Glucocorticoids: the mode of action in bullous pemphigoid

Abstract

Bullous pemphigoid (BP) is the most common of pemphigoid diseases caused by autoantibodies against the structures of dermoepidermal junction followed by complement activation, innate immune cell infiltration, neutrophil proteinase secretion and subepidermal blister formation. The first line treatment of BP is topical and systemic glucocorticoids (GC).

Regulation of the immune system and inflammatory cells is the main target of GC actions. GCs act through genomic and non-genomic mechanisms. The human glucocorticoid receptor (GR) mediates most of the biologic effects of glucocorticoids: cytosolic GR binds GCs and is capable to bind to glucocorticoid response elements in DNA and either transactivate or transrepress genes depending on the tissue and cell-type. In addition, GR exerts rapid, non-genomic effects possibly mediated by membrane-localized receptors or by translocation to mitochondria. GCs can also interact directly with several enzymes and cytokines.

As a target treatment for BP, the production of autoantibodies should be discontinued. GCs, in spite of their wide immunosuppressive actions, are weak to stop immunoglobulin G (IgG) autoantibody formation. However, both systemic and topical GCs are able to reduce the clinical symptoms of BP. GCs are used to inhibit the secondary inflammation and symptoms, such as blistering and pruritus, and it is shown that GC treatment will gradually decrease also the autoantibody formation. Our review article analyzes the mode of action of GC treatment in BP, as far it is possible due to paucity of modern immunological studies.

This article is protected by copyright. All rights reserved.

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Hepatitis due to EBV-reactivation under infliximab in a psoriasis patient

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Frequency of self-reported drug allergy

Patients reporting drug allergy are treated with second-line therapies, with possible negative clinical and health consequences.

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Cutaneous Small-Vessel Vasculitis in Two Children with Inflammatory Bowel Disease: Case Series and Review of the Literature

Abstract

Cutaneous small-vessel vasculitis (CSVV) is an infrequent manifestation of pediatric inflammatory bowel disease (IBD). We report two cases of CSVV associated with ulcerative colitis, review the literature, and discuss the diagnostic evaluation of children who present with CSVV and abdominal pain. After excluding more common causes of CSVV and abdominal pain in children, including immunoglobulin A vasculitis (previously Henoch–Schönlein purpura), infectious colitis, and drug-induced vasculitis, alternative diagnoses such as CSVV secondary to IBD or systemic vasculitis with gastrointestinal involvement must be considered.

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Palmar Eccrine Hidradenitis Secondary to Trauma from Computer Gaming in an Adolescent After Bone Marrow Transplantation

Abstract

A 14-year-old boy who had undergone a matched sibling bone marrow transplant for acute lymphoblastic leukemia presented with painful nodules on his palms after prolonged gaming on his computer and mobile phone. Histology showed a neutrophilic inflammatory infiltrate surrounding the acrosyringium and eccrine sweat coils in the deep dermis. The lesions resolved spontaneously with conservative management.

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Idiopathic Calcinosis Cutis Universalis Treated Successfully with Oral Diltiazem—A Case Report

Abstract

Idiopathic calcinosis cutis is very rare and difficult to treat. Various medical modalities of treatment described with inconsistent results include chelating agents, colchicine, and probenecid. Calcium channel blockers are known to work by inhibiting intracellular entry of calcium. We successfully treated a case of idiopathic calcinosis cutis using oral diltiazem.

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Tuberous Sclerosis Complex in 29 Children: Clinical and Genetic Analysis and Facial Angiofibroma Responses to Topical Sirolimus

Abstract

Background/Objectives

Tuberous sclerosis complex (TSC) is a genetic disorder and facial angiofibromas are disfiguring facial lesions. The aim of this study was to analyze the clinical and genetic features of TSC and to assess the treatment of facial angiofibromas using topical sirolimus in Chinese children.

Methods

Information was collected on 29 patients with TSC. Genetic analyses were performed in 12 children and their parents. Children were treated with 0.1% sirolimus ointment for 36 weeks. Clinical efficacy and plasma sirolimus concentrations were evaluated at baseline and 12, 24, and 36 weeks.

Results

Twenty-seven (93%) of the 29 patients had hypomelanotic macules and 15 (52%) had shagreen patch; 11 of the 12 (92%) who underwent genetic analysis had gene mutations in the TSC1 or TSC2 gene. Twenty-four children completed 36 weeks of treatment with topical sirolimus; facial angiofibromas were clinically undetectable in four (17%). The mean decrease in the Facial Angiofibroma Severity Index (FASI) score at 36 weeks was 47.6 ± 30.4%. There was no significant difference in the FASI score between weeks 24 and 36 (F = 1.00, p = 0.33). There was no detectable systemic absorption of sirolimus.

Conclusion

Hypomelanotic macules are often the first sign of TSC. Genetic testing has a high detection rate in patients with a clinical diagnosis of TSC. Topical sirolimus appears to be both effective and well-tolerated as a treatment of facial angiofibromas in children with TSC. The response typically plateaus after 12 to 24 weeks of treatment.

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Can You See Me Now? Video Supplementation for Pediatric Teledermatology Cases

Abstract

Background/Objectives

Digital video is widely available and is used sporadically in clinical settings to evaluate patients, but whether it helps improve clinical management has not been determined. The aim of this study was to assess whether recorded video in addition to still images can improve residents' diagnostic and management accuracy and confidence with pediatric teledermatology cases.

Methods

Dermatology residents from three programs were assigned alternately to an online survey with 15 pediatric teledermatology cases presented with still images only (still) or still images plus recorded video (mixed). Participants provided free-text diagnoses and management recommendations and rated their confidence and image quality. Responses were scored using a modified script concordance grading key based on reference panelists' responses.

Results

Thirty-one residents participated (response rate 57%). Participants in the mixed group scored significantly higher on management accuracy (87.6 ± 12.9 vs 71.7 ± 14.2; p = 0.003). Both groups performed better on more common conditions than less common conditions. The mixed group outperformed the still group on less common conditions with respect to management recommendations.

Conclusion

This novel study suggests that supplemental recorded video may improve the management accuracy of pediatric teledermatology consultations, particularly for complex cases. Residents may benefit from training in recording and interpreting video.

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See One, Do One, Teach One: No More

Anatomic dissection prior to examining and treating a living person dates back to Alexandria, Greece in the third century bc.1 For Otolaryngologist-Head and Neck Surgeons, anatomic dissections, the original "simulators," have served to prepare them for open approaches to the face, head, and neck, for temporal bone surgery, and for paranasal sinus procedures. But, as with diagnostic and surgical methods and tools, simulation tools and techniques have evolved, most rapidly over the past several years.

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Simulation in Otolaryngology

At present, simulation is widely accepted as a means of providing surgical education outside of the clinical environment. Recent advances in simulation technology and medical education reform coupled with political and societal demands have provided the impetus toward the exponential growth of this field. Research in this area supports the use of simulation to train and assess individuals in both psychomotor and team-based skills. Further benefits include translation of these improved skills to patient care practices with better patient outcomes and reduced health care costs.

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Split-Hand Malformation in a 4-Year-Old Child

Split-hand deformity is one of the milder manifestations of a congenital disorder called split-hand/split-foot malformation. We present a case of a 4-year-old child with split-hand malformation in his left hand since birth. A median cleft was present in the affected hand with absence of the 3rd and 4th digits, giving rise to a characteristic lobster-claw appearance. Functionality of the affected hand was modestly impaired. As none of the close family members of the patient had similar limb malformations, the deformity was postulated to arise most likely from a de novo mutation. The patient was discharged after the parents were provided with genetic counseling.

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Effects of anaesthesia and analgesia on long-term outcome after total knee replacement: A prospective, observational, multicentre study.

BACKGROUND: Perioperative regional anaesthesia may protect from persistent postsurgical pain (PPSP) and improve outcome after total knee arthroplasty (TKA). OBJECTIVES: Aim of this study was to evaluate the impact of regional anaesthesia on PPSP and long-term functional outcome after TKA. DESIGN: A web-based prospective observational registry. SETTING: Five Italian Private and University Hospitals from 2012 to 2015. PATIENTS: Undergoing primary unilateral TKA, aged more than 18 years, informed consent, American Society of Anesthesiologists (ASA) physical status classes 1 to 3, no previous knee surgery. INTERVENTION(S): Personal data (age, sex, BMI and ASA class), preoperative pain assessed by numerical rating scale (NRS) score, and risk factors for PPSP were registered preoperatively. Data on anaesthetic and analgesic techniques were collected. Postoperative pain (NRS), analgesic consumption, major complications and patient satisfaction were registered up to the time of discharge. PPSP was assessed by a blinded investigator during a phone call after 1, 3 and 6 months, together with patient satisfaction, quality of life (QOL) and walking ability. MAIN OUTCOME MEASURES: Experience of PPSP according to the type of peri-operative analgesia. RESULTS: Five hundred sixty-three patients completed the follow-up. At 6 months, 21.6% of patients experienced PPSP, whereas autonomy was improved only in 56.3%; QOL was worsened or unchanged in 30.7% of patients and improved in 69.3%. Patients receiving continuous regional anaesthesia (epidural or peripheral nerve block) showed a lower NRS through the whole peri-operative period up to 1 month compared with both single shot peripheral nerve block and those who did not receive any type of regional anaesthesia. No difference was found between these latter two groups. Differences in PPSP at 3 or 6 months were not significantly affected by the type of anaesthesia or postoperative analgesia. A higher NRS score at 1 month, younger age, history of anxiety or depression, pro-inflammatory status, higher BMI and a lower ASA physical status were associated with a higher incidence of PPSP and worsened QOL at 6 months. CONCLUSION: Continuous regional anaesthesia provides analgesic benefit for up to 1 month after surgery, but did not influence PPSP at 6 months. Better pain control at 1 month was associated with reduced PPSP. Patients with higher expectations from surgery, enhanced basal inflammation and a pessimistic outlook are more prone to develop PPSP. TRIAL REGISTRATION: Clinicaltrials.gov identifier: NCT02147730 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. http://ift.tt/OBJ4xP (C) 2017 European Society of Anaesthesiology

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Indicadores de calidad del Programa de Detección Precoz de Hipoacusia Permanente del Hospital Padre Hurtado

Introducción: La detección precoz de hipoacusia permanente en lactantes beneficia el desarrollo integral del paciente. Los programas cuyo objetivo es la identificación universal de hipoacusia debieran tener como meta determinados criterios de calidad en su ejecución. Objetivo: El objetivo del presente trabajo es comunicar los resultados del Programa de Detección Precoz de Hipoacusia en el Hospital Padre Hurtado. Material y método: Se incluyen los recién nacidos entre el 1 de enero de 2014 y el 31 de agosto de 2016. Los pacientes sin factores de riesgo para hipoacusia congénita se evalúan con examen de emisiones otoacústicas, y los pacientes con factores de riesgo con potenciales auditivos automatizados de tronco encefálico. Refieren aquellos pacientes con exámenes alterados en forma uní o bilateral. La etapa diagnóstica incluye potenciales auditivos evocados con tono, impedanciometría de alta frecuencia y audiometría de refuerzo visual. Los pacientes con diagnóstico de hipoacusia permanente son amplificados e inician proceso de habilitación. Resultados: En el período de estudio el universo a evaluar fue de 12.313 recién nacidos. Se completó la etapa de pesquisa en 98.4% con una tasa de referencia de 0.6%. 79 pacientes pasaron a etapa diagnóstica, completaron su evaluación antes de 3 meses en 95% de los casos. Se confirmó hipoacusia sensorioneural en 7 casos, con una tasa de 0.56 por 1.000 recién nacidos vivos. En 57% de los pacientes se amplificaron antes de los seis meses de vida. Conclusiones: El Programa de Hipoacusia Congénita del Hospital Padre Hurtado cumple con los indicadores de calidad recomendados en los ítemes de pesquisa y diagnóstico. En la etapa de habilitación con audffonos esto se realiza antes de los seis meses de vida sólo en 57% de los casos.

