bluuebunny/crossref_metadata_2025_split huggingface dataset:
Systematic Review of Surgical Outcomes Following Repair of Patulous Eustachian Tube
Authors: Ikeda Ryoukichi , Hidaka Hiroshi , Kikuchi Toshiaki , Ohta Nobuo , Kawase Tetsuaki , Katori Yukio , Kobayashi Toshimitsu 
Published: July 2020

Objective:To perform a systematic review and meta-analysis of surgical outcomes following repair of patulous Eustachian tube (PET).Data Sources:Analysis of cases collected from studies published between January 1990 and December 2018 and identified using PubMed, Google Scholar, and Cochrane databases.Study Selection:Articles addressing interventions to treat PET were selected.Data Extraction:The database was searched using the keywords “patulous Eustachian tube treatment” and yielded 1,370 studies. Twenty studies were eligible for inclusion.Data Synthesis:A total of 914 cases were evaluated for surgical approach, patient outcomes, and complications. The reported techniques were categorized by the procedure type, including ventilation tube insertion, mass loading of the tympanic membrane, ET injection, plug surgery, shim surgery, tuboplasty, and ET closure. Overall PET symptom improvement were reported for ventilation tube insertion (79 cases, mean: 58%, 95% CI: 47–69%), mass loading of the tympanic membrane (43 cases, mean: 50%, 95% CI: 32–69%), ET injection (139 cases, mean: 47%, 95% CI: 38–56%), plug surgery (386 cases, mean: 81%, 95% CI: 77–85%), shim surgery (122 cases, mean: 62%, 95% CI: 53–70%), tuboplasty (105 cases, mean: 41%, 95% CI: 31–51%), and ET closure (40 cases, mean: 66%, 95% CI: 49–80%). A low incidence of minor complications was reported.Conclusions:This systematic review describes clinical outcome data following surgical management of PET. Plug surgery and shim surgery show relatively high effectiveness and safety. Further prospective studies that compare surgical approaches for PET are needed.
Natural History of Growing Sporadic Vestibular Schwannomas: An Argument for Continued Observation Despite Documented Growth in Select Cases
Authors: Marinelli John P. , Lees Katherine A. , Lohse Christine M. , Driscoll Colin L.W. , Neff Brian A. , Link Michael J. , Van Gompel Jamie J. , Carlson Matthew L. 
Published: July 2020

Objective:
Definitive treatment of sporadic vestibular schwannoma (VS) following documented growth is common practice at most centers in the United States. However, as a natural extension of this paradigm, very little evidence exists surrounding the natural history of growing tumors. The primary objective of the current work was to describe the natural history of sporadic VS following documentation of initial tumor growth.

Study Design:
Retrospective cohort study.

Setting:
Tertiary referral center.

Patients:
Patients diagnosed with sporadic VS between January 1, 2001 and December 31, 2015 who elected continued observation despite having volumetric growth ≥20% of original tumor volume on interval magnetic resonance imaging (MRI).

Main Outcome Measure:
Survival free of subsequent volumetric growth.

Results:
Of 361 patients undergoing observation with serial imaging during the study period, 85 patients met inclusion criteria at a median age of 66 years (interquartile ranges [IQR] 55–71). Within this cohort, 40 patients demonstrated subsequent volumetric growth at a median of 1.7 years (IQR 1.0–2.6) from the date of initial MRI that documented growth. The median volumetric growth was 43% (IQR 28–57), and the median growth rate was 0.026 cm3 per year (IQR 0.009–0.107). Survival free of subsequent volumetric growth rates (95% CI; number still at risk) at 1, 2, 3, 4, and 5 years were 93% (87–99; 75), 67% (58–79; 45), 54% (43–67; 29), 44% (33–59; 19), and 41% (30–56; 12), respectively.

Conclusions:
In a cohort exclusively comprised of sporadic VS with documented growth, over 40% of tumors demonstrated no subsequent volumetric growth after 5 years of continued MRI surveillance. These data challenge the supposition that once growth occurs, all tumors will exhibit sustained growth. Continued observation after documented growth of sporadic VS is a reasonable consideration in appropriately selected cases.
Fluctuations in Subjective Tinnitus Ratings Over Time: Implications for Clinical Research
Authors: Chen Jenny X. , Whitton Jonathon P. , Parthasarathy Aravindakshan , Hancock Kenneth E. , Polley Daniel B. 
Published: July 2020

Objective:
Patients with chronic, subjective tinnitus are often administered a battery of audiometric tests to characterize their tinnitus percept. Even a comprehensive battery, if applied just once, cannot capture fluctuations in tinnitus strength or quality over time. Moreover, subjects experience a learning curve when reporting the detailed characteristics of their tinnitus percept, such that a single assessment will reflect a lack of familiarity with test requirements. We addressed these challenges by programming an automated software platform for at-home tinnitus characterization over a 2-week period.

