bluuebunny/crossref_metadata_2025 huggingface dataset:
Rival Visions of Parsimony
Authors: Gunitsky Seva
Published: May 2019

“Parsimony” is a vague and divisive concept in political science. I identify three distinct but often conflated conceptions of parsimony. The aesthetic conception emphasizes a theory's elegance and clarity; the ontological conception, drawing upon the hard sciences, posits that the world is governed by simple fundamental laws. Neither applies in international relations theory or to social science more broadly. Instead, only the epistemological conception—abstracting from reality to highlight recurring patterns and build testable propositions—justifies parsimony. This view is not a naive simplification of the world but a self-conscious capitulation to its complexity. Though both critics and supporters of parsimony often do not distinguish among these three “visions,” doing so has important implications for how we think about evaluating theories.
Antitrust intent in an age of algorithmic nudging
Authors: Zingales Nicolo
Published: May 2019

This article revisits the role of intent for the purposes of establishing an abuse of dominance under EU competition law. It does so by reviewing cases where the outcome hinged on the existence of anticompetitive intent, and eliciting from that a set of guiding principles. The consistency with those principles of the recent decision in Google Shopping is then discussed. After a critical examination of the definition of abusive conduct used by the Commission and the extent to which it satisfies the intent requirement in antitrust, the broader implications for providers of algorithmic intermediation services are explored.

Two recommendations are offered to ameliorate the status quo. First, courts and competition authorities should only take into account a defendant’s subjective state of mind under a ‘qualified intent’ test: a test requiring proof of immediate, substantial and foreseeable anticompetitive effects arising from a purported conduct. Secondly, to constrain the scope of application of the actionable duty of algorithmic self-scrutiny, regulators should introduce a negligence-based ‘safe harbor’. This safe harbour, mimicking the specialized regime of intermediary liability for information society service providers, would enable continued investment and innovation in algorithmic services while also promoting adherence to cross-industry best practices in algorithmic design.
Can Attractive Sticky Traps Be Used to Protect Horses From the Bites of Stomoxys calcitrans (L.) (Diptera: Muscidae)
Authors: Tam Tracey L , Hogsette Jerome , TenBroeck Saundra
Published: May 2019

The stable fly, Stomoxys calcitrans (L.), is a bloodsucking ectoparasite that causes irritation and distress to livestock, wildlife, and humans. Both sexes are vicious blood-feeders that feed on a variety of animals. Optically attractive sticky traps have been used to capture stable flies, and some companies claim that sticky traps can protect animals from the bites of stable flies. To further investigate the protective ability of sticky traps, Home and Garden Mosquito (HGM) traps were selected for evaluations at the University of Florida Horse Teaching Unit (HTU). Broodmares coated with fluorescent dust were either tethered to a post in the center of a paddock or released untethered into a paddock. HGM sticky traps were placed at the four compass points and four selected distances from the paddock center to capture stable flies before (unmarked) or after (marked) they visited the horses. More than 40% of flies captured on traps placed closest to the horses were marked. This indicates that the traps did not prevent the flies from visiting the horses. A percentage of marked and unmarked stable flies showed signs of blood in their guts indicating recent feeding. For unknown reasons, the number of stable flies marked with Signal Green dust exceeded the numbers marked with other colors. Although the HGM traps caught ample numbers of stable flies, the traps did not prevent stable flies from feeding on the horses. More work is needed to determine optimal trap placement and densities required to maximize stable fly management with traps.
Mapping the stability of stellar rotating spheres via linear response theory
Authors: Rozier S , Fouvry J-B , Breen P G , Varri A L , Pichon C , Heggie D C
Published: May 2019

Rotation is ubiquitous in the Universe, and recent kinematic surveys have shown that early-type galaxies and globular clusters are no exception. Yet the linear response of spheroidal rotating stellar systems has seldom been studied. This paper takes a step in this direction by considering the behaviour of spherically symmetric systems with differential rotation. Specifically, the stability of several sequences of Plummer spheres is investigated, in which the total angular momentum, as well as the degree and flavour of anisotropy in the velocity space are varied. To that end, the response matrix method is customized to spherical rotating equilibria. The shapes, pattern speeds and growth rates of the systems’ unstable modes are computed. Detailed comparisons to appropriate N-body measurements are also presented. The marginal stability boundary is charted in the parameter space of velocity anisotropy and rotation rate. When rotation is introduced, two sequences of growing modes are identified corresponding to radially and tangentially biased anisotropic spheres, respectively. For radially anisotropic spheres, growing modes occur on two intersecting surfaces (in the parameter space of anisotropy and rotation), which correspond to fast and slow modes, depending on the net rotation rate. Generalized, approximate stability criteria are finally presented.
CNIT: a fast and accurate web tool for identifying protein-coding and long non-coding transcripts based on intrinsic sequence composition
Authors: Guo Jin-Cheng , Fang Shuang-Sang , Wu Yang , Zhang Jian-Hua , Chen Yang , Liu Jing , Wu Bo , Wu Jia-Rui , Li En-Min , Xu Li-Yan , Sun Liang , Zhao Yi
Published: May 2019

