasonal changes in precipitation and tidal cycles. It is not known to what extent a hydrodynamic model may be able to predict seed dispersal.

Here we parameterised a simulation model (SisBaHia) to estimate maximum seed dispersal distances per tidal cycle, which were then compared with data from in situ seed dispersal experiments. The study was conducted along a 27‐km stretch of a tributary of the Amazon estuary, using the seeds of a widely distributed riparian tree—Carapa guianensis (Meliaceae).

Based on the simulation model, maximum potential seed dispersal distance was higher in the rainy season (c. 8.7 km) when compared with the dry season (5.6 km), for one tidal cycle (12 hr). The seeds of C. guianensis were dispersed further during the ebb than flood tide during the rainy season, the period of seed dispersal. Average dispersal distances observed in seed dispersal experiments conducted during the rainy season, and those predicted by SisBaHia for the same period were within the same order of magnitude.

The results of this study confirm that the period of higher precipitation provides favourable hydrological conditions for hydrochory in the Amazon river‐estuary complex. The fact that the time taken for the tide to fall is longer in relation to the time taken for it to rise also favours the arrival of the seeds in the main channel of the Amazon, thereby increasing the probability of long‐distance dispersal events.
Western medicine in Africa, part II: Ethiopia, East and Southern Africa to 1900
Authors: Roberts Jonathan 
Published: August 2017

AbstractBefore 1900, Western medicine struggled to attract patients in Africa. As part 2 of the history of European medical practice in Africa before 1900, this article describes how practitioners from Europe tried to win over patients in Ethiopia and East and Southern Africa, regions that already had pluralistic healing networks of herbal and spiritual therapies. Western medicine began at a disadvantage because it could offer little to combat the diseases of the tropics or fight epidemics sweeping the continent from Asia. However, over the course of several centuries, emissaries, missionaries, and explorers from Europe were able to bring a unique set of European healing ideas, practices, and medicines to the continent with them. African patients showed skepticism towards practices that they regarded as overly invasive, such as surgery at hospitals, but they were open to experimenting with pharmaceuticals as treatments for illnesses for which they had no cure. Western medicine did not “save” the people of Africa nor did it achieve hegemony, but by 1900, it was able to gain a footing on the Horn of Africa, on the coast of East Africa, and throughout Southern Africa.
Adipokines, psoriasis, systemic inflammation, and endothelial dysfunction
Authors: Lynch Maeve , Ahern Tomas , Sweeney Cheryl M. , Malara Anna , Tobin Anne‐Marie , O'Shea Donal , Kirby Brian 
Published: August 2017

AbstractAdipokines are secreted by white adipose tissue, an active endocrine organ, and play a role in the regulation of metabolic functions such as lipid metabolism, inflammation, and vascular homeostasis. Adipokines are secreted in excess in obesity and contribute to the development of associated comorbidities such as metabolic syndrome and atherosclerosis. Psoriasis, a chronic immune‐mediated skin disease, is associated with obesity and increased cardiovascular risk. Understanding the role of adipokines in psoriasis may in part explain the association between psoriasis and cardiovascular disease. This review summarizes the data regarding key adipokines in patients with psoriasis and the change in adipokine profiles with psoriasis therapy. Adipokines may be mediators of cutaneous inflammation suggesting a role in the pathophysiology of psoriasis and the development of comorbidities.
Postural change in convulsive seizures: a retrospective review of video‐electroencephalographic recordings
Authors: Rigg Jane , Irwin Kimberley , Tremayne Fred , Reutens David 
Published: August 2017

AbstractBackgroundProne position has been identified as an important risk factor for sudden unexpected death in epilepsy raising the possibility of avoidance of this posture in sleep as a preventative measure.AimsTo evaluate the potential utility of prone posture position, we studied patterns of postural change during generalised tonic clonic seizures.MethodsVideo‐electroencephalographic recordings of patients undergoing investigations at the Royal Brisbane and Women's Hospital between 2005 and 2013 were reviewed independently by two raters. Head and truncal positions (left and right lateral, supine, sitting, prone) at seizure onset and offset, before and after nurse interventions were recorded. Post‐ictal postural changes and evidence of stertorous respirations were also recorded.ResultsThirty‐one seizures from 27 patients were included in the study. One seizure began with the patient asleep in the prone position. One patient became prone during a seizure, having previously been asleep in the left lateral position. Nine patients changed position during a seizure. Seven of these patients were sitting or in a lateral position at the time of seizure onset, two patients were supine at seizure onset. No patient rolled by more than 90° during a seizure. Post‐ictal stertorous respirations were observed in 14 patients, one of whom was prone.ConclusionThe incidence of patients attaining a prone position during a seizure was low. Given that no patient rolled more than 90°, patients are least likely to attain a prone position if they are supine at the beginning of a seizure.
Associations between Maternal Behaviors at 1 Year and Child Language at 2 Years in a Cohort of Women Experiencing Adversity
Authors: Smith Jodie , Levickis Penny , Eadie Tricia , Bretherton Lesley , Conway Laura , Goldfeld Sharon 
Published: August 2017

