An unusual foreign object mimicking an odontoma in a patient with cleft alveolus: a case report

The habit of inserting foreign objects into body cavities is seen in children and in adults with intellectual disability. Usually, the foreign objects cause chronic inflammation and local tissue destruction, w...

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Delayed response to maintenance therapy after first-line chemotherapy in metastatic intrahepatic cholangiocarcinoma: a case report

Intrahepatic cholangiocarcinoma is an aggressive tumor originating in the epithelium of the bile duct, often associated with distant dissemination. The prognosis is poor and treatment is challenging due to low...

http://ift.tt/2xt1b3I

Cancer risk in patients with Behçet’s disease: A nationwide population-based dynamic cohort study from Korea

A variety of rheumatic diseases are associated with an increased risk of malignancy.BD patients had a higher risk of leukemia, lymphoma, oropharyngeal cancer, thyroid cancer, and prostate cancer, compared to controls in Korea.Physicians should carefully monitor patients with BD for the potential development of malignancy.

http://ift.tt/2wQexrp

Practical aspects of immunoglobulin replacement

A woman was diagnosed with common variable immunodeficiency (CVID) at the age of 42 years. She began having infections when she was 24 years of age. Her history included chronic sinusitis and 3 bacterial pneumonias at different times in both lungs. Her infections necessitated antibiotic therapy almost every other month. A specialist in allergy and immunology finally made the diagnosis of immune deficiency and, specifically, CVID on testing. Her immunoglobulin levels were extremely low: serum IgG, 250 mg/dL; IgA, less than 7 mg/dL; and IgM, 40 mg/dL.

http://ift.tt/2wPiBIn

Idiopathic CD4 lymphocytopenia

Idiopathic CD4 lymphocytopenia (ICL) is a rare condition characterized by an unexplained deficit of circulating CD4 T cells leading to increased risk of serious opportunistic infections. The pathogenesis, etiology, clinical presentation, and best treatment options remain unclear.

http://ift.tt/2yCWcLH

Kounis syndrome should be excluded when physicians treat patients with anaphylaxis

The article, "Epinephrine Use for Anaphylaxis: Too Seldom, Too Late," by Chooniedass et al1 was an interesting read. Therefore, we would like to introduce Kounis syndrome, which is defined as the concurrence of acute coronary syndromes with conditions associated with mast cell and platelet activation and involving interrelated and interacting inflammatory cells in the setting of allergic or hypersensitivity and anaphylactic or anaphylactoid insults.2 Akoz et al3 reported that the annual incidence of Kounis syndrome at the emergency department among all admissions and patients with allergy was 19.4 per 100,000 (27 of 138,911) and 3.4% (27 of 793), respectively.

http://ift.tt/2wQtPMH

Expression of immunoglobulin D is increased in chronic rhinosinusitis

Immunoglobulin (Ig) D is largely localized to the upper airway and reacts with colonizing respiratory pathogens.

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Instructions for Authors

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Comparison of fractional exhaled nitric oxide levels measured using the NIOX VERO and NOA 280i

Fractional exhaled nitric oxide (FeNO) is a type 2 biomarker of eosinophilic airway inflammation and predicts the likelihood of corticosteroid responsiveness in patients with eosinophilic asthma.1,2 Several devices are available to measure FeNO levels for research purposes or in clinical practice worldwide, including the Sievers Nitric Oxide Analyzer (NOA 280i; GE Analytical Instruments, Boulder, Colorado), a stationary chemiluminescence analyzer, and the NIOX VERO (Aerocrine AB, Solna, Sweden), a portable electrochemical analyzer.

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Information for Readers

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An analysis of insurance and other factors associated with asthma-related emergency department visits, 2009–2014

Asthma is a common chronic airway disorder characterized by periods of reversible airflow obstruction known as asthma attacks. Although no cure for asthma is known, control of the frequency and intensity of exacerbations and associated functional limitations is desirable. Uncontrolled asthma has a significant cost to families and society, including costs associated with work and school absenteeism, emergency department (ED) visits, and hospitalizations. Costs associated with asthma, including costs of children and adults missing school or work after an asthma attack, continue to increase.

http://ift.tt/2yD3Zcu

Risk factors for severe anaphylaxis in the United States

Anaphylaxis is an acute systemic allergic reaction and may be life-threatening.

http://ift.tt/2wRnzVb

Contemporary issues in anaphylaxis and the evolution of epinephrine autoinjectors

Food allergy and anaphylaxis appear to be increasing in the United States, especially in young children, and preparedness is paramount to successful emergency management in the community. Although the treatment of choice for anaphylaxis is epinephrine delivered by autoinjection, some devices are challenged by less user-friendly designs or pose the risk of injury, especially in young patients. Human factors engineering has played a larger role in the development of more recent epinephrine autoinjector technologies and will continue to play a role in the evolution and future design of epinephrine autoinjectors.

http://ift.tt/2yD3RK2

Influence of rhinitis control and inspiratory loop flattening on perceived asthma control

Patient-reported outcome measures in asthma and rhinitis are important tools for acute and longitudinal disease monitoring. The Asthma Control Test (ACT)1,2 and the Rhinitis Control Assessment Test (RCAT)3 are 2 brief, valid measures that can be used to monitor the assessment of patient control of these respective conditions. Children and adults with asthma are frequently noted to have comorbid allergic or chronic (nonallergic) rhinitis and vice-versa.4 These conditions can influence each other, and there are emerging hypotheses that suggest there is "one airway" from nose to lung.

http://ift.tt/2wQtM3t

This book is a comprehensive collection of the experimental techniques that are most commonly used for...

This book is a comprehensive collection of the experimental techniques that are most commonly used for basic science research. It is divided into 15 chapters, each chapter representing a different laboratory technique. Within every chapter is a detailed and well-rounded discussion of that particular research method, including descriptions of relevant scientific principles, basic science concepts, and laboratory protocols. The book provides a bridge between basic research and clinical science by including relevant applications, typical scenarios or case studies, and potential limitations, with troubleshooting tips for each experimental method.