Study Design:
Prospective case series.

Setting:
Tertiary referral center, patients’ homes.

Interventions:
Following an initial clinic visit, 25 subjects with chronic subjective tinnitus returned home with a tablet computer and calibrated headphones to complete questionnaires, hearing tests, and tinnitus psychoacoustic testing. We repeatedly characterized loudness discomfort levels and tinnitus matching over a 2-week period.

Main Outcome Measures:
Primary outcomes included intrasubject variability in loudness discomfort levels, tinnitus intensity, and tinnitus acoustic matching over the course of testing.

Results:
Within-subject variability for all outcome measures could be reduced by approximately 25 to 50% by excluding initial measurements and by focusing only on tinnitus matching attempts where subjects report high confidence in the accuracy of their ratings.

Conclusions:
Tinnitus self-report is inherently variable but can converge on reliable values with extended testing. Repeated, self-directed tinnitus assessments may have implications for identifying malingerers. Further, these findings suggest that extending the baseline phase of tinnitus characterizations will increase the statistical power for future studies focused on tinnitus interventions.
Inner Ear Enhancement With Delayed 3D-FLAIR MRI Imaging in Vestibular Schwannoma
Authors: Bowen Andrew J. , Carlson Matthew L. , Lane John I. 
Published: July 2020

Objectives:
To evaluate blood–labyrinth barrier permeability using delayed gadolinium enhanced three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) in patients with untreated sporadic vestibular schwannoma (VS) to interrogate the etiopathogenesis of progressive sensorineural hearing loss.

Design:
Prospective case series.

Setting:
Single institution tertiary care center.

Participants:
Patients with previously untreated small to medium-sized unilateral VS undergoing 3D-FLAIR imaging 10 minutes (immediate) and 5 to 8 hours (delayed) after administration of intravenous gadolinium.

Main Outcome Measures:
Comparison of cochlear and vestibular signal intensity ratios (SIRs) across immediate and delayed images and across the tumor and nontumor ear.

Results:
Six of eight (75%) patients demonstrated asymmetric enhancement of inner ear structures on delayed contrast-enhanced imaging. Delayed mean cochlea and vestibule SIRs were significantly greater than immediate SIRs (cochlea: 1.91 versus 1.21 [p = 0.02]; vestibule 1.74 versus 1.15 [p = 0.02]). A higher vestibule SIR was statistically significantly associated with poorer pure-tone average and word recognition scores (p = 0.001).

Conclusion:
Ears with VS exhibited increased enhancement on delayed 3D FLAIR imaging. These findings suggest that alterations in blood–labyrinth barrier permeability may explain progressive sensorineural hearing loss in a subset of patients with untreated VS.
Uniform Registration Agreements on Cholesteatoma Care: A Nationwide Consensus Procedure
Authors: ten Tije Fleur A. , Pauw Robert J. , Braspenning Jozé C. , Hemler Raphael J. B. , ter Schiphorst Annette J. , Hensen Erik F. , van der Putten Lisa , Kramer Sophia E. , Merkus Paul 
Published: July 2020

Background:
To coordinate and align the content for registration of cholesteatoma care.

Methods:
Systematic Delphi consensus procedure, consisting three rounds: two written sessions followed by a face-to-face meeting. Before this procedure, input on important patient outcomes was obtained. Consensus was defined as at least 80% agreement by participants. Hundred-thirty-six adult patients who had undergone cholesteatoma surgery and all ENT surgeons of the Dutch ENT Society were invited. The consensus rounds were attended by ENT surgeons with cholesteatoma surgery experience. Feasibility and acceptability of outcome measures and reporting agreements were assessed in round 1 by 150 ENT surgeons. In round 2 definitions were narrowed and context information to interpret outcome measure were questioned. In round 3, the results, amendments, and the open-ended points were discussed to reach agreement.

Results:
Most important outcome measures are: 1) the presence or absence of a cholesteatoma in the first 5 years after surgical removal of cholesteatoma, 2) hearing level after surgical removal of cholesteatoma, and 3) the documented assessment of patient's complaints with a validated patient reported outcome measures questionnaire (PROM). Furthermore, consensus was reached on the registration of cholesteatoma type (residual/recurrent), localization of cholesteatoma, and reporting of the presence of cholesteatoma in the follow-up.

