bluuebunny/crossref_metadata_2025 huggingface dataset:
Evaluating and sharing global genetic ancestry in biomedical datasets
Authors: Harismendy Olivier , Kim Jihoon , Xu Xiaojun , Ohno-Machado Lucila
Published: December 2018

Genetic ancestry is a critical co-factor to study phenotype-genotype associations using cohorts of human subjects. Most publicly available molecular datasets are, however, missing this information or only share self-reported race and ethnicity, representing a limitation to identify and repurpose datasets to investigate the contribution of ancestry to diseases and traits. We propose an analytical framework to enrich the metadata from publicly available cohorts with genetic ancestry information and a resulting diversity score at continental resolution, calculated directly from the data. We illustrate this framework using The Cancer Genome Atlas datasets searched through the DataMed Data Discovery Index. Data repositories and contributors can use this framework to provide genetic diversity measurements for controlled access datasets, minimizing the work involved in requesting a dataset that may ultimately prove inadequate for a researcher’s purpose. With the increasing global scale of human genetics research, studies on disease risk and susceptibility would benefit greatly from the adequate estimation and sharing of genetic diversity in publicly available datasets following a framework such as the one presented.
The past and future of USTC: an interview with USTC President Xinhe Bao
Authors: Zhao Weijie , Yi Xiaosu
Published: December 2018

The University of Science and Technology of China (USTC) is located in Hefei, the capital of Anhui province, and has its own characteristics among the universities in China. Established by the Chinese Academy of Sciences (CAS), USTC is distinctively tinted with a scientific color. It is also famous for its ‘Special Class for the Gifted Young’ and is considered one of the best Chinese universities in the fields of science and technology (S&T). Recently, National Science Review interviewed Professor Xinhe Bao, the President of USTC, about the characteristics of the university and the education and research in China. Xinhe Bao is an academician of CAS and has made seminal contributions in catalysis and energy chemistry in the past decades. Before joining USTC, he had worked at Dalian Institute of Chemical Physics (DICP), CAS and Fudan University (Shanghai), and thus possesses an in-depth understanding of the education and research in China.
Looking Back at 2018
Authors: Kennedy Maureen Shawn
Published: December 2018

A review of a year some labeled ‘toxic.’
The Top News Stories of 2018: More Extreme Weather Amid Environmental Protection Rollbacks
Authors: McSpedon Corinne
Published: December 2018

Deregulation continues despite worsening climate outlook.
HIV Drug May Increase Risk of Neural Tube Birth Defects
Authors: Aschenbrenner Diane S.
Published: December 2018

The Food and Drug Administration is investigating early reports from an ongoing clinical trial in Botswana that the HIV drug dolutegravir may increase the risk of neural tube birth defects if used from the time of conception through the first trimester of pregnancy.

A pregnancy test is now recommended for women of childbearing age prior to starting HIV treatment with dolutegravir.
First Drug Approved for Metastatic or Locally Advanced Cutaneous Squamous Cell Carcinoma
Authors: Aschenbrenner Diane S.
Published: December 2018

Cemiplimab-rwlc (Libtayo) is the first drug approved to treat metastatic or locally advanced cutaneous squamous cell carcinoma in patients who are not candidates for curative surgery or curative radiation.

Immune-mediated adverse effects can be serious and can affect any organ or tissue. Infusion reactions are also possible. Embryo–fetal toxicity can occur if given during pregnancy.
Low RN Staffing Levels Linked to Missed Care
Authors: Rosenberg Karen , Mechcatie Elizabeth
Published: December 2018

According to this study:

Low levels of RN staffing in hospitals are associated with reports of missed care.
Timing of Second Stage Pushing Doesn't Affect Spontaneous Vaginal Delivery Rate
Authors: Rosenberg Karen , Mechcatie Elizabeth
Published: December 2018

According to this study:

Among nulliparous women receiving neuraxial anesthesia, the spontaneous vaginal delivery rate is similar in those who use immediate and delayed pushing approaches during the second stage of labor.
Early, Nurse-Directed Sepsis Care
Authors: Ferguson Alice , Coates Daniel Evan , Osborn Scott , Blackmore Christopher Craig , Williams Barbara
Published: December 2018

Background: Sepsis is one of the leading causes of hospital mortality and readmission. For the past 20 years, sepsis research has focused on best practices for treating patients with the most severe manifestations of sepsis, while the treatment of patients outside of critical care or ED settings, who have early or less severe signs and symptoms of sepsis, have received little attention.

Objective: The goal of this quality improvement (QI) initiative was to promote early recognition and treatment of sepsis through the establishment of a multidisciplinary, executive-led sepsis guiding team that leveraged nursing skills and expertise.

Methods: To meet this objective, we decided to speed the initiation of sepsis treatment at our medical center, going beyond the Surviving Sepsis Campaign guidelines in place at the time and setting as targets the identification and treatment within one hour of all inpatients and ED patients with suspected sepsis, regardless of their illness severity or care unit. Our early intervention strategy incorporated a nurse-directed ED Code Sepsis, based on the characterization of sepsis as a systemic inflammatory response syndrome—a criterion widely used at the start of this QI initiative—and an inpatient Power Hour, which authorized nurses to initiate order sets independently for lactate levels, blood cultures, and fluid boluses when they suspected sepsis. The order sets both improved bundle adherence and signaled the pharmacy to expedite antibiotic preparation and delivery. To gauge the effects of our initiative, we conducted a retrospective, interrupted time-series cohort evaluation, using the in-hospital sepsis-related mortality rate as the primary outcome, and considered as process metrics the initiation of ED Code Sepsis and the inpatient Power Hour, order set use, bundle adherence, and sepsis-related rapid response team (RRT) calls.

