scholarly journals Pain in adult myotonic dystrophy type 1: relation to function and gender

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Gro Solbakken ◽  
Sissel Løseth ◽  
Anne Froholdt ◽  
Torunn D. Eikeland ◽  
Terje Nærland ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Gender Differences ◽  
Pain Intensity ◽  
Myotonic Dystrophy ◽  
Forced Vital Capacity ◽  
Respiratory Function ◽  
Vital Capacity ◽  
Disease Duration ◽  
Autism Spectrum

Abstract Background Pain is prevalent in myotonic dystrophy 1 (DM1). This study investigated whether CTG repeat size, disease duration, BMI and motor and psychological function were related to pain in adult patients with DM1, and if there were gender differences regarding intensity and location of pain. Method Cross-sectional design. Pain was investigated in 50 genetically confirmed DM1 patients by combining clinical assessment and self-reports of pain intensity and locations. Pain scoring results were related to CTG size, disease duration, muscle strength, walking capacity measured by 6-min walk test, activity of daily life by Katz ADL Index, respiratory function by Forced Vital Capacity and BMI. In addition, the degree of reported pain was related to Quality of life measured by WHOQOL-BREF; fatigue was measured by Fatigue severity scale; psychological functions were measured by Beck Depression Inventory, Beck Anxiety Inventory, IQ and Autism spectrum Quotient. Results Pain was reported in 84% of the patients and was significantly correlated with CTG size (r = 0.28 p = 0.050), disease duration (r = 0.38 p = 0.007), quality of life (r = − 0.37 p = 0.009), fatigue (r = 0.33 p = 0.02) and forced vital capacity (r = − 0.51, p = 0.005). Significant gender differences, with higher scores for females, were documented. In male subjects the number of pain locations was significantly correlated with quality of life and the autism quotient. In females, pain intensity was significantly correlated with activity, respiratory function and BMI. Conclusions Pain in DM1 was prevalent, with a strong association to lung function and other aspects of the disease. Significant gender differences were present for pain intensity and number of pain locations. How pain was related to other symptoms differed between male and female subjects. Our findings highlight the importance of assessments of pain in DM1 patients.

scholarly journals ILD-specific health-related quality of life in systemic sclerosis-associated ILD compared with IPF

2020 ◽  
Vol 7 (1) ◽  
pp. e000598
Author(s):  
Michael T Durheim ◽  
Anna-Maria Hoffmann-Vold ◽  
Tomas M Eagan ◽  
Arnt-Ove Hovden ◽  
May Brit Lund ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Function ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
Health Related Quality ◽  
Related Quality ◽  
Health Related

IntroductionIdiopathic pulmonary fibrosis (IPF) and systemic sclerosis-associated interstitial lung disease (SSc-ILD) are fibrotic ILDs with divergent disease populations. Little is known about health-related quality of life (HRQL) in SSc-ILD relative to IPF.MethodsWe used the Kings Brief Interstitial Lung Disease Questionnaire (K-BILD) to compare HRQL in a cross-sectional study of 57 patients with IPF and 29 patients with SSc-ILD. Analysis of covariance was used to adjust for age, gender and lung function.ResultsThe unadjusted mean K-BILD score was 63.1 (95% CI 57.1 to 69.1) among patients with SSc-ILD, as compared with 54.7 (51.8–57.5) among those with IPF (p=0.005). However, this difference in HRQL was attenuated after adjustment for age, gender and lung function. In a multivariable model, only forced vital capacity was associated with K-BILD scores. K-BILD scores were correlated with both forced vital capacity and with other relevant HRQL measures, regardless of ILD diagnosis.DiscussionPatients with SSc-ILD may have better ILD-specific quality of life than patients with IPF, but this difference appears to be driven primarily by better lung function. These results underscore the impact of lung function on HRQL in fibrotic ILD and the utility of K-BILD to assess HRQL in SSc-ILD.

Forced Vital Capacity and Quality of Life: BOLD Study Cross-Sectional Analysis

2021 ◽  
Author(s):  
Emmanouil Bagkeris ◽  
Jaymini Patel ◽  
Christer Janson ◽  
Andre Amaral ◽  
Peter Burney
Keyword(s):  
Quality Of Life ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
Cross Sectional ◽  
Cross Sectional Analysis ◽  
Sectional Analysis

Quality of life in SSc-ILD patients: Understanding the impact of the ILD and the needs of the SSc-ILD patients and their need for caregivers in France

2021 ◽  
pp. 239719832110139
Author(s):  
Yannick Allanore ◽  
Joel Constans ◽  
Dominique Godard ◽  
Gerard de Pouvourville ◽  
Stephane Bouee ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
Disease Score ◽  
System P ◽  
The Impact