As more and more high-throughput data has been produced by next-generation sequencing, it is still a challenge to classify RNA transcripts into protein-coding or non-coding, especially for poorly annotated species. We upgraded our original coding potential calculator, CNCI (Coding-Non-Coding Index), to CNIT (Coding-Non-Coding Identifying Tool), which provides faster and more accurate evaluation of the coding ability of RNA transcripts. CNIT runs ∼200 times faster than CNCI and exhibits more accuracy compared with CNCI (0.98 versus 0.94 for human, 0.95 versus 0.93 for mouse, 0.93 versus 0.92 for zebrafish, 0.93 versus 0.92 for fruit fly, 0.92 versus 0.88 for worm, and 0.98 versus 0.85 for Arabidopsis transcripts). Moreover, the AUC values of 11 animal species and 27 plant species showed that CNIT was capable of obtaining relatively accurate identification results for almost all eukaryotic transcripts. In addition, a mobile-friendly web server is now freely available at http://cnit.noncode.org/CNIT.
Difficult-to-treat gout flares: eligibility for interleukin-1 inhibition in private practice is uncommon according to current EMA approval
Authors: Pascart Tristan , Norberciak Laurène , Ea Hang-Korng , Graf Sahara , Guggenbuhl Pascal , Lioté Frédéric
Published: May 2019

Objective

The objective was to determine the proportion of patients with difficult-to-treat or difficult-to-prevent acute gout attacks eligible for IL-1 inhibition.

Methods

Participants included in the French cross-sectional GOSPEL cohort (n = 1003 gout patients) were examined for contraindications and intolerance to standard of care (SoC) drugs of gout flares (colchicine, non-steroidal anti-inflammatory drugs and systemic glucocorticoids). Patients were classified as definitely eligible for first-line IL-1 inhibition (canakinumab) according to European summary of product characteristics (contraindications/intolerance to SoC and at least three flares per year) without any other anti-inflammatory options (contraindications/intolerance only), or potentially eligible (precaution of use). Eligibility to receive IL-1 during an on-going flare related to insufficient efficacy was assessed (second-line eligibility).

Results

Definite first-line eligibility for IL-1 therapy was found in 10 patients (1%) and contraindication to all SoC therapies in nine patients who had presented <3 flares in the past 12 months. At least precaution of use for SoC therapies was noted for 218/1003 patients (21.7%). Of 487 patients experiencing flares at baseline, 114 (23.4%) were still experiencing pain scored ⩾4/10 numeric scale on day 3, one of whom could not receive further SoC drugs. Only nine of them had three or more flares in the past year and were eligible for second-line IL-1 inhibition.

Conclusion

Despite significant numbers of patients without any SoC anti-inflammatory therapeutic options for gout flares, eligibility for IL-1 inhibition therapy according to current European approval is rare.
UNCERTAINTY OF MEASUREMENT IN THE RESPONSE TEST OF A THYROID MONITOR
Authors: Yunoki Akira
Published: May 2019

A test device consisting of an I-131 source, a phantom on a 3D moving table, and a NaI(Tl) scintillation detector on a fixed flat table was developed to carry out response tests of a thyroid monitor. A measurement uncertainty due to variation of configuration of the source and the detector, as well as other factors, was estimated. The estimated relative standard uncertainty of measurement at a source–detector distance of 100 mm, which was deduced to be the optimum, was 1.9%. This is sufficiently small for calibrating a thyroid monitor used for radiation protection.
MEASUREMENT OF RADON SOIL GAS IN AND AROUND BHARATHINAGARA, MANDYA DISTRICT
Authors: Shashikumar T S , Revanna S , Ramachandra M N , Ashok G V , Ningappa C , Gowda Shivalinge
Published: May 2019

Radon activity concentration in soil gas has been studied in and around Bharathinagara, Mandya district (12° 13|| N and 77° 20|| E) using Solid State Nuclear Track Detectors with Twin cup dosimeter. The activity concentration of 222Rn in soil gas was studied at two depths. Radon in soil gas was found to increase with depth and decrease with increase in moisture content of the soil. Radon in soil gas was found to be higher in winter season which varies from 0.22 ± 0.01 to 1.31 ± 0.01 kBq/m3 with a Geometric mean value of 0.56 ± 0.01 kBq/m3 in 1 m depth and lower radon soil gas was found to be 0.16 ± 0.01 to 0.60 ± 0.01 kBq/m3 with a Geometric mean value of 0.30 ± 0.01 kBq/m3 in 0.5 m depth during summer season. The activity concentrations of radon soil gas from in and around Bharathinagara are lower compared to those in other parts of the world.
Association of Pediatric Resident Physician Depression and Burnout With Harmful Medical Errors on Inpatient Services
Authors: Brunsberg Katherine A. , Landrigan Christopher P. , Garcia Briana M. , Petty Carter R. , Sectish Theodore C. , Simpkin Arabella L. , Spector Nancy D. , Starmer Amy J. , West Daniel C. , Calaman Sharon
Published: May 2019

Purpose

To determine whether higher rates of medical errors were associated with positive screenings for depression or burnout among resident physicians.

Method

The authors conducted a prospective cohort study from 2011 to 2013 in seven pediatric academic medical centers in the United States and Canada. Resident physicians were screened for burnout and depression using the Maslach Burnout Inventory–Human Services Survey (MBI-HSS) and Harvard Department of Psychiatry/National Depression Screening Day Scale (HANDS). A two-step surveillance methodology, involving a research nurse and two physician reviewers, was used to measure and categorize errors. Bivariate and mixed-effects regression models were used to evaluate the relationship between burnout, depression, and rates of harmful, nonharmful, and total errors.

Results

A total of 388/537 (72%) resident physicians completed the MBI-HSS and HANDS surveys. Seventy-six (20%) and 178 (46%) resident physicians screened positive for depression and burnout, respectively. Screening positive for depression was associated with a 3.0-fold higher rate of harmful errors (incidence rate ratio = 2.99 [95% CI 1.40–6.36], P = .005). However, there was no statistically significant association between depression and total or nonharmful errors or between burnout and harmful, nonharmful, or total errors.