Results: Over the course of the seven-year pre- to postintervention evaluation period, ED sepsis bundle adherence increased from 40.5% to 73.7% (P < 0.001), with a mean triage to antibiotic time of 80 minutes. Sepsis-related RRT calls decreased from 2.2% to 0.85% (P < 0.001). And the in-hospital sepsis-related mortality rate dropped from 12.5% to 8.4% (P < 0.001) with an absolute reduction of 4.5 deaths per 100 sepsisrelated discharges.

Conclusion: This project demonstrates that using nurse-directed care to promote timely identification and early treatment of sepsis in the ED and in inpatient settings can improve bundle adherence and reduce in-hospital sepsis-related mortality rates.
A Lie by Omission?
Authors: Yingling Charles
Published: December 2018

The connection between self-disclosure and authenticity in the nurse–patient relationship.
Electromagnetic Interference with Protocolized Electrosurgery Dispersive Electrode Positioning in Patients with Implantable Cardioverter Defibrillators
Authors: Schulman Peter M. , Treggiari Miriam M. , Yanez N. David , Henrikson Charles A. , Jessel Peter M. , Dewland Thomas A. , Merkel Matthias J. , Sera Valerie , Harukuni Izumi , Anderson Ryan B. , Kahl Ed , Bingham Ann , Alkayed Nabil , Stecker Eric C.
Published: December 2018

Editor’s Perspective

What We Already Know about This Topic

What This Article Tells Us That Is New

Background

The goal of this study was to determine the occurrence of intraoperative electromagnetic interference from monopolar electrosurgery in patients with an implantable cardioverter defibrillator undergoing surgery. A protocolized approach was used to position the dispersive electrode.

Methods

This was a prospective cohort study including 144 patients with implantable cardioverter defibrillators undergoing surgery between May 2012 and September 2016 at an academic medical center. The primary objectives were to determine the occurrences of electromagnetic interference and clinically meaningful electromagnetic interference (interference that would have resulted in delivery of inappropriate antitachycardia therapy had the antitachycardia therapy not been programmed off) in noncardiac surgeries above the umbilicus, noncardiac surgeries at or below the umbilicus, and cardiac surgeries with the use of an underbody dispersive electrode.

Results

The risks of electromagnetic interference and clinically meaningful electromagnetic interference were 14 of 70 (20%) and 5 of 70 (7%) in above-the-umbilicus surgery, 1 of 40 (2.5%) and 0 of 40 (0%) in below-the-umbilicus surgery, and 23 of 34 (68%) and 10 of 34 (29%) in cardiac surgery. Had conservative programming strategies intended to reduce the risk of inappropriate antitachycardia therapy been employed, the occurrence of clinically meaningful electromagnetic interference would have been 2 of 70 (2.9%) in above-the-umbilicus surgery and 3 of 34 (8.8%) in cardiac surgery.

Conclusions

Despite protocolized dispersive electrode positioning, the risks of electromagnetic interference and clinically meaningful electromagnetic interference with surgery above the umbilicus were high, supporting published recommendations to suspend antitachycardia therapy whenever monopolar electrosurgery is used above the umbilicus. For surgery below the umbilicus, these risks were negligible, implying that suspending antitachycardia therapy is likely unnecessary in these patients. For cardiac surgery, the risks of electromagnetic interference and clinically meaningful electromagnetic interference with an underbody dispersive electrode were high. Conservative programming strategies would not have eliminated the risk of clinically meaningful electromagnetic interference in either noncardiac surgery above the umbilicus or cardiac surgery.
Surgical Treatment of Lenke Type 5 Adolescent Idiopathic Scoliosis
Authors: Yoshihara Hiroyuki
Published: December 2018

Study Design.

A systematic review.

Objective.

To systemically review the previous literature regarding surgical treatment of Lenke type 5 adolescent idiopathic scoliosis (AIS).

Summary of Background Data.

The Lenke classification was published in 2001 as the classification of AIS. Since then, numerous articles have been published reporting the outcomes of surgical treatment of Lenke type 5 AIS.

Methods.

The electronic databases PubMed, EMBASE, and Web of Science were queried up to Aug 2018 for articles regarding surgical treatment of Lenke type 5 AIS. Surgical variables, radiographic assessments, and clinical outcomes of surgical treatment of Lenke type 5 AIS were summarized.

Results.

Fifty studies met the inclusion criteria. The average fused levels, % correction of thoracolumbar/lumbar curve at final follow-up, and % correction of thoracic curve at final follow-up for anterior and posterior procedures were reported to be 3.6–5.3 and 4.3–7.8 levels, 53–86 and 55–94% and 17–52 and 19–67%, respectively. Average coronal balance was imbalanced (≥20 mm) at preoperation in 22/43 reporting study groups and balanced (<20 mm) at final follow-up in all 37 reporting study groups. Scoliosis Research Society Version 22 scores showed no difference between anterior and posterior procedures in most of the reporting studies (5/6).

Conclusion.

Overall, the outcomes of surgical treatment of Lenke type 5 AIS are excellent. The thoracic curve was spontaneously corrected after surgery and coronal balance after surgery was better than before surgery. Both anterior and posterior procedures demonstrated satisfactory outcomes.

