Head and Neck Medicine by Alexandros G.Sfakianakis: 08/30/17

Τετάρτη 30 Αυγούστου 2017

Clinicopathologic and immunophenotypic features of eosinophilic fasciitis and morphea profunda: A comparative study of 27 cases

Eosinophilic fasciitis (EF) and morphea profunda (MP) are inflammatory and sclerosing disorders of the subcutis that can exhibit clinical and pathologic presentations that overlap.

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Managing sharps injuries and other occupational exposures to HIV, HBV, and HCV in the dermatology office

Dermatologists and their staff are at risk for needlestick injuries and exposures to body fluids. Despite the availability of treatment to reduce the risk of blood-borne infection, many exposures go unreported. This paper identifies current recommendations and the specific details for response to occupational exposures to HIV, hepatitis B virus, and hepatitis C virus in the dermatology office. Issues surrounding each virus are discussed individually, and a summary step-by-step algorithm of how to proceed in the event of an occupational exposure is presented.

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Anaesthetic depth control using closed loop anaesthesia delivery system vs. target controlled infusion in patients with moderate to severe left ventricular systolic dysfunction

To compare the efficacy of anaesthetic depth control using Closed Loop Anaesthesia Delivery System (CLADS) and Target Controlled Infusion (TCI) in patients with moderate to severe left ventricular dysfunction (LVSD).

http://ift.tt/2wTQ2cq

Determination of ochratoxin A in tissues of wild boar ( Sus scrofa L.) by enzymatic digestion (ED) coupled to high-performance liquid chromatography with a fluorescence detector (HPLC-FLD)

Abstract

Ochratoxin A (OTA) is a secondary toxic metabolite synthesized by Aspergillus or Penicillium species, which can contaminate various crops. The International Agency for Research on Cancer (IARC) classified OTA as a group 2B possible human carcinogen. The aim of the present study was to assess OTA concentrations in tissues of wild boar (Sus scrofa L.) from Tuscany (Italy). Over a period of 2 years, samples of muscle, liver, and kidney from 48 wild boars were collected and concentrations of OTA were determined by enzymatic digestion (ED) coupled to high-performance liquid chromatography with a fluorescence detector (HPLC-FLD). The highest concentrations of OTA were found in the kidneys of the 48 wild boars analyzed. No difference in concentrations was found based on years of collection and sex while a significantly higher OTA concentration was found in the kidney of the young wild boars with respect to the adult one. Monitoring the quality of meat destined for transformation is a priority in order to decrease the possibility of toxin carry-over to humans. The present study showed that contamination of wild boar meat products by OTA represents a potential emerging source of OTA.

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Evaluation of periprocedural hypersensitivity reactions

Identifying the cause of periprocedural hypersensitivity reactions (HSRs) remains challenging because of the multitude of medications involved. Antibiotics are the most common cause in the United States, whereas neuromuscular blocking agents are most common in Europe.

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Unique heterozygous presentation in an infant with DNA ligase IV syndrome

DNA ligase IV deficiency, or ligase 4 (LIG4) syndrome, is a rare autosomal recessive disorder associated with impaired DNA damage-repair mechanisms, resulting in different clinical presentations. The repair of DNA double-stranded breaks through the nonhomologous end-joining pathway is essential in the early stages of B- and T-cell differentiation as they undergo recombination of variable, diverse, and joining gene segments to generate antigen-specific cell receptors.1 Defects in this pathway, as seen in ligase IV deficiency, lead to an extreme form of severe combined immunodeficiency disease with a lymphocyte phenotype described as T-cell negative, B-cell negative, and natural killer cell positive (T−B−NK+).

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Parental perception of anxiety in children with eosinophilic esophagitis in a tertiary care center

Eosinophilic esophagitis (EoE) is a chronic, immune-mediated, atopic disease characterized by eosinophil infiltration into the esophageal epithelium, resulting in fibrosis and dysfunction.1 During the past 20 years, its prevalence has increased exponentially in children in Western countries up to 50.5 in 10,000, which is similar to that of pediatric inflammatory bowel disease.1 The pediatric population presents with a broad spectrum of symptoms that vary with age. These, in ascending order of age, include feeding difficulty, failure to thrive, acid reflux, vomiting, abdominal pain, dysphagia, and food impaction; in contrast, adults primarily present with dysphagia and food impaction.

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Filaggrin mutations and Molluscum contagiosum skin infection in patients with atopic dermatitis

Although mutations in the filaggrin (FLG) gene have been reported to predispose patients with atopic dermatitis (AD) skin infection susceptibility, to date, the data reported in the literature are still controversial.

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Management around invasive procedures in mastocytosis

Mastocytosis is a chronic hematologic disorder that is characterized by the accumulation of aberrant mast cells and typically involves the skin and/or bone marrow. Patients with mastocytosis are at increased risk of anaphylaxis. Based on theoretical assumptions, medical procedures requiring general anesthesia or radiocontrast media are deemed hazardous for patients with mastocytosis. The objective of this article is to provide a comprehensive overview of the actual risk of iatrogenic anaphylaxis and provide recommendations for daily practice.

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Identification and validation of asthma phenotypes in Chinese population using cluster analysis

Asthma is a heterogeneous airway disease, so it is crucial to clearly identify clinical phenotypes to achieve better asthma management.

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Eosinophil-derived neurotoxin as a biomarker for disease severity and relapse in recalcitrant atopic dermatitis

Eosinophils are encountered in many skin diseases, but the role of eosinophils in atopic dermatitis (AD) remains uncertain.

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Clinical and biological response to rituximab treatment in 3 patients with acquired C1-inhibitor deficiency

Acquired C1 inhibitor (C1-INH) deficiency (ACID) is a rare cause of recurrent subcutaneous and submucosal edema without urticaria. The mechanisms of ACID include excessive C1-INH consumption because of an underlying lymphoid malignant tumors (often undiagnosed) or the presence of anti–C1-INH autoantibodies, often with an isolated monoclonal gammopathy or a lymphoproliferative disorder.1–3 The primary therapy for ACID is treatment of the underlying condition; however, management is complex, and all patients must have a medication for acute angioedema.

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On ciprofloxacin concentration in chronic rhinosinusitis

Publication date: Available online 30 August 2017
Source:Acta Otorrinolaringológica Española
Author(s): José Gameiro dos Santos, Rosário Figueirinhas, José P. Liberal, João C. Almeida, Joana Sousa, Amílcar Falcão, Corália Vicente, João Paço, Cecília A. Sousa
ObjectiveConsidering that all the evidence indicates that chronic rhinosinusitis without nasal polyps (CRSsNP) and chronic rhinosinusitis with nasal polyps (CRSwNP) are distinct entities, the aim of this study was to compare the concentrations obtained in plasma and in sinonasal mucosa with oral and nasal topical ciprofloxacin, in patients with and without nasal polyps, without evaluating the effectiveness of the use of an antibiotic.MethodsProspective clinical study with single-blind randomization. The population consisted of patients with chronic rhinosinusitis with eligible for endonasal surgery, over 18 years old. It took place between January 2010 and December 2014. A single preoperative dose of ciprofloxacin (oral or nasal topic- spray, gel or drops) was given and samples of plasma and nasal mucosa (inferior turbinate, middle turbinate, ethmoid and maxillary sinus) were collected prior to surgery. The plasma and mucosal ciprofloxacin concentrations were assayed with high performance liquid chromatography (HPLC) with fluorescence detection (FD).ResultsThe oral ciprofloxacin achieved better mucosal concentrations but had a significant plasmatic expression in all patients. None of the topical formulations achieved measurable ciprofloxacin plasmatic levels. Among the topical formulations, the gel had the best mucosal results, despite the existence of polyposis.