Conclusions

Resident physicians with a positive depression screen were three times more likely than those who screened negative to make harmful errors. This association suggests resident physician mental health could be an important component of patient safety. If further research confirms resident physician depression increases the risk of harmful errors, it will become imperative to determine what interventions might mitigate this risk.
Comparison of Preoperative Parameter Measurements Using an Optical Biometer, Automated Keratometer, and a Placido-Based Topographer Module
Authors: Gill Ekjyot S. , Le Christopher , Joseph Jacob , Munir Wuqaas M.
Published: May 2019

Objectives:

To assess the agreement of corneal power, corneal astigmatism, axis location, and astigmatic vector component measurements using a Lenstar LS900, a T-cone add-on, and an ARK 1S autorefractor, furthermore, to assess instrument agreement in a subset of astigmatic eyes.

Methods:

Retrospective analysis of keratometric readings from 3 instruments for 66 eyes undergoing cataract surgery at the Maryland Veterans Affairs Medical Center from November 2014 to 2015. Agreement in corneal power, mean corneal power, axis location, and astigmatic vector components (J0, J45) between devices was evaluated. Comparability was assessed using intraclass correlation, analysis of variance (ANOVA) one-way analysis, Bland–Altman analysis, and power vector analysis. Intraclass correlation was then assessed for a subset of 42 eyes with corneal astigmatism greater than or equal to 1 D.

Results:

The intraclass correlation between instruments was 0.941 for corneal power, 0.788 for corneal astigmatism, 0.932 for J0, and 0.809 for J45. The ANOVA one-way repeated P values comparing Kf, Ks, Km, corneal astigmatism, axis location, J0, and J45 were all greater than 0.05. Power vector analysis showed nearly all values between instruments to be within ±0.5 D. Finally, the intraclass correlation between instruments in eyes with ≥1 D of corneal astigmatism as measured by the T-cone was 0.946 for corneal power, 0.837 for corneal astigmatism, 0.948 for J0, and 0.950 for J45.

Conclusions:

The high level of agreement between all devices indicates the instruments may be used interchangeably. Our data suggest that this may also be true for the subset of eyes with greater than or equal to 1 D in astigmatism, highlighting the toric intraocular lens population.
Causes and Management Outcomes of Acquired Corneal Opacity in a Preschool Age (0–5 Years) Group: A Hospital-Based Study
Authors: Maharana Prafulla K. , Nawaz Shah , Singhal Deepali , Jhanji Vishal , Agarwal Tushar , Sharma Namrata , Vajpayee Rasik B.
Published: May 2019

Purpose:

To evaluate the causes and management outcomes of acquired corneal opacity in a preschool age group (0–5 years) at a tertiary care hospital.

Methods:

Medical records of all cases (0–5 years) with acquired corneal opacity presenting to the cornea clinic of a tertiary eye care hospital from February 2013 to January 2014 were evaluated for age of onset, age at presentation, sex, laterality, cause of opacity, visual acuity, nutritional status, and socioeconomic class of the parents. The etiology of corneal opacity and the type of intervention with outcome at 3 months follow-up were recorded.

Results:

A total of 106 cases were included in the study. The most common cause of corneal scarring was healed infective keratitis (35.8%). Chemical injury, mechanical trauma, and keratomalacia were the other causes, affecting 21.8%, 20.8%, and 16% of the cases, respectively. Optical iridectomy was the most commonly performed procedure (35.8%), followed by lens aspiration with intraocular lens implantation (17.9%) and penetrating keratoplasty (17%). The mean corrected visual acuity (spectacle or contact lens) at the time of presentation and at 3 months after treatment was 2.9 ± 0.3 (perception of light) and 2.2 ± 0.9 (hand motions) logarithm of minimum angle of resolution (logMAR) units, respectively.

Conclusions:

Chemical injury and keratomalacia are the major causes of acquired corneal opacity in preschool age groups in India and are associated with poor visual prognosis.
Reduced excitatory neurotransmitter levels in anterior insulae are associated with abdominal pain in irritable bowel syndrome
Authors: Bednarska Olga , Icenhour Adriane , Tapper Sofie , Witt Suzanne T. , Tisell Anders , Lundberg Peter , Elsenbruch Sigrid , Engström Maria , Walter Susanna
Published: May 2019

Irritable bowel syndrome (IBS) is a visceral pain condition with psychological comorbidity. Brain imaging studies in IBS demonstrate altered function in anterior insula (aINS), a key hub for integration of interoceptive, affective, and cognitive processes. However, alterations in aINS excitatory and inhibitory neurotransmission as putative biochemical underpinnings of these functional changes remain elusive. Using quantitative magnetic resonance spectroscopy, we compared women with IBS and healthy women (healthy controls [HC]) with respect to aINS glutamate + glutamine (Glx) and γ-aminobutyric acid (GABA+) concentrations and addressed possible associations with symptoms. Thirty-nine women with IBS and 21 HC underwent quantitative magnetic resonance spectroscopy of bilateral aINS to assess Glx and GABA+ concentrations. Questionnaire data from all participants and prospective symptom-diary data from patients were obtained for regression analyses of neurotransmitter concentrations with IBS-related and psychological parameters. Concentrations of Glx were lower in IBS compared with HC (left aINS P < 0.05, right aINS P < 0.001), whereas no group differences were detected for GABA+ concentrations. Lower right-lateralized Glx concentrations in patients were substantially predicted by longer pain duration, while less frequent use of adaptive pain‐coping predicted lower Glx in left aINS. Our findings provide first evidence for reduced excitatory but unaltered inhibitory neurotransmitter levels in aINS in IBS. The results also indicate a functional lateralization of aINS with a stronger involvement of the right hemisphere in perception of abdominal pain and of the left aINS in cognitive pain regulation. Our findings suggest that glutaminergic deficiency may play a role in pain processing in IBS.
Summer Adaptive Sports Technology, Equipment, and Injuries
Authors: Matsuwaka Sean T. , Latzka Erek W.
Published: May 2019