Conclusion:
Consensus was reached on the content and method of registration of cholesteatoma care based on patient's and ENT surgeons input. Three outcome measures were defined. National agreements on the method and content of registration will facilitate monitoring and feedback to the ENT surgeon about the cholesteatoma care.
Cochlear Implant Infections and Outcomes: Experience From a Single Large Center
Authors: Vijendren Ananth , Ajith Amritha , Borsetto Daniele , Tysome James R. , Axon Patrick R. , Donnelly Neil P. , Bance Manohar L. 
Published: July 2020

Objective:
To review our experience with cochlear implant infections over the past 5 years, the management strategy and to identify predictive factors that led to explantation.

Study Design:
Retrospective record-base case series of cochlear implant infections.

Setting:
Tertiary otology and implant center.

Patients:
All patients who had cochlear implantation over a period of 5 years.

Intervention(s):
None.

Main Outcome Measure(s):
To identify risk factors, rates and outcomes of cochlear implant infections, and to formulate strategies to develop clearer management protocols to prevent cochlear implant explantation.

Results:
Of 704 implanted patients, 22 suffered a postoperative soft tissue infection (3%). Fifty-nine percent of these infected patients resulted in explantation, giving an explantation rate of 1.8% over the whole study population. One hundred percent of the infected implants identified as having either Staph. Aureus or Pseudomonas spp. as the single causative organism resulted in explantation.

Conclusions:
There is a high rate of explantation when infection is detected. Currently there is no clear consensus on medical management, such as choice of antibiotics or length of antibiotic course. A registry of cochlear implants would facilitate standard reporting methods for severity and type of infection, to be able to pool data across centers and form a more robust management protocol for cochlear implant infections.
Maternal Microchimerism in Cord Blood and Risk of Celiac Disease in Childhood
Authors: Tapia German , Mortimer Georgina , Ye Jody , Mårild Karl , Chipper‐Keating Saranna , Gillard Benjamin T. , Viken Marte K. , Lie Benedicte A. , Stene Lars C. , Gillespie Kathleen M. , Størdal Ketil 
Published: July 2020

ABSTRACTObjectives:During pregnancy, small quantities of maternal cells are naturally transmitted to the fetus. This transmission, termed maternal microchimerism (MMc), has been implicated in autoimmune diseases but its potential role is unclear. We aimed to investigate if MMc at birth predicted childhood celiac disease (CD) risk, a common immune‐mediated enteropathy often presenting in childhood.Methods:We designed a case‐control study, nested in the Norwegian Mother, Father and Child Cohort. Participants were HLA class II typed to determine noninherited, nonshared maternal alleles (NIMA). Droplet digital (dd) PCR assays specific for common HLA class II NIMAs (HLA‐DQB1*03:01, *04:02 and *06:02/03) were used to estimate the quantity of maternal DNA, as a marker of maternal cells, in cord blood DNA from 124 children who later developed clinically diagnosed CD (median age at end of study 7.4 years, range 3.6–12.9) and 124 random controls. We tested whether presence of MMc was associated with CD using logistic regression, and compared ranks between cases and controls.Results:MMc, for example, maternal HLA antigens not inherited by the child, was found in 42% of cases and 43% of controls, and not associated with CD (odds ratio [OR] 0.97, 95% confidence interval [CI] 0.58–1.60). The ranks of MMc quantities in cases and controls were also similar (Mann‐Whitney U‐test, P = 0.71). The subgroup with HLA‐DQB1:03*01 as their NIMA had a potential association with MMc, where levels greater than median was associated with CD (OR 3.78, 95% CI 1.28–11.18).Conclusion:MMc measured in cord blood was not associated with later risk of CD.
Using Eosinophil Biomarkers From Rectal Epithelial Samples to Diagnose Food Protein‐induced Proctocolitis
Authors: de Boer Justin , Deb Chirajyoti , Bornstein Jeffrey , Horvath Karoly , Mehta Devendra , Smadi Yamen 
Published: July 2020

