UNASSIGNED: Dystrophic epidermolysis bullosa (DEB), a rare genetic skin disease caused by loss-of-function mutations in COL7A1, the gene encoding type VII collagen (COL7), is characterized by skin blistering, scarring, and extracutaneous manifestations that markedly reduce patient quality-of-life. Beremagene geperpavec-svdt (\'B-VEC\') is a gene therapy employing a non-integrating, replication-defective herpes simplex virus type 1 (HSV-1)-based vector encoding two copies of full-length human COL7A1 to restore COL7 protein after topical administration to DEB wounds. B-VEC was approved in the United States in 2023 as the first topical gene therapy and the first approved treatment for DEB. However, few providers have experience with use of this gene therapy.
UNASSIGNED: Data was obtained through literature review and the experience of providers who participated in the B-VEC clinical study or initiated treatment after B-VEC approval.
UNASSIGNED: This review discusses the burden of disease, describes the clinical trial outcomes of B-VEC, and provides physician and patient/caregiver recommendations as a practical guide for the real-world use of B-VEC, which can be administered in-office or at the patient\'s home.
UNASSIGNED: By continuing to optimize the practical aspects of B-VEC administration, the focus will continue to shift to patient-centric considerations and improved patient outcomes.

The Spanish Guideline on the Management of Asthma, better known by its acronym in Spanish GEMA, has been available for more than 20 years. Twenty-one scientific societies or related groups both from Spain and internationally have participated in the preparation and development of the updated edition of GEMA, which in fact has been currently positioned as the reference guide on asthma in the Spanish language worldwide. Its objective is to prevent and improve the clinical situation of people with asthma by increasing the knowledge of healthcare professionals involved in their care. Its purpose is to convert scientific evidence into simple and easy-to-follow practical recommendations. Therefore, it is not a monograph that brings together all the scientific knowledge about the disease, but rather a brief document with the essentials, designed to be applied quickly in routine clinical practice. The guidelines are necessarily multidisciplinary, developed to be useful and an indispensable tool for physicians of different specialties, as well as nurses and pharmacists. Probably the most outstanding aspects of the guide are the recommendations to: establish the diagnosis of asthma using a sequential algorithm based on objective diagnostic tests; the follow-up of patients, preferably based on the strategy of achieving and maintaining control of the disease; treatment according to the level of severity of asthma, using six steps from least to greatest need of pharmaceutical drugs, and the treatment algorithm for the indication of biologics in patients with severe uncontrolled asthma based on phenotypes. And now, in addition to that, there is a novelty for easy use and follow-up through a computer application based on the chatbot-type conversational artificial intelligence (ia-GEMA).
La Guía Española para el Manejo del Asma, mejor conocida por su acrónimo en español, GEMA, está a nuestra disposición desde hace más de veinte años. Veintiuna sociedades científicas o grupos relacionados, tanto de España como de otros países, han participado en la preparación y desarrollo de la edición actualizada de GEMA que, de hecho, se ha posicionado en la actualidad a nivel mundial como la guía de referencia sobre asma en lengua española.Su objetivo es prevenir y mejorar la situación clínica de las personas con asma, aumentando el conocimiento de los profesionales sanitarios involucrados en su cuidado. Su propósito es convertir la evidencia científica en recomendaciones prácticas sencillas y fáciles de seguir. Por lo tanto, no se trata de una monografía que reúna todo el conocimiento científico sobre la enfermedad, sino más bien de un documento conciso con lo esencial, diseñado para ser aplicado rápidamente en la práctica clínica de rutina. Las recomendaciones son necesariamente multidisciplinares, están desarrolladas para ser útiles y una herramienta indispensable para médicos de diferentes especialidades, así como para profesionales de enfermería y farmacia.Seguramente, los aspectos más destacados de la guía son las recomendaciones para: establecer el diagnóstico del asma utilizando un algoritmo secuencial basado en pruebas diagnósticas objetivas; el seguimiento de los pacientes, preferentemente basado en la estrategia de lograr y mantener el control de la enfermedad; el tratamiento según el nivel de gravedad del asma utilizando seis escalones, desde la menor hasta la mayor necesidad de medicamentos, y el algoritmo de tratamiento basado en fenotipos para la indicación de biológicos en pacientes con asma grave no controlada. A esto se suma ahora una novedad para su fácil uso y seguimiento a través de una aplicación informática basada en la inteligencia artificial conversacional de tipo chatbot (ia-GEMA).

