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.

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.

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.

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.

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.