Αρχειοθήκη ιστολογίου

Τετάρτη 14 Δεκεμβρίου 2016

Selection of epitopes from self-antigens for eliciting Th2 or Th1 activity in the treatment of autoimmune disease or cancer

Abstract

Vaccines have been valuable tools in the prevention of infectious diseases, and the rapid development of new vectors against constantly mutating foreign antigens in viruses such as influenza has become a regular, seasonal exercise. Harnessing the immune response against self-antigens is not necessarily analogous or as achievable by iterative processes, and since the desired outcome includes leaving the targeted organism intact, requires some precision engineering. In vaccine-based treatment of autoimmunity and cancer, the proper selection of antigens and generation of the desired antigen-specific therapeutic immunity has been challenging. Both cases involve a threshold of existing, undesired immunity that must be overcome, and despite considerable academic and industry efforts, this challenge has proven to be largely refractory to vaccine approaches leveraging enhanced vectors, adjuvants, and administration strategies. There are in silico approaches in development for predicting the immunogenicity of self-antigen epitopes, which are being validated slowly. One simple approach showing promise is the functional screening of self-antigen epitopes for selective Th1 antitumor immunogenicity, or inversely, selective Th2 immunogenicity for treatment of autoimmune inflammation. The approach reveals the importance of confirming both Th1 and Th2 components of a vaccine immunogen; the two can confound one another if not parsed but may be used individually to modulate antigen-specific inflammation in autoimmune disease or cancer.



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Rinite alérgica local [Livre artigo]

João Ferreira de Mello Junior
Braz J Otorhinolaryngol 2016;82:621-2

Resumo - Texto Completo - PDF

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Perfil audiológico de pacientes tratados de câncer na infância [Livre artigo]

Patricia Helena Pecora Liberman, Maria Valéria Schmidt Goffi-Gomez, Christiane Schultz, Paulo Eduardo Novaes, Luiz Fernando Lopes
Braz J Otorhinolaryngol 2016;82:623-9

Resumo - Texto Completo - PDF

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Definição do ponto de referência endoscópica nasal ao acesso cirúrgico à base anterior por estudo anatômico em cadáveres [Livre artigo]

Andressa Vinha Zanuncio, Paulo Fernando Tormin Borges Crosara, Helena Maria Gonçalves Becker, Celso Gonçalves Becker, Roberto Eustáquio dos Santos Guimarães
Braz J Otorhinolaryngol 2016;82:630-5

Resumo - Texto Completo - PDF

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Adaptação e validação transcultural da Sinus and Nasal Quality of Life Survey (SN-5) para o português brasileiro [Livre artigo]

Priscila Regina Candido Espinola Uchoa, Thiago Freire Pinto Bezerra, Élcio Duarte Lima, Marco Aurélio Fornazieri, Fabio de Rezende Pinna, Fabiana de Araújo Sperandio, Richard Louis Voegels
Braz J Otorhinolaryngol 2016;82:636-42

Resumo - Texto Completo - PDF

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Associação de zumbido e perda auditiva em distúrbios otológicos: estudo epidemiológico de uma década em uma população do Sul da Índia [Livre artigo]

Santoshi Kumari Manche, Jangala Madhavi, Koralla Raja Meganadh, Akka Jyothy
Braz J Otorhinolaryngol 2016;82:643-9

Resumo - Texto Completo - PDF

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Relação entre perda auditiva senil e atividade vestibular [Livre artigo]

Hanifi Kurtaran, Baran Acar, Emre Ocak, Emre Mirici
Braz J Otorhinolaryngol 2016;82:650-3

Resumo - Texto Completo - PDF

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Efeitos do tempo de permanência de splints intranasais sobre a colonização bacteriana, complicações no pós-operatório e desconforto do paciente após septoplastia [Livre artigo]

Abdullah Karatas, Filiz Pehlivanoglu, Mehti Salviz, Nuray Kuvat, Isil Taylan Cebi, Burak Dikmen, Gonul Sengoz
Braz J Otorhinolaryngol 2016;82:654-61

Resumo - Texto Completo - PDF

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O papel do volume plaquetário médio e a relação neutrófilos/linfócitos em abscesso periamigdaliano [Livre artigo]

Mehmet Şentürk, İsa Azgın, Gültekin Övet, Necat Alataş, Betül Ağırgöl, Esra Yılmaz
Braz J Otorhinolaryngol 2016;82:662-7

Resumo - Texto Completo - PDF

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Vertigem refratária episódica: papel da gentamicina intratimpânica e potencial evocado miogênico vestibular [Livre artigo]

Erika Celis-Aguilar, Ramon Hinojosa-González, Olivia Vales-Hidalgo, Heloisa Coutinho-Toledo
Braz J Otorhinolaryngol 2016;82:668-73

Resumo - Texto Completo - PDF

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Tradução para o português e validação do questionário de controle da rinite Rhinitis Control Assessment Test (RCAT) [Livre artigo]

Pedro Henrique Fernandes, Fausto Matsumoto, Dirceu Solé, Gustavo Falbo Wandalsen
Braz J Otorhinolaryngol 2016;82:674-9

Resumo - Texto Completo - PDF

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Comprometimento na qualidade de vida de pacientes com câncer de cabeça e pescoço e de seus cuidadores: estudo comparativo [Livre artigo]

Laís Rigoni, Raphaella Falco Bruhn, Rafael De Cicco, Jossi Ledo Kanda, Leandro Luongo Matos
Braz J Otorhinolaryngol 2016;82:680-6

Resumo - Texto Completo - PDF

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Carcinoma ex-adenoma pleomórfico derivado de adenoma pleomórfico recorrente mostra diferença importante por array CGH em comparação com adenoma pleomórfico recorrente sem transformação maligna [Livre artigo]

Fernanda Viviane Mariano, Karina Giovanetti, Luis Fernando Vidal Saccomani, André Del Negro, Luiz Paulo Kowalski, Ana Cristina Victorino Krepischi, Albina Altemani
Braz J Otorhinolaryngol 2016;82:687-94

Resumo - Texto Completo - PDF

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Comparação de enxertos com fáscia do músculo temporal e cartilagem de espessura total em timpanoplastias tipo 1 em crianças [Livre artigo]

Yakup Yegin, Mustafa Çelik, Arzu Karaman Koç, Levent Küfeciler, Mustafa Suphi Elbistanlı1, Fatma Tülin Kayhan
Braz J Otorhinolaryngol 2016;82:695-701

Resumo - Texto Completo - PDF

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Resultados de diferentes técnicas de reconstrução do nervo facial [Livre artigo]

Aboshanif Mohamed, Eigo Omi, Kohei Honda, Shinsuke Suzuki, Kazuo Ishikawa
Braz J Otorhinolaryngol 2016;82:702-9

Resumo - Texto Completo - PDF

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Presbiacusia: será que temos uma terceira orelha? [Livre artigo]

Luis Roque Reis, Pedro Escada
Braz J Otorhinolaryngol 2016;82:710-4

Resumo - Texto Completo - PDF

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Leiomiossarcoma da tireoide: apresentação de dois casos e revisão da literatura [Livre artigo]

Mehmet İlhan Şahin, Alperen Vural, İmdat Yüce, Sedat Çağlı, Kemal Deniz, Ercihan Güney
Braz J Otorhinolaryngol 2016;82:715-21

