The importance of data structure in statistical analysis of dendritic spine morphology

Publication date: 15 February 2018
Source:Journal of Neuroscience Methods, Volume 296
Author(s): Veerle Paternoster, Anto P. Rajkumar, Jens Randel Nyengaard, Anders Dupont Børglum, Jakob Grove, Jane Hvarregaard Christensen
BackgroundDendritic spine morphology is heterogeneous and highly dynamic. To study the changing or aberrant morphology in test setups, often spines from several neurons from a few experimental units e.g. mice or primary neuronal cultures are measured. This strategy results in a multilevel data structure, which, when not properly addressed, has a high risk of producing false positive and false negative findings.MethodsWe used mixed-effects models to deal with data with a multilevel data structure and compared this method to analyses at each level. We apply these statistical tests to a dataset of dendritic spine morphology parameters to illustrate advantages of multilevel mixed-effects model, and disadvantages of other models.ResultsWe present an application of mixed-effects models for analyzing dendritic spine morphology datasets while correcting for the data structure.Comparison with existing methodsWe further show that analyses at spine level and aggregated levels do not adequately account for the data structure, and that they may lead to erroneous results.ConclusionWe highlight the importance of data structure in dendritic spine morphology analyses and highly recommend the use of mixed-effects models or other appropriate statistical methods to deal with multilevel datasets. Mixed-effects models are easy to use and superior to commonly used methods by including the data structure and the addition of other explanatory variables, for example sex, and age, etc., as well as interactions between variables or between variables and level identifiers.



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Chemotherapy is administered to a minority of hospitalized patients with diffuse large B-cell lymphoma and is associated with less likelihood of death during hospitalization

Publication date: April 2018
Source:Cancer Epidemiology, Volume 53
Author(s): Anita J. Kumar, Tobi Henzer, Angie Mae Rodday, Susan K. Parsons
BackgroundWhile treatment of DLBCL is largely outpatient, some patients require planned or unplanned admissions for chemotherapy, new diagnosis, relapse, or toxicity. We examined risk factors for receipt of inpatient chemotherapy and death during hospitalization.MethodsWe analyzed data from the 2012–2013 HCUP-NIS. We identified patient and hospital characteristics that were associated with chemotherapy administration and death.ResultsChemotherapy was given in 3260/11,150 (29.2%) of hospitalizations. Younger age, urban teaching hospitals, fewer chronic conditions, male sex, non-Medicare insurance, and "less likelihood of dying" were associated with chemotherapy. Chemotherapy portended lower odds of death; older age and longer hospitalizations were associated with increased odds of death.ConclusionWe identified socio-demographics and clinical characteristics associated with inpatient chemotherapy in DLBCL patients. Chemotherapy is associated with lower odds of death during hospitalization, suggesting that most chemotherapy is given appropriately to non-critically ill patients. Clinical acuity is a stronger predictor of death than socio-demographics.



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Exploring determinants of care-seeking behaviour of oral cancer patients in India: A qualitative content analysis

Publication date: April 2018
Source:Cancer Epidemiology, Volume 53
Author(s): Hemamalini Rath, Swikant Shah, Gaurav Sharma, Ekagrata Mishra
BackgroundA major public health concern in India is the high morbidity and mortality rates of oral cancer because of late diagnosis. Among the several determinants of this late diagnosis, the most important is the healthcare-seeking behaviour of the oral cancer patients. The aim of this study was to explore the care-seeking behaviour and its determinants among oral cancer patients.MethodsA face-to-face in-depth interview was conducted among 70 oral cancer patients using a semi-structured questionnaire, and qualitative content analysis of the results was performed.ResultsAll the patients had squamous-cell carcinoma and none had attended any screening programme. The most common site affected was the buccal mucosa with a non-healing wound. Most of the patients contacted a doctor available nearby; only 7% of patients consulted a dentist. Only one patient approached a traditional healer. The median patient delay was 30 (4–365) days and the professional delay was 40 (4–650) days. Enablers included determinants such as increasing symptoms (80%), influence of the society (74%), fear (10%), and social media (3%). The main barriers were lack of awareness (97%), hope that the lesion will heal spontaneously (90%), lack of perception of seriousness (64%), financial constraints (55%), provider switching (47%), and missed diagnosis (44%).ConclusionThe care-seeking path among oral cancer patients is complex, customised, and influenced by multiple patient-related and system-related factors.



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Comparison of two exhaled biomarkers in children with and without sleep disordered breathing

Publication date: Available online 10 February 2018
Source:Sleep Medicine
Author(s): Mario Barreto, Paolo Montuschi, Melania Evangelisti, Susanna Bonafoni, Manuela Cecili, Rugia Shohreh, Giuseppe Santini, Maria Pia Villa
ObjectiveAirway oxidative stress and inflammation are likely to be involved in sleep disordered breathing (SDB) in children. We aimed to measure concentrations of 8-isoprostane (8-IsoP) in the exhaled breath condensate (EBC) and exhaled nitric oxide (FENO) in patients with SBD and healthy children, in order to assess the relationship between these two biomarkers, disease severity, and overnight changes.MethodsPatients with SDB (n=46) and healthy controls (n=20) aged 4.5-15.1 years (M/F: 36/30) underwent exhaled measurements. Patients with SDB underwent standard polysomnography to define primary snoring (PS: AHI<1) and obstructive sleep apnea (OSA). Upon awakening the following morning, FENO was measured and EBC was collected for the measurement of EBC 8-IsoP.ResultsOSA patients yielded higher awakening levels of 8-IsoP in EBC than PS patients and control subjects. The 8-IsoP levels, though not FENO, correlated with AHI (r= 0.40, p= 0.003) and SaO2 (r= -0.50, p= 0.001). Cut-off levels of 8-IsoP predicted OSA with a high AUC value (0.84, p=0.000). Sensitivity and specificity for 8-IsoP levels above the percentile 50 (33.3 pg/mL) were 76.5% and 78.1%, respectively. 8-IsoP levels did not change from the evening to morning session, whereas morning FENO levels rose significantly only in patients with mild OSA (p=0.03).ConclusionLevels of 8-IsoP, though not FENO, distinguish children with OSA from those with PS or healthy, correlate with disease severity and closely predict OSA in the whole sample.



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100 Case Reviews in Neurosurgery, Michele R. Aizenberg, Mike Y. Chen, Rahul Jandial. Elsevier (2016), 544 pages

Publication date: April 2018
Source:World Neurosurgery, Volume 112
Author(s): Won Kim




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Virtual Reality Glasses and “Eye-Hands Blind Technique” for Microsurgical Training in Neurosurgery

Publication date: April 2018
Source:World Neurosurgery, Volume 112
Author(s): Joham Choque-Velasquez, Roberto Colasanti, Juhani Collan, Riina Kinnunen, Behnam Rezai Jahromi, Juha Hernesniemi
ObjectiveMicrosurgical skills and eye-hand coordination need continuous training to be developed and refined. However, well-equipped microsurgical laboratories are not so widespread as their setup is expensive. Herein, we present a novel microsurgical training system that requires a high-resolution personal computer screen, smartphones, and virtual reality glasses.MethodsA smartphone placed on a holder at a height of about 15–20 cm from the surgical target field is used as the webcam of the computer. A specific software is used to duplicate the video camera image. The video may be transferred from the computer to another smartphone, which may be connected to virtual reality glasses.ResultsUsing the previously described training model, we progressively performed more and more complex microsurgical exercises. It did not take long to set up our system, thus saving time for the training sessions.ConclusionOur proposed training model may represent an affordable and efficient system to improve eye-hand coordination and dexterity in using not only the operating microscope but also endoscopes and exoscopes.



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Occipital Artery to Middle Cerebral Artery Bypass in Cases of Unavailable Superficial Temporal Artery

Publication date: April 2018
Source:World Neurosurgery, Volume 112
Author(s): Tsukasa Hirano, Takeshi Mikami, Hime Suzuki, Toru Hirano, Yusuke Kimura, Katusya Komatsu, Yukinori Akiyama, Masahiko Wanibuchi, Nobuhiro Mikuni
In neurosurgery, extracranial-to-intracranial (EC-IC) bypass surgery is necessary for patients who have undergone surgery in which the superficial temporal artery (STA) was already used for a different bypass procedure or was damaged. Here we report our experience with EC-IC bypass using the occipital artery (OA) in patients in whom the STA was unavailable, and discuss the technical considerations and pitfalls. Five patients with ischemic-onset moyamoya disease and atherosclerotic disease were included. Two patterns of skin incisions were planned according to the OA pathway and recipient artery location. In one of these methods, a skin incision is made above the OA, and a craniotomy is performed under this incision after OA dissection. In the other method, a skin incision is made above the OA to enable its dissection, and a craniotomy is performed via a separate skin incision. No major perioperative complications developed in any of the 5 cases, and bypass patency was confirmed in all patients. There was a significant difference between the preoperative and postoperative asymmetry ratios of the mean transit time values. Our findings suggest that OA-to-middle cerebral artery (MCA) bypass is a simple and effective technique in patients in whom the STA was already used or was damaged by previous intracranial revascularization or craniotomy. This procedure could be an alternative to STA-MCA bypass in patients without an available STA.



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Making Anatomic Preparations of the Human Brain Using Colored Silicone for Vascular Perfusion Staining (Technical Description)

Publication date: April 2018
Source:World Neurosurgery, Volume 112
Author(s): Mikhail A. Shkarubo, Alexey N. Shkarubo, Georgiy F. Dobrovolsky, Georgiy A. Polev, Ilia V. Chernov, Dmitry N. Andreev, Vasiliy V. Karnaukhov, Konstantin V. Koval
BackgroundA thorough understanding of the central nervous system anatomy is a fundamental part of a neurosurgeon's training. Development of novel neurosurgical approaches and optimization of existing ones directly depend on the comprehensive study of topographic anatomy of the head and neck using cadaveric specimens. Staining the vessels of the arterial and venous systems of a cadaveric specimen aids a detailed study of the vascularization of anatomic structures of the brain. Here we describe an alternative expedited method for creating anatomic preparations of the human brain using colored silicone for vascular perfusion staining.MethodsA nonfixed, whole cadaver (with the head intact), without central nervous system pathology, no later than 48 hours after death, is used to prepare the human brain specimen through staining of arterial and venous vessels.ResultsOur proposed method provides a fast and inexpensive approach to making anatomic preparations of the human brain with staining of the vascular system. The preparations thus obtained can be used for educational purposes, aiding the study of normal and topographic anatomy of the brain, as well as the development of new microsurgical and/or endoscopic approaches.ConclusionsIn experienced hands, our proposed method allows the creation of anatomic preparations relatively fast, at low cost, and with excellent quality.



