scholarly journals SAPHO Syndrome and Pustulotic Arthro-Osteitis (PAO)

2021 ◽  
Author(s):  
Mitsumasa Kishimoto ◽  
Yoshinori Taniguchi ◽  
Shigeyoshi Tsuji ◽  
Yoko Ishihara ◽  
Gautam A Deshpande ◽  
...  

ABSTRACTS Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis (SAPHO) syndrome is a rare inflammatory osteo-articular disorder, which encompassed many diseases, including pustulotic arthro-osteitis (PAO). Bone and joint manifestations, including osteitis, synovitis and hyperostosis, are the hallmark of the SAPHO syndrome and affect a variety of regions of the body. Recent GRAPPA survey indicated that more than 80 percent of cases of SAPHO syndrome in Japan were thought to be PAO, originally proposed by Sonozaki et al. in 1981, whereas severe acne was the most commonly reported skin ailment amongst participants with SAPHO syndrome in Israel. SAPHO syndrome is a rare disease and adequate data regarding its prevalence remains unavailable, whereas prevalence of PPP was reported to be 0.12 % in Japan and 10-30% of patients with PPP had PAO. SAPHO syndrome and PAO are predominantly found in patients in the third through fifth decades of life, and a female predominance are seen in both groups. The diagnosis of SAPHO syndrome/PAO is typically made by a rheumatologist or dermatologist. Identification of a variety of the clinical, radiological, and laboratory features outlined, as well as diagnostic criteria, are used to make the diagnosis. Goals for treating patients with SAPHO syndrome/PAO seek to maximize health-related quality of life by improving skin and articular symptoms, preventing structural changes and destruction, and normalizing physical function and social participation. Finally, we review the non-pharmacological (ie: smoking cessation and controlling focal infections) and pharmacological managements including NSAIDs, bisphosphonates, cs DMARDs, bDMARDs, and other treatments for SAPHO syndrome/PAO.

Pulmonology ◽  
2021 ◽  
Author(s):  
Jhonatan Betancourt-Peña ◽  
Juan Carlos Ávila-Valencia ◽  
Diana Milena Diaz-Vidal ◽  
Vicente Benavides-Córdoba

2008 ◽  
Vol 6 (1) ◽  
pp. 106 ◽  
Author(s):  
Nadja Vasiljevic ◽  
Sonja Ralevic ◽  
Jelena Marinkovic ◽  
Nikola Kocev ◽  
Milos Maksimovic ◽  
...  

2020 ◽  
Author(s):  
Gillian Yeowell ◽  
Danielle Burns ◽  
Francis Fatoye

Abstract Background Urea cycle disorders (UCD) are a group of conditions of inborn errors of metabolism, typically presenting neonatally. Excess ammonia builds rapidly within the body, risking hyperammonemic episodes and potentially death. Long-term management of the condition includes restrictive protein consumption, pharmacological interventions and, in extreme cases, liver transplantation. Pharmacological treatments such as sodium benzoate and sodium phenylbuturate have proven effective but not without a multitude of negative attributes including poor taste, elevated volume and associated gastrointestinal discomfort that impacts on health-related quality of life. Glycerol phenylbutyrate (GPB) has recently become a widely available pharmacological treatment with early reports of improved qualities. The following study aims to explore the burden of pharmacological treatment on health-related quality of life in people with a UCD. Results 9 carers of people living with a UCD were interviewed regarding their experiences of pharmacological treatment in relation to their, and their dependent’s health-related quality of life after transitioning to GPB. Three main themes were identified following data analysis: psychological health, physical health and social participation. Carers struggled with anxiety surrounding their dependent’s condition and the relentless battle of administering medication. Medication administration was perceived to have improved since the transition to GPB, alleviating distress for both carer and dependent. Issues involving schooling were commonly described, ranging from difficulties integrating their dependent into mainstream schooling and the impact of treatment on participation in school and extracurricular activities. Based upon participant’s experiences, it could be suggested that some burden had been relieved by the transition to GPB. However, it appeared that the burden would persist despite treatment, owing to the continuing nature of the condition. Conclusions Adhering to a strict pharmacological regime caused immense stress for both carers and dependents, severely impacting on typical social activities such as eating at a restaurant or going on holiday. GPB was perceived to have alleviated some burden in terms of administration. Practitioners should consider these findings when making clinical decisions for UCD patients and the effect of pharmacological treatment on health-related quality of life. Emotional support resources should be made readily available to families to assist with daily living.