Resumo - Texto Completo - PDF

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Estudo do microfonismo coclear na neuropatia auditiva [Livre artigo]

Ilka do Amaral Soares, Pedro de Lemos Menezes, Aline Tenório Lins Carnaúba, Kelly Cristina Lira de Andrade, Otávio Gomes Lins
Braz J Otorhinolaryngol 2016;82:722-36

Resumo - Texto Completo - PDF

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Rouquidão: apresentação incomum de linfoma primário de tireoide com infiltração da laringe [Livre artigo]

Ozan Gökdoğan, Ahmet Koybasioglu, Erkin Ismail, Timucin Erol, Gokcen Alagoz, Banu Yagmurlu, Seref Komurcu
Braz J Otorhinolaryngol 2016;82:737-40

Resumo - Texto Completo - PDF

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Lipoma do ângulo pontocerebelar em pacientes assintomáticos: relato de caso [Livre artigo]

Ricardo Rodrigues Figueiredo, Andréia Aparecida de Azevedo, João Alfredo de Mesquita Rodrigues Figueiredo, Norma de Oliveira Penido
Braz J Otorhinolaryngol 2016;82:741-2

Resumo - Texto Completo - PDF

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Nachsorge des Endometriumkarzinoms

Zusammenfassung

Hintergrund

Obwohl die Prognose des Endometriumkarzinoms i. Allg. gut ist, kommt es bei ca. 15 % der im Stadium I und II erkrankten Patientinnen zu einem Rezidiv. Durch die im Einzelfall zugrunde liegende Primärtherapie resultieren unterschiedliche Nebenwirkungen und Langzeitfolgen.

Ziel

In der Übersichtsarbeit sollen die Ziele sowie die derzeit empfohlenen Maßnahmen und Untersuchungsintervalle für die Nachsorge des Endometriumkarzinoms dargestellt werden.

Material und Methoden

Eine selektive PubMed-Literaturrecherche zu Themen der Nachsorge des Endometriumkarzinoms wurde durchgeführt.

Ergebnisse

Das Ziel der Tumornachsorge ist es zum einen, zumeist erneut kurativ therapierbare Rezidive rechtzeitig zu entdecken, darüber hinaus aber auch Vorsorge vor Zweitmalignomen zu bieten. Ferner sollen Nebenwirkungen und Folgen der stattgehabten Behandlung erkannt, gelindert und behoben werden. Hierzu werden die spezifischen Empfehlungen und die vorliegende Datenlage vorgestellt.

Schlussfolgerung

Die Nachsorgeuntersuchung erfolgt im Wesentlichen klinisch und symptomorientiert in zunehmenden Zeitintervallen. Die Wertigkeit verschiedener Untersuchungsmaßnahmen wird diskutiert. Langfristiges Ziel der Nachsorge ist es, die Lebensqualität der Patientinnen zu erhalten.



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Value of postmortem studies in deceased neonatal and pediatric intensive care unit patients

Abstract

Worldwide, various autopsy studies have shown a decrease in the diagnostic error rate over the last years. The cause of this positive development is mainly due to the improvement of modern medicine. However, intensive care unit patients are thought to have a higher risk for diagnostic errors, which is documented in several studies in the adult population. In contrast, there is only limited information about diagnostic errors in pediatrics, particularly in pediatric and neonatal intensive care units. The aims of this study were to analyze the spectrum of childhood death, determine the prevalence and distribution of autopsy-confirmed diagnostic errors, and describe patient characteristics that might have influenced the discordance between antemortem and postmortem findings. We analyzed 143 autopsy reports from 2004 to 2013 and correlated these with clinical reports. The overall autopsy rate during this interval was 20.3%. The leading causes of death were congenital malformations (28%), diseases closely associated with perinatal disorders (25%), disorders of the cardiovascular system (18%), and infections (15%). Additional findings were obtained in 23% of the autopsies. Major diagnostic errors were found in 6%, the lowest reported value in a developed country as yet. Most cases (75%) showed complete concordance between clinical diagnoses and postmortem findings, in line with improvements in diagnostic and therapeutic processes over the last decades. In conclusion, autopsy of neonates, infants, and children represents an important tool for monitoring the quality of pediatric and neonatal medical care.



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The clinical examination of non-word repetition tasks in identifying Persian-speaking children with primary language impairment

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Publication date: February 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 93
Author(s): Yalda Kazemi, Soodeh Saeednia
ObjectiveThis study aimed to examine the diagnostic accuracy of a non-word repetition (NWR) test in identifying Persian-speaking preschool children with specific/primary language impairment (PLI). Children with PLI show consistently poorer performance in non-word repetition tasks than their typically developing language (TDL) counterparts. It is assumed that the ability to repeat non-words triggers language skills and that the absence of this ability may be responsible for language impairment in PLI children.MethodsTwenty preschool children with PLI participated in this study and were compared with 31 peers whose language skills were developing typically. The TDL children were randomly selected from daycare centers, and the children with PLI were referred by qualified speech-language pathologists from speech therapy clinics. A Persian NWR test was administered and scored using two levels of scoring: item-level scoring and syllable-level scoring.Data were analyzed in two phases. The first phase aimed to determine any differences between the two groups of children in terms of NWR ability. The second phase examined the diagnostic measures of the test.ResultsThe results of first phase documented that both scoring methods for the NWR test significantly differentiated between children with PLI and their normal peers. The second phase indicated that both scoring methods for the NWR test have good sensitivity and specificity in differentiating Persian-speaking children with PLI from their normal peers.ConclusionNon-word repetition can be a reliable clinical marker of PLI in Persian-speaking preschool children.



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A 45-Second Self-Test for Cardiorespiratory Fitness: Heart Rate-Based Estimation in Healthy Individuals

http://otorhinolarygology.blogspot.com/2016/12/a-45-second-self-test-for.html


Alexandros Sfakianakis
Anapafseos 5 . Agios Nikolaos
Crete.Greece.72100
2841026182
6948891480

Acute skin and hair symptoms followed by severe, delayed eye complications in subjects using the synthetic opioid MT-45

Summary

Background

The introduction of unclassified new psychoactive substances (NPS) on the recreational drugs market through open online sale ('legal highs' or 'Internet drugs') continues unabated and represents a growing health hazard. The use of NPS has resulted in numerous, severe, adverse events and fatalities, due to unintended overdose or unknown toxic side-effects.

Objectives

To try to find a possible common underlying cause for the skin–hair–eye symptoms complex observed in three men.

Methods

From late 2013 to mid-2014, three Swedish men aged 23–34 years with a history of recreational drug use independently presented with similar and very remarkable clinical signs, requiring extensive examination and prolonged treatment.

Results

Common clinical signs included hair depigmentation, hair loss, widespread folliculitis and dermatitis, painful intertriginous dermatitis, dry eyes, and elevated liver enzymes. Two of them also showed transverse white Mees' lines (leukonychia striata) on the fingernails and toenails, suggesting a temporary, drug-induced, disorganized keratinization. The clinical signs gradually disappeared over time. However, later on, two developed severe bilateral secondary cataracts requiring surgery. Because drug tests within the Swedish STRIDA project had demonstrated intake of the NPS opioid MT-45 in all patients, this was suspected to be the common causative agent.

