Descripción del crecimiento del fémur proximal mediante tomografía en pacientes pediátricos sin enfermedad de cadera / Description of proximal femoral growth in pediatric patients without hip disorders using tomography

Objetivo: Describir el crecimiento femoral proximal en una población latinoamericana a través del desplazamiento femoral, el ángulo cervico-diafisario, la longitud del cuello femoral y el diámetro de la cabeza femoral evaluados con tomografía computarizada en pacientes pediátricos sin enfermedad de cadera. Materiales y métodos: Estudio retrospectivo que evaluó imágenes de tomografía computarizada de caderas de pacientes sanos <18 años. Se tomaron las siguientes medidas: desplazamiento femoral, longitud del cuello femoral, diámetro de la cabeza femoral y ángulo cervico-diafisario. Resultados:El desplazamiento femoral aumenta 1,96 mm hasta los 12.5 años, y desde los 12.5 hasta los 16 años, aumenta 1,2 mm. Se constató un aumento lineal del crecimiento de la longitud del cuello femoral. El ángulo cervico-diafisario disminuyó progresivamente hasta los 12 años. A partir de ese momento, la curva se aplanó. Se observó un aumento anual del diámetro de la cabeza femoral de 1,56 mm hasta los 13 años y de 0,62 mm anuales, en adelante. Conclusiones: Las medidas descritas en este estudio son esenciales para el seguimiento, el diagnóstico o el abordaje conductual en múltiples cuadros articulares de cadera durante el crecimiento. Se expone la necesidad de realizar estudios más amplios para establecer rangos de normalidad en la población local con las herramientas tecnológicas disponibles, que fundamenten una referencia para la restauración de la anatomía en la cirugía de preservación.Palabras clave: Cabeza femoral; cuello femoral; desarrollo humano. Nivel de Evidencia :IV

Introduction: This study aims to perform a descriptive analysis of proximal femoral growth in a Latin-American population through femoral offset, neck-shaft angle, femoral neck length, and femoral head diameter evaluated through computed tomography in pediatric patients without hip pathology. Materials and methods: Retrospective study evaluating CT images of the hips of healthy patients under 18 years. The following measurements were taken by a trained orthopedist: femoral offset, femoral neck length, femoral head diameter, and neck-shaft angle. Results:Femoral offset increases by 1.96 mm until age 12.5. From 12.5 to 16 years of age, it increases by 1.2 mm. A constant rise in the growth of the femoral neck length was found. The neck-shaft angle presented a progressive decrease until age 12. After that point, the curve flattened. An increase in femoral head diameter of 1.56 mm per year was observed until age 13 and then 0.62 mm per year. Conclusions: The measurements evaluated in this study are essential for the diagnosis, follow-up, and treatment approach in hip pathologies during growth. More extensive research is needed to define normal ranges that will serve as a baseline for anatomy restoration in hip joint preservation surgery. Level of Evidence: IV

The caregiver's perspective on end-of-life inpatient palliative care: a qualitative study.

INTRODUCTION: Understanding patient and caregiver experience is key to providing person-centered care. The palliative care approach includes holistic assessment and whole-person care at the end of life, that also involves the patient's family and loved ones. The aim of this study was to describe the way that family caregivers experienced patients' deaths during their loved ones' last hospital admission, comparing inpatient palliative care (PCU) and non-palliative care (Non-PCU) units. METHODS: A qualitative case study approach was implemented. Family caregivers of terminally ill patients admitted to the Infanta Elena Hospital (Madrid, Spain) between 2016 and 2018 were included using purposeful sampling. Eligible caregivers were first-degree relatives or spouses present during the patient's last hospital admission. Data were collected via in-depth interviews and researchers' field notes. Semi-structured interviews with a question guide were used. A thematic inductive analysis was performed. The group of caregivers of patients admitted to the PCU unit and the group of caregivers of patients admitted to Non-PCU were analyzed separately, through a matrix. RESULTS: In total 24 caregivers (12 from the PCU and 12 from Non-PCU units) were included. Two main themes were identified: caregivers' perception of scientific and technical appropriateness of care, and perception of person-centred care. Scientific appropriateness of care was subdivided into two categories: diagnostic tests and treatment, and symptom control. Perception of person-centred care was subdivided as: communication, emotional support, and facilitating the farewell process. Caregivers of patients admitted to a PCU unit described their experience of end-of-life care as positive, while their Non-PCU unit counterparts described largely negative experiences. CONCLUSIONS: PCU provides a person-centered approach to care at the end of life, optimizing treatment for patients with advanced disease, ensuring effective communication, establishing a satisfactory professional relationship with both patients and their loved ones, and facilitating the farewell process for family caregivers.

