V kazuistice prezentujeme případ patnáctiletého chlapce se vzácnou primární imunodeficiencí, tzv. syndromem XMEN. Tento syndrom je charakterizován zvýšenou náchylností k chronické EBV infekci a lymfoproliferacím asociovaným s EBV, dalšími klinickými znaky bývají sinopulmonární infekce, otitidy, lymfadenopatie, dysgamaglobulinemie a autoimunitní cytopenie. XMEN syndrom je způsoben mutacemi genu MAGT1, který kóduje přenašeč hořečnatých kationtů magnesium transporter protein 1. Klíčovým bodem kazuistiky je strastiplná diagnostická cesta pacienta, jemuž přes postupné návštěvy celé řady specialistů chybělo komplexní zhodnocení stavu a zachycení souvislostí různých klinických symptomů, které přinesla až návštěva v ambulanci alergologa/klinického imunologa
In this case report, we present a case of a 15 year old boy with a rare primary immunodeficiency called XMEN syndrome. XMEN syndrom is characterized by increased susceptibility to chronic EBV infection and EBV-associated lymphoproliferation, sinopulmonary and ear infections, lymphadenopathy, dysgammaglobulinemia, and autoimmune cytopenias. XMEN disease is caused by loss of function mutations in the gene MAGT1, which codes magnesium transporter protein 1.The critical point of the case report is the difficult diagnostic journey of the patient, who, despite a series of visits to several specialists, lacked a comprehensive assessment of his condition. A visit to an allergist/ clinical immunologist only gave a grasp of the context of various clinical symptoms.
Construct: The Knowledge of Malnutrition - Geriatric 2.0' (KoM-G 2.0) instrument was designed to quantify nursing staff malnutrition knowledge in inpatient medical and rehabilitation care facilities, as well as home health care. It has been used to assess grasp of current clinical practice guidelines and proficiency in addressing issues related to malnutrition. It provides insight into familiarity with and capacity to tackle issues pertaining to malnutrition in clinical practice. Furthermore, it has been used assess the effectiveness of educational interventions aimed at improving nursing professionals knowledge and awareness of malnutrition. Background: The quality of nursing education affects malnutrition risk assessment, monitoring of food intake, and effectiveness of nutrition care. Improvements in malnutrition education require determining the current level of knowledge and benchmarking with other countries. In the Czech Republic, no nationwide assessment of nursing staff malnutrition knowledge has ever been conducted. Approach: The purpose of the study was to translate the KoM-G 2.0 instrument, gather initial validity evidence, and evaluate nursing staff knowledge of malnutrition in inpatient medical, rehabilitation care facilities, and home care in the Czech Republic. All inpatient healthcare facilities and home healthcare facilities in the Czech Republic were invited to participate. The Czech version of the internationally standardized KoM-G 2.0 (KoM-G 2.0 CZ) was used to assess nursing staff malnutrition knowledge between 3 February 2021 and 31 May 2021. A total of 728 nurses began the questionnaire, and 465 (63.9%) of respondents completed it and were included in the study. Data analyses examined instrument difficulty, discriminability, and reliability, as well as sources of variation in knowledge scores. Findings: The psychometric characteristics of the KoM-G 2.0 CZ instrument included the difficulty index Q (0.61), the discriminant index (ULI 0.29, RIT 0.38, upper-lower 30% 0.67), and Cronbach alpha (0.619). The overall mean of correct answers was 6.24 (SD 2.8). There was a significant impact of educational attainment and nutrition training on KoM-G 2.0 CZ scores. Conclusions: Our findings provide initial validity evidence that KoM-G 2.0 CZ is useful and appropriate for assessing malnutrition knowledge among Czech nursing staff. Our research identified gaps in knowledge and examples of good practice in understanding malnutrition that can be applied internationally. The knowledge of academic nurses was greater; therefore, we suggest they play a key role in nutritional care. We recommend continuous education to improve understanding of malnutrition in this setting.