Conclusions

These cases highlight the importance for physicians and health professionals to consider the increasing number of novel, untested recreational drugs, as a potential cause of unusual and otherwise unrecognized clinical signs and symptoms.



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Early Detection of Xeroderma in Older Patients

This study discusses how clinicians might look for white scale sign to detect and diagnose xeroderma in aging patients.

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Answers to CME examination



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Valuable or vain: An ethical analysis of free skin cancer screening

See related articles on pages 129 and 181

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CME examination



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Reply to: “On the utility of soak and smear”

To the Editor: We thank our colleagues, Drs James, Gutman, and Kirby, for their comments. It is an honor to have the physicians who published the first study on soak and smear reflect on our work.1

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Prevention and management of glucocorticoid-induced side effects: A comprehensive review

Systemic glucocorticoids are an essential therapy for a range of conditions, but their multiple side effects can produce significant morbidity for patients. The objective of this review is to discuss these side effects while addressing 3 questions: 1) What dose and duration of glucocorticoid therapy should prompt concern for individual side effects?; 2) How should clinicians counsel patients about these complications?; and 3) How can these problems be prevented or managed? To accomplish these objectives, we have created a series of tables and algorithms based on a review of relevant data to guide counseling, prophylaxis, and management of 11 glucocorticoid side effects.

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Tender nodule on the finger

A 27-year-old man presented with a tender lesion on the fifth finger on his left hand. This lesion initially started as a linear laceration that he obtained while skinning a sheep 2 to 3 weeks earlier. The physical examination revealed a tender nodule with a central hemorrhagic crust, a white middle ring, and a violaceous periphery over the left fifth extensor finger (Fig 1). A shave biopsy specimen was obtained to confirm the diagnosis (Fig 2).

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Information for Readers



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Pruritic annular papules and plaques on the face and back

A 35-year-old previously healthy man presented with a 1-year history of multiple pruritic lesions on both his face and back. The lesions presented with crops of papules measuring 1 to 2 mm in diameter, grouped to form annular plaques, with central hyperpigmentation (Fig 1). Antifungal treatment, including oral terbinafine for 2 weeks and topical bifonazole for 1 month, was given in another hospital without any improvement. A skin biopsy specimen was obtained from the patient's face. The histopathologic examination revealed a heavy infiltrate of eosinophils and lymphocytes surrounding the hair follicles and sebaceous glands.

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The natural history of actinic keratoses in organ transplant recipients

To the Editor: Actinic keratoses (AKs) are common, highly dynamic, sun-induced lesions that frequently regress and recur.1,2 We sought to investigate whether there is an association between the degree of AK turnover and squamous cell carcinoma (SCC) incidence.

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Violaceous papules and plaques associated with Crohn's disease

A 25-year-old woman with Crohn's disease that was well controlled with infliximab presented with a 10-month history of violaceous plaques. Physical examination revealed multiple red to violaceous papules and plaques on the extensor surfaces of her elbows, knees, toes, fingers, and dorsal aspects of her feet (Fig 1). She denied fever, arthralgias, pain, or other systemic symptoms. The skin lesions had been unsuccessfully treated with oral prednisone, intralesional triamcinolone, topical clobetasol, and pimecrolimus.

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Reply to: “Is treatment of acne as simple as encouraging primary care physicians to prescribe more retinoids?”

To the Editor: Defining the role of the primary care provider in the management of skin diseases is a multifactorial issue. Perhaps from the dermatologist's perspective it might seem ideal for dermatologists to manage all cases of acne, as dermatologists have extensive training and experience with the condition. But acne is highly prevalent, and the existing shortage of dermatologists may make having every patient with acne see a dermatologist unfeasible, if not undesirable.1,2

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Hyperkeratotic plaques on the trunk and extremities

An 8-year-old white boy presented for a lifelong skin condition. At birth he had widespread blisters and erosions and later developed generalized, thick scaly plaques on his body and thinner scaling on his face. Biopsy specimens obtained for electron microscopy performed at birth revealed dyscohesive keratinocytes and clumped keratin intermediate filaments of suprabasal keratinocytes (Fig 1). The physical examination revealed generalized, thick, scaly plaques predominantly involving the flexural surfaces and brown hyperkeratotic, verrucous, scaly plaques on his trunk, legs, and arms (Fig 2).

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Change of Address



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Iotaderma #275



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Use of a cotton-tipped applicator for superior clinical diagnosis and biopsy of suspected basal cell carcinoma of the nasal ala

Although the nose is a common location for basal cell carcinoma (BCC), the nasal ala represents a location where visualization and biopsy of suspected BCC can prove especially difficult. The ala's angulated structure and background features, such as telangiectases, can make the characteristic features of BCC challenging to appreciate (Fig 1). In addition, biopsy of the region can be cumbersome because of the inherent flexures of the nasal ala.

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A double-blind, placebo-controlled, phase-II clinical trial to evaluate oral simvastatin as a treatment for vitiligo

To the Editor: Vitiligo is an autoimmune disease caused by autoreactive CD8+ T lymphocytes that target melanocytes, and interferon-γ-induced CXCL10 plays an important role.1 Simvastatin inhibits interferon-γ signaling by blocking activation of STAT12 and prevented and reversed disease in our mouse model.3 A case report described a patient with vitiligo who repigmented with simvastatin.4 We conducted a small, randomized, double-blind, placebo-controlled, phase II clinical trial to test simvastatin as a treatment for vitiligo.

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Wisely choosing thin melanomas for sentinel lymph node biopsy

To the Editor: The routine use of sentinel lymph node (SLN) biopsy (SLNB) in the management of thin melanomas is controversial. For this reason, we read with great interest the article of Wat et al1 in which the authors examined 1072 cases of melanoma submitted to SLNB. Among these, 171 cases were thin melanomas and 15 of them (8.8%) showed SLNB positivity. Today, the scientific community is precisely focusing on the clinical significance of different histologic subtypes of thin melanoma. The current staging system for melanoma of the American Joint Committee on Cancer uses Breslow thickness as the primary attribute and up to 1-mm-thick melanoma is defined as "thin" because it shows good prognosis after surgical excision, with 10-year survival of 85% to 90% in case of tumor-free margin of at least 1 cm.

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Total body skin exams (TBSEs): Saving lives or wasting time?

The central ethical issue raised by this scenario is whether it is morally obligatory to perform TBSEs, or at least offer them, to all new dermatology patients and at routine screening visits for patients at risk for skin cancer. Those supporting TBSEs make claims that the practice increases detection of skin cancer (general beneficence or utility), represents a professional duty to patients (deontology), and upholds patient preferences (autonomy). Those supporting lesion-directed exams over TBSEs suggest that the time saved from more focused exams permits seeing more patients while only making a small sacrifice in skin cancer detection, a utilitarian claim.

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A proactive approach to ending the use of university debit cards for indoor tanning

To the Editor: Indoor tanning is associated with increased risk of melanoma, particularly when used during adolescence and early adulthood.1 The ability of young adults to use indoor tanning is partially limited by the cost of purchasing these services.2 Many universities sponsor debit cards that parents can fund to support student expenses. Students can use these debit cards at various vendors that have entered into an agreement with the university, including tanning salons.3 Recently, a review of the top 125 colleges ranked by US News and World Report found that 14.4% of universities have a campus debit card that can be used to purchase tanning services.