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Encuesta sobre el conocimiento en la indicación de implantes cocleares para el tratamiento de la hipoacusia en España

Publication date: Available online 30 August 2017
Source:Acta Otorrinolaringológica Española
Author(s): Manuel Manrique, Ángel Ramos, Beatriz Pradel, Carlos Cenjor, Diego Calavia, Constantino Morera
IntroducciónEste trabajo pretende analizar el conocimiento del especialista en Otorrinolaringología en España sobre los criterios de indicación de los implantes cocleares (IC) y, según los resultados, considerar si es necesario implementar medidas de formación dirigidas a mejorar el conocimiento en esta área.Material y métodosSe diseña un cuestionario dirigido a conocer el nivel de conocimiento sobre la indicación de IC en personas con hipoacusia. Este cuestionario recogía información demográfica de los encuestados y conocimientos sobre aspectos como indicaciones convencionales y emergentes de los IC, características técnicas de los IC y resultados en la población implantada.ResultadosUn total de 222 especialistas españoles en Otorrinolaringología respondieron el cuestionario (10,29% de la muestra encuestada).ConclusionesUn 50% de los encuestados mostraron unos conocimientos medios-altos sobre IC. Datos epidemiológicos sugieren que un alto porcentaje de adultos con sorderas poslocutivas candidatos a un IC no son referidos para tratamiento. El desconocimiento de los criterios para la indicación de IC por parte de los especialistas en Otorrinolaringología puede contribuir a una inadecuada orientación de los pacientes potencialmente candidatos a un IC. Las mayores deficiencias se encontraron en las indicaciones más emergentes de un IC. Dentro de los profesionales de la Otorrinolaringología los mayores conocimientos sobre IC se concentraron en aquellos que trabajaban en hospitales terciarios, especializados en Otología y Otoneurología, y desempeñaban su actividad laboral en el sector público y privado. Este estudio sugiere la conveniencia de incrementar acciones formativas sobre IC en los profesionales de la Otorrinolaringología, especialmente para otorrinolaringólogos generales.IntroductionThis study aims to analyse the knowledge of cochlear implant (CI) candidacy criteria of otorhinolaryngology specialists in Spain, and from the results, consider whether it is necessary to implement training measures aimed at improving knowledge in this area.Material and methodsA questionnaire was designed for measuring the level of knowledge of cochlear implant candidacy criteria (CI) in people with hearing loss. The questionnaire collected the demographic data of the respondents and their knowledge on the conventional and emergency indications for CI, technical characteristics of CI and results in the implanted population.ResultsA total of 222 Spanish specialists in otorhinolaryngology answered the questionnaire (10.29% of the sample surveyed).ConclusionsThe 50% of all respondents showed a medium-high knowledge about CI. Epidemiological data suggest that a high percentage of adults with postlocutive deafness and candidates for a CI are not referred for treatment. The lack of knowledge about the criteria for the indication of CI by otorhinolaryngology specialists may contribute to inadequate guidance of patients who are potential candidates for CI. The greatest shortcomings are found in the most emergency indications for a CI. Among otorhinolaryngology professionals, the greatest knowledge about CI is found in those who work in tertiary hospitals in the areas of otology and otoneurology, either in the public or private sector. This study suggests that training on CI should be increased for otorhinolaryngology professionals, especially for general otorhinolaryngologists.

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Maxillary hydatid cyst

Publication date: Available online 30 August 2017
Source:Acta Otorrinolaringológica Española
Author(s): Satvinder Singh Bakshi, Roopa Urs

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Toll-like receptor 5 and 7 expression may impact prognosis of HPV-positive oropharyngeal squamous cell carcinoma patients

Abstract

A large subset of oropharyngeal squamous cell carcinomas (OPSCCs) is associated with HPV infection and has better outcome than non-viral-related tumors. Various malignancies also carry a role for TLRs, key activators of inflammation and innate immunity. We examined the expression of TLRs in OPSCC, and their association with HPV status and treatment outcome. TLR 5, 7, 9, and p16 were studied by immunohistochemistry and HPV status was detected with in situ hybridization in 202 tumors of consecutively treated OPSCC patients using tissue microarray method. The relations between TLR expression and HPV status, p16 expression, clinicopathological factors, and survival were analyzed. TLR 5, 7, and 9 expression patterns differed between HPV-positive and -negative tumors, and they were statistically significantly associated with history of smoking, heavy drinking, tumor site, grade, size (T), metastasis (N), and stage. Moreover, in HPV-positive tumors the expression of TLR 5 and 7 correlated with tumor recurrence. After adjustment, among HPV-positive OPSCC patients, high TLR 5 and low TLR 7 expression were associated with poor disease-specific survival. Our results indicate that TLR 5 and 7 may have a role in the prognostication of HPV-positive OPSCC, however, further studies are needed to clarify the comprehensive role of these TLRs in OPSCC.

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Demographic and clinicopathological characteristics of nasopharyngeal carcinoma and survival outcomes according to age at diagnosis: A population-based analysis

Publication date: October 2017
Source:Oral Oncology, Volume 73
Author(s): San-Gang Wu, Xu-Lin Liao, Zhen-Yu He, Li-Ying Tang, Xue-Ting Chen, Yan Wang, Qin Lin
ObjectiveTo investigate the demographic features, clinicopathological characteristics and treatment outcomes of patients with nasopharyngeal carcinoma (NPC) according to age at diagnosis.MethodsWe assessed demographic and clinicopathological variables extracted from the Surveillance, Epidemiology, and End Results database (2004–2014). The Cox proportional hazards regression model was used to perform univariate and multivariate analyses of NPC-related mortality (cause-specific survival).ResultsA total of 3880 patients were analyzed. Median age was 55-years-old; 108 (2.8%), 508 (13.1%), 1876 (48.4%), 1240 (32.0%), and 148 (3.8%) patients were aged 1–19, 20–39, 40–59, 60–79, and 80–99-years-old, respectively. Younger patients tended to be black and present with poorly/undifferentiated disease and advanced tumor and nodal category compared to older patients. Younger patients were more likely to receive chemotherapy than older patients. In multivariate analyses, age at diagnosis was an independent prognostic factor for cause-specific survival. Increasing age at diagnosis was associated with a significantly higher risk of NPC-related mortality. Compared to patients aged 1–19-years-old, the hazard ratios for patients aged 20–39, 40–59, 60–79, and 80–99-years-old were 2.030 (95% confidence interval 1.004–4.104), 2.871 (1.474–5.590), 4.443 (2.273–8.683), and 12.024 (5.855–24.695), respectively. With the exception of black patients (P=0.100), older age was associated with poor survival in all demographic and clinical subgroups.ConclusionAlthough younger patients tended to have advanced disease at diagnosis, older age at diagnosis was associated with a higher risk of NPC-related mortality.

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Epigenetic therapy regulates the expression of ALDH1 and immunologic response: Relevance to the prognosis of oral cancer

Publication date: October 2017
Source:Oral Oncology, Volume 73
Author(s): Ming-Shao Tsai, Wen-Cheng Chen, Chia-Hsuan Lai, Yu-Yen Chen, Miao-Fen Chen
ObjectivesAldehyde dehydrogenase 1 (ALDH1) is associated with tumorigenesis, and shown to identify cancer stem cells (CSC)-like cells. We aimed to investigate the significance of ALDH1 in oral squamous cell carcinoma (OSCC) and its correlation with DNMT3b and immune evasion in the present study.MethodsWe retrospectively analyzed the clinical outcomes of OSCC patients and examined its correlation with the levels of ALDH1 in tumors and circulating myeloid-derived suppressor cells (MDSCs) in the peripheral blood. Furthermore, the relationships between the DNMT3b, ALDH1 expression, and immune response were examined via clinical specimens and cellular and animal experiments. We also investigated the therapeutic potential of DNA hypomethylating agents in OSCC.ResultsOur data revealed that the levels of ALDH1 expression were linked to treatment resistance, CSC-like properties, higher circulating MDSC and poor prognosis for OSCC. The radiation resistance noted in ALDH1-positive tumors was associated with augmented radiation-induced increases in the expression of programmed death ligand (PD-L1) and the activation of MDSCs. Furthermore, there was a positive link between ALDH1 and DNMT3b expression shown by clinical specimens and cellular experiments. DNA hypomethylating agents attenuated the radioresistance of ALDH1-positive cancer cells associated with the decreased ALDH1 and the increased DNA damages. In addition, the activation of MDSCs and the expression of PD-L1 were significantly attenuated by epigenetic therapy.ConclusionsOur findings suggested that ALDH1 played an important role in treatment response and the tumor-promoting microenvironment in OSCC. Moreover, epigenetic therapy could be a promising strategy for the treatment of OSCC.

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Incidence and dosimetric parameters for brainstem necrosis following intensity modulated radiation therapy in nasopharyngeal carcinoma

Publication date: October 2017
Source:Oral Oncology, Volume 73
Author(s): Yang-Chan Li, Fo-Ping Chen, Guan-Qun Zhou, Jin-Han Zhu, Jiang Hu, De-Hua Kang, Chen-Fei Wu, Li Lin, Xiao-Ju Wang, Jun Ma, Ying Sun
ObjectivesTo clarify the incidence of brainstem toxicity and perform a dose-volume analysis for the brainstem after long-term follow-up of a large cohort of nasopharyngeal carcinoma (NPC) patients who underwent intensity-modulated radiation therapy (IMRT).Materials and methodsAll patients with NPC treated with IMRT at Sun Yat-sen University Cancer Center between April 2009 and March 2012 were retrospectively reviewed. A total of 1544 patients with follow-up >12months and detailed treatment plan data were included. Radiotherapy was administered using the simultaneous integrated boost technique in 2.0–2.48Gy per fractions/28–33 fractions. Brainstem necrosis was defined as lesions with high signal intensity on T2-weighted images and low signal intensity on T1-weighted images, with or without enhancement after administration of contrast in follow-up MRI.ResultsAfter median follow-up of 79.7months (range, 12.2–85.6months), 2/1544 (0.13%) patients developed brainstem necrosis after intervals of 12.3 and 18.5months. Actuarial incidence of brainstem necrosis was 0.07%, 0.13%, 0.13% and 0.13% after 1, 2, 3 and 5years, respectively. Overall, 384 (24.9%), 153 (9.9%), 67 (4.3%), 39 (2.5%), 78 (5.1%), and 114 (7.4%) patients had excessive doses of Dmax≥64Gy, D1cc>59Gy, D2cc>59Gy, aV50>5.9cc, aV55>2.7cc and aV60>0.9cc respectively, of whom only two developed brainstem necrosis.ConclusionsBrainstem necrosis is rare in NPC. The definitive criteria based on conventional radiotherapy cannot accurately predict the occurrence of brainstem necrosis after IMRT, thus more flexible definitive criteria with strict restrictions need to be defined.

