scholarly journals Fatigue in adults with Osteogenesis Imperfecta

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Arjan G. J. Harsevoort ◽  
Koert Gooijer ◽  
Fleur S. van Dijk ◽  
Daniëlle A. F. M. van der Grijn ◽  
Anton A. M. Franken ◽  
...  

Abstract Background Osteogenesis Imperfecta (OI) is characterized by bone fragility, and features such as blue sclerae, dentinogenesis imperfecta, hearing loss, ligamentous laxity and short stature can be present. It has long been assumed that the functional ability and quality of life of patients with OI depends primarily on the severity of skeletal deformities. However, fatigue is often mentioned in clinic by patients with all types of OI as an important modifier of their quality of life and does not always seem to be related to their functional ability. The aim of this study is to investigate whether adults with Osteogenesis Imperfecta are significantly more fatigued than the normal population. Methods The Fatigue Severity Scale (FSS) was distributed by mobile phone application among 151 adult patients with different OI types. Results of the FSS in the OI group were compared with two control populations from America (n = 20) and the Netherlands (n = 113). Results Ninety-nine patients (OI type 1 (n = 72), OI type 3 (n = 13), OI type 4 (n = 14) completed the FSS questionnaire. The mean FSS score of this cohort was 4.4 and significantly higher than the control populations (2.3/2.9). 65% of our cohort reported at least moderate fatigue compared with 2 control populations from America and the Netherlands. Conclusion Fatigue in patients with OI is a frequently encountered problem in our expert clinic but research into this topic is sparse. This pilot study is the largest study to date investigating fatigue in patients with OI and results have been compared with two control groups. The mean FSS score of 4.4 in the OI group indicates that people with OI are generally significantly more fatigued than the control population. Further evaluation of fatigue and its influencers in a larger group of OI patients is important for future management.

2010 ◽  
Vol 30 (S 01) ◽  
pp. S104-S106
Author(s):  
W. Miesbach ◽  
L. M. Sahner ◽  
A. Kurth ◽  
B. Habermann

Summary Purpose Purpose of this retrospective study was to evaluate our own results after total knee replacement in patients with haemophilia. Patients, material, method: 30 patients with haemophilia who underwent total knee replacement between 1987 and 2005 were included. We used the clinical and radiological Knee Society Score. Furthermore, the Petterson and the Arnold and Hilgartner score were applied. Results: The mean age at the time of surgery was 43.2 (27–66). At the time of follow- up examination the mean age was 51.6 (30–82) years. The mean follow-up was 7.1 (2–20) years. Preoperative, he mean Arnold and Hilgartner score was 4.17 (± 0.59) and the mean Petterson-Score was 9 ± 2.29. Compared to the preoperative deficiency in knee function (KSS-Score 88.17 ± 33.58) an improvement with 166.67 (± 22.73) points was seen. 1 patient showed an aseptic loosening after 11 years. Discussion: Total knee replacement in patients with haemophilia improves knee function and quality of life. The results of our study represent results in earlier published studies. Compared to a non-haemo-philic normal population the rate of perioper-ative complications was not increased.


2021 ◽  
Author(s):  
Han Zhang ◽  
Yi Peng ◽  
Pengwei Hou ◽  
Zhu Chen ◽  
Guoqiang Xing ◽  
...  

Abstract Purpose The epidemiological and clinical features, pathogenesis, and complications of patients with COVID-19 in the acute phase have been well described, but the long-term prognosis and rehabilitation of the patients remain largely unknown.Methods 39 COVID-19 patients were followed up at 3, 6, and 12 months after discharge. The mental health, pulmonary function, exercise capacity, and quality of life were assessed by Self-Depression Scale and the Self Anxiety Scale, pulmonary function test, 6MWT, 36-Item Short-Form, respectively. Results Total 33 survivors completed the assessment, 40.8 ± 0.8 years, body mass index= 22.7 ±1.3 kg/m2. The length of hospital stays was 19.6 ± 6.6 d. One year after discharge, the mean scores of SDS and SAS showed decreasing trends from 3-months to 12-months post-discharge. 6 patients (18.2%) had FVC <80% of the predicted value, 12 patients (36.4%) had FEV1 <80% of the predicted value. And 9 (27.3%), 3 (9.1%), and 2 (6.1%) of the patients showed reduced FEF25, FEF50, and FEF75 (<70% expected values), respectively. The mean 6MWD values increased significantly from 397±25.4 m at 3-months to 514±40.8 m at 12-months.Conclusions the impaired pulmonary function in mild COVID-19 survivors was noted after 12 months discharging from hospital. The exercise capacity, mental status, and health status were lower than those of the normal population.