This practice guideline provides updated evidence for the gynecologist who performs endometrial biopsy (EB) in gynecologic clinical practice. An international committee of gynecology experts developed the recommendations according to AGREE Reporting Guideline. An adequate tissue sampling is mandatory when performing an EB. Blind methods should not be first choice in patients with suspected endometrial malignancy. Hysteroscopy is the targeted-biopsy method with highest diagnostic accuracy and cost-effectiveness. Blind suction techniques are not reliable for the diagnosis of endometrial polyps. In low resources settings, and in absence of the capacity to perform office hysteroscopy, blind techniques could be used for EB. Hysteroscopic punch biopsy allows to collect only limited amount of endometrial tissue. grasp biopsy technique should be considered first choice in reproductive aged women, bipolar electrode chip biopsy should be preferred with hypotrophic or atrophic endometrium. EB is required for the final diagnosis of chronic endometritis. There is no consensus regarding which endometrial thickness cut-off should be used for recommending EB in asymptomatic postmenopausal women. EB should be offered to young women with abnormal uterine bleeding and risk factors for endometrial carcinoma. Endometrial pathology should be excluded with EB in nonobese women with unopposed hyperestrogenism. Hysteroscopy with EB is useful in patients with abnormal bleeding even without sonographic evidence of pathology. EB has high sensitivity for detecting intrauterine pathologies. In postmenopausal women with uterine bleeding, EB is recommended. Women with sonographic endometrial thickness > 4 mm using tamoxifen should undergo hysteroscopic EB.

The assessment of parent-child interactions and relationships (PCIR) plays an important role for many diagnostic purposes in child and adolescent psychology and psychological health care. While child and adolescent psychology has been intensively researched, the field still faces a lack of knowledge about health care practice. To offer knowledge about practical routine needs and derived needs in these domains, we aimed to obtain information from professionals who routinely assess PCIR.We aimed to gain a basic description of task-specific diagnostic fields, professional staff and their education, their clients, key diagnostic questions, observational settings, guidelines used in assessing PCIR and professionals\' personal understanding of PCIR. To gain information on how professionals assess PCIR, we used an online survey containing multiple choice questions and rating scales.We describe differences between task-specific diagnostic fields of inpatient and outpatient settings, consulting and officially appointed surveyors for court decision. Only responses from professionals performing PCIR are analyzed (N = 166). PCIR is regularly used for more than a half of children between 0-12 years of age and for more than a third of adolescents for answering a broad spectrum of diagnostical questions. We describe differences for nearly all facets of PCIR except for the content related domain. Based on these differences between task-specific fields, we give suggestions for standardized documentation of PCIR and how findings from this study can be used for scientific development.
Zusammenfassung Die Diagnostik der Eltern-Kind-Interaktion und deren Beziehung (EKIB) stellt für viele psychologische Fragestellungen im Kindes- und Jugendalter einen wichtigen Baustein dar, der intensiv beforscht wird. Zur Praxis einer EKIB liegen bislang keine Informationen vor, um eine praxisbezogene und wissenschaftliche Diskussion anzuregen. Die Beschreibung soll einen Einblick in die diagnostische Praxis ermöglichen. Deskriptiv beschreibt die Studie Unterschiede zwischen den Tätigkeitsfelder in einer Klinik, in der Praxis, in der Beratung oder als Gutachter/in hinsichtlich der Durchführung, der Klientel, des/der Diagnostiker/in und dessen/ deren Ausbildung, der diagnostischen Fragestellungen, der Beobachtungssettings sowie die Bedeutung einer EKIB für die eigene Arbeit und die Umsetzung von Empfehlungen und Standards. Unterschiede zwischen den Tätigkeitsfeldern stationärer und ambulanter Therapie, Beratung und gerichtlicher Sachverständiger/innen werden deskriptiv beschrieben. Nur Teilnehmende, die selbst eine EKIB durchführen (N = 166), wurden ausgewertet. Eine EKIB wird von mehr als der Hälfte der Teilnehmenden bei Kindern zwischen 0 und 12 Jahren standardmäßig für ein breites Spektrum an Fragestellungen durchgeführt und von mehr als einem Drittel bei Jugendlichen bis zum 18. Lebensjahr eingesetzt. Es zeigten sich Unterschiede zwischen den Tätigkeitsfeldern in vielen Bereichen, jedoch nicht in den inhaltlichen Aspekten. Auf Basis der beschriebenen Variationen im diagnostischen Prozess wird eine standardisierte Dokumentation vorgeschlagen sowie Entwicklungsfelder für die Forschung diskutiert.

OBJECTIVE: To present a practical how-to guide on implementing a coping skills intervention for patients with cancer facing surgery.
METHODS: A review of the literature on coping and coping skills and on assessing the impact on patients\' emotional status.
CONCLUSIONS: Developing and presenting a brief coping skills program is possible within the confines of perioperative preparation for oncology patients. Such coping skills help develop a sense of self-efficacy, personal control, and resilience. Patients are responsive to the program and seem to practice their home assignments in a satisfactory manner.
CONCLUSIONS: Oncology nurses can implement some of or all of the program with their patients. The coping skills fall under the mandate of a variety of health care professionals and are readily available to present to patients. The preoperative period is a suitable time to engage patients to learn coping skills.