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Pneumocystis Pneumonia in Patients With Autoimmune Blistering Diseases

This analysis assesses the incidence of Pneumocystis pneumonia in patients with autoimmune blistering diseases receiving no routine prophylaxis.

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Canakinumab for Severe Hidradenitis Suppurativa

This case report describes the use of canakinumab to treat 2 patients with severe hidradenitis suppurativa.

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Incorrectly Reported Request for Reply

In the Letter to the Editor titled "Types of Shade Vary in Protection Just Like Sunscreens," published online on June 21, 2017, and commenting on the Original Investigation titled "Sun Protection by Beach Umbrella vs Sunscreen With a High Sun Protection Factor: A Randomized Clinical Trial" by Ou-Yang et al, it was incorrectly reported that Dr Ou-Yang, corresponding author of the Original Investigation, had been shown a copy of the letter and offered an opportunity to reply. Dr Ou-Yang had not seen the letter at that time nor received the request for reply. The Letter to the Editor has been corrected online.

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Utility of Microbiology in Suspected Dermatological Infections

This medical record review analyzes data from the Partners Healthcare registry to determine the value of microbiological studies in suspected skin tissue infections.

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Uniform Nomenclature to Describe Clinical Features of Pigmented Lesions

This Editorial describes how uniform terminology to describe features of malignancy facilitates communication among physicians, trainees, and disciplines.

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Laser Treatment of Café au Lait Macules Based on Morphologic Features

This case series compares the responses of café au lait lesions to laser treatment based on their morphologic characteristics, ie, ill-defined vs well-defined borders.

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FcεRI stimulation in BMMC and basophils induces HSP70 expression and its release. Extracellular HSP70 induces degranulation and mediator release via phosphorylation of LAT.

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Red Powder Used in Hindu Ceremonies Could Have Unsafe Lead Levels

Sindoor powder sold in the United States and India could have unsafe levels of lead, researchers warn.
Reuters Health Information

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Cost-of-illness of psoriasis - results of a German cross-sectional study

Abstract

Background

Though psoriasis poses a substantial chronic socio-economic burden, few studies have addressed the economic impact in Germany.

Objectives

The objective was to evaluate the annual costs of psoriasis in Germany from the societal perspective.

Methods

A cross-sectional study was performed in randomly selected German dermatology practices and clinics in 2013/2014 using standardised questionnaires of illness-related costs. Costs were grouped by perspective and category as well as analysed by sex and age. Group differences were tested by non-parametric tests.

Results

Complete data were obtained from 1,158 patients in 132 centres. Annual average costs for patients with psoriasis: Total costs € 5,543 ± € 8,044, systemic treatment costs (paid by the statutory health insurances (SHI)) € 3,733 ± € 7,322, out-of-pocket costs € 224 ± € 406, total SHI costs € 4,940 ± € 7,533, direct costs € 5,164 ± € 7,581 and indirect costs € 379 ± € 2,087. Significant higher costs in male and significant lower costs in 65+-year-old patients were found.

Conclusions

Psoriasis induces a considerable economic burden. Between 2003 and 2014, costs have markedly shifted from hospital, out-of-pocket and indirect costs towards systemic drug costs.

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Acanthoma planoepitheliale hyperkeratoticum, lesions associated with BRAF- inhibitor in metastatic melanoma

Abstract

A 68-year-old woman was diagnosed with BRAF V600 mutation positive melanoma of unknown primary (MUP) with metastasis to the lung, liver and brain. According to local standards dabrafenib monotherapy was started in July 2016. Control CT performed after two months revealed disease stabilization. The tolerance of therapy was good, but after 2 months of treatment with dabrafenib the patient developed disseminated skin lesions with extended seborrheic keratosis G2 (according to international common toxicity criteria CTC).

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CTLA-4/CD80 pathway regulates T cell infiltration into pancreatic cancer

Abstract

The ability of some tumors to exclude effector T cells represents a major challenge to immunotherapy. T cell exclusion is particularly evident in pancreatic ductal adenocarcinoma (PDAC), a disease where blockade of the immune checkpoint molecule CTLA-4 has not produced significant clinical activity. In PDAC, effector T cells are often scarce within tumor tissue and confined to peritumoral lymph nodes and lymphoid aggregates. We hypothesized that CTLA-4 blockade, despite a lack of clinical efficacy seen thus far in PDAC, might still alter T cell immunobiology, which would have therapeutic implications. Using clinically relevant genetic models of PDAC, we found that regulatory T cells (Tregs), which constitutively express CTLA-4, accumulate early during tumor development but are largely confined to peritumoral lymph nodes during disease progression. Tregs were observed to regulate CD4⁺, but not CD8⁺, T cell infiltration into tumors through a CTLA-4/CD80 dependent mechanism. Disrupting CTLA-4 interaction with CD80 was sufficient to induce CD4 T cell infiltration into tumors. These data have important implications for T cell immunotherapy in PDAC and demonstrate a novel role for CTLA-4/CD80 interactions in regulating T cell exclusion. In addition, our findings suggest distinct mechanisms govern CD4⁺ and CD8⁺ T cell infiltration in PDAC.

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A Study of Apatinib and Tegafur Gimeracil Oteracil in Locally Advanced Squamous Cell Carcinoma of the Head and Neck

Condition: Head and Neck Squamous Cell Carcinoma
Interventions: Drug: Apatinib Mesylate Tablets; Drug: Tegafur Gimeracil Oteracil Potassium Capsules
Sponsor: Shanghai Ninth People's Hospital Affiliated to Shanghai Jiao Tong University
Not yet recruiting - verified August 2017

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Assessing Impact of CCO's PSO & PC Pathway in Ambulatory HNC Clinics

Conditions: Head and Neck Cancer; Psychosocial Care; Supportive Care; Psychosocial Intervention
Intervention: Other: PSOPC pathway approach
Sponsor: Sunnybrook Health Sciences Centre
Not yet recruiting - verified August 2017

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Nivolumab + Chemoradiation in Stage II-IVB Nasopharyngeal Carcinoma

Condition: Nasopharyngeal Carcinoma
Interventions: Drug: Nivolumab; Drug: Cisplatin; Radiation: Radiation Therapy
Sponsors: University of California, San Francisco; National University Cancer Institute, Singapore; Bristol-Myers Squibb
Not yet recruiting - verified August 2017

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Dural ectasia associated with Mounier-Kuhn syndrome: a novel association in the context of spinal stenosis

We present the case of a patient undergoing lumbar spine decompression for stenosis with a history of Mounier-Kuhn syndrome. The patient presented with axial lumbar spine pain over 6 months with progressive radicular pain to the left L3 dermatome. MRI confirmed spinal stenosis at L3/4 level with associated dural ectasia. The patient had an uneventful spinal decompression with resolution of radicular symptoms and axial spine pain. Dural ectasia poses a significant risk when operating on the lumbosacral spine. Larger cerebrospinal fluid volumes and a capacious dural canal can result in anaesthetic and orthopaedic complications such as inadequate spinal anaesthesia, complicated epidural analgesia, intraoperative dural tears and difficult pedicle screw insertion due to narrow pedicles. This is the first case in the literature detailing the association between dural ectasia and Mounier-Kuhn syndrome. We recommend adequate spinal imaging in patients with Mounier-Kuhn syndrome to exclude dural ectasia prior to undergoing lumbosacral spinal procedures.

