Ganglioneuroblastoma is a tumor of peripheral neuroblastic tissue which occurs predominantly in the pediatric age group; it is a rare occurrence in the newborn period with only one case reported at birth to date.
http://ift.tt/2wkbnZC
Δευτέρα 28 Αυγούστου 2017
Ganglioneuroblastoma in a newborn with multiple metastases: a case report
Takayasu arteritis presenting as embolic stroke
A 52-year-old Caucasian woman presented to the emergency department with symptoms of acute ischaemic stroke (right-side weakness, confusion and aphasia) that resolved completely after administration of tissue plasminogen activator. During stroke work-up, she was found to have an enhancing infiltrate of the aorta at the level of the take-off of the great vessels, most consistent with early Takayasu arteritis. After being discharged home on steroids and dual antiplatelet therapy, she returned 2 days later with re-presentation of weakness and aphasia. Further work-up revealed two intraluminal clots in the left common carotid and left internal carotid arteries that had not been discovered during previous testing. This case illustrates the need to screen for sources of embolic stroke in patients with Takayasu arteritis, especially those with recurring symptoms.
http://ift.tt/2iDQwwR
Oesophageal mastocytosis: eosinophilic oesophagitis without eosinophils?
A 59-year-old male with a history of lifelong asthma, allergic rhinitis and hypercholesterolaemia presented to the emergency department for management of severe substernal chest pain with radiating pain to his left arm, nausea and diaphoresis. Physical examination was unrevealing and a cardiac workup including cardiac enzymes, ECG, chest radiographs were negative for an underlying ischaemic event. A subsequent gastrointestinal workup including oesophageal manometry and oesophagogastroduodenoscopy revealed elevated lower oesophageal pressures and histopathology suggestive of mast cell proliferation, respectively. These findings were suggestive of oesophageal mastocytosis. Treatment with omeprazole-sodium bicarbonate, cetirizine, montelukast and oral budesonide promptly ameliorated his symptoms which have not recurred.
http://ift.tt/2gkQLMG
Continuous remission of renal cell carcinoma with tumour thrombus after severe adverse events following short-term treatment with sunitinib
A 64-year-old Japanese man with renal cell carcinoma (RCC) and tumour thrombus in the inferior vena cava was treated with sunitinib. Two weeks after treatment, he was hospitalised for disturbance of consciousness. Laboratory tests revealed high-grade hypoglycaemia, hyponatraemia, liver dysfunction and thrombocytopaenia with disseminated intravascular coagulation. Sunitinib was discontinued and the patient recovered after a protracted platelet transfusion. At 5 months after treatment, CT revealed that the tumour thrombus had disappeared and other lesions had regressed. MRI at 15 months revealed further regression and suggested the possibility of histological remission according to the signal intensity of fibrosis. A partial response persisted at 20 months after treatment, despite residual accumulation in the renal tumour evident on positron emission tomography. In summary, we present a case of locally advanced RCC accompanied by severe adverse events that showed a significant and durable response to treatment with sunitinib for just 2 weeks.
http://ift.tt/2iDQg0R
Autoinflammatory Diseases in Pediatric Dermatology-Part 1: Urticaria-like Syndromes, Pustular Syndromes, and Mucocutaneous Ulceration Syndromes
Autoinflammatory Diseases in Pediatric Dermatology–Part 2: Histiocytic, Macrophage Activation, and Vasculitis Syndromes
Diagnostic and Prognostic Relevance of the Cutaneous Manifestations of Neurofibromatosis Type 2
Impact of Vitiligo on Quality of Life
Treatment of Human Scabies with Oral Ivermectin. Eczematous Eruptions as a New Non-Reported Adverse Event
Reliability of the MDi Psoriasis Application to Aid Therapeutic Decision-Making in Psoriasis
Guitar-String Suture to Facilitate Closure of a Finger-like Flap for Reconstruction of the Nose
Acral Hemorrhagic Darier Disease
A reddish plaque in the forehead
Slow-Growing Keratotic Tumor on the Eyelid
RF-Acral Melanoma and Repetitive Injury to the Sole of the Foot
Coexistence of Sutton and Meyerson Nevi
Isolated Anterior Cervical Hypertrichosis
Edematous Dermatomyositis with Probable Evans Syndrome
Reticulate Acropigmentation of Kitamura and Nevus of Ito
Melanomas Arising on Tattoos: A Casual Association with Practical Implications
Adjuvant radiation and survival following surgical resection of sinonasal melanoma
Surgery remains the mainstay of treatment for sinonasal melanoma, but it is often difficult to obtain clear, negative margins. Therefore, patients often receive adjuvant radiation therapy (RT), however its impact on overall survival (OS) is not well understood.
http://ift.tt/2wdMIav
Effects of surgical treatment of hypertrophic turbinates on the nasal obstruction and the quality of life
Chronic hyperplasia of the inferior nasal concha is accompanied by a nasal obstruction; however, there is no standardised surgical treatment for this condition. Here, we compared the outcome of three surgical techniques frequently used to treat the hyperplasia of inferior turbinates: turbinectomy with lateralization, submucosal electrocautery and laser cautery additional to septoplasty.
http://ift.tt/2xsQuvk
Multiple correspondence analysis as a strategy to explore the association between categories of qualitative variables related to oral–maxillofacial trauma and violent crimes at the community level
The main objective of this study was to show the applicability of multiple correspondence analysis (MCA) in the detection and representation of underlying structures in large datasets used to investigate oral–maxillofacial lesions. A cross-sectional study was conducted involving the analysis of the medical–forensic and social records of 992 people who experienced oral–maxillofacial trauma resulting from non-fatal violent crimes that occurred in the community. Based on the MCA results, two distinct victim profiles were identified.
