Amniotic sac reference interval in early pregnancy between 7 and 10 weeks' gestation.

OBJECTIVE: To establish a normal reference interval for amniotic sac measurements between 7 and 10 weeks of gestation and its relative size in relation to the gestational sac and the embryo. METHOD: This was a prospective, cross-sectional study of consecutive women presenting to UCLH Early Pregnancy Unit between August 2022 to June 2023. We included live, normally sited, singleton pregnancies with a normal 20-week anomaly scan. We collected 120 cases per gestational week totaling 360 cases. We performed an inter and intra-observer variability assessment in the measurement of mean ASD in 30 patients. Regression analyses were used to establish reference intervals for GSD to CRL, ASD to CRL, GSD to ASD and GSD:ASD ratio to CRL. The fitted regression line was calculated, along with a 90% prediction interval and the R2 value. RESULTS: There was good interobserver agreement (difference 0.007mm ± 1.105 (95%CI -2.160 to 2.174)) and good intra-observer agreement between Observer A (0.007 ± 1.105 (-2.160 to 2.174)) and Observer B (-0.014 ± 0.919 (-1.814 to 1.786)) in the measurement of mean ASD in 30 patients. Regression analyses showed a highly statistically significant association between each pair of values (all p-values <0.001). There were significant quadratic associations between mean GSD and CRL (R2 = 56%) and mean GSD and ASD (R2 = 60), significant cubic association between ASD and CRL (R2 = 90%) and significant quadratic association between GSD to ASD ratio and CRL (R2 = 68%). The regression equations were used to quantify the values of ASD and GSD to ASD ratios for a range of CRL values and gestational age in days. CONCLUSION: Our study has produced comprehensive reference intervals for amniotic sac size in early pregnancy which could be used in routine clinical practice. This article is protected by copyright. All rights reserved.

Obstetric outcomes in Jehovah's Witnesses: case series over nine years in a London teaching hospital.

OBJECTIVES: The aim of the study is to assess obstetric and fetal outcomes of Jehovah's Witnesses (JWs) mothers in an inner city teaching hospital, as well as to examine the acceptance rates of various blood fractions and blood transfusion alternatives. METHODS: Case series to evaluate the maternal and fetal outcomes of JWs over a nine period between 2013 and 2021. RESULTS: There were 146 pregnancies extracted from our database, of which 10 were early pregnancy losses. Data from 136 deliveries > 24 weeks' gestation were assessed, with a mean maternal age and gestational age of 30.26 (± 5.4) years and 38.7 (± 5.3) weeks, respectively. 57% had normal vaginal deliveries, 8% had instrumental births and 35% had caesarean births. Mean estimated blood loss at caesarean was 575 (± 305.6) mls, while the overall mean estimated loss was 427.8 (± 299.8) mls. Cell salvage was performed in all caesarean sections but autologous transfusion was only necessary for 26%. Consultant presence was documented in 62% of caesarean births. The mean birthweight and 5-min Apgar scores were 3.31 (± 0.05) kg and 9.1 (± 0.09), respectively. There were no maternal deaths or admissions to the adult intensive care unit and the most serious complication was a uterine rupture following a trial of scar, after which the baby required cooling for hypoxic ischaemic encephalopathy. CONCLUSIONS: Pregnant JWs received obstetric care led by senior clinicians, with optimisation of haematinics, minimizing of blood loss at delivery and access to technology such as cell salvage.

How I maximised my training during the COVID-19 pandemic.

The COVID-19 pandemic was declared in March 2020 and London maternity units were among the first in the United Kingdom to report maternal infection and vertical transmission. To manage resources, over half of all Obstetrics and Gynaecology trainees were redeployed to support front-line specialities such as Core Medicine and Accident and Emergency. The vignettes in this article illustrate how three trainees maximised their limited training opportunities in the face of exceptional disruption, lack of surgical training opportunities and workload pressures.

A 12-week lifestyle intervention: effects on fatigue, fear, and nutritional status in children with a Fontan circulation.

