bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pilot Evaluation of Sheep as In Vivo Model for Cochlear Implantation
Authors: Kaufmann Christopher R. , Tejani Viral D. , Fredericks Douglas C. , Henslee Allan M. , Sun Daniel Q. , Abbas Paul J. , Hansen Marlan R. 
Published: February 2020

Objectives:
The rise in the use of cochlear implants (CIs) has continued to fuel research aimed at improving surgical approaches and the preservation of residual hearing. Current in vivo models involve small animals not suitable for evaluating full-sized CIs nor are prohibitively expensive nonhuman primates. The objective of this study was to develop and evaluate an in vivo model of cochlear implantation in sheep.

Methods:
Eight adult, female sheep were implanted with full-sized CIs from three manufacturers using a retrofacial approach to the round window. Partial electrode insertions were performed to a depth of 10 to 12 mm before closure. Round window electrocochleography (ECoG) and auditory brainstem responses (ABR) were conducted during and after surgery. Following a 30-day implantation, cochleae were explanted and imaged using both x-ray microscopy and histology.

Results:
The surgery was well tolerated although limited complications were observed in three of eight sheep. Electrode insertions were up to 12 mm before insertion resistance noted. ECoG and ABR responses were reduced postimplantation, reflecting changes in cochlear mechanics due to the presence of the implant, and/or insertion trauma. Histological and radiological image analysis showed the presence of intracochlear fibrosis as well as one instance of tip fold-over.

Conclusions:
The use of sheep presents a feasible live-animal model to study cochlear implantations. Full-sized implants as well as surgical techniques can be evaluated on functional outcomes such as ABR and ECoG as well as histological markers for residual hearing including intracochlear fibrosis. Use of this model and surgical approach has potential to evaluate CIs and surgical techniques in both the acute and chronic setting.
Congenital Cholesteatoma of the Sphenoid, Occipital, and Temporal Bones: 54-Year Follow Up
Authors: Maxwell Anne K. , Muelleman Thomas , Brackmann Derald E. 
Published: February 2020

Objective:
To provide long-term follow up of a unique patient with history of massive petrous apex congenital cholesteatoma.

Patient:
75-year-old man who presented at age 18 with left Gradenigo-like syndrome.

Intervention:
Staged left radical mastoidectomy and open transsphenoidal marsupialization. Followed with routine in-office cholesteatoma debridement.

Main Outcome Measures:
Bony erosion on computed tomography (CT), monitoring for new symptoms.

Results:
Interval development of sensorineural component of hearing loss due to cochlear fistulization. Very slow skull base bony erosion continues without neck destabilization. No new or recurrent cranial neuropathies have developed.

Conclusions:
Marsupialized cholesteatoma may be followed with routine debridement and interval imaging for monitoring over many decades.
Association of lymphocyte-to-monocyte ratio with total coronary plaque burden in patients with coronary artery disease
Authors: Si Yueqiao , Liu Jingyi , Shan Weichao , Zhang Ying , Han Chao , Wang Ruijuan , Sun Lixian 
Published: February 2020

Background:
Lymphocyte-to-monocyte ratio (LMR) is involved in all stages of coronary atherosclerosis and related to coronary artery disease (CAD). However, the correlation between LMR and the coronary plaque burden of CAD is not clearly elucidated. Therefore, this study aimed to investigate their correlation in patients with CAD.

Methods:
A total of 1953 consecutive eligible inpatients with suspected CAD were retrospectively included in this study. They were assigned into CAD (n = 564) and non-CAD groups (n = 1389). All patients underwent coronary computed tomographic angiography to evaluate coronary stenosis and coronary artery calcification (CAC). Spearman’s tests were used to analyze the correlation between CAC score and LMR. Multivariate logistic regression models were set up to assess the risk factors of CAD.

Results:
Patients with CAD had lower LMR value than patients without CAD (P = 0.001). LMR was negatively correlated with CAC score and was an independent risk factor of CAC score (P < 0.05). Multivariate logistic regression model showed that LMR ≤4.8 was a newly independent risk factor of CAD (all P < 0.05). Additionally, the new risk score model was compared with the Framingham model and showed that NRI was 4.9%, which proved that the new risk score model improved the prediction capability of CAD.