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 2974-2974
Author(s):  
Nora Machate ◽  
Haefaa Alchalby ◽  
Susanne Sehner ◽  
Tatjana Zabelina ◽  
Daniel Wolff ◽  
...  

Abstract Introduction Myelofibrosis (MF) is a clonal hematological disorder classified as a myeloproliferative neoplasm (MPN). Bone marrow fibrosis, splenomegaly and extramedullary hematopoiesis are hallmarks of the disease. Symptomatic burden is present in most of the affected patients and can compromise quality of life (QoL) by its severity. Most patients suffer from constitutional symptoms such as fatigue, pruritus, night sweats, bone pain, fever, early satiety and weight loss. Currently allogeneic stem cell transplantation (HSCT) is the only curative therapy but it is associated with a high morbidity and mortality. Infectious complications, acute and chronic GvHD are major complications which influence quality of life after transplantation. Until now, no systematic evaluation of QoL after allogeneic stem cell transplantation for myelofibrosis patients exists. Methods In this cross-sectional study we examined 77 patients (male=37, female=40) with a median age of 61 years (range, 40 to 79 years) who received an allogeneic HSCT with a busulfan-based reduced-intensity conditioning from related (n=20) or unrelated donor (n=54) in the period from 1999 to 2011. Diagnosis was primary myelofibrosis in 47 patients and secondary myelofibrosis in 30 patients. At time of transplantation all patients had advanced disease and were classified by the Lille score as high-risk (n=18), intermediate-risk (n=34) and low-risk (n=12) patients. At time of evaluation, 72 patients were in complete remission, 5 patients were not in complete remission, 25 patients suffered from GvHD (limited=14, extensive=11). The patient population was divided into four groups according to the time between transplantation and evaluation of QoL. First group = 6-36 months (n=24); second group= 36-60 months (n=16); third group = 60-85 months (n=22); fourth group 85-146 months after transplantation (n=15). QoL was measured by using the two validated questionnaires FACT-BMT and MPN-SAF. By means of statistical analysis (SPSS; ANCOVA) it was examined whether there are significant differences between the groups. Results According to the MPN-SAF Total Symptom score (the lower the score the better is the Result: possible range= 0-100, the mean scores of the groups one to four are as followed: group1: 18.9 (SD =11.3), group2: 21.1 (SD=17.9), group 3:19.3 (SD=15.5) and group 4: 18.9 (SD=12.4). There were no significant differences between the groups (p=0,962). Unfortunately no MPN-SAF Total Symptoms score was available before transplantation, but according to the mean value of MPN described in the literature (mean=25.3; SD=17.2) the mean score after transplantation (mean=19.5; SD=14.0) of our study population was lower. The mean scores of the FACT-BMT from group one to four were 115.3 (SD=16.6); 120.9 (SD=17.5); 112.4 (SD=15.5) and 115.3 (SD=20.9) There were also no significant differences between the 4 groups (p=0.541). We investigated whether there are significant differences of the results in both questionnaires regarding gender (male/female), donor (unrelated/related), GvHD (yes/no), HLA (matched/mismatched), primary or secondary MF (post-PV/post-ET/PMF), remission (complete/not complete), but none of the variables did influence QoL significantly. The subscales BMTS, FACT-G, and its subscales Physical Well-Being (PWB), Functional Well-Being (FWB), Emotional Well-Being (EWB), Social Well-Being (SWB), of the FACT-BMT were calculated and analyzed by ANCOVA. No significant differences were found among the groups. Comparing the mean value of the FACT-G (mean=85.7; SD=13,1; range= 60-108)and its subscales PWB (mean=22.7; SD= 4.5), SWB (mean=22.4; SD=4.7), EWB (mean 19.9; SD=3.1), FWB (mean=20.6; SD=4.7) of our entire patient population with the mean value of a normal population FACT-G (mean=80.1; SD=18.1; range= 15.4-108), PWB (mean=22.7; SD= 5.4), SWB (mean= 19.1; SD=6.8), EWB (mean=19.9; SD=4.8) and FWB (mean=18.5; SD=6.8) our population has even the same or higher scores. Conclusion This cross-sectional study shows that after dose reduced allogeneic stem cell transplantation in MF-patients Physical and Emotional Well Being is almost as good as that of the normal healthy population whereas Social and Functional Well Being seem to be classified as even better than that of a normal population. Disclosures: No relevant conflicts of interest to declare.