Variations in parenting have been suggested as contributing to a higher prevalence of language difficulties in children experiencing economic, environmental, and social adversity. Within these cohorts, the contribution of responsive and intrusive parenting to child language has been investigated; specific responsive and intrusive behaviors encapsulated within these parenting styles have yet to be fully examined. Additionally, the role of the mother–child dynamic in moderating mother–child associations has also not been explored. This study aimed to augment current research by identifying specific responsive and intrusive maternal behaviors associated with child language in a cohort experiencing adversity, as well as exploring the role of the fluency and connectedness of mother–child conversation in moderating associations (n = 249). Specific behaviors and the fluency and connectedness of the mother–child interaction were coded from free‐play videos at child age 12 months. Child language measures were derived from transcripts of free play at 24 months. Linear regression models were used to examine maternal–child associations. The moderating role of fluency and connectedness was then explored. Maternal imitations were positively associated with the child's total words; successful redirectives were negatively associated with the child's mean length of turn. Both associations were moderated by the fluency and connectedness of the interaction.
Policy choices in dementia care—An exploratory analysis of the Alberta continuing care system (ACCS) using system dynamics
Authors: Cepoiu‐Martin Monica , Bischak Diane P. 
Published: August 2017

AbstractBackgroundThe increase in the incidence of dementia in the aging population and the decrease in the availability of informal caregivers put pressure on continuing care systems to care for a growing number of people with disabilities. Policy changes in the continuing care system need to address this shift in the population structure. One of the most effective tools for assessing policies in complex systems is system dynamics. Nevertheless, this method is underused in continuing care capacity planning.MethodsA system dynamics model of the Alberta Continuing Care System was developed using stylized data. Sensitivity analyses and policy evaluations were conducted to demonstrate the use of system dynamics modelling in this area of public health planning. We focused our policy exploration on introducing staff/resident benchmarks in both supportive living and long‐term care (LTC).ResultsThe sensitivity analyses presented in this paper help identify leverage points in the system that need to be acknowledged when policy decisions are made. Our policy explorations showed that the deficits of staff increase dramatically when benchmarks are introduced, as expected, but at the end of the simulation period, the difference in deficits of both nurses and health care aids are similar between the 2 scenarios tested.Modifying the benchmarks in LTC only versus in both supportive living and LTC has similar effects on staff deficits in long term, under the assumptions of this particular model.ConclusionThe continuing care system dynamics model can be used to test various policy scenarios, allowing decision makers to visualize the effect of a certain policy choice on different system variables and to compare different policy options. Our exploration illustrates the use of system dynamics models for policy making in complex health care systems.
Clinical and economic evaluation of a Case Management Service for patients with back pain
Authors: Smith Judith , Yeowell Gillian , Fatoye Francis 
Published: August 2017

AbstractAimTo evaluate the clinical‐effectiveness and resource use associated with Case Management Services in a UK private sectorDesignAn observational cohort study based on prospective data collection of patient reported outcome measures and data collection from an existing administrative health database.SettingA United Kingdom (UK) private healthcare providerParticipantsConsecutive referrals into the Case Management Service over a three‐month period.InterventionsParticipants were managed within the Case Management Service in accordance with usual pathways.Main Outcome MeasuresOutcome measures included EQ‐5D‐5L and PSFS. Measurements were taken at baseline, and repeated at the point at which the client's case was closed. The CARE measure examined patient reported experience.Cost Consequence analysis was completed using existing data for the same period of time in two separate years: 2014, two years after CMS implementation, and 2011, prior Case Management Service developmentResultsThere was a statistically significant improvement in quality of life (EQ‐5D‐5L p < 0.0001; EQ‐VAS p < 0.001) and functional ability (p < 0.001) following the Physiotherapy Case Management. The CARE measure showed high levels of patient satisfaction with 96% of clients rating their individual case manager as good to excellent. The cost consequences analysis showed a cost reduction in therapy resource utilisation by £252 842 (from £10 772 875 to £10 520 034).ConclusionsThe Case Management Service showed the provision to be providing significant improvement in quality of life and functional outcomes within efficient use of resources, and service users are highly satisfied with their experience.
Knowledge and practice of, and attitudes toward, pulp therapy in deciduous teeth among pediatric dentists in India
Authors: Nayak Ullal A. , Wadhwa Saakshe , Kashyap Nilotpol , Prajapati Deepesh , Mahuli Amit V. , Sharma Reena 
Published: August 2017

