bluuebunny/crossref_metadata_2025_split huggingface dataset:
A Case Report of Donnai-Barrow Syndrome
Authors: Robinson Melissa K. , Coe Kristi , Bradshaw Wanda T. 
Published: July 2020

Background:
A genetic disorder should be considered when an infant presents with multiple congenital anomalies. Because of the acute presentation of an infant with multiple life-threatening defects, a genetic diagnosis of a rare disorder took weeks to delineate.

Clinical Findings:
This case describes a late preterm infant who presented at birth with congenital diaphragmatic hernia, tetralogy of Fallot, cleft lip, low-set ears, and hypertelorism.

Primary Diagnosis:
Donnai-Barrow syndrome was the final diagnosis confirmed by a defect observed on the LRP2 (2q31.1) gene using sequence analysis. This is a rare disorder that presents with a variety of phenotypic features in infants.

Interventions:
Initial neonatal resuscitation in the delivery room included intubation, positive pressure ventilation, and oxygen supplementation. Extracorporeal membrane oxygenation therapy was initiated from day of life 3 to 15. Initial surgery included correction of the congenital diaphragmatic hernia, and further surgical procedures included tracheostomy, gastrostomy tube, circumcision, ventricular septal defect repair, and cleft lip repair. Physical, occupational, and speech therapies were also initiated.

Outcomes:
The infant was transported to a pediatric rehabilitation facility at 6 months of life for further management of his chronic lung disease requiring tracheostomy with ventilator dependence.

Practice Recommendations:
Early recognition and diagnosis of genetic syndromes can improve family education and guide treatment interventions. An underlying syndrome should be suspected when an infant presents with multiple congenital defects. Infants with Donnai-Barrow syndrome should have thorough cardiac, neurologic, ophthalmologic, audiologic, and renal examinations due to the gene mutation effects on those systems.
Factors Associated With the Need for Pharmacological Management of Neonatal Opioid Withdrawal Syndrome
Authors: Scott Lisa Fath , Guilfoy Veronica , Duwve Joan M. , Rawl Susan M. 
Published: July 2020

Background:Neonatal opioid withdrawal syndrome (NOWS) is a significant and growing health problem that affects more than 23,000 infants annually, with an estimated hospital cost of more than $720 million.Purpose:The purpose of this study was to examine factors associated with the need to initiate medication for the treatment of NOWS.Methods:A retrospective review of medical records was conducted of 204 infants born to mothers who used opioids during pregnancy from April 2011 to September 2017. Associations between maternal, infant, and environmental factors and the need for neonatal pharmacological management were examined using χ2,ttests, and regression analysis.Results:Of 204 neonates exposed to opioids prenatally, 121 (59%) developed symptoms of NOWS, requiring treatment with morphine. Neonates requiring morphine had significantly higher gestational ages (37.7 weeks vs 36.4 weeks;P< .001), and mothers were present at the neonate's bedside for a lower proportion of their total hospital stay (57% vs 74% of days;P< .001). Maternal factors associated with the need for neonatal medication treatment included the mother's reason for opioid use (P= .014), primary type of opioid used (P< .001), tobacco use (P= .023), and use of benzodiazepines (P= .003).Implications for Practice:This research provides information regarding the proportion of infants exposed to opioids prenatally who develop NOWS that requires treatment, as well as maternal, infant, and environmental factors associated with the need for neonatal medication use.Implications for Research:Future research is needed to examine these relationships prospectively in a larger and more diverse sample.
Tackling global challenges in pediatric rheumatology
Authors: Lewandowski Laura B. 
Published: July 2020

Purpose of the review
To highlight the current challenges in diagnosis and clinical care of pediatric rheumatic disease and barriers to research and education of pediatric rheumatologists worldwide.

