Evaluation of ear, nose and throat involvement in pemphigus vulgaris in comparison with pemphigus severity scoring systems: a cross-sectional study

Pemphigus vulgaris (PV) frequently affects the mucous membranes of the ear, nose, and throat (ENT). Since ENT examination is not a routinely performed procedure, the exact involvement of PV remains unrecognized. The available severity scoring systems (Pemphigus Disease Area Index (PDAI) and Autoimmune Bullous Skin Disorder Intensity Score (ABSIS)) for PV do not include a full ENT examination. This study was designed to evaluate the real extent of PV in ENT areas and to find out the specific scores which indicate the need for ENT examination. The patients were evaluated for ENT manifestations by endoscopic examination whether or not they exhibited symptoms. PDAI, ABSIS, and ENT scores were calculated, and the results were compared for correlation and significance. The mucosal involvement was more severe when scored by ENT examination than when assessed by PDAI or ABSIS. The ENT score was significantly associated with symptoms and endoscopic findings, especially when PDAI ≥15 and/or ABSIS ≥17. ENT endoscopic examination could result in more accurate grading in PV. In particular, performing such an examination should be considered in patients, especially when PDAI ≥15 and/or ABSIS ≥17, regardless of ENT symptoms. </p

Evaluation of ear, nose and throat involvement in pemphigus vulgaris in comparison with pemphigus severity scoring systems: a cross-sectional study

Pemphigus vulgaris (PV) frequently affects the mucous membranes of the ear, nose, and throat (ENT). Since ENT examination is not a routinely performed procedure, the exact involvement of PV remains unrecognized. The available severity scoring systems (Pemphigus Disease Area Index (PDAI) and Autoimmune Bullous Skin Disorder Intensity Score (ABSIS)) for PV do not include a full ENT examination. This study was designed to evaluate the real extent of PV in ENT areas and to find out the specific scores which indicate the need for ENT examination. The patients were evaluated for ENT manifestations by endoscopic examination whether or not they exhibited symptoms. PDAI, ABSIS, and ENT scores were calculated, and the results were compared for correlation and significance. The mucosal involvement was more severe when scored by ENT examination than when assessed by PDAI or ABSIS. The ENT score was significantly associated with symptoms and endoscopic findings, especially when PDAI ≥15 and/or ABSIS ≥17. ENT endoscopic examination could result in more accurate grading in PV. In particular, performing such an examination should be considered in patients, especially when PDAI ≥15 and/or ABSIS ≥17, regardless of ENT symptoms. </p

Epidemiology of pemphigus in Turkey: One-year prospective study of 220 cases

Pemphigus is a group of rare and life-threatening autoimmune blistering diseases of the skin and mucous membranes. Although they occur worldwide, their incidence shows wide geographical variation, and prospective data on the epidemiology of pemphigus are very limited. Objective of this work is to evaluate the incidence and epidemiological and clinical features of patients with pemphigus in Turkey. All patients newly diagnosed with pemphigus between June 2013 and June 2014 were prospectively enrolled in 33 dermatology departments in 20 different provinces from all seven regions of Turkey. Disease parameters including demography and clinical findings were recorded. A total of 220 patients were diagnosed with pemphigus during the 1-year period, with an annual incidence of 4.7 per million people in Turkey. Patients were predominantly women, with a male to female ratio of 1:1.41. The mean age at onset was 48.9 years. Pemphigus vulgaris (PV) was the commonest clinical subtype (n=192; 87.3%), followed by pemphigus foliaceus (n=21; 9.6%). The most common clinical subtype of PV was the mucocutaneous type (n=83; 43.2%). The mean Pemphigus Disease Area Index was 28.14±22.21 (mean ± Standard Deviation).  The incidence rate of pemphigus in Turkey is similar to the countries of South-East Europe, higher than those reported for the Central and Northern European countries and lower than the countries around the Mediterranean Sea and Iran. Pemphigus is more frequent in middle-aged people and is more common in women. The most frequent subtype was PV, with a 9-fold higher incidence than pemphigus foliaceus.   </p

Is there any predictive equation to determine resting metabolic rate in ultra-endurance athletes?

