bluuebunny/crossref_metadata_2025_split huggingface dataset:
Benign vocal fold lesions: update on nomenclature, cause, diagnosis, and treatment
Authors: Naunheim Matthew R. , Carroll Thomas L. 
Published: September 2017

Purpose of review
The management of benign vocal fold lesions (BVFLs) continues to evolve. This article will review the recent literature surrounding the nomenclature, cause, diagnosis, and treatment of BVFLs, including polyps, nodules, cysts, and reactive lesions.

Recent findings
The taxonomy of vocal fold lesions has been refined, and it serves as a common descriptive language for diagnosis, treatment algorithms, and reporting of outcomes. Though these lesions are known to be the result of phonotrauma, investigation into inflammatory mediators, apoptosis, and laryngopharyngeal reflux provide further elucidation of their underlying pathophysiology. The future of diagnosis, for which direct fiberoptic visualization and stroboscopy are the current gold standard, may utilize narrow-band imaging and videokymography; the clinical utility of ultrasound and optical coherence tomography is more remote. Angiolytic laser therapy and intralesional steroid injection are acceptable options for treatment of BVFLs. Voice therapy and microsurgical excision are mainstays of treatment.

Summary
Recent clinical and basic science research has expanded upon an extensive literature surrounding the nomenclature, cause, diagnosis, and treatment of benign subepithelial vocal fold lesions. There remains a strong need for the advancement of rigorous diagnostic principles, evaluation of therapeutic interventions, and development of best practices guidelines.
Status dystonicus in childhood
Authors: Lumsden Daniel E. , King Mary D. , Allen Nicholas M. 
Published: September 2017

Purpose of review
Dystonia is a common paediatric neurological condition. At its most severe, dystonia may lead to life-threatening complications, a state termed status dystonicus. This review provides an update on the definition, causes, management and outcome of childhood status dystonicus.

Recent findings
High-quality studies in childhood status dystonicus are lacking, though an increasing number of case series have been published. Status dystonicus appears to occur more frequently in children compared with adults, with a clear precipitant identified in around two-thirds of cases. Although febrile illness remains the commonest trigger for status dystonicus, unplanned interruption to deep brain stimulation (DBS) is increasingly reported as a precipitant. In parallel with this, neurosurgical intervention for status dystonicus appears to have become more widely used, though optimum timing and patient selection remains unclear. In most cases, a multistaged approach is required; we propose an ‘ABCD’ approach – Addressing precipitants, Beginning supportive measures, Calibrating sedation and Dystonia specific medications. Outcomes following status dystonicus appear to have slightly improved in recent years, potentially as a consequence of increasing use of DBS, though mortality has remained around 10%.

Summary
Future work is needed to inform evidence-based guidelines for the management of status dystonicus. One of many pressing questions is the precise indication, and timing of interventions such as DBS.
P2X7R Blockade Prevents NLRP3 Inflammasome Activation and Pancreatic Fibrosis in a Mouse Model of Chronic Pancreatitis
Authors: Zhang Gui-Xian , Wang Man-Xue , Nie Wei , Liu Da-Wei , Zhang Yi , Liu Hong-Bin 
Published: September 2017

Objectives
The aim of this study was to investigate the role of P2X7R (purinergic 2X7 receptor) and NLRP3 (NACHT, LRR, and PYD domains–containing protein 3) inflammasome activation in the process of pancreatic fibrosis in a mouse model of chronic pancreatitis (CP).

Methods
Chronic pancreatitis was induced by repeated intraperitoneal injections of 50 μg/kg cerulein for 6 weeks in mice. P2X7R antagonist oxidized ATP (OxATP) or brilliant blue G (BBG) was administered after the last cerulein injection for 2 weeks. Pancreatic chronic inflammation and fibrosis were evaluated by histological score, Sirius red staining, and alpha-smooth muscle actin immunohistochemical staining. We further determined pancreatic P2X7R, NLRP3, and caspase-1 expressions in gene and protein levels and the pancreatic concentrations of caspase-1, interleukin 1β (IL-1β), and IL-18.

Results
The pancreatic P2X7R, NLRP3, and caspase-1 expressions in gene and protein levels and the pancreatic concentrations of caspase-1, IL-1β, and IL-18 were all reduced significantly in both the OxATP and BBG groups (P < 0.05). The pancreatic chronic inflammation and the fibrosis indices were all remarkably attenuated (P < 0.05).