This article describes a qualitative case study focusing on family caregivers' perception of end-of-life care during their loved ones' dying process in their last hospital admission. Differences were observed between palliative care and non-palliative care groups regarding the perception of scientific appropriateness of care and person-centered nature of care as reported by caregivers.

End of Life Cost Savings in the Palliative Care Unit Compared to Other Services.

CONTEXT: Hospital deaths carry a significant healthcare cost that has been confirmed to be lower when palliative care units (PCUs) are available. OBJECTIVES: To compare the last admission hospital health care cost of dying in a first-level hospital between the PCU and the rest of the hospital services. METHODS: A retrospective, comparative, observational study evaluating costs from the payer perspective on treatments and diagnostic-therapeutic tests performed on patients who die in first-level hospital, comparing whether they were treated by the PCU or another unit (Non-PCU). Patients with a mortality risk >2 were included according to the Severity of Illness Index (SOI) and Risk of Mortality (MOR). All cost express in €, median per patient and interquartile range (IQR). RESULTS: From 1,833 patients who died, 1,389 were included, 442 (31.1%) treated by PCU and 928 (68.9%) Non-PCU. Statistical differences were found for the last admission total cost (€262.8 (€470.1) for PCU versus €515.3 (€980.48) in Non-PCU), daily total cost (€74.27 (€127.4) vs €115.8 (€142.4) Non-PCU). Savings were maintained when the sample was broken down by diagnosis-related group (DRG) and a multivariate analysis was performed to determine how the different patients baseline characteristics between PCU and Non-PCU patients influenced the results obtained. CONCLUSIONS: Data from this study show that cost is significantly lower when the patients are treated by a PCU during their last hospital stay when they pass away.

"This Project Helped Me to Grow": Experiences of Teaching Palliative Care Using the EDUPALL Project Medical Undergraduate Curriculum.

Background: The WHO has proclaimed that palliative care (PC) should be integrated as a routine element of all undergraduate medical and nursing education. The EDUPALL Erasmus+project produced a PC curriculum for undergraduate medical education based on the European Association for Palliative Care (EAPC) recommendations for undergraduate training. This was tested in four Romanian Faculties of Medicine: Universities of Transilvania, Iasi, Targu Mures, and Timisoara. The aim of this study is to describe teachers' satisfaction and views on the effectiveness of the EDUPALL curriculum and supporting learning materials. Methods: We conducted nine semistructured interviews with teachers involved in EDUPALL implementation in their universities. Interviews were transcribed and collected data underwent thematic analysis. Kirkpatrick's four-level evaluation model of training was employed to synthesize the outcomes into final categories of reaction, learning, behavior, and results. Results: Data were categorized against Kirkpatrick's four levels as follows: Level 1 (Reaction) EDUPALL curriculum-a good standard with achievable goals; Level 2 (Learning) Personal appraisal and development needs of the teaching faculty; Level 3 (Behavior) Application of competencies and student feedback; and Level 4 (Results): Faculty- and country-level Impact of the EDUPALL project. Conclusion: EDUPALL curriculum is a good and adaptive model to teach PC at Faculties of Medicine, considered by teachers as a way of bridging an existing training gap for medical students in building essential competencies in symptom management, communication, spirituality, and self-awareness.

