Stiff skin syndrome (SSS) is a rare, autosomal dominant cutaneous disorder with progressive, symmetric, sclerotic skin changes of the shoulders, hips, and thighs. In a recent publication, a distinct segmental variant of SSS was proposed. In this report we discuss the case of a boy with segmental SSS and review the current literature.
Fungal recognition by Dectin-1 receptor triggers a series of cellular mechanisms involved in a protective activation of the immune system. In this study we aimed to evaluate the participation of Dectin-1 receptor in the induction of IL-8, TNF-α, IL-12, IL-10 and IL-17A secretion by human monocytes activated with different cytokines, and challenged in vitro with Paracoccidioides brasiliensis (P. brasiliensis). Our results show that monocytes challenged with P. brasiliensis (Pb265) are able to produce IL-12, IL-8, IL-17, IL-10, and TNF-α. Dectin-1 receptor blockage decreased the IL-12, IL-17, IL-10, and TNF-α levels indicating the participation of such receptor in the induction of these cytokines. Only IL-8 production was not affected by the blockage. Cells activation with different cytokines showed that GM-CSF was able to induce secretion of all cytokines and the receptor blockage prior to the challenge also decreased the cytokine secretion, except IL-8. Monocytes activated with TNF-α promoted IL-8, IL-10, and TNF-α production, while stimulation with IFN-γ promoted mainly IL-12 and TNF-α. Thus, these findings bring new and important knowledge about Dectin-1 participation in cytokines production by monocytes challenged with Pb265.
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Fungal sensitization in adults is associated with severe asthma but prevalence and clinical significance of fungal sensitization remains unclear in paediatric population.
To study association of fungal sensitization with disease severity in children with persistent asthma
One hundred children with persistent asthma in age group 7-15 years, symptom duration >2 years and forced expiratory volume in first second >50% of expected were enrolled. Skin prick test (SPT) to 8 fungal antigens and total serum IgE were done. Fungal sensitization was described as positive SPT (wheel diameter more than 3mm larger than the negative control) to any of the fungal antigens and total serum IgE >200ng/ml.
Seventeen patients showed evidence of fungal sensitization, of which, six demonstrated sensitization to multiple fungi. 17.6% patients with fungal sensitization had severe asthma as compared to 2.4% patients without fungal sensitization (p value 0.032). Significant increase in family history of allergic comorbidities was noted among patients with fungal sensitization (47.1% vs 21.7%, p value 0.03). The most common implicated organism in fungal sensitized patients was Aspergillus flavus (47.1%).
The results of this study, a first among Indian children with asthma, suggest that children with fungal sensitization have more severe asthma as compared to children without fungal sensitization.
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Posaconazole is an extended-spectrum triazole antifungal used in the treatment and prophylaxis of Aspergillus infections. It is available as oral suspension (POS-Liq) and delayed-release tablets (POS-Tab).
The aim of this longitudinal, retrospective study was to compare the clinical effectiveness, toxicity, and pharmacokinetics of POS-Liq vs. POS-Tab in lung transplant recipients (LTx-recipients), who were treated with both formulations subsequently.
Twenty-four consecutive LTx-recipients with 191 documented posaconazole trough levels (PTLs) for POS-Liq or POS-Tab were included. The administered daily doses were 300mg for POS-Tab and 600mg (prophylaxis) or 800mg (therapy) for POS-Liq. Target PTLs were ≥700ng/ml (prophylaxis) and ≥1250ng/ml (therapy).
The overall prophylactic and therapeutic response rates were 78% and 67%, respectively. No cases of hepatotoxicity or QT-prolongation were observed with either formulation. The achieved target PTLs were doubled under POS-Tab compared to POS-Liq with fewer risk factors for sub-therapeutic PTLs. Concomitant administration of POS-Tab significantly reduced the tacrolimus concentration-to-dose ratio (p=0.001).
We suggest the use of POS-Tab is appropriate for prophylaxis and therapy of Aspergillus infections in LTx-recipients, since POS-Tab displayed more reliable PTLs with no added adverse events. However, we recommend regular drug monitoring for POS-Liq and for therapy with POS-Tab and that immunosuppressant levels are monitored closely when the posaconazole formulation is switched.
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Recurrent vulvovaginal candidiasis (RVVC) is acommon opportunistic, mucosal fungal infection, predominantly caused by the fungus Candida albicans.Mannose-binding lectin (MBL) is an acute-phase protein that plays a key role in the innate immunity defense against infectious disease.
The present study was conducted to evaluate the relationship between the MBL serum level and the relative expression of MBL mRNA in RVVC using real-time PCR for the first time.
The case-control study included 40 female participants suffering from RVVC and 40 healthy individuals. The MBL serum level was measured using a commercial ELISA kit. The relative mRNA expression of the MBL gene was quantified using real-time PCR. Data analysis was done by SPSS software.