As adaptive sports grow in popularity, it is increasingly important to understand the injuries for which their athletes are at risk. This population is challenging to study given its small size and diversity of its participants; accordingly, research is mostly low quality because of limited sample sizes and study durations. Summer adaptive sports account for 22 of 28 Paralympic sports, with the most frequently studied being wheelchair basketball, rugby, tennis, athletics, swimming, and soccer. Injuries vary by sport because of differences in contact level, limbs utilized, and athlete impairments. Equipment changes and technological advances, especially within wheelchair and amputee sports, have increased the level of competition and reduced injury rates. Fortunately, the majority of injuries across adaptive sports are minor and do not result in significant time off from sport. Still, even minor injuries can negatively impact these athletes’ mobility and activities of daily living compared to the nondisabled population.
Shoulder Rehabilitation Protocol and Equipment Fit Recommendations for the Wheelchair Sport Athlete With Shoulder Pain
Authors: Riley Allison H. , Callahan Carrine
Published: May 2019

Shoulder injuries in wheelchair sport athletes is the most commonly reported injury site and can directly impact not only sport performance, but everyday function and mobility. There is abundant research on shoulder rehabilitation for able bodied athletes, but minimal specifically related to wheelchair sport athletes. The purpose of this paper is to outline an exercise protocol and wheelchair sport equipment fit guidelines based off current research and expert opinion. Further research is needed on the effectiveness of a wheelchair athlete specific shoulder rehabilitation protocol, and biomechanical analysis of ideal wheelchair sport equipment fit to reduce shoulder injury and optimize performance.
Winter Adaptive Sports Participation, Injuries, and Equipment
Authors: Oh Hanna , Johnson Wade , Syrop Isaac P.
Published: May 2019

As the participation rate and popularity of winter adaptive sports increases, understanding injury patterns and equipment is crucial for athletes, coaches, athletic trainers, sports physicians, prosthetists/orthotists, and all the staff involved. While the inaugural Winter Paralympics in 1976 had 17 participating countries, the most recent paralympic games in Pyeongchang, South Korea had >500 athletes from 49 different countries competing in 6 disciplines: alpine skiing, nordic skiing, sledge hockey, wheelchair curling, biathlon, and snowboarding. In this paper, we review participation rates, injury trends and equipment, with a focus on the 3 most popular events: alpine skiing, nordic skiing, and sledge hockey. Despite injury risk within this athletic community, there are documented advantages to physical movement, and for each individual the risks of injury have to be weighted with the benefits of competing. Overall, there is a need for educational efforts regarding the development, access, and participation to injury prevention programs with an integrated and multidisciplinary approach.
Adaptive Sports Injury Epidemiology
Authors: Rudolph Lauren , Willick Stuart , Teramoto Masaru , Cushman Daniel M.
Published: May 2019

The benefit of sport for athletes with impairments is well established. However, sport participation is not without risk. The existing literature informs us that injury patterns are sport and disability specific. Further research is needed to study injuries in this population.
Treatment of HeartMate II Short-to-Shield Patients With an Ungrounded Cable: Indications and Long-Term Outcomes
Authors: Coyle Laura , Graney Nicole , Gallagher Colleen , Paliga Robin , Yost Gardner , Pappas Pat , Macaluso Gregory , Tatooles Antone
Published: May 2019

Despite modifications and a procedure to externally replace the distal portion of the percutaneous lead, damage to the wiring insulation causing an electrical short to ground, referred to as a short to shield (STS), has become an important factor in the longevity of the HeartMate II left ventricular assist device (LVAD). Device exchange has been the suggested treatment option. The aim of this study was to evaluate the long-term clinical outcomes of patients with an STS supported on an ungrounded cable. A retrospective review of all patients (n = 479) implanted with a HeartMate II at our center between January 2008 and December 2017 was performed. Patients with a documented STS maintained on an ungrounded cable were examined. Patient characteristics, time from device implantation to STS, treatment strategies, and duration of support on an ungrounded cable were summarized. The association between support with an ungrounded cable and clinical outcomes was evaluated. A total of 53 (11% of 479) patients (83% males and 81% destination therapy) with an STS were supported on an ungrounded cable for a median duration of 195 days (range 2 days to 3.3 years). Patients were more active (New York Heart Association [NYHA] p < 0.001, 6 minute walk test [6MWT] p = 0.003) and had a trend toward increased weight gain (p = 0.055) from time of implant to STS. Duration of support before the STS was 1.9 years (range 165 days to 8.6 years). Twenty-two patients were treated directly with an ungrounded cable and 31 patients underwent an external driveline repair and still required an ungrounded cable within 2 days (range 0 days to 1.3 years). During the study period, 38 patients were maintained on an ungrounded cable: 21 patients were ongoing for 299 days (range 114 days to 2.8 years), 11 patients transplanted after 79 days (range 7–295 days), four patients died because of comorbid conditions after 1.6 years (range 141 days to 3.2 years), one patient exchanged for thrombosis after 229 days, and one patient explanted after 279 days. The other 15 patients developed a phase-to-phase electrical short after 51 days (range 2 days to 3.3 years): 14 patients underwent a successful pump exchange and one patient transplanted within 2 days. No patients died because of support with an ungrounded cable or worsening lead damage necessitating device exchange. With extended durations of support, some patients with a HeartMate II LVAD will experience device failure in the form of an STS. Select patients with an STS can be safely supported on an ungrounded cable for several years with close monitoring. This treatment approach should be considered before a device exchange.
Noninvasive Neurological Monitoring in Extracorporeal Membrane Oxygenation
Authors: Cho Sung-Min , Ziai Wendy , Mayasi Yunis , Gusdon Aaron M. , Creed Jennifer , Sharrock Matthew , Stephens Robert Scott , Choi Chun Woo , Ritzl Eva K. , Suarez Jose , Whitman Glenn , Geocadin Romergryko G.
Published: May 2019

