bluuebunny/crossref_metadata_2025_split huggingface dataset:
Malformations Associated With Pediatric Congenital Cholesteatomas
Authors: Distinguin Léa , Simon François , Alvin Fiona , Couloigner Vincent , Leboulanger Nicolas , Garabédian Noel , Loundon Natalie , Denoyelle Françoise 
Published: July 2020

Objective:
To describe malformations associated with pediatric congenital cholesteatomas of the middle ear.

Study Design:
Retrospective study.

Setting:
Tertiary referral center.

Patients:
One hundred and seventy-three cases of middle ear congenital cholesteatoma (CC) in 171 children operated between 2007 and 2017.

Interventions:
Demographic, clinical, and surgical data were collected from operative reports.

Main Outcome Measures:
We first described the type and rate of malformations associated with CC. Secondly, we compared cholesteatoma features in two subgroups: anterior superior (AS) versus posterior superior (PS) starting point. Third, we compared demographic, clinical, and surgical data between patients with and without malformation.

Results:
CC was associated with malformations in 17 cases (17/173; 9.8%). The main malformation was preauricular fistula (8/173; 4.6%). Other malformations were: one first branchial cleft, two labio palatine cleft, one nasal cyst, two preauricular fibrochondroma, and five other malformations. PS congenital cholesteatomas were diagnosed in older children (4.6 versus 8.6 years, p < 0.05) and had greater extension in middle ear than the AS cholesteatoma (39.7% versus 95.8%, p < 0.05). We did not find any significant difference between these two groups regarding the associated malformations. We did not find a difference in clinical presentation of CC between patients with and without associated malformation.

Conclusions:
We found various associated malformations in 9.8% of CC cases with no statistical difference in the malformation rate between AS and PS groups. All the malformations were located in the craniofacial region suggesting that genes implicated in craniofacial development may play a role in the pathophysiology of CC.
The Risks of Being Otologist, an Ergonomic and Occupational Hazard Review
Authors: Stern Shavit Sagit , Golub Justin S. , Lustig Lawrence R. 
Published: July 2020

Objective:
To review occupational ergonomic risks for the Otologist and Neurotologist.

Data Sources:
MEDLINE, OVID, PubMed, and Google Scholar.

Study Selection:
A search was conducted to identify all studies in the English language that involve ergonomic-related risks for surgeons.

Results:
Occupational hazards, particularly musculoskeletal disorders (MSDs), are common in the surgical community in general and among Otolaryngologists in particular. Very few studies have been conducted assessing MSDs specific to Otologists and Neurotologists. However, extrapolating from other surgical professions with similar ergonomic postures in the operation room and office, one can infer that cervical and lumbar pain are related to prolonged static sitting and neck flexion when working with a microscope and begins early in training. Early institution of correct ergonomic training is feasible and may be effective. Improved ergonomic habits include upright sitting, avoidance of neck flexion, initiating short breaks, and the use of chairs with arm and back support. Future technologies incorporated into otologic surgery should have improved ergonomic design.

Conclusions:
Otologists and Neurotologists are exposed to MSDs directly related to their work demands. Incorporating healthy ergonomics into surgical training as well adopting correct posture and the use equipment designed for back support may help mitigate the long-terms risks of MSD.
Dietary treatment to lower cholesterol and triglyceride and reduce cardiovascular risk
Authors: Welty Francine K. 
Published: July 2020

Purpose of review
To provide an update on dietary measures to lower levels of LDL-C and triglyceride and reduce cardiovascular (CVD) outcomes.

Recent findings
Fifty-year follow-up in the Seven Countries Study confirmed that cholesterol levels correlate with saturated fat intake and all-cause mortality and age at death. In the PURE study, refined carbohydrate increased CVD risk whereas saturated fat did not despite increasing LDL-C levels; limitations are discussed. Reports on CVD risk with eggs provide conflicting results. Plant-based diets with healthful complex carbohydrates reduced CVD. The REDUCE-IT trial lowered triglyceride 21.6% and reduced CVD events 26.1% with an omega-3 fatty acid, An omega-3 fatty acid index at least 4% with EPA and docosahexaenoic acid prevented coronary plaque progression. A clinician guide to counsel patients on nutrition and heart healthy diets was recently published.