Introduction: Children and adolescents with a Fontan circulation are less physically active compared to healthy peers. In the current study, effects of a 12-week lifestyle intervention on fatigue, fears regarding exercise, caloric intake, rest energy expenditure (REE), and body composition were measured in children with a Fontan circulation. Methods: This study was a semi-cross-over randomized controlled trial. The lifestyle intervention consisted of a 12-week high-weight resistance training (three supervised training sessions a week) supported by high-protein diet (>2 g/kg) and tailored recommended caloric intake. Fatigue (measured by the validated PedsQol Multidimensional Fatigue Scale), fears regarding exercise (measured on a fear thermometer), REE (measured using indirect calorimetry), caloric intake and body composition using air displacement plethysmography, and four-skinfold method were measured before and after the intervention and control period. Results: Twenty-seven pediatric Fontan patients, median age 12.9 years (IQR: 10.5-16.2), of the included 28 patients successfully completed the program. Before training, both child- and parent-reported levels of fatigue were significantly worse on all domains (general, sleep/rest, and cognitive fatigue) compared to healthy peers. After training, parent-reported fatigue significantly improved on the general and cognitive fatigue domains [effect size +16 points (7-25), p < 0.001, and +10 points (2-17), p = 0.015, compared to the control period]. Before training, fear regarding exercise scored on the fear thermometer was low for both children and parents (median score 1 and 2, respectively, on a scale of 0-8). After training, child-reported fear decreased further compared to the control period [effect size -1.4 points (-2.3 to -0.6), p = 0.001]. At baseline, children had increased REE +12% compared to reference values, which did not change after exercise. Children ate an average of 637 calories below recommended intake based on REE, caloric deficit became smaller after the intervention, and protein intake increased compared to the control period [-388 calories (-674 to -102), p = 0.008, and +15 g (0.4-30), p = 0.044]. Body fat percentage did not change significantly. Conclusion: A 12-week lifestyle intervention improved parent-reported fatigue symptoms in the children, further decreased child-reported fears, and increased caloric and protein intake.

Diagnostic accuracy of nerve excitability and compound muscle action potential scan derived biomarkers in amyotrophic lateral sclerosis.

BACKGROUND AND PURPOSE: The lack of reliable early biomarkers still causes substantial diagnostic delays in amyotrophic lateral sclerosis (ALS). The aim was to assess the diagnostic accuracy of a novel electrophysiological protocol in patients with suspected motor neuron disease (MND). METHODS: Consecutive patients with suspected MND were prospectively recruited at our tertiary referral centre for MND in Utrecht, The Netherlands. Procedures were performed in accordance with the Standards for Reporting of Diagnostic Accuracy. In addition to the standard diagnostic workup, an electrophysiological protocol of compound muscle action potential (CMAP) scans and nerve excitability tests was performed on patients' thenar muscles. The combined diagnostic yield of nerve excitability and CMAP scan based motor unit number estimation was compared to the Awaji and Gold Coast criteria and their added value was determined. RESULTS: In all, 153 ALS or progressive muscular atrophy patients, 63 disease controls and 43 healthy controls were included. Our electrophysiological protocol had high diagnostic accuracy (area under the curve [AUC] 0.85, 95% confidence interval [95% CI] 0.80-0.90), even in muscles with undetectable axon loss (AUC 0.78, 95% CI 0.70-0.85) and in bulbar-onset patients (AUC 0.85, 95% CI 0.73-0.95). Twenty-four of 33 (73%) ALS patients who could not be diagnosed during the same visit were correctly identified, as well as 8/13 (62%) ALS patients not meeting the Gold Coast criteria and 49/59 (83%) ALS patients not meeting the Awaji criteria during this first visit. CONCLUSIONS: Our practical and non-invasive electrophysiological protocol may improve early diagnosis in clinically challenging patients with suspected ALS. Routine incorporation may boost early diagnosis, enhance patient selection and generate baseline measures for clinical trials.