Conclusion:
LMR ≤4.8 is a new independent risk factor of CAD. LMR value was negatively correlated with CAC score and could be used as a new marker to evaluate the coronary plaque burden of CAD.
The utility of liver biopsy in 2020
Authors: Khalifa Ali , Rockey Don C. 
Published: February 2020

Purpose of review
Over the past decade, imaging modalities and serological tests have emerged as important tools in the evaluation of liver diseases, in many cases supplanting the use of liver biopsy and histological examination. Nonetheless, the accuracy and diagnostic value of these methods may not always be conclusive and the assessment of liver histology often remains the gold standard for diagnostic evaluation. The purpose of this review is to summarize the current role of liver biopsy in contemporary hepatology practice.

Recent findings
Technical factors were found to influence the diagnostic value of liver biopsy and histological examination of the liver, including specimen number and size (preferably ≥3 nonfragmented specimens of >20 mm in length), needle diameter (1.6 mm Menghini), number of passes (mean 2.5), imaging-guidance, and operator experience. Liver biopsy was demonstrated to be diagnostically valuable in the evaluation of persistently abnormal liver tests of unclear cause, with histology pointing to a specific diagnosis in 84% of patients. Although coagulation abnormalities continue to be an important concern when performing liver biopsy, their influence on complication risk remains unclear. Implementation of less stringent preprocedural coagulation thresholds decreased preprocedural transfusions without increasing the bleeding rate. Serious complications associated with percutaneous liver-biopsy (PLB) and transjugular liver-biopsy are similar, but pain appears to be more common with PLB.

Summary
Histopathological evaluation continues to be fundamentally important in assessing hepatic disease, and liver histology remains the most accurate approach to assess fibrosis and assign prognosis.
NR1H4‐related Progressive Familial Intrahepatic Cholestasis 5
Authors: Himes Ryan W. , Mojarrad Majid , Eslahi Atieh , Finegold Milton J. , Maroofian Reza , Moore David D. 
Published: February 2020

ABSTRACTPathogenic sequence variants in the nuclear bile acid receptor FXR, encoded by NR1H4, have been reported in a small number of children with low‐γ‐glutamyl transferase (GGT) cholestasis progressing to liver failure. We describe 3 additional children from 2 unrelated families with cholestasis and liver failure because of pathologic variants in NR1H4. One patient underwent liver transplantation and has had good clinical outcomes in 6 years of follow‐up. Although that patient has biochemical evidence of increased bile acid synthetic activity, he has not experienced post‐transplant diarrhea or allograft steatosis, as has been reported among other transplanted patients.
Differences in Management of Eosinophilic Esophagitis in Europe
Authors: Tourlamain Gilles , Garcia‐Puig Roger , Gutiérrez‐Junquera Carolina , Papadopoulou Alexandra , Roma Eleftheria , Kalach Nicolas , Oudshoorn Johanna , Sokollik Christiane , Karolewska‐Bochenek Kasia , Oliva Salvatore , Strisciuglio Caterina , Bauraind Olivia , Auth Marcus Karl‐Heinz , Thomson Mike , Otte Sebastian , Rok Orel , Dias Jorge Amil , Tzivinikos Christos , Urbonas Vaidotas , Kostovski Aco , Zevit Noam , Velde Saskia Vande ,   ESPGHAN EGID Working group
Published: February 2020