Summary
Based on the evidence, individuals should continue to minimize saturated fats and refined carbohydrates, eliminate trans-fat and increase fruits, vegetables, whole grains, low-fat dairy, and fish or other omega-3 fatty acids. Adhering to a Mediterranean diet is strongly recommended because of lowering CVD and total mortality. High-dose omega-3 fatty acids lower triglyceride, reduce CVD and prevent coronary plaque progression.
Update on adrenarche
Authors: Witchel Selma Feldman , Pinto Bianca , Burghard Anne Claire , Oberfield Sharon E. 
Published: July 2020

Purpose of review
Adrenarche is the pubertal maturation of the innermost zone of the adrenal cortex, the zona reticularis. The onset of adrenarche occurs between 6 and 8 years of age when dehydroepiandrosterone sulfate (DHEAS) concentrations increase. This review provides an update on adrenal steroidogenesis and the differential diagnosis of premature development of pubic hair.

Recent findings
The complexity of adrenal steroidogenesis has increased with recognition of the alternative ‘backdoor pathway’ and the 11-oxo-androgens pathways. Traditionally, sulfated steroids such as DHEAS have been considered to be inactive metabolites. Recent data suggest that intracellular sulfated steroids may function as tissue-specific intracrine hormones particularly in the tissues expressing steroid sulfatases such as ovaries, testes, and placenta.

Summary
The physiologic mechanisms governing the onset of adrenarche remain unclear. To date, no validated regulatory feedback mechanism has been identified for adrenal C19 steroid secretion. Available data indicate that for most children, premature adrenarche is a benign variation of development and a diagnosis of exclusion. Patients with premature adrenarche tend to have higher BMI values. Yet, despite greater knowledge about C19 steroids and zona reticularis function, much remains to be learned about adrenarche.
In the Short‐term, Milk Fat Globule Epidermal Growth Factor‐8 Causes Site‐specific Intestinal Growth in Resected Piglets
Authors: Turner Justine M. , George Petro , Lansing Marihan , Slim George , Wizzard Pamela R. , Nation Patrick , Brubaker Patricia L. , Wales Paul W. 
Published: July 2020

ABSTRACTObjectives:Short bowel syndrome (SBS) remains the leading cause of neonatal intestinal failure. Milk fat globule epidermal growth factor‐8 (MFG‐E8), present in human milk, has homology with epidermal growth factor (EGF), known to enhance adaptation in SBS. In this pilot study, the role of oral MFG‐E8 treatment in SBS was explored in neonatal piglets.Methods:Neonatal piglets underwent 75% intestinal resection, either distal (jejunal‐colonic [JC] anastomosis) or mid‐intestinal (jejunal‐ileal [JI] anastomosis). Piglets were randomized to intragastric treatment with MFG‐E8 (5 mg/kg per day) or saline and were maintained on parenteral nutrition and enteral nutrition for 7 days. Adaptation was assessed by intestinal length and weight, histopathology, fecal fat analysis and RT‐qPCR analysis of mucosal transcripts, including growth factors.Results:JI piglets demonstrated intestinal lengthening (P < 0.001), 2‐fold greater in ileum than jejunum (P = 0.02), where lengthening was increased by MFG‐E8 treatment (P = 0.02). JC piglets did not exhibit jejunal lengthening, regardless of treatment. Fat absorption was greater for JI piglets (P = 0.02), unaffected by treatment. In JI piglets, expression of Egf was increased in the ileum (P < 0.01) and MFG‐E8 treatment increased Egfr (receptor) expression (P = 0.02).Conclusions:MF‐EG8 demonstrated site‐specific trophic effects, only with JI anatomy. This may limit the utility of this treatment for SBS, except for rare patients with retained ileum. The mechanisms of these site‐specific effects, however, and the role of MFG‐E8 in neonatal gut growth and in diseases, such as necrotizing enterocolitis that commonly target ileum, warrant further exploration.
WD Repeat Domain 1 (WDR1) Deficiency Presenting as a Cause of Infantile Inflammatory Bowel Disease
Authors: Millstead Jenna , Kamat Aarti , Duffner Ulrich , Abdel‐Mageed Aly , Freswick Peter , Dickens David , Stumph Jennifer , Prokop Jeremy W. , Hartog Nicholas L. 
Published: July 2020