Level of Evidence: 4
Enhanced Perioperative Care for Major Spine Surgery
Authors: Dagal Armagan , Bellabarba Carlo , Bransford Richard , Zhang Fangyi , Chesnut Randall M. , O’Keefe Grant E. , Wright David R. , Dellit Timothy H. , Painter Ian , Souter Michael J.
Published: December 2018

Study Design.

The enhanced perioperative care (EPOC) program is an institutional quality improvement initiative. We used a historically controlled study design to evaluate patients who underwent major spine surgery before and after the implementation of the EPOC program.

Objective.

To determine whether multidisciplinary EPOC program was associated with an improvement in clinical and financial outcomes for elective adult major spine surgery patients.

Summary of Background Data.

The enhanced recovery after surgery (ERAS) programs successfully implemented in hip and knee replacement surgeries, and improved clinical outcomes and patient satisfaction.

Methods.

We compared 183 subjects in traditional care (TRDC) group to 267 intervention period (EPOC) in a single academic quaternary spine surgery referral center. One hundred eight subjects in no pathway (NOPW) care group was also examined to exclude if the observed changes between the EPOC and TRDC groups might be due to concurrent changes in practice or population over the same time period. Our primary outcome variables were hospital and intensive care unit lengths of stay and the secondary outcomes were postoperative complications, 30-day hospital readmission and cost.

Results.

In this highly complex patient population, we observed a reduction in mean hospital length of stay (HLOS) between TRDC versus EPOC groups (8.2 vs. 6.1 d, standard deviation [SD] = 6.3 vs. 3.6, P < 0.001) and intensive care unit length of stay (ILOS) (3.1 vs. 1.9 d, SD = 4.7 vs. 1.4, P = 0.01). The number (rate) of postoperative intensive care unit (ICU) admissions was higher for the TRDC n = 109 (60%) than the EPOC n = 129 (48%) (P = 0.02). There was no difference in postoperative complications and 30-day hospital readmissions. The EPOC spine program was associated with significant average cost reduction—$62,429 to $53,355 (P < 0.00).

Conclusion.

The EPOC program has made a clinically relevant contribution to institutional efforts to improve patient outcomes and value. We observed a reduction in HLOS, ILOS, costs, and variability.

Level of Evidence: 3
Preoperative Risk Stratification in Spine Tumor Surgery
Authors: Lakomkin Nikita , Zuckerman Scott L. , Stannard Blaine , Montejo Julio , Sussman Eric S. , Virojanapa Justin , Kuzmik Gregory , Goz Vadim , Hadjipanayis Constantinos G. , Cheng Joseph S.
Published: December 2018

Study Design.

A retrospective review of prospectively collected data.

Objective.

The purpose of this study is to compare and validate several preoperative scores for predicting outcomes following spine tumor resection.

Summary of Background Data.

Preoperative risk assessment for patients undergoing spinal tumor resection remains challenging. At present, few risk assessment tools have been validated in this high-risk population.

Methods.

The 2008 to 2014 National Surgical Quality Improvement database was used to identify all patients undergoing surgical resection of spinal tumors, stratified as extradural, intradural extramedullary, and intramedullary based on CPT codes. American Society of Anesthesiologists (ASA) score, modified Charlson Comorbidity Index (CCI), and modified Frailty Index (mFI) were computed. A binary logistic regression model was used to explore the relationship between these variables and postoperative outcomes, including mortality, major and minor adverse events, and hospital length of stay (LOS). Other significant variables such as demographics, operative time, and tumor location were controlled for in each model.

Results.

Two thousand one hundred seventy patients met the inclusion criteria. Higher CCI scores were independent predictors of mortality (OR = 1.24, 95% CI: 1.14–1.36, P < 0.001), major adverse events (OR = 1.07, 95% CI: 1.01–1.31, P = 0.018), minor adverse events (OR = 1.15, 95% CI: 1.10–1.20, P < 0.001), and prolonged LOS (OR = 1.14, 95% CI: 1.09–1.19, P < 0.001). Patients’ mFI scores were significantly associated with mortality and LOS, but not major or minor adverse events. ASA scores were not associated with any outcome metric when controlling for other variables.

Conclusion.

The CCI demonstrated superior predictive capacity compared with mFI and ASA scores and may be valuable as a preoperative risk assessment tool for patients undergoing surgical resection of spinal tumors. The validation of assessment scores is important for preoperative risk stratification and improving outcomes in this high-risk group.

Level of Evidence: 3
Mineralized Collagen Modified Polymethyl Methacrylate Bone Cement for Osteoporotic Compression Vertebral Fracture at 1-Year Follow-up
Authors: Zhu Jinjin , Zhang Kai , Luo Kefeng , Qiu Zhiye , Yang Shuhui , Cui Fuzhai , Weng Xisheng , Jiang Guoqiang
Published: December 2018

Study Design.

Retrospective comparative study.

Objective.

This study aimed to compare the clinical effects and imaging features of polymethyl methacrylate (PMMA) bone cement with and without mineralized collagen (MC) in percutaneous kyphoplasty (PKP) for osteoporotic vertebral compression fractures (OVCFs).

Summary of Background Data.

PKP with PMMA is widely performed for OVCF. However, numerous complications have also been reported about the PMMA bone cement. Moreover, PMMA bone cement with and without MC have not been compared with respect to their postoperative efficacy and long-term follow-up.

Methods.