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Table of Contents

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Disseminated Mycobacterium avium intracellulare leading to protein-losing enteropathy in an elderly man with idiopathic CD4 lymphocytopenia

Idiopathic CD4 lymphocytopenia (ICL) is a clinical diagnosis in which CD4+ T lymphocytes constitute fewer than 300 cells/μL or fewer than 20% of total T cells without evidence of human immunodeficiency virus (HIV) or any defined immunodeficiency or therapy associated with decreased levels of CD4+ T cells.1 Mycobacterium is one of the most common opportunistic infections in ICL. We report on a unique case of a 64-year-old man with ICL who developed protein-losing enteropathy (PLE) secondary to disseminated Mycobacterium avium intracellulare (MAI) infection that resolved with successful treatment of the mycobacterium infection.

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Editorial Board

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VideoEndocrinology™ High-Impact Videos

VideoEndocrinology™
The Official Journal of: American Thyroid Association

FREE ACCESS through October 9, 2017.
Watch now:

Thyroid Fine Needle Aspiration Smear Preparation
Juan Munoz Pena, Jeffrey Arnold

Transoral Lingual Thyroidectomy for Obstructive Symptoms
Seth Kay, David J. Terris

Population-Based Assessment of Complications After Surgery for Thyroid Cancer
Maria Papaleontiou, Megan R. Haymart

 

The post VideoEndocrinology™ High-Impact Videos appeared first on American Thyroid Association.

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Correlation among ocular surface disease, xerostomia, and nasal symptoms in patients with differentiated thyroid carcinoma subjected to radioiodine therapy: A prospective comparative study

Abstract

Background

Some complications of radioiodine therapy have been reported, but the involvement of the eyes and adnexa is rarely discussed. The purpose of this study was to determine the correlation among ocular surface changes, xerostomia, and changes in the nasal mucosa associated with radioiodine therapy.

Methods

Patients subjected to radioiodine therapy (group 1) or not subjected (group 2) were prospectively evaluated by examinations of the ocular surface and tear film, saliva production, and nasal endoscopy. Ocular and nasal symptoms and xerostomia were evaluated using questionnaires.

Results

Evaluation of the ocular surface did not indicate significant differences between the groups. Nasal endoscopy revealed higher mucosal pallor in group 1 and worsening of the endoscopic appearance. Worsening of ocular symptoms and nasal symptoms, xerostomia, and a significant decrease in salivary production was also observed in group 1.

Conclusion

Subjective worsening of xerostomia, xerophthalmia, nasal symptoms, and changes in the nasal mucosa in group 1 was observed.

http://ift.tt/2fmMgxB

Prognostic nomogram for disease-specific survival of carcinoma ex pleomorphic adenoma of the salivary gland

Abstract

Background

The purpose of this study was to establish an effective prognostic nomogram for carcinoma ex pleomorphic adenoma (Ca-ex-PA) of the salivary gland.

Methods

The nomogram was based on a retrospective study on 223 patients who underwent surgical operation for Ca-ex-PA from 2001 to 2010. The predictive accuracy and discriminative ability of the nomogram were determined by concordance index (C-index) and calibration curve. The results were validated using bootstrap resampling and a prospective study on 111 patients operated on from 2011 to 2012.

Results

On multivariate analysis of the primary cohort, independent factors for disease-specific survival (DSS) were age, tumor diameter, degree of capsule invasion, lymph node metastasis, and the interaction between tumor site and histological subtype, which were all selected into the nomogram. The C-index of the nomogram for predicting DSS was 0.90 and 0.86 in the primary cohort and validation cohort, respectively.

Conclusion

The proposed nomogram resulted in more accurate prognostic predictions for patients with Ca-ex-PA.

http://ift.tt/2wgiGk0

Survival outcomes for stage-matched endoscopic and open resection of olfactory neuroblastoma

Abstract

Background

Advanced-stage olfactory neuroblastoma requires multimodal therapy for optimal outcomes. Debate exists over endoscopic endonasal surgery in this situation. Stage-matched open and endoscopic surgical therapy were compared.

Methods

Patients from 6 cancer institutions were assessed. Stratification included dural involvement, Kadish stage, nodal disease, Hyams' grade, approach, and margin status. At follow-up, local control, nodal status, and evidence of distant metastases were recorded with any subsequent therapy. Statistical analyses to identify risk factors for developing recurrence and survival differences were performed.

Results

One hundred nine patients were assessed (age 49.2 ± 13.0 years; 46% women) representing Kadish A stage (10%), Kadish B stage (25%), and Kadish C stage (65%). The majority of the patients (61.5%) underwent endoscopic resection, 53.5% within Kadish C stage. Within-stage survival analysis favored endoscopic subgroup for Kadish C stage (log-rank P = .017) nonsignificant for Kadish B stage (log-rank P = .39).

Conclusion

Stage-matched survival was better for the endoscopically treated group compared to the open surgery group, with high negative margin resections obtained.

http://ift.tt/2fl9KDd

Upregulation of pAKT(Ser473) expression is involved in progression of HPV-positive oropharyngeal squamous cell carcinoma

Abstract

Background

PIK3CA alterations have been shown to be a frequent event in oropharyngeal squamous cell cancer (SCC), especially in human papillomavirus (HPV)-related tumors.

Methods

Tissue microarrays (TMAs) were used to evaluate pAKT(Ser473)/(Thr308), total protein kinase B (AKT)(pan) and phosphatase and tensin homolog (PTEN) expression in primary tumors and corresponding nodal disease in oropharyngeal SCC. The HPV status was determined in regard of HPV16 DNA and RNA. Survival analysis was performed by using Kaplan-Meier curves, log-rank testing, and multivariate Cox regression analysis.

Results

HPV16 is a prognostic predictive marker for advanced oropharyngeal SCC. pAKT(Ser473) and PTEN are highly expressed in HPV-related oropharyngeal SCCs in contrast to pAKT(Thr308). The pAKT(Ser473) expression increased from primary tumors to progressive nodal disease (21.1%; P < .011).