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Introduction: Quality indicators of the newborn hearing screening program in Hospital Padre Hurtado. Aim: Asses the accomplishment of quality indicators of the newborn hearing screening program in Hospital Padre Hurtado, Chile, as proposed by the Joint Committee on Infant Hearing Loss (JCIH). Material and method: Two stage screening protocol: otoacoustic emissions for babies in the well-infant nursery and automated auditory brainstem responses for those in the intensive care unit orwith risk factors. If they fail one or both ears they proceed to a comprehensive audiological assessment. Results: 12.313 live births between 01/01/2014 and 108/31/16, 12.103 were screened before discharge (98.4%). 79 cases proceeded to diagnostic assessment, referral rate 0.6%. 95% infants completed audiological evaluation before three months, seven cases were diagnose with permanent sensorineural hearing loss for a prevalence of 0.56 per 1000 live births. Amplification was provided before 6 months of age in 57% of deaf children. Conclusions: Quality indicators of the JCIH are met by our newborn hearing screening program with the exception of adequate timing for the provision of hearing aids: 57% before six months of age.

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Nuevo método para evaluar el Test Subjetivo Vertical

Introducción: La prueba Visual Subjetiva Vertical (SVV por sus siglas en inglés) corresponde a una prueba de bajo costo y complejidad que permite el estudio de la función vestibular "estática". En el año 2009 se valida la Prueba del Balde con igual confianza, sensibilidad y especificidad, que la prueba de domo. Sin embargo, diversos factores impresionan disminuir su precisión. Objetivo: Proponer un nuevo método utilizando un programa computacional y una interfaz pantalla-paciente, que permita disminuir la influencia de variables y aumentar la precisión de la evaluación. Material y método: En 43 voluntarios sin historia de patología vestibular y en 32 pacientes con patología vestibular se compararon los resultados de la aplicación de SVV en dos modalidades: prueba del balde tradicionalyuna prueba computarizada propuesta en el presente artículo. Resultados: En nuestro estudio el SVV con balde muestra 57% de sensibilidad y 90% de especificidad, mientras el SVV digital tiene 74% de sensibilidad y 93% de especificidad, el cual también presentó significativamente una menor desviación estándar. Conclusión: En suma, el SVV computarizado arroja un resultado más preciso que SVV con balde, con mejor utilidad clínica al tener mayor de discriminación con mejores perfiles de sensibilidad/especificidad.

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Background: Subjective Visual Vertical Test (SVV) is a low-cost and simple evaluation that allows the physician to study the static vestibular function of a patient. In 2009 the Bucket Test was validated as a high confidence, sensitivity and specificity comparable to the hemispheric dome testing device. Although, its result can be affected by multiple variables. Aim: To propose a new method to evaluate SVV using a computer software interface, that allows a reduction ofvariables therefore increasing its precision. Material and method: In a sample of 43 volunteers with no previous history of vestibular pathologies and 32 patients with diagnosed vestibular pathologies we compared the results of 2 different modules of SVV testing: a traditional bucket test and a computerized test proposed in the present article. Results: Bucket test SVV for this research showed a sensitivity of 57% and a specificity of 90%, meanwhile Computerized SVV had a sensitivity of 74% and a specificity of 93%, which also presented a significant smaller standard deviation. Conclusion: In summary, digital SVV testing grants more accurate result in comparison to the Bucket Test, with a better clinical performance due to an improved discriminatory capacity with better Sensitivity/Specificity profiles.

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Detección temprana de la hipoacusia con emisiones acústicas

Introducción: La hipoacusia neonatal es considerada un problema de salud pública. Su diagnóstico precoz y rehabilitación adecuada previenen la consecuencia más importante de la hipoacusia infantil: crecer sin un lenguaje. Objetivo: Analizar los resultados obtenidos en el Programa Universal de Detección Precoz de la Hipoacusia Infantil implementado en el Complejo Hospitalario Universitario Insular Materno Infantil de Las Palmas de Gran Canaria desde enero de 2007 hasta diciembre de 2013. Material y método: Se estudiaron 44.597 recién nacidos, mediante otoemisiones acústicas en ambas fases del programa. La fase diagnóstica es llevada cabo en la Unidad de Hipoacusia del Servicio de Otorrinolaringología. Resultados: Desde enero de 2007 y diciembre de 2013 nacieron 46.587 niños, se le practicaron el screening a 44.597, de los cuales, 41.621 presentan OEA positivas, 1.233 tienen OEA ausentes. En la segunda fase 8.193 presentaron otoemisiones positivas en ambos oídos, 649 no pasaron la segunda fase. Presentaron factores de riesgo para hipoacusia un total de 9.581 niños. La media de derivación a la fase diagnóstica fue de 5.69%, alcanzó una cobertura del 95,73%. 32 niños fueron diagnosticados entre los 6 y 8 meses ingresaron al Programa de Implantes Cocleares siendo intervenidos quirúrgicamente entre los 9 y 16 meses. Conclusión: El programa es adecuado a nuestro funcionamiento, alcanzando el mismo un porcentaje de cobertura superiordel 95% valor ampliamente satisfactorio teniendo en cuenta las recomendaciones de la CODEPEH.

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Introduction: Neonatal hearing loss is a public health problem early diagnosis and (re) habilitation adequate prevent the most important consequence of infant hearing loss: growing up without a language. Aim: To analyze the results obtained in the Universal Program for Early Detection of Infant Hearing Loss implemented in the Complejo Hospitalario Universitario Insular Materno Infantil, in Las Palmas de Gran Canaria, from January 2007 to December 2013. Material and method: We studied otoacoustic emissions in both phases ofthe program for 44 597 newborns. The diagnostic phase was carried out at the Hearing Loss Unit, Department of Otorhinolaryngology. Results: Between January 2007 and December 2013, 46,587 children were born, 44,597 underwent screening, of which 41,621 have positive otoacustic emissions and in 1233 these are absent. In 8,193 the secondphase gave positive otoacustic emissions in both ears and 649 failed the second phase. 9,581 newborns presented risk factors associated with hearing loss. The average referral to diagnostic phase was 5.69%, the program reacheda coverage of 95.73%. 32 children diagnosedbetween 6and8months entered the Cochlear Implant Program being operated on between 9 and 16 months. Conclusion: The Program in our hospital has proved adequate, achieving a coverage rate above 95%, highly satisfactory value considering the recommendations of the CODEPEH.

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Análisis de tiempos de espera en pacientes con cáncer de cabeza y cuello en el Hospital San Juan de Dios

Introducción: Los cánceres de cabeza y cuello (CCyC) son reconocibles precozmente mediante la cínica y exámenes poco invasivo, sin embargo, existe importante demora entre las primeras molestias del paciente y el tratamiento. Objetivo: Describir los tiempos involucrados entre los primeros síntomas y el tratamiento. Material y método: Estudio descriptivo retrospectivo, mediante evaluación de fichas de pacientes diagnosticados y tratados por CCyC en otorrinolaringología del Hospital San Juan de Dios durante 18 meses. Resultados: Fueron elegibles 33 casos. El 93,7% correspondieron a carcinomas escamosos. Fueron comprometidos principalmente laringe (45,4%) y orofaringe (24,2%). El 90,2% se diagnosticó en estadio avanzado. Los pacientes demoraron 17 semanas en consultar desde sus primeros síntomas. Pasó una semana para ser evaluados por el especialista. Este tardó una semana en obtener una biopsia. El resultado histológico tomó tres semanas y el tratamiento se realizó a las cinco semanas. Quienes recibieron cirugía como primer tratamiento tardaron cuatro semanas en recibirla. Quienes fueron a quimiorradioterapia como coadyuvancia esperaron 11,5 semanas. Mientras, quienes recibieron quimiorradioterapia como único tratamiento, tardaron 8 semanas. Conclusión: Los principales retrasos en el manejo de estos pacientes son: la demora del paciente en consultar, el informe histológico y el acceso al tratamiento al conocerse el diagnóstico, especialmente cuando el tratamiento es la radio-quimioterapia.

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Introduction: Head and neck cancers (HNC) are recognizable by clinical and early minimally invasive tests, however, there is significant delay between the first symptoms and treatment. Aim: To describe the times involved between the first symptoms and treatment. Material and method: Descriptive and retrospective study, by evaluating records of patients diagnosed and treated for HNC in otolaryngology at the Hospital San Juan de Dios, for 18 months. Results: 33 cases were eligible. 937% corresponded to squamous cell carcinoma. The sites most committed were larynx (45.4%) and (24.2%); the diagnosis was made in advanced stages in 90.2% of the cases. The patients took 17 weeks to consult since their first symptoms. It took 1 week to be evaluated by the specialist. It took 1 week to get a biopsy. The histological result took 3 weeks and treatment was performed at 5 weeks. Who received surgery as first treatment took 4 weeks to receive it. Who went to chemo-radiotherapy as co-adjuvant waited 11.5 weeks. Those receiving chemo-radiotherapy as the only treatment, 8 weeks. Conclusion: The major delays are the patient delay in consulting, the time delay in the histological report and access to treatment when knowing the diagnosis, especially when treatment is radio-chemotherapy.

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Estimación de la incidencia del cáncer de laringe en Chile según la aplicación de un formulario de registro digital

Introducción: El cáncer de laringe es una neoplasia frecuente en otorrinolaringología. Actualmente la incidencia ajustada por edad en Chile de cáncer laríngeo se proyecta en base al Registro Poblacional de Cáncer de la provincia de Los Lagos (International Agency for Research on Cancer - WHO), siendo ésta de 1,3 x100.000 habitantes. Objetivo: Estimar la incidencia del cáncer de laringe en Chile medida mediante un formulario de registro digital en hospitales y clínicas del país que realizan diagnóstico y tratamiento a esta patología, entre septiembre de 2015 y septiembre de 2016. Material y metodo: Estudio descriptivo prospectivo. Se utilizó un formulario de registro digital. Se incluyeron pacientes con diagnóstico histológico de cáncer de laringe entre septiembre de 2015 y septiembre de 2016. Resultados: Se registraron un total de 134 carcinomas escamosos de laringe. 15 (11%) de sexo femenino y 119 (89%) de sexo masculino. La incidencia fue de 2,13 casos por cada 100.000 habitantes, al estandarizarla por edad fue de 2,12 por cada 100.000 habitantes. Conclusión: La estimación del presente estudio es más alta que la reportada en los registros nacionales. Es necesario crear un registro poblacional más representativo de la realidad nacional, y así conocer la magnitud real de los casos de cáncer en el país.