2014 ◽  
Vol 7 (3) ◽  
pp. 96-99
Author(s):  
SS Bist ◽  
Sarita Mishra ◽  
Vinish Agrawal ◽  
Priyanka Gairola

ABSTRACT Introduction Chronic rhinosinusitis (CRS) is a global health problem affecting approximately 14% of the population of all age groups. CRS has a significant adverse impact on quality of life of the patients. Objective The aim of this study was to compare the quality of life of patients suffering from CRS with that of normal population and to assess the association between objective nasal endoscopic score, self-rated symptom score and quality of life (QoL) score. Methodology This was a prospective study conducted over a period of 12 months. A total of 110 cases of CRS and 50 normal subjects taken as controls were recruited from out patients department. Patients diagnosed as a case CRS were subjected to rhinosinusitis disability index (RSDI), subjective QoL questionnaires and self-rated symptom score in their vernacular language followed by diagnostic nasal endoscopy, using 0° rigid endoscope (4 mm). Results This study revealed that the mean ± SD total QoL score in the patients was 50.2 ± 14.9 (15-94) which was significantly affected as compared to control group where it was only 18.2 ± 9.15 (3-47). The mean ± SD of the functional, emotional and physical domains were 17.6 ± 5.46 (4-34), 16.5 ± 8.29 (5-88) and 16.5 ± 5.84 (2-35) respectively. There was no correlation between endoscopic score and the patient's self-rated symptom score or RSDI score. The correlation between the self-rated symptom score and total QoL score was significant (p = 0.02). Conclusion On comparing the QoL in the patients of CRS with the controls, it was observed that QoL was affected more in the patients of CRS in the physical, functional and emotional domain of their life. CRS has considerable adverse impact on QoL of patients and RSDI is a valuable tool in assessing the QoL in patients of CRS. How to cite this article Gairola P, Bist SS, Mishra S, Agrawal V. Assessment of Quality of Life in Patients of Chronic Rhinosinusitis. Clin Rhinol An Int J 2014;7(3):96-99.


2019 ◽  
Vol 11 (1) ◽  
pp. 9-18
Author(s):  
Abdul Wakhid ◽  
Ana Puji Astuti ◽  
Maya Kurnia Dewi