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Percutaneous ligature of the superficial temporal artery: A simple technique to reduce bleeding

Excision of skin lesions within close proximity of the branches of the superficial temporal artery can be challenging because bleeding from severed arteries readily results in a loss of visibility.

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Deliberate chemical dermatoglyphic burns in a political refugee

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Publication date: Available online 14 December 2016
Source:Annales de Dermatologie et de Vénéréologie
Author(s): P. Charlier, A. Haroun, C. Hervé




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Inequalities in access to biological treatments for psoriasis: Results from the Italian Psocare Registry

Abstract

Background

Limited evidence is available on the impact of socioeconomic factors in drug prescriptions for psoriasis.

Objectives

To investigate factors influencing prescription of conventional versus biological treatment for psoriatic patients, based on the Psocare registry with a special focus on socioeconomic factors.

Methods

This was a cross-sectional study evaluating the baseline data of patients included in the Italian Psocare Registry. All of the consecutive adult patients with a diagnosis of chronic plaque psoriasis (Ps) or psoriatic arthritis and who were prescribed a systemic treatment for Ps at the participating centres were included in this study. Univariate and multivariate analyses of the baseline factors associated with a biologics prescription were performed.

Results

From September 2005 to September 2009, 12,838 patients were identified. A multivariate analysis revealed that, among other factors, completing a level of education higher than lower secondary school and being employed as a manager or a professional were independent factors associated with a biologics prescription at entry in the registry. Additional analyses on the association between these two variables and a severe Ps condition (i.e., psoriasis area and severity index [PASI] score > 20) revealed a significant increasing trend of severe disease towards lower educational attainment, while unemployed patients were more likely to have a more severe condition as compared to the other categories of workers.

Conclusions

We documented inequalities of drug prescriptions for Ps in Italy, with a trend towards a higher frequency of prescription for more expensive biologics in higher socio-economic sectors of the population.

This article is protected by copyright. All rights reserved.



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Amianto. Cáncer de laringe

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Publication date: Available online 14 December 2016
Source:Acta Otorrinolaringológica Española
Author(s): Luis Sánchez Galán




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Electrocauterización endoscópica para el tratamiento de las fístulas congénitas del seno piriforme en pediatría. Serie de casos

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Publication date: Available online 14 December 2016
Source:Acta Otorrinolaringológica Española
Author(s): Giselle Cuestas, Flavia Doormann, Verónica Rodríguez, Patricio Bellia Munzón, Gastón Bellia Munzón
Las fístulas del seno piriforme son anomalías infrecuentes de los arcos branquiales. La mayoría se localizan en el lado izquierdo y se extienden desde el ápex del seno piriforme de la hipofaringe hasta la glándula tiroides o el espacio peritiroideo.El diagnóstico se sospecha ante la presencia de tiroiditis aguda supurada o abscesos cervicales laterales recurrentes, y se confirma mediante la visualización endoscópica del orificio de la fístula. El tratamiento clásico consiste en la exéresis del trayecto fistuloso por vía cervical, con o sin lobectomía tiroidea. Sin embargo, se han desarrollado alternativas menos invasivas y con menos riesgos de complicaciones que obliteran el trayecto de la fístula, como la electrocauterización endoscópica.Describimos nuestra experiencia con 7 pacientes que presentaban esta afección, tratados con cauterización endoscópica utilizando electrobisturí de radiofrecuencia, y evaluamos la eficacia y la seguridad del tratamiento realizado.Pyriform sinus fistulas are rare anomalies of the branchial arches. Most of them are located on the left side. They extend from the apex of the pyriform sinus of the hypopharynx to the thyroid gland or adjacent tissues.The diagnosis is suspected in the presence of acute suppurative thyroiditis or recurrent cervical abscesses, and is confirmed by endoscopic visualization of the fistula hole. The traditional treatment consists of excision of the fistulous tract, with or without thyroid lobectomy, by cervical approach. However, less invasive alternatives that obliterate the path of the fistula have been developed, such as endoscopic electrocautery.We describe our experience with 7 patients with this condition, who were treated with endoscopic cauterization using radiofrequency electrocautery, and we evaluate the effectiveness and safety of the treatment performed.



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Use of thromboelastography in the management of liver cirrhosis and accelerated intravascular coagulation and fibrinolysis (AICF)

In the presented case, the authors describe an obese middle-aged man that presented to the emergency department for persistent oedema, scleral icterus and fatigue. He was admitted to the hospital and diagnosed with liver cirrhosis via transjugular liver biopsy. He continued to bleed from the biopsy site for 5 days from accelerated intravascular coagulation and fibrinolysis (AICF) requiring multiple transfusions of packed red blood cells, fresh-frozen plasma and cryoprecipitate. The authors then used thromboelastography (TEG) to further characterise the patient's coagulopathy, which revealed platelet inhibition. The results of the TEG significantly changed future transfusion management. Finally, the authors conducted a literature review to summarise the current literature available for the use of TEG in the management of liver cirrhosis with AICF.



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Combined central retinal vein and branch retinal artery occlusion in hyperhomocysteinaemia

Description

A woman aged 30 years reported of blurred vision in the right eye (RE) for 2 days. Visual acuity was 6/24 in the RE and 6/6 in the left eye (LE). Funduscopy of RE showed combined non-ischaemic central retinal vein occlusion (CRVO) and supero-temporal branch retinal artery occlusion (BRAO) (figure 1A). LE examination was normal. Optical coherence tomography (OCT) of the RE showed thickening of inner retinal layers corresponding to the area of BRAO (figure 1A: white arrow). Thorough systemic investigations and cardiac workup revealed raised serum homocysteine levels (37.21 μmol/L). She was started on oral folic acid and pyridoxine. Over the next 6 months, her visual acuity improved to 6/12 with clearing of retinal whitening and resolution of retinal haemorrhages (figure 1B–D).

Figure 1

Funduscopy of the right eye (RE) showing dilated torturous retinal veins with multiple retinal haemorrhages in all four quadrants...



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Iliopsoas abscess caused by chronic urolithiasis and pyelonephritis

Description

A man aged 45 years, with a history of recurrent urolithiasis and pyelonephritis, presented with a 3-month history of fever. Physical examination revealed a nodule in his right inguinal area. CT with contrast showed a right iliopsoas abscess that extended to the inguinal area and a right urethral stone (figure 1). Drainage under fluoroscopic guidance was performed, and the contrast was injected into the cavity from the drainage tube to assess the abscess cavity. The contrast study demonstrated a connection between the urethra and abscess cavity (figure 2). The patient was administered 1 g cefotiam every 8 hours. Blood, urine and fluid cultures from the abscess were positive for Escherichia coli. Surgical drainage and right nephrectomy was performed. The patient was discharged 3 months after the surgery without any complication. The penetration of an iliopsoas abscess into the urinary tract is extremely rare, while the symptoms...