From July 2016 to July 2017, 105 OVCF patients were randomly divided into two groups based on their PKP treatment: MC-PMMA group and PMMA group. Clinical operation, cement leakage, Oswestry Disability Index, visual analog scale, height of the fractured vertebrae, Cobb angle, refracture of the adjacent vertebra, recompression, and computed tomography values of the injured vertebra were compared between the two groups postoperatively and after 1-year follow-up.

Results.

Clinical operation showed no differences between the two groups. Visual analog scale scores, Oswestry Disability Index scores, and Cobb angles showed statistically significant differences between the two groups after 1-year follow-up. The height of the vertebral body showed significant difference at 3 days postoperatively and preoperatively in each group and significant difference after 1 year between the two groups. The rate of refracture and leakage of the MC-PMMA group was lower than that of the PMMA group. The computed tomography value of the MC-PMMA group was obviously higher than that of the PMMA group after 1-year follow-up.

Conclusion.

MC-modified PMMA did not change the beneficial properties of PMMA. This new bone cement has better biocompatibility, can form a stable structure in the vertebral body, and improve the prognosis of patients by reducing pain and reoperation.

Level of Evidence: 3
A New Joystick Technique for the Reduction of Unstable Trochanteric Hip Fractures: Especially With Rotational Deformity
Authors: Okazaki Atsushi , Murase Tomoo , Sakano Hiroaki , Saito Tomoyuki
Published: December 2018

We are performing surgical treatment of trochanteric hip fractures using an intramedullary nail. There are irreducible fractures in which the correct reduction cannot be performed with the usual traction and internal rotation. In particular, the rotational deformity is difficult to reduce. One of the causes of this is the instability and uncontrollability of the proximal bone fragment. To address this problem, we devised a new joystick technique. From the same skin incision as used for inserting the intramedullary nail, a 3.0 mm Kirschner wire (K-wire) is inserted into the femoral neck perpendicularly. A K-wire is inserted at the front part of the femoral neck so as not to interfere with the lag screw. The fracture is reduced by operating the proximal bone fragment by using the K-wire as a joystick. The proximal bone fragment is operated in the varus-valgus direction by moving the K-wire proximally-distally and in internal-external rotation directions by moving the K-wire anteriorly-posteriorly. No complications, such as cut-out of the femoral neck due to the K-wire or neurovascular injury, were observed. The joystick technique was used in the following situations: (1) stabilization of the proximal bone fragment, (2) rotational reduction of the proximal bone fragment, and (3) prevention of rotational deformity caused by lag screw insertion. This joystick technique is simple and less invasive so we do not consider it a problem to apply it routinely, in all cases.
A Compilation of Serum Concentrations of 12 Antipsychotic Drugs in a Therapeutic Drug Monitoring Setting
Authors: Jönsson Anna K. , Spigset Olav , Reis Margareta
Published: December 2018

Background:

No comprehensive collection of routine therapeutic drug monitoring data for antipsychotic drugs has been published.

Methods:

In this compilation, data on 12 antipsychotics are presented. The drugs included are amisulpride (n = 506), aripiprazole (n = 1610), clozapine (n = 1189), flupentixol (n = 215), haloperidol (n = 390), olanzapine (n = 10,268), perphenazine (n = 1065), quetiapine (n = 5853), risperidone (n = 3255), sertindole (n = 111), ziprasidone (n = 1235), and zuclopenthixol (n = 691). Because only one sample per patient is included, the number of patients equals the number of samples. For each drug, median serum concentrations as well as that of the 10th and 90th percentiles are given for a range of daily doses. Comparisons are made between males and females, between patients younger than 65 years and 65 years and older, and between those treated with a low and a high dose of each drug. The concentration-to-dose (C/D) ratio is the primary variable used in these comparisons. Coefficients of variation (CVs) for the serum concentrations of each drug within and between subjects are presented.

Results:

In general, the C/D ratios were higher in females than in males, higher in those 65 years and older than in younger subjects, and lower in those treated with higher doses than in those treated with lower doses. CVs between individuals were larger than within subjects, and the CVs were highest for the drugs with short elimination half-lives.

Conclusions:

For each antipsychotic drug, the results presented can serve as a reference tool for pharmacokinetic interpretation of the individual patient's serum drug level. The compiled serum concentrations and the C/D ratios can support the physician's decision when individualizing dosing and determining treatment strategies for a specific patient.
Microvasculature of the Optic Nerve Head and Peripapillary Region in Patients With Primary Open-Angle Glaucoma
Authors: Nascimento e Silva Rafaella , Chiou Carolina A. , Wang Mengyu , Wang Haobing , Shoji Marissa K. , Chou Jonathan C. , D’Souza Erica E. , Greenstein Scott H. , Brauner Stacey C. , Alves Milton R. , Pasquale Louis R. , Shen Lucy Q.
Published: December 2018

Purpose:

To assess optic nerve head (ONH) and peripapillary microvasculature in primary open-angle glaucoma (POAG) of mild to moderate severity using swept-source optical coherence tomography angiography (OCTA).

Materials and Methods:

In a cross-sectional study, swept-source OCTA images were analyzed for 1 eye from each of 30 POAG patients with glaucomatous Humphrey visual field loss and 16 controls. The anatomic boundary of ONH was manually delineated based on Bruch’s membrane opening and large vessels were removed from en face angiography images to measure vessel density (VD) and the integrated OCTA by ratio analysis signal (IOS), suggestive of flow, in the ONH and peripapillary region. POAG subgroup analysis was performed based on a history of disc hemorrhage (DH) matched by visual field mean deviation (MD).