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Prohibited Arc Angles During Leksell Frame-Based Stereotaxy

Publication date: April 2018
Source:World Neurosurgery, Volume 112
Author(s): Holger Joswig, Andrew G. Parrent, Keith W. MacDougall, David A. Steven
BackgroundDuring Leksell frame-based stereotaxy the lateral trajectory can be obstructed by the arc supports at certain combinations of the X-coordinate and arc angle.ObjectiveTo provide a matrix with usable and prohibited combinations of the X-coordinate and arc angle to faciliate finding lateral stereotactic trajectories with the Leksell frame in standard configuration.MethodsVarious combination settings of the X-coordinate and arc angle were tested for a Leksell Multi-Purpose Stereotactic Arc mounted on a Leksell Coordinate Frame G.ResultsA matrix of usable and prohibited combinations of the X-coordinate and arc angle is provided.ConclusionsThe matrix will help to facilitate surgical work flow during stereotactic procedures involving the Leksell frame.



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Anti-Yo-Associated Paraneoplastic Cerebellar Degeneration Manifesting as Acute Cerebellitis with Posterior Cranial Fossa Hypertension

Publication date: April 2018
Source:World Neurosurgery, Volume 112
Author(s): Alejandro Enríquez-Marulanda, Luis David Beltrán-Osorio, Luis Alberto Escobar, Ana María Granados, Fernando Velásquez-Lasprilla, Jorge Luis Orozco
BackgroundParaneoplastic cerebellar degeneration (PCD) is a rare complication of some malignant cancers. It is most commonly described in women with gynecologic or breast malignancies; however, there have been reports in other types of cancers. Symptoms include ataxia, dysarthria, and tremors, which could be the first manifestations of an underlying malignancy.Case DescriptionA 50-year-old woman had an acute PCD with anti-Yo antibodies from an underlying breast invasive ductal carcinoma. She presented with intracranial hypertension in the posterior cranial fossa that required an emergent decompressive craniectomy.ConclusionsPCD is an uncommon disease that may manifest initially as posterior cranial fossa hypertension and subsequent acute hydrocephalus owing to diffuse cerebellar swelling. To our knowledge, this is the first described case of an anti-Yo PCD that has manifested as acute posterior cranial fossa hypertension owing to diffuse cerebellar edema. Early diagnosis and treatment should be pursued to improve long-term outcomes.



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Clinical benefit of improved Prehospital stroke scales to detect stroke patients with large vessel occlusions: results from a conditional probabilistic model

Clinical scales to detect large vessel occlusion (LVO) may help to determine the optimal transport destination for patients with suspected acute ischemic stroke (AIS). The clinical benefit associated with impr...

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A dual tracer ratio method for comparative emission measurements in an experimental dairy housing

Publication date: April 2018
Source:Atmospheric Environment, Volume 179
Author(s): Joachim Mohn, Kerstin Zeyer, Margret Keck, Markus Keller, Michael Zähner, Jernej Poteko, Lukas Emmenegger, Sabine Schrade
Agriculture, and in particular dairy farming, is an important source of ammonia (NH3) and non-carbon dioxide greenhouse gas (GHG) emissions. This calls for the development and quantification of effective mitigation strategies. Our study presents the implementation of a dual tracer ratio method in a novel experimental dairy housing with two identical, but spatially separated housing areas. Modular design and flexible floor elements allow the assessment of structural, process engineering and organisational abatement measures at practical scale. Thereby, the emission reduction potential of specific abatement measures can be quantified in relation to a reference system. Emissions in the naturally ventilated housing are determined by continuous dosing of two artificial tracers (sulphur hexafluoride SF6, trifluoromethylsulphur pentafluoride SF5CF3) and their real-time detection in the ppt range with an optimized GC-ECD method. The two tracers are dosed into different experimental sections, which enables the independent assessment of both housing areas. Mass flow emissions of NH3 and GHGs are quantified by areal dosing of tracer gases and multipoint sampling as well as real-time analysis of both tracer and target gases. Validation experiments demonstrate that the technique is suitable for both areal and point emission sources and achieves an uncertainty of less than 10% for the mass emissions of NH3, methane (CH4) and carbon dioxide (CO2), which is superior to other currently available methods. Comparative emission measurements in this experimental dairy housing will provide reliable, currently unavailable information on emissions for Swiss dairy farming and demonstrate the reduction potential of mitigation measures for NH3, GHGs and potentially other pollutants.

Graphical abstract

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Summertime diurnal variations in the isotopic composition of atmospheric nitrogen dioxide at a small midwestern United States city

Publication date: April 2018
Source:Atmospheric Environment, Volume 179
Author(s): Wendell W. Walters, Huan Fang, Greg Michalski
The nitrogen and oxygen stable isotopes (δ¹⁵N & δ¹⁸O) of nitrogen oxides (NOx = nitric oxide (NO) + nitrogen dioxide (NO2)) may be a useful tool for partitioning NOx emission sources and for evaluating NOx photochemical cycling, but few measurements of in situ NOx exist. In this study, we have collected and characterized the diurnal variability in δ¹⁵N and δ¹⁸O of NO2 from ambient air at a small Midwestern city (West Lafayette, IN, USA, 40.426° N, 86.908° W) between July 7 to August 5, 2016, using an active sampling technique. Large variations were observed in both δ¹⁵N(NO2) and δ¹⁸O(NO2) that ranged from −31.4 to 0.4‰ and 41.5–112.5‰, respectively. Daytime averages were −9.2 ± 5.7‰ (x̅ ± 1σ) and 86.5 ± 14.1‰ (n = 11), while nighttime averages were −13.4 ± 7.3‰ and 56.3 ± 7.1‰ (n = 12) for δ¹⁵N(NO2) and δ¹⁸O(NO2), respectively. The large variability observed in δ¹⁵N(NO2) is predicted to be driven by changing contributions of local NOx emission sources, as calculated isotope effects predict a minor impact on δ¹⁵N(NO2) relative to δ¹⁵N(NOx) that is generally less than 2.5‰ under the sample collection conditions of high ozone concentration ([O3]) relative to [NOx]. A statistical δ¹⁵N mass-balance model suggests that traffic-derived NOx is the main contributor to the sampling site (0.52 ± 0.22) with higher relative contribution during the daytime (0.58 ± 0.19) likely due to higher traffic volume than during the nighttime (0.47 ± 0.22). The diurnal cycle observed in δ¹⁸O(NO2) is hypothesized to be a result of the photochemical cycling of NOx that elevates δ¹⁸O(NO2) during the daytime relative to the nighttime. Overall, this data suggests the potential to use δ¹⁵N(NO2) for NOx source partitioning under environmental conditions of high [O3] relative to [NOx] and δ¹⁸O(NO2) for evaluating VOC-NOx-O3 chemistry.



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Sleep disorders associated with risk of rheumatoid arthritis

Abstract

Background

Immune disorders impair sleep quality and sleep disorders (SDs) may derange immune function.

Purpose

The study evaluated the incidence and risk of rheumatoid arthritis (RA) in patients with SDs using a nationwide cohort.

Methods

We recognized the patients with SDs from 1998 to 2002 by using the Taiwan National Health Insurance Research Database. One control patient for each SD patient was randomly selected and matched based on the proportion of age, sex, and index year. We calculated the person years of follow-up for each participant from the index date to RA diagnosis, censoring, or until December 31, 2011. The risk of RA was estimated by using Cox models incorporating demographics and comorbidities.

Results

We enrolled 65,754 patients with SDs and 65,753 controls and followed for 637,906 and 662,514 person-years, respectively. The patients with SDs exhibited a 1.49-fold greater risk of RA development compared with the comparison cohort when we adjusted for covariates. The patients with sleep apnea (SA) showed the greatest incidence density rate of RA, followed by those with non-apnea SDs and the non-SD cohort (4.11, 3.29, and 2.15 per 10,000 person-years, respectively). The SA cohort had a 2.56-fold adjusted hazard ratio (aHR) of RA (95% confidence interval [CI] = 1.11–5.91) and the non-apnea SD cohort had a 1.47-fold aHR of RA (95% CI = 1.18–1.84) compared with the non-SD cohort. Women with SDs presented a considerable risk of developing RA.

Conclusions

This nationwide cohort study indicates that SDs are associated with the risk of RA development.

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Three-dimensional imaging assessment of anatomic invasion and volumetric considerations for chemo/radiotherapy-based laryngeal preservation in T3 larynx cancer

Publication date: April 2018
Source:Oral Oncology, Volume 79
Author(s): Mona Kamal, Sweet Ping Ng, Salman A. Eraj, Crosby D. Rock, Brian Pham, Jay A. Messer, Adam S. Garden, William H. Morrison, Jack Phan, Steven J. Frank, Adel K. El-Naggar, Jason M. Johnson, Lawrence E. Ginsberg, Renata Ferrarotto, Jan S. Lewin, Katherine A. Hutcheson, Carlos E. Cardenas, Mark E. Zafereo, Stephen Y. Lai, Amy C. Hessel, Randal S. Weber, G. Brandon Gunn, Clifton D. Fuller, Abdallah S.R. Mohamed, David I. Rosenthal
ObjectivesTo investigate the impact of 3-Diminsional (3D) tumor volume (TV) and extent of involvement of primary tumor on treatment outcomes in a large uniform cohort of T3 laryngeal carcinoma patients treated with nonsurgical laryngeal preservation strategies.Materials and MethodsThe pretreatment contrast-enhanced computed tomography images of 90 patients with T3 laryngeal carcinoma were reviewed. Primary gross tumor volume (GTVp) was delineated to calculate the 3D TV and define the extent of invasion. Cartilage and soft tissue involvement was coded. The extent of invasion was dichotomized into non/limited invasion versus multiple invasion extension (MIE), and was subsequently correlated with survival outcomes.ResultsThe median TV was 6.6 cm³. Sixty-five patients had non/limited invasion, and 25 had MIE. Median follow-up for surviving patients was 52 months. The 5-year local control and overall survival rates for the whole cohort were 88% and 68%, respectively. There was no correlation between TV and survival outcomes. However, patients with non/limited invasion had better 5-year local control (LC) than those with MIE (95% vs 72%, p = .009) but did not have a significantly higher rate of overall survival (OS) (74% vs 67%, p = .327). In multivariate correlates of LC, MIE maintained statistical significance whereas baseline airway status showed a statistically significance trend with poor LC (p = .0087 and 0.06, respectively). Baseline good performance status was an independent predictor of improved OS (p = .03) in multivariate analysis.ConclusionThe extent of primary tumor invasion is an independent prognostic factor of LC of the disease after definitive radiotherapy in T3 larynx cancer.



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Comments on “Compositional and functional variations of oral microbiota associated with the mutational changes in oral cancer” by Yang et al.

