Impairment of lung volume perception and breathing control in hypermobile Ehlers-Danlos syndrome.

Breathing difficulties and exertional dyspnea are frequently reported in hypermobile Ehlers-Danlos syndrome (hEDS); however, they are not clearly explained. An impaired proprioception or the addition of a cognitive task could influence ventilatory control. How can the perception of lung volume be measured? Is lung volume perception impaired in hEDS patients? Is the breathing control impaired during a cognitive task in hEDS patients? A device was developed to assess the accuracy of lung volume perception in patients with hEDS and matched control subjects. In the second step, ventilation was recorded in both groups with and without a cognitive task. Two groups of 19 subjects were included. The accuracy of lung volume perception was significantly (P < 0.01) lower at 30% of inspired vital capacity in patients with hEDS in comparison to the control group, and they showed erratic ventilation (based on spatial and temporal criteria) when performing a cognitive task. These data support the influence of the proprioceptive deficit on ventilatory control in hEDS patients. These elements may help to understand the respiratory manifestations found in hEDS. Future research should focus on this relationship between lung volume perception and ventilation, and could contribute to our understanding of other pathologies or exercise physiology.Trial registration number: ClinicalTrials.gov, NCT05000151.

Self- and Caregiver-Reported Participation, Quality of Life, and Related Mood and Behavior Challenges in People Living With Dystrophinopathies.

BACKGROUND: Previous studies of quality of life (QOL), mood, and behavior in muscular dystrophy focus on caregiver perceptions. This cross-sectional study aims to determine the prevalence of clinically significant mood and behavior problems by both patient and caregiver report and assess relationship between mood/behavior and QOL. METHODS: Forty-one patients with dystrophinopathies (Duchenne muscular dystrophy [DMD] and Becker muscular dystrophy [BMD]) were recruited through the University of Virginia Neuromuscular Clinic. Each patient and caregiver dyad completed questionnaires, including the Behavior Assessment System for Children, 2nd Edition (BASC-2); the Pediatric Quality of Life Inventory for DMD (PedsQL-DMD); Children's Depression Inventory, 2nd Edition; and Screen for Child Anxiety Related Disorders. RESULTS: Persons with dystrophinopathies rated most of their behavior and adaptive skills similarly to the general population. Sixty-four percent of parent assessments rated clinically significant problems on the BASC-2. Worse BASC-2 scores for self- and parent assessments correlated with lower (worse) scores in the Worry and Communication PedsQL domains. Patient-reported QOL scores were higher than parent-reported scores in each domain except Worry. CONCLUSIONS: Individuals with DMD/BMD rate their adaptive skills, behavioral symptoms, externalizing and internalizing problems, and school problems more positively than parents/caregivers. Obtaining self-report data is a worthwhile endeavor that can add value to intervention planning, with the ultimate goal of optimizing QOL.

The Standardized Pressure Injury Prevention Protocol (SPIPP) Checklist 2.0: Content validation.

BACKGROUND: Pressure injury prevention is complex, and rates continue to rise. Checklists reduce human error, improve adherence and standardization with complex processes, focus attention on evidence-based practices derived from clinical practice guidelines and are arranged in a systematic manner to manage the entirety of a patient's risk for preventable outcomes. The original Standardized Pressure Injury Prevention Protocol was created to provide a checklist of pressure injury prevention measures but needed revision and validation. PURPOSE: This article describes the revision and content validity testing of the Standardized Pressure Injury Prevention Protocol Checklist 2.0 that took place in 2022. METHODS: Using the International 2019 Clinical Practice Guideline as a foundation, items were identified/revised, and expert review of the items was obtained. The Standardized Pressure Injury Prevention Protocol 2.0 underwent three rounds of revision by experts from the National Pressure Injury Advisory Panel. A panel of eight national experts completed the content validity survey. Individual item content validity index and total scale content validity index were used to summarize the content validity survey scores. RESULTS: The individual item content validity index scores ranged from 0.5 to 1.0. One item (using a mirror to look at heels) was rated as 0.5, three items were 0.75, 20 items were 0.875 and 23 items were 1.0. The item scoring 0.5 was deleted. Those items scoring 0.75 were revised using the content experts' recommendations. The total scale content validity index was 0.93. CONCLUSION: The Standardized Pressure Injury Prevention Protocol 2.0 provides a standardized checklist of evidence-based items that operationalize a rigorous clinical practice guideline for the prevention of pressure injuries. Early intervention using a standardized approach and evidence-based checklist that can be integrated into the workflow of the direct-care nurse and provider provides the best opportunity for successful and sustainable pressure injury prevention.