Optimal neurologic monitoring methods have not been characterized for patients on extracorporeal membrane oxygenation (ECMO). We assessed the feasibility of noninvasive multimodal neuromonitoring (NMN) to prognosticate outcome. In this prospective observational study, neurologic examinations, transcranial Doppler (TCD), electroencephalography (EEG), and somatosensory evoked potentials (SSEPs) were performed at prespecified intervals. Outcome at discharge was defined as favorable when modified Rankin Scale (mRS) 0–3; unfavorable when mRS >3. Of 20 patients (median age 60 years), 17 had TCDs, 13 had EEGs, and seven had SSEPs. With NMN, 17 (85%) were found to have neurologic complications. Fourteen (70%) had unfavorable outcomes. The unfavorable outcome was associated with absent EEG reactivity, coma, central cannulation, higher transfusion requirement, and higher Acute Physiology and Chronic Health Evaluation II and Sepsis-related Organ Failure Assessment scores. Seven patients had both SSEPs and EEGs and exhibited intact N20 responses despite poor outcomes. Four of these seven showed absent EEG reactivity despite intact N20. Eighteen thromboembolic events were observed, 14 of which had positive microembolic signals (MESs) in TCD. All 10 patients with arterial-sided thrombotic events had positive MES. NMN caused no adverse effects. NMN during ECMO is feasible and found high neurologic complication rate. EEG and TCD showed potential for prognostication of neurologic outcome.
Clinical implication of maximal voluntary ventilation in myotonic muscular dystrophy
Authors: Suh Mi Ri , Kim Dong Hyun , Jung Jiho , Kim Bitnarae , Lee Jang Woo , Choi Won Ah , Kang Seong-Woong
Published: May 2019

Patients with myotonic muscular dystrophy type 1 (DM1) tend to exhibit earlier respiratory insufficiency than patients with other neuromuscular diseases at similar or higher forced vital capacity (FVC). This study aimed to analyze several pulmonary function parameters to determine which factor contributes the most to early hypercapnia in patients with DM1.

We analyzed ventilation status monitoring, pulmonary function tests (including FVC, maximal voluntary ventilation [MVV], and maximal inspiratory and expiratory pressure), and polysomnography in subjects with DM1 who were admitted to a single university hospital. The correlation of each parameter with hypercapnia was determined. Subgroup analysis was also performed by dividing the subjects into 2 subgroups according to usage of mechanical ventilation.

Final analysis included 50 patients with a mean age of 42.9 years (standard deviation = 11.1), 46.0% of whom were male. The hypercapnia was negatively correlated with MVV, FVC, forced expiratory volume in 1 second (FEV1), and their ratios to predicted values in subjects with myotonic muscular dystrophy type 1. At the same partial pressure of carbon dioxide, the ratio to the predicted value was lowest for MVV, then FEV1, followed by FVC. Moreover, the P values for differences in MVV and its ratio to the predicted value between ventilator users and nonusers were the lowest.

When screening ventilation failure in patients with DM1, MVV should be considered alongside other routinely measured parameters.
A mutation in PIK3CD gene causing pediatric systemic lupus erythematosus
Authors: Li Guo-Min , Liu Hai-Mei , Guan Wan-Zhen , Xu Hong , Wu Bing-Bing , Feng Jia-Yan , Sun Li
Published: May 2019

Rationale:

Gain of function (GOF) mutations in PIK3CD gene encoding PI3K p110δ were recently associated with a novel combined immune deficiency characterized by recurrent sinopulmonary infections, CD4
+ lymphopenia, reduced class-switched memory B cells, lymphadenopathy, cytomegalovirus and/or epstein-Barr virus (EBV) viremia, and EBV-related lymphoma. A subset of affected patients also had elevated serum IgM.

Patient concerns:

We report a patient who was diagnosed with systemic lupus erythematosus (SLE) at a young age and was recently found to carry heterozygous mutations in PIK3CD. The patient not only presented with recurrent sinopulmonary infections, CD4
+ lymphopenia, lymphadenopathy, EBV viremia, and elevated serum IgM, but also met classification criteria of SLE based on persistent proteinuria and hematuria, leukopenia and anemia, low level of serum complement, and positive autoantibody for antinuclear antibodies.

Diagnoses:

Activated PI3Kδ syndrome.

Interventions:

Oral prednisolone and hydroxychloroquine combined with mycophenolate mofetil was given to the patient. He was currently receiving intravenous immunoglobulin per month in association with hydroxychloroquine, low-dose prednisolone, and mycophenolate mofetil.

Outcomes:

At present, the level of complement restored to normal, hematuria and proteinuria disappeared, and liver function returned to normal.