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Introduction: The squamous cell carcinoma of the larynx is a frequent neoplasms within otorhinolaryngology Currently the age standardized incidence of Chile for laryngeal cancer is based on the cancerpopulation registration of the province of Los Lagos (International Agency for Research on Cancer - WHO), which is 1.3 x 100,000 inhabitants. Aim: To estimate the incidence of laryngeal cancer in Chile using a digital registry form in hospitals and clinics of the country that diagnose and treat this condition between September 2015 and September 2016. Material and method: A descriptive prospective study was conducted. We use a digital registration form. We included patients with clinical diagnosis of laryngeal cancer between September 2015 and September 2016. Data was analyzed with descriptive statistics. Results: A total of 134 squamous cell carcinomas of the larynx were registered. 15 (11%) were female and 119 (89%) were male, with a mean age of65years. The incidence was of 2.13 cases per 100,000 inhabitants, and the age-standardized incidence was of 2.12 per 100,000 inhabitants. Conclusion: In this study the incidence is greater than the one reported in other registrations. It is necessary a more representative population registration of the national reality in order to estimate accurately the cancer cases in the country.

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Angiomiolipoma de cavidad nasal: Reporte de un caso y revisión de la literatura

El angiomiolipoma de cavidad nasal es un tumor hamartomatoso extremadamente infrecuente compuesto por adipocitos maduros, espacios vasculares con escaso tejido elástico y la presencia de haces de células musculares lisas maduras. Se manifiesta principalmente por obstrucción nasal unilateral y epistaxis recurrente. Se presenta el caso de una paciente de 73 años con historia crónica de obstrucción nasal y epistaxis recurrente izquierda asociada a rinorrea y descarga posterior intermitente. La tomografía computarizada (TC) y resonancia nuclear magnética (RNM) demuestran una lesión vascular circunscrita en fosa nasal izquierda. La angiografía demostró irrigación exclusiva de la arteria esfenopalatina izquierda. Se realizó extirpación de la lesión por abordaje endonasal endoscópico previa embolización arterial. La revisión de la literatura mundial muestra que es el duodécimo caso de angiomiolipoma de cavidad nasal reportado.

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Angiomyolipoma of nasal cavity is an extremely rare hamartoma tumor composed of mature adipocytes, vascular spaces with lack of elastic tissue, and the presence of bundles of mature smooth muscle cells. It manifests itself mainly by unilateral nasal obstruction and recurrent epistaxis. We present the case of a 73-years-old woman with chronic history of left-sided nasal obstruction and recurrent epistaxis associated with rhinorrhea and intermittent post nasal drip. Computed tomography and magnetic resonance imaging demonstrate a vascular lesion inside the left nasal cavity. Angiography showed irrigation exclusively by the left sphenopalatine artery Surgical excision was performed by endoscopic endonasal approach after arterial embolization. World literature review showed that this is the twelfth reported case of angiomyolipoma of the nasal cavity.

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Pólipo bilateral de cuerda vocal: Presentación de un caso clínico

Los pólipos de cuerda vocal son lesiones laríngeas benignas. Se asocian a micro-traumatismos por mal uso vocal que generan remodelación de la lámina propia y el epitelio. Es más frecuente en hombres entre los veinte y cuarenta años. En la gran mayoría de los casos se presenta de manera unilateral. Si bien estas lesiones están bien documentadas en la literatura, es raro encontrar presentaciones bilaterales, por lo que su enfrentamiento y manejo puede ser discutible. Se presenta el caso de una paciente de sexo femenino de 41 años, fumadora, que consulta por disfonía de larga data. Se diagnostican pólipos bilaterales de cuerda vocal, realizando una intervención quirúrgica en un tiempo, con un resultado favorable.

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Vocal cord polyps are benign Iaryngeal lesions. They are associated to micro traumatisms because ofvocal misuse, generating a remodelation of the lamina propria and the epithelium. It is more common in men between twenty and forty years of age. In the vast majority of cases it unilaterally occurs. While these are well documented injuries in the literature, it is rare to find bilateral presentations, so their confrontation and management may be debatable. We present the case of a female patient, smoker, who consulted for chronic dysphonia. Bilateral vocal cord polyps were diagnosed, performing a bilateral resection with a positive outcome.

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Biopsia endoscópica de osteocondroma de fosa infratemporal

Presentamos el caso de una mujer de 13 años con un gran tumor de características óseas en la fosa infratemporal derecha, el cual fue biopsiado mediante un abordaje endoscópico transeptal transpterigoídeo. La biospia mostró un osteocondroma. Describimos el caso y discutimos sus aspectos relevantes.

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We report the case of a 13-year-old woman with a large tumor with osseous appearance in her right infratemporal fossa, which was biopsied through an endoscopic transpterygoid approach. The biopsy showed an osteocondroma. We described the case and discuss its relevant aspects.

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Condrosarcoma de la laringe: Reporte de un caso y revisión de la literatura

El cáncer de laringe es un tumor relativamente raro en Ecuador (0,5/100000 varones). Las lesiones neoplásicas de laringe son usualmente epiteliales y el tipo histológico más frecuente es el carcinoma escamocelular. El tumor mesenquimal más común es el condrosarcoma. En los 31 años de existencia del Registro Nacional de Tumores de Ecuador éste es el primer caso de condrosarcoma de laringe registrado. Por este motivo, hemos decidido reportar el manejo diagnóstico y terapéutico de este caso.

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Laryngeal cancer is a relatively uncommon tumor en Ecuador (0,5/100000 males). These neoplastic lesions usually epithelial and the most frequent histological type is squamous cell carcinoma. The most common mesenchymal tumor is chondrosarcoma. No case of this type of laryngeal tumor has been registered since the foundation of the National Cancer Registry of Ecuador thirty-one years ago. For this reason, we have decided to report the diagnostic and therapeutic management of his case.

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Estenosis de la apertura piriforme y síndrome de incisivo central único: Casos clínicos

El síndrome incisivo central único es un trastorno que involucra anomalías de la línea media. Se puede presentar con dificultad respiratoria poniendo en peligro la vida del recién nacido, debido a malformaciones nasales. Estas malformaciones incluyen atresia de coanas y la estenosis de la apertura del orificio piriforme. Debe pensarse en esta última en todo recién nacido con estridor y dificultad respiratoria de grado variable, asociado a la dificultad de pasar una sonda a través de la región anterior de las fosas nasales. El diagnóstico se confirma por tomografía computarizada del macizo craneofacial y el examen nasofibroscópico. La conducta terapéutica dependerá de la gravedad de los síntomas, pero en general si es que no hay compromiso respiratorio severo, se prefiere una conducta expectante, y esperar el crecimiento craneofacial del niño, para aumentar permeabilidad nasal. Presentamos dos casos clínicos.

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Solitary median maxillary central incisor syndrome is a rare disorder involving midline abnormalities. It may present with life threatening respiratory distress in the neonate secondary to nasal malformations. These include choanal atresia and pyriform aperture stenosis. The last to be thought in any newborn with stridor and respiratory distress associated with the difficulty of passing a tube through the anterior region of the nostrils. The diagnosis is confirmed by craniofacial CT scan and nasolaryngoscope evaluation. The therapeutic approach depends on the severity of symptoms.

http://ift.tt/2vk2Ibl

Abordaje cerrado en patología de vía aérea superior con el uso de microelectrodos: Nuestra experiencia

El láser como alternativa a la cirugía abierta de la vía aérea superior ha venido a modificar la forma de abordaje de las patologías en esta área, pero no deja de ser un procedimiento costoso que no está al alcance de todos los servicios. Por este motivo se han reinventado nuevas formas de abordaje que cumplan los mismos requisitos tanto de la cirugía abierta como con láser pero con un menor coste. Presentamos una serie de 30 casos realizados en un período de 6 años por motivos tanto tumorales como no, en los que se realizaron abordajes cerrados a través de microcirugía con disección mediante microelectrodos. Obteniendo pocas complicaciones y una disminución de la estancia hospitalaria significativa. Con lo cual nos parece una técnica eficiente para abordajes de este tipo.

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The laser as an alternative to open surgery of the upper airway has come to change the form of approaching the disease in this area, but it is still an expensive procedure that is not available to all services. For this reason a new ways of approach to meet the same requirements both open as laser but at a lower cost surgery. We present a series of 30 cases performed over a period of 6 years for reasons as much tumor, which closed approaches through microsurgical dissection were performed using microelectrodes. Obtaining few complications and significant decreased hospital stay. Our considerations is it seems an efficient technique for such approaches.

http://ift.tt/2vkp6Bw

Presentación inusual de fracturas laríngeas: Reporte de dos casos de fracturas laríngeas no traumáticas

Las fracturas laríngeas se producen principalmente en el contexto de traumas cervicales, ahorcamiento o estrangulamiento. Las fracturas laríngeas no traumáticas son excepcionales, existiendo escasos reportes en la literatura. A continuación, presentamos dos casos de fracturas laríngeas no traumáticas evaluadas en nuestro servicio.

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Laryngeal fractures occur mainly in the context of cervical trauma, hanging or strangulation. Nontraumatic laryngeal fractures are rare and there are fewreports in the literature. We present two cases of nontraumatic laryngeal fractures evaluated in our service.

http://ift.tt/2vk3N2I

Utilidad de imagen de banda estrecha en un caso de melanoma mucoso de septum nasal

En los últimos años, se está utilizando la fibrolaringoscopía con imagen de banda estrecha (NBI) como técnica novedosa para observar el patrón específico de microvas-cularización de una lesión concreta a evaluar. Es conocida por su utilidad en el diagnóstico de otras lesiones de vías aerodigestivas superiores, fundamentalmente laríngea y digestiva. Los melanomas mucosos son tumores infrecuentes, que suelen localizarse a nivel del área rinosinusaly que comportan un manejo y pronóstico distinto con respecto a los melanomas cutáneos. Se presenta el caso clínico de una paciente mujer con anamnesis, exploración y fibrolaringoscopía con imagen de banda estrecha, compatible con melanoma mucoso de fosa nasal izquierda. El tratamiento realizado fue quirúrgico, sin necesidad de tratamiento coadyuvante, y no presenta evidencia de enfermedad al año postseguimiento.

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In recent years, it is being used fibrolaryngoscopy with narrowband image (NBI) as a novel technique to observe the specific pattern of microvasculature of a particular lesion. NBI is known for its usefulness in the diagnosis of other lesions of the upper aerodigestive tract, (primarily laryngeal and digestive lesions). Mucosal melanomas are rare tumors, which are usually located at the level of rhino-sinusal area and involving a different prognosis and management regarding cutaneous melanomas. We report a female patient case with anamnesis, clinical examination and NBI compatible with mucosal melanoma of left nostril. Surgical treatmentwas performed without adjuvant therapy, and there is no evidence of disease at one year post-monitoring.

http://ift.tt/2vkfbvG

Linfoma T/NK extraganglionar tipo nasal: Caso clínico

El linfoma T/NK extraganglionar tipo nasal es un linfoma extraganglionar, habitualmente expresa el fenotipo NK y VEB positivo. Cursa ocasionando necrosis y angioinvasión afectando de manera preferente estructuras mediofaciales. Característicamente es muy agresivo. Presentamos un caso con una sobrevida de siete meses a partir de los primeros síntomas y realizamos revisión de la literatura.