Sintomas de disfunções psicológicas após adoecimento crítico / Symptoms of psychological dysfunction after critical illness

Objetivo:estimar a prevalência e identificar fatores associados aos sintomas de ansiedades e/ou depressão. Método: Estudo transversal realizado em um hospital de grande porte. Os dados foram coletados através de ficha sociodemográfica e clínica e escalas para triagem de sintomas de ansiedade e depressão. Nas análises, empregou-se estatística descritiva e modelo de regressão. Resultados: Foram incluídos 83 pacientes, majoritariamente homens, com idade média de 47 anos, cor parda, baixo nível educacional, casados, católicos e com renda mensal inferior a um salário mínimo. 36,3% apresentaram sintomas de ansiedade, 29,5% de depressão e 21% de ambos. Sexo masculino e idade mais jovem associaram-se como proteção para o desenvolvimento de sintomas psicológicos. Conclusão: Pessoas submetidas a cuidados intensivos enfrentam disfunções psicológicas após a alta, portanto, torna-se pertinente a implementação de medidas assistenciais e políticas públicas para o enfrentamento desses prejuízos.(AU)

Objective: To estimate the prevalence and identify factors associated with symptoms of anxiety and/or depression. Method: Cross-sectional study carried out in a large hospital. Data was collected using a sociodemographic and clinical form and scales to screen for symptoms of anxiety and depression. Descriptive statistics and a regression model were used in the analysis. Results: 83 patients were included, mostly men, with an average age of 47, brown, with a low level of education, married, Catholic and with a monthly income of less than one minimum wage. 36.3% had symptoms of anxiety, 29.5% of depression and 21% of both. Male gender and younger age were associated as protective factors for the development of psychological symptoms. Conclusion: People undergoing intensive care face psychological dysfunction after discharge, so it is pertinent to implement care measures and public policies to deal with this damage.(AU)

Objetivo: Estimar la prevalencia e identificar factores asociados a síntomas de ansiedad y/o depresión. Método: Estudio transversal realizado en un gran hospital. Los datos se recogieron mediante un formulario sociodemográfico y clínico y escalas de detección de síntomas de ansiedad y depresión. En los análisis se utilizó estadística descriptiva y un modelo de regresión. Resultados: Fueron incluidos 83 pacientes, en su mayoría hombres, con edad media de 47 años, morenos, con bajo nivel de escolaridad, casados, católicos y con renta mensual inferior a un salario mínimo. 36,3% presentaban síntomas de ansiedad, 29,5% de depresión y 21% de ambos. El sexo masculino y la menor edad se asociaron como factores protectores para el desarrollo de síntomas psicológicos. Conclusión: Las personas sometidas a cuidados intensivos enfrentan disfunción psicológica después del alta, por lo que es pertinente implementar medidas de atención y políticas públicas para enfrentar este daño.(AU)

Multiple Sustainable Benefits of a Rehabilitation Program in Therapeutic Management of Hypermobile Ehlers-Danlos Syndrome: A Prospective and Controlled Study at Short- and Medium-Term.

OBJECTIVE: To evaluate the effects of a 9-week rehabilitation program (RP) for patients with hypermobile Ehlers-Danlos syndrome (hEDS) in the short- and medium-term. DESIGN: Nonrandomized controlled trial with 6 months follow-up. SETTING: Outpatient rehabilitation program. PARTICIPANTS: A referred sample of 36 hEDS patients were assessed for eligibility (N=36), 25 were included, 22 completed the RP and 19 completed the follow-up. INTERVENTIONS: A 9 -week control period without intervention followed by a 9-week RP. MAIN OUTCOME MEASURE: Functional exercise capacity was used as a primary outcome measure. Balance, kinesiophobia, fatigue, pain, quality of life, anxiety, depression, and hyperventilation were measured as secondary outcomes. RESULTS: No significant change was observed during the 9-week control period before the RP. There was a significant improvement immediately after the RP for the functional exercise capacity, balance with eyes closed, fatigue, and quality of life (P<.05). Even more improvements were found 6 weeks after the end of the RP, and there was still an improvement after 6 months in functional exercise capacity, kinesiophobia, depression, hyperventilation, and some components of the quality of life. CONCLUSION: This study supports the effectiveness of an RP as a useful management tool for hEDS patients.

The Hidden Disease: Delayed Diagnosis in Duchenne Muscular Dystrophy and Co-Occurring Conditions.