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Vertical muscle transposition with silicone band belting in VI nerve palsy

A woman aged 60 years developed a Millard-Gubler syndrome after a diagnosis of a cavernous angioma in the median and paramedian areas of the pons. In this context, she presented a right VI nerve palsy, right conjugate gaze palsy, facial palsy and left hemiparesis. To improve the complete VI nerve palsy, we planned a modified transposition approach, in which procedure we made a partial transposition of vertical rectus with a silicone band that was fixated posteriorly. After the procedure, the patient gained the ability to slightly abduct the right eye. We found no compensatory torticollis in the primary position of gaze. There was also an improvement of elevation and depression movements of the right eye. We obtained satisfactory results with a theoretically reversible technique, which is adjustable intraoperatively with no need of muscle detachment, preventing anterior segment ischaemia and allowing simultaneous recession of the medial rectus muscles, if necessary.



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Indian Journal of Medical and Paediatric Oncology (Indian J Med Paediatr Oncol)

EDITORIAL COMMENTARY

Classical or pylorus-preserving pancreatoduodenectomy in pancreatic and periampullary cancer: "The jury is still out!" [pg. 209]
Savio George Barreto
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REVIEW ARTICLES

Should every patient with pancreatic cancer receive perioperative/neoadjuvant therapy? [pg. 211]
Ulrich Nitsche, Bo Kong, Alexander Balmert, Helmut Friess, Jörg Kleeff
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Skin: A mirror of internal malignancy [pg. 214]
Rita V Vora, RahulKrishna S Kota, Nilofar G Diwan, Nidhi B Jivani, Shailee S Gandhi
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Status of barium studies in the present era of oncology: Are they a history? [pg. 223]
Abhishek Mahajan, Subash Desai, Nilesh Pandurang Sable, Meenakshi Haresh Thakur
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ORIGINAL ARTICLES

Protection behaviors for cytotoxic drugs in oncology nurses of chemotherapy centers in Shiraz hospitals, South of Iran [pg. 227]
Khadijeh Abbasi, Maryam Hazrati, Abolfazl Mohammadbeigi, Jasem Ansari, Mahboubeh Sajadi, Azam Hosseinnazzhad, Esmail Moshiri
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Outcomes, cost comparison, and patient satisfaction during long-term central venous access in cancer patients: Experience from a Tertiary Care Cancer Institute in South India [pg. 232]
K Govind Babu, MC Suresh Babu, D Lokanatha, Gita R Bhat
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Effect of areca nut chewing and maximal mouth opening in schoolgoing children in Ahmedabad [pg. 239]
Azizfatema Munawer Khan, Megha S Sheth, Romsha R Purohit
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Clinicopathological features and outcomes in advanced nonsmall cell lung cancer with tailored therapy [pg. 242]
Stalin Bala, Sadashivudu Gundeti, Vijay Gandhi Linga, Lakshmi Srinivas Maddali, Raghunadha Rao Digumarti, Shantveer G Uppin
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Clinicopathological profile of gastrointestinal lymphomas in Kashmir [pg. 251]
Mehnaaz Sultan Khuroo, Summyia Farooq Khwaja, Ajaz Rather, Zhahid Hassan, Ruby Reshi, Naira Sultan Khuroo
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Metabolic toxicities in patients undergoing treatment for nonhematological malignancy: A cross-sectional study [pg. 256]
Subhash Gupta, Kunhi Parambath Haresh, Soumyajit Roy, Lakhan Kashyap, Narayan Adhikari, Rambha Pandey, Dayanand Sharma, Pramod Kumar Julka, Goura Kishor Rath
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Managing metastatic renal cell carcinoma-challenges, pitfalls, and outcomes in the real world [pg. 260]
Karnam Ashok Kumar, Gundeti Sadashivudu, KV Krishnamani, Vijay Gandhi Linga, Lakshmi Srinivas Maddali, Raghunadha Rao Digumarti
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Evaluation of thyroid lesions by fine-needle aspiration cytology based on Bethesda system for reporting thyroid cytopathology classification among the population of South Bihar [pg. 265]
Richa Bhartiya, Mahasweta Mallik, Nawanita Kumari, Brijendra Narayan Prasad
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Oxaliplatin-related neuropathy in Indian patients – no difference between generic and original molecules [pg. 271]
Bhawna Sirohi, Vikas Ostwal, Shaheenah Dawood, Gilberto Lopes, Sanjay Talole, Chaitali Nashikkar, Shailesh Shrikhande
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Burden of cervical cancer and role of screening in India [pg. 278]
Saurabh Bobdey, Jignasa Sathwara, Aanchal Jain, Ganesh Balasubramaniam
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Cognizance and utilization about breast cancer screening among the health professional female students and staffs of University Kuala Lumpur, Royal College of Medicine Perak, Malaysia [pg. 286]
ATM Emdadul Haque, Muhammad Afif Bin Mohd Hisham, Noor Azwa Laili Binti Ahmad Adzman, Nur Atiqah Binti Azudin, Nursakinah Binti Shafri, Mainul Haque
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CASE REPORTS

Juvenile granulosa cell tumor associated with Ollier disease [pg. 293]
Abhilasha Ashok Sampagar, Rahul R Jahagirdar, Vibha Sanjay Bafna, Sandip P Bartakke
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Thymoma masquerading as transfusion dependent anemia [pg. 296]
Javvid Muzamil, Aejaz Aziz Shiekh, Gull Mohammad Bhat, Abdul Rashid Lone, Shuaeb Bhat, Firdousa Nabi
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PRACTITIONER SECTION

A rare case of lung cancer presenting as an ischioanal fossa mass [pg. 300]
Nishitha Shetty, Ranvijay Singh, Maryam Naveed, Ashwini M Ronghe, Falguni Shashikant Barot
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Multiple solitary extramedullary anaplastic plasmacytomas [pg. 303]
Sandesh Madi, Vishnu Senthil, Monappa Naik, Sandeep Vijayan
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LETTERS TO EDITOR

Folate supplementation in transfusion-dependent thalassemia: Do we really need such high doses? [pg. 305]
Gaurav Tripathi, Manas Kalra, Amita Mahajan
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Early tumor shrinkage as an "on-treatment" clinical predictor of long-term outcome in solid organ cancers [pg. 306]
Pratishtha Banga Chaudhari
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Cancer risk of general people due to using joss stick for religious worshiping [pg. 307]
Beuy Joob, Viroj Wiwanitkit
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A rare case of hepatoid carcinoma of the ovary with pancytopenia and hypocellular marrow [pg. 307]
Manoj Lakhotia, Hans Raj Pahadiya, Akanksha Choudhary, Ronak Gandhi, Ramesh Chand Purohit
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Multiple cutaneous malignancies in a child with xeroderma pigmentosum: A case report [pg. 309]
Rita V Vora, RahulKrishna SureshKumar Kota, Nilofar G Diwan
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The masquerading splenic lesion [pg. 311]
Mansoor C Abdulla, Jemshad Alungal, Ram Naryan, Neena Mampilly
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ASCO 2016 GI CANCER UPDATE

Focused update on Gastrointestinal (GI) Oncology from ASCO 2016 [pg. 314]
Ravi Kumar Paluri
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ERRATUM

Erratum: Evaluation of myeloid cells (tumor associated tissue eosinophils and mast cells) infiltration in different grades of oral squamous cell carcinoma [pg. 319]

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Erratum: Isolated humeral recurrence in endometrial carcinoma [pg. 320]

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Alexandros Sfakianakis
Anapafseos 5 . Agios Nikolaos
Crete.Greece.72100
2841026182
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A connection between neurovascular conflicts within the cerebellopontine angle and vestibular neuritis, a case controlled cohort study.