Conclusion

Activation of phosphoinositide 3-kinase (PI3K)/pAKT(Ser473) frequently occurs in advanced HPV-positive oropharyngeal SCC and elevated pAKT(Ser473) levels represent a feature during progression of oropharyngeal SCC, indicating a critical role of the mammalian target of rapamycin (mTOR) complex. Further studies are required to evaluate specific drugs targeting PI3K/AKT/mTOR in consideration of PIK3CA alterations.

http://ift.tt/2wfPutx

Prognostic biomarkers in patients with human immunodeficiency virus-positive disease with head and neck squamous cell carcinoma

Abstract

Background

We examined the prognostic value of a panel of biomarkers in patients with squamous cell carcinoma of the head and neck (SCCHN) who were human immunodeficiency virus (HIV) positive (HIV-positive head and neck cancer) and HIV negative (HIV-negative head and neck cancer).

Methods

Tissue microarrays (TMAs) were constructed using tumors from 41 disease site-matched and age-matched HIV-positive head and neck cancer cases and 44 HIV-negative head and neck cancer controls. Expression of tumor biomarkers was assessed by immunohistochemistry (IHC) and correlations examined with clinical variables.

Results

Expression levels of the studied oncogenic and inflammatory tumor biomarkers were not differentially regulated by HIV status. Among patients with HIV-positive head and neck cancer, laryngeal disease site (P = .003) and Clavien-Dindo classification IV (CD4) counts <200 cells/μL (P = .01) were associated with poor prognosis. Multivariate analysis showed that p16 positivity was associated with improved overall survival (OS; P < .001) whereas increased expression of transforming growth factor-beta (TGF-β) was associated with poor clinical outcome (P = .001).

Conclusion

Disease site has significant effect on the expression of biomarkers. Expression of tumor TGF-β could be a valuable addition to the conventional risk stratification equation for improving head and neck cancer disease management strategies.

http://ift.tt/2fmM3dN

Mutations de PNPLA1 dans les ichtyoses congénitales autosomiques récessives

Publication date: Available online 25 September 2017
Source:Annales de Dermatologie et de Vénéréologie
Author(s): O. Dereure




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Scars: Are they preventable?

While many scars do not hamper everyday life, some cause life-long problems. Find out how scars form and why it is so difficult to prevent them.

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Simplified conditions for storing and cryopreservation of dental pulp stem cells

Publication date: December 2017
Source:Archives of Oral Biology, Volume 84
Author(s): Nam Cong-Nhat Huynh, Son Hoang Le, Vu Nguyen Doan, Lan Thi Quynh Ngo, Ha Le Bao Tran
ObjectivesThis study aimed to simplify the collection, isolation and cryopreservation procedure of human dental pulp stem cells (DPSCs) to ease the establishment of dental stem cell banking.DesignExtracted third molars were collected and stored either in growth medium or in gentamicin-saline (480μg/ml) for 6, 9 or 12h. DPSCs were isolated and subjected to cryopreservation by a controlled-rate or rapid freezing method in 5 or 10% DMSO. Flow cytometry and growth pattern of DPSCs before and after cryopreservation were conducted.ResultsRate of contamination by which the extracted teeth were stored in control and gentamicin-saline were 9.1% (N=33) and 2.3% (N=43), respectively. Successful cell isolation rate of teeth preserved in gentamicin-saline at 6h (92.9%) was comparable to those of growth media group (90.3%). At 9 and 12h, the rates dropped significantly to 75% and 54%, respectively. Cryopreservation by controlled-rate freezing either in 5 or 10% DMSO resulted in a significantly higher percentage of viable cells than by rapid freezing. Cells conserved by controlled-rate freezing in 5% DMSO showed a pattern of growth similar to control unfrozen cells; 10% DMSO significantly deteriorated the growth pattern of the cells. After thawing, DPSCs conserved by controlled-rate freezing still expressed stemness characteristics, although hematopoietic stem cell markers were slightly increased.ConclusionGentamicin-saline was effective in preserving human teeth for DPSC isolation. Controlled-rate freezing in 5% DMSO gave the highest rate of cell viability. This study simplifies the storage conditions and proposes a simple method for cryopreservation of DPSCs.



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Bacterial Biofilms in Chronic Rhinosinusitis and Their Implications for Clinical Management

Abstract

To study the microbiological profile in patients with chronic rhino-sinusitis. To correlate disease severity with the presence of biofilms and host risk factors. To assess outcome of Sinus Surgery 2 weeks post operatively in terms of presence of bacteria and their ability to form biofilm. Prospective study. 50 cases of chronic rhino-sinusitis requiring Functional Endoscopic Sinus Surgery admitted in SDM Hospital, Dharwad, Karnataka were studied using intra-operative mucosal samples for microbiological analysis. The organisms isolated were tested for biofilm forming ability using three in vitro tests. Severity of disease was assessed using SNOT 22 scoring system. Of 50 cases studied, 66% showed presence of chronic rhino-sinusitis with polyposis and had higher SNOT scores compared to those without polyps. Bacterial isolates were obtained from only 17 samples. Staphylococcus species was isolated from 16 samples and Klebsiella pneumoniae from one. 11 Staph spp. isolates showed biofilm forming ability in vitro. Postoperative events in 3 cases yielded biofilm-forming Staphylococcus. Staphylococcus was the most dominant organism isolated and 11 isolates were biofilm formers. Thus the detection of biofilm forming organisms can be considered as a negative prognostic indicator and should forewarn the surgeon about the risk of recurrence.

http://ift.tt/2wPmLjn

Hörrehabilitation mithilfe von Cochleaimplantaten und kognitive Fähigkeiten

Zusammenfassung

Die Untersuchung kognitiver Fähigkeiten erlangt vor dem Hintergrund des demografischen Wandels zunehmend Relevanz. Die Frage nach dem Effekt einer Cochleaimplantation bei taub geborenen Kindern im Vergleich zu Hörgesunden stand noch bis vor wenigen Jahren im Fokus. Es stellt sich heute die Frage, ob eine Hörrehabilitation bei Älteren, z. B. mithilfe eines Cochleaimplantats (CI), einen protektiven Effekt auf die kognitiven Fähigkeiten und damit auch auf das Demenzrisiko besitzt. Die vorliegende Übersichtsarbeit behandelt die Verknüpfungen der kognitiven Fähigkeiten mit Hörstörungen und Cochleaimplantation. Historische Aspekte der Intelligenztestung werden beschrieben. In der aktuellen Literatur sind Erkenntnisse über kognitive Aspekte bei älteren hörgestörten Patienten und CI-Trägern rar. Erste Untersuchungsergebnisse weisen einen positiven Zusammenhang von Hörverbesserung und Kognition aus. Im Umgang mit kognitiven Defiziten und hörgestörten Patienten sind weitere Untersuchungen zur Erarbeitung von Handlungsempfehlungen dringend erforderlich.