Results:

POAG (mean MD±SD, −3.3±3.0 dB) and control groups had similar demographic characteristics and intraocular pressure on the day of imaging. Groups did not differ in superficial ONH VD or flow indicated by IOS (P≥0.28). POAG eyes showed significantly lower VD (39.4%±4.0%) and flow (38.8%±5.6%) in deep ONH, peripapillary VD (37.9%±2.9%) and flow (43.6%±4.0%) compared with control eyes (44.1%±5.1%, 44.7%±6.9%, 40.7%±1.7%, 47.8%±2.5%, respectively; P≤0.007 for all). In the subgroup analysis, POAG eyes with (n=14) and without DH (n=16) had similar measured OCTA parameters (P>0.99 for all).

Conclusions:

The image processing methodology based on the anatomic boundary of ONH demonstrated compromised microvasculature in the deep ONH and peripapillary region in eyes with mild to moderate POAG, regardless of the history of DH.
The Effects of Endurance Sports on Children and Youth
Authors: Egger Anthony C. , Oberle L.Morgan , Saluan Paul
Published: December 2018

In the United States, youth participation in sports continues to increase yearly. This increase in participation, in conjunction with the trend toward early sports specialization and year round training, has led to a similar increase in athletically developed injuries. These injuries vary in nature and acuity, with the type of injury often related to the athlete’s age, sport, and level of training. Endurance athletes are at an elevated risk of injury as they frequently push their body to the limit during their arduous training. Pediatric endurance athletes can be particularly vulnerable, especially to overuse injuries, given their unique and ever-changing physiological state. It is important to understand the specific challenges facing not only the physical, but also the emotional well-being of these pediatric endurance athletes to maximize performance while minimizing injury and potential long-term sequelae.
Comparative Therapeutic Assessment of Atrial Fibrillation in Heart Failure With Reduced Ejection Fraction—A Network Meta-Analysis
Authors: Malik Aaqib H. , Aronow Wilbert S.
Published: December 2018

Background:

Catheter ablation (CA) continues to prove to be an intriguing therapeutic option for the management of atrial fibrillation (AF) especially in patients with heart failure with reduced ejection fraction (HFrEF). Recent data have suggested that CA may be a viable first-line strategy for these patients.

Study Question:

Is CA more effective in managing patients with AF with HFrEF compared to optimal medical treatment and anti-arrhythmic drugs?

Data Sources:

Randomized controlled trials (RCTs) comparing CA, medical treatment, or antiarrhythmic drugs to each other or a placebo group for the treatment of AF in HFrEF. We performed a comprehensive search in PubMed, Embase, and Cochrane library to identify relevant RCTs.

Study Design:

Our primary outcomes of interest were all-cause mortality, hospitalization for heart failure, and the percentage change in left ventricular ejection fraction. Also, we looked at functional outcomes such as Minnesota Living with Heart Failure Questionnaire and 6-minute walking distance. We used event rates for categorical variables and mean differences between the groups for the continuous variables. We used a frequentist approach employing a graph theory methodology to construct a network meta-analysis model.

Results:

We ended up with 17 RCTs with 5460 participants and 5 different treatments in our network meta-analysis. Compared to optimal medical therapy, CA was effective in reducing all-cause mortality odds ratio (OR) 0.44 (95% confidence interval, 0.27–0.74; P-value: <0.001) and hospitalization for heart failure OR 0.41 (0.28–0.59; P-value: <0.001). CA also resulted in improvement in left ventricular ejection fraction OR 9.34 (7.13–11.55; P-value: <0.001), Minnesota Living with Heart Failure Questionnaire OR −7.75 (−13.98 to −1.52; P-value: <0.01), and 6MWT OR 27.30 (5.27–49.33; P-value: <0.02).

Conclusions:

CA is the most effective and safe treatment for AF patients with HFrEF. We should consider this as a first-line therapy for the management of these patients.
Management of Peutz‐Jeghers Syndrome in Children and Adolescents
Authors: Latchford Andrew , Cohen Shlomi , Auth Marcus , Scaillon Michele , Viala Jerome , Daniels Richard , Talbotec Cecile , Attard Thomas , Durno Carol , Hyer Warren
Published: December 2018

Peutz‐Jeghers syndrome (PJS) is a well‐described inherited syndrome, characterized by the development of gastrointestinal polyps, and characteristic mucocutaneous freckling. Development of small bowel intestinal polyps may lead to intussusception in children may require emergency laparotomy with potential loss of bowel. Gastrointestinal polyps may lead to bleeding and anemia. This European Society for Paediatric Gastroenterology Hepatology and Nutrition position paper provides a guide for diagnosis, assessment, and management of PJS in children and adolescents and guidance on avoiding complications from PJS or from the endoscopic procedures performed on these patients.

This is the first position paper regarding PJS published by European Society for Paediatric Gastroenterology Hepatology and Nutrition. Literature from PubMed, Medline, and Embase was reviewed and in the absence of evidence, recommendations reflect the opinion of pediatric and adult experts involved in the care of polyposis syndromes. Because many of the studies that form the basis for the recommendations were descriptive and/or retrospective in nature, some of the recommendations are based on expert opinion. This position paper will be helpful in the appropriate management and timing of procedures in children and adolescents with PJS.
Facial Onset Motor and Sensory Neuronopathy Syndrome With a Novel TARDBP Mutation
Authors: Zhang Qin , Cao Bei , Chen Yongping , Liang Yan , Wei Qianqian , Zhou Dong , Shang Huifang
Published: December 2018

Introduction:

Facial onset sensory and motor neuronopathy (FOSMN) syndrome was a rare and slowly progressive neurodegenerative disorder, which heralded by sensory symptoms within the face, and followed by evolution of sensory and motor deficits in the face and limbs. The underlying pathogenesis of FOSMN remains to be fully elucidated.