The MBL concentration was significantly higher in the participants suffering from RVVC compared to the control group (0.330 ng/ml versus 0.253 ng/ml). The prognostic value (P<0.001) for RVVC diagnosis has been calculated. Quantitative RT-PCR results from 35 samples showed a low to significant values for mRNA levels corresponding to MBL gene expression (1-352 folds) (P<0.001).
The results of this study suggest that MBL plays a main role in the innate immunity and it is also affected by environmental factors and other genetic variations. Therefore, the MBL gene expression profile does not reflect precise phenotypic levels in the serum.
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Publication date: Available online 6 November 2017
Source:Actas Dermo-Sifiliográficas (English Edition)
Author(s): A. de Quintana-Sancho, M.T. Conde Calvo
Publication date: Available online 6 November 2017
Source:Actas Dermo-Sifiliográficas (English Edition)
Author(s): Pedro Vilas Boas, Antonio Ruedas-Martínez, Ofelia Baniandrés-Rodriguez, Cristina Ciudad-Blanco
Publication date: Available online 6 November 2017
Source:Actas Dermo-Sifiliográficas (English Edition)
Author(s): P. García-Montero, J.M. Segura Palacios, M.de Troya Martín
Publication date: Available online 6 November 2017
Source:Actas Dermo-Sifiliográficas (English Edition)
Author(s): L. Campos Muñoz, A. Fueyo Casado, E. López Bran
Publication date: Available online 6 November 2017
Source:Actas Dermo-Sifiliográficas (English Edition)
Author(s): J.F. Millán-Cayetano, J. del Boz, P. García-Montero, M. de Troya-Martín
Publication date: Available online 6 November 2017
Source:Actas Dermo-Sifiliográficas (English Edition)
Author(s): J.F. Millán-Cayetano, I.C. Abitei, P. García-Montero, L. Padilla-España
Publication date: Available online 6 November 2017
Source:Actas Dermo-Sifiliográficas (English Edition)
Author(s): S. Córdoba, E. Rojas, A. Garrido-Ríos, J. Borbujo
Publication date: Available online 6 November 2017
Source:Actas Dermo-Sifiliográficas (English Edition)
Author(s): A. Imbernón-Moya, E. Fernández-Cogolludo, M.Á. Gallego-Valdés
Publication date: Available online 6 November 2017
Source:Actas Dermo-Sifiliográficas (English Edition)
Author(s): O. Corral-Magaña, J. Escalas Taberner, A. Bauzá Alonso, A. Martin-Santiago
The DIEP flap has reduced the incidence of abdominal wall complications compared to the TRAM flap [1]. However, this risk still remains [1-2] because the anterior sheath of the rectus muscle needs to be incised longitudinally to dissect the deep inferior epigastric vessels. We believe that robotic dissection of the deep inferior epigastric vessels may reduce donor site morbidity by preserving the anterior sheath of the rectus muscle as much as possible. The aim of this study was to assess the technical feasibility of DIEP flap harvest with robotically assisted dissection of the deep inferior epigastric vessels.
Cardiac amyloidosis is a rare and still underdiagnosed disease leading a poor prognosis. We reviewed the literature regarding non-invasive imaging of cardiac amyloidosis (i.e., echocardiography, magnetic resonance, and nuclear medicine imaging) with special attention to the clinical contribution of bone-seeking radiotracers.
We performed a PubMed and Scopus literature search using keywords related to imaging of cardiac involvement in systemic amyloidosis. In particular, a review of studies published in the English literature between 2002 and 2017 dealing with clinical contribution of in vivo nuclear medicine imaging using bone-seeking radiotracers has been performed; studies that do not clearly report final diagnoses have been excluded.
Interest for cardiac amyloidosis imaging has raised in the last years; in particular, about 20 recent studies evaluating imaging with bone-seeking radiotracers have been considered for the review. Echocardiography represents the first imaging approach, although its sensitivity (in the early stage) and specificity are limited. Magnetic resonance imaging can show highly suggestive signs for cardiac amyloidosis. Imaging with bone-seeking radiotracers has high accuracy in revealing cardiac amyloidosis etiology—both sensitivity and specificity reach 100% in selected groups of patients—and performs better than other imaging modalities in revealing the early cardiac amyloidosis. PET radiotracers' contribution is still under investigation.
Imaging can aid in differentiating cardiac amyloidosis from other diseases providing prognostic information. Bone-seeking radiotracers can establish the type of amyloidosis, even without performing biopsy, in a large part of patients, and are able to early detect cardiac involvement in subjects with transthyretin gene mutations.