A connection between neurovascular conflicts within the cerebellopontine angle and vestibular neuritis, a case controlled cohort study.

Acta Otorhinolaryngol Ital. 2016 Oct;36(5):421-427

Authors: Loader B, Linauer I, Korkesch S, Krammer-Effenberger I, Zielinski V, Schibany N, Kaider A, Vyskocil E, Tscholakoff D, Franz P

Abstract
This retrospective, observer blinded case-control study aims to compare the prevalence of neurovascular conflicts (NVCs) of the vestibulocochlear nerve and the anterior inferior cerebellar artery (AICA) in patients presenting with clinical signs of acute vestibular neuritis with and without subsequent objective vestibular function loss (VFL). 58 acute cases of clinically suspected acute vestibular neuritis were investigated with same day cranial MRI at a tertiary referral centre and compared to 61 asymptomatic controls. The prevalence of NVCs in cases with objective VFL were also compared to cases without VFL. Radiologists described the NVC as "no contact" (Grade 0), "contact < 2 mm" (Grade 1), "contact > 2 mm" (Grade 2) and "vascular loop presence" (Grade 3) without knowledge of neurotological data. Neurotological data was collected without knowledge of MRI findings. Vestibular function was tested by bithermic caloric irrigation. 26 cases (45%) showed caloric VFL (Group A), whereas 32 (55%) exhibited no VFL (Group B). Group A included 13 cases with NVCs (50%), Group B included 26 NVC cases (82%) (p = 0.012) and the control group included 16 individuals (26%) (p < 0.001 for comparison of all 3 groups). Group B had a significantly higher NVC-Grading than Group A (p = 0.009). There was no statistically significant association between NVCs and either SNHL or tinnitus (p > 0.05). Our results suggest that patients presenting with clinical signs of acute vestibular neuritis who show symmetrical caloric vestibular function test results have a significantly higher NVC prevalence in the cerebellopontine angle.

PMID: 27958603 [PubMed - in process]



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Severe to profound deafness may be associated with MYH9-related disease: report of 4 patients.

Severe to profound deafness may be associated with MYH9-related disease: report of 4 patients.

Acta Otorhinolaryngol Ital. 2016 Oct;36(5):415-420

Authors: Canzi P, Pecci A, Manfrin M, Rebecchi E, Zaninetti C, Bozzi V, Benazzo M

Abstract
MYH9-related disease (MYH9-RD) is a rare genetic syndromic disorder characterised by congenital thrombocytopenia and is associated with the risk of developing progressive sensorineural hearing loss, nephropathy and presenile cataracts during childhood or adult life. All consecutive patients enrolled in the Italian Registry for MYH9-RD with severe to profound deafness were included in a retrospective study. The study population involved 147 Italian patients with MYH9-RD: hearing loss was identified in 52% of cases and only 4 patients (6%) presented severe to profound deafness at a mean age of 33 years. Deafness was associated with mild spontaneous bleeding in all patients and with kidney involvement in 3 cases. Cochlear implantation was carried out in 3 cases with benefit, and no major complications were observed. Diagnosis was performed about 28 years after the first clinical manifestation of MYH9-RD, which was never suspected by an otolaryngologist. The clinical and diagnostic aspects of 4 patients with severe to profound deafness are discussed with a focus on therapeutic implications.

PMID: 27958602 [PubMed - in process]



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Open partial horizontal laryngectomies: is it time to adopt a modular form of consent for the intervention?

Open partial horizontal laryngectomies: is it time to adopt a modular form of consent for the intervention?

Acta Otorhinolaryngol Ital. 2016 Oct;36(5):403-407

Authors: Giordano L, Di Santo D, Crosetti E, Bertolin A, Rizzotto G, Succo G, Bussi M

Abstract
Nowadays, open partial horizontal laryngectomies (OPHLs) are well-established procedures for treatment of laryngeal cancer. Their uniqueness is the possibility to modulate the intervention intraoperatively, according to eventual tumour extension. An OPHL procedure is not easy to understand: there are several types of procedures and the possibility to modulate the intervention can produce confusion and lack of adherence to the treatment from the patient. Even if the surgery is tailored to a patient's specific lesion, a unified consent form that discloses any possible extensions, including a total laryngectomy, is still needed. We reviewed the English literature on informed consent, and propose comprehensive Information and Consent Forms for OPHLs. The Information Form is intended to answer any possible questions about the procedure, while remaining easy to read and understand for the patient. It includes sections on laryngeal anatomy and physiology, surgical aims and indications, alternatives to surgery, complications, and physiology of the operated larynx. The Consent Form is written in a "modular" way: the surgeon defines the precise extension of the lesion, chooses the best OPHL procedure and highlights all possible expected extensions specific for the patient. Our intention, providing these forms both in Italian and in English, is to optimise communication between the patient and surgeon, improving surgical procedure arrangements and preventing any possible misunderstandings and medico-legal litigation.

PMID: 27958601 [PubMed - in process]



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Comparison between videofluoroscopy, fiberoptic endoscopy and scintigraphy for diagnosis of oro-pharyngeal dysphagia.

Comparison between videofluoroscopy, fiberoptic endoscopy and scintigraphy for diagnosis of oro-pharyngeal dysphagia.

Acta Otorhinolaryngol Ital. 2016 Oct;36(5):395-402

Authors: Fattori B, Giusti P, Mancini V, Grosso M, Barillari MR, Bastiani L, Molinaro S, Nacci A

Abstract
The purpose of this study was to compare videofluoroscopy (VFS), fiberoptic endoscopic evaluation of swallowing (FEES) and oro-pharyngo- oesophageal scintigraphy (OPES) with regards to premature spillage, post-swallowing residue and aspiration to assess the reliability of these tests for detection of oro-pharyngeal dysphagia. Sixty patients affected with dysphagia of various origin were enrolled in the study and submitted to VFS, FEES and OPES using a liquid and semi-solid bolus. As a reference, we used VFS. Both the FEES and the OPES showed good sensitivity with high overall values (≥ 80% and ≥ 90% respectively). The comparison between FEES vs VFS concerning drop before swallowing showed good specificity (84.4% for semi-solids and 86.7% for liquids). In the case of post-swallowing residue, FEES vs VFS revealed good overall validity (75% for semi-solids) with specificity and sensitivity well balanced for the semi-solids. OPES vs. VFS demonstrated good sensitivity (88.6%) and overall validity (76.7%) for liquids. The analysis of FEES vs. VFS for aspiration showed that the overall validity was low (≤ 65%). On the other hand, OPES demonstrated appreciable overall validity (71.7%). VFS, FEES and OPES are capable of detecting oro-pharyngeal dysphagia. FEES gave significant results in the evaluation of post-swallowing residues.

PMID: 27958600 [PubMed - in process]



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Association between oral habits, mouth breathing and malocclusion.

Association between oral habits, mouth breathing and malocclusion.