Corrigendum: Magnetization Transfer Ratio in Lower Limbs of Late Onset Pompe Patients Correlates With Intramuscular Fat Fraction and Muscle Function Tests.

[This corrects the article DOI: 10.3389/fneur.2021.634766.].

Magnetization Transfer Ratio in Lower Limbs of Late Onset Pompe Patients Correlates With Intramuscular Fat Fraction and Muscle Function Tests.

Objectives: Magnetization transfer (MT) imaging exploits the interaction between bulk water protons and protons contained in macromolecules to induce signal changes through a special radiofrequency pulse. MT detects muscle damage in patients with neuromuscular conditions, such as limb-girdle muscular dystrophies or Charcot-Marie-Tooth disease, which are characterized by progressive fiber loss and replacement by fatty tissue. In Pompe disease, in which there is, in addition, an accumulation of glycogen inside the muscle fibers, MT has not been tested yet. Our aim is to estimate MT ratio (MTR) in the skeletal muscle of these patients and correlate it with intramuscular fat fraction (FF) and results of muscle function tests. Methods: We obtained two-point axial Dixon and Dixon-MT sequences of the right thigh on a 1.5 Teslas MRI scanner in 60 individuals, including 29 late onset Pompe disease patients, 2 patients with McArdle disease, and 29 age and sex matched healthy controls. FF and MTR were estimated. Muscle function using several muscle function tests, including quantification of muscle strength, timed test quality of life scales, conventional spirometry obtaining forced vital capacity while sitting and in the supine position, were assessed in all patients. Results: MTR was significantly lower in Pompe patients compared with controls (45.5 ± 8.5 vs. 51.7 ± 2.3, Student T-test, p < 0.05). There was a negative correlation between the MTR and FF muscles studied (correlation coefficient: -0.65, Spearman test: p < 0.05). MTR correlated with most of the muscle function test results. We analyzed if there was any difference in MTR values between Pompe patients and healthy controls in those muscles that did not have an increase in fat, a measure that could be related to the presence of glycogen in skeletal muscles, but we did not identify significant differences except in the adductor magnus muscle (48.4 ± 3.6 in Pompe vs. 51 ± 1.3 in healthy controls, Student T-test = 0.023). Conclusions: MTR is a sensitive tool to identify muscle loss in patients with Pompe disease and shows a good correlation with muscle function tests. Therefore, the MT technique can be useful in monitoring muscle degeneration in Pompe disease in clinical trials or natural history studies.

Does Delirium Phenomenology in Persons with Advanced Cancer Follow a Specific Pattern?

Objective: Recognizing delirium phenomenology (DP) aids the early diagnosis of this syndrome and improves quality of life in patients with advanced cancer. The aim of this study was to identify the neurobehavioral and cognitive patterns of delirium-related symptoms in persons with advanced cancer. Methods: We conducted an observational comparative prospective study on delirium in patients with advanced cancer in different palliative care settings, assessing the presentation/evolution of DP with the Memorial delirium assessment scale (MDAS). Results: Two hundred twenty-seven patients were enrolled on hospital/hospice admission. Of these, 57 were admitted with delirium, 170 without delirium, and 31 developed delirium during hospitalization. Of the 88 patients admitted with delirium or who developed it during hospitalization, only 32 underwent two consecutive MDAS evaluations (at diagnosis and after one week). Delirium resolved in 22 patients (first average MDAS score 10.08 vs. second 3.6 [p < 0.001]). Disorientation, short-term memory, and memory span were altered in all patients with unresolved delirium. The same features were altered in 18 (80%), 17 (80%), and 16 (70%) of the patients with resolved delirium, respectively, and in 58 (35%), 114 (67%), and 38 (23%) of no-delirium patients, respectively. Conclusion: Cognitive-related symptoms appear to be the most prevalent and earliest signs of DP in patients with advanced cancer.