AbstractAimThe aim of the present study was to assess the knowledge and practice of, and attitudes toward, pulp therapy in deciduous dentition among pediatric dentists.MethodsA cross‐sectional, observational survey was conducted using a closed‐ended, multiple‐choice questionnaire evaluating the knowledge and practice of, and attitudes toward, pulp therapy in deciduous dentition, which was formulated and sent to 360 pediatric dentists across India. Descriptive statistics were done, followed by χ2‐test to test the association between years of experience and the questionnaire items.ResultsA total of 3.5% of pediatric dentists performed their treatment using a rubber dam in all cases; 30% preferred to use local anesthesia prior to indirect pulp therapy (IPT). Traditional indirect pulp capping was preferred to IPT, and only 48% of them believed in not removing it completely. Pulpotomy was preferred over IPT (70%) when there was a probability of pulp exposure following complete caries excavation. Calcium hydroxide, along with iodoform, was the material of choice for the obturation of primary teeth (73.5%). A stainless steel crown was placed after pulp therapy in the primary tooth (86.3%). Most dentists believed requested immediate and 3‐month follow up.ConclusionThe survey helps in assessing whether our views or approaches are in line with recent trends.
White spot lesions: Does etching really matter?
Authors: Abufarwa Moufida , Voorhees Robert D. , Varanasi Venu G. , Campbell Phillip M. , Buschang Peter H. 
Published: August 2017

AbstractAimThe clinical significance of acid etching prior to orthodontic bonding is controversial. In the present study, we evaluated the effect of 15 seconds of acid etching on enamel demineralization.MethodsTwenty‐seven human molars were sectioned and assigned to two groups. Under standardized conditions, the enamel surfaces were imaged using FluoreCam to obtain baseline data. Group 1 was etched using 37% phosphoric acid for 15 seconds, rinsed with water, and then imaged again; group 2 was only rinsed with water. Water rinse was collected for calcium chemical analysis using inductively‐coupled plasma auger electron spectrometry. Both groups were subjected to 9 days of pH cycling, after which final FluoreCam images were obtained.ResultsGroup 1 showed a significant increase in lesion area (P=.012), decrease in light intensity (P=.009), and decrease in impact (P=.007) after acid etching. The amount of calcium that leached out over the 15 seconds was 14 ppm ±2.4 (0.35 mmol/L±0.06). Following pH cycling, there was no statistically‐significant between‐group difference in overall enamel demineralization.ConclusionInitial demineralization caused by 15 seconds of acid etching does not increase enamel susceptibility to further demineralization. This suggests that acid etching does not increase the risk of developing white spot lesions during orthodontics.
How Do Quasi‐Random Option Grants Affect CEO Risk‐Taking?
Authors: SHUE KELLY , TOWNSEND RICHARD R. 
Published: August 2017

ABSTRACTWe examine how an increase in stock option grants affects CEO risk‐taking. The overall net effect of option grants is theoretically ambiguous for risk‐averse CEOs. To overcome the endogeneity of option grants, we exploit institutional features of multiyear compensation plans, which generate two distinct types of variation in the timing of when large increases in new at‐the‐money options are granted. We find that, given average grant levels during our sample period, a 10% increase in new options granted leads to a 2.8% to 4.2% increase in equity volatility. This increase in risk is driven largely by increased leverage.
Streptococcal toxic shock syndrome occurring in the third trimester of pregnancy: A case report
Authors: Irani Mohamad , McLaren Rodney , Savel Richard H. , Bogatyryova Oksana , Khoury‐Collado Fady 
Published: August 2017

AbstractGroup A streptococcal (GAS) toxic shock syndrome (TSS) is a rare life‐threatening illness. Most reported cases have occurred in the post‐partum period. Here, we report a rare case of a primigravid who developed GAS TSS in the third trimester. We also review the potential preventive measures and treatment modalities for this syndrome. A 29‐year‐old primigravid presented at 36 weeks' gestation with diarrhea, abdominal pain, fever, and fetal bradycardia. She underwent an emergency cesarean section and was subsequently diagnosed with GAS TSS. She had a complicated post‐partum course marked by a 3‐month hospital stay and major sequelae. Her infant died on post‐partum day 4. GAS TSS should be considered in the differential diagnosis of pregnant patients presenting with fever and rapid onset of septic shock. A consideration to treat GAS that is detected incidentally during routine screening for group B streptococcus is suggested.