OBJECTIVE: Early diagnosis of Duchenne muscular dystrophy (DMD) is important for timely intervention to prolong function and preserve quality of life. The prevalence of various neurocognitive disorders is known to be higher in patients with DMD than the general population. In this study, we highlight cases of delayed DMD diagnosis that resulted from misattribution of early motor symptoms to co-occurring neurocognitive conditions. We also investigate the difference in age at DMD diagnosis in the setting of specific co-occurring neurocognitive conditions. METHOD: In this study, we reviewed 40 consecutive patients seen at a Certified Duchenne Care Center, excluding siblings of already-diagnosed patients. We highlight cases of significant delay in DMD diagnosis in the setting of co-occurring neurocognitive diagnoses. We also investigate the association of autism spectrum disorder (ASD), attention-deficit/hyperactivity disorder (ADHD), intellectual disability, and speech/language delay on age of DMD diagnosis. RESULTS: The prevalence of co-occurring neurocognitive diagnoses was 73.1% in patients diagnosed at or after age 5 years vs. 35.7% in those diagnosed before age 5 years. The average age of DMD diagnosis was 6.6 years in patients with any co-occurring neurocognitive diagnoses and 4.9 years in patients without ( p = 0.09). Individual analysis of ASD and ADHD showed significant differences. A greater number of co-occurring conditions were associated with an increased age at DMD diagnosis ( R2 = 0.87, p < 0.001). CONCLUSION: The data suggest an association between the presence of co-occurring neurocognitive conditions and a later age of DMD diagnosis. One cost-effective diagnostic step that can be implemented by all pediatric practitioners is testing serum creatinine kinase (CK) in any child with motor delays or hypotonia, even in the context of other behavioral or cognitive disabilities.

Enhanced photo-reactivity of polyanthracene in the VIS region.

The wavelength-dependent photo-reactivity of polyanthracene was explored upon UV-C and VIS light irradiation. The material was prepared via one-pot chemical oxidation route using FeCl3 as oxidizing agent. A decrease in surface hydrophobicity of a polyanthracene-coated poly(methylmethacrylate) substrate from 109.11° to 60.82° was observed upon UV-C exposure for 48 hrs which was attributed to increase in oxygen content at the surface, as validated by energy dispersive X-ray spectroscopy. Upon exposure to ultraviolet-visible LEDs, photo-dimerization of polyanthracene in solution occurred and was monitored using UV-VIS spectroscopy. The photo-dimer product formation decreased from 381 nm to 468 nm and was found to be higher for the polyanthracene material compared to the monomer anthracene. At 381 nm, photo-dimerization of the material was found to be approx. 4x more efficient than the non-substituted monomer counterpart. Results obtained show that photo-dimerization of polyanthracene will proceed upon exposure with visible light LEDs with reduction in efficiency at longer wavelengths. To compensate, irradiation power of the light source and irradiation time were increased.

Use of quality improvement methodology to improve care of women with hypertensive disease in pregnancy and haemorrhage in Yemen (low-income, high-insecurity setting).

This safety improvement project used quality improvement methods in a Comprehensive Emergency Obstetric and Newborn Care facility in Yemen, managed by an international humanitarian non-governmental organisation. It is responsible for about 6500 deliveries per annum. Following a local review of maternal deaths and serious incidents in 2020-2021, care for women with severe hypertensive disease in pregnancy and postpartum haemorrhage were highlighted as potential areas for improvement. These are also the two most common reasons recorded for maternal mortality in Yemen (and in many low-income countries worldwide). We also wanted to foster an open and honest safety culture within the department that encouraged learning from error.We used an inclusive approach in designing the improvements, with change ideas collated via frontline doctors, midwives and nurses working in the maternity unit. Data were collected via manual audit, and through routinely collected data. We focused on the following measures: number of incidents reported per month, documentation quality of antihypertensive prescriptions, fluid restriction practices in women with severe pre-eclampsia, number of minutes taken to control severe hypertensive episodes, postpartum haemorrhage identification rates and tranexamic acid stock usage. We evaluated the efficacy of team simulation training through precourse and postcourse questionnaires.We found demonstrable improvement in our measures relating to treatment of women with hypertensive disorders of pregnancy, and in postpartum haemorrhage identification and treatment. Team simulation training was a difficult intervention to sustain but was received well with positive results during our test session. Incident reporting showed a temporary increase, but this effect was not sustained.We concluded that quality improvement methodology is a valuable tool even in challenged healthcare settings such as this one, in an active conflict zone. Behaviour change in team culture and safety culture is harder to sustain and demonstrate without a long-term strategy.

Management of Hyperactive Delirium in the Pediatric Intensive Care Unit: Case Series of Three Young Children.

Children in the intensive care unit (ICU) are at high risk of developing delirium, given their underlying disease processes, the adverse effects of treatments and medications, and the stressful, abnormal environment. If prevention and nonpharmacologic measures to treat delirium are unsuccessful, atypical antipsychotics are considered, although they are not approved by Food and Drug Administration for the treatment of pediatric delirium and could have significant adverse side effects. This case report presents three pediatric patients with hyperactive ICU delirium that risked life-threating complications who were successfully treated with short courses of atypical antipsychotic medications.