Case Report:

A 40-year-old man was admitted to our hospital with facial sensory deficits spreading in a rostral-caudal manner. He then developed diffuse fasciculation, bulbar signs, atrophy and weakness of facial, neck, and limb muscles progressively, a process resembling amyotrophic lateral sclerosis. Neurophysiological studies demonstrated abnormal blink reflexes and some denervation-reinnervation changes in electromyogram. He was diagnosed with FOSMN syndrome clinically. A novel heterozygous Gly386Glu mutation in the transactive response DNA-binding protein (TARDBP) gene was found. The patient had no response to immunologic treatment and finally died of respiratory failure.

Conclusions:

This is the first time that a novel mutation in TARDBP gene was identified in a patient with FOSMN syndrome, which further suggested a link between FOSMN and amyotrophic lateral sclerosis. Our findings widen the spectrum of TARDBP-related motor neuron diseases.
Sturge-Weber Syndrome Coexisting With Moyamoya Disease in the Fifth Decade
Authors: Hao Zilong , Lai Xiaohui
Published: December 2018

Sturge-Weber syndrome (SWS) is a sporadically occurring neurocutaneous syndrome characterized by port-wine stain over the face, ocular abnormalities (glaucoma and choroidal hemangioma), and leptomeningeal angiomas. It is usually diagnosed in infancy, but it may occasionally present in adulthood with seizures or stroke-like episodes. Here, we report a 46-year-old male patient, having SWS coexisting with moyamoya disease, attending our hospital due to sudden loss of consciousness. We also searched PubMed (from its earliest date to August 2014) for case reports mentioning that SWS presents in adulthood. We identified 31 patients. The common clinical manifestation are seizures, stroke-like episodes, and migraine-like headaches.On the basis of our findings in this patient, we would recommend that patients with a port-wine nevus of the face should be given further investigation to rule out an intracranial vascular malformation, especially if seizures, stroke-like episodes, or migraine-like headaches are present.
Generalized Convulsive Status Epilepticus Secondary to Jarisch-Herxheimer Reaction in Neurosyphilis
Authors: Rissardo Jamir P. , Caprara Ana L.F. , Silveira Juliana O.F.
Published: December 2018

Introduction:

Jarisch-Herxheimer reaction (JHR) is defined as a transient immunologic reaction, classically seen in patients during syphilis treatment. JHR is a rare cause of status epilepticus (SE) in neurosyphilis. We describe a case of generalized convulsive SE (GCSE) probably secondary to JHR in a patient with neurosyphilis.

Case Report:

A 23-year-old man presented with progressive behavioral changes with 4 months of onset and pupillary alteration with 9 months of onset. He had experienced blurred vision and had noticed mydriasis in the left eye. Brain magnetic resonance imaging was normal; however, sexually transmitted infections were not investigated. After 1 month, the blurred vision disappeared, but mydriasis remained. Three months passed, and he reported progressive behavioral changes. Laboratory tests were within the normal limits, except by positive blood venereal disease research laboratory. Later, the cerebrospinal fluid analysis was requested, which revealed a positive venereal disease research laboratory. Electroencephalogram and brain magnetic resonance imaging were normal. Treatment was started with crystalline penicillin G. A GCSE began 12 hours after the first dose of penicillin. He did not develop classic signs and symptoms of the JHR. An EEG showed periodic lateralized epileptiform discharges.

Conclusions:

To the author’s knowledge, there are 3 cases in the literature of nonconvulsive SE with JHR in neurosyphilis, and the present report was the first to describe the occurrence of GCSE secondary to JHR in neurosyphilis. Another important finding, in this case, was the isolated unilateral mydriatic tonic pupil, rarely reported as an early isolated symptom of neurosyphilis. In this way, it is essential that patients presenting with isolated unilateral tonic pupil receive an adequate investigation for syphilis.
Are Postprocedural Blood Pressure Goals Associated With Clinical Outcome After Mechanical Thrombectomy for Acute Ischemic Stroke?
Authors: Blech Benzion , Chong Brian W. , Sands Kara A. , Wingerchuk Dean M. , Jackson William T. , Marks Lisa A. , O’Carroll Cumara B
Published: December 2018