Conclusions
P2X7R antagonist OxATP and BBG significantly decreased pancreatic chronic inflammation and fibrosis in a mouse CP model and suggested that blockade of P2X7R-NLRP3 inflammasome signaling pathway may represent a novel therapeutic strategy for CP and its fibrotic process.
Severe Neonatal Cholestasis in Cerebrotendinous Xanthomatosis
Authors: Gong Jing‐Yu , Setchell Kenneth D.R. , Zhao Jing , Zhang Wujuan , Wolfe Brian , Lu Yi , Lackner Karolin , Knisely A.S. , Wang Neng‐Li , Hao Chen‐Zhi , Zhang Mei‐Hong , Wang Jian‐She 
Published: September 2017

ABSTRACTObjectives:Cerebrotendinous xanthomatosis (CTX) is caused by defects in sterol 27‐hydroxylase (CYP27A1, encoded by CYP27A1), a key enzyme in the bile acid synthesis pathway. CTX usually presents as neurologic disease in adults or older children. The rare reports of CTX manifest as neonatal cholestasis assess the cholestasis as transient, with patient survival. Our experience differs.Methods:Homozygous or compound heterozygous CYP27A1 mutations were detected in 8 neonatal cholestasis patients by whole exome sequencing, panel sequencing, or Sanger sequencing. Their clinical and biochemical data were retrospectively reviewed. Immunostaining for CYP27A1 was conducted in liver of 4 patients. Mass spectrometry was used to analyze patients’ urine samples.Results:All 8 infants had severe cholestasis. Five died from, or were transplanted for, liver failure; 3 cleared their jaundice eventually. Marking for CYP27A1 was weak or absent in 3 of the 4 patient specimens. Mass spectrometry of urine revealed a predominance of sulfated and doubly conjugated (sulfated‐glucuronidated) bile alcohols. No patient harbored a putatively pathogenic mutation in genes other than CYP27A1 that have been implicated in cholestatic liver disease.Conclusions:CTX manifest as neonatal cholestasis has a bile acid profile different from CTX manifest in later life, and thus may be overlooked. Immunostaining, mass spectrometry of urine, and genetic studies can support one another in making the diagnosis. A substantial proportion of CTX patients with severe neonatal cholestasis may die or need liver transplantation. CTX manifest in infancy as severe cholestasis warrants further investigation of biochemical diagnostic criteria and best management.
Body Composition Predicts Growth in Infants and Toddlers With Chronic Liver Disease
Authors: Hurtado-López Erika F. , Vásquez-Garibay Edgar M. , Trujillo Xóchitl , Larrosa-Haro Alfredo 
Published: September 2017

ABSTRACT
This cross-sectional study was conducted on 15 infants and toddlers with chronic liver disease to validate arm anthropometry as an accurate measure of body composition (BC) compared to dual-energy x-ray absorptiometry and to predict growth from BC. The z score means of the anthropometric indicators were <−2 standard deviation, except for body fat index and subscapular skinfold, which were between −2 and +2 standard deviation. Fat mass was predicted by arm adiposity indicators and fat-free mass by arm muscle area. Bone mineral content explained 87% of variation in length. Two multiple regression models predicted length: 1 with fat mass plus fat-free mass; and the second with fat mass and bone mineral content. These observations suggest that arm anthropometry is a useful tool to estimate BC and the nutritional status in infants and toddlers with chronic liver disease. Length and head circumference can be predicted by fat mass, fat-free mass, and bone mineral content.
Cholangiocarcinoma Among Children and Adolescents
Authors: Newsome Jennifer R. , Venkatramani Rajkumar , Heczey Andras , Danysh Heather E. , Fishman Douglas S. , Miloh Tamir 
Published: September 2017