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Decreased severity of Shiga toxin-producing Escherichia coli haemolytic uraemic syndrome (STEC-HUS) in a child with type 1 von Willebrand disease

Shiga toxin-producing Escherichia coli-associated haemolytic uraemic syndrome (STEC-HUS) is characterised by haemolytic anaemia, thrombocytopenia and acute kidney injury. Von Willebrand Factor (vWF) is an important mediator of normal thrombi formation and indirect evidence suggests that vWF may play an important role in Shiga toxin-induced thrombi formation. Clinical evidence supporting the role of vWF in STEC-HUS is lacking. A 10-year-old girl with type 1 von Willebrand Disease (vWD) had a mild case of STEC-HUS, with nadir haemoglobin 7.3 g/dL and platelet count 105x10⁹ cells/L and peak serum creatinine 0.56 mg/L and lactate dehydrogenase 741 U/L. This is the first report of STEC-HUS in a patient with vWD. We speculate that the quantitative deficiency of vWF associated with type 1 vWD may have attenuated the course of disease by reducing platelet aggregation, complement activation and thrombi formation. This case adds to a growing literature supporting a link between vWF and STEC-HUS.

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Primary mediastinal seminoma presenting with superior vena caval obstruction

Description

A previously well 29-year-old man presented with a 2-week history of progressive orthopnoea, dry cough, neck and facial swelling, and night sweats. On further questioning, he described 2 kg of weight loss and increasing swallowing difficulties over the previous year. An oesophagogastroduodenoscopy performed 1 year prior was unremarkable. A chest radiograph demonstrated a large mediastinal mass (figure 1A), and a subsequent CT scan revealed a large anterior mediastinal mass measuring 11 cm in diameter. There was central necrosis and complete occlusion of the superior vena cava (SVC; figure 1C,D). An urgent biopsy of the mass was performed, and immunohistochemistry showed malignant cells positive for OCT3/4, placental alkaline phosphatase, D2-40 and CD117 but negative for pancytokeratin, S100, CD30, human chorionic gonadotropin (HCG), alpha-foetoprotein (AFP), CD45, CD20 and CD3. There were no masses found elsewhere on CT imaging of the chest, abdomen, pelvis and head, or on testicular ultrasonography....

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Duplex gall bladder: bystander or culprit

Gall bladder (GB) duplication is a rare anatomical malformation, which can be detected by preoperative imaging study. We present a case of duplex gall bladder in a 14-year-old boy who presented with abdominal pain. On ultrasound, he had right nephrolithiasis and duplex gall bladder. Duplex gall bladder was confirmed on MR cholangiopancreatography. There was a dilemma for surgical management of duplex gall bladder; however, he became asymptomatic after conservative treatment. Prophylactic surgery is not recommended for asymptomatic incidentally detected duplex gall bladder. Radiologists and paediatric surgeons should be sensitised about the exact anatomy of this entity.

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Food challenges, the risks and benefits

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Issue Information

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Applying prevention concepts to anaphylaxis: A call for worldwide availability of adrenaline auto-injectors

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Forthcoming Meetings

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Cover Image

The cover image, by P. Brinkman et al., is based on the Original Article Exhaled breath profiles in the monitoring of loss of control and clinical recovery in asthma, DOI: 10.1111/cea.12965.

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Cirtical role for Salmonella effector SopB in regulating inflammasome activation

Publication date: October 2017
Source:Molecular Immunology, Volume 90
Author(s): Gui-Qiu Hu, Pei-Xuan Song, Wei Chen, Shuai Qi, Shui-Xing Yu, Chong-Tao Du, Xu-Ming Deng, Hong-Sheng Ouyang, Yong-Jun Yang
ObjectiveSalmonella is known to evolve many mechanisms to avoid or delay inflammasome activation which remain largely unknown. In this study, we investigated whether the SopB protein critical to bacteria virulence capacity was an effector that involved in the regulation of inflammasome activation.MethodsBMDMs from NLRC4-, NLRP3-, caspase-1/-11-, IFI16- and AIM2-deficient mice were pretreated with LPS, and subsequently stimulated with a series of SopB-related strains of Salmonella, inflammasome induced cell death, IL-1β secretion, cleaved caspase-1 production and ASC speckle formation were detected.ResultsWe found that SopB could inhibit host IL-1β secretion, caspase-1 activation and inflammasome induced cell death using a series of SopB-related strains of Salmonella; however the reduction of IL-1β secretion was not dependent on sensor that contain PYD domain, such as NLRP3, AIM2 or IFI16, but dependent on NLRC4. Notably, SopB specifically prevented ASC oligomerization and the enzymatic activity of SopB was responsible for the inflammasome inhibition. Furthermore, inhibition of Akt signaling induced enhanced inflammasome activation.ConclusionsThese results revealed a novel role in inhibition of NLRC4 inflammasome for Salmonella effector SopB.

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Interaction of Zap70 and CXCR4 receptor at lamellipodia that determines the directionality during Jurkat T cells chemotaxis

Publication date: October 2017
Source:Molecular Immunology, Volume 90
Author(s): Chia-Yun Wu, Yi-Ying Tsai, Sheng-Yi Chen, Yu-Ping Lin, Jyh-Wei Shin, Chia-Ching Wu, Bei-Chang Yang
Directional migration of T-lymphocytes is a key process during immune activation and is tightly regulated both temporally and spatially. The initial cell membrane protrusion at a particular site is critical for determining the direction of cell migration. In this study, we found that ZAP-70 protein appeared not only at the margin of the spreading areas of polarized Jurkat T cells but also formed clusters near the center of the cell body on a fibronectin plate. Specifically, some pZAP-70 was located at the lamellipodia/filopodia and was closely associated with the most extended membrane contact. To visualize the dynamic distribution of ZAP-70 on migrating Jurkat T cells, we generated a fluorescent ZAP-70-EGFP fusion protein (hZAP70G). Expression of the hZAP70G in P116 cells, a ZAP-70 defective Jurkat derivative, restored its chemotactic migration toward SDF-1, adhesion to fibronectin matrix, and integrin activation. In addition, the distribution of hZAP70G protein is associated with changes in cell shape, specifically the membrane protrusion step, forming filopodia/lamellipodia and a retracting uropod. Furthermore, SDF-1 stimulated the formation of ZAP-70 and CXCR4 complex. CXCR4 was observed mainly at the leading edge of migrating cell. The localization of ZAP-70 at the very front edge of protruding lamellipodia was close to CXCR4 and a part of them were overlapped. Collectively, our data describe the critical early step of directional cell movement toward SDF-1 that ZAP-70 is recruited to the CXCR4 at the leading edge of membrane and consequently modulates lamellipodia/filopodia formation and integrin activation.

http://ift.tt/2vJDS0K

Role of neuropilin-2 in the immune system

Publication date: October 2017
Source:Molecular Immunology, Volume 90
Author(s): S. Schellenburg, A. Schulz, D.M. Poitz, M.H. Muders
Neuropilins (NRPs) are single transmembrane receptors with short cytoplasmic tails and are dependent on receptors like VEGF receptors or Plexins for signal transduction. NRPs are known to be important in angiogenesis, lymphangiogenesis, and axon guidance. The Neuropilin-family consists of two members, Neuropilin-1 (NRP1) and Neuropilin-2 (NRP2). They are up to 44 % homologous and conserved in all vertebrates. High levels of NRP2 are found on immune cells. Current research is very limited regarding the functions of NRP2 on these cells. Recent evidence suggests that NRP2 is important for migration, antigen presentation, phagocytosis and cell–cell contact within the immune system. Additionally, posttranslational NRP2 modifications like polysialylation are crucial for the function of some immune cells. This review is an overview about expression and functions of NRP2 in the immune system.

http://ift.tt/2vrUzT9

The circular RNA of peripheral blood mononuclear cells: Hsa_circ_0005836 as a new diagnostic biomarker and therapeutic target of active pulmonary tuberculosis

Publication date: October 2017
Source:Molecular Immunology, Volume 90
Author(s): Ze-Gang Zhuang, Jun-Ai Zhang, Hou-Long Luo, Gan-Bin Liu, Yuan-Bin Lu, Nan-Hai Ge, Bi-Ying Zheng, Rui Xi Li, Chen Chen, Xin Wang, Yu-Qing Liu, Feng-Hui Liu, Yong Zhou, Xiao-Zhen Cai, Zheng W. Chen, Jun-Fa Xu
It has been reported that circular RNA (circRNA) is associated with human cancer. However, few studies have been reported in active pulmonary tuberculosis (APTB). The global circRNA expression was detected in the peripheral blood mononuclear cells (PBMCs) of APTB patients (n=5) and health controls (HC) (n=5) by using high-throughput sequencing. According to the systematical bioinformatics analysis, the basic content of circRNAs and their fold changes in the two groups were calculated. We selected 6 significant differentially expressed circRNAs, hsa_circ_0005836, hsa_circ_0009128, hsa_circ_0003519, hsa_circ_0023956, hsa_circ_0078768, and hsa_circ_0088452 and validated the expression in PBMCs from APTB (n=10) and HC (n=10) by real-time quantitative reverse transcription-polymerase chain reactions (qRT-PCRs). Further, the verification of these specific circRNAs (hsa_circ_0005836 and hsa_circ_0009128) between APTB (n=34) and HC (n=30) in PBMCs was also conducted by qRT-PCRs. The RNA-seq data showed the significant differential expression of the 523 circRNAs between the APTB and HC groups (199 circRNAs were significantly up-regulated and 324 circRNAs were down-regulated). Hsa_circ_0005836 and hsa_circ_0009128 expression was significantly down-regulated in the PBMCs of APTB (P<0.05) in the samples of APTB compared to HC in our study. The gene ontology based enrichment analysis of the circRNA-miRNA-mRNAs network showed that cellular catabolic process (P=7.10E-08), regulation of metabolic process (P=2.10E-06), catalytic activity (P=3.67E-08), protein binding (P=1.71E-07), cell part (P=3.46E-06), intracellular part (P=1.71E-07), and intracellular (P=3.67E-08) were recognized in the comparisons between APTB and HC. Based on KEGG analysis, HTLV-I infection, regulation of actin cytoskeleton, neurotrophin signaling pathway and mTOR signaling pathway were relevant during tuberculosis bacillus infection. We found for the first time that hsa_circ_0005836 and hsa_circ_0009128 were significantly down-regulated in the PBMCs of APTB compared with HC. Our findings indicate hsa_circ_0005836 might serve as a novel potential biomarker for TB infection.