Physical Training and Healthy Diet Improved Bowel Symptoms, Quality of Life, and Fatigue in Children With Inflammatory Bowel Disease.

OBJECTIVES: Physical activity programs have been suggested as adjunctive therapy in adult inflammatory bowel disease (IBD) patients. We assessed the effects of a 12-week lifestyle intervention in children with IBD. METHODS: This study was a randomized semi-crossover controlled trial, investigating a 12-week lifestyle program (3 physical training sessions per week plus personalized healthy dietary advice) in children with IBD. Endpoints were physical fitness (maximal and submaximal exercise capacity, strength, and core stability), patient-reported outcomes (quality of life, fatigue, and fears for exercise), clinical disease activity (fecal calprotectin and disease activity scores), and nutritional status (energy balance and body composition). Change in maximal exercise capacity (peak VO 2 ) was the primary endpoint; all others were secondary endpoints. RESULTS: Fifteen patients (median age 15 [IQR: 12-16]) completed the program. At baseline, peak VO 2 was reduced (median 73.3% [58.8-100.9] of predicted). After the 12-week program, compared to the control period, peak VO 2 did not change significantly; exercise capacity measured by 6-minute walking test and core-stability did. While medical treatment remained unchanged, Pediatric Crohn's Disease Activity Index decreased significantly versus the control period (15 [3-25] vs 2.5 [0-5], P = 0.012), and fecal calprotectin also decreased significantly but not versus the control period. Quality of life (IMPACT-III) improved on 4 out of 6 domains and total score (+13 points) versus the control period. Parents-reported quality of life on the child health questionnaire and total fatigue score (PedsQoL Multidimensional Fatigue Scale) also improved significantly versus the control period. CONCLUSIONS: A 12-week lifestyle intervention improved bowel symptoms, quality of life, and fatigue in pediatric IBD patients.

Physical training and high-protein diet improved muscle strength, parent-reported fatigue, and physical quality of life in children with Pompe disease.

Exercise has proven to be an effective adjuvant treatment to enzyme replacement therapy (ERT) in mildly affected adult Pompe patients. The aim of this study was to investigate the effects of a 12-week tailored lifestyle intervention, consisting of physical training and a high protein diet (2 grams/kg), in children with Pompe disease. This randomized controlled semi-crossover trial investigated the effects of a lifestyle intervention on the primary outcome: exercise capacity. Secondary outcomes were: muscle strength, core stability, motor function, physical activity levels, quality of life, fatigue, fear of exercise, caloric intake, energy balance, body composition, and safety. Fourteen Pompe patients with a median age of 10.6 [IQR: 7.2-14.5], of whom six classic infantile patients, participated in the lifestyle intervention. At baseline, patients had a lower exercise capacity compared to healthy peers (median 70.3% [IQR: 54.8%-98.6%] of predicted). After the intervention, absolute Peak VO2 improved significantly (1279 mL/min [1012.5-2006] vs. 1352 mL/min [1101.5-2069], p = 0.039), but not compared to the control period. Muscle strength of the hip flexors, hip abductors, elbow extensors, neck extensors, knee extensors, and core stability improved significantly compared to the control period. Children reported a significant increase on the change in health domain of quality of life, parents reported significantly better scores on the quality of life domains: physical functioning, change in health, family cohesion, and fatigue. A 12-week tailored lifestyle intervention for children with Pompe disease seemed safe and led to improvements in muscle strength, core stability, quality of life, and parent-reported fatigue. Pompe patients with a stable disease trajectory seemed to benefit the most from the intervention.

Effects of enzyme replacement therapy on cardiac function in classic infantile Pompe disease.