ABSTRACTObjective:Cholangiocarcinoma (CCA) is a biliary malignancy found primarily in adults. The incidence of CCA in children is unknown. The aim of this study was to describe characteristics of CCA in children and adolescents.Methods:Using the Surveillance, Epidemiology, and End Results Program (SEER 18) database, we identified incident cases of CCA diagnosed at <20 years of age during the period of 1973 to 2013. Additionally, we reviewed published case reports describing pediatric patients with CCA. We calculated descriptive statistics for CCA cases identified in SEER and in case reports. Kaplan–Meier analysis was performed to determine median and 3‐year overall survival (OS) rates.Results:We identified 15 children and adolescents diagnosed as having CCA from SEER 18 with an incidence rate of 0.0036 per 100,000. Two‐thirds of cases were male, and the majority were white (n = 10). The median age at diagnosis was 17 years (range: 11–19 years). Nine tumors were intrahepatic, 3 extrahepatic, and 3 unspecified. One‐third had distal metastases at diagnosis. Eight patients underwent surgical resection including liver transplant in two. Six patients were alive at the time of follow‐up. Patients without surgical treatment did not survive. Three‐year OS was 50%. Twenty‐two children with CCA were found in the literature with a median age at diagnosis of 15 years (range: 3–18 years). Half were male, and 90% had an underlying gastrointestinal comorbidity. Three‐year OS was 35.3%.Conclusions:CCA in children and adolescents is rare with poor survival. A high proportion of cases had a history of biliary disease. Surgical resection is necessary for cure.
Evans Syndrome as First Manifestation of Primary Immunodeficiency in Clinical Practice
Authors: Martínez-Valdez Libny , Deyà-Martínez Angela , Giner María T. , Berrueco Rubén , Esteve-Solé Ana , Juan Manel , Plaza-Martín Ana M. , Alsina Laia 
Published: September 2017

Background:
Evans syndrome (ES) is a rare immune disorder in children, manifested by simultaneous or sequential autoimmune cytopenias (ACs) of unknown cause and having a chronic course with periods of exacerbation and remission. Some primary immunodeficiencies (PIDs) may present with autoimmune manifestations without infections, masking suspicion of them. The PIDs that can typically manifest as ES are autoimmune lymphoproliferative syndrome and common variable immunodeficiency (CVID).

Materials and Methods:
Review of clinical charts and laboratory results of pediatric patients followed-up in the outpatient clinic of PID with a diagnosis of ES and humoral immunodeficiency.

Results:
Three pediatric patients, a boy and 2 girls, presented with corticosteroid-dependent ES. In the diagnostic approach, autoimmune lymphoproliferative syndrome was ruled out, and during follow-up, patients showed laboratory signs of humoral immune deficiency and were diagnosed with CVID. After initiating the recommended treatment for CVID with AC, patients improved without new exacerbations.

Conclusions:
These cases highlight the importance of detection of possible PID in the context of ES and the establishment of CVID treatment to control AC.
Ambulatory High-dose Methotrexate Administration in Pediatric Osteosarcoma Patients at a Single Institution in Argentina
Authors: Villegas Rubio Jose A. , Cacciavillano Walter , Rose Adriana , Zubizarreta Pedro , Scopinaro Marcelo 
Published: September 2017

Background:
The purpose of this study was to evaluate the feasibility and safety of ambulatory high-dose methotrexate (HDMTX) administration with oral hydration, alkalinization, and leucovorin rescue. HDMTX (12 g/m2) was given intravenously over 4 hours after urine alkalinization. Families and patients were instructed to continue ambulatory oral hydration and alkalinization to monitor urine pH and to adjust bicarbonate according to our institution’s treatment algorithm. Clinical status and MTX levels were controlled every 24 hours, and oral leucovorin dose was adjusted accordingly.

Results:
From April 2007 to December 2010, 150 of 447 courses of HDMTX (31.4%) were given on an outpatient basis, and 91.2% were successfully completed. The main causes of failure were poor oral tolerance (n=6) and fever (n=4). Most patients (81%) had MTX levels of <10 μmol/L 24 hours post-HDMTX; only in 1 course the levels were >50 μmol/L (50.96 μmol/L). Neutropenia grade III/IV was observed in 18.3% of the courses, grade III/IV leukopenia in 2.7%, and grade III/IV thrombocytopenia and anemia in 4.7%. Around 39% were associated with grade III/IV hepatic toxicity (asymptomatic hypertransaminasemia), grade III-IV gastrointestinal toxicity (vomiting and diarrhea) (5%), grade III-IV mucositis (4%), and none of the patients developed renal toxicity.