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Extranodal NK/T-cell lymphoma nasal type, is an extranodal lymphoma, usually with an NK-cell phenotype and EBV possitive. It causes necrosis and angioinvasion, and it is most commonly presenting in the midfacial area. Characteristically it is very aggressive. A case with survival of seven months from the first symptoms is reported and a review of the literature is made.

http://ift.tt/2vjSGXI

Cierre de fístula oroantral con injerto de hueso vómer

Presentamos un caso de fístula oroantral y rinosinusitis maxilar, resuelto por abordaje combinado endoscópico, nasal e intraoral en el cual se utilizó colgajo de mucosa palatina y hueso vómer para el cierre de la misma. Describimos el caso de una paciente femenina de 66 años de edad, que consultó por presentar cacosmia, algia facial izquierda y rinorrea posterior purulenta, 3 semanas posterior a extracción de segundo molar superior izquierdo, la tomograffa axial computarizada (TC) de senos paranasales evidenció velamiento total maxilar izquierdo, parcial etmoidal izquierdo y defecto óseo en reborde alveolar superior izquierdo. Se realizó toma de fragmento de hueso vómer. Seguidamente abordaje de cavidad antral izquierda por vía endoscópica; e intraoral, se concluyó disección, cierre óseo y mucoso de la fístula.

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We report a case of an oroantral fistula and maxillary rhinosinusitis, that was resolved by combined approach, in which palatal mucosa flap and vomer bone was used for its closure. We describe the case of a female patient of 66 years old, who consulted for having cacosmia, left facial pain and purulent rhinorrhea, after left second molar extraction. CT-scan sinus showed the total left maxillary sinus, partial left ethmoid opacity and bone defect in left alveolar ridge. A vomer bone graft was taken from the nasal septum; left maxillary sinus surgerywas done by endoscopic approach and intraoral closure of bony and mucosa fístula was concluded.

http://ift.tt/2vkvwk9

Carcinoma familiar de tiroides no medular: Reporte de un caso clínico y revisión de la literatura

El carcinoma tiroideo familiar no medular (CFTNM) representa aproximadamente entre el 3,2% y 9,6% de todos los cánceres de tiroides, y se define por la presencia de cáncer diferenciado de tiroides en 2 o más familiares, en ausencia de otros síndromes familiares conocidos o exposición a radiación. Si bien su fisiopatología es aún incierta, algunos investigadores postulan un patrón de herencia dominante con penetrancia incompleta, no habiendo aún un gen específico responsable. Esta entidad suele presentarse a una menor edad y con características más agresivas que en su forma esporádica. Dado el interés por conocer la presentación de esta enfermedad y las recomendaciones para su manejo, se presenta el caso de una paciente diagnosticada con cáncer papilar de tiroides con el antecedente de 4 familiares con la misma patología. Actualmente el tamizaje mediante ecografía cervical y biopsia por punción aspiración con aguja fina de los nódulos tiroideos es el examen de elección ante la presencia del antecedente de CFTNM, ya que aún no hay estudios genéticos disponibles. La tiroidectomía total más disección ganglionar es el tratamiento de elección. Debido al comportamiento más agresivo y peor pronóstico del CFTNM, es necesaria un alto índice de sospecha y una investigación completa en la presencia de un componente familiar.

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The non-familial medullary thyroid carcinoma (FNMTC) represents approximately between 3.2 and 9.6% of all thyroid cancers, and is defined by the presence of differentiated thyroid cancer in 2 or more families in the absence of other known familial syndromes or radiation exposure. Although the pathophysiology is still uncertain, some investigators posit a dominant pattern of inheritance with incomplete penetrance, but still there is no specific gene responsible. It occurs at a younger age and with more aggressive characteristics than the sporadic form. Because of the interest in learning about the presentation of this disease and its recommendations, we present the case of a patient diagnosed with papillary thyroid cancer with a history of 4 family with the same pathology. Actually cervical screening by ultrasound and the fine needle aspiration biopsy (FNAB) of thyroid nodules is the examination of choice in the presence of a history of FNMTC, since no genetic studies yet available. Total thyroidectomy with lymph node dissection is the treatment of choice. Because the more aggressive behavior and poor prognosis of FNMTC, a high index of suspicion and a full investigation is required in the presence of a familial component.

http://ift.tt/2vk2EZ9

Uso de probióticos en otorrinolaringología

En los humanos las infecciones más frecuentes son las respiratorias, siendo la principal indicación de antibióticos en niños. El uso indiscriminado de antibióticos lleva a la aparición de gérmenes multirresistentes. Uno de los objetivos actuales en salud es la prevención de las enfermedades infecciosas para disminuir el uso de antibióticos. Una estrategia postulada recientemente para prevenir infecciones respiratorias es el uso de probióticos.

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In humans, the most frequent infections are respiratory, being the main indication of antibiotics in children. The indiscriminate use of antibiotics leads to the emergence of multi-resistant germs. One of the current health objectives is the prevention of infectious diseases so we can reduce the use of antibiotics. A potential strategy for preventing respiratory infections is the use of probiotics.

http://ift.tt/2vke13s

Superviviencia en cáncer de laringe: A propósito de un artículo

En los humanos las infecciones más frecuentes son las respiratorias, siendo la principal indicación de antibióticos en niños. El uso indiscriminado de antibióticos lleva a la aparición de gérmenes multirresistentes. Uno de los objetivos actuales en salud es la prevención de las enfermedades infecciosas para disminuir el uso de antibióticos. Una estrategia postulada recientemente para prevenir infecciones respiratorias es el uso de probióticos.

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In humans, the most frequent infections are respiratory, being the main indication of antibiotics in children. The indiscriminate use of antibiotics leads to the emergence of multi-resistant germs. One of the current health objectives is the prevention of infectious diseases so we can reduce the use of antibiotics. A potential strategy for preventing respiratory infections is the use of probiotics.

http://ift.tt/2v0DzzP

A Cost Comparison for Telehealth Utilization in the Kidney Transplant Waitlist Evaluation Process.

Background: There have been limited publications on telehealth utilization in transplantation with no prior reports of telehealth-related costs for pretransplant evaluations. The aim of this study is to compare costs throughout the evaluation process for those patients assessed initially by telehealth to those seen in-person. Methods: All patients approved for kidney transplant waitlist evaluation at our center from March 2013 thru May 2016 with decisions were included in this study. Patients approved for evaluation were scheduled for either an initial telehealth or in-person visit, partly based on patient factors. Clinically-related and travel-related costs were calculated. Time estimates for patient time needed to complete visit, time from application approval to initial visit, and time from application approval to decision were obtained. Comparisons were made using t tests. Results: Thirty-nine months were included for 302 patients. All categories of clinically or travel-related costs were significantly less for the telehealth cohort (p

http://ift.tt/2u5Eou2

Refractory Vascular Rejection in a Hand Allograft in the Presence of Antibodies Against Angiotensin II (Type 1) Receptor.

No abstract available

http://ift.tt/2ulzWmh

Gender equity in Transplantation: A Report from the Women in Transplantation Workshop of the Transplantation Society of Australia and New Zealand.

The exponential growth of young talented females choosing science and medicine as their professional career over the past decade is substantial. Currently, more than half of the Australian medical doctoral graduates and early career researchers are comprised of women, but less than 20% of all academic professorial staff are female. The loss of female talent in the hierarchical ladder of Australian academia is a considerable waste of government investment, productivity and scientific innovation. Gender disparity in the professional workforce composition is even more striking within the field of transplantation. Women are grossly under-represented in leadership roles, with currently no female Heads of Unit in any of the Australian and New Zealand Transplanting centres. At the same time, there is also gender segregation with a greater concentration of women in lower-status academic position compared to their male counterparts. Given the extent and magnitude of the disparity, the Women In Transplantation Committee, a subcommittee of the Transplantation Society of Australia and New Zealand established a workshop comprising 8 female clinicians/scientists in transplantation. The key objectives were to i) identify potential gender equity issues within the transplantation workforce, ii) devise and implement potential strategies and interventions to address some of these challenges at a societal level, iii) set realistic and achievable goals to enhance and facility gender equality, equity and diversity in transplantation. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.

http://ift.tt/2u5HVIN

Aus der Gutachtenpraxis: Was der medizinische Gutachter über technische Akustik und Lärmmesstechnik wissen sollte

Laryngo-Rhino-Otol 2017; 96: 478-484
DOI: 10.1055/s-0043-104094

© Georg Thieme Verlag KG Stuttgart · New York

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Antibiotika-Therapien erhöhen das Risiko für eine chronische Rhinosinusitis

Laryngo-Rhino-Otol 2017; 96: 436-437
DOI: 10.1055/s-0043-105200

© Georg Thieme Verlag KG Stuttgart · New York

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Singultus – Diagnostik und Therapie

Laryngo-Rhino-Otol 2017; 96: 446-455
DOI: 10.1055/s-0043-104086

Der Schluckauf (Singultus) ist ein reflektorischer Bewegungsablauf mit diffus im Thorax verteilten Afferenzen und Efferenzen und kontrovers diskutierter funktioneller Relevanz. In seiner physiologischen Form handelt es sich um eine meist spontan sistierende Bagatellbeschwerde, die nur selten zum Arztkontakt führt. Ein anhaltender quälender Singultus kann jedoch zu einer erheblichen Beeinträchtigung der Lebensqualtiät führen. Ein solcher chronischer Singultus wird im Allgemeinen durch eine Dauer von mehr als 48 h definiert. Häufigste zugrundeliegende Erkrankung ist der gastroösophageale Reflux. Die weiteren Ursachen für einen chronischen Singultus sind vielfältig und betreffen multiple Organsysteme mit zum Teil schwerwiegenden Grunderkrankungen. In einigen Fällen können sich auch neurologische Erkrankungen mit einem Schluckauf präsentieren, zum Teil ist er sogar das einzige Symptom im Rahmen der Erstmanifestation. In der zerebralen Bildgebung findet sich dann häufig eine Läsion der Medulla oblongata. Eine Neuromyelitis optica und ein ischämischer Hirninfarkt mit Wallenbergsyndrom sind 2 häufigere zugrundeliegende neurologische Erkrankungen, aber auch andere entzündliche und vaskuläre Erkrankungen und Tumorerkrankungen des zentralen Nervensystems sind beschrieben. Für ein optimales evidenzbasiertes Management der Diagnostik und Therapie des chronischen Singultus gibt es keine hinreichenden Daten. Die Suche nach der Grunderkrankung erfordert oft ein interdisziplinäres Vorgehen von Internisten, Neurologen und HNO-Ärzten. Bereits vor Abschluss der Diagnostik oder bei nicht behebbarer Ursache kann eine symptomatische Therapie erforderlich sein. Der anhaltende Schluckauf stellt auch ein häufiges Problem in der onkologischen Palliativversorgung dar. Die medikamentöse Therapie des Singultus ist oft diffizil. Protonenpumpenhemmer oder Prokinetika bei gastroösophagealer Ursache bzw. Baclofen mit oder ohne Gabapentin bei anderer Ursache gehören dabei zu den Mitteln der ersten Wahl. Weitere Alternativen sind andere Antikonvulsiva, Neuroleptika, Antidepressiva und Kalziumantagonisten. Für den therapierefraktären Verlauf stehen grundsätzlich auch invasive Verfahren wie z. B. die selektive Phrenicusblockade zur Verfügung. Insgesamt wären mehr Studien zum Singultus wünschenswert, um der therapeutischen und diagnostischen Herausforderung gerecht zu werden, die dieses Symptom für Neurologen darstellen kann.
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© Georg Thieme Verlag KG Stuttgart · New York

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Leserbrief von Prof. Dr. med. Dr. med. dent. Dr. h. c. Ralf Siegert