Objectives: The objectives of this study were to describe the impact of systemic sclerosis associated interstitial lung disease, on quality of life, to estimate the correlation between quality of life and severity of lung disease and to assess the impact of interstitial lung disease on caregivers. Methods: Seven investigators included systemic sclerosis associated interstitial lung disease patients from December 2019 to April 2020. Sociodemographics and clinical data were collected. Patients reported outcomes and questionnaires were used with 1 generic patients reported outcome (EQ-5D-5L), 1 specific PRO (Brief Interstitial Lung Disease) and 2 self-reported questionnaires on impact of SSc complications and impact on caregivers. The correlation between forced vital capacity and EQ-5D-5L score was estimated with a multivariate linear regression model adjusted on several covariates. Results: In all, 89 patients were included. 26.4% were males, mean age was 58.2 ± 14.5 years. Mean EQ-5D-5L score = 0.79 ± 0.22 (median = 0.85). Mean EQ-5D-5L visual analog scale score = 60.8 ± 20.4 (median = 61.5). Mean King’s Brief Interstitial Lung Disease score = 58.4 ± 12.7 (median = 58.0). After adjustment on covariates, a significant correlation between forced vital capacity and EQ-5D-5L score was found with an increase of 0.003 of the EQ-5D-5L score for a 1% increase of FVC (p = 0.0096). No significant correlation between forced vital capacity and the EQ-VAS and King’s Brief Interstitial Lung Disease score were found. The impact of SSc on other organs was significantly correlated with EQ- 5D-5L score, respectively, for the impact scores on the lung system (p = 0.0003), heart system (p = 0.0182), Raynaud’s syndrome (p = 0.0015), digestive system (p = 0.0032), joints/muscles (p = 0.0003), skin (p < 0.0001), kidney (p = 0.0052) and gastro-oesophageal reflux (p = 0.0063). Significant correlations between King’s Brief Interstitial Lung Disease score and lung system (p < 0.0001), heart system (p < 0.0001), digital ulcers (p = 0.058), digestive system (p < 0.0001), kidney (p = 0.0004), skin (p = 0.0499) and gastro-oesophageal reflux (p = 0.0033) scores were found 68.5% of patients reported their need for a caregiver to help them in their daily life activities. Conclusion: Our study highlighted the strong burden of systemic sclerosis associated interstitial lung disease` for patients, especially with an impact on quality of life, on other organs manifestations and need for caregivers in their daily life.

Development and Validation of a Questionnaire to Assess Parent Reported Quality of Life Pre and Post Vision Therapy in a Population with Autism Spectrum Disorder

2019 ◽  
pp. 195-207
Keyword(s):  
Quality Of Life ◽  
Autism Spectrum Disorder ◽  
Social Behavior ◽  
Psychometric Properties ◽  
Autism Spectrum ◽  
Spectrum Disorder ◽  
Visual Behavior ◽  
The Mean ◽  
Vision Therapy

Background: Autism spectrum disorder is characterized in part by atypical behavior in the communication, social, and visual domains. Success in vision therapy is judged not only by changes in optometric findings, but through improvement in quality of life involving communication, social behavior and visual behavior. It would therefore be beneficial to have a validated questionnaire to assess parent reported quality of life pre and post vision therapy specific to patients with autism spectrum disorder. To our knowledge, a questionnaire of this nature has not been previously published in the literature. Methods: Questionnaire items were generated through surveying medical literature based on symptoms in three different categories: visual behavior, social behavior and communication. A pool of 34 questions was developed initially and then with thorough discussion with other experts, a 20-point questionnaire was developed with each item reflected in the construct concept. A draft of 20 questions was then sent to 10 subject experts with clinical experience in the field for more than 20 years, to review the pooled items. Validity and reliability was established prior to assessing the psychometric properties of the ASD/QOL-VT. Prospective observational study was conducted for a duration of 18 months. The study included individuals undergoing vision therapy in the age range of 3 to 15 years who had been diagnosed with ASD. The questionnaire was administered to parents of these children prior to the start of vision therapy. All subjects completed a minimum of 60 vision therapy sessions. The questionnaire was readministered after completing 60 sessions of vision therapy. Results: Cronbach’s alpha value for this questionnaire was 0.93, which reflected very good internal consistency. Factorial analysis yielded four factors with an Eigen value exceeding 1.0 which accounted for 68% variation in the model. The Cronbach alpha value for subscales identified by factorial analysis is 0.97 indicating excellent internal reliability. The mean pre vision therapy social behavior, communication and visual behavior score was 12.0±3.21, 17.07±4.57 and 26.97±6.41 respectively. The mean post vision therapy scores for social behavior, communication and visual behavior was 8.27±4.16, 11.33±5.27 and 17.93±6.52 respectively. On paired t test, the mean difference in score was statistically significant with P<0.001 in all three subcategories. Conclusions: Our study presents the development of a valid and reliable parent questionnaire, the ASD/QOL-VT, that judges communication, social behavior, and visual behavior in autism. Results of the study conducted indicate that vision therapy can result in significant improvements in the quality of life of patients with ASD as judged by their parents. This is evidenced by statistically significant changes in psychometric properties of the ASD/QOL-VT in social behavior, communication and visual behavior.