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Clinical evaluation of macrophages in cancer: role in treatment, modulation and challenges

Abstract

The focus of immunotherapeutics has been placed firmly on anti-tumour T cell responses. Significant progress has been made in the treatment of both local and systemic malignancies, but low response rates and rising toxicities are limiting this approach. Advancements in the understanding of tumour immunology are opening up a new range of therapeutic targets, including immunosuppressive factors in the tumour microenvironment. Macrophages are a heterogeneous group of cells that have roles in innate and adaptive immunity and tissue repair, but become co-opted by tumours to support tumour growth, survival, metastasis and immunosuppression. Macrophages also support tumour resistance to conventional therapy. In preclinical models, interference with macrophage migration, macrophage depletion and macrophage re-education have all been shown to reduce tumour growth and support anti-tumour immune responses. Here we discuss the role of macrophages in prognosis and sensitivity to therapy, while examining the significant progress which has been made in modulating the behaviour of these cells in cancer patients.

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Korean Cancer Study Group: Translational bIomarker Driven UMbrella Project for Head and Neck (TRIUMPH), Esophageal Squamous Cell Carcinoma- Part 1 (HNSCC)]

Conditions:   HNSCC;   Head and Neck Neoplasms
Interventions:   Drug: BYL719;   Drug: Poziotinib;   Drug: Nintedanib;   Drug: Abemaciclib;   Drug: Durvalumab,Tremelimumab
Sponsors:   Seoul National University Hospital;   Korean Cancer Study Group;   Chungnam National University Hospital
Recruiting - verified September 2017

http://ift.tt/2fLM0J2

Study to Evaluate the Safety and Tolerability of IACS-010759 in Subjects With Advanced Solid Tumors and Lymphoma

Conditions:   Malignant Neoplasm of Breast;   Malignant Neoplasms of Bone and Articular Cartilage;   Malignant Neoplasms of Digestive Organs;   Malignant Neoplasms of Female Genital Organs;   Malignant Neoplasms of Lip Oral Cavity and Pharynx;   Malignant Neoplasms of Male Genital Organs;   Malignant Neoplasms of Mesothelial and Soft Tissue;   Malignant Neoplasms of Respiratory and Intrathoracic Organs;   Malignant Neoplasms of Thyroid and Other Endocrine Glands;   Malignant Neoplasms of Urinary Tract;   Malignant Neoplasms Stated as Primary Lymphoid Haematopoietic
Intervention:   Drug: IACS-010759
Sponsor:   M.D. Anderson Cancer Center
Not yet recruiting - verified September 2017

http://ift.tt/2xqCaGv

Evaluation of a Web-based Decision Aid Tool for Larynx Cancer (T3/T4) Patients.

Condition:   Larynx Cancer
Interventions:   Other: Interviews;   Other: Treatmentchoice Decisional Tool;   Other: Questionnaires;   Other: Interviews with stakeholders
Sponsors:   Maastricht Radiation Oncology;   MUMC;   The Netherlands Cancer Institute
Recruiting - verified September 2017

http://ift.tt/2fLVtzX

Phase I Study of Intratumoral CV8102

Conditions:   Melanoma (Skin);   Squamous Cell Carcinoma of the Skin;   Carcinoma, Squamous Cell of Head and Neck;   Carcinoma, Adenoid Cystic
Interventions:   Biological: CV8102;   Biological: CV8102 + anti-PD-1 therapy
Sponsor:   CureVac AG
Not yet recruiting - verified September 2017

http://ift.tt/2xqBOzF

Aspirin Improve Survival of N2-3 Nasopharyngeal Carcinoma Patients

Condition:   Nasopharyngeal Carcinoma
Interventions:   Radiation: Radiotherapy;   Drug: Concurrent chemotherapy;   Drug: Aspirin
Sponsor:   Sun Yat-sen University
Not yet recruiting - verified September 2017

http://ift.tt/2jZxkuv

Die intraoperative Parathormonmessung als bester Indikator einer postoperativen symptomatischen Hypokalzämie

Zusammenfassung

Hintergrund

Das Ziel der Studie ist es, Risikofaktoren für eine passagere postoperative Hypokalzämie (HC) aufzuzeigen und Cut-off-Werte für perioperative Parathormon (PTH)- und Kalziumparameter (Ca) zu bestimmen, um die Morbidität aufgrund einer symptomatischen HC zu vermindern.

Material und Methoden

Am Luzerner Kantonsspital wurden im Rahmen einer retrospektiven Studie zwischen 2006 und 2013 insgesamt 353 Patienten hinsichtlich des Risikos einer HC nach totaler Thyreoidektomie statistisch ausgewertet. Zu definierten Zeitpunkten wurden die Laborwerte für Kalzium und PTH im Serum bestimmt.

Ergebnisse

Die Prävalenz der postoperativen laborchemischen HC lag bei 43 %. Eine symptomatische HC entwickelten 10 % der Patienten. Signifikante Risikofaktoren einer laborchemischen und symptomatischen HC waren hierbei die Kalziumwerte (prä-, intra, 4 h und 1 Tag postoperativ), die PTH-Werte (intraoperativ, 4 h und 1 Tag postoperativ) sowie der PTH-Abfall. Die PTH-Werte präoperativ, das Alter der Patienten, das Gewicht der Schilddrüse, die Diagnose sowie das Geschlecht stellten keine signifikanten Risikofaktoren dar. In der ROC-Analyse („receiver operating characteristics") zeigte die Kalziummessung 4 h postoperativ die beste Vorhersagekraft bzgl. laborchemischer HC, wohingegen die PTH-Messung intraoperativ am aussägekräftigsten bzgl. der symptomatischen HC war.