Logoterapi merupakan terapi untuk menemukan makna positif dibalik sebuah kejadian yang tidak diharapkan. Logoterapi dilaksanakan secara individu maupun berkelompok dalam bentuk konseling dan berorientasi pada pencarian makna hidup individu. Tujuan logoterapi meningkatkan makna pengalaman hidup individu yang diarahkan kepada pengambilan keputusan yang bertanggung jawab. Penelitian ini dilakukan dengan menggunakan rancangan pre-experiment dengan metode pre and post test group, artinya pengumpulan data dilakukan terhadap responden untuk membandingkan kualitas hidup sebelum dan sesudah dilakukan intervensi. Teknik pengambilan sampel dilakukan dengan metode total sampling yaitu pengambilan seluruh sampel dengan tetap memperhatikan kriteria yang telah ditetapkan. Jumlah pasien yang menjalani hemodialisis di RSUD Ungaran sebanyak 21 orang dan di RSUD Ambarawa sebanyak 25 pasien. Analisis data dilakukan dengan menggunakan uji t test dependent. Hasil penelitian didapatkan bahwa dari 46 responden didapatkan rata-rata skor kualitas hidup pasien yang mejalani hemodialisis sebesar 60.22 dengan skor terrendah 55 dan skor tertinggi 69. Bahwa dari 46 responden didapatkan rata-rata skor kualitas hidup pasien yang mejalani hemodialisis sebesar 88.72 dengan skor terrendah 79 dan skor tertinggi 103. Hasil uji statistik dengan uji t test dependent diketahui ada pengaruh logoterapi terhadap kemampuan memaknai hidup pada klien yang menjalani hemodialisis di RSUD Kabupaten Semarang (p value: 0,0001). Saran perlunya peningkatan kemampuan perawat dalam memberikan layanan kesehatan termasuk pemberian atau pemanduan penemuan makna hidup bagi pasien hemodialysis, agar selain dengan hemodialysis, ada faktor internal dari pasien yang dapat dijadikan sebagai motivasi untuk sembuh dari penyakit.   Kata Kunci: Logoterapi, kualitas hidup   IMPROVE THE QUALITY OF LIFE OF PATIENTS WITH RENAL FAILURE WHO UNDERWENT HEMODIALYSIS   ABSTRACT Logotherapy is a therapy to discover the positive meaning behind an unexpected event. Logotherapy is carried out individually or in groups in the form of counseling and oriented to the search for the meaning of individual life. This study aims to improve the quality of life of patients with renal failure who underwent hemodialysis. This research was conducted by using pre-experiment with pre-post test study. The sampling technique was done by the convenience sampling. The number of patients undergoing hemodialysis as many as 46 respondents. Data analysis was done by using test t test dependent. The result showed that from 46 respondents got the mean of quality of life of patients who had hemodialysis 60.22 with lowest score 55 and highest score 69. Whereas from 46 respondents got the mean score of life quality of patients who had hemodialysis 88.72 with score the lowest score 79 and the highest score 103. The result of statistical test with t test dependent is known there is influence of logoterapi to the ability of meaningful life on client who undergo hemodialysis at Semarang Regency hospitals (p value: 0.0001). Advice on the need to improve the nurse's ability to provide health services, including the provision or guidance of the discovery of the meaning of life for hemodialysis patients, in addition to hemodialysis, there are internal factors of the patient that can be used as a motivation to recover from illness.   Keywords: Logotherapy, quality of life, kidney failure.  


2019 ◽  
pp. 195-207

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.


2003 ◽  
Vol 128 (1) ◽  
pp. 17-26 ◽  
Author(s):  
David J. Kay ◽  
Richard M. Rosenfeld

OBJECTIVE: The goal was to validate the SN-5 survey as a measure of longitudinal change in health-related quality of life (HRQoL) for children with persistent sinonasal symptoms. DESIGN AND SETTING: We conducted a before and after study of 85 children aged 2 to 12 years in a metropolitan pediatric otolaryngology practice. Caregivers completed the SN-5 survey at entry and at least 4 weeks later. The survey included 5 symptom-cluster items covering the domains of sinus infection, nasal obstruction, allergy symptoms, emotional distress, and activity limitations. RESULTS: Good test-retest reliability ( R = 0.70) was obtained for the overall SN-5 score and the individual survey items ( R ≥ 0.58). The mean baseline SN-5 score was 3.8 (SD, 1.0) of a maximum of 7.0, with higher scores indicating poorer HRQoL. All SN-5 items had adequate correlation ( R ≥ 0.36) with external constructs. The mean change in SN-5 score after routine clinical care was 0.88 (SD, 1.19) with an effect size of 0.74 indicating good responsiveness to longitudinal change. The change scores correlated appropriately with changes in related external constructs ( R ≥ 0.42). CONCLUSIONS: The SN-5 is a valid, reliable, and responsive measure of HRQoL for children with persistent sinonasal symptoms, suitable for use in outcomes studies and routine clinical care.