Lessons:

SLE may be a novel phenotype of GOF mutation in PI3CKD gene (GOF PIK3CD).
Presence of diabetic retinopathy is lower in type 2 diabetic patients with non-alcoholic fatty liver disease
Authors: Zhang Meng , Li Li , Chen Jing , Li Bei , Zhan Yutao , Zhang Chuan
Published: May 2019

To analyze the association between non-alcoholic fatty liver disease (NAFLD) and the presence of diabetic retinopathy (DR) in patients with type 2 diabetes mellitus (T2DM).

Total 411 T2DM patients were divided into NAFLD and control groups. NAFLD was diagnosed by ultrasound. Retinopathy was diagnosed by fundus photography. All patients were screened based on medical history, physical examinations, and laboratory measurements.

The prevalence of NAFLD and DR in T2DM patients was 60.8% and 40.9%, respectively. The presence of DR was associated with diabetes duration, systolic blood pressure (SBP), glycated hemoglobin (HbA1c), and proteinuria (all P < .001) using univariate and multivariate regression analyses. The prevalence of DR was lower in patients with NAFLD than those without NAFLD (37.2% vs 46.6%, P = .065), and significantly lower in patients with moderate and severe NAFLD (30.2% vs 46.6%, P = .012; 14.3% vs 46.6%, P = .024). The presence of DR in NAFLD patients was associated with diabetes duration (P = .032) in Chi-squared analysis.

NAFLD and DR were highly prevalent in T2DM patients. Diabetes duration, SBP, HbA1c, and proteinuria were risk factors for DR in T2DM patients. The presence of DR was lower in T2DM patients with NAFLD, which was mainly due to their shorter diabetes duration.
Dual arterial blood supply D-pouch in a patient with ulcerative colitis undergoing proctocolectomy and ileal pouch-anal anastomosis
Authors: Hu Hang , Zhang Yichao , Qian Qun , Xu Ming , Chen Min , Jiang Congqing , Ding Zhao
Published: May 2019

Introduction:

Pouchitis is the most common complication in Ulcerative colitis (UC) patients after restorative proctocolectomy with ileal pouch-anal anastomosis (RP-IPAA) and ischemia may be a significant contributing factor. Tension and blood supply are the primary concerns while performing the procedure. A dual arterial blood supply technique is designed to decrease tension while ensuring sufficient blood perfusion.

Patient concerns:

A 61-year-old female patient with 14 years history of UC wanted to seek surgical treatment.

Diagnoses:

Ulcerative colitis.

Interventions:

After physical examination and treatment of parenteral nutrition, the patient underwent a D-pouch with dual arterial blood supply after total proctocoloectomy.

Outcomes:

The patient recovered well and was discharged 10 days after her procedure. Postoperatively dual arterial blood supply to the D-pouch was demonstrated by computed tomography angiography (CTA).

Conclusion:

D-pouch with dual arterial blood supply is feasible and safe in patients with UC undergoing RP-IPAA.
Distal femoral physeal fractures after neonatal osteomyelitis
Authors: Wu Xing , Xia Jingdong , Li Jun , Sun Jie , Shen Xiantao
Published: May 2019

Rationale:

The physeal separations and septic osteomyelitis in newborns are unusual, which represents a challenging problem in diagnosis and treatment.

Patient concerns:

A 2-day-old mature male suddenly complained by parents about minimal swelling around the left knee, decreased left extremity motion and no fever.

Diagnosis:

Preliminary x-rays of the lower extremities demonstrated a displaced distal femoral physeal, Laboratory investigation indicated infection. Magnetic resonance imaging and ultrasound showed displaced distal femoral physeal. A needle aspiration confirmed the diagnosis.

Intervention:

Debridement and ultrasound guide reduction with pinning of physeal separations was performed.

Outcome:

At 5 years later, his last follow-up showed that there was only 1.6 cm limb-length discrepancy without angular deformity, the child did not report any pain and was perfectly able to perform his daily activities.

Lessons:

Distal femoral physeal fractures after neonatal osteomyelitis requires immediate and reliable decision for management. We point out the important role of the application of sonography, which is helpful to make an early diagnosis and guide reduction and percutaneous pinning of distal femoral physeal fractures.
Tracheal atresia with a cephalically developed lung bud
Authors: Lee Na Mi , Yi Dae Yong , Yun Sin Weon , Chae Soo Ahn , Lim In Seok
Published: May 2019

Rationale:

Tracheal atresia (TA) involves complete or partial tracheal absence below the larynx. It involves failure of complete separation of the laryngotracheal diverticulum from the foregut at the 4th gestational week. In TA, the trachea and main bronchi generally proceed in the normal caudal directions.

Patient concerns:

At the gestational age of 34 weeks and 6 days, a male baby weighing 2290 g was born via cesarean section. A brisk bag was used, and mask ventilation was performed, but was not effective. Intubation was attempted; however, the endotracheal tube did not advance below the vocal cord.

Diagnosis:

Tracheal atresia.

Interventions:

An emergent open neck exploration could not reveal the trachea. On computed tomography, the tracheoesophageal fistula (TEF) started from the lower esophagus just above the gastroesophageal junction. The trachea arose upward to form the main bronchus.

Outcomes:

His parents signed the “Do not resuscitate” form due to poor outcome. On the 23rd day of birth, the baby expired.