OBJECTIVE: Patients with classic infantile Pompe disease are born with a hypertrophic cardiomyopathy, which resolves after treatment with Enzyme replacement therapy (ERT). We aimed to assess potential deterioration of cardiac function over time using myocardial deformation analysis. METHODS: Twenty-seven patients treated with ERT were included. Cardiac function was assessed at regular time intervals (before and after start with ERT) using conventional echocardiography and myocardial deformation analysis. Separate linear mixed effect models were used to asses temporal changes within the first year and the long-term follow-up period. Echocardiograms of 103 healthy children served as controls. RESULTS: A total of 192 echocardiograms were analyzed. Median follow-up was 9.9 years (IQR: 7.5-16.3). Mean LVMI before start of ERT was increased 292.3 g/m2 (95% CI: 202.8-381.8, mean Z-score + 7.6) and normalized after 1 year of ERT 87.3 g/m2 (CI: 67.5-107.1, mean Z-score + 0.8, p < 0.001). Mean shortening fraction was within normal limits before start of ERT, up to 22 years of follow-up. Cardiac function measured by RV/LV longitudinal, and circumferential strain was diminished before start of ERT, but normalized (<-16%) within 1 year after start of ERT, and all remained within normal limits during follow-up. Only LV circumferential strain gradually worsened in Pompe patients (+0.24%/year) during follow-up compared to controls. LV longitudinal strain was diminished in Pompe patients, but did not change significantly over time compared to controls. CONCLUSION: Cardiac function, measured using myocardial deformation analysis, normalizes after start of ERT, and seems to remain stable over a median follow-up period of 9.9 years.

The Importance of Consumer Authorities for the Production and Maintenance of Trust and Social Capital in Consumer Markets.

Trust is a valuable resource that varies between countries. This paper suggests that consumers' trust in retailers and service providers, facilitating interactions and transactions between sellers and buyers in impersonal markets, is best understood as generalized trust. The paper is based on 28 037 respondents' evaluations of consumer conditions in 30 European countries. The material reveals large country-to-country variations in the percentages of residents who trust public authorities to protect their consumer rights. Moreover, there are large differences in the percentages who trust retailers and service providers to respect their rights as consumers. A multilevel path analysis supports the paper's main hypothesis that fair and effective consumer authorities enhance generalized trust in the markets. The analyses also demonstrate that fair and effective consumer institutions contribute to more equality in the markets. It is argued that consumer markets are important arenas for the maintenance and production of trust and social capital. And that generalized trust produced in markets will probably extend to, and be valuable for, the wider society.

Salivary immune markers are not associated with self-reported childhood maltreatment or psychopathology in adults.

BACKGROUND: Psychological stress has repeatedly been found to be associated with pro-inflammatory markers in blood, and neuro-inflammation may play a role in the development of psychopathology after early life stress. Salivary immune testing is a novel method to non-invasively assess immune functioning. We examined a large range of salivary immune markers in relation to self-reported childhood maltreatment and psychopathology in an adult sample. METHODS: Participants (N = 118, 51% female, mean age = 46.6 yrs, range 22-64) were drawn from a cross-sectional three-generation study, and supplied 2 ml of saliva via passive drool. They reported on childhood maltreatment experiences and on psychopathological symptoms in the last 6 months. Hair cortisol was additionally assessed in a subsample (n = 68). Levels of IL1ß, IL6, IL8, IFNγ, TNFα, tIgE, sIgA, FLCƛ, and FLCƙ were assessed. RESULTS: Linear mixed model analyses showed that several salivary immune markers were associated with age (sIgA and IgE), BMI (sIgA, IL1ß, and IL6), sex (FLCs and IgE), and bad health (IL6, IL8, TNFα). No associations with (anti-inflammatory) medication use or oral health problems were found. Notably, no associations between the immune markers and self-reported childhood maltreatment, psychopathology, or hair cortisol were found. CONCLUSIONS: Salivary immune measures were found to be sensitive to individual differences in age, sex, health and BMI. However. in the current sample there was no indication of inflammation in relation to chronic psychological stress. Larger studies, including participants with higher stress levels, are needed to further examine associations between salivary immune markers and psychological stress.

Composite endpoint for ALS clinical trials based on patient preference: Patient-Ranked Order of Function (PROOF).