Recent findings
Recent studies and reports demonstrate a paucity of studies on epidemiology, outcomes, and management guidelines from many regions of the world. There have been noteworthy efforts to bridge the gap in under resourced areas. An analysis of the global burden of rheumatic disease has demonstrated that while understudied, musculoskeletal diseases are prevalent and increasingly contribute to loss of years of healthy life. In juvenile idiopathic arthritis, two milestone publications in global pediatric rheumatology have recently been published. An international study evaluated the epidemiology, treatment, and outcomes of juvenile idiopathic arthritis and demonstrated global diversity in both clinical manifestations and outcomes. Notably, the first guidelines for managing pediatric rheumatic disease in a less resourced setting have been published for juvenile idiopathic arthritis. This document offers the first publication targeted to address challenges faced by pediatric rheumatology caregivers in low-resourced settings. These documents serve as exemplars for international collaboration in pediatric rheumatology and can be used as models for other pediatric rheumatic disease research. Other efforts are making progress in various arenas towards increasing access to care, education, and training in pediatric rheumatology.

Summary
The global burden of rheumatic disease in the pediatric population is poorly understood but unrecognized disease greatly impacts the overall morbidity and mortality in this population. More studies in lesser resourced regions are needed to prioritize access to pediatric rheumatology care and prioritize a further increase in research capacity and education moving forward.
Optimal Technical Factors During Operative Management of Low-Energy Femoral Neck Fractures
Authors: Schottel Patrick C. , Blankstein Michael , Sprague Sheila , Swiontkowski Marc , Bzovsky Sofia , Bhandari Mohit , Schemitsch Emil H. ,   on behalf of the FAITH Investigators
Published: July 2020

Objective:
To determine if cancellous screw (CS) and sliding hip screw (SHS) technical factors during low-energy femoral neck fracture fixation affects a 24-month revision surgery rate and health-related quality of life (HRQL).

Design:
Prospective randomized controlled study.

Setting:
International, multicenter.

Patients:
Eight hundred ninety-eight femoral neck fracture patients age 50 years and older.

Intervention:
Patients were randomized to fracture stabilization with either CSs or a SHS device as part of the Fixation Using Alternative Implants for the Treatment of Hip Fractures (FAITH) trial. CS technical factors analyzed included screw diameter, short versus long screw threads, screw number and formation, screw orientation, and washer use. SHS technical factors studied were side plate length, supplemental screw use, lag screw position, and tip–apex distance.

Main Outcome Measurements:
Revision surgeries within 24 months to promote fracture healing, relieve pain, treat infection, or improve function. In addition, HRQL measures were collected, including the SF-12 Physical Component Score and Western Ontario and McMaster Universities Osteoarthritis Index.

Results:
The 3-screw inverted triangle pattern had a significantly lower revision surgery rate than a 3-screw triangle formation (P = 0.004). No other CS or SHS technical factors were predictive of revision surgery or affected a patient's HRQL (P > 0.05).

Conclusions:
A 3-screw inverted triangle pattern was superior to a 3-screw triangle formation. However, injury and patient factors such as fracture displacement, age, smoking status and sex play a more significant role in clinical outcomes for low-energy femoral neck fracture treatment.

Level of Evidence:
Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.
Why Make the Cut? Trochanteric Slide Osteotomy Can Improve Exposure to the Anterosuperior Acetabulum
Authors: Phelps Kevin D. , Crickard Colin V. , Li Katherine , Harmer Luke S. , Andrews McArthur Erica , Sample Robinson Katherine , Sims Stephen H. , Hsu Joseph R. 
Published: July 2020

Objective:
To define relative increases in visual bony surface area and access to critical landmarks with the addition of a trochanteric slide osteotomy to a Kocher–Langenbeck approach.

Methods:
A Kocher–Langenbeck approach followed by a trochanteric slide osteotomy was sequentially performed on 10, fresh-frozen, hemipelvectomy cadaveric specimens. Visual and palpable access to relevant surgical landmarks was recorded. Calibrated digital photographs were taken of each approach and analyzed using Image J.

Results:
The acetabular surface area exposed was 27.66 (±6.67) cm2 for a Kocher–Langenbeck approach. This increased to and 41.82 (±7.97) cm2 with the addition of a trochanteric osteotomy. The exposed surface area was increased by 51.2% for the trochanteric osteotomy (P < 0.001). The superior margin of the acetabulum could be visualized and palpably accessed in both exposures. Access to the more anterosuperior portions of the acetabulum was consistently possible in the trochanteric osteotomy but not with the Kocher–Langenbeck approach.