From European Association for Palliative Care Recommendations to a Blended, Standardized, Free-to-Access Undergraduate Curriculum in Palliative Medicine: The EDUPALL Project.

The World Health Organization recommends that "palliative care should be integrated as a routine element of all Undergraduate Medical Education." However, the provision of training for medical undergraduates is variable; only 18% of 51 European countries have mandatory training in palliative medicine. EDUPALL is an ERASMUS+ funded international collaborative project to develop and pilot an undergraduate program for training in palliative medicine. The objective of this study was to critically review and revise current European Association for Palliative Care (EAPC) Recommendations for the Development of Undergraduate Curricula in Palliative Medicine and translating these into an updated curriculum document. Clinicians, academics, and researchers from Romania, Ireland, Germany, Austria, Spain, and the United Kingdom reviewed the EAPC recommendations using a variant of consensus methodology, Nominal Group Technique. From the updated document, four working-groups translated each recommendation into a specific learning objective, and developed associated learning outcomes, stratified by domain: attitude, cognition, and skills. The outcomes and objectives were organized into discrete teaching units and transferred into a curriculum template, identifying notional hours, teaching, and assessment strategies. To ensure quality control, the draft template was circulated to experts from 17 European countries, together with a brief survey instrument, for peer review purposes. All 17 reviewers returned overwhelmingly positive comments. There was large agreement that: the teaching units were logically organized; learning outcomes covered core training needs; learning objectives provided guidance for teaching sessions; learning modalities were appropriately aligned; and assessment strategies were fit for purpose. An updated and standardized curriculum was developed, which provides a platform for the sequential development of the next phases of the EDUPALL project.

Follow-up of late-onset Pompe disease patients with muscle magnetic resonance imaging reveals increase in fat replacement in skeletal muscles.

BACKGROUND: Late-onset Pompe disease (LOPD) is a genetic disorder characterized by progressive degeneration of the skeletal muscles produced by a deficiency of the enzyme acid alpha-glucosidase. Enzymatic replacement therapy with recombinant human alpha-glucosidase seems to reduce the progression of the disease; although at the moment, it is not completely clear to what extent. Quantitative muscle magnetic resonance imaging (qMRI) is a good biomarker for the follow-up of fat replacement in neuromuscular disorders. The aim of this study was to describe the changes observed in fat replacement in skeletal muscles using qMRI in a cohort of LOPD patients followed prospectively. METHODS: A total of 36 LOPD patients were seen once every year for 4 years. qMRI, several muscle function tests, spirometry, activities of daily living scales, and quality-of-life scales were performed on each visit. Muscle MRI consisted of two-point Dixon studies of the trunk and thigh muscles. Computer analysis of the images provided the percentage of muscle degenerated and replaced by fat in every muscle (known as fat fraction). Longitudinal analysis of the measures was performed using linear mixed models applying the Greenhouse-Geisser test. RESULTS: We detected a statistically significant and continuous increase in mean thigh fat fraction both in treated (+5.8% in 3 years) and in pre-symptomatic patients (+2.6% in 3years) (Greenhouse-Geisser p < 0.05). As an average, fat fraction increased by 1.9% per year in treated patients, compared with 0.8% in pre-symptomatic patients. Fat fraction significantly increased in every muscle of the thighs. We observed a significant correlation between changes observed in fat fraction in qMRI and changes observed in the results of the muscle function tests performed. Moreover, we identified that muscle performance and mean thigh fat fraction at baseline visit were independent parameters influencing fat fraction progression over 4 years (analysis of covariance, p < 0.05). CONCLUSIONS: Our study identifies that skeletal muscle fat fraction continues to increase in patients with LOPD despite the treatment with enzymatic replacement therapy. These results suggest that the process of muscle degeneration is not stopped by the treatment and could impact muscle function over the years. Hereby, we show that fat fraction along with muscle function tests can be considered a good outcome measures for clinical trials in LOPD patients.