Background and purpose
Patients with severe, progressive multiple sclerosis (MS) have complex physical and psychosocial needs, typically over several years. Few treatment options are available to ...prevent or delay further clinical worsening in this population. The objective was to develop an evidence‐based clinical practice guideline for the palliative care of patients with severe, progressive MS.
Methods
This guideline was developed using the Grading of Recommendations Assessment, Development and Evaluation methodology. Formulation of the clinical questions was performed in the Patients–Intervention–Comparator–Outcome format, involving patients, carers and healthcare professionals (HPs). No uniform definition of severe MS exists: in this guideline, constant bilateral support required to walk 20 m without resting (Expanded Disability Status Scale score > 6.0) or higher disability is referred to. When evidence was lacking for this population, recommendations were formulated using indirect evidence or good practice statements were devised.
Results
Ten clinical questions were formulated. They encompassed general and specialist palliative care, advance care planning, discussing with HPs the patient’s wish to hasten death, symptom management, multidisciplinary rehabilitation, interventions for caregivers and interventions for HPs. A total of 34 recommendations (33 weak, 1 strong) and seven good practice statements were devised.
Conclusions
The provision of home‐based palliative care (either general or specialist) is recommended with weak strength for patients with severe, progressive MS. Further research on the integration of palliative care and MS care is needed. Areas that currently lack evidence of efficacy in this population include advance care planning, the management of symptoms such as fatigue and mood problems, and interventions for caregivers and HPs.
Mutations in the PARK2 (PRKN) gene are the most common cause of autosomal-recessive (AR) juvenile parkinsonism and young-onset Parkinson's disease (YOPD). >100 different variants have been reported, ...including point mutations, small indels and single or multiple exon copy number variations.
Mutation screening of PARK2 was performed in 225 Serbian PD patients (143 males and 82 females) with disease onset before 50 years and/or positive family history with apparent AR inheritance. All coding regions and their flanking intronic sequences were amplified and directly sequenced. Whole exon multiplications or deletions were detected using Multiple Ligation Probe Amplification (MLPA) method.
We identified 12 PD patients with PARK2 mutations (5.3%). Five patients (2.2%) had biallelic mutations and seven (3.1%) were single mutation carriers. Patients with compound heterozygous mutations had earlier onset of the disease compared to non-carriers (p = 0.005) or heterozygotes (p = 0.001). Other clinical features in mutation carriers were not different compared to non-carriers.
In our cohort, sequence and dosage variants were equally represented in patients, inducing their first symptoms mainly before the age of 30. For efficient genetic testing strategy, patients with early, especially juvenile onset of PD were strong candidates for both dosage and sequence variants screening of PARK2 gene.
•Mutation screening of PARK2 was performed in 225 Serbian PD patients.•Five patients had biallelic mutations and seven were single mutation carriers.•Compound heterozygotes had earlier disease onset than non-carriers or heterozygotes.•Other clinical features did not differ comparing mutation carriers and non-carriers.
Despite the availability of a safe and effective vaccine since 1982, overall coverage of hepatitis B vaccination among healthcare workers (HCWs) has not reached a satisfactory level in many countries ...worldwide. The aim of this study was to estimate the prevalence of hepatitis B vaccination, and to assess the predictors of hepatitis B vaccination status among HCWs in Serbia. Of 380 randomly selected HCWs, 352 (92.6%) were included in the study. The prevalence of hepatitis B vaccination acceptance was 66.2%. The exploratory factor analyses using the vaccination-refusal scale showed that items clustered under 'threat of disease' explained the highest proportion (30.4%) of variance among those declining vaccination. The factor analyses model of the potential reasons for receiving the hepatitis B vaccine showed that 'social influence' had the highest contribution (47.5%) in explaining variance among those vaccinated. In the multivariate adjusted model the following variables were independent predictors of hepatitis B vaccination status: occupation, duration of work experience, exposure to blood in the previous year, and total hepatitis B-related knowledge score. Our results highlight the need for well-planned national policies, possibly including mandatory hepatitis B immunisation, in the Serbian healthcare environment.
Background and purpose
Patient and public involvement in clinical practice guideline development is recommended to increase guideline trustworthiness and relevance. The aim was to engage multiple ...sclerosis (MS) patients and caregivers in the definition of the key questions to be answered in the European Academy of Neurology guideline on palliative care of people with severe MS.
Methods
A mixed methods approach was used: an international online survey launched by the national MS societies of eight countries, after pilot testing/debriefing on 20 MS patients and 18 caregivers, focus group meetings of Italian and German MS patients and caregivers.