http://ift.tt/2wjX6vI
Success rates and complications of autologous onlay bone grafts and sinus lifts in patients with congenital hypodontia and after trauma
Autogenous bone remains the gold standard for augmentation of the alveolar ridge in congenital hypodontia and appreciable post-traumatic deformity. This generally reflects the volume of material required for such defects and the osteogenic potential of the grafts. Morbidity at the donor site and success rates may lead to autogenous grafts being superseded by xenografts or alloplastic materials in the future, but we know of little evidence to confirm this. All patients having augmentation of the alveolar ridge or sinus lift to enable subsequent placement of implants between 01 January 2009 and 31 December 2016 were identified from a prospectively-gathered database held at the Queen Elizabeth Hospital, Birmingham.
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Emergency consultation for epistaxis: A bad predictor for overall health?
To compare the mortality rate of a large epistaxis cohort with the fatalities of the general Swiss population and to evaluate significant risk factors for impending early death.
http://ift.tt/2xIci5z
Long-term voice outcome after thyroidectomy using energy based devices
Voice dysfunction is frequently reported after thyroidectomy even in absence of vocal fold paralysis. The energy-based devices such as Harmonic scalpel (HS) or LigaSure (LS) are commonly used in conventional thyroidectomy. The objective of this study was to investigate the long-term voice outcome after total thyroidectomy using energy based devices.
http://ift.tt/2wNyZZq
Cytomegalovirus Viral Load in Bronchoalveolar Lavage to Diagnose Lung Transplant Associated CMV Pneumonia.
Background: The diagnostic yield for cytomegalovirus (CMV) PCR viral load in Bronchoalveolar Lavage (BAL) or in plasma to diagnose CMV pneumonia in lung transplant recipients remains uncertain, and was investigated in a large cohort of consecutive lung transplant recipients. Methods: Bronchoscopies within the first year of lung transplantation with CMV detectable in BAL by PCR (ie, viral load >=273 IU/mL) were included (66 recipients; 145 bronchoscopies); at each bronchoscopy episode 2 independent experts reviewed clinical and laboratory information to determine whether the patient at that time fulfilled the criteria for CMV pneumonia per current international recommendations. Corresponding plasma CMV PCR viral load determined at time of the bronchoscopy (n=126) was also studied. Optimal CMV PCR viral load cut off for CMV pneumonia diagnosis was determined using receiver operating characteristics (ROC). Results: CMV was detected in BAL with CMV PCR in 145 episodes, and 34 (23%) of these episodes fulfilled the criteria for CMV pneumonia. The AUC-ROC for CMV in BAL was 90% at the optimum cut off (4545 IU/mL) with a corresponding sensitivity of 91% and specificity of 77% (in plasma the corresponding values were 274 IU/mL, 63% and 76%, respectively). Conclusions: CMV PCR viral load in BAL had a high performance to diagnose CMV pneumonia in lung transplant recipients; plasma CMV viral load did not reliably aid as a diagnostic tool. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.
http://ift.tt/2xHBeK9
http://ift.tt/2xHBeK9
Sofosbuvir-based Regimens in HIV/HCV Coinfected Patients after Liver Transplantation: Results from the ANRS CO23 CUPILT Study.
Background: A recurrence of hepatitis C virus after liver transplantation affects survival in HIV/HCV coinfected patients. This study assessed the efficacy and safety of sofosbuvir-based regimens in HIV/HCV coinfected patients following liver transplantation. Methods: 29 HIV/HCV coinfected transplanted patients receiving tacrolimus, cyclosporine or everolimus-based immunosuppressive therapy were enrolled in the CUPILT cohort. Their antiviral treatment combined sofosbuvir, daclatasvir with or without ribavirin (n=10/n=6), or sofosbuvir, ledipasvir with or without ribavirin (n=2/n=11). Results: The median delay between liver transplantation and treatment initiation was 37.5 months (IQR 14.4-99.2). The breakdown of HCV genotypes was: G1: 22 patients (75.9%), G3: 3 patients (10.3%) and G4: 4 patients (13.8%). The treatment indications were HCV recurrence (>= F1 n=23) or fibrosing cholestatic hepatitis (n=6). Before starting sofosbuvir, the HCV viral load and CD4 count were 6.7 log10 IU/mL (IQR 5.9-7.2), and 342 cells/mm3 (IQR 172-483), respectively. At W4, the HCV viral load was
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http://ift.tt/2xHmo6F
Swine Leukocyte Antigen (SLA) Class II is a Xenoantigen.