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Elevated intrathymic sphingosine-1-phosphate promotes thymus involution during sepsis

Publication date: October 2017
Source:Molecular Immunology, Volume 90
Author(s): Laura Kuchler, Lisa K. Sha, Annika K. Giegerich, Tilo Knape, Carlo Angioni, Nerea Ferreirós, Martina V. Schmidt, Andreas Weigert, Bernhard Brüne, Andreas von Knethen
Sepsis mouse models revealed thymus atrophy, characterised by decreased thymus weight and loss of thymocytes due to apoptosis. Mice suffered from lymphopenia, a lack of T cells in the periphery, which attenuates their ability to fight against recurring and secondary infections during sepsis progression. Key players in thymus atrophy are IL-6, which is directly involved in thymus involution, and the sphingosine-1-phosphate − sphingosine-1-phosphate receptor 1 signaling, influencing thymocytes emigration. In healthy individuals a sphingosine-1-phosphate (S1P) gradient from lymphoid organs to the circulatory system serves as signal for mature T cell egress. In the present study we investigated, whether inhibition of S1P generation improves thymus involution. In sepsis, induced by cecal ligation and puncture (CLP), S1P in the thymus increased, while it decreased in serum, thus disrupting the naturally occurring S1P gradient. As a potential source of S1P we identified increased numbers of apoptotic cells in the thymic cortex of septic mice. Pharmacological inhibition of the S1P generating sphingosine kinases, by 4- [[4-(4-Chlorophenyl)-2-thiazolyl]amino]phenol (SK I–II), administered directly following CLP, prevented thymus atrophy. This was reflected by lymphocytosis, diminished apoptosis, decreased IL-6 expression, and an unaltered thymus weight. In addition SK I-II-treatment preserved the S1P balance and prevented S1P-dependent internalization of the sphingosine-1-phosphate receptor 1. Our data suggest that inhibition of sphingosine kinase and thus, S1P generation during sepsis restores thymic T cell egress, which might improve septic outcome.

http://ift.tt/2vrXlrn

Interaction of extremophilic archaeal viruses with human and mouse complement system and viral biodistribution in mice

Publication date: October 2017
Source:Molecular Immunology, Volume 90
Author(s): Linping Wu, Kristine Buch Uldahl, Fangfang Chen, Halli Benasutti, Deborah Logvinski, Vivian Vu, Nirmal K. Banda, Xu Peng, Dmitri Simberg, Seyed Moein Moghimi
Archaeal viruses offer exceptional biophysical properties for modification and exploration of their potential in bionanotechnology, bioengineering and nanotherapeutic developments. However, the interaction of archaeal viruses with elements of the innate immune system has not been explored, which is a necessary prerequisite if their potential for biomedical applications to be realized. Here we show complement activation through lectin (via direct binding of MBL/MASPs) and alternative pathways by two extremophilic archaeal viruses (Sulfolobus monocaudavirus 1 and Sulfolobus spindle-shaped virus 2) in human serum. We further show some differences in initiation of complement activation pathways between these viruses. Since, Sulfolobus monocaudavirus 1 was capable of directly triggering the alternative pathway, we also demonstrate that the complement regulator factor H has no affinity for the viral surface, but factor H deposition is purely C3-dependent. This suggests that unlike some virulent pathogens Sulfolobus monocaudavirus 1 does not acquire factor H for protection. Complement activation with Sulfolobus monocaudavirus 1 also proceeds in murine sera through MBL-A/C as well as factor D-dependent manner, but C3 deficiency has no overall effect on viral clearance by organs of the reticuloendothelial system on intravenous injection. However, splenic deposition was significantly higher in C3 knockout animals compared with the corresponding wild type mice. We discuss the potential application of these viruses in biomedicine in relation to their complement activating properties.

http://ift.tt/2vJ6EhT

Exogenous T3 toxicosis following consumption of a contaminated weight loss supplement

Summary

A 42-year-old male presented with a one-week history of palpitations and sweating episodes. The only significant history was of longstanding idiopathic dilated cardiomyopathy. Initial ECG demonstrated a sinus tachycardia. Thyroid function testing, undertaken as part of the diagnostic workup, revealed an un-measureable thyroid-stimulating hormone (TSH) and free thyroxine (T₄). Upon questioning the patient reported classical thyrotoxic symptoms over the preceding weeks. Given the persistence of symptoms free tri-iodothyronine (T₃) was measured and found to be markedly elevated at 48.9 pmol/L (normal range: 3.1–6.8 pmol/L). No goitre or nodular disease was palpable in the neck. Historically there had never been any amiodarone usage. Radionucleotide thyroid uptake imaging (¹²³I) demonstrated significantly reduced tracer uptake in the thyroid. Upon further questioning the patient reported purchasing a weight loss product online from India which supposedly contained sibutramine. He provided one of the tablets and laboratory analysis confirmed the presence of T₃ in the tablet. Full symptomatic resolution and normalised thyroid function ensued upon discontinuation of the supplement.

Learning points:

Free tri-iodothyronine (T₃) measurement may be useful in the presence of symptoms suggestive of thyrotoxicosis with discordant thyroid function tests.

Thyroid uptake scanning can be a useful aid to differentiating exogenous hormone exposure from endogenous hyperthyroidism.

Ingestion of thyroid hormone may be inadvertent in cases of exogenous thyrotoxicosis.

Local infiltration analgesia: a 2-year follow-up of patients undergoing total hip arthroplasty

Abstract

Purpose

Local infiltration analgesia (LIA) is commonly used for postoperative pain management following total hip arthroplasty (THA). However, the long-term effects of the component drugs are unclear. The aim of our study was to investigate functional outcome, quality of life, chronic post-surgical pain, and adverse events in patients within 2 years of undergoing THA.

Methods

The study was a secondary analysis of data from a previous larger study. Eighty patients were randomized to receive either intrathecal morphine (Group ITM) or local infiltration analgesia (Group LIA) for pain management in a double-blind study. The parameters measured were patient-assessed functional outcome [using the Hip dysfunction and Osteo-arthritis Outcome Score (HOOS) questionnaire], health-related quality of life [using the European Quality of Life–5 dimensions (EQ-5D) questionnaire and the 36-Item Short Form Health Survey (SF-36) score], and pain using the numeric rating score (NRS), with persistent post-surgical pain having a NRS of > 3 or a HOOS pain sub-score of > 30. All complications and adverse events were investigated during the first 2 years after primary surgery.

Results

Pain intensity and rescue analgesic consumption were similar between the groups after hospital discharge. No differences were found in HOOS or SF-36 score between the groups up to 6 months after surgery. A significant group × time interaction was seen in the EQ 5D form in favor of the LIA group. No between-group difference in persistent post-surgical pain was found at 3 or 6 months, or in adverse events up to 2 years after surgery.

Conclusion

Analysis of functional outcome, quality of life, and post-discharge surgical pain did not reveal significant differences between patients receiving LIA and those receiving ITM. LIA was found to be a safe technique for THA during the long-term follow-up. However, it should be noted that these conclusions are based on a limited number of patients.

http://ift.tt/2wIEwQL

Vocal fold leukoplakia: incidence, management, and prevention.