ABSTRACTObjectives:The gold standard diagnostic procedure for food protein‐induced proctocolitis (FPIP) requires flexible sigmoidoscopy (FS). To date there is no validated, noninvasive test to confirm FPIP diagnosis. Eosinophil‐derived neurotoxin (EDN), a product of eosinophil (EOS) degranulation, has been shown to correlate with eosinophil infiltration in other tissues. Our objective was to compare EDN concentrations in rectal epithelial samples from infants with FPIP with those from a control population.Methods:Children who underwent routine FS at Arnold Palmer Hospital for Children were enrolled in an IRB‐approved, prospective, open‐label pilot study between July 2017 and May 2019. We obtained rectal epithelial samples via: rectal swab, cytology brushing through FS, and rectal biopsy through FS. We then measured EDN levels in the samples and compared levels found in infants with FPIP against levels found in the control group. FPIP was defined as more than 60 EOS per 10 high‐power fields (HPF) in rectal epithelial tissue obtained via rectosigmoid biopsy.Results:Twenty‐four patients were enrolled. The control group (n = 13) included patients with normal histopathology (84% boys, mean age 19 months, SD 6 months) and the FPIP group (n = 11) included patients with FPIP confirmed via biopsy (45% boys, mean age 6.9 months, SD 9 months). EDN concentration was significantly higher in the FPIP group than in the control group, for 2 sampling methods: rectal biopsy (183.6 ± 114.6 vs 76.6 ± 71.0 μg/mL; P = 0.010) and rectal swab (66.2 ± 64.8 vs 20.4 ± 22.2 μg/mL; P = 0.025).Conclusions:EDN concentrations measured from rectal swab and rectal biopsy samples is elevated and may be a useful tool to screen for FPIP in children.
Hepatitis C in 2020
Authors: Leung Daniel H. , Squires James E. , Jhaveri Ravi , Kerkar Nanda , Lin Chuan‐Hao , Mohan Parvathi , Murray Karen F. , Gonzalez‐Peralta Regino P. , Roberts Eve A. , Sundaram Shikha S. 
Published: July 2020

ABSTRACTIn 1989, a collaboration between the Centers for Disease Control (CDC) and a California biotechnology company identified the hepatitis C virus (HCV, formerly known as non‐A, non‐B hepatitis virus) as the causative agent in the epidemic of silent posttransfusion hepatitis resulting in cirrhosis. We now know that, the HCV genome is a 9.6 kb positive, single‐stranded RNA. A single open reading frame encodes a 3011 amino acid residue polyprotein that undergoes proteolysis to yield 10 individual gene products, consisting of 3 structural proteins (core and envelope glycoproteins E1 and E2) and 7 nonstructural (NS) proteins (p7, NS2, NS3, NS4A, NS4B, NS5A, and NS5B), which participate in posttranslational proteolytic processing and replication of HCV genetic material. Less than 25 years later, a new class of medications, known as direct‐acting antivirals (DAAs) which target these proteins, were introduced to treat HCV infection. These highly effective antiviral agents are now approved for use in children as young as 3 years of age and have demonstrated sustained virologic responses exceeding 90% in most genotypes. Although tremendous scientific progress has been made, the incidence of acute HCV infections has increased by 4‐fold since 2005, compounded in the last decade by a surge in opioid and intravenous drug use. Unfortunately, awareness of this deadly hepatotropic virus among members of the lay public remains limited. Patient education, advocacy, and counseling must, therefore, complement the availability of curative treatments against HCV infection if this virus is to be eradicated.
Dyssynergic Defecation and Anal Sphincter Disorders in Children in High‐Resolution Anorectal Manometry Investigation
Authors: Makosiej Ryszard , Makosiej Aleksandra , Bossowski Artur , Kolejwa Michał , Wawrusiewicz‐Kurylonek Natalia , Łęgowik Anna , Gościk Joanna , Piaseczna‐Piotrowska Anna , Czkwianianc Elżbieta 
Published: July 2020

ABSTRACTObjectives:Dyssynergic defecation is a common disorder in children with functional constipation (FC) because of relaxation disorders of the sphincter apparatus and intra‐rectal pressure during defecation. The aim of the study was to determine frequency and type of dyssynergic defecation and to assess pressure in the anal canal poles during simulated evacuation and function of puborectalis muscle in defecation in children with FC.Methods:Three‐dimensional (3D) high‐resolution anorectal manometries (3D HRAM) were performed in 131 children with FC. In the manometric test, resting pressure measurements were assessed in 4 measuring poles of the anal canal.Results:One hundred thirty‐one children ages 5 to 17 years (mean age 10.2; SD ± 3.8; median 10) were involved in the study (69 girls and 62 boys). Dyssynergic defecation was shown in 106/131 (80.9%) examined children. A statistically significant difference between the age of examined children (P < 0.02) and intrarectal pressures at the anal canal measuring points (left P < 0.009, right P < 0.005, anterior P < 0.01) was found. Correlation between the residual pressure values in lateral anal canal measurement poles and intrarectal pressure was demonstrated in all types of dyssynergy (left: r = 0.69, P < 0.0005; right: r = 0.74, P < 0.0005). In a group of 53/131 (40.5%) children, 3D HRAM showed a rectal pressure increase during simulated defecation, because of the dysfunction of the puborectalis muscle.Conclusion:The increase in sphincter pressure in lateral and posterior poles in I and II types of dyssynergia and in lateral poles in other types of dyssynergia may depend on relaxation disorders of the puborectalis muscle during defecation.
Outcomes and Predictors for Re‐stenosis of Esophageal Stricture in Epidermolysis Bullosa
Authors: Pope Elena , Mansour Mark , Berseneva Maria , Liy‐Wong Carmen , Salas Julio , Fuentes Ignacia , Yubero Maria Joao , Palisson Francis , Martinez Anna , Mellerio Jemima , Lara‐Corrales Irene , Yang Anes , Murrell Dedee , Torres‐Pradilla Mauricio , Lucky Anne 
Published: July 2020