Publication date: Available online 10 February 2018
Source:Oral Oncology
Author(s): Divya Gopinath, Rohit Kunnath Menon




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Sleep disorders associated with risk of rheumatoid arthritis

Abstract

Background

Immune disorders impair sleep quality and sleep disorders (SDs) may derange immune function.

Purpose

The study evaluated the incidence and risk of rheumatoid arthritis (RA) in patients with SDs using a nationwide cohort.

Methods

We recognized the patients with SDs from 1998 to 2002 by using the Taiwan National Health Insurance Research Database. One control patient for each SD patient was randomly selected and matched based on the proportion of age, sex, and index year. We calculated the person years of follow-up for each participant from the index date to RA diagnosis, censoring, or until December 31, 2011. The risk of RA was estimated by using Cox models incorporating demographics and comorbidities.

Results

We enrolled 65,754 patients with SDs and 65,753 controls and followed for 637,906 and 662,514 person-years, respectively. The patients with SDs exhibited a 1.49-fold greater risk of RA development compared with the comparison cohort when we adjusted for covariates. The patients with sleep apnea (SA) showed the greatest incidence density rate of RA, followed by those with non-apnea SDs and the non-SD cohort (4.11, 3.29, and 2.15 per 10,000 person-years, respectively). The SA cohort had a 2.56-fold adjusted hazard ratio (aHR) of RA (95% confidence interval [CI] = 1.11–5.91) and the non-apnea SD cohort had a 1.47-fold aHR of RA (95% CI = 1.18–1.84) compared with the non-SD cohort. Women with SDs presented a considerable risk of developing RA.

Conclusions

This nationwide cohort study indicates that SDs are associated with the risk of RA development.

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Temporal Bone Meningo-Encephalic-Herniation: Etiological Categorization and Surgical Strategy.

Temporal Bone Meningo-Encephalic-Herniation: Etiological Categorization and Surgical Strategy.

Otol Neurotol. 2018 Mar;39(3):320-332

Authors: Grinblat G, Dandinarasaiah M, Prasad SC, Piras G, Piccirillo E, Fulcheri A, Sanna M

Abstract
OBJECTIVE: To study the clinical presentation, intraoperative findings and surgical management in meningo-encephalic-herniation (MEH) based on the etiology.
STUDY DESIGN: A retrospective clinical study and is a follow-up on the previously published report in 2009.
SETTING: A quaternary referral otology and skull base center PATIENTS AND METHODS:: The inclusion criteria were intraoperatively verified MEH in patients with a minimum follow-up of 12 months, which yielded 262 operated ears. The data were extracted regarding demographics, laterality, clinical presentation, past surgeries, contralateral-ear condition, intraoperative findings, complications, recurrences, revision-surgeries, audiometric-data, and follow-up.
RESULTS: The mean age at surgery was 49.7 years with the involvement of right-ear in 53.8% of patients. Lesions were categorized based on the etiology as chronic-otitis-media with/without cholesteatoma-MEH (COM/CHOL-MEH)-47.7%, iatrogenic-MEHs -20.9%; traumatic-MEHs -8% and spontaneous-MEHs -23.3%. At presentation, hearing loss (100 and 98.2%) and otorrhea (65.6 and 49.1%) were predominant in COM/CHOL-MEHs and iatrogenic-MEHs, respectively. On the other hand, meningitis (23.9 and 14.3%) and cerebrospinal fluid-leak (52.4 and 42.8%) were more pronounced in spontaneous and traumatic MEHs, respectively. Surgical approaches included 1) transmastoid, 2) middle-cranial-fossa-approach, 3) combined, and 4) middle-ear-obliteration (MEO) techniques. A total of 52.8% of COM/CHOL-MEHs and 49.1% of iatrogenic-MEHs underwent MEO. Middle-cranial-fossa approach was predominantly used in spontaneous-MEHs (52.5%) and traumatic-MEHs (38.1%). The defect was mostly single (75.2%). Smaller, multiple, bilateral lesions were more common in spontaneous-MEHs with tegmen-tympani involvement (57.4%).
CONCLUSION: Incorporating etiology into MEHs is a key-step that can be used as a guidance in choosing the right surgery. MEO is a part of armamentarium, and should be used whenever needed, if the objective is performing a definitive surgery.

PMID: 29424819 [PubMed - in process]

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Alloplastic temporomandibular joint replacement systems: a systematic review of their history

Publication date: Available online 9 February 2018
Source:International Journal of Oral and Maxillofacial Surgery
Author(s): N. De Meurechy, M.Y. Mommaerts
This systematic review provides an overview of the historical evolution of the prosthetic temporomandibular joint and addresses the challenges and complications faced by engineers and surgeons, in an effort to shed light on why only a few systems remain available. A better understanding of the history of temporomandibular joint prostheses might also provide insights into the origin of the negative public opinion of the prosthesis, which is based on outdated information. A computerized search using the PubMed Central, ScienceDirect, Wiley Online, Ovid, and Cochrane Library databases was performed following the PRISMA guidelines. Out of 7122 articles identified, 41 met the inclusion criteria for this systematic review. Although several historical reviews have been published previously, none has covered such an extensive time period or has described all designs. Furthermore, besides providing a historical overview, this review discusses the rationale behind the evolution in design and biomaterials, which have largely contributed to the outcomes of the prosthetic systems.



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Reply to “10-10 Electrode System for EEG Recording”

We thank Prof. Nuwer for providing additional information on the 10-10-system (Nuwer, 2018) which helps to put the development of the current EEG-nomenclature and related discussions in the correct historical context to which Prof Nuwer contributed significantly. We, as the international clinical neurophysiological community, continue to try to improve not only the nomenclature, but our clinical tools and their implementation to improve the patient care – which would have not been possible without the commitment of colleagues active in the field like Prof Nuwer.

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Comparison of two exhaled biomarkers in children with and without sleep disordered breathing

Airway oxidative stress and inflammation are likely to be involved in sleep disordered breathing (SDB) in children. We aimed to measure concentrations of 8-isoprostane (8-IsoP) in the exhaled breath condensate (EBC) and exhaled nitric oxide (FENO) in patients with SBD and healthy children, in order to assess the relationship between these two biomarkers, disease severity, and overnight changes.

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Incidence and morphometry of sellar bridges and related foramina in dry skulls: Their significance in middle cranial fossa surgery

The current study investigated the incidence, morphology and morphometry of the ossified ligaments expanding between petrous bone and posterior clinoid processes and in between the anterior, middle and posterior clinoid processes. Side symmetry, gender dimorphism and age influence were also studied.

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Optic nerve sheath diameter based on preoperative brain computed tomography and intracranial pressure are positively correlated in adults with hydrocephalus

Publication date: April 2018
Source:Clinical Neurology and Neurosurgery, Volume 167
Author(s): Hyung-Chul Lee, Won-Jong Lee, Yun-Sik Dho, Won-Sang Cho, Yong Hwy Kim, Hee-Pyoung Park
ObjectiveThe usefulness of optic nerve sheath diameter (ONSD) in predicting increased intracranial pressure (ICP) is not well established in adults with hydrocephalus. In this retrospective study, we evaluated the correlation between ONSD measured on brain computed tomography (CT) and ICP in adults with hydrocephalus.Patients and MethodsONSDs were measured on preoperative brain CT images from 64 adult patients with hydrocephalus who underwent extracranial ventricular drainage or a ventriculoperitoneal shunt in 2016. ICP was defined as ventricular fluid pressure.ResultsThe ONSD measured on preoperative CT was greater in patients (n = 8) with a higher ICP (>20 mmHg, 5.8vs. 4.9 mm, P = 0.001). The ONSD was linearly correlated with ICP (r = 0.543, P < 0.001) and was predictive of increased ICP with an area under the receiver operating characteristic curve of 0.834. The optimal cut-off value of 5.3 mm ONSD yielded 88% sensitivity and 79% specificity. The inter-class coefficient of ONSD on preoperative CT was 0.882. The correlation between ONSD on preoperative CT and ICP was detected only in patients with communicating and non-communicating hydrocephalus (r = 0.437 and r = 0.585, P = 0.037 and P = 0.002, respectively).ConclusionONSD measured on preoperative brain CT was linearly correlated with ICP in adult patients with communicating and non-communicating hydrocephalus, and it was a predictor of increased ICP with good discrimination and high inter-observer reliability. These results suggest that preoperative ONSD measurement on brain CT can be helpful to safely manage such patients by providing information about ICP.



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The surgical management of dysphagia secondary to diffuse idiopathic skeletal hyperostosis

Publication date: April 2018
Source:Clinical Neurology and Neurosurgery, Volume 167
Author(s): Y.C. Lui Jonathan, P. Sayal, G. Prezerakos, V. Russo, D. Choi, A.T.H. Casey
ObjectiveThis study reviews the management pathway and surgical outcomes of patients referred to and operated on at a tertiary neurosurgical centre, for dysphagia associated with anterolateral cervical hyperostosis (ACH) in diffuse idiopathic skeletal hyperostosis (DISH).Patients & MethodsElectronic patient records for 6 patients who had undergone anterior cervical osteophytectomy for dysphagia secondary to ACH were reviewed. ACH diagnosis was made by an Ear, Nose and Throat (ENT) specialist and patients were referred to a neurosurgical-led multidisciplinary team (MDT) for review. A senior radiologist performed imaging measurements and vertebral level localization was confirmed via barium-swallow video-fluoroscopy. Speech and language therapists (SLTs) determined the suitability of pre-operative conservative management. Patients were followed-up post-operatively with clinical and radiological assessments.Results6 patients (Male to female ratio, 6:0; mean age, 59 years) were referred to a tertiary neurosurgical centre with DISH related dysphagia, an average of 25 months after ENT review (range, 14–36 months) between 2005 and 2016. The vertebral levels implicated in dysphagia ranged from C2 to T1 with a median of 4 vertebral levels involved. The most frequently affected vertebral levels were C4-6 (all 6 patients). The average antero-posterior height (as measured on axial images) of the most prominent osteophyte was 15.9 mm (range 12.0–20.0 mm).Patients underwent elective cervical osteophytectomy on average 10.8 months after neurosurgical review (range, 3–36 months). One patient had a post-operative haematoma needing evacuation and prolonged hospital stay. The average duration of follow-up was 42.3 months. All our patients maintained good symptomatic resolution without osteophyte recurrence.ConclusionsAll our patients experienced significant and sustained clinical improvement. Anterior cervical osteophytectomy consistently leads to improvement in symptomatic ACH patients without recurrence. Early referral to a neurosurgical multi-disciplinary team (MDT) is indicated in ACH related dysphagia, once conservative management has failed.