Laryngo-Rhino-Otol 2017; 96: 440-441
DOI: 10.1055/s-0043-103502

© Georg Thieme Verlag KG Stuttgart · New York

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Fragen für die Facharztprüfung

Laryngo-Rhino-Otol 2017; 96: 497-498
DOI: 10.1055/s-0043-104095

© Georg Thieme Verlag KG Stuttgart · New York

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Antwort auf den Leserbrief

Laryngo-Rhino-Otol 2017; 96: 441-442
DOI: 10.1055/s-0037-1606176

© Georg Thieme Verlag KG Stuttgart · New York

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Spontanes retropharyngeales Hämatom mit letalem Ausgang

Laryngo-Rhino-Otol 2017; 96: 473-475
DOI: 10.1055/s-0043-112162

© Georg Thieme Verlag KG Stuttgart · New York

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Einlage eines tracheobronchialen Bifurkationsstents zur Behandlung ­malignombedingter tracheobronchialer Stenosen und Fisteln

Laryngo-Rhino-Otol 2017; 96: 443-444
DOI: 10.1055/s-0043-104092

© Georg Thieme Verlag KG Stuttgart · New York

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Laryngopharyngeale Refluxerkrankung (LPRD) bei Kindern

Laryngo-Rhino-Otol 2017; 96: 485-496
DOI: 10.1055/s-0043-109188

Zwischen 5 und 20 % aller Kinder leiden an einer gastroösophagealen Refluxerkrankung (GERD), die zu Erosionen, Ulzera und Metaplasien der Ösophagusschleimhaut führen kann. Überwindet der Mageninhalt in pathologischer Häufigkeit und Intensität auch den kindlichen oberen Ösophagussphinkter, liegt eine laryngopharyngeale Refluxerkrankung (LPRD) vor. Die mit einer kindlichen LPRD assoziierten Symptome und Folgeerkrankungen sind äußerst vielfältig.LPRD in children is characterized by symptoms, clinical findings, and sequelae caused by the reflux of gastric acid, bile acid or refluxate containing pepsin beyond the esophagus. For diagnostic procedures and therapy of gastroesophageal reflux disease (GERD) in children and adults widely accepted guidelines have been established. However, diagnosis and therapy of LPRD in children remains a continuous issue of inter- and intradisciplinary discussions. Although both GERD and LPRD in children are reflux-induced diseases, these two entities are different in symptoms, cause, diagnostic procedures, and therapy. Thus, the terms GERD and LPRD are not eligible to be used as synonyms.Otorhinolaryngologists are becoming more and more involved in the management of children with suspicious LPRD. With flexible transnasal laryngopharyngoscopy being one of the most important diagnostic tools for LPRD detection, otorhinolaryngologists play an important role in the interdisciplinary diagnostic network of physicians treating children with suspected LPRD. The present article highlights age-dependent clinical symptoms, diagnostic tools, differential diagnoses, and adequate therapy for pediatric LPRD.
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© Georg Thieme Verlag KG Stuttgart · New York

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Chirurgie der inneren Nase

Laryngo-Rhino-Otol 2017; 96: 499-502
DOI: 10.1055/s-0043-104090

© Georg Thieme Verlag KG Stuttgart · New York

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Kommentar der Schriftleitung

Laryngo-Rhino-Otol 2017; 96: 433-434
DOI: 10.1055/s-0043-104084

Liebe Leserinnen und Leser,genau, Sie lesen richtig: Ich bin der Neue.Nach mehreren Jahren sehr erfolgreicher Schriftleitungstätigkeit durch meinen hochgeschätzten, lieben Kollegen Orlando Guntinas-Lichius habe ich jetzt mit diesem Juli-Heft die federführende Schriftleitung innerhalb unseres aus Stefan Dazert, Orlando Guntinas-Lichius, Gerd Rasp und mir bestehenden Schriftleitungsteams übernommen. Ich möchte bei dieser Gelegenheit Orlando Guntinas-Lichius im Namen der gesamten Schriftleitung meinen herzlichen Dank für die ausgezeichnete Arbeit der letzten Jahre aussprechen.
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© Georg Thieme Verlag KG Stuttgart · New York

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An unbiased in vivo functional genomics screening approach in mice identifies novel tumor cell-based regulators of immune rejection

Abstract

The clinical successes of immune checkpoint therapies for cancer make it important to identify mechanisms of resistance to anti-tumor immune responses. Numerous resistance mechanisms have been identified employing studies of single genes or pathways, thereby parsing the tumor microenvironment complexity into tractable pieces. However, this limits the potential for novel gene discovery to in vivo immune attack. To address this challenge, we developed an unbiased in vivo genome-wide RNAi screening platform that leverages host immune selection in strains of immune-competent and immunodeficient mice to select for tumor cell-based genes that regulate in vivo sensitivity to immune attack. Utilizing this approach in a syngeneic triple-negative breast cancer (TNBC) model, we identified 709 genes that selectively regulated adaptive anti-tumor immunity and focused on five genes (CD47, TGFβ1, Sgpl1, Tex9 and Pex14) with the greatest impact. We validated the mechanisms that underlie the immune-related effects of expression of these genes in different TNBC lines, as well as tandem synergistic interactions. Furthermore, we demonstrate the impact of different genes with previously unknown immune functions (Tex9 and Pex14) on anti-tumor immunity. Thus, this innovative approach has utility in identifying unknown tumor-specific regulators of immune recognition in multiple settings to reveal novel targets for future immunotherapies.

http://ift.tt/2uWUapJ

Thyroid Open Access Articles

OPEN ACCESS
Read now:

Latest Impact Factor: 5.515
The Official Journal of: American Thyroid Association

No Effect of Levothyroxine and Levothyroxine-induced Subclinical Thyrotoxicosis on the Pharmacokinetics of Sorafenib in Healthy Male Subjects
Funan Huang, Antoinette Ajavon-Hartmann, Erya Huang, John Lettieri, Rong Liu, Carol Pena, Matthias Berse

Prevalence and characterization of thyroid hemiagenesis in Japan: The Fukushima Health Management Survey
Satoru Suzuki, Sanae Midorikawa , Takashi Matsuzuka, Toshihiko Fukushima, Yuko Ito, Hiroki Shimura, Hideto Takahashi, Tetsuya Ohira, Akira Ohtsuru, Masafumi Abe, Shinichi Suzuki, Shunichi Yamashita

Sorafenib in Japanese Patients with Locally Advanced or Metastatic Medullary Thyroid Carcinoma and Anaplastic Thyroid Carcinoma
Yasuhiro Ito, Naoyoshi Onoda, Ken-ichi Ito, Iwao Sugitani, Shunji Takahashi, Iku Yamaguchi, Koki Kabu, Katsuya Tsukada

Defining Radioiodine-refractory Differentiated Thyroid Cancer: Efficacy and Safety of Lenvatinib by Radioiodine-refractory Criteria in the SELECT Trial
Naomi Kiyota, Bruce Robinson, Manisha H Shah MD, Ana O. Hoff, Matthew Taylor, Di Li, Corina E. Dutcus, Eun Kyung Lee, Sung-Bae Kim, Makoto Tahara

The post Thyroid Open Access Articles appeared first on American Thyroid Association.

http://ift.tt/2vul7To

Multicenter Benchmark Study Reveals Significant Variation in Thyroid Testing in the United States

Thyroid , Vol. 0, No. 0.


http://ift.tt/2uZSMRz

Conventional osteosarcoma of the mandible successfully treated with radical surgery and adjuvant chemotherapy after responding poorly to neoadjuvant chemotherapy: a case report

Osteosarcoma, the most common primary bone malignancy, has an extremely poor prognosis and a high rate of local recurrence and distal metastases. Because osteosarcomas of the head and neck region are rare, acc...

http://ift.tt/2uZCKYf

Age Range Error in Key Points, Abstract, Introduction, and Figure Caption

In the Original Investigation titled "Trends in Nationwide Herpes Zoster Emergency Department Utilization From 2006 to 2013," published online on June 21, 2017, there was an age range error repeated in the Key Points, Abstract, Introduction, and Figure 2 caption. The range given as "18 to 19" should have been "less than 20." This article was corrected online.

http://ift.tt/2uWM00y

Patient Perceptions of Primary Care–Based Skin Cancer Screening

This analysis of a series of interviews seeks to better understand patient perceptions of primary care–based skin cancer screening.

http://ift.tt/2w6If7z

Treatment of Hailey-Hailey Disease With Low-Dose Naltrexone

This case series investigates whether low-dose naltrexone is an effective treatment for recalcitrant Hailey-Hailey disease.

http://ift.tt/2uW3kmq

Repurposing Medications

My dad grew up on a chicken farm. One winter in my youth, while at an extended family gathering in his little farm town in mid-Missouri, I was complaining to him about my severe chapped lips. "Well, farmers around here would tell you to put chicken [manure] on chapped lips. That's a sure cure." Looking around the circle of conversation, I saw several relatives, farmers, and locals affirming this statement. For the next dozen years I was perplexed by the recommendation and silent about any further lip concerns.

http://ift.tt/2w6G4AZ

Secukinumab-Induced Psoriasiform Eruption

This case report describes a patient with secukinumab-induced psoriasiform eruption.

http://ift.tt/2uWwjXt

Naltrexone Treatment of Familial Benign Pemphigus (Hailey-Hailey Disease)

This case series evaluates whether low-dose naltrexone is efficacious and safe in the treatment of recalcitrant familial benign pemphigus (Hailey-Hailey disease).

http://ift.tt/2w6VeG6

Risk prediction instruments in geriatric surgery are available but often ignored

No abstract available

http://ift.tt/2viKiYu

Compressed air injection technique for Shamrock lumbar plexus block

No abstract available

http://ift.tt/2viVJiI

History of anaesthesia: The ketamine story – past, present and future

No abstract available

http://ift.tt/2vj6oKs

Rocuronium is more hepatotoxic than succinylcholine in vitro

BACKGROUND: The development of liver failure is a major problem in critically ill patients. The hepatotoxicity of many drugs, as one important reason for liver failure, is poorly screened for in human models. Rocuronium and succinylcholine are neuromuscular blocking agents used for tracheal intubation and for rapid-sequence induction. OBJECTIVE: We used an in-vitro test with a permanent cell line and compared rocuronium and succinylcholine for hepatotoxicity. DESIGN: In-vitro study. SETTING: A basic science laboratory, University Hospital Rostock, Germany. MATERIAL/(PATIENTS): The basic test compound is the permanent human liver cell line HepG2/C3A. In a standardised microtitre plate assay the toxicity of different concentrations of rocuronium, succinylcholine and plasma control was tested. INTERVENTIONS: After two incubation periods of 3 days, the viability of cells (XTT test, lactate dehydrogenase release and trypan blue staining), micro-albumin synthesis and the cytochrome 1A2 activity (metabolism of ethoxyresorufin) were measured. MAIN OUTCOME MEASURES: Differences between rocuronium and succinylcholine were assessed using the Kruskal–Wallis one-way test and two-tailed Mann–Whitney U test. RESULTS: Rocuronium, but not succinylcholine, led to a significant dose-dependent decrease of viability, albumin synthesis and cytochrome 1A2 activity of test cells. CONCLUSION: An in-vitro test with a cell line showed hepatotoxicity of rocuronium that was dose-dependent. Further studies are needed to investigate the underlying mechanisms of the effects of rocuronium on hepatic cellular integrity. TRIAL REGISTRATION: Not suitable.