BACKGROUND: Patients with amyotrophic lateral sclerosis (ALS) show considerable variation in symptoms. Treatments targeting an overall improvement in symptomatology may not address what the majority of patients consider to be most important. Here, we propose a composite endpoint for ALS clinical trials that weighs the improvement in symptoms compared with what the patient population actually wants. METHODS: An online questionnaire was sent out to a population-based registry in The Netherlands. Patients with ALS were asked to score functional domains with a validated self-reported questionnaire, and rank the order of importance of each domain. This information was used to estimate variability in patient preferences and to develop the Patient-Ranked Order of Function (PROOF) endpoint. RESULTS: There was extensive variability in patient preferences among the 433 responders. The majority of the patients (62.1%) preferred to prioritise certain symptoms over others when evaluating treatments. The PROOF endpoint was established by comparing each patient in the treatment arm to each patient in the placebo arm, based on their preferred order of functional domains. PROOF averages all pairwise comparisons, and reflects the probability that a patient receiving treatment has a better outcome on domains that are most important to them, compared with a patient receiving placebo. By means of simulation we illustrate how incorporating patient preference may upgrade or downgrade trial results. CONCLUSIONS: The PROOF endpoint provides a balanced patient-focused analysis of the improvement in function and may help to refine the risk-benefit assessment of new treatments for ALS.

Severe chickenpox disease and seroprevalence in Sweden - implications for general vaccination.

OBJECTIVES: To describe the current panorama of severe chickenpox disease and seroprevalence in Sweden, as a basis for the approaching decision on universal vaccination. METHODS: Patients discharged with an International Classification of Diseases 10th revision-code for chickenpox (B01-B01.9) in eight pediatric and infectious diseases departments in Stockholm and Gothenburg in 2012-2014 were included in the study and their medical charts were reviewed. Further, residual serum samples collected from 11 laboratories across Sweden were analyzed for varicella zoster IgG-antibodies to investigate age-specific seroprevalence. RESULTS: A total of 218 children and 46 adults were included in this hospital-based study; 87.2% of children and 63.0% of adults had complications. An underlying condition was not associated with an increased risk of complication. Dehydration (31.7%), bacterial skin infections (29.8%) and neurological involvement (20.6%) were the most frequent complications in children. Among adult cases, 63% were born abroad. The seroepidemiological analysis included 957 patient samples. Seroprevalence was 66.7% at 5 years and 91.5% at 12 years. Infants and adolescents/adults were overrepresented among admitted patients compared to seroprevalence data. CONCLUSIONS: Half of all complications in hospitalized chickenpox cases were seen in previously healthy children, which supports universal childhood vaccination. Adult migrants was a risk group for chickenpox hospitalization. Age-specific seroprevalence was similar to neighboring countries.

Inhibition of HERV-K (HML-2) in amyotrophic lateral sclerosis patients on antiretroviral therapy.

Reactivation of Human Endogenous Retrovirus K (HERV-K), subtype HML-2, has been associated with pathophysiology of amyotrophic lateral sclerosis (ALS). We aimed to assess the efficacy of antiretroviral therapy in inhibiting HML-2 in patients with ALS and a possible association between the change in HML-2 levels and clinical outcomes. We studied the effect of 24-weeks antiretroviral combination therapy with abacavir, lamivudine, and dolutegravir on HML-2 levels in 29 ALS patients. HML-2 levels decreased progressively over 24 weeks (P = 0.001) and rebounded within a week of stopping medications (P = 0.02). The majority of participants (82%), defined as "responders", experienced a decrease in HML-2 at week 24 of treatment compared to the pre-treatment levels. Differences in the evolution of some of the clinical outcomes could be seen between responders and non-responders: FVC decreased 23.69% (SE = 11.34) in non-responders and 12.71% (SE = 8.28) in responders. NPI score decreased 91.95% (SE = 6.32) in non-responders and 53.05% (SE = 10.06) in responders (P = 0.01). Thus, participants with a virological response to treatment showed a trend for slower progression of the illness. These findings further support the possible involvement of HML-2 in the clinical course of the disease.