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Extracranial arterial wall volume is increased and shows relationships with vascular MRI measures in idiopathic Parkinson’s disease

Publication date: Available online 10 February 2018
Source:Clinical Neurology and Neurosurgery
Author(s): Stephen Ball, Sarah Al-Bachari, Laura M. Parkes, Hedley C.A. Emsley, Charles N. McCollum
ObjectiveIdiopathic Parkinson's disease (IPD) is the second most common neurodegenerative disorder, often complicated by dementia. Cardiovascular risk factors and spontaneous cerebral emboli (SCE) are strongly associated with Alzheimer's (AD) and vascular dementia (VaD). We measured SCE in the middle cerebral artery and arterial wall volume in the extracranial arteries in patients with IPD and controls, and explored the relationships with structural and physiological MRI brain neurovascular measures.Patients and MethodsArterial wall volume over 2cm of the axillary and internal carotid arteries (ICA) bilaterally was measured by 3-D tomographic ultrasound in 15 IPD patients and 16 age/gender matched controls. SCE were counted by Transcranial Doppler (TCD) using international consensus criteria. Venous to arterial circulation shunting (v-aCS), usually through a patent foramen ovale (PFO), was measured using a TCD technique with intravenous microbubble contrast. Structural and physiological MRI brain neurovascular measures, acquired separately, comprised white matter lesion volume (WMLV), cerebral blood flow (CBF) and arterial arrival time (AAT).ResultsMean (95% CI) axillary and ICA wall volume was higher in IPD patients at 523mm³ (446, 600) and 455mm³ (374, 536) respectively compared with 412mm³ (342, 483) and 408 mm³ (362, 454) in controls being significant for the axillary artery (p = 0.04).Cerebral WMLV was related to mean arterial wall volume for both axillary (r = 0.555, p = 0.009) and ICA (r = 0.559, p = 0.026) in all participants.SCE were detected in four IPD patients and three controls (p = 1.00). Two IPD patients and three controls were positive for a v-aCS equivalent to PFO (p = 0.477).ConclusionAlthough frequent in AD and VaD, neither SCE nor v-aCS were associated with IPD. This is the first study to demonstrate arterial wall volume is increased in IPD and relates to WMLV.



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Re: Acrylic stent to aid placement of footplate of palatal distractor during surgically-assisted rapid palatal expansion

I read with interest the recent paper by Payne et al1 and commend them on their use of a stent to indicate the position of the abutment plates of the transpalatal distractor.2 I would, however, like to raise two important points.

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Re: Wrong tooth extraction: an examination of “Never Event” data

I read the recent article by Pemberton et al1 with interest and would like to draw attention to a recent experience in our department.

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Comments on “Compositional and functional variations of oral microbiota associated with the mutational changes in oral cancer” by Yang et al.

We read with great interest the article titled "Compositional and functional variations of oral microbiota associated with the mutational changes in oral cancer" by Yang et al. [1]. We greatly appreciate the author's efforts for exploring the association between mutations in oral cancer and microbiome, thus opening up an interesting avenue in oral microbiome research. Host genetics has already been demonstrated to influence the gut microbiome [2] and the authors of this paper have reported an association between mutations seen in oral cancer with the salivary microbiome.

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Sporadic pediatric papillary thyroid carcinoma harboring the ETV6/NTRK3 fusion oncogene in a 7-year-old Japanese girl: a case report and review of literature

Journal Name: Journal of Pediatric Endocrinology and Metabolism
Issue: Ahead of print


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CyberKnife robotic radiosurgery in the multimodal management of acromegaly patients with invasive macroadenoma: a single center’s experience

Abstract

Surgery is the primary treatment for acromegaly. However, surgery may not be curative of some tumors, particularly invasive macroadenomas. Adjuvant radiation, specifically robotic stereotactic radiosurgery (rSRS), may improve the endocrine outcome. We retrospectively reviewed hormonal and radiological data of 22 acromegalic patients with invasive macroadenomas treated with rSRS at Stanford University Medical Center between 2000 and 2016. Prior to treatment, the tumor's median maximal diameter was 19 mm (2.5–50 mm). Cavernous sinus invasion occurred in 19 patients (86.3%) and compression of the optic chiasm in 2 (9.0%). At last follow up, with an average follow up of 43.2 months, all patients had a reduction in their IGF-1 levels (median IGF-1% upper limit of normal (ULN) baseline: 136% vs last follow up: 97%; p = 0.05); 9 patients (40.9%) were cured, and 4 (18.1%) others demonstrated biochemical control of acromegaly. The median time to cure was 50 months and the mean interval to cure or biochemical control was 30.3 months (± 24 months, range 6–84 months). Hypopituitarism was present in 8 patients (36.3%) and new pituitary deficits occurred in 6 patients with a median latency of 31.6 ± 14.5 months. At final radiologic follow-up, 3 tumors (13.6%) were smaller and 19 were stable in size. The mean biologically effective dose (BED) was higher in subjects cured compared to those with persistent disease, 163 Gy3 (± 47) versus 111 Gy3 (± 43), respectively (p = 0.01). No patient suffered visual deterioration. Robotic SRS is a safe and effective treatment for acromegaly: radiation-induced visual complications and hypopituitarism is rare.

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Ectopic impulse generation in peripheral nerve hyperexcitability syndromes and amyotrophic lateral sclerosis

Publication date: Available online 10 February 2018
Source:Clinical Neurophysiology
Author(s): Yu-ichi Noto, Neil G. Simon, Alexis Selby, Nidhi Garg, Kazumoto Shibuya, Nortina Shahrizaila, William Huynh, José M. Matamala, Thanuja Dharmadasa, Susanna B. Park, Steve Vucic, Matthew C. Kiernan
ObjectiveTo elucidate differences in the distribution and firing frequency of fasciculations between peripheral nerve hyperexcitability syndromes and amyotrophic lateral sclerosis (ALS) and to explore the generator site of fasciculations.MethodsUltrasound of 14 preselected muscles was performed in patients with peripheral hyperexcitability and ALS. The distribution and firing frequency of fasciculations were calculated. Cortical excitability assessment was also done by threshold tracking transcranial magnetic stimulation.ResultsIn total, 518 muscles from 37 peripheral hyperexcitability patients and 756 muscles from 54 ALS patients were examined. Regarding the detection rate, 74% of muscles in ALS patients demonstrated fasciculations, compared with 34% of muscles in peripheral hyperexcitability patients (P <0.001). The number of unique repeating focal muscle fasciculation movements per muscle and firing frequency of individual fasciculations in ALS were both significantly higher than those in peripheral hyperexcitability (P <0.001). Furthermore, cortical silent period duration negatively correlated with the number and firing frequency of fasciculations in ALS (P <0.05). A similar relationship was not evident in peripheral hyperexcitability.ConclusionsALS patients exhibited a widespread distribution of fasciculations, whereas peripheral hyperexcitability patients had a lower limb-dominant pattern.SignificanceA significant proportion of fasciculations in ALS may be influenced by changes in central excitability.



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Reply to “10-10 Electrode System for EEG Recording”

Publication date: Available online 10 February 2018
Source:Clinical Neurophysiology
Author(s): Margitta Seeck, Thomas Bast, Frans Leijten, Bin He, Sándor Beniczky




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Auditory and language development in Mandarin-speaking children after cochlear implantation

Publication date: Available online 7 February 2018
Source:International Journal of Pediatric Otorhinolaryngology
Author(s): Xing Lu, Zhaobing Qin
ObjectiveTo evaluate early auditory performance, speech perception and language skills in Mandarin-speaking prelingual deaf children in the first two years after they received a cochlear implant (CI) and analyse the effects of possible associated factors.MethodsThe Infant-Toddler Meaningful Auditory Integration Scale (ITMAIS)/Meaningful Auditory Integration Scale (MAIS), Mandarin Early Speech Perception (MESP) test and Putonghua Communicative Development Inventory (PCDI) were used to assess auditory and language outcomes in 132 Mandarin-speaking children pre- and post-implantation.ResultsChildren with CIs exhibited an ITMAIS/MAIS and PCDI developmental trajectory similar to that of children with normal hearing. The increased number of participants who achieved MESP categories 1–6 at each test interval showed a significant improvement in speech perception by paediatric CI recipients. Age at implantation and socioeconomic status were consistently associated with both auditory and language outcomes in the first two years post-implantation.ConclusionMandarin-speaking children with CIs exhibit significant improvements in early auditory and language development. Though these improvements followed the normative developmental trajectories, they still exhibited a gap compared with normative values. Earlier implantation and higher socioeconomic status are consistent predictors of greater auditory and language skills in the early stage.



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Cochlear implantation in children with auditory neuropathy spectrum disorder: A multicenter study on auditory performance and speech production outcomes

Publication date: Available online 7 February 2018
Source:International Journal of Pediatric Otorhinolaryngology
Author(s): Ahmad Daneshi, Marjan Mirsalehi, Seyed Basir Hashemi, Mohammad Ajalloueyan, Mohsen Rajati, Mohammad Mahdi Ghasemi, Hesamaldin Emamdjomeh, Alimohamad Asghari, Shabahang Mohammadi, Mohammad Mohseni, Saleh Mohebbi, Mohammad Farhadi
ObjectivesTo evaluate the auditory performance and speech production outcome in children with auditory neuropathy spectrum disorder (ANSD). The effect of age on the outcomes of the surgery at the time of implantation was also evaluated.MethodsCochlear implantation was performed in 136 children with bilateral severe-to- profound hearing loss due to ANSD, at four tertiary academic centers. The patients were divided into two groups based on the age at the time of implantation; Group I: Children ≤24 months, and Group II: subjects >24 months. The categories of auditory performance (CAP) and speech intelligibility rating (SIR) scores were evaluated after the first and second years of implantation. The differences between the CAP and SIR scores in the two groups were assessed.ResultsThe median CAP scores improved significantly after the cochlear implantation in all the patients (p value < 0.001). The improvement in the CAP scores during the first year in Group II was greater than Group I (p value: 0.007), but the improvement in CAP scores tended to be significantly higher in patients who were implanted at ≤24 months (p value < 0.001). There was no significant difference between two groups in SIR scores at first-year and second-year follow-ups. The evaluation of the SIR improvement revealed significantly higher values for Group I during the second-year follow-up (p value: 0.003).ConclusionThe auditory performance and speech production skills of the children with ANSD improved significantly after cochlear implantation, and this improvement was affected by age at the time of implantation.



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Treatment of extensive post tonsillectomy oropharyngeal stenosis secondary to fibromatosis

Publication date: Available online 2 February 2018
Source:International Journal of Pediatric Otorhinolaryngology
Author(s): Brian Chang, Jennifer F. Ha, David Zopf
Aggressive fibromatosis is an uncommon, benign tumor of fibroblastic origin with high potential for local invasion. Less than a quarter of these lesions are located in the head and neck, and although extremely rare, associations have been demonstrated with physical trauma. We describe a unique case of oropharyngeal fibromatosis with traumaticetiology, managed successfully with surgical excision of the lesion with negative surgical margins. A 5-year old patient was found to have an aggressive fibromatosis causing oropharyngeal stenosis following tonsillectomy. We demonstrate that surgical resection with a clear margin allowed for alleviation of stenosis without recurrences reported since the procedure.