Conclusions:
A trochanteric osteotomy may visually improve access to the most anterosuperior acetabulum but does not significantly improve surgical access to relevant portions of the superior acetabulum when compared with a Kocher–Langenbeck approach.
Therapeutic Facet Joint Interventions in the Lumbar Spine
Authors: Mazmudar Aditya , Nayak Rusheel , Patel Alpesh A. 
Published: July 2020

Objective:
In this narrative review, the current literature on therapeutic interventions for low back pain of facet joint etiology is assessed from an economic value perspective.

Summary of Background Data:
The efficacy and economics of facet joint interventions in the treatment of lumbar back pain is a controversial topic. Trends show that facet joint interventions are becoming increasingly used, perhaps as physicians become more averse to treating chronic low back pain with opioids. With the emphasis on value-based spine care and changing reimbursement models, the perspective of rigorously evaluating the outcomes these interventions provide and the costs they incur is particularly relevant.

Discussion:
Although the evidence is noted to be limited, most systematic reviews fail to demonstrate the therapeutic utility of intra-articular facet joint injections in low back pain because of high study heterogeneity. A few good quality studies and systematic reviews describe moderate evidence for the utilization of therapeutic medial branch blocks and radiofrequency neurotomies in alleviating facet joint pain.

Conclusion:
Consequently, there is a need for high-quality cost-effectiveness studies for facet joint interventions so that evidence-based and economically viable solutions can be used to optimize patient care at a societally affordable price.
Patient Outcomes Following Short-segment Lumbar Fusion Are Not Affected by PI-LL Mismatch
Authors: Divi Srikanth N. , Kepler Christopher K. , Hilibrand Alan S. , Goyal Dhruv K.C. , Mujica Victor E. , Radcliff Kristen E. , Anderson David Greg , Kurd Mark F. , Rihn Jeffrey A. , Kaye Ian D. , Woods Barrett R. , Vaccaro Alexander R. , Schroeder Gregory D. 
Published: July 2020

Study Design:
This is a retrospective cohort study.

Objective:
The objective of this study was to further elucidate the relationship between pelvic incidence-lumbar lordosis (PI-LL) mismatch and surgical outcomes in patients undergoing short segment lumbar fusions for degenerative lumbar disease.

Summary of Background Data:
There are few studies examining the relationship between spinopelvic parameters and patient reported outcome measurements (PROMs) in short segment lumbar degenerative disease.

Materials and Methods:
A retrospective review was conducted at single academic institution. Patients undergoing 1- or 2-level lumbar fusion were retrospectively identified and separated into 2 groups based on postoperative PI-LL mismatch ≤10 degrees (NM) or PI-LL mismatch >10 degrees (M). Outcomes including the Physical Component Score (PCS)-12, Mental Component Score (MCS)-12, Oswestry Disability Index (ODI), Visual Analog Scale (VAS) back and leg scores were analyzed. Absolute PROM scores, the recovery ratio and the percentage of patients achieving minimum clinically important difference between groups were compared and a multiple linear regression analysis was performed.

Results:
A total of 306 patients were included, with 59 patients in the NM group and 247 patients in the M group. Patients in the M group started with a higher degree of PI-LL mismatch compared with the NM group (22.2 vs. 7.6 degrees, P<0.001) and this difference increased postoperatively (24.7 vs. 2.5 degrees, P<0.001). There were no differences between the 2 groups in terms of baseline, postoperative, or Δ outcome scores (P>0.05). In addition, having a PI-LL mismatch was not found to be an independent predictor of any PROM on multivariate analysis (P>0.05).

Conclusion:
The findings in this study show that even though patients in the M group had a higher degree of mismatch preoperatively and postoperatively, there was no difference in PROMs.

Level of Evidence:
Level III.
New targets and technologies for CAR-T cells
Authors: Freitag Fabian , Maucher Marius , Riester Zeno , Hudecek Michael 
Published: July 2020

Purpose of review
Immunotherapy with gene-engineered chimeric antigen receptor (CAR)-T cells has curative potential in advanced malignancies and undergoes a surging preclinical and clinical development. Here, we present a selection of new targets and technologies that illustrate the progress that is being made with the aspiration to make CAR-T cell therapy a universally applicable and effective treatment in cancer medicine.