Publication date: Available online 6 November 2017
Source:Journal of Plastic, Reconstructive & Aesthetic Surgery
Author(s): Samuel Struk, Benjamin Sarfati, Nicolas Leymarie, Antoine Missistrano, Heba Alkhashnam, Françoise Rimareix, Frédéric Kolb, Jean-François Honart
Die Fox-Fordyce-Erkrankung (FFD), auch bekannt als apokrine Miliaria, ist eine seltene, chronische Dermatose, die durch hautfarbene bis hellbraune oder gelbliche Papeln und Juckreiz v. a. in Bereichen mit apokrinen Drüsen charakterisiert ist. Typischerweise sind postpubertale Mädchen und Frauen zwischen 13 und 35 Jahren betroffen. Die Ätiologie ist bisher nicht gänzlich geklärt, wobei eine hormonelle Komponente diskutiert wird. Des Weiteren wurden verschiedene Exazerbationsfaktoren beschrieben, unter anderem lasergestützte Epilationen sowie eine Hyperhidrose. Die Behandlung der FFD gestaltet sich als relativ schwierig, die bisher beschriebenen Therapien zeigten unterschiedliche Erfolge.
Acne inversa (AI) ist eine chronisch rezidivierende, entzündliche Dermatose mit dem Charakter einer Systemerkrankung. AI ist durch typische Hautveränderungen in Körperarealen mit apokrinen Drüsen gekennzeichnet. Die Versorgung der AI-Patienten ist in Deutschland nach wie vor unzureichend. Diese Situation lässt sich durch folgende Anstrengungen wahrscheinlich maßgeblich verbessern: 1) die Verkürzung der Zeitspanne zwischen der ersten Manifestation der Erkrankung und Diagnosestellung/Beginn der Therapie; 2) die fundierte Erforschung der AI-Pathogenese mit dem Ziel der Identifizierung von Therapietargets und Blutbiomarkern; 3) die Etablierung einer Methode zur Quantifizierung des Schweregrads der Erkrankung, die sowohl die klinische Beurteilung als auch objektive Laborparameter und die Selbsteinschätzung des Patienten (z. B. Dermatology Life Quality Index [DLQI]) berücksichtigt; 4) die Erarbeitung eines klaren Algorithmus für die interdisziplinäre Behandlung der Patienten, die neben der Therapie der Hautläsionen auch lebensstiländernde Maßnahmen einschließt und die systemischen Veränderungen bei AI berücksichtigt. Dieser Beitrag beschreibt die aktuellen Probleme der medizinischen Versorgung der AI-Patienten und zeichnet Wege auf, wie wir die beschriebenen Ziele erreichen können.
In this introduction to a special subsection of Theoretical Medicine and Bioethics comprising separate reviews of the Springer Handbook of the Philosophy of Medicine, The Routledge Companion to Philosophy of Medicine, and The Bloomsbury Companion to Contemporary Philosophy of Medicine, I compare the three texts with respect to their overall organization and their approach to the relation between the science and the art of medicine. I then indicate two areas that merit more explicit attention in developing a comprehensive philosophy of medicine going forward: health economics and systematic relations within the field as a whole. The reviews that follow speak for themselves.
Numerous studies have demonstrated the negative effects of elevated O3 on leaf photosynthesis. Within trees, a portion of respired CO2 is assimilated by woody tissue photosynthesis, but its response to elevated O3 remains unclear. Saplings of two evergreen tree species, EuCahetus dunnii Maiden (E. dunnii) and Osmanthus fragrans (Thunb.) Lour. (O. fragrans), were exposed to non-filtered air (NF), 100 nmol mol−1 O3 air (E1) and 150 nmol mol−1 O3 air (E2) in open-top chambers from May 5 to September 5, 2016 (8 h a day; 7 days a week) in subtropical China. In this study, O3 fumigation significantly reduced leaf net photosynthesis rate in both two tree species on most measurements. However, compared with leaf net photosynthesis rate, woody tissue gross photosynthesis rate showed less negative response to O3 fumigation and was even stimulated to increase. Refixation rate reflects the utilization efficiency of the respired CO2 by woody tissue photosynthesis. During the experiment period, E1 and E2 both increased refixation rate in O. fragrans compared with NF. Whereas for E. dunnii, E1 increased refixation rate until 81 days after starting of fumigation and then decreased it, and E2 decreased it all the time. Refixation rate had a significant positive correlation with woody tissue chlorophyll contents, indicating that the response of refixation rate to elevated O3 may relate to chlorophyll contents. All these suggested that under O3 fumigation, when atmospheric CO2 uptake and fixation by leaf is limited, woody tissue photosynthesis can contribute more to the total carbon assimilation in trees. The findings help to understand the significance of woody tissue photosynthesis under elevated O3 conditions.