ABSTRACTInfantile and very early onset inflammatory bowel disease (VEOIBD) are a rare phenomenon wherein patients develop intestinal inflammation with typical IBD symptoms before ages 2 and 6, respectively. In recent years, there has been an increasing number of monogenetic immunological disorders identified that lead a child to develop VEOIBD. We present a case of an infant boy who presented with hematochezia and thrombocytopenia in the first week of life and developed IBD by the age of 1 month. Additional clues to his diagnosis included lymphopenia and nuclear herniation observed in his neutrophils. Compound heterozygous damaging variants were identified in WD Repeat Domain 1 (WDR1) by whole‐exome sequencing (WES) and represents a novel cause of VEOIBD. Our patient's IBD and immunologic phenotype was successfully treated by hematopoietic stem cell transplant (HSCT).
A Narrow Window
Authors: Runde Joseph , Mears Macy , Guandalini Stefano , Jericho Hilary 
Published: July 2020

ABSTRACTAn expanding gluten‐free marketplace has left children with celiac disease and their families with a host of new dietary options. The quality of these foods is inconsistent and processed items may be high in caloric content while lacking nutritional value. Assessing the dietary preferences of a cohort of children with celiac disease via cross‐sectional survey, we find that these processed food items have become a staple of the gluten‐free diet, and in many cases, these foods are consumed to the exclusion of healthy alternatives. Furthermore, children with celiac disease and their families become less interested in dietary education over time, indicating that the greatest opportunity for imparting a healthy diet may occur at the time of diagnosis.
Does the Treatment After Kasai Procedure Influence Biliary Atresia Outcome and Native Liver Survival?
Authors: Pietrobattista Andrea , Mosca Antonella , Liccardo Daniela , Alterio Tommaso , Grimaldi Chiara , Basso MariaSole , Saffioti Maria Cristina , Corte Claudia Della , Spada Marco , Candusso Manila 
Published: July 2020

ABSTRACTObjectives:Biliary atresia (BA) is a rare and progressive idiopathic disease affecting the biliary tract that can lead to end‐stage liver disease. The main treatment is Kasai portoenterostomy (KP). The use of adjuvant therapy (AT; prophylactic antibiotics and steroids) after KP aims to prevent cholangitis and reduce the need for liver transplantation (LT), but there is a lack of evidence on their effectiveness. We investigated the impact of significant changes in the post‐KP protocol on the overall outcomes of BA.Methods:We enrolled 43 consecutive infants undergoing KP at Bambino Gesù Children's Hospital between July 2012 and October 2018. We compared AT (AT group; n=25) against no treatment (AT‐free group; n = 18).Results:No significant differences in anthropometric and laboratory parameters were shown between the 2 groups at baseline and every study evaluation (1, 3, and 6 months). The incidences of clinical complications of liver disease were similar. Six months post‐KP, the achievement of serum total bilirubin ⩽1.5 mg/dL and satisfactory Pediatric End‐Stage Liver Disease scores were not significantly different between the 2 groups. Cholangitis was observed in 30% of patients in the first 6 months postoperatively: 33% and 28% in the AT‐free and AT groups, respectively (P = 0.18). Survival to LT listing at 12 months and without LT at 24 months were not significantly different between the 2 groups (P > 0.05).Conclusions:AT after KP confirmed conflicting results; therefore, multicentered, prospective, randomized control studies are needed to better understand its utility after KP, especially in the multidrug resistance spread era.
Marijuana Use, Sexual Behaviors, and Prevalent Sexually Transmitted Infections Among Sexually Experienced Males and Females in the United States: Findings From the National Health and Nutrition Examination Surveys
Authors: Patel Eshan U. , White Jodie L. , Gaydos Charlotte A. , Quinn Thomas C. , Mehta Shruti H. , Tobian Aaron A.R. 
Published: July 2020

Background
Several national guidelines consider illicit drug use as an indication for testing and/or counseling for some sexually transmitted infections (STIs). The legal and social landscape of marijuana use is changing, and its relevance with STI risk is unclear.

Methods
Sex-specific prevalence of T. vaginalis and/or C. trachomatis infection was examined by past-year marijuana use (no vs yes) among 2958 sexually experienced, 20- to 39-year-old participants of the 2013–2016 National Health and Nutrition Examination Surveys. Prevalence ratios (PRs) with 95% confidence intervals [CIs] were estimated by Poisson regression. Adjusted PRs (aPR) were estimated following propensity score covariate-adjustment accounting for sociodemographics, alcohol use, injection drug use, depression, and age at sexual debut.