Lessons:

Our case involved TA with tracheoesophageal fistula forming between the trachea and main bronchi in a cephalic direction as detected on computed tomography. Further, the trachea arose from the gastroesophageal junction; thus, it does not belong to any Floyd classification. Herein, we report a TA case with a cephalically developed lung bud.
Treatment outcomes of percutaneous transhepatic variceal embolization versus transjugular intrahepatic portosystemic shunt for gastric variceal bleeding
Authors: Zhang Kai , Sun Xiaoyan , Wang Guangchuan , Zhang Mingyan , Wu Zhe , Tian Xiangguo , Zhang Chunqing
Published: May 2019

There have been few studies comparing percutaneous transhepatic variceal embolization (PTVE) and transjugular intrahepatic portosystemic shunt (TIPS) for the prevention of recurrent gastric variceal bleeding (GVB).

Compare the outcomes of these 2 procedures in patients with GVB.

A total of 74 cirrhosis patients with GVB who underwent TIPS and modified PTVE were enrolled. The rebleeding and mortality rates, portal vein pressure (PVP) variation, and rates of hepatic encephalopathy (HE) were compared between the 2 groups.

A total of 43 PTVE and 31 TIPS patients were enrolled in this study. The difference of rebleeding rate in the 2 groups was not statistically significant (P 
= .190). The difference of early rebleeding rates and cumulative rebleeding-free rates were all not statistically significant (P 
= .256, P = .200). The difference of mortality rates in the 2 groups was not statistically significant (χ2 = 1.206, P = .272). The rate of HE in TIPS group was statistically higher than that in PTVE group (P < .0001).

Both PTVE and TIPS were effective for preventing rebleeding of GVs. There were no significant differences in rebleeding and mortality rates. The incidence of HE after TIPS was higher than PTVE.
Electronic fetal monitoring characteristics of a patient with sudden onset of placental abruption and intrauterine fetal demise
Authors: Zhang Yan , Zuo Xiaohang , Yuan Ting , Teng Yue
Published: May 2019

Introduction:

Placental abruption (PA) is a serious complication of pregnancy, associated with significant perinatal complications, including intrauterine fetal demise (IUFD). Continuous electronic fetal monitoring (EFM) has been widely applied in China in recent decades. Exploration of potentially PA-specific patterns of EFM contributes to early detection of PA occurrence.

Patient concerns and diagnosis:

A 33-year-old woman (gravida 3, para 1) was referred to our hospital at 33+3 weeks gestation due to non-reassuring fetal heart rate (FHR) pattern, and suffered sudden onset of severe PA and subsequent intrauterine fetal demise.

Interventions:

We analyzed the characteristics of her non-stress tests (NSTs) 1 day and 10 min before the detection of PA, aiming to explore potentially PA-specific patterns of EFM and provide reference for early detection of asymptomatic PA occurrence in obstetric practice.

Outcomes:

Unfavored characteristics of FHR patterns before PA onset are analyzed.

Conclusion:

For those who sense decreased fetal movements (DFMs), a NST and a biophysical profile (BPP) are recommended for exclusion of potential adverse maternal and fetal complications.
Clinical characterization of icotinib-induced chemoresistance in erlotinib-treated lung adenocarcinoma patient with EGFR mutations
Authors: Wu Ka , Guo Chao , Li Rong
Published: May 2019

Rationale:

Mounting evidences reveal that mutation of epidermal growth factor receptor (EGFR) may induce the resistance of tyrosine kinase inhibitors (TKIs). TKI-resistant lung cancer cells are sensitive to inhibition of the EGFR pathway. This case report aimed to characterize the therapeutic benefits of erlotinib, a targeted drug, on an advanced lung cancer patient with somatic EGFR mutation.

Patient concerns:

A 52-year-old non-smoking Chinese woman was suffered from pneumonia-based chest pains, and the patient was diagnosed as advanced lung cancer through medical imaging, thoracoscopy, and pathological examination.

Diagnoses:

Blood tests, pathological examination, thoracoscopy, computed tomography (CT)/positron emission computed tomography (PET) scans, next-generation sequencing (NGS) testing were subjected to the patient's samples before and after targeted drug treatments.

Interventions:

After icotinib-induced resistance, the chemoresistance mechanism was involved in EGFR mutations before being prescribed with erlotinib.

Outcomes:

The therapeutic effectiveness of icotinib for 4-month showed undetected carcinomatous metastasis. The lung tumor sizes were reduced, and improved quality of life (QOL) was described by the patient. Followed by monotherapy with erlotinib for 1.5-year, the icotinib-resistant patient benefited from longer survival rate without tumor enlargement and neoplastic metastasis. In therapeutic duration of erlotinib, T790M mutation of EGFR, R248W mutation of tumor protein p53 (TP53), K844S mutation of retinoblastoma protein 1 (RB1) were identified through NGS test.

Lessons:

In conclusion, the anti-cancer benefits of icotinib and erlotinib against advanced lung cancer may contribute to suppress neoplastic growth and metastasis. Further, erlotinib exerts potent efficacy for extended survival rate of patient because detectable mutations may not or limitedly induce erlotinib-resistance. In addition, reduced circulating hormones by menopause may enhance the therapeutic effectiveness of erlotinib.
Predictive factors for the development of renal insufficiency following partial nephrectomy and subsequent renal function recovery
Authors: Lee Kwang Suk , Kim Dae Keun , Kim Kwang Hyun , Bang Woo Jin , Kim Hyung Joon , Park Sung Yul , Rha Koon Ho , Chung Byung Ha , Cho Jin Seon , Koo Kyo Chul
Published: May 2019

