Surgical treatment of enterovirus D68 brainstem encephalitis-induced dysphagia

Cluster of acute flaccid paralysis and cranial nerve dysfunction was associated with a 2014 outbreak of enterovirus D68 (EV-D68) respiratory illness in US. We describe a 33 year-old male patient of refractory dysphagia due to EV-D68-induced brainstem encephalitis successfully treated by surgery. Following acute upper respiratory tract infection, he developed dysphagia and bilateral facial paralysis. A coughing reflex was readily produced when the laryngopharyngeal fiberscope touched the epiglottis, however, water infusion induced only very weak and slow swallowing reflex, suggesting that only motor component was impaired but sensory function was preserved during swallowing.

http://ift.tt/2Bjhaza

Lysosomal Acid Lipase Deficiency: Report of Five Cases across the Age Spectrum

Lysosomal acid lipase (LAL) deficiency is an autosomal recessive lysosomal storage disorder caused by mutations in the LIPA gene that leads to premature organ damage and mortality. We present retrospective data from medical records of 5 Brazilian patients, showing the broad clinical spectrum of the disease.

http://ift.tt/2DTU9WD

Differential effects of secukinumab vs. ustekinumab for treatment of psoriasis on quality of life, work productivity and activity impairment: a structural equation modelling analysis

Abstract

Purpose

This study examined direct and indirect (mediated) effects of secukinumab vs. ustekinumab on quality of life, work productivity, and activity impairment based on psoriasis severity and symptoms.

Methods

Analyses were based on data from the CLEAR study. Structural equation modelling (SEM) examined the effects of secukinumab vs. ustekinumab on the Dermatology Life Quality Index (DLQI) and on the Work Productivity and Activity Impairment (WPAI) questionnaire using Psoriasis Area Severity Index (PASI) severity and symptoms (pain, itching, and scaling) as potential mediators. Analyses were conducted primarily for patients achieving PASI 90 response (indicating a 90% or greater reduction in PASI from baseline) at week 16 (repeated at week 52) and for PASI 50, 75, and 100.

Results

Results at weeks 16 and 52 showed that the effect of treatment on change in DLQI score was mediated by PASI 90 response and by improvements in itching and scaling. Achieving any PASI response as early as week 16 directly resulted in significantly better WPAI scores. At week 52, both PASI response and improvement in scaling directly resulted in significantly better WPAI scores. Pain, itching, and scaling were correlated (r = 0.51 to 0.68); improvement in any of these had a significant effect (directly or indirectly) on WPAI. All results favoured secukinumab over ustekinumab.

Conclusion

The results underscore the important role of both PASI response and reduction in symptoms on improvements in health-related quality of life and work and daily activity in favour of secukinumab vs. ustekinumab.

This article is protected by copyright. All rights reserved.

http://ift.tt/2BhpRdv

How should minimally important change (MIC) scores for the Patient Oriented Eczema Measure (POEM) be interpreted? A validation using varied methods

Summary

Background

The Patient Oriented Eczema Measure (POEM), scored 0-28, is the core outcome instrument recommended for measuring patient-reported atopic eczema symptoms in clinical trials. To date, two published studies have broadly concurred that the MIC of the POEM is 3 points. Further assessment of the minimally important change (MIC) of POEM in different populations, and using a variety of methods, will improve interpretability of the POEM in research and clinical practice.

Objectives

To calculate the smallest detectable change in the POEM and estimate the MIC of the POEM using a variety of methods in a trial dataset of children with moderate to severe atopic eczema.

Methods

This study used distribution-based and anchor-based methods to calculate the MIC of the POEM in children with moderate to severe eczema.

Results

Data was collected from 300 children. The smallest detectable change was 2.12. The MIC estimates were 1.07 (0.2 SD) and 2.68 (0.5 SD) based on distribution-based methods, were 3.09 to 6.13 based on patient-reported anchor-based methods, and were 3.23 to 5.38 based on investigator-reported anchor-based methods.

Conclusions

We recommend the following thresholds are used to interpret changes in POEM scores: ≤ 2, unlikely to be a change beyond measurement error; 2.1 to 2.9, a small change detected that is likely to be beyond measurement error but may not be clinically important; 3 to 3.9, probably a clinically important change; 4+, very likely to be a clinically important change.