Purpose of review: The purpose of this article is to provide an update on English-language literature evaluating the current understanding of incidence, management, and prevention of vocal fold leukoplakia focusing on premalignancy. Recent findings: Recent studies have continued to try to elucidate factors influencing recurrence and progression of dysplastic disease, though results vary. Although advanced diagnostic techniques have attempted to predict disease behavior, tissue diagnosis continues to be essential. Studies reinforce the necessity of removal of disease as the primary treatment, whereas use of photoangiolytic lasers via the ablative technique has increased, as has transition of some patients into the office-based settings for treatment. Although genetic and molecular testing may hold promise for predictive purposes, further study is necessary, and chemotherapy for recalcitrant disease continues to be an area of study with few case studies being reported. Summary: Management of vocal fold leukoplakia continues to progress in terms of identification and treatment. Although many work to advance our knowledge in the field and push treatment strategies toward newer avenues, pathologic diagnosis, eradiation of disease on the basis of experience and skill, and close surveillance continue to be paramount. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

http://ift.tt/2xwQVom

Voice rest after laryngeal surgery: what's the evidence?.

Purpose of review: This article aims to review previous research reports and to summarize current strategies for the optimal duration of voice rest and the effect of phonatory stimulation after phonomicrosurgery. Recent findings: Voice rest is commonly recommended after laryngeal surgery to prevent worsening of vocal fold injuries. However, there are no established standard protocol for voice rest, and the type and duration of voice rest vary among clinicians. The most effective duration of voice rest is unknown. Recently, early vocal stimulation was recommended as a means to improve wound healing, on the basis of the basic and clinical researches. Summary: It seems that early vocal stimulation may enhance the wound healing process in the vocal fold. More basic and clinical researches are warranted to investigate appropriate timing of initiation of stimulation, as well as the type and amount of stimulation that are available for human. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

http://ift.tt/2whvI3g

Postmenopausal mild hirsutism and hyperandrogenemia due to granulosa cell tumor of the ovary: a case report

Among classes of ovarian tumor, granulosa cell tumors are the least common. In approximately 10% of cases of granulosa cell tumor, androgen will be secreted which will present with hirsutism and hyperandrogene...

http://ift.tt/2vrxvUI

Trichoscopy – a useful tool in the preliminary differential diagnosis of autoimmune bullous diseases

Abstract

Background

Scalp is a common location of autoimmune bullous diseases. Trichoscopy is a noninvasive method for diagnosing hair and scalp diseases. Data on trichoscopy in autoimmune bullous diseases are limited to the studies on pemphigus including a small number of patients. Trichoscopic characteristics of bullous pemphigoid and dermatitis herpetiformis were not reported to date. The aim of the study was to determine the value of trichoscopy in the differential diagnosis of pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, and dermatitis herpetiformis.

Methods

Trichoscopy was used to evaluate scalp lesions in 68 patients (26 with pemphigus vulgaris, 17 with pemphigus foliaceus, 17 with bullous pemphigoid, and 8 with dermatitis herpetiformis). The working magnification was 20-fold and 70-fold.

Results

The most frequent trichoscopic feature of autoimmune bullous diseases was extravasations. They occurred in 76.9% of patients with pemphigus vulgaris, 70.6% of patients with pemphigus foliaceus, 76.5% of patients with bullous pemphigoid, and 100% of patients with dermatitis herpetiformis. Yellow hemorrhagic crusts occurred in, respectively, 73.1%, 70.6%, 64.7%, and 35.5% of the cases. Yellow diffuse scaling and tubular scaling occurred more frequently in pemphigus foliaceus (52.9% and 41.2%, respectively). Clustered dotted vessels were characteristic for dermatitis herpetiformis (5/8, 62.5%). Dotted vessels with whitish halo were a hallmark of pemphigus vulgaris. A trichoscopic algorithm for the differential diagnosis of autoimmune bullous diseases was developed.

Conclusions

Autoimmune bullous diseases present characteristic trichoscopic patterns. Trichoscopy can be regarded as a rapid in-office preliminary diagnostic method in the differential diagnosis of these diseases.

http://ift.tt/2vqHLwe

The implications of pneumomediastinum and subcutaneous emphysema for the paediatric otolaryngologist

Publication date: October 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 101
Author(s): Chloe Swords, Neil Cheng-Wen Tan, Nico Jonas
Three patients presented within a 6-month period with pneumomediastinum. The underlying cause in each was distinct. One case occurred due to blunt laryngeal trauma and required urgent surgical intervention due to a decompensating airway. The second case was related to tracheal perforation secondary to a myofibroblastic tracheal tumour and the final case was related to adenovirus upper respiratory tract infection. Pneumomediastinum may be spontaneous or secondary to an underlying cause. Children should be managed using a multidisciplinary approach. Investigation and management should be influenced by clinical stability and invasive procedures should only be considered in patients who exhibit respiratory distress.

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Otologic manifestations of Larsen syndrome

Publication date: October 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 101
Author(s): Letícia Helena de Sousa Marques, Daniela Vieira Martins, Gabriel Liria Juares, Fábio Tadeu Moura Lorenzetti, Rafael da Costa Monsanto
ObjectiveTo describe and discuss otologic manifestations of Larsen syndrome, based on a case report and a systematic review of the literature.Materials and methodsWe performed a PubMED database search, and we selected studies reporting otolaryngologic manifestations secondary to Larsen syndrome. The selected articles were read in full, and three researchers independently extracted data from the studies. In parallel, we report the case of a 14-year-old patient who had hearing loss secondary to Larsen syndrome.ResultsFifteen studies met our selection criteria. Seven studies reported hearing loss in patients with Larsen syndrome (4 had conductive hearing loss and 3 had mixed hearing loss). The conductive hearing loss may be secondary to ossicular malformations and/or middle ear effusions. Other causes for conductive hearing loss are mesenchymal remnants in the middle ear, Eustachian tube dysfunction, and cleft palate. Surgical management of the hearing loss is possible in selected cases, although the surgical and anesthetic risks should be considered. Hearing aids seem to be safe and effective treatment options for the hearing loss secondary to Larsen syndrome.ConclusionAlthough rare, patients with Larsen syndrome may have hearing loss. The most frequent type of deficit is conductive, either secondary to malformation of the ossicles or middle ear effusion. Possible surgical correction of these abnormalities should be weighed against the anesthetic risks of these patients.

http://ift.tt/2vE6dGo

Anatomical measurement of the ossicles in patients with congenital aural atresia and stenosis

Publication date: October 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 101
Author(s): Jieying Li, Keguang Chen, Chenlong Li, Dongming Yin, Tianyu Zhang, Peidong Dai
ObjectivesOur aims were to measure and compare anatomical parameters of the ossicles in normal, congenital aural stenosis (CAS), and congenital aural atresia (CAA) ears.MethodsThis retrospective study was performed using three-dimensional reconstructed images derived from computed tomography scans of 20 normal subjects, 20 CAS patients, and 20 CAA patients.ResultsThe lengths of the malleus handle and long process of the incus were greater in normal ears than in CAS and CAA ears (all P < 0.05). The angles of the incudostapedial joint and between the short and long processes of the incus were smaller in normal ears than in CAS and CAA ears (all P < 0.05). There were no significant differences in the positions of the malleus head and incudomalleolar joint, the size of the malleus head, the length of the short process of the incus, or the angle of the incudomalleolar joint (P > 0.05).ConclusionsAnatomical parameters of the lower part, but not of the upper part, of the ossicular chain in CAS and CAA ears differed from those in normal ears. Different branchial arch origins of the upper and lower parts of the ossicular chain may explain these findings. Dysplasia of the second arch, which develops into the lower part of the ossicular chain, may contribute to ossicular malformation in CAA and CAS. Accurate radiographic measurement of malformed ossicles may be useful for reconstructive surgery of CAA and CAS using the patient's native ossicular chain and for choosing an appropriate place for active middle ear implants.