Background: Over 130 000 patients in the United States alone need a life-saving organ transplant. Genetically modified porcine organs could resolve the donor organ shortage, but human xenoreactive antibodies destroy pig cells and are the major barrier to clinical application of xenotransplantation. The objective of this study was to determine whether waitlisted patients possess preformed antibodies to swine leukocyte antigen (SLA) class II, homologs of the class II human leukocyte antigens (HLA). Methods: Sera from people currently awaiting solid organ transplant were tested for IgG binding to class II SLA proteins when expressed on mammalian cells. Pig fibroblasts were made positive by transfection with the class II transactivator (CIITA). As a second expression system, transgenes encoding the alpha and beta chains of class II SLA were transfected into Human embryonic kidney (HEK293) cells. Results: Human sera containing IgG specific for class II HLA molecules exhibited greater binding to class II SLA positive cells than to SLA negative cells. Sera lacking antibodies against class II HLA showed no change in binding regardless of the presence of class II SLA. These antibodies could recognize either SLA-DR or SLA-DQ complexes. Conclusions: Class II SLA proteins may behave as xenoantigens for people with humoral immunity towards class II HLA molecules. Copyright (C) 2017 Wolters Kluwer Health, Inc. All rights reserved.
http://ift.tt/2wNBPh6
http://ift.tt/2wNBPh6
Granulomatous skin reactions after tumor vaccine in two patients
Abstract
Two patients with HNPCC-syndrome (Hereditary Non-Polyposis Colorectal Cancer), an autosomal dominant genetic disorder in which patients are at higher risk of developing colorectal cancer, presented in our out-patient clinic with asymptomatic skin alterations at the injection sites of an experimental tumor-specific vaccine. The vaccine was given as part of a study in which colorectal carcinoma patients with lymph node and organ metastases and high microsatellite instability (MSI-H) received a vaccine containing fragments of specific tumor proteins. Both patients, a 60-year-old and 54-year-old male, had noted multiple slow-growing, firm, indolent erythematous to violaceous nodes of up to 4 cm in diameter on both upper arms at former injection sites approximately one year after receiving the tumor-specific vaccination (figure 1).
This article is protected by copyright. All rights reserved.
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The usefulness of investigating the possible underlying conditions in rosacea
Abstract
The paper entitled "Rosacea and demodicidosis with gain of function mutation in STAT1″ by Second et al.1 is of indubitable interest and prompted us to make some observations. The Authors described a patient with cutaneous and ocular rosacea that they related to demodicidosis since oral ivermectin improved the cutaneous lesions1. However, the Authors did not demonstrate by skin scraping nor by standardized skin surface biopsy (SSSB) an excessive number of Demodex folliculorum (DF) mites in the pilosebaceous units to justify an oral antiparasitic treatment as a drug of first choice1.
This article is protected by copyright. All rights reserved.
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Trends in restorative composites research: what is in the future?
Abstract Clinical trials have identified secondary caries and bulk fracture as the main causes for composite restoration failure. As a measure to avoid frequent reinterventions for restoration replacement, composites with some sort of defense mechanism against biofilm formation and demineralization, as well as materials with lower susceptibility to crack propagation are necessary. Also, the restorative procedure with composites are very time-consuming and technically demanding, particularly concerning the application of the adhesive system. Therefore, together with bulk-fill composites, self-adhesive restorative composites could reduce operator error and chairside time. This literature review describes the current stage of development of remineralizing, antibacterial and self-healing composites. Also, an overview of the research on fiber-reinforced composites and self-adhesive composites, both introduced for clinical use in recent years, is presented.
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Should my composite restorations last forever? Why are they failing?
Abstract Composites resins have become the first choice for direct anterior and posterior restorations. The great popularity is related to their esthetic appearance and reduced need of sound tissue removal as compared with former treatments. Several studies have demonstrated that composite restorations may last long in clinical service. In this review we discuss the factors playing a role on the long-term longevity. Composite restorations have demonstrated a good clinical performance with annual failure rates varying from 1% to 3% in posterior teeth and 1% to 5% in anterior teeth. Factors related to the patients such as caries risk and occlusal stress risk, in addition to socioeconomic factors, may affect the survival significantly. Characteristics of the clinical operators, particularly their decision making when it comes to observing or approaching an existing restoration, are decisive for longevity. Cavity features such as the number of restored walls, composite volume, and presence of endodontic treatment are of major importance and may dictate the service time of the restorative approach. The choice of restorative composite seems to have a minor effect on longevity provided that appropriate technical procedures are used. The main reasons for failure in posterior teeth are secondary caries and fracture (restoration or tooth/restoration), while in anterior teeth esthetic concerns are the main reasons leading to restoration failures. Composite resin restorations can be considered a reliable treatment as long as both the professional and the patient are aware of the factors involved in restoration failures.
http://ift.tt/2wMupuq
Bonding efficiency and durability: current possibilities
Abstract Bonding plays a major role in dentistry nowadays. Dental adhesives are used in association with composites to solve many restorative issues. However, the wide variety of bonding agents currently available makes it difficult for clinicians to choose the best alternative in terms of material and technique, especially when different clinical situations are considered. Moreover, although bonding agents allow for a more conservative restorative approach, achieving a durable adhesive interface remains a matter of concern, and this mainly due to degradation of the bonding complex in the challenging oral environment. This review aims to present strategies that are being used or those still in development which may help to prevent degradation. It is fundamental that professionals are aware of these strategies to counteract degradation as much as possible. None of them are efficient to completely solve this problem, but they certainly represent reasonable alternatives to increase the lifetime of adhesive restorations.