- MeSH
- Adult MeSH
- Clinical Competence standards MeSH
- Middle Aged MeSH
- Humans MeSH
- Nursing Staff education MeSH
- Malnutrition * MeSH
- Cross-Sectional Studies MeSH
- Surveys and Questionnaires MeSH
- Psychometrics * MeSH
- Health Knowledge, Attitudes, Practice MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Geographicals
- Czech Republic MeSH
INTRODUCTION: Upper limb (UL) impairment is common in people with multiple sclerosis (pwMS), and functional recovery of the UL is a key rehabilitation goal. Technology-based approaches, like virtual reality (VR), are increasingly promising. While most VR environments are task-oriented, our clinical approach integrates neuroproprioceptive 'facilitation and inhibition' (NFI) principles. To advance this, we developed immersive VR software based on NFI principles targeting UL function and sit-to-stand ability. This study aims to evaluate the effectiveness of this VR therapy compared with conventional NFI-based physical therapy in pwMS. Our study uniquely applies advanced imaging techniques, along with biological molecular assessments, to explore adaptive processes induced by VR rehabilitation. METHODS AND ANALYSIS: This double-arm, randomised, assessor-blinded, controlled trial runs over 2 months (1 hour, 2 times per week). PwMS with mild to severe disability will receive either VR therapy or real-world physical therapy. Primary outcomes include the nine-hole peg test, box and block test, handgrip strength, tremor and five times sit-to-stand test. Secondary measures include the Multiple Sclerosis Impact Scale, the 5-level EQ-5D questionnaire and kinematic analysis. Adaptive processes will be monitored using imaging techniques (functional MRI and tractography), molecular genetic methods (long non-coding RNAs) and immune system markers (leukocytes, dendritic cells). The International Classification of Functioning, Disability and Health brief set for MS will map the bio-psycho-social context of participants. ETHICS AND DISSEMINATION: This project and its amendments were approved by the Ethics Committee of the Institute for Clinical and Experimental Medicine and Thomayer Hospital (1983/21+4772/21 (G-21-02) and the Ethics Committee of Kralovske Vinohrady University Hospital (EK-VP/38/0/2021) in Prague, Czechia (with single enrolment). The findings of this project will be disseminated through scientific publications, conferences, professional networks, public engagement, educational materials and stakeholder briefings to ensure a broad impact across clinical, academic and public domains. TRIAL REGISTRATION NUMBER: clinicaltrials.gov (NCT04807738).
- MeSH
- Adult MeSH
- Double-Blind Method MeSH
- Upper Extremity * physiopathology MeSH
- Quality of Life * MeSH
- Humans MeSH
- Magnetic Resonance Imaging * methods MeSH
- Posture MeSH
- Randomized Controlled Trials as Topic MeSH
- Multiple Sclerosis * diagnostic imaging MeSH
- Hand Strength MeSH
- Physical Therapy Modalities * MeSH
- Virtual Reality Exposure Therapy methods MeSH
- Virtual Reality MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- Male MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Clinical Trial Protocol MeSH
Recent reviews have highlighted conflicting findings regarding the validity of finger flexor strength and endurance tests in sport climbers, often due to small sample sizes and low ecological validity of the tests used. To address these gaps, 185 male and 122 female climbers underwent maximal finger flexor strength, intermittent and continuous finger flexor endurance, and the finger hang tests in a sport-specific setting to determine the predictive and concurrent validity of these tests. The finger hang test showed the strongest relationship to climbing ability for both sexes (R ≈ 0.75). However, despite its widespread use as an endurance test, the finger hang was found to be primarily determined by finger strength, explaining 65% and 80% of the variance in males and females, respectively. Finger strength emerged as the dominant factor, explaining the majority of variance in climbing ability (males 68%; females 64%), followed by intermittent endurance (males 28%; females 34%). These findings emphasize finger strength as the primary predictor of climbing ability and highlight the importance of intermittent endurance testing for assessing climbing-specific endurance of the finger flexors. No significant differences were found between male and female climbers in finger flexor strength and endurance when normalized to body mass.
- MeSH
- Adult MeSH
- Physical Endurance * physiology MeSH
- Mountaineering * physiology MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Fingers * physiology MeSH
- Reproducibility of Results MeSH
- Sex Factors MeSH
- Hand Strength physiology MeSH
- Muscle Strength physiology MeSH
- Exercise Test methods MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Male MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Validation Study MeSH
BACKGROUND: Impaired physical performance and muscle strength are recognized risk factors for fragility fractures, frequently associated with osteoporosis and sarcopenia. However, the integration of muscle strength and physical performance in the comprehensive assessment of fracture risk is still debated. Therefore, this cross-sectional study aimed to assess the potential role of hand grip strength (HGS) and short physical performance battery (SPPB) for predicting fragility fractures and their correlation with Fracture Risk Assessment Tool (FRAX) with a machine learning approach. METHODS: In this cross-sectional study, a group of postmenopausal women underwent assessment of their strength, with the outcome measured using the HSG, their physical performance evaluated using the SPPB, and the predictive algorithm for fragility fractures known as FRAX. The statistical analysis included correlation analysis using Pearson's r and a decision tree model to compare different variables and their relationship with the FRAX Index. This machine learning approach allowed to create a visual decision boundaries plot, providing a dynamic representation of variables interactions in predicting fracture risk. RESULTS: Thirty-four patients (mean age 63.8±10.7 years) were included. Both HGS and SPPB negatively correlate with FRAX major (r=-0.381, P=0.034; and r=-0.407, P=0.023 respectively), whereas only SPPB significantly correlated with an inverse proportionality to FRAX hip (r=-0.492, P=0.001). According to a machine learning approach, FRAX major ≥20 and/or hip ≥3 might be reported for an SPPB<6. Concurrently, HGS<17.5 kg correlated with FRAX major ≥20 and/or hip ≥3. CONCLUSIONS: In light of the major findings, this cross-sectional study using a machine learning model related SPPB and HGS to FRAX. Therefore, a precise assessment including muscle strength and physical performance might be considered in the multidisciplinary assessment of fracture risk in post-menopausal women.