Mechanical thrombectomy (MT) is the standard of care for patients who present with an acute ischemic stroke within 6 hours of symptom onset, and up to 24 hours in appropriately selected patients. However, optimal postoperative management of these patients remains uncertain, especially with regard to blood pressure control. To review the existing literature to define potential blood pressure goals in the immediate postoperative period in patients who undergo MT for acute ischemic stroke. The topic was defined through a clinical scenario and the subsequent development of a targeted clinical question. A literature search was performed, with relevant articles selected, one of which, a prospective observational study, was critically appraised. Participants included neurology residents and consultants, a medical librarian, clinical epidemiologists, as well as content experts from vascular neurology and interventional neuroradiology. Permissive hypertension (defined as <220/120 or <180/105 mm Hg as per the American Heart Association/American Stroke Association guidelines) may be harmful in the postoperative period following MT, especially in patients who were successfully recanalized. Moderate blood pressure control (<160/90) was found to be a predictor of improved 3-month mortality on multivariable logistic regression analysis in patients who sustained successful reperfusion [odds ratio (OR), 0.08; 95% confidence interval (CI), 0.01-0.054; P=0.01]. A 10 mm Hg increase in systolic blood pressure was found to result in a lower OR of having a favorable 3-month functional independence (OR, 0.70; 95% CI, 0.56-0.85; P=0.001) as well as higher rates of 3-month mortality (OR, 1.49; 95% CI, 1.18-1.88; P=0.001). Blood pressure goals in the immediate postoperative period in patients who undergo MT should differ than those who do not undergo MT, with data suggesting that lower blood pressure than permissive hypertension may be related to improved outcomes, especially in cases of successful reperfusion. However, current data are derived from observational studies; further studies, preferably in the form of randomized-controlled trials, are needed to further clarify the relationship between postoperative blood pressures and outcomes in this patient population.
Changes in the Properties of the Preferred Retinal Locus with Eccentric Viewing Training
Authors: Hassan Shirin E. , Ross Nicole Christie , Massof Robert W. , Stelmack Joan
Published: December 2018

SIGNIFICANCE

This study explores whether eccentric viewing training (EVT) changes the properties of the retinal area used for fixation in subjects with bilateral macular disease. The data presented demonstrate the feasibility of conducting a randomized controlled trial on EVT.

PURPOSE

Patients with bilateral central scotomas adopt other retinal area(s) called preferred retinal locus to substitute the blind fovea. EVT is offered with the goal to improve functional vision by learning how to identify and use a more useful retinal area for fixation—a trained retinal locus—and/or to improve fixation stability with their existing preferred retinal locus. An observational study was conducted to determine whether patients change and adopt a new trained retinal locus location and/or if fixation stability improves after EVT.

METHODS

Seventy-six patients with bilateral central scotomas received EVT from an experienced rehabilitation therapist. Retinal locations for repeated fixations and sizes of central scotomas of the better eye were recorded before and after EVT with a scanning laser ophthalmoscope. The position and area of the 95% confidence bivariate ellipse that defines the retinal area in which 95% of fixations occur were analyzed.

RESULTS

We observed no significant difference between the size of the area of the fitted ellipses before (mean [SD], 26.4° [19.7°]) and after (mean [SD], 24.8° [20.9°]) EVT (P = .54). However, we observed a shift in the preferred retinal locus location after EVT (P < .0001). The mean (SD) shift in the position of the center of fixation after EVT was 7.5° (5.2°; range, 0.45 to 23°). This mean difference was larger than would be expected from average within-subject fixation stability.

CONCLUSIONS

The location of the retinal area used for fixation changes after EVT. Fixation stability does not change after EVT. These preliminary data are yet to be confirmed with the use of a control group.
Enhanced Recovery after Surgery Pathway for Microsurgical Breast Reconstruction: A Systematic Review and Meta-Analysis
Authors: Sebai Mohamad E. , Siotos Charalampos , Payne Rachael M. , Stone Jill P. , Seal Stella M. , Habibi Mehran , Broderick Kristen , Sacks Justin M. , Manahan Michele A. , Rosson Gedge D.
Published: December 2018

Background:

The enhanced recovery after surgery pathway was introduced in 1997 as a multimodal approach to reduce preventable postoperative harm and shorten hospital length of stay. However, there is yet no widely accepted enhanced recovery after surgery protocol for microsurgical breast reconstruction. The authors conducted a systematic review and meta-analysis of the current literature on enhanced recovery after surgery for microsurgical breast reconstruction with regard to postoperative length of stay and morbidity.

Methods:

The PubMed, Embase, Cochrane, Scopus, and Web of Science databases were searched for all studies published before June of 2016 containing original data on enhanced recovery after surgery in microsurgical breast reconstruction in relation to postoperative length of stay and morbidity. Studies were screened using eligibility criteria. Meta-analysis, odds ratio, and 95 percent confidence interval were used to pool acquired data.

Results:

The initial search identified 86 studies. Two independent screeners identified four original articles with a total of 676 patients. Length of stay was significantly shorter for patients on an enhanced recovery after surgery pathway (mean difference, −1.23; 95 percent CI, −1.50 to −0.96; p < 0.001; I
2 = 0 percent; random effects model). Enhanced recovery was not associated with changes in 30-day postoperative morbidity; specifically, no significant difference was observed in rates of partial flap loss (p = 0.44), total flap loss (p = 0.91), breast hematoma (p = 0.69), donor-site infection (p = 0.53), urinary tract infection (p = 0.29), and pneumonia (p = 0.42).

Conclusion:

The authors’ review suggests that enhanced recovery after surgery in microsurgical breast reconstruction is associated with a reduced length of stay, and is not associated with increased postoperative morbidity.
Feasibility of Simulation-Based Medical Education in a Low-Income Country
Authors: Bulamba Fred , Sendagire Cornelius , Kintu Andrew , Hewitt-Smith Adam , Musana Fred , Lilaonitkul Maytinee , Ayebale Emmanuel T. , Law Tyler , Dubowitz Gerald , Kituuka Olivia , Lipnick Michael S.
Published: December 2018