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Dental ceramics: a review of new materials and processing methods
Abstract The evolution of computerized systems for the production of dental restorations associated to the development of novel microstructures for ceramic materials has caused an important change in the clinical workflow for dentists and technicians, as well as in the treatment options offered to patients. New microstructures have also been developed by the industry in order to offer ceramic and composite materials with optimized properties, i.e., good mechanical properties, appropriate wear behavior and acceptable aesthetic characteristics. The objective of this literature review is to discuss the main advantages and disadvantages of the new ceramic systems and processing methods. The manuscript is divided in five parts: I) monolithic zirconia restorations; II) multilayered dental prostheses; III) new glass-ceramics; IV) polymer infiltrated ceramics; and V) novel processing technologies. Dental ceramics and processing technologies have evolved significantly in the past ten years, with most of the evolution being related to new microstructures and CAD-CAM methods. In addition, a trend towards the use of monolithic restorations has changed the way clinicians produce all-ceramic dental prostheses, since the more aesthetic multilayered restorations unfortunately are more prone to chipping or delamination. Composite materials processed via CAD-CAM have become an interesting option, as they have intermediate properties between ceramics and polymers and are more easily milled and polished.
http://ift.tt/2wMmldt
Efficiency of polymerization of bulk-fill composite resins: a systematic review
Abstract This systematic review assessed the literature to evaluate the efficiency of polymerization of bulk-fill composite resins at 4 mm restoration depth. PubMed, Cochrane, Scopus and Web of Science databases were searched with no restrictions on year, publication status, or article's language. Selection criteria included studies that evaluated bulk-fill composite resin when inserted in a minimum thickness of 4 mm, followed by curing according to the manufacturers' instructions; presented sound statistical data; and comparison with a control group and/or a reference measurement of quality of polymerization. The evidence level was evaluated by qualitative scoring system and classified as high-, moderate- and low- evidence level. A total of 534 articles were retrieved in the initial search. After the review process, only 10 full-text articles met the inclusion criteria. Most articles included (80%) were classified as high evidence level. Among several techniques, microhardness was the most frequently method performed by the studies included in this systematic review. Irrespective to the "in vitro" method performed, bulk fill RBCs were partially likely to fulfill the important requirement regarding properly curing in 4 mm of cavity depth measured by depth of cure and / or degree of conversion. In general, low viscosities BFCs performed better regarding polymerization efficiency compared to the high viscosities BFCs.
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Randomized clinical trials of dental bleaching – Compliance with the CONSORT Statement: a systematic review
Abstract We reviewed the literature to evaluate: a) The compliance of randomized clinical trials (RCTs) on bleaching with the CONSORT; and b) the risk of bias of these studies using the Cochrane Collaboration risk of bias tool (CCRT). We searched the Cochrane Library, PubMed and other electronic databases, to find RCTs focused on bleaching (or whitening). The articles were evaluated in compliance with CONSORT in a scale: 0 = no description, 1 = poor description and 2 = adequate description. Descriptive analyses of the number of studies by journal, follow-up period, country and quality assessments were performed with CCRT for assessing risk of bias in RCTs. 185 RCTs were included for assessment. More than 30% of the studies received score 0 or 1. Protocol, flow chart, allocation concealment and sample size were more critical items, as 80% of the studies scored 0. The overall CONSORT score for the included studies was 16.7 ± 5.4 points, which represents 52.2% of the maximum CONSORT score. A significant difference among journal, country and period of time was observed (p < 0.02). Only 7.6% of the studies were judged at "low" risk; 62.1% were classified as "unclear"; and 30.3% as "high" risk of bias. The adherence of RCTs evaluating bleaching materials and techniques to the CONSORT is still low with unclear/high risk of bias.
http://ift.tt/2wMsqXg
Light curing in dentistry and clinical implications: a literature review
Abstract Contemporary dentistry literally cannot be performed without use of resin-based restorative materials. With the success of bonding resin materials to tooth structures, an even wider scope of clinical applications has arisen for these lines of products. Understanding of the basic events occurring in any dental polymerization mechanism, regardless of the mode of activating the process, will allow clinicians to both better appreciate the tremendous improvements that have been made over the years, and will also provide valuable information on differences among strategies manufacturers use to optimize product performance, as well as factors under the control of the clinician, whereby they can influence the long-term outcome of their restorative procedures.
http://ift.tt/2wM73Fn
Polymerization shrinkage stress of composite resins and resin cements – What do we need to know?
Abstract Polymerization shrinkage stress of resin-based materials have been related to several unwanted clinical consequences, such as enamel crack propagation, cusp deflection, marginal and internal gaps, and decreased bond strength. Despite the absence of strong evidence relating polymerization shrinkage to secondary caries or fracture of posterior teeth, shrinkage stress has been associated with post-operative sensitivity and marginal stain. The latter is often erroneously used as a criterion for replacement of composite restorations. Therefore, an indirect correlation can emerge between shrinkage stress and the longevity of composite restorations or resin-bonded ceramic restorations. The relationship between shrinkage and stress can be best studied in laboratory experiments and a combination of various methodologies. The objective of this review article is to discuss the concept and consequences of polymerization shrinkage and shrinkage stress of composite resins and resin cements. Literature relating to polymerization shrinkage and shrinkage stress generation, research methodologies, and contributing factors are selected and reviewed. Clinical techniques that could reduce shrinkage stress and new developments on low-shrink dental materials are also discussed.
http://ift.tt/2xGD12a
Cutaneous sporotrichosis treated with methylene blue-daylight photodynamic therapy
Abstract
Sporotrichosis is an infection caused by the dimorphic fungus,Sporothrix schenckii. Acquisition typically occurs via cutaneous inoculation with development of a localized cutaneous and/or lymphocutaneous infection. aPDT (antimicrobial photodynamic therapy) is a process that generates reactive oxygen species (ROS) in presence of a photosensitizer, visible light and oxygen which destroys fungal and bacterial cells(1). Daylight PDT (DL-PDT) uses the visible spectrum of daylight (400-750nm) for the activation of the photosensitizer instead of an artificial light source. It is currently approved to treat actinic keratosis(2). The band of absorption of MB is between 550-700nm, with an absorption peak of 654nm.