- MeSH
- Risk Assessment MeSH
- Bone Density * physiology MeSH
- Middle Aged MeSH
- Humans MeSH
- Osteoporotic Fractures * epidemiology etiology MeSH
- Postmenopause MeSH
- Cross-Sectional Studies MeSH
- Risk Factors MeSH
- Aged MeSH
- Hand Strength MeSH
- Physical Functional Performance MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
PURPOSE OF THE STUDY: The purpose of this study was to assess the patient experience of trapeziectomy under WALANT for trapeziometacarpal joint (TMJ) osteoarthritis (OA) in a prospective study with 2-year follow-up. MATERIAL AND METHODS: The study included 23 patients with TMJ OA undergoing trapeziectomy with WALANT. All patients were seen by a hand therapist preoperatively and at 3, 12, and 24 months postoperatively. At each visit, VAS pain scores, thumb range of motion, grip strength, and Disabilities of the Arm, Shoulder and Hand (DASH) score were assessed. The Picker Patient Experience (PPE-15) questionnaire was administered within 2 weeks of surgery. RESULTS: All 23 patients completed the PPE-15 questionnaire. Their mean age was 64 years. The 21 patients who remained at the 24-month follow-up all said they would choose the same anaesthesia method again. At this follow-up, VAS pain scores, thumb range of motion, key pinch grip and DASH scores had improved significantly, while thumb opposition and hand grip strength remained largely unchanged. The majority of patients felt well informed before and during the procedure, and all patients rated pain relief as good or satisfactory. Nearly 40% of patients reported receiving inadequate information about the postoperative medications. DISCUSSION: Patients have a positive attitude to trapeziectomy with WALANT, and seem to prefer WALANT over other methods of anaesthesia. Trapeziectomy with WALANT for TMJ OA is a safe procedure and appears to give a functional outcome similar to trapeziectomy under general anaesthesia. CONCLUSIONS: Trapeziectomy with WALANT for TMJ OA is safe, preferred by patients and has similar clinical outcome as trapeziectomy in general anesthesia. KEY WORDS: trapeziectomy, osteoarthritis, WALANT.
- MeSH
- Carpometacarpal Joints surgery physiopathology MeSH
- Middle Aged MeSH
- Humans MeSH
- Anesthesia, Local * methods MeSH
- Pain Measurement MeSH
- Metacarpal Bones surgery MeSH
- Follow-Up Studies MeSH
- Osteoarthritis * surgery physiopathology MeSH
- Thumb surgery physiopathology MeSH
- Prospective Studies MeSH
- Surveys and Questionnaires MeSH
- Range of Motion, Articular * MeSH
- Aged MeSH
- Hand Strength MeSH
- Patient Satisfaction MeSH
- Trapezium Bone * surgery MeSH
- Treatment Outcome MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
This study focuses on understanding the intentions and perspectives of the youth in Ukraine regarding their return to their native cities post-de-occupation. In the context of sustainable regional development, the research aims to grasp the complexities of the youth's mindset, which is crucial for effective policy-making and strategic planning in the post-occupation period. The study utilized a mixed-methods approach, combining quantitative surveys and qualitative focus group discussions. The quantitative phase involved a survey, targeting youth aged 14-35 and its district, to gather data on their willingness to return to de-occupied territories, trust in local institutions, and views on reconstruction efforts. This was followed by qualitative research through structured focus groups, segmented into participants with varying attitudes towards returning. The study highlights the importance of stability, economic growth, and the rebuilding of trust from the youth's viewpoint. It emphasizes the critical role of young people as key stakeholders in the reconstruction and planning processes.
- Publication type
- Journal Article MeSH
Juvenile hormone (JH) signaling is realized at the gene regulatory level by receptors of the bHLH-PAS transcription factor family. The sesquiterpenoid hormones and their synthetic mimics are agonist ligands of a unique JH receptor (JHR) protein, methoprene-tolerant (MET). Upon binding an agonist to its PAS-B cavity, MET dissociates from a cytoplasmic chaperone complex including HSP83 and concomitantly switches to a bHLH-PAS partner taiman, forming a nuclear, transcriptionally active JHR heterodimer. This course of events resembles the vertebrate aryl hydrocarbon receptor (AHR), activated by a plethora of endogenous and synthetic compounds. Like in AHR, the pliable PAS-B cavity of MET adjusts to diverse ligands and binds them through similar mechanisms. Despite recent progress, we only begin to discern agonist-induced conformational shifts within the PAS-B domain, with the ultimate goal of understanding how these localized changes stimulate the assembly of the active JHR complex and, thus, fully grasp the mechanism of JHR signaling.