Patients who undergo partial nephrectomy (PN) may exhibit renal function insufficiency, and a subset of these patients achieves renal function recovery. We evaluated the predictors of renal insufficiency and subsequent renal function recovery following PN. Data on 393 patients who underwent PN for solid renal tumors between March 2001 and November 2013, obtained from 6 institutions, were retrospectively reviewed. Renal insufficiency was defined as new onset of chronic kidney disease stage ≥3 postoperatively on the second of 2 consecutive tests. Renal function recovery was defined as an estimated glomerular filtration rate ≥60 ml/minute/1.73 m2 following renal insufficiency. Tumor complexity was stratified according to the RENAL classification system. The median (interquartile range) age, tumor size, and follow-up period were 53 (45–63) years, 2.6 (1.9–3.8) cm, and 36 (12–48) months, respectively. Tumors were of low complexity in 258/393 (65.6%) of cases. Renal insufficiency developed in 54/393 (13.5%) patients, in which age ≥60 years and preoperative creatinine ≥1.1 mg/ml were independent predictors. Tumor complexity, clamp type, and operative method were not significant prognostic factors. Among patients with newly developed renal insufficiency, 18/54 (33.3%) patients exhibited renal function recovery within a median period of 18 months, of which preoperative creatinine <1.1 mg/ml was an independent predictor. Age ≥60 years and preoperative creatinine ≥1.1 mg/ml were risk factors for renal insufficiency following PN. Patients with renal insufficiency whose preoperative creatinine was <1.1 mg/ml were likely to have renal function recovery.
Progress towards improving homing and engraftment of hematopoietic stem cells for clinical transplantation
Authors: Huang Xinxin , Broxmeyer Hal E.
Published: May 2019

Purpose of review

Hematopoietic cell transplantation (HCT) is a life-saving treatment for a variety of hematological and nonhematological disorders. Successful clinical outcomes after transplantation rely on adequate hematopoietic stem cell (HSC) numbers, and the homing and subsequent short-term and long-term engraftment of these cells in the bone marrow. Enhancing the homing capability of HSCs has the potential for high impact on improving HCT and patient survival.

Recent findings

There are a number of ways to enhance HSC engraftment. Neutralizing negative epigenetic regulation by histone deacetylase 5 (HDAC5) increases surface CXCR4 expression and promotes human HSC homing and engraftment in immune-deficient NSG (NOD.Cg-Prkdcscid IL2rgtm1Wjl/Sz) mice. Short-term treatment of cells with glucocorticoids, pharmacological stabilization of hypoxia-inducible factor (HIF)-1α, increasing membrane lipid raft aggregation, and inhibition of dipeptidyl peptidase 4 (DPP4) facilitates HSC homing and engraftment. Added to these procedures, modulating the mitochondria permeability transition pore (MPTP) to mitigate ambient air-induced extra physiological oxygen stress/shock (EPHOSS) by hypoxic harvest and processing, or using cyclosporine A during air collection increases functional HSC numbers and improves HSC engraftment.

A better understanding of the regulation of human HSC homing mediated by various signaling pathways will facilitate development of more efficient means to enhance HCT efficacy.
Successful Treatment of an EBV-positive Diffuse Large B-Cell Lymphoma in a Patient With Trisomy 21
Authors: Putti Maria C. , Marzollo Antonio , Carraro Elisa , Boaro Maria P. , Massano Davide , Mussolin Lara , Lovisa Federica , Tumino Manuela , Calore Elisabetta , Mainardi Chiara , Varotto Elena , d’Amore Emanuele S.G. , Pillon Marta
Published: May 2019

Diffuse large B-cell Lymphoma (DLBCL) secondary to a chronic severe Epstein-Barr virus (EBV) infection has not been previously described in a patient with trisomy 21. Here we report the case of a 14-year-old girl with trisomy 21 with impaired control of EBV and DLBCL. She was cured with dose-adapted chemotherapy and hematopoietic stem cell transplantation without severe treatment-related toxicity. We describe the first case of EBV-positive DLBCL in a patient with trisomy 21 and we propose a treatment modality for this rare entity.
Bevacizumab Use in Refractory Adult Pilocytic Astrocytoma
Authors: Carabenciov Ivan D. , Bhargav Adip G. , Uhm Joon H. , Ruff Michael W.
Published: May 2019

Pilocytic astrocytomas (PA) are highly vascular tumors with vascular endothelial growth factor (VEGF)–vascular endothelial growth factor receptor-2 (VEGFR-2) signaling present in the tumor vasculature. PA may, therefore, be responsive to VEGF blockade with bevacizumab (BEV). Data regarding the use of BEV in refractory PA in adults are limited primarily to case reports and case series of patients with recurrent PA. We conducted a single-center, retrospective cohort study from 2009 to 2018. We screened 426 patients with pathologically confirmed PA. We identified 5 adult patients with PA who received BEV at our institution with sufficient clinical follow-up to derive evidence of the efficacy and toxicity. All 5 patients experienced tumor progression after initial therapies which included surgery, radiation, and chemotherapy. Four patients received BEV as monotherapy, whereas 1 received BEV with the continuation of previously initiated alkylating chemotherapy (temozolomide). The average duration of BEV therapy was 10.2 months (range, 1 to 20 mo) with an average follow-up of 47 months (range, 6 to 112 mo). One patient had a severe necrotizing rash in areas of skin contact and discontinued after 1 cycle of BEV. All patients had stabilization per RANO criteria, with 1 patient experiencing progression after 10 months on treatment. One patient had disease progression 5 years after completion of BEV, but the tumor responded to repeat treatment with BEV. Our institution’s experience with the use of BEV in recurrent PA is in line with previous reports of therapeutic benefit in recurrent adult PA.