This article is protected by copyright. All rights reserved.
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Immune deficiency and rosacea
Abstract
They wonder whether the patients that we reported definitely had demodicidosis associated to rosacea, and above all provide, they their own interesting findings in 60 patients with rosacea, findings that we will not comment here.We agree that skin scraping with a D. folliculorum count is certainly the less unreliable way to support a diagnosis of demodicidosis. We did not perform this test, since in our experience, strong itching in papulo-pustular rosacea in individuals with immune deficiency is always associated with D. folliculorum proliferation.
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Chronic Urticaria and the Metabolic Syndrome: a Cross-sectional Community-based Study of 11,261 Patients
Abstract
Background
Emerging evidence suggests that chronic urticaria (CU) is associated with chronic, low grade, inflammatory process.
Objective
To evaluate the association between CU and metabolic syndrome and its components in a large community-based medical database.
Methods
A cross-sectional study of CU patients and matched controls was performed. CU was defined as eight urticaria diagnoses (with each two diagnoses registered within a period of six weeks) from 2002 to 2012. Data regarding the prevalence of metabolic syndrome, its components, and possible complications were collected.
Results
The study included 11,261 patients with CU and 67,216 controls. In a univariate analysis, CU was significantly associated with higher body mass index (BMI) and a higher prevalence of obesity, diabetes, hyperlipidemia, hypertension, metabolic syndrome, chronic renal failure, and gout. Multivariate analysis demonstrated a significant association between CU and metabolic syndrome (OR = 1.12, 95% CI 1.1-1.2, p < 0.001) and its components- obesity (OR = 1.2, 95% CI 1.1-1.3, p < 0.001), diabetes (OR = 1.08, 95% CI 1.01-1.15, p = 0.001), hyperlipidemia (OR = 1.2, 95% CI 1.1-1.2, p < 0.001), and hypertension (OR = 1.1, 95% CI 1.1-1.2, p < 0.001).
Conclusions
CU patients may have one or more undiagnosed components of metabolic syndrome despite their young age. Thus, appropriate targeted screening is advised.
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Long-Term Efficacy and Safety of Sonidegib in Patients With Locally Advanced and Metastatic Basal Cell Carcinoma: 30-Month Analysis of the Randomized Phase 2 BOLT Study
Abstract
Background
Patients with locally advanced basal cell carcinoma (laBCC) or metastatic BCC (mBCC), 2 difficult-to-treat populations, have had limited treatment options. Sonidegib, a hedgehog pathway inhibitor (HPI), was approved in laBCC based on results from the BOLT trial.
Objective
To evaluate long-term efficacy and safety of sonidegib in laBCC and mBCC in the BOLT 18- and 30-month analyses.
Methods
BOLT (NCT01327053, ClinicalTrials. gov), a double-blind phase 2 study, enrolled patients from July 2011 until January 2013. Eligible HPI-treatment naïve patients with laBCC not amenable to curative surgery/radiotherapy or mBCC were randomized 1:2 to sonidegib 200 mg (laBCC, n = 66; mBCC, n = 13) or 800 mg (laBCC, n = 128; mBCC, n = 23). Tumor response was assessed per central and investigator review.
Results
With 30 months of follow-up, among patients treated with sonidegib 200 mg (approved dose), objective response rates were 56.1% (central) and 71.2% (investigator) in laBCC and 7.7% (central) and 23.1% (investigator) in mBCC. Tumor responses were durable: median duration of response was 26.1 months (central) and 15.7 months (investigator) in laBCC and 24.0 months (central) and 18.1 months (investigator) in mBCC. Five patients with laBCC and 3 with mBCC in the 200-mg arm died. Median overall survival was not reached in either population; 2-year overall survival rates were 93.2% (laBCC) and 69.3% (mBCC). In laBCC, efficacy was similar regardless of aggressive or nonaggressive histology. Sonidegib 200 mg continued to have a better safety profile than 800 mg, with lower rates of grade 3/4 adverse events (43.0% vs 64.0%) and adverse events leading to discontinuation (30.4% vs 40.0%).
Conclusion
Sonidegib continued to demonstrate long-term efficacy and safety in these populations. These data support the use of sonidegib 200 mg per local treatment guidelines.
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Different Expression Patterns of Plasma Th1-, Th2-, Th17-, and Th22-Related Cytokines Correlate with Serum Autoreactivity and Allergen Sensitivity in Chronic Spontaneous Urticaria
Abstract
Background
Clinical features and basophil activation levels correlate with serum autoreactivity and allergen sensitivity in patients with chronic spontaneous urticaria (CSU).
Objectives
To explore the relationship of the expression patterns of plasma T-helper cell (Th) 1, Th2, Th17, and Th22-related cytokines with the serum autoreactivity and the allergen sensitivity in CSU.
Method
Twenty related cytokines were measured and analysed in 60 CSU patients, 15 acute urticaria patients, 10 patients with atopic dermatitis (AD), and 15 healthy persons, respectively. Autologous serum skin testing (ASST) and skin prick testing (SPT) were performed to detect autoreactivity and allergy sensitivity, respectively. The protein-protein interaction of cytokines and the molecular pathways were analysed by Search Tool for the Retrieval of Interacting Genes/Proteins (STRING) and KyotoEncyclopedia of Genes and Genomes (KEGG database), respectively.