ABSTRACTObjectives:The aim of the study was to assess differences in the diagnosis and management of eosinophilic esophagitis (EoE) by European pediatric (PG) and adult gastroenterologists (AG), and their self‐reported adherence to guidelines.Methods:A multiple‐choice questionnaire gauged the diagnostic and management strategies of gastroenterologists treating children or adults in 14 European countries and the United Arab Emirates (UAE).Results:Questionnaires were completed by 465 PG and 743 AG. PG were significantly more likely to take biopsies in patients with symptoms of esophageal dysfunction (86.2% PG vs 75.4% AG, P < 0.001) and to perform endoscopic follow‐up (86.3% PG vs 80.6% AG, P < 0.001). After failure of proton‐pump inhibitors (PPIs), topical steroids were the preferred second‐line therapy; however, PG opted more frequently for elimination diets (47.5% PG vs 13.7% AG, P < 0.001). More PG than AG indicated having read recent guidelines (89.4% PG vs 58.2% AG, P < 0.001). Geographic differences in practice were reported, with respondents from the United Kingdom, Portugal, and Spain more often adhering to recommended biopsy protocols. Physicians in the UAE, France, Lithuania, and Poland tended to opt for steroid therapy or elimination diets as first‐line therapy, in contrast to most other countries.Conclusions:Significant differences in general practice between PG and AG were demonstrated with notable divergence from consensus guidelines. International practice variations are also apparent. Among other strategies, educational activities to highlight current recommendations may help harmonize and optimize clinical practice.
Adherence to Gluten‐free Diet in a Celiac Pediatric Population Referred to the General Pediatrician After Remission
Authors: Sbravati Francesca , Pagano Sara , Retetangos Cristiana , Spisni Enzo , Bolasco Giulia , Labriola Flavio , Filardi Maria C. , Grondona Ana G. , Alvisi Patrizia 
Published: February 2020

ABSTRACTObjectives:Assessment of adherence to gluten‐free diet in celiac disease (CD) is generally recommended. Few data are available about consequences of transition from the referral center to the general pediatrician (GP) once remission is achieved.Methods:Adherence was assessed in patients referred to the GP for an annual basis follow‐up, called back for re‐evaluation. Immunoglobulin A (IgA) antitissue transglutaminase (anti‐tTG) antibodies and the Biagi score (BS) were determined at last follow‐up at the referral center (V1), and at re‐evaluation (V2). Patients were classified as adherent (BS 3–4, IgA anti‐tTG <7 U/mL) and nonadherent (BS 0–2, IgA anti‐tTG ≥7). Scores of adherence were correlated with personal and clinical data.Results:We evaluated 200 patients. Overall, we found good adherence rates in 94.95% of patients at V1 and 83.5% at V2. IgA anti‐tTG were negative in 100% at V1 and 96.97% at V2. BS is 3 to 4 in 94.5% at V1 and 84% at V2. Adherence at V2 was significantly worse than V1 (P < 0.001). No significant associations were found between scores of adherence and sex, symptoms and age at diagnosis, family history of CD, comorbidity, and diagnosis by endoscopy. Age 13 years or older represents a risk factor for lack of compliance at V1 (P = 0.02) and V2 (P = 0.04), and foreign nationality at V2 (P = 0.001).Conclusions:The BS, serology, and a clinical interview, integrated, are reliable tools for assessing pediatric adherence to gluten‐free diet. We argue that referring patients to the GP after remission of CD is important, but the process must be improved and recommendations are required.
Factors Associated With Diagnostic Error on Admission to a PICU: A Pilot Study
Authors: Cifra Christina L. , Ten Eyck Patrick , Dawson Jeffrey D. , Reisinger Heather Schacht , Singh Hardeep , Herwaldt Loreen A. 
Published: February 2020

Objectives:
Diagnostic errors can harm critically ill children. However, we know little about their prevalence in PICUs and factors associated with error. The objective of this pilot study was to determine feasibility of record review to identify patient, provider, and work system factors associated with diagnostic errors during the first 12 hours after PICU admission.

Design:
Pilot retrospective cohort study with structured record review using a structured tool (Safer Dx instrument) to identify diagnostic error.

Setting:
Academic tertiary referral PICU.

Patients:
Patients 0–17 years old admitted nonelectively to the PICU.

Interventions:
None.

Measurements and Main Results:
Four of 50 patients (8%) had diagnostic errors in the first 12 hours after admission. The Safer Dx instrument helped identify delayed diagnoses of chronic ear infection, increased intracranial pressure (two cases), and Bartonella encephalitis. We calculated that 610 PICU admissions are needed to achieve 80% power (α = 0.05) to detect significant associations with error.