Results
Past-year marijuana use was reported by 27.3% and 36.3% of females and males, respectively. Male and female past-year marijuana users were more likely to have new and multiple sexual partners in the past year (P < 0.05). Past-year marijuana use was associated with prevalent C. trachomatis and/or T. vaginalis infection among females (7.4% vs. 2.9%; PR, 2.57 [95% CI, 1.62–4.07]) and males (4.0% vs. 1.1%; PR, 3.59 [95% CI, 1.96–6.58]), but this association was attenuated after propensity score covariate adjustment among females (aPR, 1.15 [95% CI, 0.72–1.83]) and males (aPR, 2.10 [95% CI, 0.88–5.02]). Additional adjustment for new or multiple sexual partners further attenuated the associations (aPRs, 1.02 [95% CI, 0.65–1.51] and 1.91 [95% CI, 0.82–4.47] for females and males, respectively).

Conclusions
Sexually transmitted infection prevalence was higher among persons with a past-year history of marijuana use; however, this association was not significant after accounting for measured confounders. Additional work is needed to characterize STI prevalence by the mode, duration, and frequency of marijuana use.
Specific Biomarker Expression Patterns in the Diagnosis of Residual and Recurrent Endometrial Precancers After Progestin Treatment
Authors: Chen Hao , Lucas Elena , Strickland Amanda L. , Carrick Kelley , Gwin Katja , Castrillon Diego H. , Rivera-Colon Glorimar , Niu Shuang , Molberg Kyle H. , Zheng Wenxin 
Published: July 2020

Background:
Conservative management with progestin is a treatment option for atypical hyperplasia (AH). However, pathologic diagnosis of residual/recurrent lesions is often problematic because of the profound morphologic changes induced by progestin and the lack of established diagnostic criteria for progestin-treated residual AH.

Methods:
We conducted a longitudinal study of 265 endometrial biopsies from 54 patients with a history of AH on progestin therapy. Patient outcomes were divided into 3 categories after morphologic review and immunohistochemical staining with phosphatase and tensin homolog (PTEN) and paired box 2 (PAX2): (1) persistent or residual disease; (2) recurrent disease; (3) complete response. All specimens were classified into 3 categories based on morphology: (1) persistent/recurrent disease (nonresponse), (2) morphologically uncertain response, (3) optimally treated (complete response). The staining patterns of PTEN/PAX2 were tracked over time in individual patients and correlated with morphologic findings before and after progestin therapy.

Results:
Our data showed that aberrant expression patterns of PTEN and/or PAX2 were identified in 48 (88.9%) of the 54 primary biopsies and persisted in persistent/recurrent AH across serial endometrial biopsies (n=99, P<0.00001), while normal PTEN and PAX2 expressions were consistently observed in optimally treated cases (n=84, P<0.00001). More importantly, follow-up biopsies that showed a morphologically uncertain response but a PTEN/PAX2 expression pattern identical to the initial biopsy were significantly correlated with persistent or recurrent disease (n=18, P=0.000182), as evidenced by areas with morphologic features diagnostic of AH on subsequent biopsy.

Conclusions:
Biomarker PTEN/PAX2 signatures offer a valuable diagnostic aid to identify residual AH in progestin-treated endometrial samples for which the biomarker status from preprogestin treated AH is known. The findings of this study are promising for a possible future change of diagnostic practice.
Analysis of Risk Factors for Patient Safety Events Occurring in the Emergency Department
Authors: Alsabri Mohamed , Boudi Zoubir , Zoubeidi Taoufik , Alfaki Ibrahim Abdalla , Levy Phillip , Oneyji Churchill , Shan Liu , Camargo Carlos A. , Michel Philippe , Tazarourte Karim , Hachimi-Idrissi Said , Grossman Shamai , Bellou Abdelouahab 
Published: July 2020

Objective
The aim of the study was to describe and analyze the risk factors associated with patient safety events (PSEs), defined as adverse events (AEs), preventable AEs (PAEs), and near-miss events (NMEs), in the emergency department (ED).

Methods
It was a retrospective cohort study using ED patients’ data retrieved from January 2010 to December 2016. Quality assurance issues (QAIs) used as triggers included the following: issues during procedural sedation, death within 24 hours of admission, patients’ and physicians’ complaints, returns to the ED within 72 hours, and transfers to an intensive care unit within 24 hours.