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Changes in tonal audiometry in children with progressive sensorineural hearing loss and history of Neonatal Intensive Care Unit discharge. A 20 year long-term follow-up

Publication date: October 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 101
Author(s): Carlos F. Martínez-Cruz, Adrián Poblano, Patricia García-Alonso Themann
ObjectiveNewborns from Neonatal intensive care units (NICU) are at high-risk for sensorineural hearing loss (SNHL) a follow-up is needed for early diagnosis and intervention. Our objective here was to describe the features and changes of SNHL at different periods during a follow-up of almost 20 years.MethodsRisk factors for SNHL during development were analyzed. The audiological examination included: Brainstem auditory evoked potentials (BAEP), and Transient evoked otoacoustic emissions (TEOAE). At birth; tonal audiometry (between 125 and 8000 Hz), and tympanometry were performed at 5, 10, 15, and 20 years of age.ResultsSixty-five percent of cases presented bilateral absence of BAEP. At 5 years of age, the most frequent SNHL level was severe (42.5%), followed by moderate (22.5%), and profound level (20%), in all cases, the SNHL was symmetrical with a predominance of lesion for the high frequencies. Exchange transfusion was associated with a higher degree of SNHL (OR = 6.00, CI = 1.11–32.28, p < 0.02). In 55%, SNHL remained stable, but in 40% of the cases it was progressive. At the end of the study six cases with moderate loss progressed to the severe level and seven cases with severe level progressed to profound.ConclusionsForty percent of infants with SNHL discharged from NICU may present a progression in the hearing loss. Exchange transfusion was associated with a higher degree of SNHL. NICU graduates with SNHL merit a long-term audiological follow-up throughout their lifespan.

http://ift.tt/2wILlSq

Segmental deficiency of cervical tracheal rings masquerading as complete tracheal rings: A case report and literature review

Publication date: October 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 101
Author(s): Andre M. Wineland, James R. Thomsen, April Landry, Dan Benscoter, Michael Rutter
Congenital deficiency of distal tracheal rings is a rare anomaly and has been previously reported in the literature. Here we report the first case deficient tracheal rings confined to the cervical trachea. Patient was transferred to our institution for management of what was initial thought to be complete tracheal rings. The patient was successfully managed with a tracheal resection and short segment cervical slide tracheoplasty. Presentation, surgical approach, histological findings, and literature review are described.

http://ift.tt/2wnss4U

Gemcitabine-induced chronic systemic capillary leak syndrome

A 56-year-old woman presented with anasarca, hypoalbuminaemia and hypotension following cycle 3 day 1 of adjuvant gemcitabine for stage II pancreatic cancer. Due to the temporal nature of presentation, suspicion for gemcitabine-induced capillary leak syndrome was included in the differential diagnosis. Vascular endothelial growth factor levels were elevated at 707 pg/mL (reference range: 9–86 pg/mL). Corticosteroids were initiated, resulting in complete resolution of symptoms and hypotension. The patient suffered relapse of symptoms on discontinuation of steroids, further supporting chronic capillary leak syndrome.

http://ift.tt/2wSaNFc

Cyclopia: isolated and with agnathia-otocephaly complex

Cyclopia is a rare form of lethal holoprosencephaly (HPE) due to incomplete cleavage of prosencephalon during embryogenesis, leading to failure of the orbits of the eye to divide into two cavities. We report two cases, one with cyclopia and another case of cyclopia with agnathia–otocephaly complex (AOC). AOC (also known as agnathia–microstomia–synotia syndrome) is a rare lethal congenital malformation of the first branchial arch characterised by the association of agnathia (agenesis of mandible) or mandibular hypoplasia, melotia (anteromedial malposition of ears), microstomia (small mouth), aglossia or microglossia (absent or rudimentary tongue). These two reported cases had in common a single eye and alobar HPE. The first case was live born and the second stillborn. Both mothers did not have antenatal care.

http://ift.tt/2whMPBZ

Prevalence of Severe and Profound Sensorineural Hearing Loss in School Children in West Bengal, India

Abstract

There is pressing need for reliable information about prevalence of sensorineural hearing loss in children in India. Without this basic information it is difficult to plan services on a rational basis. In this stratified cross sectional study, we aimed to determine the prevalence of severe and profound sensorineural hearing loss in school children in West Bengal, India. We hypothesized that all children with severe or profound hearing loss in elementary schools (age 6–14 years) would be among those identified as 'children with special need' because of their marked problems with hearing, speech or communication. First stratum of our study included all 6–14 years old mainstream elementary school 'children with special need', with known or suspected hearing problem. Second stratum covered all 6–14 years old hearing impaired children attending special schools. Comprehensive audiological assessment of 10,763 'children with special need' in mainstream elementary schools identified 3984 children showing severe or profound SNHL (≥71 dB). A further 1022 children were identified with similar loss in special schools, giving a total of 5006 children with severe or profound sensorineural loss among a school population of 8,654,057, with estimated prevalence of 0.58 (CI 0.57–0.59) per 1000 children. This is the first large scale study of the epidemiology of severe and profound sensorineural hearing loss in school children in India. Although further studies are needed from other parts of India, in the interim, findings of this study can form the basis for planning resource allocation, service provision, investigation into etiology and prevention of sensorineural deafness in children in India.

http://ift.tt/2iJ1OAj

Prevalence of Severe and Profound Sensorineural Hearing Loss in School Children in West Bengal, India

Abstract

http://ift.tt/2iJ1OAj

The views of children and young people on the use of silk garments for the treatment of eczema: a nested qualitative study within the CLOTHES randomised controlled trial

Summary

Background

Many children suffer with skin diseases, but to date, most dermatological research has been done 'on' rather than 'with' children; in this study we actively sought the experiences of children and young people. Atopic eczema (AE) is a chronic, itchy, inflammatory skin condition that affects around 20% of children and can impact on health and wellbeing for children and their families. The role of specialist clothing in the management of AE is poorly understood.

Objectives

The aim of this study, which was nested in a randomised controlled trial was to qualitatively examine child participants' experiences of using silk garments for the treatment of AE.

Methods

Eighteen children aged 5-15, who took part in the CLOTHES trial, participated in age-appropriate individual interviews or focus groups.

Results

Thematic analysis generated 4 themes directly related to the garments: i) expectations of the garments; ii) wearing the silk garments; iii) did they help? and iv) thoughts about the garments. The conclusions from this nested qualitative study are that: there was some limited improvement in eczema for some children but that the hoped for 'miracle cure' did not transpire. A mixed picture of knowledge, beliefs and experiences of using the silk garments emerged.

Conclusion

Engaging children in the evaluation of the garments provided first hand nuanced insights that enhanced understanding of the CLOTHES study as a whole. This nested study demonstrates that children can and indeed want to be engaged in dermatological research in meaningful ways that add to our understanding of treatment options.

http://ift.tt/2vISopH

Prevalence and determinants of seborrheic dermatitis in a middle aged and elderly population: the Rotterdam Study

Abstract

Background

Seborrheic dermatitis is a chronic relapsing inflammatory skin disease with unclear pathophysiological mechanisms.

Objective

To establish which lifestyle and physiological determinants are associated with seborrheic dermatitis.

Methods

Seborrheic dermatitis was diagnosed by a trained physician during a full body skin examination within the Rotterdam Study, a prospective population-based cohort study in middle aged and elderly. The current design is a comparative cross-sectional study embedded in the Rotterdam Study. Potential factors were identified from the literature and analysed in a multivariable logistic regression, including: age, sex, obesity, skin colour, stress, depression, education level, hypertension, climate, xerosis cutis, alcohol and tobacco use.

Results

Of the 5,498 participants, 788 participants were diagnosed with seborrheic dermatitis (14.3%). We found associations between seborrheic dermatitis and male sex (adjusted OR 2.09; 95% CI: 1.77-2.47), darker skin (adjusted OR 0.39; 95% CI: 0.22-0.69), season (summer vs winter: adjusted OR 0.63; 95% CI: 0.48-0.82) and generalized xerosis cutis (adjusted OR 1.41; 95% CI 1.12-1.80).

Conclusion

Seborrheic dermatitis is one of most common inflammatory dermatoses in middle and elderly aged individuals, especially during winter period. Men, and people with a light and dry skin were most likely to have seborrheic dermatitis.

http://ift.tt/2vqMzBS

Exogenous T3 toxicosis following consumption of a contaminated weight loss supplement

Summary

Learning points:

Free tri-iodothyronine (T₃) measurement may be useful in the presence of symptoms suggestive of thyrotoxicosis with discordant thyroid function tests.

Thyroid uptake scanning can be a useful aid to differentiating exogenous hormone exposure from endogenous hyperthyroidism.

Ingestion of thyroid hormone may be inadvertent in cases of exogenous thyrotoxicosis.

Medicines and supplements sourced online for weight loss may contain thyroxine (T₄) or T₃ and should be considered as a cause of unexplained exogenous hyperthyroidism.

http://ift.tt/2wh0OYz

Lone Hepatocellular Carcinoma: An Isolated Chest Wall Malignancy

Herein we describe the case of an elderly diabetic gentleman presenting with a two-week history of dyspnea and nonproductive cough, found to have a large left anterolateral chest wall mass. Further characterization through computed tomography (CT) of the chest revealed a soft tissue mass in the left anterior lower hemithorax found to be hepatocellular carcinoma (HCC). The liver, spleen, and pancreas were unremarkable. Diagnostic labs were unremarkable. The patient had no history of hepatitis, alcohol abuse, or illicit substance use. Pathological examination and immunohistochemical staining of the chest mass biopsy were consistent with metastatic hepatocellular carcinoma (HCC). The patient opted to pursue no further medical intervention and expired two weeks later. To the authors' knowledge, this is one of very few descriptions of isolated hepatocellular carcinoma found in the absence of a primary liver lesion and classical risk factors for hepatocarcinogenesis. This case highlights that HCC may present independently of liver lesions seen on imaging in a patient without clear signs or symptoms of liver. HCC should be considered in cases of isolated tumors with unclear primaries as ectopic carcinogenesis and occult primary malignancy are possibilities.

http://ift.tt/2x3qhWx

Failure of initial disease control in bullous pemphigoid: a retrospective study of hospitalized patients in a single tertiary center

Abstract

Background

Bullous pemphigoid (BP) is the most prevalent autoimmune blistering skin disease in Western countries and in Israel. Initial disease control is achieved in 60–90% of BP patients within 1–4 weeks of corticosteroid therapy. In the remainder of patients, recalcitrant disease is controlled with additional immunosuppressive treatment.