Schlussfolgerung

Sowohl Kalzium‑/PTH-Werte als auch ein Abfall der PTH-Werte im Verlauf sind signifikante Risikofaktoren einer postoperativen HC. Präoperativ ist nur die Kalziummessung prognostisch signifikant. Die intraoperative PTH-Messung ist im klinischen Setting der sinnvollste und sensitivste Faktor, um eine postoperative passagere HC frühzeitig zu erkennen.

http://ift.tt/2fl96W5

Frequent oncogenic BRAF V600E mutation in odontogenic keratocyst

Publication date: November 2017
Source:Oral Oncology, Volume 74
Author(s): Yong Hoon Cha, Eunae Sandra Cho, Hee Eun Kang, Jaemin Ko, Woong Nam, Hyung Jun Kim, Nam Hee Kim, Hyun Sil Kim, In-Ho Cha, Jong In Yook
ObjectivesOdontogenic keratocyst (OKC), also known as keratocystic odontogenic tumor (KCOT), has clinical significance due to its high incidence as well as high recurrence rate after surgical enucleation. Current clinical management for OCK is entirely dependent on surgical approach. While various genetic alterations, such as PTCH1 mutation and loss of heterozygosity in tumor suppressor genes, have been reported, the molecular background of OKC is not well-understood. Although recent identification of BRAF V600E mutation and subsequent activation of mitogen-activated protein kinase (MAPK) pathway in ameloblastoma and odontogenic tumors provide additional options with targeted therapeutics, the molecular background of OKC is not well understood.Materials and methodsIn this study, we examined BRAF V600E mutation from paraffin embedded OKC samples by tumor cell enriched microdissection and TA cloning of amplified DNA. We further examined the relationship between BRAF V600E mutation and clinical parameters.ResultsWe found frequent BRAF V600E mutation in OKC (24 of 38 samples, 63.2%). However, BRAF V600E mutational status is not related with clinical indexes such as size, location, and recurrence. In orthokeratinized odontogenic cyst, there is one case of BRAF 600E mutation from 11 samples (9.1%).ConclusionThese results indicate that BRAF V600E mutation occurs in OKCs at a high rate and plays an important role in the pathogenesis of OKCs.



http://ift.tt/2wgwxqB

Polarized transilluminating dermoscopy: Bedside trichoscopic diagnosis of trichothiodystrophy

Abstract

Trichothiodystrophy is a rare autosomal recessive disorder resulting in a broad range of systemic abnormalities. Polarizing microscopy of the hair reveals the pathognomic "tiger tail" of alternating light and dark bands, but the need for a microscope prevents rapid bedside diagnosis. We describe a new technique for the bedside diagnosis of trichothiodystrophy using a handheld polarizing dermatoscope, precluding the need for microscopic examination.

http://ift.tt/2yAVRZZ

Self-limited neonatal periumbilical erythema

Abstract

We report three cases of neonatal, noninfectious, periumbilical erythema that resolved shortly after umbilical stump detachment. We hypothesize that these infants experienced an inflammatory and vasodilatory response during the normal umbilical cord separation process. We propose a new term: self-limited neonatal periumbilical erythema.

http://ift.tt/2wOW3aU

Comparison of psoriasis and atopic dermatitis guidelines—an argument for aggressive atopic dermatitis management

Abstract

The development of effective systemic treatments has revolutionized the treatment of inflammatory skin diseases. The availability of safe new treatments and the understanding of psoriasis as a systemic disease with comorbidities and effects on quality of life have driven the current aggressive treatment paradigm of psoriasis. Historically the morbidity of atopic dermatitis (AD) has been dismissed, given the perception of AD as "just" a rash. Differences in the guidelines for psoriasis and AD management may suggest variations in the current conceptualization of disease severity and effects on quality of life. Published guidelines from the American Academy of Dermatology for the management of psoriasis and AD were reviewed. We recorded the similarities and differences in disease assessment and therapy. The threshold to use biologic agents for moderate to severe psoriasis highlights the aggressive nature of modern psoriasis treatment. AD guidelines include an assessment of quality of life but do not designate a disease severity threshold for systemic treatment. AD and psoriasis have a tremendous effect on quality of life. The AD guidelines have a less aggressive approach to disease management than the psoriasis guidelines. We should think critically about rapid advancement to systemic agents in AD management, especially now that more and better agents are being developed.

http://ift.tt/2yCccxg

Inpatient management of children with recessive dystrophic epidermolysis bullosa: A review

Abstract

Recessive dystrophic epidermolysis bullosa is a disorder marked by skin and mucosal blistering after minimal trauma. Even the most routine procedures in the hospital, if done incorrectly, can precipitate extensive skin loss, pain, and scarring. Most providers have little experience working with patients with this degree of skin fragility. When a person with recessive dystrophic epidermolysis bullosa is admitted to the hospital, there are multiple considerations to keep in mind while strategizing an effective care plan: avoidance of new blisters with a "hands-off" approach; careful consideration of all indwelling devices; symptomatic management of pain, itch, and anxiety; coordination of dressing changes; aggressive treatment of skin infections; environmental and staffing considerations; and awareness of other chronic complications that affect care, such as anemia, malnutrition, and chronic pain. To minimize discomfort for patients with recessive dystrophic epidermolysis bullosa during the hospital stay, inpatient care teams should understand these considerations and modify the care plan accordingly. Prior preparation by the hospital facility and inpatient care team will facilitate the delivery of safe and effective care and greatly improve the overall patient experience.

http://ift.tt/2wNsVka

Pediatric vaccine-strain herpes zoster: a case series

Abstract

Background/objectives

Herpes zoster (HZ) is caused by reactivation of the varicella zoster virus (VZV), and typically causes a painful, vesicular, dermatomal rash in adults over the age of fifty. However, HZ has been known to present in immunocompetent pediatric patients, which account for under 1% of total cases. Pediatric cases are typically caused by natural infection with VZV, but among vaccinated children up to half of cases can be due to vaccine-strain VZV. We present two such cases of vaccine-strain HZ in pediatric patients.