Conclusions:
Ambulatory HDMTX administration is feasible and safe in a population with poor resources in a developing country.
The Impact of Fluid Overload on Outcomes in Children Treated With Extracorporeal Membrane Oxygenation: A Multicenter Retrospective Cohort Study*
Authors: Selewski David T. , Askenazi David J. , Bridges Brian C. , Cooper David S. , Fleming Geoffrey M. , Paden Matthew L. , Verway Mark , Sahay Rashmi , King Eileen , Zappitelli Michael 
Published: September 2017

Objective:
To characterize the epidemiology of fluid overload and its association with mortality and duration of extracorporeal membrane oxygenation in children treated with extracorporeal membrane oxygenation.

Design:
Retrospective cohort study.

Setting:
Six tertiary children’s hospital ICUs.

Patients:
Seven hundred fifty-six children younger than 18 years old treated with extracorporeal membrane oxygenation for greater than or equal to 24 hours from January 1, 2007, to December 31, 2011.

Interventions:
None.

Measurements and Main Results:
Overall survival to extracorporeal membrane oxygenation decannulation and hospital discharge was 74.9% (n = 566) and 57.7% (n = 436), respectively. Median fluid overload at extracorporeal membrane oxygenation initiation was 8.8% (interquartile range, 0.3–19.2), and it differed between hospital survivors and non survival, though not between extracorporeal membrane oxygenation survivors and non survivors. Median peak fluid overload on extracorporeal membrane oxygenation was 30.9% (interquartile range, 15.4–54.8). During extracorporeal membrane oxygenation, 84.8% had a peak fluid overload greater than or equal to 10%; 67.2% of patients had a peak fluid overload of greater than or equal to 20% and 29% of patients had a peak fluid overload of greater than or equal to 50%. The median peak fluid overload was lower in patients who survived on extracorporeal membrane oxygenation (27.2% vs 44.4%; p < 0.0001) and survived to hospital discharge (24.8% vs 43.3%; p < 0.0001). After adjusting for acute kidney injury, pH at extracorporeal membrane oxygenation initiation, nonrenal complications, extracorporeal membrane oxygenation mode, support type, center and patient age, the degree of fluid overload at extracorporeal membrane oxygenation initiation (p = 0.05), and the peak fluid overload on extracorporeal membrane oxygenation (p < 0.0001) predicted duration of extracorporeal membrane oxygenation in survivors. Multivariable analysis showed that peak fluid overload on extracorporeal membrane oxygenation (adjusted odds ratio, 1.09; 95% CI, 1.04–1.15) predicted mortality on extracorporeal membrane oxygenation; fluid overload at extracorporeal membrane oxygenation initiation (adjusted odds ratio, 1.13; 95% CI, 1.05–1.22) and peak fluid overload (adjusted odds ratio, 1.18; 95% CI, 1.12–1.24) both predicted hospital morality.

Conclusions:
Fluid overload occurs commonly and is independently associated with adverse outcomes including increased mortality and increased duration of extracorporeal membrane oxygenation in a broad pediatric extracorporeal membrane oxygenation population. These results suggest that fluid overload is a potential target for intervention to improve outcomes in children on extracorporeal membrane oxygenation.
A Novel Registration-Based Semiautomatic Mandible Segmentation Pipeline Using Computed Tomography Images to Study Mandibular Development
Authors: Chuang Ying Ji , Doherty Benjamin M. , Adluru Nagesh , Chung Moo K. , Vorperian Houri K. 
Published: September 2017

Objective
We present a registration-based semiautomatic mandible segmentation (SAMS) pipeline designed to process a large number of computed tomography studies to segment 3-dimensional mandibles.

Method
The pipeline consists of a manual preprocessing step, an automatic segmentation step, and a final manual postprocessing step. The automatic portion uses a nonlinear diffeomorphic method to register each preprocessed input computed tomography test scan on 54 reference templates, ranging in age from birth to 19 years. This creates 54 segmentations, which are then combined into a single composite mandible.

Results
This pipeline was assessed using 20 mandibles from computed tomography studies with ages 1 to 19 years, segmented using both SAMS-processing and manual segmentation. Comparisons between the SAMS-processed and manually-segmented mandibles revealed 97% similarity agreement with comparable volumes. The resulting 3-dimensional mandibles were further enhanced with manual postprocessing in specific regions.