Background and Objectives: Hysteroscopy is a reliable technique which is highly useful for the evaluation and management of intrauterine pathology. Recently, the widespread nature of in-office procedures without the need for anesthesia has been requesting validation of practical approach in order to reduce procedure-related pain. In this regard, we performed a comprehensive review of literature regarding pain management in office hysteroscopic procedures. Materials and Methods: MEDLINE, EMBASE, The Cochrane Library (Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials, Cochrane Methodology Register), Global Health, Health Technology Assessment Database and Web of Science, other research registers (for example Clinical Trials database) were searched. We searched for all original articles regarding pain relief strategy during office hysteroscopy, without date restriction. Results have been collected and recommendations have been summarized according to the Appraisal of Guidelines for Research and Evaluation (AGREE) tool. Moreover, the strength of each recommendation was scored following the Grading of Recommendations Assessment (GRADE) system, in order to present the best available evidence. Results: Both pharmacological and non-pharmacological strategies for pain management are feasible and can be applied in office setting for hysteroscopic procedures. The selection of strategy should be modulated according to the characteristics of the patient and difficulty of the procedure. Conclusions: Accumulating evidence support the use of pharmacological and other pharmacological-free strategies for reducing pain during office hysteroscopy. Nevertheless, future research priorities should aim to identify the recommended approach (or combined approaches) according to the characteristics of the patient and difficulty of the procedure.

Estimation of effective dose and dose to the lens of the eye for workers involved in interventional procedures is challenging. The interventional procedures in question involve high doses and, due to this, workers need to wear protective garments. As a result, various methodologies have been developed to assess the effective dose and dose to the lens of the eye. In the present study, measurements from four European dosimetry services, over and under protective garments, have been collected and analysed in order to provide practical guidelines based on the routine use of personal dosemeters from staff in interventional workplaces. The advantages and limitations of using one or two dosemeters are discussed.

OBJECTIVE: This specific review aims to expose clinicians, researchers and administrators in hospitals to the importance, procedures and safety of fMRI studies to promote the increased utilisation of such studies in different geographical places worldwide. The child\'s brain is developing rapidly, both structurally and functionally. These functional changes can only be detected using functional scans generated from an MRI machine and referred to as a functional MRI (fMRI). This method may be used clinically in complex medical and surgical conditions (e.g., epilepsy surgery), but these days are often used for research purposes. However, due to ethical and logistical considerations, fMRI in the paediatric population is not widely and equally used in different geographical places.
CONCLUSIONS: The benefits of using this method to define the functional changes occurring in the developing brain are discussed in this review, along with desensitisation methods recommended when working with this vulnerable population in research and even in a clinical setting.

There is currently a paucity of neuroscientific data recorded from more severely affected individuals with autism spectrum conditions (ASC). Enabling data collection to take place in a more familiar environment, that is, at home, may increase access to research participation in this group. Here, we present a new accessible method of studying brain activity of autistic individuals outside the laboratory in their home environment, using mobile electroencephalography (EEG) technology. The primary aim of the present study was to test the feasibility of acquiring good quality EEG data from autistic children at home, assessed via a set of objective data quality metrics, and to develop a list of practical guidelines on how to successfully conduct an EEG experiment in such a naturalistic setting based directly upon participants\' views. To demonstrate the utility of this method, we evaluated the EEG signal quality recorded from 69 children with ASC at home using a gel-based Eego Sports mobile EEG system. Five key indicators of data quality were assessed. Our results demonstrate that it is possible to record high quality EEG signal from children with ASC at home, generating data that could address a number of research questions. A user experience survey identified areas of good practice, which researchers should take into consideration when designing mobile EEG studies aiming to acquire data from children with ASC at a home environment.

Coronavirus disease 2019 (COVID-19), with an increasing number of deaths worldwide, has created a tragic global health and economic emergency. The disease, caused by severe acute respiratory syndrome coronavirus 2019 (SARS-CoV-19), is a multi-system inflammatory disease with many of COVID-19-positive patients requiring intensive medical care due to multi-organ failures. Biomarkers to reliably predict the patient\'s clinical cause of the virus infection, ideally, to be applied in point of care testing or through routine diagnostic approaches, are highly needed. We aimed to probe if routinely assessed clinical lab values can predict the severity of the COVID-19 course. Therefore, we have retrospectively analyzed on admission laboratory findings in 224 consecutive patients from four hospitals and show that systemic immune inflammation index (SII) is a potent marker for predicting the requirement for invasive ventilator support and for worse clinical outcome of the infected patient. Patients\' survival and severity of SARS-CoV-2 infection could reliably be predicted at admission by calculating the systemic inflammatory index of individual blood values. We advocate this approach to be a feasible and easy-to-implement assay that may be particularly useful to improve patient management during high influx crisis. We believe with this work to contribute to improving infrastructure availability and case management associated with COVID-19 pandemic hurdles.