- MeSH
- Juvenile Hormones * metabolism MeSH
- Signal Transduction MeSH
- Basic Helix-Loop-Helix Transcription Factors metabolism genetics chemistry MeSH
- Animals MeSH
- Check Tag
- Animals MeSH
- Publication type
- Journal Article MeSH
- Review MeSH
BACKGROUND AND OBJECTIVES: Skeletal muscle is characterized by its mass, strength and performance. These normative values are pivotal in defining sarcopenia. Sarcopenia is associated with poor outcome of numerous medical and surgical conditions. This study aimed to establish normative benchmarks for skeletal muscle mass, strength and performance within the context of the Asian (Indian) population. METHODS: Our investigation utilized the computed tomography (CT) skeletal muscle index (SMI), handgrip strength (HGS), gait velocity and chair-stand test to construct reference values for muscle characteristics in the Indian population. RESULTS: The SMI analysis incorporated 1485 cases of acute abdomen (54.7%) males). The calculated SMI (kg/m2) was 38.50 (35.05-42.30) in males and 36.30 (32.20-41.20) in females (p = 0.510). The study also involved 3083 healthy individuals (67.6% males) evaluated for muscle strength and performance between August 2017 and August 2018. Notably, HGS (kg force) was recorded at 34.95 (26.50-43.30) in males and 25.50 (18.60-31.20) in females (p < 0.001). Gait velocity (metres/second) exhibited values of 1.25 (1.04-1.56) in males and 1.24 (1.03-1.56) in females (p = 0.851). Additionally, chair-stand test (seconds) results were 10.00 (9.00-13.00) in males and 12.00 (10.00-14.00) in females (p < 0.001). CONCLUSIONS: The investigation determined that males had greater muscle strength and performance than females. But gender wise, there was no significant difference in muscle mass. Interestingly, our population's muscle parameters were consistently lower compared to western literature benchmarks. These normative values will help to define sarcopenia parameters in our population, which have prognostic value in multiple ailments.
- MeSH
- Adult MeSH
- Muscle, Skeletal * diagnostic imaging physiology anatomy & histology MeSH
- Middle Aged MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Tomography, X-Ray Computed * MeSH
- Reference Values MeSH
- Walking Speed physiology MeSH
- Sarcopenia * diagnosis physiopathology MeSH
- Aged MeSH
- Hand Strength * physiology MeSH
- Muscle Strength * physiology MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Geographicals
- India MeSH
OBJECTIVE: The purpose of this study was to establish a consensus on the surgical technique for sentinel lymph node (SLN) dissection in cervical cancer. METHODS: A 26 question survey was emailed to international expert gynecological oncology surgeons. A two-step modified Delphi method was used to establish consensus. After a first round of online survey, the questions were amended and a second round, along with semistructured interviews was performed. Consensus was defined using a 70% cut-off for agreement. RESULTS: Twenty-five of 38 (65.8%) experts responded to the first and second rounds of the online survey. Agreement ≥70% was reached for 13 (50.0%) questions in the first round and for 15 (57.7%) in the final round. Consensus agreement identified 15 recommended, three optional, and five not recommended steps. Experts agreed on the following recommended procedures: use of indocyanine green as a tracer; superficial (with or without deep) injection at 3 and 9 o'clock; injection at the margins of uninvolved mucosa avoiding vaginal fornices; grasping the cervix with forceps only in part of the cervix is free of tumor; use of a minimally invasive approach for SLN biopsy in the case of simple trachelectomy/conization; identification of the ureter, obliterated umbilical artery, and external iliac vessels before SLN excision; commencing the dissection at the level of the uterine artery and continuing laterally; and completing dissection in one hemi-pelvis before proceeding to the contralateral side. Consensus was also reached in recommending against injection at 6 and 12 o'clock, and injection directly into the tumor in cases of the tumor completely replacing the cervix; against removal of nodes through port without protective maneuvers; absence of an ultrastaging protocol; and against modifying tracer concentration at the time of re-injection after mapping failure. CONCLUSION: Recommended, optional, and not recommended steps of SLN dissection in cervical cancer have been identified based on consensus among international experts. These represent a surgical guide that may be used by surgeons in clinical trials and for quality assurance in routine practice.
- MeSH
- Sentinel Lymph Node Biopsy methods MeSH
- Indocyanine Green MeSH
- Consensus MeSH
- Humans MeSH
- Lymph Node Excision methods MeSH
- Lymphatic Metastasis pathology MeSH
- Lymph Nodes pathology MeSH
- Uterine Cervical Neoplasms * surgery pathology MeSH
- Check Tag
- Humans MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