The aim was to assess the ability of microcosms (laboratory-scale shallow ponds) as a post polishing stage for the remediation of artificial textile wastewater comprising two commercial dyes (basic red 46 (BR46) and reactive blue 198 (RB198)) as a mixture. The objectives were to evaluate the impact of Lemna minor L. (common duckweed) on the water quality outflows; the elimination of dye mixtures, organic matter, and nutrients; and the impact of synthetic textile wastewater comprising dye mixtures on the L. minor plant growth. Three mixtures were prepared providing a total dye concentration of 10 mg/l. Findings showed that the planted simulated ponds possess a significant (p < 0.05) potential for improving the outflow characteristics and eliminate dyes, ammonium-nitrogen (NH4-N), and nitrate-nitrogen (NO3-N) in all mixtures compared with the corresponding unplanted ponds. The removal of mixed dyes in planted ponds was mainly due to phyto-transformation and adsorption of BR46 with complete aromatic amine mineralisation. For ponds containing 2 mg/l of RB198 and 8 mg/l of BR46, removals were around 53%, which was significantly higher than those for other mixtures: 5 mg/l of RB198 and 5 mg/l of BR46 and 8 mg/l of RB198 and 2 mg/l of BR46 achieved only 41 and 26% removals, respectively. Dye mixtures stopped the growth of L. minor, and the presence of artificial wastewater reduced their development.
To determine the reference intervals (RefInt) of the quantitative morphometric parameters of femoroacetabular impingement (FAI) in asymptomatic hips with computed tomography (CT) and determine their dependence on age, side, limb dominance and sex.
We prospectively included 590 patients and evaluated 1111 hips with semi-automated CT analysis. We calculated overall, side- and sex-specific parameters for imaging signs of cam [omega and alpha angle (α°)] and pincer-type morphology [acetabular version (ACvers), lateral centre-edge angle (LCEA) and cranio-caudal coverage].
Hip shape was symmetrical and did not depend on limb dominance. The 95% RefInt limits were sex-different for all cam-type parameters and extended beyond current abnormal thresholds. Specifically, the upper limits of RefInt for α° at 12:00, 1:30 and 3:00 o'clock positions were 56°, 70° and 58°, respectively, and 45° for LCEA. Acetabular morphology varied between age groups, with a trend toward an LCEA/ACvers increase over time.
Our morphometric measurements can be used to estimate normal hip morphology in asymptomatic individuals. Notably they extended beyond current thresholds used for FAI imaging diagnosis, which was most pronounced for cam-type parameters. We suggest the need to reassess α° RefInt and consider a 60° threshold for the 12:00/3:00 positions and 65-70° for other antero-superior positions.
• Hip shape is symmetrical regardless of limb dominance.
• Pincer/cam morphology is frequent in asymptomatic subjects (20 and 71%, respectively).
• LCEA and acetabular version increases with age (5-7° between opposite age groups).
• Femoral morphology is stable after physeal closure (in the absence of pathology).
• Alpha and omega angle thresholds should be set according to sex.
Sceptics vis-à-vis introspection often base their scepticism on 'phenomenological disputes', 'introspective disagreement', or 'introspective disputes' (ID) (see Kriegel in Phenomenol Cogn Sci 6(1):115–136, 2007; Bayne and Spener in Philos Issues 20(1):1–22, 2010; Schwitzgebel in Perplexities of consciousness, MIT Press, Cambridge, 2011): introspectors massively diverge in their opinions about experiences, and there seems to be no method to resolve these issues. Sceptics take this to show that introspection lacks any epistemic merit. Here, I provide a list of paradigmatic examples, distill necessary and sufficient conditions for IDs, present the sceptical argument encouraged by IDs, and review the two main strategies (resolution and containment) to reject such a scepticism. However, both types of strategies are unsatisfactory. In order to save introspection from the looming sceptical threat, I advocate a deflationary strategy, based on either an 'Argument from Perceptual Kinship' or an 'Argument from Ownership'. In the end, there cannot be any genuine IDs, for nothing can fulfil the reasonable conditions for IDs. What looks like IDs may instead be indicators of phenomenal variation. Debates that look like IDs may then arise even if introspection were a perfect method to know one's mind. Thus, scepticism vis-à-vis introspection based on IDs rests on shaky grounds.
Pemphigus is a general term for a rare group of autoimmune diseases which result in the formation of blisters on the skin and oral cavity. Although there is no way to prevent autoimmune diseases, some factors may trigger pemphigus initiation in susceptible individuals or be exacerbated in affected patients. Recognition of these triggers, based on the latest studies and experiences is essential and should be updated every few years. In this study, several triggers, including different drugs and treatments, diseases, vaccines, genetic factors, nutrients, micronutrients, pregnancy, stress, and various other triggers have been discussed. Some possible triggers, such as blood antigens and the effect of seasons have also been discussed briefly. Moreover, some protective factors against pemphigus have been reviewed. Considering the molecular mechanism of pemphigus and immune response alteration during this disease, some possible triggers have been suggested and discussed. Although those triggers may be a real threat, more studies are needed to support these hypotheses.