Methods

This is a retrospective study of two cases seen at UCLA-affiliated sites. PCR and Sanger sequencing, using previously described PCR primers, determined the presence of two vaccine-strain-specific single nucleotide polymorphisms.

Results

We report two cases of vaccine-strain HZ in immunocompetent pediatric patients who had previously received the varicella vaccine, affecting the right thigh in the first patient and the left leg in the second. Varicella-strain VZV positivity was confirmed by PCR. Both patients had received the varicella vaccine at 12 months of age. Both patients achieved complete resolution of symptoms after 7-day courses of acyclovir.

Conclusions

While vaccination against VZV has overall reduced the incidence of both varicella and HZ in US children, given the widespread use of the VZV vaccine, awareness of the possibility of vaccine-induced HZ remains an important consideration.

http://ift.tt/2xA8Bl1

Treatment of immune thrombocytopenic purpura associated with cytomegalovirus infection in a child with pre-B cell acute lymphoblastic leukaemia after central nervous system relapse

A 13-year-old male patient with a history of pre-B cell acute lymphoblastic leukaemia (ALL) with isolated central nervous system relapse on maintenance chemotherapy presented with severe thrombocytopenia refractory to platelet transfusions. The patient showed only modest responses to two courses of intravenous immunoglobulin and steroids. He was found to be positive for cytomegalovirus (CMV) with modest viral load. His thrombocytopenia normalised with rituximab therapy and CMV treatment supporting the diagnosis of CMV-associated immune thrombocytopenic purpura (ITP). Following treatment, the patient continued to have a stable platelet count well above the threshold for transfusions. He continued to be intermittently treated for CMV when viral loads became detectable. This report discusses the unique management considerations of ITP in a patient undergoing therapy for ALL with a review of previously reported cases and discusses the possibility of CMV viraemia as a modulating factor.

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Unusual presentation of silently growing abdominal aortic aneurysm causing biliary obstruction

Biliary obstruction is a rare presentation of abdominal aortic aneurysm (AAA). The most common symptoms of AAA are abdominal or back pain and limb ischaemia from thromboembolism. We report a case of a 67-year-old male who was diagnosed with obstructive jaundice secondary to an AAA. CT angiogram revealed compression of the common bile duct by the large AAA, causing diffuse intrahepatic and extrahepatic ductal dilatation. Surgical repair of the aortic aneurysm was successful, and patient's symptoms improved.

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The eye as a complement dysregulation hotspot

Abstract

Complement turnover is tightly regulated throughout the human body in order to prevent over-activation and subsequent damage from inflammation. In the eye, low-level complement activation is maintained to provide immune tolerance in this immune privileged organ. Conversely, the complement system is suppressed in the cornea to protect it from continuous immunological insult. Over-activation of the complement cascade has been implicated in the disease progression of glaucoma and diabetic retinopathy and is now known to be a central driver in the pathogenesis of age-related macular degeneration (AMD). Indeed, it is with AMD where the most recent and exciting work has been carried out with complement-based therapies entering into clinical trials. However, the success of these trials will depend upon delivering the therapeutics to the correct anatomical sites within the eye, so a full understanding of how complement regulation is compartmentalized in the eye is required, a topic that will be highlighted in this review.

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Paclitaxel-related scleredema-like skin changes in a patient with breast cancer

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A composite of exhaled LTB4, LXA4, FeNO and FEV1 as an “asthma classification ratio” characterizes childhood asthma

Abstract

Background

Aberrant generation of eicosanoids is associated with asthma, but the evidence remains incomplete and its potential utility as biomarkers is unclear. Major eicosanoids in exhaled breath condensates (EBCs) were assessed as candidate markers for childhood asthma.

Methods

Ten exhaled eicosanoid species was evaluated using ELISA in the discovery phase, followed by prediction model-building and validation phases.

Results

Exhaled LTB₄, LTE₄, PGE₂ and LXA₄ showed significant difference between asthmatics (N=60) and controls (N=20). For validation, an expanded study population consisting of 626 subjects with asthma and 161 healthy controls were partitioned into a training subset to establish a prediction model and a test sample subset for validation. Receiver Operating Characteristic (ROC) analyses of the training subset revealed the level of exhaled LTB₄ to be the most discriminative among all parameters, including FeNO, and a composite of exhaled LTB₄, LXA₄, together with FeNO and FEV₁, distinguishing asthma with high sensitivity and specificity. Further, the Youden index (J) indicated the cut point value of 0.598 for this composite of markers as having the strongest discriminatory ability (sensitivity = 85.2% and specificity = 83.6%). The predictive algorithm as "asthma classification ratio" was further validated in an independent test sample with sensitivity and specificity being 84.4% and 84.8%, respectively.

Conclusions

In a pediatric study population in Taiwan, the levels of exhaled LTB₄, LTE₄, LXA₄ and PGE₂ in asthmatic children were significantly different from those of healthy controls, and the combination of exhaled LTB₄ and LXA₄, together with FeNO and FEV₁, best characterized childhood asthma.

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Comparative Study of New Autologous Material, Bone-Cartilage Composite Graft (BCCG), for Ossiculoplasty with Polycel® and Titanium

Abstract

Objective

Ossiculoplasty is a surgical procedure that recreates sound transmission of the middle ear in conductive hearing loss. Various materials have been used for ossicular reconstruction, but the most ideal material for ossiculoplasty remains controversial. The purpose of this study is to introduce a novel method of autologous ossiculoplasty, bone-cartilage composite graft (BCCG), and to compare its surgical results with different types of ossiculoplastic prostheses.

Study Design

A retrospective study was performed in a tertiary referral center.

Methods

Data of 275 patients who received ossiculoplasty using the three different materials of BCCG, Polycel^®, and titanium were analyzed according to type of ossiculoplasty: partial or total ossicular replacement prosthesis (PORP or TORP). Hearing results, complication rates, and clinical parameters including age, sex, past history, preoperative diagnosis, and surgery type were compared among different groups.