The management of women with thoracic endometriosis: a national survey of British gynaecological endoscopists.

OBJECTIVES: This study evaluates current national opinions on screening, diagnosis, and management of thoracic endometriosis. BACKGROUND: Thoracic endometriosis is a rare but serious condition with four main clinical presentations: pneumothorax, haemoptysis, haemothorax, and pulmonary nodules. There are no specialist centres in the United Kingdom despite growing patient desire for recognition, investigation, and treatment. METHODS: We distributed a multiple-choice email survey to senior members of the British Society for Gynaecological Endoscopy. Descriptive statistics were used to present the results. Results: We received 67 responses from experienced clinicians having provided over 800 combined years of endometriosis patient care. The majority of respondents managed over 100 endometriosis patients annually, for more than five years. Over one third had never managed a patient with symptomatic thoracic endometriosis; just 9% had managed more than 30 cases over the course of their career. Screening varied by modality with only 4% of clinicians always taking a history of respiratory symptoms while 69% would always screen for diaphragmatic endometriosis during laparoscopy. The management of symptomatic thoracic endometriosis varied widely with the commonest treatment being surgery followed by hormonal therapies. Regarding management, 71% of respondents felt the team should comprise of four or more different specialists, and 56% believed care should be centralised either regionally or nationally. CONCLUSIONS: Thoracic endometriosis is poorly screened for amongst clinicians with varied management lacking a common diagnostic or therapeutic pathway in the United Kingdom. Specialists expressed a preference for women to be managed in a large multidisciplinary team setting at a regional or national level.

Improved Accuracy of Cardiac Tissue-Level Simulations by Considering Membrane Resistance as a Cellular-Level Optimization Objective.

Cardiac cellular models are utilized as the building blocks for tissue simulation. One of the imprecisions of conventional cellular modeling, especially when the models are used in tissue-level modeling, stems from the mere consideration of cellular properties (e.g., action potential shape) in parameter tuning of the model. In our previous work, we put forward an accurate framework in which membrane resistance (Rm) reflecting inter-cellular characteristics, i.e., electrotonic effects, was considered alongside cellular features in cellular model fitting. This paper, for the first time, examines the hypothesis that considering Rm as an additional optimization objective improves the accuracy of tissue-level modeling. To study this hypothesis, after cellular-level optimization of a well-known model, source-sink mismatch configurations in a 2-dimensional model are investigated. The results demonstrate that including Rm in the optimization protocol yields a substantial improvement in the relative error of the critical transition border which is defined as the minimum window size between source and sink that wave propagates. Model developers can utilize the proposed concept during parameter tuning to increase the accuracy of models.

Associations between illness cognitions and health-related quality of life in the first year after diagnosis of amyotrophic lateral sclerosis.

OBJECTIVE: To describe illness cognitions among patients with amyotrophic lateral sclerosis (ALS), to study cross-sectional associations between illness cognitions and health-related quality of life (HRQoL) and to study the predictive value of illness cognitions measured shortly after the diagnosis for HRQoL at follow-up. METHODS: Prospective longitudinal design. We administered Self-report questionnaires at study onset (n = 72) and follow-up (n = 48). Median follow-up period was 10.0 months. At baseline median ALS Functional Rating Scale-Revised was 43, median time since onset of symptoms was 13.6 months, 79% of patients presented with spinal onset. Illness cognitions Helplessness, Acceptance and Disease Benefits were measured with the Illness Cognitions Questionnaire (ICQ) and HRQoL with the ALS Assessment Questionnaire (ALSAQ-40). Correlational and regression analyses were used. RESULTS: Patients experienced more Helplessness at follow-up. We found no significant changes in Acceptance or Disease Benefits at follow-up. In cross-sectional analyses, Helplessness was independently related to worse HRQoL at baseline (ß = 0.44; p = .001) and Acceptance and Disease Benefits were independently related to worse HRQoL at follow-up (ß = -0.17, p = .045) and (ß = -0.186, p = .03 respectively). Longitudinal analyses showed that, adjusted for disease severity at baseline, Helplessness at baseline was a predictor of worse HRQoL at follow-up (ß = 0.43; p = .006). None of the illness cognitions were a significant predictor of HRQoL with adjustment for baseline HRQoL. CONCLUSION: Helplessness was independently associated with HRQoL in the cross-sectional and longitudinal analyses. These results can help us identify patients shortly after diagnosis who might benefit from psychological interventions.