Results
Th1/Th2 and Th17 related cytokines were significantly elevated and correlated with disease activity in CSU than in healthy controls. Interleukin (IL)-6, IL-10, and IL-13 were significantly higher in acute urticaria than in CSU patients. Granulocyte/macrophage colony-stimulating factor (GM-CSF), IL-10 and IL-17 were significantly higher in ASST + than in ASST- CSU patients. IFN-γ, IL-2, IL-12p70, and IL-21 were significantly higher in SPT + than in SPT- CSU patients. The plasma levels of interferon-γ, IL-2, and IL-21 varied among ASST+/SPT+, ASST+/SPT-, ASST-/SPT+, and ASST-/SPT- CSU subgroups, which appeared to involve the positive regulation of the Janus kinase-signal transducer and activator of transcription (Jak-STAT) signaling pathway.
Conclusion
This study indicates acute urticaria elicits a more prominent Th2 immune response than CSU. There was association between different expression patterns of plasma Th1-, Th2-, Th17-, and Th22-related cytokines and serum autoreactivity or allergen sensitivity in CSU. Further studies on the JAK-STAT pathway in the pathogenesis of CSU is warranted.
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Dermoscopy and the diagnosis of primary cutaneous B-cell lymphoma
Abstract
Background
Primary cutaneous B-cell lymphomas (PCBCL) are frequently misdiagnosed and a biopsy is needed to attain the correct diagnosis.
Objective
To characterize the dermoscopic features of PCBCL.
Methods
In this retrospective observational study we analyzed the pathology reports of 172 newly diagnosed PCBCL for the initial clinical differential diagnosis. The dermoscopic images of 58 PCBCL were evaluated for dermoscopic features. Two dermoscopy experts, who were blinded to the diagnosis and the study objective, evaluated images from 17 cases for a dermoscopic differential diagnosis.
Results
Of 172 biopsy-proven PCBCL lesions, cutaneous lymphoma was suspected by the clinician in 16.3%; the leading diagnosis was basal cell carcinoma in 17.4%, and other skin neoplasms in 21%. Studying 58 PCBCL dermoscopic images, we most frequently identified salmon-colored background/area (79.3%) and prominent blood vessels (77.6%), mostly of serpentine (linear-irregular) morphology (67.2%). Dermoscopic features did not differ significantly by subtype or location. Blinded evaluation by dermoscopy experts raised a wide differential diagnosis including PCBCL, arthropod bite, basal cell carcinoma, amelanotic melanoma and scar/keloid.
Conclusions
Two dermoscopic features, salmon-colored area/background and serpentine vessels, are frequently seen in PCBCL lesions. These characteristic dermoscopic features, although not specific, can suggest a possible diagnosis of PCBCL.
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http://ift.tt/2vwPaq1
Dermoscope-guided lesional biopsy to diagnose EMA+ CK7+ CK20+ extramammary Paget's disease with an extensive lesion
Abstract
Dermoscopy has been reported to facilitate the diagnosis of extramammary Paget's disease (EMPD).1-3 We report here the first application of dermoscope-guided lesional biopsy to confirm the diagnosis of EMPD. A 60-year-old male presented with a pruritic and painful rash over his left groin region for six months.
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Obstetric Outcomes in Women with Nonalcoholic Fatty Liver Disease
Metabolic Syndrome and Related Disorders , Vol. 0, No. 0.
http://ift.tt/2wc4b3c
Correction: Cerebral venous sinus thrombosis during superselective intra-arterial infusion of cisplatin and concomitant radiotherapy for maxillary squamous cell carcinoma
Okamura S, Saito Y, Mori H, Yamasoba T. Cerebral venous sinus thrombosis during superselective intra-arterial infusion of cisplatin and concomitant radiotherapy for maxillary squamous cell carcinoma. BMJ Case Rep. Published Online First: 12 May 2017. doi:10.1136/bcr-2017-220591
The line '(1) the indwelling catheter in;' should read:
(1) the indwelling catheter in the common carotid artery decreased brain venous flow;
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Correction: Vitamin A deficiency due to chronic malabsorption: an ophthalmic manifestation of a systemic condition
Cheshire J, Kolli S. Vitamin A deficiency due to chronic malabsorption: an ophthalmic manifestation of a systemic condition. BMJ Case Rep. Published Online First: 10 May 2017. doi:10.1136/bcr-2017-220024
In this article, the author affiliations are incorrect; the correct affiliations are listed below.
James Cheshire: Heart of England NHS Foundation Trust, Birmingham, UK
Sai Kolli: Department of Ophthalmology, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK
http://ift.tt/2gjAlnD
A Rare, Unforeseen Complication Of Facial Skin Resurfacing
Erosive pustular dermatitis (EPD), a rare condition that typically affects actinically damaged skin on the scalp, may also emerge on the face after cosmetic resurfacing, researchers have found.
Reuters Health Information
http://ift.tt/2gd80zm
Age-related histologic and biochemical changes in auricular and septal cartilage
Objectives/Hypothesis
To characterize the histologic and biochemical properties of auricular and septal cartilage and analyze age-related changes in middle-aged to older adults.
Study Design
Cross-sectional study of auricular and septal cartilage from 33 fresh cadavers.
Methods
Auricular and septal cartilage specimens were stained using Safranin O for glycosaminoglycans, Verhoeff's stain for elastin, and Masson's trichrome for collagen. Percentage of tissue stained, cell density and size were quantified. Relationships between donor characteristics and histologic properties were evaluated using mixed model analyses.