Results

Ossiculoplasty with BCCG showed satisfactory hearing outcomes and the lowest complication rate among the three different materials. In particular, its extrusion rate was 0%.

Conclusion

We propose that the BCCG technique is a useful alternative method for ossiculoplasty, with proper patient selection.

This article is protected by copyright. All rights reserved.

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The effect of side of implantation on unilateral cochlear implant performance in patients with prelingual and postlingual sensorineural hearing loss – a systematic review

Abstract

Objective

Cerebral lateralization of language processing leads to a right ear advantage in normal hearing subjects. The aim of this study was to present a systematic overview of the effect of implantation side on postoperative cochlear implant performance in patients with symmetrical severe to profound sensorineural hearing loss.

Data sources

PubMed, Embase and The Cochrane Library databases.

Research methods

Databases were searched from database inception up to January 9^th 2017 for cochlear implant and side and all synonyms. Title, abstract and full-text of retrieved articles were screened for eligibility. Then, directness of evidence and risk of bias were assessed. For the included articles, study characteristics and outcome data (hearing and language development) were extracted.

Results

2541 unique articles were screened, of which twenty were eligible for critical appraisal. No randomized controlled trials were identified. Twelve studies with a high directness of evidence remained for data extraction. Four of six studies including children with prelingual sensorineural hearing loss and four of seven studies investigating adults with postlingual sensorineural hearing loss found a right ear advantage in at least one outcome measurement related to cochlear implant performance.

Conclusion

The available evidence on the effect of side of implantation is of low quality, since study populations and outcome measures are heterogeneous. The majority of studies reveals evidence for a right ear advantage in prelingually deafened children as well as postlingually deafened adults. In view of the present evidence and since no left ear advantage was identified, we cautiously advise implanting the cochlear implant in the right ear when other prognostic factors do not favour the left ear and sensorineural hearing loss is symmetrical.

This article is protected by copyright. All rights reserved.

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Phase I/II trial of dendritic cell-based active cellular immunotherapy with DCVAC/PCa in patients with rising PSA after primary prostatectomy or salvage radiotherapy for the treatment of prostate cancer

Abstract

Objective

Immunotherapy of cancer has the potential to be effective mostly in patients with a low tumour burden. Rising PSA (prostate-specific antigen) levels in patients with prostate cancer represents such a situation. We performed the present clinical study with dendritic cell (DC)-based immunotherapy in this patient population.

Materials and methods

The single-arm phase I/II trial registered as EudraCT 2009-017259-91 involved 27 patients with rising PSA levels. The study medication consisted of autologous DCs pulsed with the killed LNCaP cell line (DCVAC/PCa). Twelve patients with a favourable PSA response continued with the second cycle of immunotherapy. The primary and secondary objectives of the study were to assess the safety and determine the PSA doubling time (PSADT), respectively.

Results

No significant side effects were recorded. The median PSADT in all treated patients increased from 5.67 months prior to immunotherapy to 18.85 months after 12 doses (p < 0.0018). Twelve patients who continued immunotherapy with the second cycle had a median PSADT of 58 months that remained stable after the second cycle. In the peripheral blood, specific PSA-reacting T lymphocytes were increased significantly already after the fourth dose, and a stable frequency was detected throughout the remainder of DCVAC/PCa treatment.

Summary

Long-term immunotherapy of prostate cancer patients experiencing early signs of PSA recurrence using DCVAC/PCa was safe, induced an immune response and led to the significant prolongation of PSADT. Long-term follow-up may show whether the changes in PSADT might improve the clinical outcome in patients with biochemical recurrence of the prostate cancer.

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Efficacy of dexmedetomidine on perioperative morbidity during nasal surgery: A meta-analysis

Objectives

The goal of this meta-analysis was to perform a systematic review of the literature on the effect of dexmedetomidine on perioperative morbidity following nasal surgery and on the adverse effects of dexmedetomidine.

Data Sources

MEDLINE, Scopus, and Cochrane Database of Systematic Reviews.

Review Methods

Two authors independently searched scientific and medical databases from their inception of article collection to March 2017. Studies that compared perioperative dexmedetomidine administration (dexmedetomidine group) with another agent under monitored anesthesia care (MAC) or general anesthesia (control group) with outcomes of interest that were perioperative pain intensity; rescue analgesic consumption; or adverse effects such as hemodynamic instability, nausea, and vomiting (PONV), and operative bleeding were included in the analysis.

Results

Perioperative pain scores and postoperative need for analgesics were significantly decreased in the dexmedetomidine group versus control group (other agent or general anesthesia). In subgroup analysis according to anesthesia type (general anesthesia and other sedatives in MAC), dexmedetomidine showed a similar effect on bradycardia, hypotension, and desaturation with general anesthesia, but it reduced PONV effectively compared with general anesthesia. Additionally, dexmedetomidine satisfied patient significantly. By contrast, compared with other sedative under MAC, it provoked bradycardia significantly.

Conclusion

This meta-analysis showed that systemic administration of dexmedetomidine efficiently can decrease intraoperative and postoperative pain without adverse effects such as nausea, vomiting, and respiratory depression. Dexmedetomidine also can decrease analgesic consumption. However, clinicians should be aware of the potential for intraoperative bradycardia; patients also should be educated regarding these possibilities.

Level of Evidence

NA. Laryngoscope, 122, 2017

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Long-term impact of endoscopic orbital decompression on sinonasal-specific quality of life

Objective

Endoscopic orbital decompression (EOD) is the workhorse surgical intervention for severe thyroid eye disease in Graves disease. Although EOD is a safe and effective procedure. The objective of this study is to determine the impact of orbital decompression on long-term sinonasal-specific quality of life.

Methods

Retrospective study of 27 patients who underwent EOD by a single surgeon. The primary endpoint was change in preoperative 22-item Sinonasal Outcomes Test (SNOT-22) score at a minimum of 1 year. The secondary endpoint was to determine whether the performance of septoplasty for surgical access in patients without nasal obstruction impacted domain 1 (i.e., rhinologic domain) and total SNOT-22 scores.