In the process of hair treatment, various cationic actives contained in hair care products can be absorbed into hair fiber to modulate the physicochemical properties of hair such as color, strength, style and volume. There have been very limited studies on the binding and partition properties of hair care actives to hair. This study aimed to investigate the pH effects on cationic solute absorption into hair and binding to keratin.
The keratin binding and hair partition properties of three cationic solutes (theophylline, nortriptyline and amitriptyline) have been measured at different pH using fluorescence spectroscopy and equilibrium absorption experiment. The binding constants, thermodynamic parameters and hair-water partition coefficients determined at different pH were compared and analyzed.
Increasing the pH from 2.0 to 6.0 resulted in the net charge of hair keratin changed from positive to negative. As a consequence, the binding constants of the three cationic solutes with keratin increased with the increasing pH. This correlated with the variation of the electrostatic interaction between cationic solutes and keratin from repulsion to attraction. The positive ΔH and ΔS values indicated that hydrophobic interaction also played a major role in the binding of the three cationic solutes to keratin. There was a good correlation between solutes binding to keratin and hair-water partition of solutes.
It appears that solute binding to hair keratin is driven first by hydrophobic interaction and then by electrostatic interaction. The fitted thermodynamic parameters suggested that hydrophobic interaction dominates for the binding of the three cationic solutes to keratin. That binding of cationic solutes to keratin correlates with the partition of the solutes to hair could provide theoretical guidance for further developing mathematical models of hair partition and penetration properties.
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Audiometric tests provide information about hearing in otitis media with effusion (OME). Questionnaires can supplement this information by supporting clinical history-taking as well as potentially providing a standardized and comprehensive assessment of the impact of the disease on a child. There are many possible candidate questionnaires. This study aimed to assess the quality and usability of parent / child questionnaires in OME assessment.
Fifteen, published questionnaires, commonly used in audiological departments (ABEL, CHAPS, CHILD, COW, ECLiPS, ELF, FAPC, HL-7, LIFE-R Student, LIFE-UK IHP, LittlEARS, LSQ, OM-6, OMQ-14, PEACH) were assessed according to the following 8 criteria: conceptual clarity, respondent burden, reliability, validity, normative data, item bias, ceiling/ floor effects, and administrative burden.
ECLiPS, LittlEARS and PEACH scored highest overall based on the assessment criteria established for this study. None of the questionnaires fully satisfied all 8 criteria. Although all questionnaires assessed issues considered to be of at least adequate relevance to OME, the majority had weaknesses with respect to the assessment of psychometric properties, such as item bias, floor/ceiling effects or measurement reliability and validity. Publications reporting on the evaluation of reliability, validity, normative data, item bias and ceiling/floor effects were not available for most of the questionnaires.
This formal evaluation of questionnaires, currently available to clinicians, highlights three questionnaires as potentially offering a useful adjunct in the assessment of OME in clinical or research settings. These were the ECLiPS, which is suitable for children aged 6 years and older, and either the LittlEARS or the PEACH for younger children. The latter two are narrowly focused on hearing, whereas ECLiPS has a broader focus on listening, language and social difficulties.
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to compare the results of tissue preservation techniques of soft palate surgeries including expansion sphincter pharyngoplasty (ESP) and barbed reposition pharyngoplasty (BRP) for patients suffering from obstructive sleep apnea (OSA) with the traditional uvulopalatopharyngoplasty (UPPP).
interventional comparative study.
Morgagni- Pierantoni Hospital.
seventy five patients were included in the study, divided into three groups with 25 patients per group: UPPP, ESP or BRP.
Polysomnography was done for all patients pre- and post-operatively, the post-operative results were recorded at least six months after surgery. All patients were assessed pre-operatively using drug induced sleep endoscopy. Epworth Sleepiness scale and body mass index were registered for all patients before and after surgery.
the mean of pre- and post-operative differences of apnea hypopnea index values were higher in BRP group than ESP: 15.76±14.5 Vs 10.13±5.3; P <0.05 and UPPP groups: 15.76± 14.5 Vs 6.08±5.5; P <0.0005. The mean of differences of oxygen desaturation index values was higher in BRP group than UPPP group: 15.09±17.6 Vs 7.13±6.8; P <0.0005, but not significantly higher than ESP group: 15.09± 17.6 Vs 6.48±7.9; P >0.05. The mean of differences of ESS values was higher in BRP group than ESP group: 5.52 ±4.1 Vs 4.84±3.3; P <0.005 and UPPP groups: 5.52 ±4.1Vs 1.36±1.9; P <0.005. Finally, the pre- and post-operative mean of differences of lowest oxygen saturation values were not statistically significant among the three groups (P >0.05).