Results
Of 1199 participants, 951 (79%) completed the whole online survey and 934 from seven countries were analysed: 751 (80%) were MS patients (74% women, mean age 46.1) and 183 (20%) were caregivers (36% spouses/partners, 72% women, mean age 47.4). Participants agreed/strongly agreed on inclusion of the nine pre‐specified topics (from 89% for ‘advance care planning’ to 98% for ‘multidisciplinary rehabilitation’), and <5% replied ‘I prefer not to answer’ to any topic. There were 569 free comments: 182 (32%) on the pre‐specified topics, 227 (40%) on additional topics (16 guideline‐pertinent) and 160 (28%) on outcomes. Five focus group meetings (three of MS patients, two of caregivers, and overall 35 participants) corroborated the survey findings. In addition, they allowed an explanation of the guideline production process and the exploration of patient‐important outcomes and of taxing issues.
Conclusions
Multiple sclerosis patient and caregiver involvement was resource and time intensive, but rewarding. It was the key for the formulation of the 10 guideline questions and for the identification of patient‐important outcomes.
Abstract Aim Our aim was to estimate the incidence of acute and late genitourinary toxicity in patients treated with three-dimensional conformal radiotherapy (3DCRT) for localised prostate cancer and ...to estimate the possible influence of individual and clinical characteristics. Materials and methods Between September 2009 and September 2013, 225 patients with localised prostate cancer were treated with 3DCRT. Ninety-four patients with an estimated risk of lymph node involvement ≤15%, according to the Roach formula, were evaluated in this study. All patients received a total dose of 72 Gy in 36 fractions. Acute and late genitourinary toxicity were graded according to the European Organization for Research and Treatment of Cancer radiation morbidity scoring scale. Characteristics such as age, smoking status, previous abdominal or pelvic surgery (PAPS), diabetes mellitus and the use of diuretics were analysed as possible predictive factors of toxicity. The median follow-up was 27 months. Results Grade ≥2 acute toxicity during 3DCRT developed in 25 of 94 patients (26.5%). Predictive factors of acute genitourinary toxicity grade ≥2 in the multivariate logistic regression analysis (MVA) were current smoking status ( P = 0.003), PAPS ( P = 0.012) and the use of diuretics ( P = 0.017). The 2 and 3 year cumulative risk of late genitourinary toxicity grade ≥1 was 25.3% and 30.2%, respectively. In the MVA, acute genitourinary toxicity was significantly associated with late genitourinary toxicity ( P = 0.024). Conclusion Current smoking status, PAPS and the use of diuretics have a significant effect on the occurrence of acute genitourinary toxicity grade ≥2. The occurrence of any grade of acute genitourinary toxicity has a significant influence on the development of any grade of late genitourinary toxicity.
Objective
Sexual dysfunction (SD) is a common but often overlooked symptom in multiple sclerosis (MS). The aim of this study was to estimate the frequency, type, and intensity of SD in our patients ...with MS and to investigate its influence on all the domains of quality of life.
Methods
The study population comprised a cohort of 109 patients with MS (McDonald's criteria, 2001). SD was quantified by a Szasz sexual functioning scale. Health-related quality of life was measured by a disease-specific instrument MSQoL-54 (Serbian version).
Results
The presence of at least one symptom of SD was found in about 84% of the men and in 85% of the women. The main complaints in women were reduced libido, difficulties in achieving orgasm, and decreased vaginal lubrication; in men, the main complaints were reduced libido, incomplete erections, and premature ejaculation. In women, statistically significant negative correlations between the presence and level of SD and quality of life domains were reached for all subscales (P < 0.01), except for the Pain subscale (P = 0.112). In men, negative correlations were also observed for all domains, but they were statistically significant for physical health, physical role limitations, social function, health distress, sexual function, and sexual function satisfaction (P < 0.01). We found that the presence of all the analyzed types of sexual problems statistically significantly lowered scores on the sexual function and the sexual function satisfaction subscales in both men and women (P < 0.01). The most prominent impact on both domains was observed for the total loss of erection in men and for anorgasmia in women.
Conclusions
Our results reveal that frequent occurrence of SD in MS patients prominently affects all aspects of their quality of life.
•Nineteen questionnaires from European MS registries were analysed.•Aim and focus as well as number of patients and inclusion criteria vary considerably.•Most of the MS registries collect data within ...common general categories.•There are more pronounced differences regarding specific subcategories.
We present a prospective study of 240 patients with medication overuse headache (MOH) treated with drug withdrawal and prophylactic medications. At 1-year follow-up, 137 (57.1%) patients were without ...chronic headache and without medication overuse, eight (3.3%) patients did not improve after withdrawal and 95 (39.6%) relapsed developing recurrent overuse. Age at time of MOH diagnosis, regular use of benzodiazepines, frequency and Migraine Disability Assessment (MIDAS) score of chronic headache, age at onset of primary headache, frequency and MIDAS score of primary headache, ergotamine compound overuse and daily drug intake were significantly different between successfully and unsuccessfully treated patients. Multivariate analysis determined the frequency of primary headache disorder, ergotamine overuse and disability of chronic headache estimated by MIDAS as independent predictors of treatment efficacy at 1-year follow-up.
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