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Granulomatosis with polyangiitis presenting as facial nerve palsy in a teenager

Publication date: Available online 7 February 2018
Source:International Journal of Pediatric Otorhinolaryngology
Author(s): James C. Wang, Brittany A. Leader, Ryan A. Crane, Bernadette L. Koch, Matthew M. Smith, Stacey L. Ishman
Granulomatosis with polyangiitis (GPA, previously known as Wegener's granulomatosis) is an autoimmune systemic small-vessel vasculitis, associated with the presence of anti-neurophil cytoplasmic antibodies with a cytoplasmic staining pattern (c-ANCA). It is characterized by necrotizing granulomas, usually affecting the airways and kidneys. GPA should be considered when patients do not improve despite adequate treatment of otologic symptoms, when patients have unspecific symptoms suggesting systemic disease (e.g. fever, malaise), or when other organs are involved (kidney, lungs, etc.). We present an interesting case of a 14-year-old female with eight-weeks of bilateral otalgia, unilateral facial nerve palsy, decreased appetite, and fatigue refractory to steroid, anti-viral, and antibiotic treatment ultimately diagnosed with GPA.



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Volume relationships between cricoid and main stem bronchi in children using three-dimensional computed tomography imaging

Publication date: Available online 5 February 2018
Source:International Journal of Pediatric Otorhinolaryngology
Author(s): Tariq M. Wani, Ayman Mohammad AlAhdal, Mohammed Hakim, Tanveer Hussein, Abdul Basit Mir, Ravees Jan, Dmitry Tumin, Joseph D. Tobias
BackgroundThere are limited data to guide the selection of the appropriate sized endobronchial tube for main stem intubation to provide one-lung ventilation in children. The relationship between the cricoid and the main bronchi (right and left) has been previously evaluated using two-dimensional computed tomography (CT) imaging and video-bronchoscopic images. The present study defines the three-dimensional, CT-derived volume-based relationships between the right main-stem bronchus (RMB), left main-stem bronchus (LMB), and the cricoid ring.MethodsThe three-dimensional CT images of 35 children, less than 8 years of age, undergoing radiological evaluation unrelated to airway or mediastinal symptomatology were examined. The images of the airway column were evaluated at the level of the cricoid and main stem bronchi (right and left). Volumes were calculated and comparisons made between these levels.ResultsThere was no statistically significant difference based on gender for the cricoid and main stem bronchi volumes. A statistically significant difference was observed between the cricoid and left main stem bronchi volumes as well as between the right and left main stem bronchi.ConclusionThe relationship (ratio) between the volumes of the cricoid and main stem bronchi remains constant by age. The cricoid dimensions can be used to predict the main stem bronchi dimensions.



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Bony cochlear nerve canal stenosis in pediatric unilateral sensorineural hearing loss

Publication date: March 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 106
Author(s): Chae-Hyun Lim, Ji Hyung Lim, Doyoun Kim, Ho sung Choi, Dong-Hee Lee, Dong-Kee Kim
ObjectivesThis study was performed to evaluate the frequency of bony cochlear nerve canal (BCNC) stenosis and its clinical significance in pediatric patients with unilateral sensorineural hearing loss (SNHL) of unknown etiology.Materials and methodsWe analyzed the medical records and temporal bone computed tomography (CT) results of patients less than 13 years of age with a diagnosis of unilateral SNHL of unknown etiology between July 2007 and July 2017. We compared the BCNC diameter between both sides and analyzed the age at diagnosis, degree of hearing loss, and accompanying inner ear anomalies.ResultsIn 42 patients, the mean age at diagnosis was 7.4 ± 3.6 years, and the average hearing level in the affected ear was 87.9 ± 20.0 dB HL (decibels hearing level). The average diameter of the BCNC was 1.22 ± 0.75 mm on the affected side and 1.96 ± 0.52 mm on the normal side. The most suitable criterion for BCNC stenosis appeared to be a diameter of 1.2 mm by the recursive partitioning procedure. With application of this criterion, the rate of BCNC stenosis was significantly greater on the affected side than on the normal side (52.4% vs. 4.8%, respectively; P < 0.05). A narrow internal acoustic canal was found in two patients, and vestibular and cochlear anomalies were found in three patients each.ConclusionsOur results suggest that it is reasonable to set a diameter of 1.2 mm as a cutoff for BCNC stenosis, and also that BCNC stenosis is a common cause of unilateral SNHL of unknown etiology in childhood.



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Emberger syndrome: A rare association with hearing loss

Publication date: Available online 7 February 2018
Source:International Journal of Pediatric Otorhinolaryngology
Author(s): Faisal Zawawi, Meirav Sokolov, Thomas Mawby, Karen A. Gordon, Blake C. Papsin, Sharon L. Cushing
Emberger Syndrome (ES) is a rare genetic disorder characterized by lymphedema and myelodysplasia. It is also associated with hearing loss. The genetic mutations associated with ES are not part of the comprehensive 80 gene next generation sequencing (NGS) panel. As a result, the otolaryngologist should maintain an index of suspicion for ES in any child with SNHL who presents repeatedly with recurrent infections, lymphedema and/or cutaneous warts. This paper describes the clinical evolution and management of two children who were followed up for hearing loss and eventually were diagnosed with ES.



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Pattern of allergic rhinitis among children in Ekiti, Nigeria

Publication date: March 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 106
Author(s): Waheed Atilade Adegbiji, Gabriel Toye Olajide, Anthony Oyebanji Olajuyin, Shuaib Kayode Aremu, Akanbi Ganiyu Olusola
BackgroundAllergic rhinitis is a chronic and recurrent nasal condition. It is often neglected in children with late presentation. This study aimed at determining the prevalence, sociodemographic features, comorbid illnesses, complications and quality of life in children with allergic rhinitis in the study institution.Materials and methodsThis is a prospective hospital based study of children with allergic rhinitis in Ekiti state university teaching hospital, Ado Ekiti. The study was carried out in ENT department over a period of two years (between June 2015 to May 2017). Informed consent was obtained from the parents/guardian/patients and consented patients were enrolled into the study. Data were obtained by pretested interviewers assisted questionnaire. Details of their history, physical examination and investigations were carried out and findings were documented. All data obtained were descriptively analysed using SPSS version 18.0 and presented in simple tables and charts. Ethical clearance was sought for and obtained from the ethical committee of the hospital.ResultsA total of 4341 patients were seen out of which 265 were children with allergic rhinitis. Prevalence of allergic rhinitis in children in this study was 6.1%. There were 63.0% males with male to female ratio of 2:1. Allergic rhinitis was peaked at preschool age group (1–5 years) accounted for 47.9%.A total of 42.3% participants were living in urban setting while 57.7% were from rural setting. Majorities (40.4%) of the patients were in nursery and parent's major occupation was mainly farming in 27.2%.There was positive family history of allergy in 54.7% patients. Perennial allergic rhinitis were noted in 63.8% patients while seasonal allergic rhinitis were noted in 36.2% patients. Major form of allergens was inhalant 81.8% and the least form of allergen was ingestant 5.7%.The commonest identified trigger factors among the study population were as follows: dust, cold weather and smoke which were accounted for 59.6%, 37.4% and 18.9% of the study patients respectively. Other noted triggering factors were soap and perfume which accounted for 4.2% and 1.1% respectively.Major associated comorbid illnesses among the patients were tonsils hypertrophy, adenoid hypertrophy and inferior turbinate hypertrophy which accounted for 55.5%, 46.4% and 40.4% respectively. Clinical presentations of allergic rhinitis in this study were mainly 75.8% nasal blockage, 65.3% runny nose and 8.5% recurrent sneezing.Commonest complications of allergic rhinitis were 35.1% pharyngitis, 32.1% otitis media and 28.3% headache.Treatment of allergic rhinitis leads to improvement on the clinical features in 90.1% patients. No significant improvement in clinical features were noticed in 9.8% patients. None of the studied patients reported worse clinical condition after treatment of allergic rhinitis. No mortality was recorded from allergic rhinitis in this study.ConclusionAllergic rhinitis affect all paediatric age group and there were delayed presentation in the participants. There were associated comorbid illnesses, complications and affectation of quality of life at presentation in majority of the patients.



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Healthcare and psychosocial experiences of individuals with craniofacial microsomia: Patient and caregivers perspectives

Publication date: Available online 7 February 2018
Source:International Journal of Pediatric Otorhinolaryngology
Author(s): Daniela V. Luquetti, Michelle R. Brajcich, Nicola M. Stock, Carrie L. Heike, Alexis L. Johns
ObjectiveCraniofacial microsomia (CFM) is primarily characterized by underdevelopment of the ear and mandible, with several additional possible congenital anomalies. Despite the potential burden of care and impact of CFM on multiple domains of functioning, few studies have investigated patient and caregiver perspectives. The objective of this study was to explore the diagnostic, treatment-related, and early psychosocial experiences of families with CFM with the aim of optimizing future healthcare delivery.MethodsForty-two caregivers and nine adults with CFM responded to an online mixed-methods survey. Descriptive statistics and qualitative methods were used for the analysis.ResultsSurvey respondents reported high rates of subspecialty evaluations, surgeries, and participation in therapies. Some participants reported receiving inaccurate or incomplete information about CFM and experienced confusion about etiology. Communication about CFM among family members included mostly positive messages. Self-awareness of facial differences began at a mean age of three years and teasing at mean age six, with 43% of individuals four years or older reporting teasing. Teasing often involved name-calling and frequent reactions were ignoring and negative emotional responses. Participants ranked "understanding diagnosis and treatment" as a top priority for future research and had the most questions about etiology and treatment guidance.ConclusionsThe survey results on the healthcare and psychosocial experiences from birth through adulthood of individuals with CFM reinforce the need for ongoing psychological assessment and intervention. Healthcare provision could be improved through establishing diagnostic criteria and standardized treatment guidelines, as well as continued investigation of CFM etiology.