BRP can be considered an effective procedure on the basis of the post-operative outcomes. ESP still proves to be a good technique especially when performed by experienced surgeons. Both techniques proved to be superior to UPPP.
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Tinnitus and sleep are closely related. One of the most important aspects of tinnitus is its association with sleep disturbance. According to the literature, up to 71% of patients with tinnitus report sleep problems (1); furthermore, insomnia is also associated with more distressing tinnitus (2). Conversely, sleep disturbance was proposed as one of the causes of chronic tinnitus (3). The relationship of tinnitus with sleep-disordered breathing (SDB) and obstructive sleep apnea (OSAS) has rarely been mentioned (4). In a recent population-based case-control study, the risk of tinnitus was found to increase 1.36 times in patients with OSAS. However, no detailed grading or severity of SDB (or OSAS) and tinnitus was provided. The purpose of this study was to investigate the exact relationship between tinnitus and SDB and delineate the incidence and severity of SDB in patients with chronic tinnitus; moreover, our study reports the preliminary outcome of tinnitus after intervention with continuous positive airway pressure (CPAP) in selected patients with moderate to severe SDB.
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There should be continued support and drive for innovation and implementation of smartphone-based access to local guidelines.
This is the first study, that we're aware of, assessing the use of a smartphone-based clinical handbook in ENT surgery.
Use of a smartphone-based handbook is superior to printed or intranet-based guidelines.
Use of smartphone apps can support junior doctors with delivery of care.
Smartphone-based access to guidelines encourage learning and education to improve the induction process for new doctors.
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Vitamin D (VD) deficiency is associated with higher risk of cancer, possibly due to anti-proliferative, anti-angiogenic, pro-apoptotic, cell-differentiating and anti-invasive effects. The anti-carcinogenic role of VD in melanoma is still a matter of debate. Loss of nuclear and cytoplasmic vitamin D Receptor (VDR) expression in melanoma cells has been reported.
To analyze VDR immunohistochemical expression in benign dermal nevi (DN) and MM.
A case-control study evaluated nuclear and cytoplasmic VDR immunohistochemical staining in 54 DN and 55 MM tissue samples.
There was a significantly higher cytoplasmic VDR-positivity in DN compared with MM (59% vs. 16%, P<0.0001). The mean VDR cytoplasmic expression was also higher in DN vs. MM (P<0.0001). No differences in nuclear VDR-positivity were observed between groups, but mean nuclear VDR expression was significantly lower in DN vs. MM (P=0.02). The loss of cytoplasmic VDR in MM was associated with Clark level, Tumor staging and pTNM AJCC staging (P=0.004, P=0.009 and P=0.02, respectively).
Alterations in VDR expression and localization are found in MM compared with DN. Loss of cytoplasmic VDR was associated with melanoma tumor size, suggesting that loss of cytoplasmic VDR may be a prognostic factor.
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Ethnic differences and temporal trends in the epidemiology of chronic spontaneous urticaria (CSU) are not well understood, especially in East Asia.1-3 The aim of our study was to investigate trends in the prevalence and incidence of CSU among adult Korean subjects from 2006 to 2014 using a nationwide, population-based study.
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The diagnosis of psoriasis in adults and children is made clinically, for both patient management and the selection of participants in research. Diagnostic criteria provide a structure for clinical assessment, which in turn helps standardise patient recruitment into clinical trials and case definitions in observational studies.
The aim of this systematic review was to identify and critically appraise the published studies to date that had a primary research aim to develop or validate diagnostic criteria for psoriasis.
A search of Ovid MEDLINE and Ovid Embase was conducted in October 2016. The primary objective was sensitivity and specificity of diagnostic criteria for psoriasis. Secondary objectives included diagnostic recommendations, applicability to children and study characteristics. Diagnostic accuracy studies were critically appraised for risk of bias using the QUADAS-2 tool.
Twenty-three studies met the inclusion criteria.None detailed clinical examination-based diagnostic criteria. The included criteria varied from genetic and molecular diagnostic models to skin imaging, histopathology, questionnaire-based, computer-aided and traditional Chinese medicine criteria. High sensitivity and specificity (>90%) were reported in many studies. However, the study authors often did not specify how criteria would be used clinically or in research. This review identified studies with varyingrisk of bias and due to each study developing separate criteria meta-analysis was not possible.
Clinical examination-based diagnostic criteria are currently lacking for psoriasis. Future research could follow an international collaborative approach and employ high quality diagnostic accuracy study design. Existing and newly developed criteria require validation.