Objectives

We aimed to evaluate the rate of BP patients who needed adjuvant therapy to achieve initial disease control and to identify potential predictors for recalcitrant disease.

Methods

We conducted a retrospective study of newly diagnosed BP patients who were hospitalized at the Tel Aviv Sourasky Medical Center between the years 2008–2014. We performed statistical analyses to assess the association between clinical factors and failure of initial disease control.

Results

Among 114 hospitalized patients with newly diagnosed BP, 1.8% presented with oral mucosa involvement. Seven patients (6.1%) required systemic agents in addition to corticosteroids to achieve disease control. Hypertension (P = 0.048), involvement of the head region (P = 0.042), and metformin treatment (P = 0.02) were significantly more prevalent among patients with recalcitrant disease.

Conclusions

The low frequency of recalcitrant BP (6.1%) and the rarity of involvement of the oral mucosa (1.8%) in this study suggest that Israeli BP patients present a milder phenotype compared to similar patients from other geographic areas. Hypertension, head involvement, and metformin therapy were found to be significantly associated with the need for adjuvant therapy to achieve initial control, suggesting that these parameters may serve as predictors of treatment response in BP.

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IgA multiple myeloma in a patient with an IgG pemphigus foliaceus-like exanthem

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Direct oral anticoagulant medications in calciphylaxis

Abstract

Background

Recent studies suggest that calciphylaxis is a thrombotic condition in which arteriolar thrombosis leads to painful skin infarcts and consequent morbidity and mortality. Paradoxically, warfarin is implicated as a risk factor for calciphylaxis. Our objective is to report the use of oral direct thrombin and factor Xa inhibitors (termed direct oral anticoagulants [DOACs]) in patients with calciphylaxis.

Methods

We retrospectively reviewed records of 16 patients with calciphylaxis who received concomitant administration of novel anticoagulants. Patient data, including demographics, comorbidities, other treatments, and adverse events, were abstracted from the health records.

Results

Eleven patients (69%) had chronic kidney disease (stage ≥3A), and eight (50%) received dialysis. Apixaban was the most frequently used agent (n = 11 [69%]). Dabigatran (n = 4 [25%]) and rivaroxaban (n = 2 [13%]) were reserved for patients with mild renal impairment (stage ≤2). One clinically relevant but nonmajor bleeding event occurred. There were no major bleeding events. Nine patients (56%) were alive at last follow-up, and five (31%) had complete resolution of their calciphylaxis (mean follow-up, 523 days; range, 26–1884 days).

Conclusion

DOACs were safe and well tolerated in patients with calciphylaxis, in this initial experience. Several patients had improvement or resolution of calciphylaxis in response to therapy that included DOACs. The degree of renal impairment should guide DOAC choice. Randomized trials are required to determine treatment efficacy.

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Acne treatment in antiquity: can approaches from the past be relevant in the future?

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Vocal fold leukoplakia: incidence, management, and prevention.

Voice rest after laryngeal surgery: what's the evidence?.

Isoflurane Preconditioning Alleviated Murine Liver Ischemia and Reperfusion Injury by Restoring AMPK/mTOR-Mediated Autophagy.

BACKGROUND: Isoflurane has a pharmacological preconditioning effect against ischemia injury in the heart, kidney, and brain, but whether and how isoflurane preconditioning protects livers against ischemia and reperfusion (IR) injury is unclear. METHODS: Mice were randomly divided into an isoflurane preconditioning (ISO) group and control group, receiving 1.5% isoflurane or carrier gas for 40 minutes, respectively (n = 8/group). A partial warm liver IR model was used, and liver injury was evaluated. Primary hepatocytes were pretreated with 1.5% isoflurane for 2 hours before the induction of cell death by hydrogen peroxide. Cell death and survival were evaluated with the lactate dehydrogenase and cell counting kit-8 assay. Autophagy and regulatory molecules in stressed livers and hepatocytes were analyzed by Western blot (n = 6/group). An autophagy inhibitor (3-methyladenine [3-MA]) and 5' adenosine monophosphate-activated protein kinase (AMPK) inhibitor (dorsomorphin) were administered in vivo (n = 8/group) and in vitro (n = 6/group). RESULTS: Compared to that observed in the control group, mice in the ISO group showed reduced liver injury (alanine aminotransferase [ALT] levels, control versus ISO group, 8285 +/- 769 vs 4896 +/- 917 U/L, P

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Theoretical Modeling of Coagulation Management With Therapeutic Plasma or Prothrombin Complex Concentrate.

Prothrombin complex concentrates (PCCs) have been associated with a possible risk of thromboembolic complications, potentially attributable to an increased ratio of the plasma concentration of factor II (FII) to antithrombin (AT). We developed a mathematical model to examine the relationship between amounts of PCC or therapeutic plasma administered, and plasma levels of FII and AT. The model showed that PCC produces substantial increases in plasma levels of FII but only small changes in AT, increasing the FII:AT ratio. Therapeutic plasma was shown to have only modest effects on levels of FII or AT, unless high doses are used. This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. (C) 2017 International Anesthesia Research Society

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Coblation Versus Conventional Tonsillectomy: A Double Blind Randomized Controlled Trial

Abstract

Post tonsillectomy bleeding and pain are two main problems following traditional tonsillectomy. Coblation therapy (controlled ablation) was first used in tonsillectomy in 2001. A great amount of literature debated around its use with controversial opinions regarding its benefits, efficacy, and cost. This is a prospective double-blind randomized controlled study that compares between coblation tonsillectomy and conventional tonsillectomy as regard operative time, operative blood loss, time needed to return back to the normal activity and diet, and incidence of postoperative hemorrhage whether primary or secondary. The study included 1004 patients with mean age of 10.4 years (range from 4 to 35 years). The first group (coblation tonsillectomy group) included 507 patients, age ranging from 4 to 35 years with mean age 15.1 year. This group included 277 female (54.6%) and 230 male (45.4%). The second group (conventional tonsillectomy group) included 497 patients, age ranging from 4 to 24 years and mean age 14.7 years. This group included 274 female (55.1%) and 223 male (44.9%). Coblation tonsillectomy offers significant advantages over dissection method with less operative time, decreased intraoperative blood loss, early restoration of daily activities and normal diet. However coblation tonsillectomy is associated with a higher incidence of secondary hemorrhage.

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Coblation Versus Conventional Tonsillectomy: A Double Blind Randomized Controlled Trial

Abstract

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Comparison of the laryngeal mask airway supreme and the i-gel in paralysed elderly patients: A randomised controlled trial.

BACKGROUND: The laryngeal mask airway supreme (LMA-S) and i-gel are both popular second-generation supraglottic airway devices that have been widely studied in surgical patients, but their differences in clinical performance in the elderly are not clear. OBJECTIVE: We compared the efficacy and safety of the LMA-S and i-gel in anaesthetised and paralysed elderly patients. DESIGN: A prospective, randomised and parallel study. SETTING: Single-centre trial, study period January 2014 from to October 2016. PATIENTS: One hundred and six elderly patients who underwent urological or orthopaedic surgery with an expected duration less than 2 h. INTERVENTION: Patients were allocated to either the LMA-S (n = 53) or i-gel (n = 53) group. All insertions were performed in a standardised manner according to the manufacturers' instructions. MAIN OUTCOME MEASURES: Our primary endpoint was the rate of successful insertion at the first attempt. The adequacy of positive pressure ventilation and airway sealing, fibreoptic laryngoscopy grades and stability of airway maintenance during anaesthesia were also assessed. RESULTS: Although the rate of successful insertion at the first attempt was similar between the two groups (94.3 vs. 82.7%, P = 0.072), more patients required device manipulation during insertion with the LMA-S than the i-gel (42.3 vs. 18.9%, P = 0.011). Good fibreoptic laryngoscopy grades were significantly more common with the i-gel than the LMA-S (79.3 vs. 55.8%, P = 0.042), and peak inspiratory pressures were lower in the i-gel group both immediately after insertion and at the end of surgery. Leak pressures were significantly higher in the i-gel group than the LMA-S group, both immediately after insertion and at the end of surgery (25.8 vs. 23.0, P = 0.036; and 28.1 vs. 23.7, P

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Αρχειοθήκη ιστολογίου

Τετάρτη 30 Αυγούστου 2017

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