Conclusions:
Our pilot study found four patients with diagnostic error out of 50 children admitted nonelectively to a PICU. Retrospective record review using a structured tool to identify diagnostic errors is feasible in this population. Pilot data are being used to inform a larger and more definitive multicenter study.
The Evolution of Breast Satisfaction and Well-Being after Breast Cancer: A Propensity-Matched Comparison to the Norm
Authors: Mundy Lily R. , Rosenberger Laura H. , Rushing Christel N. , Atisha Dunya , Pusic Andrea L. , Hollenbeck Scott T. , Hyslop Terry , Hwang E. Shelley 
Published: February 2020

Background:
Breast cancer survival continues to improve, with women living longer after treatment. It is not well understood how long-term satisfaction and well-being differ following treatment or how types of reconstruction differ when compared to the norm.

Methods:
In a propensity-matched sample, the authors compared patient-reported outcomes in breast cancer patients at various time intervals from surgery with normative BREAST-Q data. All data were obtained using the Army of Women, an online community fostering breast cancer research. Breast cancer patients were stratified by surgical treatment and reconstruction type. Regression lines were estimated and differences in slope tested between cancer patients and noncancer controls.

Results:
The authors compared normative (n = 922) and breast cancer (n = 4343) cohorts in a propensity-matched analysis. Among the breast cancer patients, 49.4 percent underwent lumpectomy, 17.0 percent underwent mastectomy, 21.7 percent underwent implant reconstruction, and 11.9 percent underwent autologous reconstruction. Median time since surgery was 4.7 years, with 21.1 percent more than 10 years after surgery. At the time of survey, breast cancer patients reported higher Satisfaction with Breasts and Psychosocial Well-being scores compared to noncancer controls (p < 0.01), with the cohorts undergoing lumpectomy and autologous reconstruction both reporting higher scores than the normative controls. After mastectomy, scores averaged lower than the noncancer controls, but improved over time. However, all breast cancer groups reported significantly lower Physical Well-being scores than the noncancer cohort (all p < 0.01).

Conclusions:
Breast cancer patients undergoing lumpectomy or autologous reconstruction reported higher psychosocial well-being compared to noncancer controls. These differences were influenced both by time since treatment and by choice of surgical procedure.
Obesity and Breast Reconstruction: Complications and Patient-Reported Outcomes in a Multicenter, Prospective Study
Authors: Srinivasa Dhivya R. , Clemens Mark W. , Qi Ji , Hamill Jennifer B. , Kim Hyungjin M. , Pusic Andrea L. , Wilkins Edwin G. , Butler Charles E. , Garvey Patrick B. 
Published: February 2020

Background:
The authors’ purpose was to evaluate the effects of body mass index, as defined by World Health Organization criteria, on complications and patient-reported outcomes in implant-based and autologous breast reconstruction.

Methods:
Complications and BREAST-Q patient-reported outcomes were analyzed 2 years after breast reconstruction for women from 11 participating sites. Separate mixed-effects regressions were performed to assess body mass index effects on outcomes.

Results:
A total of 2259 patients (1625 implant-based and 634 autologous) were included. Women with class II/III obesity had higher risks of any complication in both the implant (OR, 1.66; p = 0.03) and autologous (OR, 3.35; p < 0.001) groups, and higher risks of major complications in both the implant (OR, 1.71, p = 0.04) and autologous (OR, 2.72; p = 0.001) groups, compared with underweight/normal weight patients. Both class I (OR, 1.97; p = 0.03) and class II/III (OR, 3.30; p = 0.001) obesity patients experienced higher reconstructive failures in the implant cohort. Class I obesity implant patients reported significantly lower Satisfaction with Breasts scores (mean difference, –5.37; p = 0.007). Body mass index did not significantly affect patient-reported outcomes for autologous reconstruction patients.

Conclusions:
Obesity was associated with higher risks for complications in both implant-based and autologous breast reconstruction; however, it only significantly affected reconstruction failure and patient-reported outcomes in the implant reconstruction patients. Quality-of-life benefits and surgical risk should be presented to each patient as they relate to her body mass index, to optimize shared decision-making for breast reconstruction.