Recent findings
There is a rich pipeline of new target antigens for CAR-T cells in hematology and oncology that are rated based on uniformity but also stability of expression on tumor cells under therapeutic pressure. New technologies in CAR-T cell engineering are directed at neutralizing inhibitory ligands and factors in the tumor microenvironment, preventing CAR-T cell exhaustion and enhancing selectivity for tumor cells with ‘smart’ CAR designs. The manufacture of CAR-T cells using virus-free protocols is anticipated to reduce supply-chain complexity and to improve patient access.

Summary
CD19 CAR-T cell therapy is an approved treatment for B-cell leukemia and -lymphoma and considering the current ‘target and technology’ pipeline, we anticipate that additional CAR-T cell products will accomplish their ‘breakthrough’ and clinical proof-of-concept in other indications in hematology and in oncology. Technologies to enhance therapeutic index and facilitate manufacturing will be key for assuring availability and accessibility of CAR-T cell products and their implementation into routine clinical practice.
Clinical Presentation and Outcome of Patients With Spontaneous Coronary Artery Dissection Versus Atherosclerotic Coronary Plaque Dissection
Authors: Daoulah Amin , Al-Faifi Salem M. , Madan Mina , Arafat Amr A. , Hersi Ahmad S. , Alasmari Abdulaziz , Clarkson Francis A. , Ball Vincent , Alkhushail Abdullah , Khan Abdul Salim , Asrar Farhan M. , Haq Ejazul , Alama Mohamed Nabil , Faden Mazen S. , Lotfi Amir 
Published: July 2020

Background:
Atherosclerotic coronary plaque dissection (ACPD) is one cause of acute coronary syndrome (ACS) caused by underlying atherosclerosis. Spontaneous coronary artery dissection (SCAD) occurs outside the setting of atherosclerosis among young women and individuals with few or no conventional atherosclerotic risk factors, and has emerged as an important cause of ACS, and sudden death. A comparison between ACPD and SCAD has not been previously addressed in the literature. Our study will compare ACPD and SCAD.

Methods:
Patients with confirmed diagnosis of SCAD and ACPD were retrospectively identified from 30 centers in 4 Arab Gulf countries between January 2011 and December 2017. In-hospital (ventricular tachycardia/ventricular fibrillation, myocardial infarction (MI), percutaneous coronary intervention, dissection extension, cardiogenic shock, death, implantable cardioverter-defibrillator placement) and follow-up (MI, de novo SCAD, spontaneous superior mesenteric artery dissection, death) events were compared between them.

Results:
Eighty-three cases of SCAD and 48 ACPD were compared. ACPD patients were more frequently male (91.67% vs. 49.40%, P < 0.001) and older (58.5 vs. 44, P < 0.001). Cardiovascular risk factors were more prevalent in patients with ACPD, including diabetes mellitus (60.4% vs. 25.3%), dyslipidemia (62.5% vs. 38.5%), and hypertension (62.5% vs. 31.3%), P < 0.001. Hospital presentation of ST-elevation MI was diagnosed in 48% of SCAD versus 27% of ACPD patients (P = 0.012). SCAD patients received medical-only treatment in 40% of cases and ACPD in 21% (P = 0.042). In-hospital and follow-up events were comparable in both groups (P = 0.25).

Conclusions:
Despite a completely different pathophysiology of ACS between SCAD and ACPD, in-hospital and follow-up events were comparable.
COVID-19 Recommendations From Ophthalmic and Plastic Reconstructive Surgery Societies Worldwide
Authors: Nguyen Anne X. , Gervasio Kalla A. , Wu Albert Y. 
Published: July 2020

Purpose:
The COVID-19 pandemic has brought unprecedented challenges for oculoplastic surgeons worldwide, in terms of care delivery, medical equipment and at-risk patient management. To date, there are no centralized or compiled international COVID-19 guidelines for oculoplastic surgeons.

Methods:
We examined COVID-19 guidelines published by oculoplastic societies worldwide. All countries around the world were initially considered in this study, but only 9 oculoplastic societies met the inclusion criteria: (1) publicly available guidelines displayed on the oculoplastic society’s website, or (2) guidelines received from the oculoplastic society after contacting them twice using the contact information on their website.