ABSTRACTBackground:Esophageal strictures are the common gastrointestinal complications in patients with epidermolysis bullosa (EB) requiring dilation. There is limited information on the best type of intervention, outcomes, and predictors for re‐stenosis.Objectives:We aimed to investigate the frequency, clinical presentation of esophageal strictures in EB patients, and to ascertain the predictors of re‐stenosis.Methods:We conducted a retrospective, multicenter cohort study involving 7 specialized, international EB centers on patients who were 0 to 50 years of age. Descriptive statistics and hazard risks for re‐stenosis were calculated.Results:We identified 125 patients with 497 esophageal stricture episodes over a mean period of observation of 17 (standard deviation [SD] = 11.91) years. Dilations were attempted in 90.74% of episodes, using guided fluoroscopy 45.23%, retrograde endoscopy 33.04%, and antegrade endoscopy 19.07%. Successful dilation was accomplished in 99.33% of attempts. Patients experienced a median of 2 (interquartile range [IQR]: 1–7) stricture episodes with a median interval between dilations of 7 (IQR: 4–12) months. Predictors for re‐stenosis included: number of strictures (2 vs 1 stricture: χ2 = 4.293, P = 0.038, hazard ratio [HR] = 1.294 (95% confidence interval [CI]: 1.014‐‐1.652 and 3 vs 1 stricture:χ2 = 7.986, P = 0.005, HR = 1.785 [95% CI: 1.194, 2.667]) and a long (≥1 cm) segment stricture (χ2 = 4.599, P = 0.032, HR = 1.347 (95% CI: 1.026‐‐1.769). Complications were more common with the endoscopic approach (8/86, antegrade endoscopy; 2 /149, retrograde endoscopy vs 2/204, fluoroscopy; χ2 = 17.39, P‐value <0.000).Conclusions:We found excellent dilation outcomes irrespective of the dilation procedure; however, with higher complications in the endoscopic approach. Long (>1 cm) segment involvement and multiple locations were predictive of stricture reoccurrence.
Two‐hour Observation After Liver Biopsy in Children
Authors: Alomari Mohammed H. , Kerr Cindy L. , Chewning Rush , Chaudry Gulraiz , Shaikh Raja , Padua Horacio , Landrigan‐Ossar Mary , Alomari Ahmad I. 
Published: July 2020

ABSTRACTObjective:Following percutaneous liver biopsy performed at our institution on an outpatient basis, children traditionally were observed for 4 hours then discharged after verifying a stable hematocrit level. In June 2015, we adopted a quality improvement project with shorter 2‐hour observation for patients with no known risks and the hematocrit test was abandoned.The purpose of this study is to evaluate the clinical and economic outcomes of early discharge of children following liver biopsy.Methods:We analyzed data on 2 groups of children who underwent ultrasound‐guided nontargeted core needle liver biopsy performed on outpatient basis. Group A (100 procedures with 4‐hour postprocedural observation time and hematocrit test) was compared with group B (100 procedures with 2‐hour observation without hematocrit test).Results:Group A consisted of 92 patients (43 boys; 49 girls) with a mean age of 11.1 years and mean weight of 52.6 kg. Group B had 92 patients (47 boys; 45 girls) with a mean age of 8.9 years and mean weight of 40.5 kg. The mean length of observation was 281 minutes (range 204–540 minutes) and 147 minutes (range 86–332 minutes) for groups A and B, respectively. The mean recovery charges were reduced by 35% per procedure in group B. The tissue obtained was sufficient for pathologic diagnosis in all procedures. There were no biopsy‐related complications in either group.Conclusions:Enhanced recovery with early discharge of low‐risk children after 2‐hour observation following percutaneous liver biopsy can be safely implemented without adversely affecting the outcome. Shorter postbiopsy observation can be cost‐saving and may potentially improve patient satisfaction.