Summary Statement

Simulation is relatively new in many low-income countries. We describe the challenges encountered, solutions deployed, and the costs incurred while establishing two simulation centers in Uganda. The challenges we experienced included equipment costs, difficulty in procurement, lack of context-appropriate curricula, unreliable power, limited local teaching capacity, and lack of coordination among user groups. Solutions we deployed included improvisation of equipment, customization of low-cost simulation software, creation of context-specific curricula, local administrative support, and creation of a simulation fellowship opportunity for local instructors. Total costs for simulation setups ranged from US $165 to $17,000. For centers in low-income countries trying to establish simulation programs, our experience suggests that careful selection of context-appropriate equipment and curricula, engagement with local and international collaborators, and early emphasis to increase local teaching capacity are essential. Further studies are needed to identify the most cost-effective levels of technological complexity for simulation in similar resource-constrained settings.
A Systematic Review of the Educational Effectiveness of Simulation Used in Open Surgery
Authors: Heskin Leonie , Simms Ciaran , Holland Jane , Traynor Oscar , Galvin Rose
Published: December 2018

Summary Statement

The role of simulation to teach and access open surgical skills has become more prevalent in recent years. This systematic review synthesizes the totality of evidence with respect to the educational effectiveness of simulators used in open surgical training. A systematic literature search was conducted in PubMed, Embase, CINAHL, Scopus, and Web of Science. Only randomized controlled trials were included that explored the educational efficacy of theses simulators. Six randomized controlled trials were included from the 9934 studies found. The methodological quality of the included studies was variable. Overall, the use of the simulators was more educationally effective compared with standard teaching of the skill without a simulator (P < 0.05). Two studies showed that the simulator was as good as an animal model of much higher fidelity. Further studies are needed to secure higher evidence for the educational value, validity, and transferability of the skills to the hospital setting for all simulators.
Doxycycline Reduces Scar Thickness and Improves Collagen Architecture
Authors: Moore Alessandra L. , desJardins-Park Heather E. , Duoto Bryan A. , Mascharak Shamik , Murphy Matthew P. , Irizarry Dre M. , Foster Deshka S. , Jones Ruth E. , Barnes Leandra A. , Marshall Clement D. , Ransom Ryan C. , Wernig Gerlinde , Longaker Michael T.
Published: December 2018

Objective:

To investigate the effects of local doxycycline administration on skin scarring.

Background:

Skin scarring represents a major source of morbidity for surgical patients. Doxycycline, a tetracycline antibiotic with off-target effects on the extracellular matrix, has demonstrated antifibrotic effects in multiple organs. However, doxycycline's potential effects on skin scarring have not been explored in vivo.

Methods:

Female C57BL/6J mice underwent dorsal wounding following an established splinted excisional skin wounding model. Doxycycline was administered by local injection into the wound base following injury. Wounds were harvested upon complete wound closure (postoperative day 15) for histological examination and biomechanical testing of scar tissue.

Results:

A one-time dose of 3.90 mM doxycycline (2 mg/mL) within 12 hours of injury was found to significantly reduce scar thickness by 24.8% (
∗

P < 0.0001) without compromising tensile strength. The same effect could not be achieved by oral dosing. In doxycycline-treated scar matrices, collagen I content was significantly reduced (
∗

P = 0.0317) and fibers were favorably arranged with significantly increased fiber randomness (
∗

P = 0.0115). Common culprits of altered wound healing mechanics, including angiogenesis and inflammation, were not impacted by doxycycline treatment. However, engrailed1 profibrotic fibroblasts, responsible for scar extracellular matrix deposition, were significantly reduced with doxycycline treatment (
∗

P = 0.0005).

Conclusions:

Due to the substantial improvement in skin scarring and well-established clinical safety profile, locally administered doxycycline represents a promising vulnerary agent. As such, we favor rapid translation to human patients as an antiscarring therapy.
Intraoperative Evaluation of Urinary Tract Injuries at the Time of Pelvic Surgery: A Systematic Review
Authors: Siff Lauren N. , Hill Audra J. , Jallad Karl , Harnegie Mary Pat , Barber Matthew D.
Published: December 2018

Objective

The aim of this study was to determine the safety and efficacy of methods for intraoperative evaluation of urinary tract injury during pelvic surgery.

Methods

PubMed, EMBASE, CINAHL, Web of Science, Scopus, ProQuest, the Cochrane Library, and Clinicaltrials.gov were searched from 1947 to February 2018. Articles or abstracts describing the routine evaluation of urinary tract injuries during pelvic surgery in adults were included, surgical indications of urinary tract anomaly, stones, or malignancy were excluded. There were no restrictions on study design or language. Outcomes included injuries diagnosed intraoperatively, delayed diagnoses, adverse effects, subjective assessments, time to use, and cost. Data were extracted in duplicate at an individual-participant level. Prevalence of injuries, sensitivity, specificity, and predictive values of each diagnostic method were calculated. A combination of generalized linear models and a Bayesian approach were used to separately pool diagnostic accuracy measures.

Results

There were 5303 titles, 527 abstracts, and 164 full-text articles assessed for eligibility; 69 articles were analyzed. Diagnostic methods retrieved were cystoscopy using saline, dextrose or unspecified distention media, oral phenazopyridine and vitamin B, intravenous (IV) methylene blue, IV sodium fluorescein, IV indigo carmine, prophylactic retrograde ureteral stents, and transabdominal Doppler ultrasound. Prevalence of urinary tract injury ranged from 0.3% to 2.8%. Sensitivity ranged 63% to 91%, and specificity, 99.7% to 100%, with no significant differences suggested between methods.

Conclusions

All evaluable methods of intraoperative urinary tract assessment during pelvic surgery are safe and effective with specificity of greater than 99% and low rates of complications. Larger, more rigorous studies are required to evaluate the diagnostic accuracy of some newer methods.