Devrim Lanpir, Asli/0000-0002-4267-9950WOS: 000460805800004Background/aims: Only a few studies determined some equations to predict resting metabolic rate (RMR) in endurance athletes, however the validity in ultra-endurance athletes, such as triathletes and ultra-marathoners, had not been examined previously. The aim of this study was to assess the accuracy of commonly used RMR predictive equations (Harris-Benedict, Mifflin-St. Jeor, Cunningham, WHO/FAO/UNU (calculated by using body mass and height and body mass alone), Wang, and Sabounchi (Structure 4, 5, and 11) equations) comparing with measured RMR in ultra-endurance athletes. Methods: Male (n=15) and female (n=15) ultra-endurance athletes age 23 to 55 years from Ankyra Sports Club were included. The Bland-Altman plot was performed to determine mean bias and limits of agreement between measured and predicted RMRs. Linear regression analysis was used to determine the accuracy of each predictive equation by computing the standard en-or of estimate and root-mean-squared prediction error (RMSPE). Results: Mifflin-St. Jeor equation was found to be the best predictive equation with lowest RMSPE (275.85 kcal/day for men and 388.34 kcal/day for women) and mean difference (3.04 +/- 285.51 kcal/day for men and 185.57 +/- 353.10 for women) in ultra-endurance athletes. The Cunningham equation could be used in estimating RMR in male athletes (RMSPE, 310.77 kcal/day, the bias between measured vs. predicted RMR, 147.68 +/- 283.04 kcal/day). Conclusions: The Mifflin- St. Jeor and Cunningham equations for men and the Mifflin-St. Jeor equation in women could be used with caution in the absence of indirect calorimetry in ultra-endurance athletes. All other predictions significantly underestimated RMR for both sexes.Hacettepe University Ethics Board and Commission [KA-180011]; Ministry of HealthMinistry of Health - Turkey [E182]This project was approved by Hacettepe University Ethics Board and Commission (research ethic project no, KA-180011) and The Ministry of Health (research ethic project no, E182). This research received no specific grant from any funding agency. No potential conflict of interest was reported by the authors. The authors thank Ebru Aslanoglu, Aslihan Nefes, Didem Gencal, Mefaret Tekin, Eylem Orhan Aksut, Salih Sari, and Bahar Sevgen at Center of Athlete Training and Health Research for their contributions and supports

Total Dietary Antioxidant Intake Including Polyphenol Content: Is It Capable to Fight against Increased Oxidants within the Body of Ultra-Endurance Athletes?

Devrim, Asli/0000-0002-4267-9950; Knechtle, Beat/0000-0002-2412-9103; Lanpir, Asli Devrim/0000-0002-4267-9950; Rosemann, Thomas/0000-0002-6436-6306WOS:000553879300001PubMed: 32586010The role of dietary antioxidants on exhaustive exercise-induced oxidative stress has been well investigated. However, the contribution of total dietary antioxidant capacity on exogenous antioxidant defense and exercise performance has commonly been disregarded. The aims of the present investigation were to examine (i) the effects of dietary total antioxidant intake on body antioxidant mechanisms, and (ii) an exhaustive exercise-induced oxidative damage in ultra-endurance athletes. The study included 24 ultra-marathon runners and long-distance triathletes (12 male and 12 female) who underwent an acute exhaustive exercise test (a cycle ergometer (45 min at 65% VO(2)max) immediately followed by a treadmill test (75% VO(2)max to exhaustion). Oxidative stress-related biomarkers (8-isoprostaglandin F2alpha (8-iso PGF2a), total oxidant status (TOS, total antioxidant status (TAS)) in plasma were collected before and after exercise. Oxidative stress index was calculated to assess the aspect of redox balance. Blood lactate concentrations and heart rate were measured at the 3rd and 6th min after exercise. Dietary antioxidant intake was calculated using the ferric reducing ability of plasma (FRAP) assay. Dietary total antioxidant intake of the subjects was negatively correlated with pre-exercise TOS concentrations (rs = -0.641 in male, and rs = -0.741 in females) and post- vs. pre- ( increment ) 8-iso PGF2a levels (rs = -0.702 in male;p= 0.016, and rs = -0.682 in females;p= 0.024), and positively correlated with increment TAS concentrations (rs = 0.893 in males;p= 0.001, and rs = 0.769 in females;p= 0.002) and post- exercise lactate concentrations (rs = 0.795 for males;p= 0.006, and rs = 0.642 for females;p= 0.024). A positive meaningful (p= 0.013) interaction was observed between time at exhaustion and dietary antioxidant intake (rs = 0.692) in males, but not in females. In conclusion, the determination of total dietary antioxidant intake in ultra-endurance athletes may be crucial for gaining a better perspective on body antioxidant defense against exhaustive exercise-induced oxidative stress. However, the effects of dietary antioxidant on exercise performance and recovery rate needs further investigation.Ministry of Youth and Sports of TurkeyThe study was supported by The Ministry of Youth and Sports of Turkey. The authors would like to thank Erkan Tortu, Ebru Aslanoglu, Aslihan Nefes, Didem Gencal, Mefaret Tekin, Eylem Orhan Aksut, Salih Sari, and Bahar Sevgen for their help in collecting data and supports