Results
Of 383,586 ED visits, 6519 (1.7%) QAIs were reported with a PSEs incidence of 6.1%. Among the 397 PSEs, 258 were AEs including 82 PAEs, and 139 NMEs. During the 7-year period, we observed a fourfold increase in NMEs, and despite a decrease in the rate of AEs with the highest (3.1%) and lowest (0.8%) incidence in 2011 and 2016, respectively, the incidence of PAEs events remained relatively constant. Unadjusted analysis showed that ED waiting time, boarding time, ED length of stay (LOS), ED disposition, as well as diagnostic and QAIs were significantly related to PSEs (P < 0.05). Multivariable analysis showed that the type of QAIs and diagnostic were associated with PSEs (P < 0.001). Type of QAIs was a risk factor for AEs and PAEs occurrence and factors involved in NMEs were type of QAIs (P = 0.02) and ED LOS (P < 0.001). “The odds of a PSE occurring increased by 0.2% for each additional minute increase in the ED waiting time, by 5.2% for each additional boarding hour, and by 4.5% for each ED LOS hour.”

Conclusions
This study showed several potential risk factors for PSEs, especially ED LOS, type of QAIs, and diagnostic. Systematic interventions might have more impact on risk of PSE.
The Incidence and Preventability of Adverse Events in Older Acutely Admitted Patients: A Longitudinal Study With 4292 Patient Records
Authors: Schouten Bo , Merten Hanneke , Spreeuwenberg Peter M. M. , Nanayakkara Prabath W. B. , Wagner Cordula 
Published: July 2020

Objectives
Acute care crowding is a global issue, jeopardizing patient safety. An important cause of crowding is the growing number of older, vulnerable, and complex patients. This group is at higher risk of experiencing (preventable) adverse events (AEs) than younger patients.
This study aimed to identify the incidence, preventability, nature, and prevention strategies of AEs in older patients during an acute hospital admission and to assess changes over time.

Methods
We analyzed data of 4292 acutely admitted patients (70+) who died in the hospital, using data of a multicenter Dutch AE record review study (2008, 2012, 2016). Multilevel logistic regression analyses were performed to adjust for patient-mix differences and clustering on department/hospital level per year.

Results
The incidence of AEs in this group declined significantly (χ2(1) = 8.78, P = 0.003) from 10.7% (95% confidence interval [CI] =8.2–13.9) in 2008, 7.4% (95% CI = 5.6–9.7) in 2012, to 7.2% (95% CI = 5.5–9.3) in 2016. The relative preventability showed a significant parabolic trend (χ2(1) = 4.86, P = 0.027), from 46.2% (95% CI = 34.1–58.7), to 32.4% (95% CI = 21.1–46.1), to 44.6% (95% CI = 32–58). Adverse events were often related to medication (26.3% in 2008, 35.1% in 2012, and 39.5% in 2016), and the preventability in AEs related to diagnosis was highest (88.3%, 70.8%, and 79.9%).

Conclusions
The incidence of AEs in older acutely admitted patients declined over the years; however, the preventability increased again after an initial decline. This could be related to crowding or increasing complexity in the acute care chain. Further monitoring and improvement in (preventable) AE rates are necessary to pinpoint areas of improvement to make hospital care for this vulnerable group safer.
Implications of Training Pathways on Future Academic Plastic Surgeon Employment
Authors: Glener Adam D. , Glener Steven R. , Shammas Ronnie L. , Le Elliot , Cason Roger W. , Rezak Kristen , Phillips Brett T. 
Published: July 2020

Background
The limited supply of academic plastic surgery positions has led to increased demand and strong competition for these desired positions. Residents and students now seek out academic opportunities earlier in their training to account for this employment shortage. Training pathways and locations play an extremely important role in obtaining an academic position at most institutions. This study aimed to evaluate the training patterns of academic plastic surgeons in an attempt to elucidate its value and role for trainees interested in pursuing future academic careers.

Methods
All full-time faculty members at currently accredited integrated and independent plastic surgery programs were included in the study; clinical affiliates were excluded. These institutions' websites were then queried to obtain the training history of the surgeons meeting inclusion criteria. Data were entered into a centralized database from which descriptive statistics were obtained.

Results
In the 741 surgeons included in the study, 514 (69.4%) completed the independent plastic surgery track and 227 (30.6%) completed the integrated pathway. Residents completing the independent track had 20.8% and 31.7% employment at the same institution where they finished their general and plastic surgery residency, respectively. Of those completing the integrated pathway, 33.9% are employed at the same institution where they graduated from residency. In addition, 47.9% of the surgeons included in the study completed medical school, residency, or fellowship at the current institution at which they are employed. Lastly, 512 surgeons (69.4%) completed at least 1 postresidency fellowship.

Conclusions
Academic surgeons commonly complete a postresidency fellowship and are often employed at institutions where they have formerly trained. Trainees considering an academic career should consider these patterns when planning their future careers.