Conclusions
Findings are indicative of a robust pipeline that reduces manual segmentation time by 75% and increases the feasibility of large-scale mandibular growth studies.
Adaptive Statistical Iterative Reconstruction–V Versus Adaptive Statistical Iterative Reconstruction: Impact on Dose Reduction and Image Quality in Body Computed Tomography
Authors: Gatti Marco , Marchisio Filippo , Fronda Marco , Rampado Osvaldo , Faletti Riccardo , Bergamasco Laura , Ropolo Roberto , Fonio Paolo 
Published: September 2017

Objective
The aim of this study was to evaluate the impact on dose reduction and image quality of the new iterative reconstruction technique: adaptive statistical iterative reconstruction (ASIR-V).

Methods
Fifty consecutive oncologic patients acted as case controls undergoing during their follow-up a computed tomography scan both with ASIR and ASIR-V. Each study was analyzed in a double-blinded fashion by 2 radiologists. Both quantitative and qualitative analyses of image quality were conducted.

Results
Computed tomography scanner radiation output was 38% (29%–45%) lower (P < 0.0001) for the ASIR-V examinations than for the ASIR ones. The quantitative image noise was significantly lower (P < 0.0001) for ASIR-V. Adaptive statistical iterative reconstruction–V had a higher performance for the subjective image noise (P = 0.01 for 5 mm and P = 0.009 for 1.25 mm), the other parameters (image sharpness, diagnostic acceptability, and overall image quality) being similar (P > 0.05).

Conclusions
Adaptive statistical iterative reconstruction–V is a new iterative reconstruction technique that has the potential to provide image quality equal to or greater than ASIR, with a dose reduction around 40%.
Noninvasive Computed Tomography–Derived Fractional Flow Reserve Based on Structural and Fluid Analysis: Reproducibility of On-site Determination by Unexperienced Observers
Authors: Ri Keiken , Kumamaru Kanako K. , Fujimoto Shinichiro , Kawaguchi Yuko , Dohi Tomotaka , Yamada Sou , Takamura Kazuhisa , Kogure Yosuke , Yamada Norikazu , Kato Etsuro , Irie Ryusuke , Takamura Tomohiro , Suzuki Michimasa , Hori Masaaki , Aoki Shigeki , Daida Hiroyuki 
Published: September 2017

Objective
The aim of this study was to evaluate the reproducibility of computed tomography (CT)–derived fractional flow reserve (FFR) determined on site by inexperienced observers using a postprocessing software based on structural and fluid analysis.

Methods
Using 21 coronary vessels in 7 patients who underwent 320-row coronary CT angiography and catheter-FFR, 2 independent inexperienced observers (A: a student radiation technologist; B: a nonmedical staff) determined the CT-FFR using a postprocessing software. After a 20-minute training session, both observers postprocessed all vessels and readjusted their settings after another training/feedback. These CT-FFRs were compared with values determined by an expert analyst.

Results
The mean processing times were 23 ± 4 minutes (automatic), 71 ± 5 minutes (observer A), and 57 ± 7 minutes (observer B) per patient. The initial correlations with expert data were r = 0.92 (observer A) and 0.73 (observer B) and increased to 0.83 for observer B after additional training. The final absolute difference with the expert data was 0.000 to 0.020. The correlation between catheter-FFR and expert CT-FFR was r = 0.76.

Conclusions
The CT-derived FFR on-site postprocessing software showed good reproducibility for measurements by inexperienced observers.
Avoidance of Tracheostomy Using Mandibular Distraction in an Infant With Severe Condylar Dysplasia and Airway Obstruction
Authors: Archual Anthony J. , Black Jonathan S. 
Published: September 2017

Abstract
Craniofacial microsomia (CFM) is a common craniofacial anomaly characterized by asymmetric malformation of the mandible, ear, and other structures including the upper airway. The degree of mandibular hypoplasia is classified using a scheme developed by Pruzansky and modified by Kaban. Severe condylar dysplasia is the hallmark of Pruzansky–Kaban types IIB and III. Distraction osteogenesis has emerged as a treatment modality for mandibular hypoplasia in this setting for the treatment of asymmetry and airway obstruction. However, its use to resolve upper airway obstruction and avoid tracheostomy in infants with types IIB and III is not reported in the literature. The authors present a patient with successful distraction osteogenesis in a patient with CFM resulting in a Pruzansky IIB micrognathia. The authors achieved resolution of upper airway obstruction and avoidance of tracheostomy.