Author(s):  
José Ignacio Baile ◽  
Raquel María Guevara ◽  
María José González-Calderón ◽  
José David Urchaga

Excess weight has been associated with numerous psychosocial problems and is considered to be one of the most important health problems of today. The aim of this study is to analyze the relationship between weight status, which is evaluated by means of the body mass index (BMI), and the health-related quality of life (HRQoL) and life satisfaction (LS) variables in Spanish adolescents, as well as to examine whether gender influences this interrelationship. A total of 1197 subjects studying in their 1st and 4th years of high school (mean age: 14.4 years, SD: 1.69) participated in the study by completing the Health Behavior in School-Aged Children (HBSC) questionnaire. Then, the participants were grouped into the following categories: underweight, normal weight, overweight, and obese. The results show that boys have significantly higher HRQoL as well as higher levels of LS. On the other hand, only the obese group shows significantly lower scores in both HRQoL and LS than those in the normal-weight group. The interaction of weight category and gender does not have a significant impact on the variables that have been analyzed (HRQoL or LS).


2009 ◽  
Vol 137 (11-12) ◽  
pp. 684-689
Author(s):  
Dusan Mustur ◽  
Vladislava Vesovic-Potic ◽  
Tatjana Ille ◽  
Dejana Stanisavljevic ◽  
Mihailo Ille

The assessment of the quality of life as a subjective measure of therapeutic intervention outcome appears to be increasingly adopted by different fields of medicine. In contrast to conventional indicators of condition, which are recognized as objective outcomes, the health-related quality of life (HRQoL) focuses on the patient himself. HRQoL has been determined as the perception of the individual of his or her situation in the current culture and value system; it includes wishes, expectations and emotional responses of the individual related to his or her health. Chronic arthritis leads to irreversible structural changes in joints and consequently to impaired physical function and reduced HRQoL. Today there are validated disease specific ('target') questionnaires to assess HRQoL in patients with rheumatoid arthritis and ankylosing spondylitis, but not yet in patients with psoriatic arthritis. A disease specific questionnaire makes easier approach to patients suffering from a specific rheumatic condition; it enables the comparison of HRQoL among patients suffering from the different types of chronic arthritis. The most frequently used questionnaire in the assessment of the HRQoL of patients suffering from chronic arthritis is the generic MOS 36-Item Short-Form Health Survey (SF-36) which is considered as the 'gold standard'. It is a generic questionnaire for the assessment of HRQoL in patients with chronic arthritis. To our knowledge, there are only a few studies focused on the HRQoL of patients suffering from chronic arthritis in Serbia and Montenegro.


2021 ◽  
Vol In Press (In Press) ◽  
Author(s):  
Parnian Parvin ◽  
Parisa Amiri ◽  
Sara Jalali-Farahani ◽  
Mehrdad Karimi ◽  
Mina Moein Eslam ◽  
...  

Background: Maternal characteristics have been known to be associated with parenting practices, which could eventually influence their child’s weight and health-related quality of life (HRQoL). Objectives: This study aimed to assess the direct and indirect associations of maternal emotional states (depression, anxiety, and stress) with body mass index (BMI) and HRQoL in their children. Methods: This study was conducted within the framework of the Tehran lipid and glucose study (TLGS). Participants were the children (n = 231) enrolled in TLGS during 2014 - 2016, who had complete data on maternal emotional states. The body weight and height of children were measured using the standard protocol, and BMI Z-score was determined using Anthroplus. Also, HRQoL in children and emotional states in mothers were assessed using the Iranian version of the pediatric quality of life inventory (PedsQLTM4.0) and the depression, anxiety, and stress scale (DASS-21), respectively. Structural equations modeling (SEM) was used to assess the direct and indirect relations of maternal emotional states with children’s BMI Z-score and HRQoL. Results: Mean age, BMI Z-score, and HRQoL total score in children were 13.8 ± 3.1 years, 0.74 ± 1.5, and 84.7 ± 11.3, respectively. In the mothers, median DASS-21 scores (interquartile ranges) in the three scales of depression, anxiety, and stress were 4 (0 - 10), 6 (2 - 12), and 14 (8 - 20), respectively. Maternal level of education was significantly associated with the DASS-21 score (β = -0.23, 95% CI: -0.37,-0.07). Maternal DASS-21 score was significantly associated with BMI Z-score only in girls (β = 0.25, 95% CI: 0.06, 0.53). Significant determinants of HRQoL in boys were the child’s age (β = -0.21, 95% CI: -0.40, -0.01) and maternal education (β = -0.24, 95%CI: -0.44, -0.02) and emotional state (β = -0.24, 95% CI: -0.44, -0.03). The child’s age (β = -0.33, 95% CI: -0.53, -0.10) and maternal emotional state (β = -0.31, 95% CI: -0.54, -0.08) were significantly associated with HRQoL in girls. Conclusions: Our results indicated that the maternal emotional state was an important determinant of HRQoL in children, regardless of their weight status. Further research is recommended to examine the current hypothesized model in rural and suburban populations, taking into consideration more influential factors.