CLINICAL QUESTION/LEVEL OF EVIDENCE:
Risk, I.
The Use of Botulinum Toxin in Pain Management: Basic Science and Clinical Applications
Authors: Hehr Jason D. , Schoenbrunner Anna R. , Janis Jeffrey E 
Published: February 2020

Summary:
Pain is an unpleasant experience resulting from either tissue damage or insults to the somatosensory system. Approaches to pain management evolve as we better understand both pain pathways and the tools available to interrupt these. The interest surrounding botulinum neurotoxin as a chemodenervating agent has expanded to include its potential applications in painful pathologies, both within and beyond the confines of plastic surgery. In this article, the authors discuss botulinum neurotoxin’s mechanism of action as it pertains to both muscular paralysis and its interplay in the modulation of proinflammatory pain mediators. In addition, the authors review evidence supporting the use of botulinum neurotoxin in common painful conditions, in order to prepare the readership to aptly provide their patients with evidence-based recommendations. After reading this article, the participant should be able to discuss both mechanism of action and common applications of botulinum neurotoxin in painful conditions.
Mitigating Biases in a Cohort Study: A Critical Examination of the Silicone Arthroplasty in Rheumatoid Arthritis Study
Authors: Kim You J. , Chung Kevin C. 
Published: February 2020

Summary:
Research bias, or the systematic errors of a study, can arise before, during, or after a trial ends. These biases hinder the internal validity of the study, which is the accuracy of a study’s conclusions regarding the effects of an intervention on a given group of subjects. With the growing use of evidence-based medicine, there is a demand for high-quality evidence from the research community. Systematic reviews and meta-analyses of randomized controlled trials are considered the highest level of evidence, followed by individual randomized controlled trials. However, most surgical trials cannot be conducted as randomized controlled trials because of factors such as patient preferences and lack of equipoise among surgeons. Therefore, surgical trials may lack features that are held as important standards for high-quality evidence, such as randomization and blinding. To demonstrate the biases that surgical trials may encounter, the authors examined a prospective cohort study, the Silicone Arthroplasty in Rheumatoid Arthritis study. The authors focus on the challenges that arise during a surgical trial, including the design, implementation, and methods used to report the clinical evidence. By recognizing and addressing obstacles that exist in research, investigators will provide health care providers with high-quality evidence needed to make well-informed, evidence-based clinical decisions.
Polymorphisms of SOCS-1 Are Associated With a Rapid HIV Progression Rate
Authors: Hersberger Martin , Schlaepfer Erika , Buehler Marco , Bochud Pierre-Yves , Vernazza Pietro , Marti-Jaun Jacqueline , Nemeth Johannes , Zwahlen Marcel , Schmidlin Kurt , Speck Roberto F. ,   the Swiss HIV Cohort Study
Published: February 2020

Objectives:
Immune activation, among others driven by interferon (IFN)-α and IFN-γ activation, is a main feature of progressive HIV infection. Suppressor of cytokine signaling (SOCS) 1 and 3 are negative feedback regulators of the IFN-α and IFN-γ axis. Here, we analyzed the role of 9 single-nucleotide polymorphisms (SNPs) within SOCS-1 and SOCS-3 genes for their association with an HIV progression rate in a cohort of 318 rapid vs 376 slow progressors from the Swiss HIV Cohort Study.

Design and Methods:
We analyzed 9 SNPs, which we have identified in Swiss blood donors, in a cohort of HIV-infected patients (n = 1144), which have been categorized according to the decline in CD4+ T-cell counts. In all the conducted analyses, we focused on the comparison between rapid and slow progressors with regard to SNPs in SOCS-1 and SOCS-3 and with regard to haplotypes using multivariate logistic regression models.

Results:
Three SOCS-1 SNPs (rs193779, rs33989964, and rs4780355) are associated with a risk reduction for rapid progression. Two of these SNPs, rs33989964 and rs4780355, are in strong linkage disequilibrium, forming a frequent haplotype. Homozygous carriers of this haplotype are also associated with a risk reduction for rapid progression. By contrast, the minor TT genotype of rs33977706 is associated with twice the risk for rapid progression. No associations have been observed for the 4 SOCS-3 SNPs or the major SOCS-3 haplotypes.

Conclusions:
Our data suggest that SNPs in SOCS-1 are associated with HIV disease progression and speak in favor that immune activation is causal for the progressive immunodeficiency.