Results

The mean follow-up was 25.7 ± 11.4 months. Domain 1 scores significantly increased at the first postoperative visit (P ≤ 0.01) and returned to baseline values between 1 and 3 months. At 1 year, significant improvements in both total score and domain 4 and 5 (psychological and sleep dysfunction, respectively) scores were seen (P < 0.01 for all scores). Septoplasty was not associated with a significant change in SNOT-22 score at 1 year (P = 0.48).

Conclusion

Endoscopic orbital decompression is associated at 1 year with a significant improvement in sinonasal-specific quality of life, which is driven by the psychological and sleep dysfunction domains. Adjunctive septoplasty has no significant impact on SNOT-22 scores.

Level of Evidence

4. Laryngoscope, 2017

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Morbidity and mortality associated with preclinical tracheostomy models

Objectives/Hypothesis

A secure airway is critical to study obstructive disorders of the larynx and trachea in preclinical models. Tracheostomy has been described in rabbits, swine, canines, and other mammals using tracheostomy tubes or permanent stomas. No studies specifically evaluated morbidity and mortality associated with these models, and existing studies using tracheostomy make little mention of tracheostomy-related complications. We assessed the management, complications, and mortality associated with tracheostomy in a rabbit model that has recently gained significant attention.

Study Design

In vivo.

Methods

Twenty-two female rabbits underwent tubeless tracheotomy. Rabbits were monitored hourly for the first 8 hours, with progressively increasing intervals between evaluations up to 7 days. A suctioning and tracheal moisture protocol was employed, and animals with signs of crusting or impending airway compromise underwent therapeutic bronchoscopy.

Results

Nine of 22 (41%) rabbits succumbed to tracheostomy-related complications, ranging from 1 to 7 days after tracheotomy. The experiment consisted of two study groups. The preliminary group of 10 rabbits studied over a 4-day period had 40% mortality. After implementing modified preventive therapy guidelines and a new humidification system, the second group of 12 rabbits studied over a 7-day period had 42% mortality. Average time to unrecoverable complication was 2.2 days (median = 2 days). Cause of death was airway obstruction in four animals and respiratory depression in three animals, and two animals were found unresponsive.

Conclusion

Tracheostomy in preclinical rabbit models should be temporally limited, and investigators should anticipate tracheostomy-related complications during study design.

Level of Evidence

NA. Laryngoscope, 2017

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Pharmacologic management of voice disorders by general medicine providers and otolaryngologists

Objectives

1) To compare laryngeal diagnoses from general medical providers (GMP) to otolaryngologists following GMP-based medication trial, and 2) to evaluate associations between GMP medication trials and pharmacologic treatment by otolaryngologists.

Methods

Retrospective cohort analysis using large, national administrative U.S. claims database. Patients with laryngeal/voice disorders as per the International Classification of Diseases, Ninth Revision, Clinical Modification codes from January 1, 2010, to December 31, 2012, seen by a GMP and then an otolaryngologist between 2 weeks to 3 months after the GMP visit, were included. Patient demographics, comorbid conditions, medication use, and initial GMP and otolaryngology laryngeal diagnoses were collected. Logistic regression was performed to evaluate the association between GMP and otolaryngologist medication trials.

Results

A total of 12,475 unique laryngeal/voice-disordered patients met inclusion criteria. At the initial GMP visit, 15.3% received an antibiotic, 14.0% a proton pump inhibitor (PPI), and 7.7% an oral steroid. After the otolaryngology visit, increased diagnoses of vocal fold paralysis/paresis, benign vocal fold/laryngeal pathology, chronic laryngitis, and multiple diagnoses occurred. The adjusted odds for an otolaryngologist prescribing an antibiotic, PPI, or oral steroid, respectively, given that a GMP prescribed an antibiotic, PPI, or oral steroid, was roughly two to three times higher that of a GMP not prescribing the given medication.

Conclusion

Patients with structural and neuromuscular laryngeal disorders were treated with medications by GMPs, and similar mediations often were repeated after otolaryngology evaluation. These findings suggest potential areas of unnecessary pharmacologic treatment of laryngeal/voice-disordered patients.

Level of Evidence

2b. Laryngoscope, 2017

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Präoperative Bestimmung des Ursprungsnervs von Vestibularisschwannomen

Zusammenfassung

Hintergrund

Vestibularisschwannome (VS) sind gutartige Tumoren, die sich im inneren Gehörgang und im Kleinhirnbrückenwinkel entwickeln und potenziell das Hörvermögen oder den Gleichgewichtssinn beeinträchtigen. Die meisten VS haben ihren Ursprung im N. vestibularis inferior oder dem N. vestibularis superior. Die sichere Bestimmung des Ursprungsnervs könnte die präoperative Beratung von Patienten, Behandlungsauswahl sowie die operative Entscheidungsfindung und Planung verbessern. Das Ziel dieser Studie war es, ein neuartiges Scoring-System vorzustellen, das entwickelt wurde, um den Ursprungsnerv zu bestimmen.

Methoden

Der Ursprungsnerv wurde auf der Grundlage der Ergebnisse des Video-Kopfimpulstests aller Bogengänge und den zervikal sowie okulär vestibulär evozierten myogenen Potenzialmessungen vorausgesagt. Die Ergebnisse wurden mit einem Scoring-System zur Bestimmung des Tumorursprungsnervs bewertet. Die definitive Bestimmung des Ursprungsnervs erfolgte intraoperativ.

Ergebnisse

Das neuentwickelte Scoring-System wurde auf 5 aufeinander folgende Patienten angewendet, die sich einer Operation zur VS-Resektion unterzogen. In einem Fall war keine Festlegung möglich. In allen anderen Fällen entsprach der präoperativ vorhergesagte Tumorursprung dem während der Operation bestimmten Ursprungsnerv.

Schlussfolgerung

Das entwickelte Scoring-System eignet sich zur Vorhersage des Ursprungsnervs eines VS und wird demnächst in einer größeren, prospektiven Studie an Patienten mit VS evaluiert.

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Asymptomatic cutaneous lesions with zosteriform distribution

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