Results
The average donor age was 75 years (standard deviation = 11 years; range, 55–93 years). In auricular cartilage, each 1-year increase in age was associated with a 0.97% decrease in glycosaminoglycans (P < .001) and a 0.98% decrease in elastin (P < .001). In septal cartilage, glycosaminoglycans decreased 2.4% per year (P < .001). Age did not affect collagen content significantly in auricular (P = .417) or septal cartilage (P = .284). Cell density and cell size declined with age in auricular (both P < .001) and septal cartilage (P = .044, P = .032, respectively). Compared to septal cartilage in patients of all ages, auricular cartilage had more glycosaminoglycans, less collagen, higher cell density, and smaller cells.
Conclusions
In auricular and septal cartilage, glycosaminoglycans, elastin, cell density, and cell size decrease significantly with age in patients over 55 years of age. Glycosaminoglycan content declines faster with age in septal cartilage than auricular cartilage. These age-related changes may affect biomechanical properties and tissue viability, and thereby have implications for graft choice in functional, aesthetic, and reconstructive nasal surgery.
Level of Evidence
NA Laryngoscope, 2017
http://ift.tt/2iBX1Ai
Combined surgical and endovascular approach to treat a carotid cavernous fistula with associated brainstem venous congestion
Endovascular embolization is the standard approach for management of carotid cavernous fistulas (CCFs) due to the ease of access and reduced level of risk associated with the procedure compared with open surgery. We present here a case of a CCF that eventually led to the development of brainstem venous congestion from perimedullary venous drainage. This fistula was not amenable to endovascular embolization due to lack of either ophthalmic vein or petrosal sinus drainage. Therefore, a craniotomy with direct puncture of the cavernous sinus was performed, followed by coil embolization to completely treat this fistula. This case demonstrates an uncommon progression of venous drainage to Cognard grade V, rare development of symptomatic brainstem venous congestion and a unique method to combine an open surgical approach with endovascular embolization to treat CCFs.
http://ift.tt/2xr3D8j
Nrf2-peroxiredoxin I axis in polymorphous adenocarcinoma is associated with low matrix metalloproteinase 2 level
Abstract
Polymorphous adenocarcinoma (PAC) is a malignant epithelial neoplasm that affects almost exclusively the minor salivary glands, generally described as having a relatively good prognosis. Aberrant nuclear factor erythroid 2 (NF-E2)-related factor (Nrf2) activation in tumor cells has been associated with induction of antioxidant enzymes, such as peroxiredoxin I (Prx I) and increased matrix metalloproteinase (MMP) expression. In this context, the aim of the present study was to evaluate the expression of Nrf2 and correlate it with Prx I and MMP-2 secretion in PAC. Thirty-one cases of PAC from oral biopsies were selected and immunohistochemically analyzed for Nrf2 and Prx I. MMP-2 quantification was performed on primary cell cultures derived from PAC. Oral squamous cell carcinoma (OSCC) cell cultures were used as control. A high immunoexpression of Nrf2 was observed in both the cytoplasm and the nucleus of neoplastic cells from PAC. Nuclear staining for Nrf2 suggested its activation in the majority of the PAC cells, which was confirmed by the high expression of its target gene, Prx I. Quantification of MMP-2 secretion showed lower levels in PAC cell cultures when compared to OSCC cell cultures (p < 0.05). In conclusion, although Nrf2 overexpression has been frequently associated with high-grade malignancies, such relationship is not infallible and, in fact, the opposite may occur in low-grade tumors, such as PAC of minor salivary glands.
http://ift.tt/2xr8OF2
A Study to Investigate the Efficacy and Safety of Cobimetinib Plus Atezolizumab in Participants With Solid Tumors
Condition: Solid Tumors
Interventions: Drug: Cobimetinib; Drug: Atezolizumab
Sponsor: Hoffmann-La Roche
Not yet recruiting - verified August 2017
http://ift.tt/2xqTHeM
Interventions: Drug: Cobimetinib; Drug: Atezolizumab
Sponsor: Hoffmann-La Roche
Not yet recruiting - verified August 2017
http://ift.tt/2xqTHeM
Kinesio-Taping in Stroke Patients With Visuospatial Neglect
Conditions: Stroke; Motor Disorder
Interventions: Other: Kinesio taping group; Other: Sham Taping
Sponsor: Universita di Verona
Not yet recruiting - verified August 2017
http://ift.tt/2wb2WB5
Interventions: Other: Kinesio taping group; Other: Sham Taping
Sponsor: Universita di Verona
Not yet recruiting - verified August 2017
http://ift.tt/2wb2WB5
AMP-activated protein kinase and cAMP response element-binding protein activity are associated with distinct postoperative behavioral changes in rats
Abstract
Purpose
To explore the association of AMP-activated protein kinase (AMPK) and cAMP response element-binding protein (CREB) activity with distinct postoperative behavioral changes in rats.
Methods
We performed partial hepatolobectomy in adult rats and aged rats. The learning and memory ability of rats was evaluated by the Barnes maze test on postoperative days 1–4. The expressions of AMPK and phosphorylated CREB (p-CREB) in the CA1 and DG regions of the hippocampus were detected by immunohistochemical assay at 3, 6, 12 and 24 h postoperatively.