http://ift.tt/2viJmmU

Efficacy and safety of buprenorphine in peripheral nerve blocks: A meta-analysis of randomised controlled trials

BACKGROUND: The duration of analgesia provided by nerve blocks is limited if local anaesthetics are administered alone. Therefore, a variety of additives to local anaesthetics have been investigated to prolong postoperative analgesia following single-shot nerve blocks. OBJECTIVE(S): The aims of the current meta-analysis were to assess the efficacy and safety of the addition of perineural buprenorphine to local anaesthetic compared with local anaesthetic alone, or combined with systemic administration of buprenorphine, or other perineural opioids for peripheral nerve blocks. DESIGN: Systematic review and meta-analysis of randomised controlled trials (RCTs). DATA SOURCES: The following data sources were systematically searched: MEDLINE, CENTRAL and EMBASE (till 03/2016). ELIGIBILITY CRITERIA: All RCTs focusing on the efficacy and safety of perineural buprenorphine combined with local anaesthetic compared with local anaesthetic alone, or in combination with systemic buprenorphine, or other perineural opioids for peripheral nerve blocks were included. RESULTS: We included 13 RCTs (685 patients). Participants treated with perineural buprenorphine combined with local anaesthetic showed a longer duration of analgesia compared with those receiving local anaesthetic alone [mean difference 8.64 h, 95% confidence interval (CI) (6.44 to 10.85); P 

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Postoperative nausea and vomiting: solutions and questions

No abstract available

http://ift.tt/2uZxcNl

Injectate spread following anterior sub-costal and posterior approaches to the quadratus lumborum block: A comparative cadaveric study

BACKGROUND: The dermatomal level of analgesia achieved with quadratus lumborum blocks varies according to the location of injection. The most commonly used approaches are either at the postero-lateral aspect or anterior to the quadratus lumborum muscle. OBJECTIVE: To determine whether the site of injection of contrast dye around the quadratus lumborum muscle of cadavers affects the extent and mechanism of dye spread. DESIGN: Observational human cadaver study. SETTING: Cleveland Clinic cadaveric laboratory. PARTICIPANTS: Six fresh human cadavers. INTERVENTIONS: The cadavers received either a posterior quadratus lumborum block or an anterior subcostal quadratus lumborum block on each side. MAIN OUTCOME MEASURES: Cadavers were dissected to determine the extent of dye spread. RESULTS: The posterior quadratus lumborum block approach revealed consistently deep staining of the iliohypogastric, ilioinguinal, subcostal nerve, T11 to 12 and L1 nerve roots. In addition, staining of the middle thoracolumbar fascia was seen in all specimens but only variable staining of T10 nerve roots. The anterior subcostal quadratus lumborum block approach in all specimens demonstrated predictable deep staining of the iliohypogastric and ilioinguinal nerves, subcostal nerves, T11 to 12 and L1 nerve roots, and in addition traversing the arcuate ligaments to involve T9 to 12 nerve roots with variable staining of higher thoracic nerve roots. CONCLUSIONS: Our cadaveric study demonstrates that injection of dye on the posterior aspect of quadratus lumborum muscle led to injectate spread through the lateral and posterior abdominal wall but with limited cranial spread, whereas the anterior approach produced broader coverage of the lower to mid-thoracic region. Clinical translation of these findings to determine the practical significance is warranted.

http://ift.tt/2uZeCoE

Anaesthesiology and ethics: Presumed consent with real consequences

No abstract available

http://ift.tt/2vjoWdH

Thoracic paravertebral block for postoperative pain management after renal surgery: A randomised controlled trial

BACKGROUND: Thoracic paravertebral block (ThPVB) combined with general anaesthesia is used in thoracic and general surgery. It provides effective analgesia, reduces surgical stress response and the incidence of chronic postoperative pain. OBJECTIVE: To assess the efficacy of ThPVB in reducing opioid requirements and decreasing the intensity of pain after renal surgery. DESIGN: A randomised, open label study. SETTING: A single university hospital. Study conducted from August 2013 to February 2014. PARTICIPANTS: In total, 68 patients scheduled for elective renal surgery (open nephrectomy or open nephron-sparing surgery). INTERVENTIONS: Preoperative ThPVB with 0.5% bupivacaine combined with general anaesthesia, followed by postoperative oxycodone combined with nonopioid analgesics as rescue drugs. Follow-up period: 48 h. MAIN OUTCOME MEASURES: Total dose of postoperative oxycodone required, pain intensity, occurrence of opioid related adverse events, ThPVB-related adverse events and patient satisfaction. RESULTS: A total of 68 patients were randomised into two groups and, of these, 10 were subsequently excluded from analysis. Patients in group paravertebral block (PVB; n = 27) had general anaesthesia and ThPVB, and those in group general (anaesthesia) (GEN) (n = 31) formed a control group receiving general anaesthesia only. Compared with patients in group GEN, patients who received ThPVB required 39% less i.v. oxycodone over the first 48 h and had less pain at rest (P 

http://ift.tt/2viKcjA

Plasma levels of local anaesthetic following supraclavicular block

No abstract available

http://ift.tt/2viRLqo

Loss of resistance: A randomised controlled trial assessing four low-fidelity epidural puncture simulators

BACKGROUND: Detecting loss of resistance (LOR) can either be taught with dedicated simulators, with a cost ranging from €1500 to 3000, or with the 'Greengrocer's Model', requiring simply a banana. OBJECTIVES: The purpose of this study was to compare three dedicated epidural puncture training simulators and a banana in their ability to simulate LOR. Our hypothesis was that there was a difference between the four simulators when comparing the detection of LOR. DESIGN: Single-blinded, randomised, controlled study. SETTING: Department of Anaesthesiology and Pain Therapy, Bern University Hospital, Switzerland. PARTICIPANTS: Fifty-five consultant anaesthesiologists. INTERVENTIONS: The participants were asked to insert an epidural catheter in four different epidural puncture training simulators: Lumbar Puncture Simulator II (Kyoto Kagaku, Kyoto, Japan), Lumbar Epidural Injection Trainer (Erler-Zimmer, Lauf, Germany), Normal Adult Lumbar Puncture/Epidural Tissue (Simulab Corp., Seattle, Washington, USA) and a banana. The simulators were placed in identical boxes to blind the participants. MAIN OUTCOME MEASURES: The primary outcome was the detection of LOR rated on a 100-mm visual analogue scale, in which 0 mm represented 'completely unrealistic' and 100 mm represented 'indistinguishable from a real patient'. RESULTS: The mean visual analogue scale scores for LOR in the four simulators were significantly different: 60 ± 25 mm [95% confidence interval (CI), 55 to 65 mm], 50 ± 29 mm (95% CI, 44 to 55 mm), 64 ± 24 mm (95% CI, 58 to 69 mm) and 49 ± 32 mm (95% CI, 44 to 54 mm); P less than 0.001, Friedman test. CONCLUSION: Two of the three dedicated epidural simulators were rated more realistic in detecting LOR than the banana, but some participants preferred the banana to the other three simulators. Given the relative cost of a banana compared with a dedicated simulator, we suggest that a banana be used to teach the technique of LOR for epidural puncture. TRIAL REGISTRATION: KEK Nr: Req-2015-z087.

http://ift.tt/2vj0yZo

Should the transversus abdominis plane block be performed for laparoscopic colorectal surgery?

No abstract available

http://ift.tt/2uZ9osJ

Impact of reversal strategies on the incidence of postoperative residual paralysis after rocuronium relaxation without neuromuscular monitoring: A partially randomised placebo controlled trial

BACKGROUND: Electronic neuromuscular monitoring is not widely used to determine either the reversal requirements for neuromuscular block before extubation of the trachea, or to determine if there is any subsequent postoperative residual neuromuscular block (PORNB). OBJECTIVES: To investigate the incidence of PORNB using acceleromyography after spontaneous recovery of rocuronium-induced block and to compare this with the administration of sugammadex, neostigmine or a placebo. DESIGN: Partially randomised, partially randomised, placebo-controlled, double-blind, four-group parallel-arm study. SETTING: Single-centre study performed between October 2013 and December 2015 in a university hospital. PATIENTS: Of the 134 eligible patients, 128 gave their consent and 125 of these completed the study. INTERVENTIONS: Patients received general anaesthesia with propofol, sevoflurane, fentanyl and rocuronium. Neuromuscular transmission was measured by acceleromyography (TOF-Watch-SX; Organon Teknika B.V., Boxtel, the Netherlands) but the anaesthetist was blind to the results. If the anaesthetist deemed pharmacological reversal to be necessary before extubation of the trachea then patients were assigned randomly to receive either sugammadex (2.0 mg kg−1), neostigmine (0.05 mg kg−1) or a placebo. In the postanaesthesia care unit, an independent anaesthetist, unaware of the treatment given, assessed the neuromuscular function using acceleromyography. MAIN OUTCOME MEASURES: The incidence of a normalised train-of-four ratio less than 0.9 on arrival in the recovery room. RESULTS: In total, 125 patients were recruited. Neuromuscular block was allowed to recover spontaneously in 50 patients, whereas the remainder received either sugammadex (27), neostigmine (26) or placebo (22). The number of cases with PORNB were one (3.7%), four (15%), 13 (26%) and 10 (45%) after sugammadex, neostigmine, spontaneous recovery and placebo, respectively. Sugammadex and neostigmine were more effective than placebo [odds ratio (OR): 0.05, 95% confidence interval (CI): 0.005 to 0.403, P = 0.005; OR: 0.22, 95% CI: 0.056 to 0.85, P = 0.028, respectively]. Sugammadex performed better than spontaneous recovery (OR: 0.11, 95% CI: 0.014 to 0.89, P = 0.039) unlike neostigmine (OR: 0.52, 95% CI: 0.15 to 1.79, P = 0.297). Yet, antagonism (pooled data) was more effective than spontaneous recovery (OR: 0.3, 95% CI: 0.1 to 0.9, P = 0.03). CONCLUSION: Although pharmacological reversal based on clinical signs was superior to spontaneous recovery it did not prevent PORNB, irrespective of the reversal agent. TRIAL REGISTRATION: The study is registered under EUDRACT number 2013-001965-17.

http://ift.tt/2uZcux4

Reply to: postoperative nausea and vomiting: solutions and questions

No abstract available

http://ift.tt/2vjoTi1

Effects of calcium chloride coadministered with neostigmine on neuromuscular blockade recovery: A double-blind randomised study

BACKGROUND: Ionised calcium plays an important role in neuromuscular transmission, but its effects on the reversal of nondepolarising neuromuscular blockade have not been fully evaluated. OBJECTIVE: We examined whether calcium chloride coadministered with neostigmine could enhance the rate of neuromuscular recovery. DESIGN: Randomised double-blind trial. SETTING: A tertiary teaching hospital. PATIENTS: In total, 53 patients undergoing elective surgery under general anaesthesia with neuromuscular monitoring by acceleromyography using a TOF-Watch SX monitor. INTERVENTIONS: Patients were randomly allocated to receive either 5 mg kg−1 of calcium chloride (calcium group, n = 26) or the same volume of normal saline (control group, n = 27) coadministered with 25 μg kg−1 of neostigmine and 15 μg kg−1 of atropine at the end of surgery. MAIN OUTCOME MEASURES: The primary end point was the neuromuscular recovery time [time from neostigmine administration to recovery of the TOF ratio (TOFr) to 0.9]. Secondary end points included the TOFr at 5, 10 and 20 min after neostigmine administration and the incidence of postoperative residual curarisation (PORC), defined as a TOFr less than 0.9 at each time point. RESULTS: The neuromuscular recovery time was significantly faster in the calcium group than in the control group (median [Q1 to Q3]; 5.0 [3.0 to 7.0] vs. 6.7 [5.7 to 10.0] min, respectively; P = 0.007). At 5 min after neostigmine administration, the TOFr was higher [87 (74 to 100) vs. 68 (51 to 81)%, respectively; P = 0.002] and the incidence of PORC was lower (50.0 vs. 81.5%, respectively; P = 0.016) in the calcium group than in the control group. There were no differences between the two groups with respect to the TOFr or incidence of PORC at 10 and 20 min after neostigmine administration. CONCLUSION: Calcium chloride coadministered with neostigmine enhanced neuromuscular recovery in the early period of nondepolarising neuromuscular blockade reversal.