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A series of novel coumarin pyrazoline moieties combined with tetrazoles, 3-(1-phenyl-4-(1H-tetrazol-5-yl)-1H-pyrazol-3-yl)-2H-chromen-2-one, 6-chloro-3-(1-phenyl-4-(1H-tetrazol-5-yl)-1H-pyrazol-3-yl)-2H-chromen-2-one, 6-bromo-3-(1-phenyl-4-(1H-tetrazol-5-yl)-1H-pyrazol-3-yl)-2H-chromen-2-one and 6-bromo-3-(1-(4-bromophenyl)-4-(1H-tetrazol-5-yl)-1H pyrazol-3-yl)-2H-chromen-2-one 7(a-d) were designed and synthesized. Single crystal X-ray diffraction and their interactions were studied by Hirshfeld Surface Analysis. Thermal stabilities and electrochemical properties of these compounds were examined from Differential Scanning Calorimetry (DSC), Thermo gravimetric (TGA) and Cyclic Voltametric (CV) studies. Their spectroscopic properties were analysed in various alcohols and general solvents by UV-Vis absorption, fluorescence and time resolved spectroscopy. In addition, the ground and excited state electronic properties were investigated using density functional theory (DFT). The calculated highest occupied molecular orbital (HOMO) and lowest unoccupied molecular orbital (LUMO) and energy band gap (Eg) values have revealed the effect of substitution of halogens. The substitution has equally affected the ground and excited states of 7(a-d) compounds. The solvatochromism on absorption, fluorescence spectra and fluorescence lifetimes of these compounds were investigated. All these results showed the chromen-2-one of pyrazoline tetrazole derivatives could play an important role in photonic and electronic devices.
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Adverse drug reactions of the delayed type rank among the most common dermatoses and are predominantly characterized by exanthematous macular or maculopapular eruptions. However, approximately 2 % of affected individuals develop severe or even life-threatening systemic immune reactions associated with organ involvement, requiring immediate diagnosis and treatment. Numerous drugs are capable of eliciting delayed-type hypersensitivity reactions, with antibiotics, anticonvulsant drugs, and the xanthine oxidase inhibitor allopurinol being the most common. Apart from genetic susceptibility, predisposing factors for the development of drug hypersensitivity reactions include high drug doses, polypharmacy, long treatment duration, female gender, as well as acute or chronic infections.
Mit dem Begriff „Off-Label-Use" (zulassungsüberschreitende Anwendung) wird die Verordnung pharmazeutischer Präparate außerhalb ihrer zugelassenen Anwendungsgebiete beschrieben. Für seltene Krankheiten sind häufig keine oder kaum zugelassene Therapiealternativen verfügbar. Die Kosten für eine Off-Label Verordnung werden von der Gesetzlichen Krankenversicherung (GKV) nur unter bestimmten, gesetzlich geregelten Voraussetzungen übernommen. Um Regressforderungen durch die Kostenträger zu vermeiden, kann vor Verordnung ein Antrag auf Kostenübernahme ('Off-Label Antrag') gestellt werden.
Es wurde eine retrospektive Auswertung von Off-Label Anträgen, die im Zeitraum 2010-2012 in zwei Sprechstunden einer dermatologischen Hochschulambulanz gestellt wurden, durchgeführt (Autoimmun-Sprechstunde, Urtikaria-Sprechstunde). Krankenkassen, Bewilligungsraten, Gründe für Ablehnungen und die Bearbeitungsdauer wurden ausgewertet.
Die Auswertung ergab, dass 56,8 % der eingeschlossenen Off-Label Anträge (n = 44) initial bewilligt wurden. Nach bis zu zweimaligem Widerspruch gegen die Ablehnung einer Kostenübernahme betrug die Bewilligungsrate insgesamt 75 %. Die Dauer zwischen Antragstellung und Bescheid durch die Kostenträger betrug 49 Tage (Median). Bei positivem Bescheid erfolgte die Therapieeinleitung 92 Tage (Median) nach Antragstellung.
Die vorliegende Fallserie zeigt, dass die Kostenträger den beantragten Kostenübernahmen für Off-Label Therapien im überwiegenden Teil der betrachteten Fälle zustimmten. Ob die identifizierten Probleme durch die gegenwärtigen Veränderungen der gesetzlichen Rahmenbedingungen (GKV-Versorgungsstrukturgesetz, Patientenrechtegesetz) adäquat adressiert werden, sollte in einer prospektiven Studie untersucht werden.