Health-promoting factors in higher education for a sustainable working life - protocol for a multicenter longitudinal study.

BACKGROUND: The World Health Organization has highlighted the importance of health promotion for health service providers in order to ensure sustainable working life for individuals involved in providing health services. Such sustainability begins when students are preparing to manage their own future health and welfare in working life. It has been suggested that universities, employees and trainee health professionals should adopt or follow a salutogenic approach that not only complements the providing of information on known health risks but also favors health promotion strategies. This paper describes the study design and data collection methods in a planned study aiming to explore health-promoting factors for a sustainable working life among students in higher education within healthcare and social work. METHODS: This protocol describes a multicenter longitudinal study involving Swedish students on higher education programs in the healthcare and social work sectors. In 2018, the study invited students on seven education programs at six universities to participate. These programs were for qualification as: biomedical laboratory scientists (n = 121); dental hygienists (n = 87); nurses (n = 1411); occupational therapists (n = 111); physiotherapists (n = 48); radiographers (n = 60); and, social workers (n = 443). In total, 2283 students were invited to participate. Participants completed a baseline, a self-reported questionnaire including six validated instruments measuring health-promoting factors and processes. There are to be five follow-up questionnaires. Three while the students are studying, one a year after graduating, and one three years after graduating. Each questionnaire captures different health-promoting dimensions, namely: health-promoting resources (i.e. sense of coherence); occupational balance; emotional intelligence; health and welfare; social interaction; and work and workplace experiences/perceptions. DISCUSSION: This study focuses on the vastly important aspect of promoting a sustainable working life for healthcare and social work employees. In contrast to previous studies in this area, the present study uses different, validated instruments in health promotion, taking a salutogenic approach. It is hoped that, by stimulating the implementation of new strategies, the study's findings will lead to education programs that prepare students better for a sustainable working life in healthcare and social work.

Bulbar Problems Self-Reported by Children and Adults with Spinal Muscular Atrophy.

BACKGROUND: Spinal muscular atrophy (SMA) is hereditary motor neuron disorder, characterised by the degeneration of motor neurons and progressive muscle weakness. It is caused by the homozygous loss of function of the survival motor neuron (SMN) 1 gene. SMA shows a wide variability of disease severity. OBJECTIVE: To investigate self-reported bulbar problems in patients with SMA, and their relationship to age, functional motor scores and active maximum mouth opening. METHODS: We used the Diagnostic List of Dysphagia and Dysarthria in (pediatric) patients and relevant recent clinical data from the national SMA database. RESULTS: The 118 included patients with SMA frequently reported jaw problems (34%), fatigue associated with mastication (44%), choking (56%) and intelligibility problems (27%). Jaw, mastication and swallowing problems frequently occurred in combination with each other. There was an increase of reported bulbar problems in patients with SMA type 3a, older than 30 years of age, compared to younger patients of this SMA type.The Hammersmith Functional Motor Scale Expanded scores showed a negligible correlation with jaw and mastication problems, a low negative correlation with swallowing problems and a moderate negative correlation with intelligibility problems. Reduced mouth opening showed a significant, but low correlation with bulbar complaints in patients with SMA type 2. CONCLUSIONS: Fatigue associated with mastication and swallowing problems were frequently reported complaints. Patients 30 years and older with milder forms of SMA showed an increase of self-reported bulbar problems.