http://ift.tt/2viNyTS

Reply to: risk prediction instruments in geriatric surgery are available but often ignored

No abstract available

http://ift.tt/2uZqtD9

Familial aggregation of nasopharyngeal carcinoma in Taiwan

Publication date: October 2017
Source:Oral Oncology, Volume 73
Author(s): Shiang-Fu Huang, Ren-Hao Hsiao, Chi-Kuan Young, Hui-Tzu Chien, Chang-Fu Kuo, Lai-Chu See, Shue-Fen Luo, Lu-Hsiang Huang, Chun-Ta Liao, Tung-Chieh Joseph Chang
BackgroundThe incidence of nasopharyngeal carcinoma (NPC) is higher in Chinese than in Caucasian populations. Genetic, viral, and lifestyle factors may explain these ethnic differences in the incidence of NPC. In the present study, we examined the familial aggregation, heritability, and relative risks (RRs) of NPC using a nationwide database in Taiwan.MethodsA population-based family study was conducted using the Taiwan National Health Insurance Research Database. Participants included all individuals (N=23,422,955) registered with that database in 2013; of these, 17,653 had NPC. Among them, 47.45%, 57.45%, 47.29%, and 1.51% had a parent, child, sibling, and twin, respectively, with NPC.ResultsAmong the approximately 23 million Taiwan NHI beneficiaries in 2013, the relative risks (RRs) (95% confidence intervals) for NPC were 34.46 (5.12–231.77) for twins of the patients, 9.23 (6.34–13.43) for siblings, 3.80 (2.97–4.86) for parents, 3.74 (2.60–5.37) for offspring, and 1.78 (1.16–2.74) for spouses without genetic similarity. The mean age of onset in first-degree relative-affected NPC patients was 35.5years compared to 39.0years for NPC patients without affected first-degree relatives (p≤0.0001). Using a threshold liability model, the accountability for phenotypic variance of NPC was estimated to be 61.3% for genetic factors (heritability), 13.9% for shared environmental factors, and 24.8% for non-shared environmental factors. The probability of a patient with NPC to be sporadic was 82.8%.ConclusionThis population-based analysis suggested a strong familial tendency in the development of NPC. Screening of first-degree relatives of NPC patients is recommended, particularly in endemic regions.



http://ift.tt/2tZU124

Comprehensive Cancer Center in Deutschland

Zusammenfassung

Hintergrund

In den USA haben sich seit den 1970er Jahren die Comprehensive Cancer Center (CCC) als erfolgreiche Organisationsform durchgesetzt und zum Erfolg in der translationalen und klinischen Krebsforschung sowie in der interdisziplinären Patientenversorgung beigetragen.

Ergebnisse

In Deutschland wurden die ersten CCC vor 15 Jahren aufgebaut. In den folgenden Jahren wurden – mit wenigen Ausnahmen – an allen Universitätskliniken CCC gegründet. Die CCC bündeln die multidisziplinäre Patientenversorgung, die Aus- und Weiterbildung, die translationale und klinische Forschung sowie die regionale Vernetzung in der Onkologie.

http://ift.tt/2u09C1r

Épilation génitale intégrale : un rôle-clef pour le dermatologue dans la prévention des IST ?

Publication date: Available online 1 August 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): F. Desruelles




http://ift.tt/2f82o9m

Pseudomonas aeruginosa en dermatologie

Publication date: Available online 2 August 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): A. Morand, J.-J. Morand
Pseudomonas aeruginosa, microorganisme bacille Gram négatif ubiquitaire, caractérisé par sa couleur verdâtre et son odeur douceâtre, est à l'origine de dermatoses potentiellement sévères comme l'ecthyma gangrenosum qui signe une immunodépression ou révèle une septicémie notamment chez l'enfant. Cette bactérie colonise fréquemment les plaies chroniques ou brûlures graves, les dermatoses spongiotiques ou acantholytiques surtout lorsqu'elles sont étendues et localisées dans les plis. Elle nécessite une prise en charge particulière du fait de sa forte capacité de résistance aux antibiotiques et antiseptiques. Elle peut aussi se traduire par une folliculite favorisée par les sports aquatiques ou une atteinte unguéale (chloronychie).Pseudomonas aeruginosa, a ubiquitous Gram-negative bacillus characterized by its greenish color and sweetish smell, is at the origin of potentially severe forms of dermatosis, such as ecthyma gangrenosum which marks immunosuppression or reveals blood-poisoning, especially in children. It frequently colonizes chronic wounds and serious burns, and spongiotic or acantholytic dermatosis, especially when severe or localized in skinfolds. It requires special care because of its high resistance to antibiotics and antiseptics. It can also involve folliculitis favored by water sports or a nail disorder (chloronychia).



http://ift.tt/2hnYREl

M. Menière: Diagnostische Kriterien des Internationalen Klassifikationskomitees der Bárány-Gesellschaft

Zusammenfassung

Es werden die diagnostischen Kriterien für M. Menière beschrieben, die vom Klassifikationskomitee der Bárány-Gesellschaft, der Japanischen Gesellschaft für Gleichgewichtsforschung, der Europäischen Akademie für Otologie und Neurootologie (EAONO), dem Komitee für Gleichgewichtsstörungen der Amerikanischen Akademie für Otolaryngologie, Kopf- und Hals-Chirurgie (AAO-HNS) sowie der Koreanischen Gesellschaft für Gleichgewichtsstörungen erarbeitet worden sind. Diese Klassifikation enthält 2 diagnostische Kategorien: M. Menière und wahrscheinlicher M. Menière. Die Diagnose eines definitiven M. Menière basiert auf klinischen Kriterien und erfordert rezidivierende episodische Schwindelsymptome, die mit einer sensorineuralen Hörminderung im niedrigen bis mittleren Frequenzbereich assoziiert sein müssen sowie mit fluktuierenden Symptomen (Hörminderung, Tinnitus und/oder Völlegefühl) im betroffenen Ohr. Die Dauer der Attacken liegt zwischen 20 min und 12 h. Ein wahrscheinlicher M. Menière ist definiert als episodische Schwindelsymptome (Dreh- oder Schwankschwindel), assoziiert mit fluktuierenden Ohrsymptomen und einer Dauer von 20 min bis 24 h.

http://ift.tt/2uVG0VS

Therapie der Mycosis fungoides und des Sézary-Syndroms

Zusammenfassung

Die Therapie kutaner T‑Zell-Lymphome beruht auf einer exakten Einordnung der klinischen Entitäten anhand der aktuellen WHO-Klassifikation. Kurative Therapieansätze existieren aktuell nicht, daher zielt die Behandlung auf die Verbesserung der Symptomatik und Lebensqualität sowie die Reduktion der Rezidivhäufigkeit ab und ist stadiengerecht durchzuführen. Hierzu stehen sowohl hautgerichtete als auch systemische Therapien zur Verfügung. Die Phototherapie und Lokalsteroide stellen nach wie vor Verfahren der ersten Wahl zur Behandlung früher Stadien dar. Als systemische Therapeutika stehen u. a. rekombinantes Interferon-α-2a und das Retinoid-X-Rezeptor(RXR)-spezifische Retinoid Bexaroten als Erstlinienmedikamente sowie Monochemotherapeutika wie Gemcitabin und das liposomal verkapselte Doxorubicin in Zweitlinienindikation zur Verfügung. Aufgrund der häufigen Rezidive in fortgeschrittenen Stadien sind neue Alternativen notwendig. Hier erscheint insbesondere das gegen das CD30-Molekül gerichtete Fusionsprotein Brentuximab Vedotin vielversprechend.

http://ift.tt/2ukhudx

Bronchial thermoplasty induces immunomodulation with a significant increase in pulmonary CD4+25+ regulatory T cells

Publication date: Available online 1 August 2017
Source:Annals of Allergy, Asthma & Immunology
Author(s): Mateja Marc Malovrh, Aleš Rozman, Sabina Škrgat, Mira Šilar, Julij Šelb, Matjaž Fležar, Peter Korošec




http://ift.tt/2wlE7jn

Increased malignancy incidence in IgE deficient patients not due to concomitant Common Variable Immunodeficiency

Publication date: Available online 1 August 2017
Source:Annals of Allergy, Asthma & Immunology
Author(s): Denisa Ferastraoaru, Rebecca Gross, David Rosenstreich
BackgroundImmunoglobulin E (IgE) deficiency (<2.5 kU/L) has unclear clinical significance. Very little is known about the clinical characteristics of IgE deficiency in patients with Common Variable Immunodeficiency (CVID).ObjectiveTo evaluate the clinical and laboratory differences between patients with IgE deficiency and those with non-IgE deficiency with and without CVID diagnosis.MethodsThis is a retrospective study of adult patients who had total serum IgE levels measured at our facility from 2010 through 2015. Patients with IgE levels lower than 2.5 kU/L composed the IgE deficiency group. We used Clinical Looking Glass software to identify laboratory results and comorbid conditions including CVID and malignancy.ResultsThe IgE levels were measured in 2,339 patients and 63 (2.7%) had IgE deficiency. Of those with IgE deficiency, 14 of 63 (22%) had CVID diagnosis compared with only 62 of 2,276 patients (2.7%) with non-IgE deficiency and CVID. A significantly higher rate of prior malignancy was found in patients with IgE deficiency (21 of 63, 33%) compared with those with non-IgE deficiency (197 of 2,276, 8.7%; P = .001; odds ratio 5.51, 95% confidence interval 3.07–9.88). Six of 14 patients with CVID and IgE deficiency (43%) had a prior malignancy diagnosis compared with 8 of 62 patients (13%) with CVID and non-IgE deficiency (P = .009; odds ratio 10.65, 95% confidence interval 1.79–63.19). In addition to the higher rate of malignancy, patients with CVID and IgE deficiency did not have more severe disease than those with CVID and non-IgE deficiency.ConclusionThe rate of prior malignancy is significantly higher in patients with IgE deficiency than in those without IgE deficiency. Similarly, patients with CVID and IgE deficiency have a higher frequency of prior malignancy than those with CVID and non-IgE deficiency. However, patients with IgE deficiency have higher frequency of malignancy than patients with normal IgE levels even in the absence of CVID.



http://ift.tt/2vta9gP

Zika virus infection as a trigger of atopic dermatitis

Publication date: Available online 1 August 2017
Source:Annals of Allergy, Asthma & Immunology
Author(s): Wilfredo Cosme-Blanco, Erving Arroyo-Flores, Sylvette Nazario




http://ift.tt/2vsZse8

Hypogammaglobulinemia E in 216 adults with IgG subclass deficiency and respiratory tract infections

Publication date: Available online 1 August 2017
Source:Annals of Allergy, Asthma & Immunology
Author(s): James C. Barton, J. Clayborn Barton, Luigi F. Bertoli




http://ift.tt/2vtoIB8

miRNA – rola i znaczenie w astmie oskrzelowej

Publication date: Available online 1 August 2017
Source:Alergologia Polska - Polish Journal of Allergology
Author(s): Maksymilian Ziaja, Rafał Pawliczak
Chronic inflammatory airway diseases, including asthma, are a significant health problem and previous treatment methods seem to be insufficient. There is a lack of effective therapies which are adjusted to treat all classes in terms of severity, and it is probably caused by insufficient understanding of molecular mechanisms involved in its occurrence. Small non-coding microRNA molecules in response to environmental factors lead to modulate gene expression. They regulate many processes associated with physiologic functions of the immune system. Researches showed crucial role of miRNA in pathogenesis of asthma and the other chronic inflammatory airway diseases. Despite the link of microRNA with pathogenesis of inflammatory airway diseases, the role of these molecules is still unclear. More detailed knowledge of the impact of miRNA molecules on the development of bronchial asthma would allow us to better understand the genesis process and might influence the effectiveness of the bronchial asthma treatment.



http://ift.tt/2u00j1K

Role of metastasectomy and chemotherapy in carcinoma of uterine cervix

Squamous cell carcinoma of uterine cervix is potentially a curable disease; however, many patients treated with definitive chemoradiotherapy develop distant metastases, with few of them having a single metastatic deposit. There are no guidelines for the treatment of patients with oligometastatic cervical cancer.