Acta Otorhinolaryngol Ital. 2016 Oct;36(5):386-394

Authors: Grippaudo C, Paolantonio EG, Antonini G, Saulle R, La Torre G, Deli R

Abstract
The ratio of bad habits, mouth breathing and malocclusion is an important issue in view of prevention and early treatment of disorders of the craniofacial growth. While bad habits can interfere with the position of the teeth and normal pattern of skeletal growth, on the other hand obstruction of the upper airway, resulting in mouth breathing, changes the pattern of craniofacial growth causing malocclusion. Our crosssectional study, carried out on 3017 children using the ROMA index, was developed to verify if there was a significant correlation between bad habits/mouth breathing and malocclusion. The results showed that an increase in the degree of the index increases the prevalence of bad habits and mouth breathing, meaning that these factors are associated with more severe malocclusions. Moreover, we found a significant association of bad habits with increased overjet and openbite, while no association was found with crossbite. Additionally, we found that mouth breathing is closely related to increased overjet, reduced overjet, anterior or posterior crossbite, openbite and displacement of contact points. Therefore, it is necessary to intervene early on these aetiological factors of malocclusion to prevent its development or worsening and, if already developed, correct it by early orthodontic treatment to promote eugnatic skeletal growth.

PMID: 27958599 [PubMed - in process]



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Advanced oxidation protein product levels as a marker of oxidative stress in paediatric patients with chronic tonsillitis.

Advanced oxidation protein product levels as a marker of oxidative stress in paediatric patients with chronic tonsillitis.

Acta Otorhinolaryngol Ital. 2016 Oct;36(5):381-385

Authors: Ozbay I, Kucur C, Koçak FE, Savran B, Oghan F

Abstract
We aimed to determine whether advanced oxidation protein product (AOPP) levels can serve as a marker of oxidative stress in paediatric patients with chronic tonsillitis. Thirty children with chronic tonsillitis and 30 healthy children (control group) were recruited from the Otorhinolaryngology (ORL) and Paediatric Surgery departments, respectively, of Dumlupinar University Hospital. In the patient group, blood samples were collected before tonsillectomy, and tonsil tissue was sampled during the operation. Blood samples were also obtained from the control subjects. AOPP levels in the serum and tonsil tissue were measured by the spectrophotometric method. Serum AOPP levels were significantly higher in the patient group (13.1 ± 3.3 ng/ml) than in the control group (11.6 ± 2.3 ng/ml; P < 0.05). In addition, the mean AOPP level (41.9 ± 13.5 ng/mg protein) in the tonsil tissue in the patient group was significantly higher than the mean serum AOPP levels in the control and patient groups (P < 0.05). AOPP levels are elevated in the tonsil tissue and serum of patients with chronic tonsillitis compared to the serum AOPP levels in healthy controls. AOPPs may represent a novel class of pro-inflammatory molecules that are involved in oxidative stress in chronic tonsillitis. AOPPs may be used as a marker of oxidative stress in paediatric patients with chronic tonsillitis.

PMID: 27958598 [PubMed - in process]



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Salvage total laryngectomy after conservation laryngeal surgery for recurrent laryngeal squamous cell carcinoma.

Salvage total laryngectomy after conservation laryngeal surgery for recurrent laryngeal squamous cell carcinoma.

Acta Otorhinolaryngol Ital. 2016 Oct;36(5):373-380

Authors: De Virgilio A, Greco A, Bussu F, Gallo A, Rosati D, Kim SH, Wang CC, Conte M, Pagliuca G, De Vincentiis M

Abstract
The aim of the present study was to evaluate the oncological efficacy of salvage total laryngectomy in patients who had previously undergone supracricoid partial laryngectomy or transoral laser microsurgery for treatment of laryngeal squamous cell carcinoma. We retrospectively reviewed the medical, surgical and pathological records of 35 patients who underwent salvage total laryngectomy after recurrence of laryngeal cancer (following supracricoid partial laryngectomy or transoral laser microsurgery). Kaplan-Meier survival curves as well as univariate and multivariate analyses of prognostic factors were performed. No statistically significant differences were seen comparing the supracricoid partial laryngectomy group with the transoral laser microsurgery group for overall survival and disease-specific survival at 3 years (OS = 38% vs. 52%, p = 0.16; DSS = 40% vs. 61%, p = 0.057) or locoregional control at 2 years (LRC = 40% vs. 54%, p = 0.056). A trend indicating worse survival and locoregional control for supracricoid partial laryngectomy patients emerged. Preservation of the osteocartilaginous frame in transoral laser microsurgery could hypothetically result in better salvageability of anterior recurrences with extralaryngeal spread.

PMID: 27958597 [PubMed - in process]



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Lipofilling as refinement procedure in maxillo-mandibular malformations.

Lipofilling as refinement procedure in maxillo-mandibular malformations.

Acta Otorhinolaryngol Ital. 2016 Oct;36(5):368-372

Authors: Cervelli D, Gasparini G, Moro A, Pelo S, Foresta E, Grussu F, D'Amato G, De Angelis P, Saponaro G

Abstract
Maxillo-mandibular asymmetry has numerous aetiologies: congenital, traumatic, iatrogenic and post-oncologic. Patients with congenital dentofacial malformations are generally submitted to orthognathic surgery and/or additional procedures (genioplasty, alloplastic implants) with satisfactory results. However, despite achieving skeletal symmetry, noticeable facial asymmetry may persist.This study was performed in 45 patients (29 women and 16 men) operated between December 2012 and June 2014. All patients were affected by maxilla-mandibular asymmetry and underwent orthognatic surgery for hard tissue correction of the deformity. Residual facial alterations were then treated with lipofilling refinement proceure. In all cases good integration of the grafted fat was observed in the recipient sites. Retrospective analysis of photographic documentation showed progressive volumetric decrease for up to approximately 6 months after surgery; after that graft volume remained relatively stable. There were no significant surgical complications, either from the fat harvest site or the reconstructed site. Mild oedema and bruising were frequent during the first post-operative week. No haematomas, infections, vascular or nervous injuries were recorded. Twenty-four patients felt the need to have a second procedure. A second fat transfer was performed in 22 cases, and a third in 2 (total of 69 procedures). Based on the observations of our study, fat grafting is a simple, effective and reproducible technique, with a high satisfaction rate and few disadvantages or complications. We demonstrated that the success of lipofilling is dependent on the treated aesthetic subunits of the face. The malar and lateral cheek regions seem to be highly favourable for fat grafting, unlike the upper and lower lips subunits. Composite procedures using orthognathic surgery and autologous fat provide the surgeon with an additional, more customisable option for patients with maxillo-mandibular malformations.

PMID: 27958596 [PubMed - in process]



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Childhood neurofibromatosis type 2 (NF2) and related disorders: from bench to bedside and biologically targeted therapies.

Childhood neurofibromatosis type 2 (NF2) and related disorders: from bench to bedside and biologically targeted therapies.