2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Hua-hong Wu ◽  
Feng-qi Wu ◽  
Yang Li ◽  
Jian-ming Lai ◽  
Gai-xiu Su ◽  
...  

Abstract Background Juvenile idiopathic arthritis (JIA) may seriously affects patients’ quality of life (QoL), but it was rarely focused and studied in China, so we explore JIA children’s QoL using Chinese version of the PedsQL4.0 Generic Core and PedsQL3.0 Rheumatology Module scale, and analyzed the psychometric properties of these two Scales among Chinese JIA children. Methods We recruited 180 JIA patients from Children's Hospital Affiliated to Capital Institute of Pediatrics and Hebei Yanda Hospital from July 2018 to August 2019. The questionnaires include information related on JIA, PedsQL4.0 generic core and PedsQL3.0 Rheumatology Module scales. According to the disease type, onset age of and course of JIA, we divided them into different groups, then compared the QoL status among different groups. Moreover, we analyzed the reliability and validity of these two scales in these 180 JIA children. Results The mean score of PedsQL4.0 generic core scale on these 180 patients was 82.85 ± 14.82, for these in active period was 72.05 ± 15.29, in remission period was 89.77 ± 9.23; the QoL score of systemic, polyarticular and oligoarticular JIA patients were 77.05 ± 19.11, 84.33 ± 12.46 and 87.12 ± 10.23. The mean score of PedsQL3.0 Rheumatology Module scale on 180 patients was 91.22 ± 9.45, for these in active period was 84.70 ± 11.37, in remission period was 95.43 ± 4.48; the QoL score of systemic, polyarticular and oligoarticular JIA patients were 89.41 ± 11.54, 89.38 ± 10.08 and 93.71 ± 6.92. In the PedsQL 4.0 Generic Core scale, the α coefficients of total scale and almost every dimension are all greater than 0.8 except for the school activity dimension of 0.589; the correlation coefficients of 22 items’ scores (total 23 items) with the scores of dimensions they belong to are greater than 0.5 (maximum value is 0.864), and the other one is 0.406. In PedsQL3.0 Rheumatology Module scale, except for the treatment and worry dimensions of 0.652 and 0.635, the α coefficients of other dimensions and the total scale are all greater than 0.7; the correlation coefficients of all items’ score were greater than 0.5 (the maximum is 0.933, the minimum is 0.515). Conclusions The QoL of Chinese JIA children is worse than their healthy peers, these in active period and diagnosed as systemic type were undergoing worst quality of life. The reliability and validity of PedsQL 4.0 Generic Core and PedsQL3.0 Rheumatology Module scale in Chinese JIA children are satisfactory, and can be used in clinical and scientific researches.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Majid Barati ◽  
Khadijeh Bandehelahi ◽  
Tahereh Nopasandasil ◽  
Hanieh Jormand ◽  
Amir Keshavarzi

Abstract Background Substance-Related Disorders are among the most common social problems caused by using legal and illegal substances. Therefore, this study aimed at determining the quality of life (QoL) and its related factors among women with substance use disorders referring to substance abuse treatment centers in Hamadan, west of Iran. Methods This cross-sectional study was carried out on 120 Iranian female substance users recruited through the census sampling method in 2018. Data collection tools consisted of demographic characteristics and QoL assessment (SF-36). Data were analyzed using SPSS-16 via one-way analysis of variance (ANOVA) and chi-square tests. Results The mean age of the participants was 33.2 ± 12.1 years and the mean score of their total QoL was 35.35 ± 13.5. The results of multiple linear regression analysis indicated that using methamphetamine (β =  − 6.62) was the predictor of QoL in women. Moreover, there was a significant association between QoL and age (p < 0.001), educational level (p = 0.011), and age at first use (p < 0.001). Conclusion According to the results, the participants’ QoL was found to be at an unsatisfactory level. So, it is essential to implement educational help-seeking behavior for treatment and effectiveness educational, as well as holding mental health intervention, school-based substance abuse prevention, and harm reduction programs of substance use. This is especially important in adolescents, young, low-educated, early drug use, and methamphetamine user women, as it may increase the QoL


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