Die Diagnose des Sweet-Syndroms (SS) gründet sich auf eine Reihe von Kriterien, von denen mindestens zwei Haupt- und zwei Nebenkriterien erfüllt sein müssen. In einigen Fällen ist die Diagnose aufgrund des Fehlens bestimmter Kriterien jedoch nicht so einfach. Ziel der vorliegenden Studie war es, die klinischen, histopathologischen und Labormerkmale der aktuellen Diagnosekriterien für das SS zu überprüfen und ihre Aussagekraft anhand der publizierten Fälle sowie bei 40 in unserem Institut behandelten Patienten zu beurteilen. Unsere umfassende Prüfung der aktuellen Kriterien für das SS ergab, dass seit seiner Erstbeschreibung im Jahr 1964 die beiden Hauptkriterien bei allen Fällen – einschließlich unserer – durchweg vorhanden waren. Andererseits gab es hinsichtlich der Nebenkriterien deutliche Unterschiede zwischen den verschiedenen Studien und bei vielen Patienten war die Bedingung, dass zwei Nebenkriterien vorhanden sein müssen, nicht erfüllt. Wir schlagen hier zwei Gruppen von revidierten Diagnosekriterien für das SS vor. Dies geschieht mit dem Ziel, die Diagnose zu vereinfachen, Fehldiagnosen zu vermeiden und eine sofortige Behandlung zu ermöglichen. Die erste Gruppe umfasst konstante klinische und histopathologische Merkmale, die vorhanden sein müssen und die per se für die Diagnose eines SS ausreichen. Die zweite Gruppe enthält veränderliche Merkmale, deren Fehlen es nicht erlaubt, ein SS auszuschließen.
Nonmelanoma skin cancer (NMSC) is a chronic and sometimes difficult-to-treat condition affecting the quality of life (QL). The present study was conducted to investigate whether the European Organization for Research and Treatment of Cancer (EORTC) core QL Questionnaire – Cancer (QLQ-C30) is a suitable tool for the assessment of QL in patients with NMSC.
In order to define the psychometric properties of the questionnaire, the QLQ-C30 and the Dermatology Life Quality Index (DLQI) were handed out to 172 patients of the Department of Dermatology at the University Hospital Regensburg, Germany.
Internal consistencies of all multi-item scales (except one) were acceptable, with Cronbach's alpha ranging from 0.71 to 0.93. The hypothesized scale structure was supported by item/scale and interscale correlations within the QLQ-C30. Related scales of the QLQ-C30 and the DLQI correlated significantly, thus establishing construct validity. At the same time, the proportion of substantial correlations (6 % ≥ 0.40) indicated that the two questionnaires assessed distinct components of QL. The QLQ-C30 significantly differentiated between clinically distinct patient groups, indicating that severe clinical conditions were associated with greater impairment in physical, role, and cognitive functioning (p ≤ 0.030).
These results confirm the QLQ-C30 to be a suitable tool for the assessment of QL in patients with NMSC.
Nicht-melanozytärer Hautkrebs (NMSC, nonmelanoma skin cancer) ist eine chronische und mitunter schwierig zu behandelnde Erkrankung, die die Lebensqualität (LQ) beeinträchtigt. Die vorliegende Studie wurde durchgeführt um zu prüfen, ob der Kernfragebogen Quality of Life Questionnaire - Cancer (QLQ-C30) der European Organization for Research and Treatment of Cancer (EORTC) ein geeignetes Instrument für die Beurteilung der LQ bei NMSC-Patienten ist.
Zur Bestimmung der psychometrischen Eigenschaften des Fragebogens wurden der QLQ-C30 und der Dermatology Life Quality Index (DLQI) an 172 Patienten der Klinik und Poliklinik für Dermatologie des Universitätsklinikums Regensburg, Deutschland, ausgegeben.
Die interne Konsistenz aller Multi-Item-Skalen (außer einer) war akzeptabel, wobei Cronbachs Alpha-Koeffizient zwischen 0,71 bis 0,93 lag. Die angenommene Skalenstruktur wurde durch Korrelationen zwischen Items und Skalen sowie Korrelationen zwischen Skalen innerhalb des QLQ-C30 bestätigt. Verwandte Skalen des QLQ-C30 und des DLQI korrelierten signifikant und belegten damit die Konstruktvalidität. Gleichzeitig wies der Anteil substanzieller Korrelationen (6 % ≥ 0,40) darauf hin, dass mit den beiden Fragebogen unterschiedliche Aspekte der LQ beurteilt werden. Der QLQ-C30 differenzierte signifikant zwischen Patienten mit verschiedenen Erkrankungsgraden. Eine schwere Erkrankung ging mit stärkeren Beeinträchtigungen der Körperlichen, Rollen- und Kognitiven Funktion einher (p ≤ 0,030).
Diese Ergebnisse bestätigen, dass der QLQ-C30 ein geeignetes Instrument für die Beurteilung der LQ bei NMSC-Patienten darstellt.