Acta Otorhinolaryngol Ital. 2016 Oct;36(5):345-367

Authors: Ruggieri M, Praticò AD, Serra A, Maiolino L, Cocuzza S, Di Mauro P, Licciardello L, Milone P, Privitera G, Belfiore G, Di Pietro M, Di Raimondo F, Romano A, Chiarenza A, Muglia M, Polizzi A, Evans DG

Abstract
Neurofibromatosis type 2 [NF2; MIM # 101000] is an autosomal dominant disorder characterised by the occurrence of vestibular schwannomas (VSs), schwannomas of other cranial, spinal and cutaneous nerves, cranial and spinal meningiomas and/or other central nervous system (CNS) tumours (e.g., ependymomas, astrocytomas). Additional features include early onset cataracts, optic nerve sheath meningiomas, retinal hamartomas, dermal schwannomas (i.e., NF2-plaques), and (few) café-au-lait spots. Clinically, NF2 children fall into two main groups: (1) congenital NF2 - with bilateral VSs detected as early as the first days to months of life, which can be stable/asymptomatic for one-two decades and suddenly progress; and (2) severe pre-pubertal (Wishart type) NF2- with multiple (and rapidly progressive) CNS tumours other-than-VS, which usually present first, years before VSs [vs. the classical adult (Gardner type) NF2, with bilateral VSs presenting in young adulthood, sometimes as the only disease feature]. Some individuals can develop unilateral VS associated with ipsilateral meningiomas or multiple schwannomas localised to one part of the peripheral nervous system [i.e., mosaic NF2] or multiple non-VS, non-intradermal cranial, spinal and peripheral schwannomas (histologically proven) [schwannomatosis]. NF2 is caused by mutations in the NF2 gene at chromosome 22q12.1, which encodes for a protein called merlin or schwannomin, most similar to the exrin-readixin-moesin (ERM) proteins; mosaicNF2 is due to mosaic phenomena for the NF2 gene, whilst schwannomatosis is caused by coupled germ-line and mosaic mutations either in the SMARCB1 gene [SWNTS1; MIM # 162091] or the LZTR1 gene [SWNTS2; MIM # 615670] both falling within the 22q region and the NF2 gene. Data driven from in vitro and animal studies on the merlin pathway [e.g., post-translational and upstream/downstream regulation] allowed biologically targeted treatment strategies [e.g., Lapatinib, Erlotinib, Bevacizumab] aimed to multiple tumour shrinkage and/or regression and tumour arrest of progression with functional improvement.

PMID: 27958595 [PubMed - in process]



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Anaphylaxis after Zoster Vaccine: Implicating Alpha-Gal Allergy as a Possible Mechanism

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Publication date: Available online 14 December 2016
Source:Journal of Allergy and Clinical Immunology
Author(s): Cosby A. Stone, Jonathan A. Hemler, Scott P. Commins, Alexander P. Schuyler, Elizabeth J. Phillips, R. Stokes Peebles, John M. Fahrenholz

Teaser

A patient with alpha-gal allergy presented with anaphylaxis after receiving zoster vaccine. Subsequent testing of selected vaccines revealed the presence of alpha-gal allergen in MMR and zoster vaccines, which have in common a higher content of gelatin and content of bovine calf serum.


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Guidelines of the French Society of Otorhinolaryngology (SFORL) (short version). Specific treatment of epistaxis in Rendu-Osler-Weber disease

Publication date: Available online 14 December 2016
Source:European Annals of Otorhinolaryngology, Head and Neck Diseases
Author(s): L. Robard, J. Michel, V. Prulière Escabasse, E. Bequignon, B. Vérillaud, O. Malard, L. Crampette
ObjectivesThe authors present the guidelines of the French Oto-Rhino-Laryngology – Head and Neck Surgery Society (Société Française d'Oto-Rhino-Laryngologie et de Chirurgie de la Face et du Cou: SFORL) concerning specific treatment of epistaxis in Rendu-Osler-Weber disease.MethodsA multidisciplinary work-group was entrusted with a review of the scientific literature on the above topic. Guidelines were drawn up, based on the articles retrieved and the group members' individual experience. They were then read over by an editorial group independent of the work group. The final version was established in a coordination meeting. The guidelines were graded as A, B, C or expert opinion, by decreasing level of evidence.ResultsRendu-Osler-Weber disease is diagnosed from the presence of at least three of Curaçao's four criteria. In acute epistaxis, bidigital compression is recommended. Embolization is reserved for resistant epistaxis. Non-resorbable nasal packing and cauterization are contraindicated. Patient education is essential. Telangiectasia of the nasal mucosa can be treated by various local means. In the event of insufficient control, systemic administration of tranexamic acid is recommended.



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Mullerian-Type Ciliated Cyst of the Thigh with PAX-8 and WT1 Positivity: A Case Report and Review of the Literature

Mullerian-type ciliated cysts are uncommon lesions usually found in the lower extremities and perineal region of young females. They have however been reported in males and in other anatomic sites. The cyst lining is typically positive for estrogen receptor (ER), progesterone receptor (PR), PAX-8, and WT1 immunohistochemical stains. This staining pattern has led to the notion that these cysts are of Müllerian origin. The vast majority of cases are located in the dermis where the preferred nomenclature is cutaneous ciliated cyst (CCC). We report a case of Müllerian-type ciliated cyst in the thigh of a 16-year-old girl. Unlike most of the cases reported in the English literature, this cyst was not centered in the dermis. Only a few other cases of Müllerian-type ciliated cysts with no cutaneous connection have been reported. We propose the term ectopic Müllerian cyst for this rare subset of lesions that are not skin based as is the current case.

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Vertebral artery ruptures manifesting as hoarseness

Publication date: Available online 13 December 2016
Source:Brazilian Journal of Otorhinolaryngology
Author(s): Chih-Jen Yang, Sheng-Yao Cheng, Cheng-Chung Cheng, Chi-Tun Tang, Shih-Hung Tsai




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Transoral laser microsurgery versus radiation therapy in the management of T1 and T2 laryngeal glottic carcinoma: which modality is cost-effective within the UK?

Abstract

Objectives

To identify the most cost effective treatment strategy in patients with early stage (T1and T2) cancers of the laryngeal glottis.

Design

A Markov decision model populated using data from updated systematic reviews and meta analyses, with attributable costs from NHS sources. Data on local control and mortality was obtained from updates of existing systematic reviews conducted for the NICE guideline on cancer of the upper aerodigestive tract. Procedure costs were sourced from NHS reference costs 2013/14 by applying tariffs associated with the appropriate health resource group code.

Setting

The UK National Health Service.

Population

Patients with early stage (T1 and T2) cancers of the laryngeal glottis.

Interventions

Transoral laser microsurgery (TLM) and radiation therapy (RT).

Main outcome measures

Total costs, incremental costs and quality adjusted life years (QALYs) over a ten-year time horizon.

Results

RT as the initial treatment strategy was found to be more expensive (£2,654 vs £623) and less effective (QALY reduction of 0.141 and 0.04 in T1a and T1b-T2 laryngeal cancer respectively) than TLM. The dominance of TLM for T1a cancers was unchanged in most scenarios modelled in sensitivity analysis. For T1b-T2 laryngeal cancers, the result changed in numerous scenarios. In probabilistic sensitivity analysis, TLM was found to have a 71% and 58% probability of being cost-effective in T1a and T1b-T2 laryngeal cancer, respectively.

Conclusions

TLM is a cost effective strategy to adopt in the management of T1a laryngeal cancers. Uncertainty remains over the optimal strategy to adopt in T1b-T2 laryngeal cancers.

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