This article is protected by copyright. All rights reserved.

http://ift.tt/2mY74j6

In vivo dermoscopic and confocal microscopy multi-step algorithm to detect in situ melanomas

Summary

Background

Although several dermoscopic features of in situ melanoma (MIS) have been identified, data on confocal features for MIS are still lacking.

Objectives

To identify RCM (reflectance confocal microscopy) features of MIS and to develop a diagnostic score for MIS while combining dermoscopy and RCM.

Methods

120 MIS and 213 nevi (test set) were retrospectively analysed to assess the presence of dermoscopic and RCM criteria. Facial and acral lesions have been excluded. Spearman's correlation, univariate and multivariate regression models were performed to find features significantly correlated with MIS diagnosis. Multivariate results on the test set allowed the development of a multi-step algorithm, that was tested on a validation set counting 100 lesions.

Results

Dermoscopic atypical network and regression resulted as independent predicting factors for MIS diagnosis (OR=3.437, 95%CI=1.696-6.966 and OR=4.168, 95%CI=1.930-9.002 respectively). Significant confocal predictors for malignancy were epidermal pagetoid spread (OR=2.829, 95%CI=1.324-6.043) and junctional cytological atypia (OR=3.390, 95%CI=1.383-8.305 if focal, OR=8.439, 95%CI=3.213-22.165 if widespread). A multi-step diagnostic algorithm able to predict MIS with a sensitivity of 92.5% and a specificity of 61% was developed. The validation set confirmed the high diagnostic value (sensitivity 92%, specificity 58%).

Conclusions

An easy and reproducible multi-step algorithm for MIS detection is suggested, that can be routinely used in tertiary centres.

This article is protected by copyright. All rights reserved.

http://ift.tt/2BiFt0j

Analysis of the skin mycobiome in adult patients with atopic dermatitis

Abstract

With the recent availability of culture-independent sequencing methods, studies have been conducted to analyze skin microorganisms present in patients with atopic dermatitis (AD). However, the database on the skin fungal communities, "mycobiome," has been relatively restrictive compared with the bacterial world. We aimed to comparatively analyze the overall skin mycobiome between patients with AD and healthy individuals in the Korean population. We analyzed skin swab samples obtained from the antecubital fossae of 8 patients with AD and 8 healthy controls. Using sequencing method followed by direct DNA extraction and molecular PCR, taxonomic compositions of fungi at stepwise level ranks were analyzed. The phylogenic marker used was internal transcribed spacer 2 regions of DNA. We observed the tendency of higher intra- and inter-personal taxonomic diversity at genus and species levels in AD samples. Non-Malassezia fungal diversity was also noticeable in the patient group compared with healthy controls. Malassezia globosa and Malassezia restricta were prevalent in all samples across both study groups, and some Malassezia species, including Malassezia sloofiae and dermatis characterized AD. Our data might provide a new insight into the mycobiome of adult AD, which contributes to building a systemic mycobiome database in AD.

This article is protected by copyright. All rights reserved.

http://ift.tt/2F0tOpg

Seasonal changes in epidermal ceramides are linked to impaired barrier function in acne patients

Abstract

Acne skin demonstrates increased transepidermal water loss (TEWL) compared with healthy skin, which may be due, in part, to altered ceramide levels. We analysed ceramides in the stratum corneum of healthy and acne skin, and studied seasonal variation over the course of a year. Using ultraperformance liquid chromatography with electrospray ionisation and tandem mass spectrometry (UPLC/ESI-MS/MS), we identified 283 ceramides. Acne-affected skin demonstrated overall lower levels of ceramides, with notable reductions in CER[NH] and CER[AH] ceramides, as well as the acylceramides CER[EOS] and CER[EOH]; these differences were more apparent in the winter months. Lower ceramide levels reflected an increase in TEWL in acne, compared with healthy skin, which partly resolves in the summer. Individual ceramide species with 18-carbon 6-hydroxysphingosine (H) bases (including CER[N(24)H(18)], CER[N(26)H(18)], CER[A(24)H(18)], CER[A(26)H(18)]) were significantly reduced in acne skin, suggesting that CER[NH] and CER[AH] species may be particularly important in a healthy skin barrier.

This article is protected by copyright. All rights reserved.

http://ift.tt/2DAkn2H