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Challenges and outcomes of cholesteatoma management in children with Down syndrome

Publication date: March 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 106
Author(s): Saied Ghadersohi, Bharat Bhushan, Kathleen R. Billings
IntroductionThe high incidence of chronic otitis media with effusion and Eustachian tube dysfunction in children with Down syndrome (DS) may predispose them to cholesteatoma formation. Establishing the diagnosis, choosing the appropriate operative intervention, and post-operative care can be challenging.ObjectiveTo describe management strategies for cholesteatoma diagnosis, surgical treatment, and post-operative management in children with Down syndrome.MethodsRetrospective case series of 14 patients (17 total ears) with Down syndrome diagnosed with cholesteatoma over a 9-year period.ResultsA total of 14 patients with cholesteatoma (3 with bilateral disease) were analyzed. Thirteen ears (76.5%) had ≥2 tympanostomy tubes insertions prior to cholesteatoma diagnosis, and otorrhea and hearing loss were the most common presenting symptoms. Common pre-operative CT scan findings included mastoid sclerosis and ossicular erosion. The average age at first surgery was 9.8 years, and the average follow-up was 4.3 years. For acquired cholesteatoma, most ears were managed with canal wall up (CWU) approaches, but ultimately 6/15 (40.0%) required canal wall down (CWD) approaches. Postoperatively, 3 (20.0%) ears developed new tympanic membrane retraction pockets, but no recurrent cholesteatoma. Four (26.7%) ears developed recurrent disease, and 3 (20.0%) had residual disease at secondary procedures. Ossiculoplasty was performed in 4 ears. Twelve (70.6%) ears were rehabilitated with hearing aids or FM systems.ConclusionsThe diagnosis of cholesteatoma in Down syndrome was associated with otorrhea, hearing loss, and CT scan findings of ossicular erosion and mastoid sclerosis. Most cases were managed with CWU surgical approaches. Hearing aid use was common post-operatively.



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Tracheal paraganglioma presenting as stridor in a pediatric patient, case report and literature review

Publication date: Available online 7 February 2018
Source:International Journal of Pediatric Otorhinolaryngology
Author(s): Amy L. Dimachkieh, Allison Dobbie, Damon R. Olson, Mark A. Lovell, Jeremy D. Prager
ObjectiveTo review tracheal paragangliomas and describe the clinical presentation, radiologic findings, operative management, and histologic findings of a pediatric patient who presented with stridor refractory to traditional asthma therapy.MethodsChart review of an 8-year-old male who presented to a tertiary care pediatric hospital and literature review of tracheal paragangliomas.ResultsWe present the case of an 8-year-old male who presented with new-onset of wheezing and dyspnea on exertion. He was given a new diagnosis of asthma and treated with bronchodilators that failed to improve his symptoms, which progressed over 3 months until he presented urgently with biphasic stridor. Bedside flexible laryngoscopy failed to reveal an etiology. Computed tomography (CT) imaging demonstrated 17 × 12 × 16 mm exophytic mass arising from the posterior membranous trachea with extension of the mass to the border of the thyroid gland and separate from the esophagus. Magnetic resonance imaging (MRI) angiography confirmed vascular supply from the right thyrocervical trunk and inferior thyroid artery. Rigid microlaryngoscopy revealed a friable vascular polypoid mass 2 cm distal to the vocal folds with 75% obstruction of the airway from which a small biopsy was taken. Pathology confirmed paraganglioma with neuroendocrine cells arranged in "zellballen" architecture and strong immunopositivity for chromogranin and synaptophysin in the neuroendocrine cells and S100 immunopositivity in the sustentacular cells. The patient underwent complete open resection of the tumor including three tracheal rings with primary anastomosis. Final pathology confirmed paraganglioma and negative margins. Genetic screening revealed a succinate dehydrogenase complex subunit C (SDHC) germline mutation, confirming hereditary paraganglioma/pheochromocytoma syndrome. He remains well at 3 month follow up without dyspnea or stridor.ConclusionTracheal paragangliomas are exceptionally rare, with 12 reported cases. This is the only pediatric case reported. In pediatric patients with persistent airway complaints, subglottic and tracheal masses and obstruction should be considered. Due to the vascularity and endotracheal component of tracheal paragangliomas, a detailed surgical plan should consider embolization, endotracheal laser photocoagulation and electrocautery, and open surgical resection. Additionally, pediatric patients benefit from a multidisciplinary approach including radiology, endocrinology, and genetic counseling.



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Geographic health disparities in the Los Angeles pediatric esophageal foreign body population

Publication date: March 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 106
Author(s): Kevin Hur, Varun Angajala, Dennis Maceri, Christian Hochstim
ObjectiveTo assess geographical sociodemographic differences in the pediatric esophageal foreign body population of Los Angeles.MethodsWe retrospectively reviewed the medical records of 128 consecutive pediatric patients at Children's Hospital Los Angeles (CHLA) from 2014 to 2017 with a diagnosis of a retained foreign body in the esophagus removed by rigid or flexible esophagoscopy. Sociodemographic information including zip code of residence was extracted and analyzed with Chi-square, Fisher's exact test, and multivariable logistic regression.ResultsThe average age of patients with a retained esophageal foreign body in this study was 2.5 years old, 52.3% were male, 91.4% had no past medical history, 53.1% were Hispanic, 82.0% had public health insurance, and 63.3% were transfers from an outside hospital. The most common foreign body removed was a coin. There were no significant differences in gender, race, type of health insurance, or income between patients that lived within 10 miles of CHLA versus farther than 10 miles. On multivariable analysis, zip codes with a high volume of esophageal foreign bodies were more likely to be lower income neighborhoods. Gender, race, type of health insurance, and distance from CHLA were not risk factors for zip codes with a high volume of esophageal foreign bodies.ConclusionGeographic areas in the greater Los Angeles community with a high volume of retained esophageal foreign bodies requiring endoscopic removal at our institution are associated with lower income neighborhoods. Further studies should be performed to better understand health disparities within the U.S. pediatric esophageal foreign body population.



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Meta-analysis of the comorbidity rate of allergic rhinitis and asthma in Chinese children

Publication date: Available online 5 February 2018
Source:International Journal of Pediatric Otorhinolaryngology
Author(s): Wei Kou, Xuelei Li, Hongbing Yao, Ping Wei
ObjectivesAllergic rhinitis (AR) and asthma often occur concomitantly and are the two most common inflammatory conditions of the airways in children. Large-scale studies investigating the comorbidity of asthma and AR in children are rare. So, we performed a meta-analysis to describe the comorbidity rate of asthma and AR in Chinese children.MethodsWe retrieved related studies from Pubmed, Science, Springer, Elsevier, Embase, BMJ, and four Chinese biomedical databases, including Wanfang Data, VIP, CBM, and CNKI. From these individual studies, the comorbidity rate of asthma and AR in Chinese children was extracted and pooled to generate summary effect estimates in R version 3.2.3.ResultsThe meta-analysis included 25 cross-sectional studies. The results indicated that in China, the incidence of asthma in children with AR is 35.01% (95% CI: 32.32%–37.70%) and the incidence of AR in children with asthma is 54.93% (95% CI: 53.05%–56.80%).ConclusionsThe comorbidity of AR and asthma is high in Chinese children. Statistically, the prevalence of AR was higher in children with asthma, as opposed to the prevalence of asthma in children with AR. The comorbidity rate of AR and asthma signifies the importance of improving the recognition and treatment under both conditions by respiratory physicians and otolaryngologists.



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Microbiology and antibiotic therapy of subperiosteal orbital abscess in children with acute ethmoiditis

Publication date: March 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 106
Author(s): A. Coudert, S. Ayari-Khalfallah, P. Suy, E. Truy
ObjectiveThe objective of this study was to investigate the microbiological cultures and the management of acute ethmoiditis complicated by subperiosteal orbital abscess (SPOA) in a pediatric population.MethodsThe medical records of children under 18 years old was performed in a tertiary referral pediatric center from January 2009 to April 2017. Clinical examination, computed tomography scans, medical and surgical treatments were reviewed and compared to other studies in literature.ResultsOne hundred and twenty-nine children were hospitalized for acute ethmoiditis. Among them, forty eight were complicated by SPOA. The mean age of these children were 7 years (range 10 months–16 years). Thirtyfour underwent surgical drainage; for the others the medical treatment was sufficient. Microbiological samples were obtained during the surgical intervention and were contributive in 91% of cases. Streptococcus spp was the most frequently encountered bacteria (60% of cases). We also found anaerobic bacteria (12%), and Staphylococcus aureus (12%). 94% of children received two intravenous antibiotics (a third-generation cephalosporin and metronidazole) for a mean duration of four days. Then the oral treatment was based on amoxicillin-clavulanate during about 8.5 days. All children were cured without sequelae.ConclusionsFor five years Streptococcus milleri, Staphylococcus spp and anaerobic bacteria are on the rise in acute ethmoiditis complicated by SPOA. That is why antibiotics must be adapted to these bacteria even in children under ten years old.



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Human beta defensin-1 is involved in the susceptibility to adenotonsillar hipertrophy

Publication date: Available online 5 February 2018
Source:International Journal of Pediatric Otorhinolaryngology
Author(s): Luisa Zupin, Fulvio Celsi, Martina Bresciani, Eva Orzan, Domenico Leonardo Grasso, Sergio Crovella
IntroductionInnate immunity molecules are known to play a pivotal role in the homeostasis of the oral mucosa, permitting the presence of commensal microflora and, at the same time, providing a first line of defense against pathogens attempting to invade the oral cavity.Tonsils represent the local immune tissue in oral cavity, being able to provide a non-specific response to pathogens; however, in the presence of microbes or foreign materials present in the mouth tonsils could became infected and develop chronic inflammation, thus leading to hypertrophy.The etiology of the disease is multifactorial depending upon environmental and host factors, the latter including molecules of mucosal innate immunity.MethodsNinety-five children with adeno-tonsillar hypertrophy subjected to adeno-tonsillectomy were recruited at the pediatric otorhinolaryngology service of the Institute for Maternal and Child Health IRCCS Burlo Garofolo, Trieste (Italy).The specimen discard from the surgery were used for genomic DNA extraction and genotyping, for mRNA extraction and gene expression analysis, finally the samples were cut and used to prepare slides to perform immunohistochemistry.ResultsFunctional polymorphisms within DEFB1 gene, encoding the human beta defensin-1 (hBD-1), were analyzed finding association between DEFB1 rare haplotypes and susceptibility to adenotonsillar hypertrophy. DEFB1 mRNA expression was detected in the tonsils and the hBD-1 protein was localized at the epithelia of tonsils mainly in the proximity of the basal lamina.ConclusionOur findings lead us to hypothesize an involvement of hBD-1 mediated innate immunity in the modulation of the susceptibility towards adenotonsillar hypertrophy development.