We present a case of a patient with International Federation of Gynecology and Obstetrics (FIGO) Stage IIB squamous cell carcinoma of uterine cervix. She was successfully treated with concurrent chemoradiotherapy with definitive intent. One year later, she developed a solitary pulmonary nodule for which she underwent resection followed by chemotherapy. She is free of any local or distant disease at 5 years of regular follow-up.

http://ift.tt/2u02Tod

Diagnosis of acrodermatitis enteropathica in resource limited settings

Acrodermatitis enteropathica (AE) is a rare inherited zinc deficiency that usually manifests in infancy within days in cases of bottlefed infants and days to weeks after weaning in breastfed infants. It is characterised by diarrhoea, dermatitis, alopecia and systemic symptoms. We report a case of acquired nutritional AE in a 6-month-old female infant who had diarrhoeal episodes and the characteristic dermatitis lesions in the acral and anogenital regions. She responded dramatically to oral zinc supplementation.

http://ift.tt/2voNXDD

Paget-Schroetter syndrome complicated by an incidental pulmonary embolism

A young man presented with the severe right upper limb swelling following a heavy weight lifting that was thought to be caused by a biceps tendon rupture. However, subsequent investigations confirmed the diagnosis of Paget-Schroetter syndrome that was associated with an incidental pulmonary embolism. The patient underwent a successful thrombolysis followed by a surgical thoracic outlet decompression. Overall, the patient has made a good recovery.

http://ift.tt/2tZFYK1

Self-medication complicating pseudo membranous conjunctivitis

We aim to highlight the prevalence of using local medications for curing eye ailments in a rural population and the harmful consequence of resultant disease, ranging from something as mild as conjunctivitis to sight-threatening conditions. This case report will address one such incidence where in a local village, the doctor known as a registered medical practitioner) in the rural community, uses local remedies for his conjunctivitis and ends up in a condition where he cannot see anything beyond perceiving light. The membrane removal and treatment with topical antibiotic cured his infection, restoring complete recovery of vision. This incidence inspired the local village doctor to become an eye health educator. He was motivated to spread awareness about the ill effects of self-medication on eye health. He is also determined to bring all blind people and others who need eye care from his remote village to our centre.

http://ift.tt/2voBi3T

An illusion of septic shock: acute generalised exanthematous pustulosis with multiorgan dysfunction

Acute generalised exanthematous pustulosis (AGEP) is a rare cutaneous eruption, most often caused by commonly used antibiotics. It is characterised by an acute onset of non-follicular sterile pustular rash and erythema within hours or days of drug exposure and usually resolves spontaneously within 1–2 weeks once the drug is discontinued. Haemodynamic involvement in the form of shock is rare. Here, we present a severe case of AGEP, manifesting with systemic involvement and haemodynamic instability resulting in shock with multiorgan dysfunction. The associated drugs were erythromycin and fluconazole with a possible combined effect of these two drugs that resulted in systemic involvement. Our patient improved markedly, both haemodynamically and dermatologically, after discontinuation of the drugs and with systemic steroid therapy.

http://ift.tt/2u02S3D

Percutaneous valve in valve in the tricuspid position in a patient with Tetralogy of Fallot

Here, we describe a case of a successful percutaneous insertion of a transcatheter 29 mm Edwards Sapien XT valve into a tricuspid valve in a patient with repaired tetralogy of fallot.

Similar procedures have been performed with the Edwards Sapien valve and Melody valves; however, this is the first case described in the literature of an Edwards Sapien valve used in a patient with Tetralogy of Fallot.

With procedural safety being demonstrated, this case illustrates an important alternative treatment option for patients with congenital heart disease. Although long-term data is required to compare its efficacy with surgically replaced valves, percutaneous procedures can serve as a delay to surgical valve replacement which over a patients' lifetime with congenital heart disease, can minimise the amount of invasive surgeries and potential complications.

http://ift.tt/2voOZ2y

Insidious enemy: downside to prolonged survival in prostate cancer

An 83-year-old man, a prostate cancer survivor of 10 years with multiple vertebral metastases presented with sudden onset of double vision. On examination he was found to have an isolated partial left abducens palsy with no other neurological deficits. Despite having microvascular risk factors, given his history of prostate cancer, aMRI brain scan was requested to look for a neurological cause. The scan revealed a metastatic lesion in the clivus encasing the cavernous sinus and carotid artery. He was referred to his oncologist for further management, however he opted out of further treatment and succumbed to his illness a month later. This case report includes a literature review of cases with clivus metastases secondary to prostate cancer. It highlights the importance of carefully examining eye movements and having a high index of suspicion for the subtlest sign that may suggest brain metastases in elderly patients with prostate cancer with prolonged survival.

http://ift.tt/2tZBLFY

Dinosaur tail sign on spinal MRI in a patient with postdural puncture headache

Description

A 68-year-old man presented with cognitive impairment, urinary incontinence and short step gait. These symptoms, together with brain MRI finding, suggested idiopathic normal pressure hydrocephalus. After a spinal tap test to confirm the diagnosis, he suffered from postdural puncture headache. Despite an equivocal finding on spinal MR myelography (MRM), lumbosacral fat-suppressed T2-weighted image (FST2WI) clearly showed the characteristic 'Dinosaur tail sign' indicative of cerebrospinal fluid (CSF) leakage (figure 1).1 This pathognomonic sign disappeared on symptom improvement (figure 1).

Figure 1

Serial MRM and FST2WI before (A, D) and after a dural puncture (B, C, E, F). In contrast to subtle paraspinal hyperintensities on MRM (arrows, B), sagittal FST2WI shows characteristic dorsal epidural fat tissues demarcated by interspinous arched hyperintensities (dash lines, E) contributing to the 'Dinosaur tail sign' and slight epidural hyperintensity (arrowhead, E). Pathognomonic signal changes disappear on...

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Inactivation of Beclin-1-dependent autophagy promotes ursolic acid-induced apoptosis in hypertrophic scar fibroblasts

Abstract

A hypertrophic scar (HS) is caused by abnormal proliferation of dermal fibroblasts. Thus, promoting hypertrophic scar fibroblast (HSFB) apoptosis is an effective strategy for HS therapy. Ursolic acid (UA) has been widely used as an inducer of apoptosis in diverse cancers. However, whether UA plays an inhibitory role in HS formation is still unknown. In our study, UA was used to treat HSFBs and the cell viability, apoptosis, and collagen synthesis were determined by a Cell Counting Kit 8 (CCK8) assay, flow cytometry, and an H³-proline incorporation assay, respectively. Autophagy activity was detected by LC3 immunoblotting and electron microscopy, and siRNAs targeting Beclin-1 were used to inhibit autophagy. Western blotting was performed to investigate the molecular changes in HSFBs after various treatments. We found that UA inhibited collagen synthesis and induced cell apoptosis in HSFBs, evidenced by the deregulated expression of Bim, Bcl-2 and Cyto C. Furthermore, we demonstrated that US induced autophagy and inactivation of autophagy promoted UA-induced apoptosis and collagen synthesis inhibition in HSFBs. Molecular investigation indicated that UA induced autophagy through upregulation of Beclin-1 and knockdown of Beclin-1 prevent UA-induced autophagy. Overexpression of Bcl-2 prevent UA-induced autophagy, Beclin-1 upregulation, apoptosis and collagen synthesis inhibition in HSFBs. Collectively, our study demonstrated that UA is a novel agent for inhibiting HS formation by promoting apoptosis, especially in combination with an autophagy inhibitor. Our results provide strong evidence of the application of UA in clinical HS treatment.

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What about physical contacts between epidermal keratinocytes and sensory neurons?

Abstract

Recent studies have demonstrated that keratinocytes closely participate in sensory transduction, and therefore, intra-epidermal free nerve endings are not exclusive transducers of pain. This discovery implies the existence of close afferent communication from keratinocytes to sensory neurons. Although reciprocal interactions between keratinocytes and intra-epidermal free nerve endings via soluble mediators are well established, little attention has been paid to physical contacts between keratinocytes and intra-epidermal free nerve endings. This review proposes to consider the ultrastructural and functional knowledge of these contacts, in both human skin biopsies and keratinocyte-sensory neuron co-cultures to speculate on the possible existence of synaptic contacts.

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Web thickness determines the therapeutic effect of endoscopic keel placement on anterior glottic web

Abstract

This work is a retrospective analysis to investigate the critical risk factor for the therapeutic effect of endoscopic keel placement on anterior glottic web. Altogether, 36 patients with anterior glottic web undergoing endoscopic lysis and silicone keel placement were enrolled. Their voice qualities were evaluated using the voice handicap index-10 (VHI-10) questionnaire, and improved significantly 3 months after surgery (21.53 ± 3.89 vs 9.81 ± 6.68, P < 0.0001). However, 10 (27.8%) cases had web recurrence during the at least 1-year follow-up. Therefore, patients were classified according to the Cohen classification or web thickness, and the recurrence rates were compared. The distribution of recurrence rates for Cohen type 1 ~ 4 were 28.6, 16.7, 33.3, and 40%, respectively. The difference was not statistically significant (P = 0.461). When classified by web thickness, only 2 of 27 (7.41%) thin type cases relapsed whereas 8 of 9 (88.9%) cases in the thick group reformed webs (P < 0.001). These results suggest that the therapeutic outcome of endoscopic keel placement mostly depends on the web thickness rather than the Cohen grades. Endoscopic lysis and keel placement is only effective for cases with thin glottic webs. Patients with thick webs should be treated by other means.

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Prediction of Lymphatic Metastasis in Esophageal Cancer by CT Radiomics

Condition:   Esophagus Cancer
Intervention:   Procedure: preoperative enhanced chest CT examination
Sponsor:   Beijing Cancer Hospital
Recruiting - verified July 2017

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