Carnaval e "a história que a história não conta": uma análise dos sambas de enredo / Carnaval and "the story that history does not tell": an analysis of sambas de enredo

A Estação Primeira de Mangueira foi campeã dos desfiles do Carnaval de 2019 do Rio de Janeiro com o enredo "História para ninar gente grande". Após a verificação de 1.592 sambas de enredo, dos carnavais cariocas de 1939 a 2019, este artigo demonstra que foram apresentadas, em 43 composições anteriores, de escolas diversas, perspectivas semelhantes às que constam no samba-enredo da Verde e Rosa. São obras que exaltam as contribuições de negros, indígenas e populares para a História do Brasil; elencam personalidades relevantes como "outros heróis"; desconstroem a contribuição dos heróis "clássicos" da pátria; e realizam críticas à sociedade brasileira, suas mazelas e seu(s) governo(s). Finalmente, elencamos as oito principais composições que ilustram essas propostas. O objetivo é relacionar a produção cultural carnavalesca às questões do seu tempo, destacando a importância do festejo para a cultura nacional.

Estação Primeira de Mangueira was the champion of Rio de Janeiro's 2019 Carnival Parade with the samba "História para ninar gente grande". After checking 1,592 sambas from the Carnival of Rio de Janeiro, from 1939 to 2019, this article demonstrates that 43 previous compositions from different Escolas de Samba, presented similar perspectives to Mangueira: sambas that praise the contributions of black people, indigenous and popular to the history of Brazil; relevant personalities considered as "other heroes"; an deconstruction of the contribution of the "classic" heroes; and social critics to the Brazilian society, its problems and its government. Finally, we list the eight compositions that better illustrate these proposals. The objective is to relate the carnivalesque cultural production to the issues of its time, highlighting the importance of the celebration for the national culture.

Optimizing Nutrition and Bone Health in Children with Cerebral Palsy.

Children with cerebral palsy (CP) are at risk of growth and nutrition disorders. There are numerous challenges to measure and assess growth and nutritional status in children with CP. Addressing these challenges is imperative, because the consequences of poor growth and malnutrition range from decreased bone density, muscle mass, and quality of life to impacts on intellectual quotient, behavior, attention, social participation, healthcare utilization, and health care costs. In addition to discussing approaches to assess growth and nutrition, this article examines some of the methods of optimizing nutrition and bone health, including when preparing for and recovering from surgery.

Ventilatory Dysfunction in Parkinson's Disease.

In contrast to some other neurodegenerative diseases, little is known about ventilatory dysfunction in Parkinson's disease (PD). To assess the spectrum of ventilation disorders in PD, we searched for and reviewed studies of dyspnea, lung volumes, respiratory muscle function, sleep breathing disorders and the response to hypoxemia in PD. Among the studies, we identified some limitations: (i) small study populations (mainly composed of patients with advanced PD), (ii) the absence of long-term follow-up and (iii) the absence of functional evaluations under "off-drug" conditions. Although there are many reports of abnormal spirometry data in PD (mainly related to impairment of the inspiratory muscles), little is known about hypoventilation in PD. We conclude that ventilatory dysfunction in PD has been poorly studied and little is known about its frequency and clinical relevance. Hence, there is a need to characterize the different phenotypes of ventilation disorders in PD, study their relationships with disease progression and assess their prognostic value.

Patient satisfaction with the laborist model of care in a large urban hospital.

BACKGROUND: The obstetric practice environment is evolving to include more laborists staffing obstetric units, with the hope of improving quality of care and provider satisfaction, yet there are scant data on the impact of a laborist care model on patient satisfaction or delivery outcomes. We sought to assess patient satisfaction after implementation of the laborist model of obstetric care in a large urban teaching hospital. METHODS: Postpartum patients were asked to complete an anonymous survey assessing their satisfaction with care, particularly with regard to the laborist model. Survey questions included rating the overall experience of labor and delivery. All responses were based on a five-point Likert scale. Press-Ganey results were compared from before and after initiation of the model. Descriptive statistics were used to analyze the results. RESULTS: Post-laborist implementation obstetric and delivery experience surveys were collected from 4166 patients, representing a 54% response rate. Ninety percent of patients reported that they were highly satisfied with the overall experience in the labor and delivery unit. A subgroup was asked to rate their experience with the practitioner for their current delivery. Of the 687 respondents, 75% answered excellent, 18% answered good/very good, and 3.4% answered neutral. Eighty-five percent of this subgroup stated that they were informed during prenatal care that they may be delivered by someone other than the practitioner or group that they saw during the pregnancy. Thirty-seven percent (n = 1553) of the total respondents reported that they had had a previous delivery at this institution, 97% (n = 1506) of whom stated "yes" to having their next delivery at this institution. Press-Ganey results were similarly favorable in both time periods (91.3 [n = 811] versus 93.4 [n = 747], P = 0.08). CONCLUSION: Patient satisfaction does not appear to be adversely affected by initiation of the laborist model. Additional research is needed to understand further the implications of this model for provider satisfaction, and maternal and neonatal outcomes.