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Hospital cost analysis of children with preseptal cellulitis

Publication date: March 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 106
Author(s): İlknur Çağlar, Cansu Kafes, Mine Korcum, Mine Düzgöl, Ahu Kara, Süleyman Nuri Bayram, Hurşit Apa, İlker Devrim
ObjectiveHospitalization of the children with preseptal cellulitis creates a burden on healthcare costs. This study aimed to analyze the hospital costs for preseptal cellulitis and determine the factors contributing.MethodsChildren, between 1 and 18 years old, who were admitted to hospital for preseptal cellulitis from May 2013 to December 2016 were included in the study. Patients were divided into groups by age (under or equal to five years and older than five years) and by the presence of sinusitis. Demographics, length of stay and total and categorical hospital costs were evaluated retrospectively.ResultsThe study included 54 patients with a mean age of 5 years. Thirty one of the patients were under five years of age. The most common symptoms were swelling (94.4%) and redness (83.3%) around eye. Among the predisposing factors, sinusitis was the most common one (37%). The average length of stay was 4.5 days. Total hospital cost of all patients was $11,841. Antibiotic costs (37%) and inpatient floor costs (36%) were the greatest expenditures. Between age groups, length of stay was longer, and inpatient floor and antibiotic costs were significantly higher in the group of >5 years (p = 0.007, p = 0.004 and p = 0.001, respectively). In the group with sinusitis, length of stay was longer, and all hospital costs were significantly higher compared to the group without sinusitis (p < 0.001). There was a strong, positive correlation between length of stay and hospital costs (r = 0.854, n = 53, p < 0.001). Sinusitis was a significant factor (p < 0.001) for longer length of stay, but age was not (p = 0.841).ConclusionSinusitis was found to be an important factor contributing to longer length of stay and higher hospital costs for preseptal cellulitis. Oral or ambulatory intravenous antimicrobial treatment strategies might decrease the hospital expenditure in these patients; however care should be taken in the presence of sinusitis.



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Analysis of risk factors associated with unilateral hearing loss in children who initially passed newborn hearing screening

Publication date: March 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 106
Author(s): Eric N. Appelbaum, Jessica B. Howell, Derek Chapman, Arti Pandya, Kelley M. Dodson
ObjectiveTo analyze 2007 Joint Committee on Infant Hearing (JCIH) risk factors in children with confirmed unilateral hearing loss (UHL) who initially passed newborn hearing screening.MethodsRetrospective record review of 16,108 infants who passed newborn hearing screening but had one or more JCIH risk factors prompting subsequent follow-up through the universal newborn hearing screening (UNHS) program in Virginia from 2010 to 2012. The study was reviewed and qualified as exempt by the Virginia Commonwealth University Institutional Review Board (IRB) and the Virginia Department of Health.ResultsOver the 2-year study period, 14896 (4.9% of total births) children passed UNHS but had the presence of one or more JCIH risk factor. Ultimately, we identified 121 babies from this group with confirmed hearing loss (0.7%), with 48 babies (0.2%) showing UHL. The most common risk factors associated with the development of confirmed UHL after passing the initial screen were neonatal indicators, craniofacial anomalies, family history, and stigmata of syndrome associated with hearing loss.ConclusionNeonatal indicators and craniofacial anomalies were the categories most often found in children with confirmed unilateral hearing loss who initially passed their newborn hearing screen. While neonatal indicators were also the most common associated risk factor in all hearing loss, craniofacial abnormalities are relatively more common in children with UHL who initially passed newborn hearing screening. Further studies assessing the etiology underlying the hearing loss and risk factor associations are warranted.



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Tympanostomy tubes for serous otitis media and risk of recurrences

Publication date: March 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 106
Author(s): Nathalie Klopp-Dutote, Catherine Kolski, Vladimir Strunski, Cyril Page
ObjectivesTo determine the value of tympanostomy tubes (TTs) in the management of serous otitis media (SOM) and the risk factors for SOM recurrence.MethodThis single-centre cohort study was performed in the University hospital of Amiens, France; and concerned 215 under-12 children having undergone at least one bilateral TT (Shepard grommet-type) placements for SOM.ResultsThe mean TT retention time was 10 months. SOM recurred in 79 children (62.79%) and thus required a second TT placement (bilaterally in 90% of these cases). Overall, 29.3% of the patients underwent a total of two TT placements, 5.58% underwent three placements and 0.93% underwent four placements. After their first-ever TT placement, 17 children had complications: 10 cases of otorrhoea (4.6%), 4 cases of retraction pocket (1.9%) and 3 perforations of the tympanic membrane (1.4%). At last follow-up, the most common complications were tympanosclerosis (6.9%) and perforation of the tympanic membrane (6.5%). In a multivariate analysis, the only significant risk factors for SOM recurrence were age below 48 months at the time of TT placement, and a TT retention time below 9 months. In contrast, a history of allergy, gastro-oesophageal reflux, prematurity or passive smoking were not significantly associated with recurrence.ConclusionAge at the time of TT placement and the TT retention time were significantly associated with SOM recurrence. The TT retention time and the number of TT placements were not associated with the risk of long-term complications.



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Cochlear implantation in pediatric patients with Cockayne Syndrome

Publication date: March 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 106
Author(s): Renae D. Van Wyhe, Claudia V. Emery, Robert A. Williamson
Cockayne Syndrome (CS) is a rare, autosomal recessive disorder characterized by a spectrum of phenotypic abnormalities, including progressive sensorineural hearing loss (SNHL) that involves both peripheral and central components. To date, a single series of CS patients undergoing cochlear implant (CI) placement has been reported; this study reports on additional previously unreported pediatric CI recipients. Subjective benefits were noted early after activation in both patients, and speech perception scores improved over time as well, varying from 42 to 70% (versus 0–12% previously). Thus, we report that cochlear implantation in pediatric patients with CS can be effective in the management of progressive SNHL.



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Acute external otitis as debut of acute myeloid leukemia - A case and review of the literature

Publication date: March 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 106
Author(s): Joachim Slengerik-Hansen, Therese Ovesen
Acute leukemia is a well known childhood cancer. The relation between leukemia and otological symptoms has long been established but is highly rare as a debut symptom of leukemia. External otitis is a common condition affecting many children, and most cases are successively treated with topical medicine. Here we present a child with acute external otitis later shown to be the debut symptom of acute myeloid leukemia, to our knowledge the first specific case described. We have reviewed the literature to find red flags for suspicion of severe disease in case of acute external otitis.



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Vastus lateralis motor unit firing rate is higher in females with patellofemoral pain

Publication date: Available online 10 February 2018
Source:Archives of Physical Medicine and Rehabilitation
Author(s): Alessio Gallina, Michael A. Hunt, Paul Hodges, S. Jayne Garland
ObjectiveTo compare neural drive, determined from motor unit firing rate, to the vastus medialis and lateralis in females with and without patellofemoral pain.DesignCross-sectional study.SettingUniversity research laboratory.ParticipantsFemales (N=56) 19-35 years old, 36 with patellofemoral pain and 20 controls. Interventions: Not applicable.Main Outcome Measure(s)Participants sustained an isometric knee extension contraction at 10% of their maximal voluntary effort for 70s. Motor units (N=414) were identified using high-density surface electromyography. Average firing rate was calculated between 5 and 35s after recruitment for each motor unit. Initial firing rate was the inverse of the first three motor unit inter-spike intervals.ResultsIn control participants, vastus medialis motor units discharged at higher rates than vastus lateralis (p=0.001). This was not observed in females with patellofemoral pain (p=0.78) due to a higher discharge rate of vastus lateralis compared to control participants (p=0.002). No between-group differences were observed for vastus medialis (p=0.93). Similar results were obtained for the initial motor unit firing rate.ConclusionsThese findings suggest that females with patellofemoral pain have a higher neural drive to vastus lateralis but not vastus medialis, which may be a contributor of the altered patellar kinematics observed in some studies. The different neural drive may be an adaptation to patellofemoral pain, possibly to compensate for decreased quadriceps force production, or a precursor of patellofemoral pain.



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The clinical assessment of spasticity in people with spinal cord damage: recommendations from the Ability Network, an international initiative

Publication date: Available online 10 February 2018
Source:Archives of Physical Medicine and Rehabilitation
Author(s): Anand V. Nene, Alexandre Rainha Campos, Klemen Grabljevec, Arminda Lopes, Bengt Skoog, Anthony S. Burns
A thorough assessment of the extent and severity of spasticity, and its impact on functioning, is central to the effective management of spasticity in persons with spinal cord damage (SCD). These individuals, however, do not always receive adequate assessment of their spasticity. Inadequate assessment compromises management when the impact of spasticity and/or need for intervention are not fully recognized. Assessment is also central to determining treatment efficacy. A barrier to spasticity assessment has been the lack of consensus on clinical and functional measures suitable for routine clinical practice. To extend upon existing work, a working group of the Ability Network identified and consolidated information on possible measures, then synthesized and formulated findings into practical recommendations for assessing spasticity and its impact on function in persons with SCD. Sixteen clinical and functional measures that have been used for this purpose were identified using a targeted literature review. These were mapped to the relevant domains of the International Classification of Functioning, Disability and Health (ICF) to assess the breadth of their coverage; coverage of many domains was found to be lacking, suggesting a focus for future work. The advantages, disadvantages and usefulness of the measures were assessed using a range of criteria, with a focus on usefulness and feasibility in routine clinical practice. Based on this evaluation, a selection of measures suitable for initial and follow-up assessments are recommended. The recommendations are intended to have broad applicability to a variety of healthcare settings where people with SCD are managed.



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Screening for Abdominal Aortic Aneurysm During Transthoracic Echocardiography: A Systematic Review and Meta-analysis

Publication date: Available online 9 February 2018
Source:European Journal of Vascular and Endovascular Surgery
Author(s): Christos Argyriou, George S. Georgiadis, Nikolaos Kontopodis, Arun D. Pherwani, Joost A. Van Herwaarden, Constantijn E.V.B. Hazenberg, George A. Antoniou
BackgroundScreening for abdominal aortic aneurysm (AAA) during transthoracic echocardiography (TTE) may be an effective targeted screening strategy.ObjectiveThe aim was to assess the feasibility of AAA screening during TTE and to estimate the prevalence of AAA in patients undergoing TTE.MethodsElectronic bibliographic sources were interrogated using a combination of free text and controlled vocabulary searches to identify studies reporting on AAA screening during TTE. The review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement standards. Fixed effect or random effects models were used to calculate pooled prevalence estimates.ResultsTwenty observational cohort studies were identified reporting a total of 43,341 participants (23,291 men and 20,050 women). Hypertension was reported in 41% (95% CI 38–43), hypercholesterolemia in 31% (95% CI 29–32), diabetes mellitus in 20% (95% CI 19–22), and tobacco use in 37% (95% CI 35–38). The aorta was visualised in 86% (95% CI 84–88) of the screened population. The pooled prevalence of AAA in the entire screened population was 0.033 (95% CI 0.024–0.044). The pooled prevalence of AAA in men was 0.046 (95% CI 0.032–0.065) and in women it was 0.014 (95% CI 0.008–0.022). The mean age of participants in whom an AAA was detected ranged across the studies from 66 to 85 years. The mean diameter of the aneurysm identified ranged across the studies from 35 mm to 45 mm. Clinical outcomes in participants with a detected AAA were poorly reported.ConclusionsScreening for AAA during TTE may identify a population group with a high risk of AAA in whom targeted screening may be beneficial. Further research is required to investigate the cost-effectiveness and clinical benefits of AAA screening in this setting.