Health related quality of life of rosacea patients in China assessed by DLQI and willingness to pay: Cross-Sectional Questionnaire Study (Preprint)

2020 ◽  
Author(s):  
Yaqun Huang ◽  
Sha Yan ◽  
Hongfu Xie ◽  
Ben Wang ◽  
Zhixiang Zhao ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Willingness To Pay ◽  
Disease Duration ◽  
Life Quality ◽  
Cross Sectional Study ◽  
Health Related Quality ◽  
Cross Sectional ◽  
Related Quality ◽  
Health Related

BACKGROUND Rosacea is a chronic inflammatory dermatosis with facial skin involved, leading to physical and emotional problems, which greatly affected quality of life (QoL) of patients. Dermatology Life Quality Index (DLQI) and willingness to pay (WTP) are well-established instruments assessing the health-related quality of life (HRQoL), while very few studies have been focused on this topic about rosacea in China. OBJECTIVE To investigate HRQoL in Chinese rosacea patients assessed by DLQI and WTP and investigate potential predictors for patients with HRQoL severely affected. METHODS This cross-sectional study was conducted on 973 patients with rosacea. Sociodemographic data, clinical features and DLQI were collected, and WTP was assessed by three standardized items. Multivariable logistic analysis was performed to investigate independent factors influencing QoL. RESULTS 921 questionnaires were accomplished by participants. The mean DLQI score was 11.6 (median 11). Patients were willing to pay an average of $1050.2 or € 896.2 (median $431.4 or € 368.1) for complete cure. 33.3% would like to pay more than 20% of their monthly income to achieve sustainable control. There were positive correlations between WTP with DLQI (P < .05). DLQI could be independently impacted by age (21-30 and 31-40, OR = 3.242 and 3.617, respectively), the occupational requirement of appearance (high, OR = 4.410), disease duration (< 2 years, OR = 1.582), oedema (OR = 1.844) and severity of flushing, burning, stinging and pruritus (severe, OR = 2.003, 1.981, 2.491, 2.249, respectively). There were no significant associations between WTP and most of the clinical factors. CONCLUSIONS The QoL was negatively affected and should not be ignored among rosacea patients in China. Patients aged 21-40y, having occupational requirement of appearance, with the disease duration less than 2 years, and suffering severe flushing and related symptoms were more likely to have severe or very severe limitation of QoL.

Joint involvement influences quality of life in systemic lupus erythematosus patients

Lupus ◽  
2020 ◽  
pp. 096120332097903
Author(s):  
Francesco Natalucci ◽  
Fulvia Ceccarelli ◽  
Enrica Cipriano ◽  
Carlo Perricone ◽  
Giulio Olivieri ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Joint Involvement ◽  
Systemic Lupus ◽  
Median Disease Duration ◽  
Group A ◽  
Musculoskeletal Manifestations ◽  
Group B

Introduction Joint involvement represents the major determinant in quality of life (QoL)in Systemic Lupus Erhytematosus (SLE) patients. However, QoLhas been generally evaluated by non-specific questionnaires. We evaluated the relationship between SLE musculoskeletal manifestations and QoL, assessed by LupusQoL. Methods Patients with joint involvement (group A) were compared with those without this feature (group B). Disease activity was assessed by SLEDAI-2k in the whole population, while DAS28 and swollen to tender ratio were applied to assess joint activity. LupusQoL was administered to all the patients. Results Group A included 110 patients [M/F 8/102; median age 49 years (IQR 13), median disease duration 156 months (IQR 216)], group B 58 [M/F 11/47; median age 40 years (IQR 15), median disease duration 84 months (IQR 108)].We found significanlty lower values in all the LupusQoL domains except for one (burden to others) in group A in comparison with group B. A significant correlation between DAS28 values and all the LupusQoL domains in group A was found; only three domains correlated with SLEDAI-2k. Conclusions SLE-related joint involvement significantly influences disease-specific QoL. DAS28 better correlated with LupusQoL domains in comparison with SLEDAI-2k, confirming the need for specific musculoskeletal activity indices.

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