Sarcoma ◽  
2020 ◽  
Vol 2020 ◽  
pp. 1-9
Author(s):  
Milea J. M. Timbergen ◽  
Winette T. A. van der Graaf ◽  
Dirk J. Grünhagen ◽  
Eugenie Younger ◽  
Stefan Sleijfer ◽  
...  

Purpose. Desmoid-type fibromatosis (DTF) is a rare, nonmetastasising soft tissue tumour. Symptoms, unpredictable growth, lack of definitive treatments, and the chronic character of the disease can significantly impact health-related quality of life (HRQoL). We aimed at identifying the most important HRQoL issues according to DTF patients in two countries, in order to devise a specific HRQoL questionnaire for this patient group. Methods. DTF patients and healthcare providers (HCPs) from the Netherlands and the United Kingdom individually ranked 124 issues regarding diagnosis, treatment, follow-up, recurrence, living with DTF, healthcare, and supportive care experiences, according to their relevance. Descriptive statistics were used to calculate priority scores. Results. The most highly ranked issues by patients (n = 29) were issues concerning “tumour growth,” “feeling that there is something in the body that does not belong there,” and “fear of tumour growth into adjacent tissues or organs” with mean (M) scores of 3.0, 2.9, and 2.8, respectively (Likert scale 1–4). British patients scored higher on most issues compared to Dutch patients (M 2.2 vs. M 1.5). HCPs (n = 31) gave higher scores on most issues compared to patients (M 2.3 vs. M 1.8). Conclusion. This study identified the most relevant issues for DTF patients, which should be included in a DTF-specific HRQoL questionnaire. Additionally, we identified differences in priority scores between British and Dutch participating patients. Field testing in a large, international cohort is needed to confirm these findings and to devise a comprehensive and specific HRQoL questionnaire for DTF patients.


2016 ◽  
Vol 15 (2) ◽  
pp. 301 ◽  
Author(s):  
Juan Carlos Rodriguez Barrera ◽  
Miryam Bastidas ◽  
Giuseppe Genta ◽  
Patricia Olaya-Contreras

Obesity in children affects physical-and mental health and can develop anxiety, depression, as well as other disorders. The objective was to evaluate the Health-Related Quality of Live (HRQOL) in schoolchildren, according to the nutritional status, and to compare our results with those of the European Union. A cross-sectional study was conducted with 679 schoolchildren, 10-14 years, in a public school of Medellin. The KIDSCREEN -27 questionnaire was used to asses HRQOL. Women, especially with obesity, had a less favorable perception of their physical activity, health, mood and family life, while men perceived less social support from friends; they were mocked by their peers and also felt harassed. Our results are consistent with those found in the European study. The Health-Related Quality of Live in schoolchildren is not homogenous, is affected by age, sex and by the cultural constraints about the construction of the body image. Key words: Bullying, obesity, schoolchildren, overweight, quality of life, social acceptance Health Psychology, Quality of Life La obesidad afecta la salud física y mental de niños/niñas. Se evaluó la calidad de vida relacionada con la salud (CVRS) en escolares, de acuerdo al estado nutricional y se compararon los resultados con los de la Unión Europea. Se realizó un estudio transversal con 679 escolares, 10 a 14 años, de una escuela pública de Medellín, Colombia. Se empleó el KIDSCREEN-27 para medir CVRS. Las mujeres, especialmente con obesidad, tuvieron una percepción menos favorable de la actividad física, salud, estado de ánimo y vida familiar, mientras que los hombres perciben más desfavorablemente el apoyo de amigos y la aceptación social, sintiéndose objeto de burla e intimidación. Los resultados son consistentes con los encontrados en el estudio europeo. La percepción de CVRS de los escolares no es homogénea, está afectada por la edad, el sexo y los condicionantes culturales alrededor de la construcción de la imagen corporal. Palabras claves autores Aceptación social, acoso escolar, calidad de vida, obesidad escolar, sobrepeso, Palabras claves descriptores Calidad de Vida, Psicología de la Salud,