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NUP98-BPTF gene fusion identified in primary refractory acute megakaryoblastic leukemia of infancy

Abstract

The advent of large scale genomic sequencing technologies significantly improved the molecular classification of acute megakaryoblastic leukaemia (AMKL). AMKL represents a subset (∼10%) of high fatality pediatric acute myeloid leukemia (AML). Recurrent and mutually exclusive chimeric gene fusions associated with pediatric AMKL are found in 60-70% of cases and include RBM15-MKL1, CBFA2T3-GLIS2, NUP98-KDM5A and MLL rearrangements. In addition, another 4% of AMKL harbor NUP98 rearrangements (NUP98r), with yet undetermined fusion partners. We report a novel NUP98-BPTF fusion in an infant presenting with primary refractory AMKL. In this NUP98r, the C-terminal chromatin recognition modules of BPTF, a core subunit of the NURF (Nucleosome Remodeling Factor) ATP-dependent chromatin-remodeling complex, are fused to the N-terminal moiety of NUP98, creating and in frame NUP98-BPTF fusion, with structural homology to NUP98-KDM5A. The leukemic blasts expressed two NUP98-BPTF splicing variants, containing one or two tandemly spaced PHD chromatin reader domains. Our study also identified an unreported wild type BPTF splicing variant encoding for 2 PHD domains, detected both in normal cord blood CD34⁺ cells and in leukemic blasts, as with the fly BPTF homolog, Nurf301. Disease course was marked by rapid progression and primary chemoresistance, with ultimately significant tumor burden reduction following treatment with a clofarabine containing regimen. In sum, we report 2 novel NUP98-BPTF fusion isoforms that contribute to refine the NUP98r subgroup of pediatric AMKL. Multicenter clinical trials are critically required to determine the frequency of this fusion in AMKL patients and explore innovative treatment strategies for a disease still plagued with poor outcomes. This article is protected by copyright. All rights reserved.

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Ascertainment of Asthma Prognosis Using Natural Language Processing from Electronic Medical Records

Publication date: Available online 10 February 2018
Source:Journal of Allergy and Clinical Immunology
Author(s): Sunghwan Sohn, Chung-Il Wi, Stephen T. Wu, Hongfang Liu, Euijung Ryu, Elizabeth Krusemark, Alicia Seabright, Gretchen A. Voge, Young J. Juhn
NLP algorithm successfully determined asthma prognosis (i.e., no remission, long-term remission, and intermittent remission) by taking into account asthma symptoms documented in EMR, and addressed the limitations of billing code-based asthma outcome assessment.



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Lumen area change (Delta Lumen) between inspiratory and expiratory CT as a measure of severe outcomes in asthma

Publication date: Available online 10 February 2018
Source:Journal of Allergy and Clinical Immunology
Author(s): Sung Shine Shim, Mark L. Schiebler, Michael D. Evans, Nizar Jarjour, Ron L. Sorkness, Loren Denlinger, Alfonso Rodriguez, Sally Wenzel, Eric A. Hoffman, Ching-Long Lin, David S. Gierada, Mario Castro, Sean B. Fain
BackgroundQuantitative computed tomography (QCT) biomarkers of airway morphology hold potential for understanding and monitoring regional airway remodeling in asthma.ObjectiveWe sought to determine if the change of airway lumen area between TLC and FRC lung volumes measured from CT imaging data was correlated with severe outcomes in asthma patients.MethodsWe studied 152 asthma patients (90 female and 62 male) and 33 normal subjects (12 female and 21 male) with QCT. Post-processing of airways at generations 1-5 (1 = trachea) was performed for wall area percent (WA%), wall thickness percent (WT%), lumen area at baseline TLC (LATLC) and FRC (LAFRC) and low attenuation area at FRC. A new metric (reflecting remodeling and/or distal air trapping), Delta Lumen, was determined from the percent normalized difference in lumen area defined as (LATLC - LAFRC ) / LATLC x 100.ResultsPost-processing of 4501 airway segments was performed (3681 segments in the 152 subjects with asthma and 820 segments in the 33 normal subjects, range 17-28 segments per subject). Delta Lumen was negatively correlated with WT% and low attenuation area (p<0.01) in asthma subjects. Delta Lumen was significantly lower for airway generations 3-5 (segmental airways) in subjects undergoing hospitalization due to exacerbation and in refractory asthma requiring treatment with systemic corticosteroids. WT% and low attenuation area were positively, and Delta Lumen was negatively, associated with systemic corticosteroid treatment (p<0.05) suggesting reduced Delta Lumen is a potential outcomes biomarker in severe asthma.ConclusionReduced Delta Lumen of the central airways measured on QCT is a promising exploratory biomarker of unstable refractory asthma that warrants further study.



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Chronic inducible urticaria: a systematic review of treatment options

Publication date: Available online 10 February 2018
Source:Journal of Allergy and Clinical Immunology
Author(s): Corinna Dressler, Ricardo Niklas Werner, Lisa Eisert, Torsten Zuberbier, Alexander Nast, Marcus Maurer
BackgroundChronic inducible urticaria (CindU) is a condition characterized by the appearance of recurrent wheals, angioedema or both, as a response to specific and reproducible triggers.ObjectiveTo systematically assess the evidence on the efficacy and safety of treatment options for CindU. Results were used to inform the 2017 update of "The EAACI/GA²LEN/EDF/WAO Guideline for the definition, classification, diagnosis and management of Urticaria."MethodsRandomised controlled trials (RCTs) and controlled studies (CCTs) were systematically searched in various databases. Included studies were evaluated with the 'Cochrane Risk of Bias tool'. Where possible, results from single studies were meta-analyzed, applying the Mantel-Haenszel approach using a random-effects model (Der Simonian-Laird).ResultsWe identified 30 studies that included patients with cold urticaria, symptomatic dermographism, delayed pressure urticaria, or cholinergic urticaria,. No studies on other forms of CindU were eligible. Risk of bias was often rated as unclear or high. Overall, second generation antihistamines were more effective than placebo and the available data indicate that updosing may be effective. Omalizumab proved to be effective in patients with symptomatic dermographism, who did not respond to antihistamines. Detailed results are given for each type of CindU.ConclusionsThe available evidence is limited by small samples, heterogeneous efficacy outcomes, and poor reporting quality in many of the included studies. The findings are congruent with the suggested stepwise approach to treating CindUs. However, the data do not allow for drawing specific conclusions for specific subtypes of CindU.



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An ulcer on the ventral tip of tongue

Publication date: Available online 9 February 2018
Source:Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
Author(s): Hélder Antônio Rebelo Pontes, Lucas Lacerda de Souza, Anderson Mauricio Paiva e Costa, Andreia Aparecida da Silva, Fábio Luiz Neves Gonçalves, Felipe Paiva Fonseca, Flávia Sirotheau Correa Pontes




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FBW7 is associated with prognosis, inhibits malignancies and enhances temozolomide sensitivity in glioblastoma cells

Abstract

F-box and WD repeat domain-containing 7(FBW7) is a SCF-type E3 ubiquitin ligase targeting a multitude of oncoproteins for degradation. Acting as one of the most important tumor suppressor it is frequently inactivated in various tumors. In this study we aimed to evaluate the relationship of FBW7 with glioma pathology and prognosis, and examine its effect in glioma malignancies and temozolomide(TMZ)-based therapy. Clinical tissues and TCGA database analysis revealed FBW7 expression was correlated inversely with glioma histology and positively with patient survival time. Lentivirus transfection- induced FBW7 overexpression significantly suppressed proliferation, invasion and migration of U251 and U373 cells whereas knockdown of FBW7 by targeted shRNA promoted proliferation, invasion and migration of glioma cells. Most importantly, the expression level of FBW7 was found to affect 50% inhibition concentration(IC50) of U251 and the TMZ resistant variant. Combining TMZ with FBW7 overexpression notably increased the cytotoxicity than TMZ treatment alone, which was conversely attenuated by FBW7 knockdown. Moreover, flow cytometry(FC) analysis showed either overexpression of FBW7, TMZ or the combination increased proportion of G2/M arrest and apoptotic rate whereas FBW7 inhibition reduced G2/M arrest and apoptosis in U251 cells. Finally, mechanistic study found FBW7 overexpression downregulated Aurora B, Mcl1 and Notch1 levels in a time-dependent pattern and this expressional suppression was independent of TMZ. These findings collectively demonstrate the critical role of FBW7 as a prognostic factor and a potential target to overcome chemoresistance of glioblastoma.

This article is protected by copyright. All rights reserved.

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Comprehensive review of genetic factors contributing to head and neck squamous cell carcinoma development in low-risk, nontraditional patients

Abstract

Background

The past 2 decades have seen an increased incidence of head and neck squamous cell carcinoma (HNSCC) in a nontraditional, low-risk patient population (ie, ≤45 years of age, no substance use history), owing to a combination of human papillomavirus (HPV) infection and individual genetic variation.

Methods

Articles positing genetic variants as contributing factors in HNSCC incidence in low-risk, nontraditional patients were identified using a PubMed search, reviewed in detail, and concisely summarized herein.

Results

Recent data suggest that common polymorphisms in DNA repair enzymes, cell-cycle control proteins, apoptotic pathway members, and Fanconi anemia-associated genes likely modulate susceptibility to HNSCC development in low-risk, nontraditional patients.

Conclusion

At present, there is a lack of robust, comprehensive data on genetic drivers of oncogenesis in low-risk patients and a clear need for further research on genetic alterations underlying the rising incidence of HNSCC in low-risk, nontraditional patients.

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A case of a primary hepatic so-called adenosarcoma with heterotopic ossification: possibly of biliary adenofibroma origin

Publication date: March 2018
Source:Human Pathology, Volume 73
Author(s): Shiori Meguro, Satoru Yamazaki, Sayomi Matsushima, Kazuhito Kawata, Hideya Kawasaki, Takashi Tsuchida, Isao Kosugi, Yoshimasa Kobayashi, Satoshi Baba, Toshihide Iwashita
We present an autopsy case of a "so-called adenosarcoma with ossification of the liver" in a 63-year-old man. Macroscopically, the well-circumscribed tumor with portal vein invasion was observed in the right lobe of the liver. The cut surface of the tumor had a solid and microcystic appearance. Microscopically, the tumor was characterized by a benign epithelial component and a malignant mesenchymal component. We believe the presence of biliary adenofibroma–like areas and the von Meyenburg complexes suggests that the tumor is possibly associated with a biliary adenofibroma. In addition, the present tumor was unique in that it showed scattered heterotopic ossification. Immunohistochemical study showed that the mesenchymal atypical spindle cells had characteristics of undifferentiated mesenchymal cells. This is the first report of a primary hepatic so-called adenosarcoma.



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