Results
Surgery impaired the memory of aged rats but not that of adult rats on postoperative days 2 and 3 (both p < 0.05). Surgery synergistically increased the expressions of AMPK and p-CREB in the CA1 region of hippocampus in aged rats but not in adult rats at 3 and 6 h postoperatively.
Conclusion
Our data suggest that surgery induces cognitive impairment in aged rats. Surgery-induced expression of AMPK and p-CREB in the CA1 region of hippocampus may be involved in this cognitive impairment.
http://ift.tt/2wVGVXJ
Invading beyond bounds: extraintestinal Clostridium difficile infection leading to pancreatic and liver abscesses
Clostridium difficile has become a common healthcare-associated infection over the past few years and gained more attention. C. difficile was estimated to cause almost half a million infections in USA in 2011 and 29 000 died within 30 days of the initial diagnosis. Although colitis due to C. difficile is the most common presentation, there have been reported cases of extraintestinal infections. As per our review of literature, this is the third reported case of liver abscess due to the organism.
http://ift.tt/2wVAOmm
Elephantiasis nostras verrucosa: a rare complication of lower limb lymphoedema
We reported a case of a 49-year-old man who had chronic lymphoedema of bilateral lower limbs for 30 years, but he did not seek treatment. His disease was complicated with irreversible changes of elephantiasis nostras verrucosa and had recurrent admissions due to infection. He was not keen for surgical intervention. This case report illustrated a rare and non-reversible complication of a common skin disease, lymphoedema and also the importance of identifying and treating it early.
http://ift.tt/2vwmPjg
Dilemma of diagnosing sulphonylurea overdose in children: deliberations and considerations before reaching a diagnosis
A 15-year-old non-diabetic Caucasian girl presented with sudden onset of seizures, unrecordable blood glucose readings and acute renal failure. She denied any medication ingestion and no other precipitating factors were encountered for this acute presentation. She was treated with intravenous glucose infusion and hydrocortisone injection. Investigations showed a non-ketotic hypoglycaemia with high C-peptide and insulin levels. It took several days and multiple investigations to establish the exact cause of her persistent hypoglycaemia before it was concluded to be secondary to gliclazide overdose in a suicide attempt by the young girl. She made a complete recovery in a week with no apparent lasting neurological or renal impairment.
http://ift.tt/2wVvVtD
Hypertensive bipolar: chronic lithium toxicity in patients taking ACE inhibitor
A patient with bipolar I disorder has been treated with lithium and haloperidol for the last 20 years and received an ACE inhibitor for his hypertension since 9 years ago. Despite regular clinic follow-ups and blood monitoring, he recently developed tremors and delirium. On hospital admission, serum level of lithium was far above toxic level. Mental state examination revealed an anxious and disorientated man with irrelevant speech. Immediate discontinuation of lithium resulted in slow reduction of serum lithium levels and gradual resolution of tremor but his delirium persisted for 2 weeks. His condition took a turn for the worse when he developed acute renal failure and arm abscess. We discussed about lithium toxicity and the vulnerability factors which have induced delirium and renal failure in this patient.
http://ift.tt/2vvKlgF
Novel oral anticoagulant and kidney injury: apixaban-related acute interstitial nephritis
Non-vitamin K oral anticoagulants (NOACs) are being increasingly prescribed. These drugs act rapidly, have predictable dose-related anticoagulation effect and require no routine laboratory monitoring, making them attractive for both patients and healthcare providers. All NOACs are at least partially excreted thought the kidneys. Renal injury related to NOAC use is being increasingly reported. NOAC-related acute interstitial nephritis (AIN) has only been reported once and that was in context of dabigatran use. We describe the first case of apixaban-related AIN. This case adds an important differential diagnoses that should be considered for any patient presenting with renal injury while being treated with NOACs.
http://ift.tt/2wVsumx
Abdominal tuberculosis and spontaneous miscarriage
We present a case report of a 23-year-old Pakistani woman who had a second trimester spontaneous miscarriage while visiting her family in the Kingdom of Saudi Arabia. A dilatation and curettage (D&C) was done after the miscarriage. She developed sepsis and acute respiratory distress syndrome, requiring intensive care unit admission a few days after the D&C. An exploratory laparotomy was done and she was found to have a pelvic abscess. Despite adequate broad spectrum antimicrobial cover, she continued to drain a copious amount of serous fluid from the peritoneal cavity, with persistent fever and a stiff lung with difficult weaning off mechanical ventilation. Tuberculosis PCR of the peritoneal fluid came back positive. A histological finding of necrotising granuloma from the postoperative omental specimen and a positive culture from the ascitic fluid confirmed the diagnosis of Mycobacterium tuberculosis. Antituberculous treatment was started and she made a speedy recovery.
http://ift.tt/2wVFrNa
Multiple sclerotic fibromas of the skin: an important clue for the diagnosis of Cowden syndrome
Cowden syndrome is a rare autosomal dominant condition characterised by mucocutaneous hamartomas and, most importantly, predisposition to various extracutaneous benign and malignant tumours. This disorder is associated with a germline mutation in the phosphatase and tensin homologue gene, a tumour suppressor gene, located on 10q23 chromosome. The expressivity of this genodermatosis is highly variable, therefore many of the cases remain undiagnosed. Skin and mucous findings are very common in Cowden syndrome and may represent the initial clinical manifestation leading to the diagnosis. The authors describe a case of a 58-year-old man with multiple cutaneous sclerotic fibromas associated with a previously unrecognised Cowden syndrome.
http://ift.tt/2vwAxmc
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