Author(s):  
Marlena Krawczyk-Suszek ◽  
Andrzej Kleinrok

Quality of life is an important indicator of the treatment process, lifestyle, and influence of many other factors, both exogenous and endogenous, on the body. Determining the quality of life of healthy people (health-related quality of life (HRQoL), considering the influence of various factors, is important due to the possibility of making subsequent comparative analyses regarding the quality of life of people diagnosed with diseases. In addition, it allows us to identify the most crucial factors influencing the HRQoL in the process of “good aging”. The purpose of the study was to present the HRQoL level of healthy people over 65 years of age. HRQoL was measured in five-year age groups (66–70, 71–75, 76–80, >80 years), considering the analyzed factors. Finally, 1038 healthy people were included in the study. The inclusion criteria were as follows: no diagnosed chronic diseases, no permanent treatment in specialist clinics, and no constant administration of medicaments. A comparative analysis was carried out, assuming a 5% conclusion error. The SF-36 questionnaire assessing the main dimensions of the quality of life was the tool used in the study to assess the HRQoL: the physical component summary (PCS), mental component summary (MCS) and index of life quality (ILQ). The factors significantly differentiating the average level of HRQoL were as follows: gender, place of residence, education, employment status, smoking and physical activity. Relationship status (p > 0.05) was one of the analyzed factors that did not influence the differences in the average level of the perceived HRQoL. More than a twofold greater chance of a higher HRQoL was reported in the group of men under 75 years of age (66–70: OR = 2.01; 71–75: OR = 2.52) compared to the group of women. The same relationship was noted in the case of higher education in respondents up to the age of 80 (66–70: OR = 1.56; 71–75: OR = 2.16; 76–80: OR = 2.74). Smoking by people over 80 years of age significantly increased the chances of a higher HRQoL in each of the dimensions (PCS: OR = 4.09; MCS: OR = 12.64; ILQ: OR = 5.79). Age as a non-modifiable factor significantly differentiates the level of the HRQoL of healthy people over 65 years of age. The results of the conducted study on HRQoL can be helpful when comparing the HRQoL of healthy people with a group of people with chronic diseases.


2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Atefeh Velayati ◽  
Shahideh Jahanian Sadatmahalleh ◽  
Saeideh Ziaei ◽  
Anoshirvan Kazemnejad

Abstract Background The aim of this study was to provide a path model for assessing the direct and/or indirect effects of psychological/behavioral parameters on health-related quality of life among women with vaginismus. Methods A cross-sectional study was conducted on a sample of 236 women with vaginismus disorder attending to sex clinics in Tehran, Iran from April 2017 to March 2018. Data were collected using a demographic questionnaire, the marital satisfaction scale, the hospital anxiety and depression scale, the rosenberg self-esteem scale, the body image concern inventory, the short-form health survey (SF-12) and the female sexual quality of life questionnaire. In addition to descriptive statistical data, the fitness of the proposed model was investigated using path analysis. Results The results of path analysis demonstrated that the final model had a good fit to the data (Chi-Square/degrees of freedom (Normed Chi2) = 2.12, root mean square error of approximation = 0.069, goodness fit index = 0.99, both comparative fit index = 0.99 and Tucker–Lewis index = 0.96). In this model, anxiety and depression significantly predicted health-related quality of life as measured by the SF-12. Conclusions Anxiety and depression are important components in predicting health-related quality of life among those suffering from vaginismus.


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