Results:
The 9 oculoplastic societies examined include: the American Society of Ophthalmic Plastic and Reconstructive Surgery, the British Oculoplastic Surgery Society, the Canadian Society of Oculoplastic Surgery, the European Society of Ophthalmic Plastic and Reconstructive Surgery, la Sociedad Española de Cirugía Plástica Ocular y Orbitaria, la Asociación Colombiana de Cirugía Plastica Ocular, the Asia Pacific Society of Ophthalmic Plastic & Reconstructive Surgery, the Oculoplastics Association of India, and the Philippine Society of Ophthalmic Plastic and Reconstructive Surgery. They all agree that urgent procedures should not be delayed, while non-necessary procedures (including all elective clinic services) should be postponed. When adequate protective equipment is available, oculoplastic surgeons must treat urgent cases. Eight out of 9 societies have provided recommendations on personal protective equipment use in order to prevent the spread of COVID-19 and to adequately protect mucous membranes. Other recommendations provided by certain societies are related to shelter in place measures, hand hygiene and surface disinfection protocols, patient triage, and thyroid eye disease management.

Conclusions:
All 9 societies with published recommendations have provided valuable recommendations to their members, regarding urgency of care and infection control solutions (personal protective equipment, hand hygiene, telemedicine, and social isolation).
Peripheral nerve injury and sensitization underlie pain associated with oral cancer perineural invasion
Authors: Salvo Elizabeth , Campana Wendy M. , Scheff Nicole N. , Nguyen Tu Huu , Jeong Se-hee , Wall Ian , Wu Angie K. , Zhang Susanna , Kim Hyesung , Bhattacharya Aditi , Janal Malvin N. , Liu Cheng , Albertson Donna G. , Schmidt Brian L. , Dolan John C. , Schmidt Robert E. , Boada M. Danilo , Ye Yi 
Published: July 2020

Abstract:
Cancer invading into nerves, termed perineural invasion (PNI), is associated with pain. Here, we show that oral cancer patients with PNI report greater spontaneous pain and mechanical allodynia compared with patients without PNI, suggesting that unique mechanisms drive PNI-induced pain. We studied the impact of PNI on peripheral nerve physiology and anatomy using a murine sciatic nerve PNI model. Mice with PNI exhibited spontaneous nociception and mechanical allodynia. Perineural invasion induced afterdischarge in A high-threshold mechanoreceptors (HTMRs), mechanical sensitization (ie, decreased mechanical thresholds) in both A and C HTMRs, and mechanical desensitization in low-threshold mechanoreceptors. Perineural invasion resulted in nerve damage, including axon loss, myelin damage, and axon degeneration. Electrophysiological evidence of nerve injury included decreased conduction velocity, and increased percentage of both mechanically insensitive and electrically unexcitable neurons. We conclude that PNI-induced pain is driven by nerve injury and peripheral sensitization in HTMRs.
Mitochondrial bioenergetics, glial reactivity, and pain-related behavior can be restored by dichloroacetate treatment in rodent pain models
Authors: Lagos-Rodríguez Valentina , Martínez-Palma Laura , Marton Soledad , Miquel Ernesto , Escobar-Pintos Ricardo , Cassina Adriana , Lago Natalia , Cassina Patricia 
Published: July 2020

Abstract
Glial reactivity in the dorsal horn of the spinal cord is a hallmark in most chronic pain conditions. Neuroinflammation-associated reactive glia, in particular astrocytes, have been shown to exhibit reduced mitochondrial respiratory function. Here, we studied the mitochondrial function at the lumbar spinal cord tissue from complete Freund's adjuvant-induced inflammatory pain rat and chronic constriction injury mouse models by high-resolution respirometry. A significant decrease in mitochondrial bioenergetic parameters at the injury-related spinal cord level coincided with highest astrocytosis. Oral administration of dichloroacetate (DCA) significantly increased mitochondrial respiratory function by inhibiting pyruvate dehydrogenase kinase and decreased glial fibrillary acidic protein and Iba-1 immunoreactivity in spinal cord. Importantly, DCA treatment significantly reduced the ipsilateral pain-related behavior without affecting contralateral sensitivity in both pain models. Our results indicate that mitochondrial metabolic modulation with DCA may offer an alternative therapeutic strategy to alleviate chronic and persistent inflammatory pain.