FBX07-R498X mutation: Phenotypic variability from chorea to early onset parkinsonism within a family

Objective: FBXO7 mutations (PARK 15), first reported in 2008, are among the monogenic causes of early-onset parkinsonism. Classically, PARK 15 was suggested to correspond to previously described pallido-pyramidal syndrome. Here, we report clinical and genetic findings in a unique family of Kurdish origin with an FBXO7 mutation and presenting with diverse clinical phenotypes

Internalized stigma in psoriasis: A multicenter study

Internalized stigma is the adoption of negative attitudes and stereotypes of the society regarding a person's illness. It causes decreased self-esteem and life-satisfaction, increased depression and suicidality, and difficulty in coping with the illness. The primary aim of this study was to investigate the internalized stigma state of psoriatic patients and to identify the factors influencing internalized stigma. The secondary aim was to identify the correlation of internalized stigma with quality of life and perceived health status. This multicentre, cross-sectional study comprised 1485 patients. There was a significant positive correlation between mean values of Psoriasis Internalized Stigma Scale (PISS) and Psoriasis Area and Severity Index, Body Surface Area, Dermatological Life Quality Index and General Health Questionnaire-12 (P < 0.001 in all). Lower percieved health score (P = 0.001), early onset psoriasis (P = 0.016), family history of psoriasis (P = 0.0034), being illiterate (P < 0.001) and lower income level (P < 0.001) were determinants of high PISS scores. Mean PISS values were higher in erythrodermic and generalized pustular psoriasis. Involvement of scalp, face, hand, genitalia and finger nails as well as arthropathic and inverse psoriasis were also related to significantly higher PISS scores (P = 0.001). Our findings imply that psoriatic patients experience high levels of internalized stigma which are associated with psoriasis severity, involvement of visible body parts, genital area, folds or joints, poorer quality of life, negative perceptions of general health and psychological illnesses. Therefore, internalized stigma may be one of the major factors responsible from psychosocial burden of the disease

Internalized Stigma in Pediatric Psoriasis: A Comparative Multicenter Study

Background: Internalized stigma, adoption of negative attitudes and stereotypes of the society regarding persons' illness, has not been studied previously in pediatric psoriasis patients. Objective: We aimed to investigate the internalized stigma in pediatric psoriasis patients and to determine differences according to factors affecting internalized stigma compared to adult psoriasis patients. Methods: This multicenter, cross-sectional, comparative study included 125 pediatric (55 female, 70 male; mean age +/- standard deviation [SD], 14.59 +/- 2.87 years) and 1,235 adult psoriasis patients (577 female, 658 male; mean age +/- SD, 43.3 +/- 13.7 years). Psoriasis Internalized Stigma Scale (PISS), Dermatology Life Quality Index (DLQI), Perceived Health Status (PHS), and the General Health Questionnaire (GHQ)-12 were the scales used in the study. Results: The mean PISS was 58.48 +/- 14.9 in pediatric group. When PISS subscales of groups were compared, the pediatric group had significantly higher stigma resistance (p = 0.01) whereas adult group had higher scores of alienation (p = 0.01) and stereotype endorsement (p = 0.04). There was a strong correlation between mean values of PISS and DLQI (r = 0.423, p = 0.001). High internalized stigma scores had no relation to either the severity or localization of disease in pediatric group. However, poor PHS (p = 0.007) and low-income levels (p = 0.03) in both groups, and body mass index (r = 0.181, p = 0.04) in the pediatric group were related to high PISS scores. Conclusion: Internalized stigma in pediatric patients is as high as adults and is related to poor quality of life, general health, and psychological illnesses. Unlike adults, internalized stigma was mainly determined by psoriasis per se, rather than disease severity or involvement of visible body parts, genitalia or folds