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Acknowledgments: The company Manzanal Mamphel SA provided the necessary tourniquets for the pretest and post-test phases of the study (480 devices)., Among the main preventable causes of death in the area of operations is external exsanguinating hemorrhage in the extremities, hence the importance of the tourniquet as a therapeutic tool in this type of injury and, therefore, of the training of personnel participating in international missions. The main objective of this study is to determine the impact of training in the application of this device. This is a quasi-experimental, prospective, cross-sectional study, carried out with 97 healthy volunteers, military personnel who perform their work in the Royal Guard barracks of El Pardo. The study was conducted between June 2019 and July 2021. The correct determination of the device placement site and the times of correct device placement were evaluated by determining whether there was blood flow using Doppler ultrasound measurements. Statistically significant results were obtained for application time (76.68 s to 58.06 s; p < 0.001), correct device placement (p < 0.001), and achievement of complete ischemia in the upper extremity (23.7% pretest vs. 24.7% post-test; p < 0.001). In the lower extremity, after training, longer application duration (43.33 s to 47.30 s) and lower ischemia achievement (59.8% pretest vs. 37.8% post-test) were obtained. Standardized and regulated training improves device application. More intensive training is necessary to obtain better results.

A case report of an 11-year-old boy with a de novo variant in NOTCH2 and clinical features characteristic of Hajdu-Cheney syndrome is reported, with acroosteolysis of the distal phalanges of the feet and hands, generalized osteoporosis, musculoskeletal and craniofacial alterations, short stature, bowing of long bones, vertebral anomalies, genu recurvatum, hypertrichosis, joint and skin hyperlaxity, atopic dermatitis, megalocorneas, micrognathia and frequent respiratory infections, among others. Treatment is with bisphosphonates in the framework of bone density improvement and with focal vibration therapy for rehabilitation of the musculoskeletal system and gait improvement. The three generalities of this pathology—phenotypic variability, degenerative character and the presence of generalized osteoporosis and acroosteolysis of the distal phalanges—are seen in this case, whose diagnostic confirmation was made by genetic study.

Hajdu–Cheney syndrome is a rare genetic disease. Its main features include phenotypic variability, age-dependent progression and the presence of acroosteolysis of the distal phalanges and generalized osteoporosis, which have significant disabling potential. Currently, there is no effective curative treatment, so nursing care is essential to ensure the maintenance of the quality of life of these patients. The main objective of this study was to establish a specific standardized nursing care plan using the NANDA–NIC–NOC taxonomy. The application of a care plan as such would improve the quality of life of patients affected by this rare disease, will contribute to increasing healthcare professionals’ knowledge on this matter and will support future studies on this disease.

Background: The treatment associated with non-Hodgkin lymphoma patients may cause adverse effects on their physical and psychological condition. The aim of this study is to detect the response to an eight-week, 16-session, 60-min presential Qigong program in anxiety, depression and vagal nerve activity alongside a control group. Methods: A randomized controlled clinical trial was managed. Randomization was carried out by generating a numerical sequence of three cycles through the software EPIDAT 4.1. Numbers were placed in sealed opaque envelopes for assignment to the different groups. Results: Anxiety levels were substantially decreased in the experimental group, with a large effect size (F = 30.38, p < 0.00). Depression levels had an improvement in the experimental group in contrast to the control group, reaching statistical significance (F = 19.19, p < 0.00). Heart Rate Variability unveiled significant results in terms of between-group differences, with a large effect size in the HRV Index (F = 15.80, p < 0.00), SDNN (F = 8.82, p < 0.00), and RMSSD (F = 6.72, p < 0.01) in the time domain, and a medium effect size in the HF (F = 9.78, p < 0.003), LF (F = 9.78, p < 0.00), and LF/HF Ratio (F = 18.44, p < 0.00) in the frequency domain, which were all bettered in the experimental group, after the Qigong program. Conclusions: Qigong therapy can be an effective therapeutic activity in consonance with traditional medicine to improve psychological health and autonomic nervous system balance in non-Hodgkin lymphoma survivors., Education Ministry, Madrid, Spanish Government FPU16/01437

Rare diseases, also known as orphan diseases, are medical conditions that affect a small percentage of the population [...]

Breast cancer continues to be the leading cause of death in women worldwide. Mammography, which is the current gold standard technique used to diagnose it, presents strong limitations in early ages where breast cancer is much more aggressive and fatal. MiRNAs present in numerous body fluids might represent a new line of research in breast cancer biomarkers, especially oncomiRNAs, known to play an important role in the suppression and development of neoplasms. The aim of this systematic review and meta-analysis was to evaluate dysregulated miRNA biomarkers and their diagnostic accuracy in breast cancer. Two independent researchers reviewed the included studies according to the preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines. A protocol for this review was registered in PROSPERO with the registration number “CRD42021256338”. Observational case-control-based studies analyzing concentrations of microRNAs which have been published within the last 10 years were selected, and the concentrations of miRNAs in women with breast cancer and healthy controls were analyzed. Random-effects meta-analyses of miR-155 were performed on the studies which provided enough data to calculate diagnostic odds ratios. We determined that 34 microRNAs were substantially dysregulated and could be considered biomarkers of breast cancer. Individually, miR-155 provided better diagnostic results than mammography on average. However, when several miRNAs are used to screen, forming a panel, sensitivity and specificity rates improve, and they can be associated with classic biomarkers such us CA-125 or CEA. Based on the results of our meta-analysis, miR-155 might be a promising diagnostic biomarker for this patient population.

This study aimed to examine the influence of new ICTs on the recording of nursing interventions in the Emergency Unit of the High Resolution Hospital (HRH) of Loja (Granada), Spain. A descriptive observational study was conducted to analyze the evolution of the Nursing Interventions (NIC) records in the Emergency Unit of the Loja HRH (Granada) from 2017 to 2021. Results showed that 11,076 NIC registrations were exploited, which increased by 51.2% from 2017 to 2021. The linear correlation between the NIC and the years was analyzed with Spearman’s coefficient, obtaining a low level of correlation (p = 0.166), but one that is statistically significant (p < 0.001). The introduction of tablet devices in the emergency room of the Loja HRH (Granada) led to a significant increase in the percentage of NIC recorded and collated during the study period without increasing the number of emergencies attended. However, usability barriers of ICTs were detected, highlighting the need to guide and train health professionals in their use and in the culture of patient safety.

Ehlers–Danlos syndrome type arthrochalasia (aEDS) is a rare genetic disease characterized by severe generalized joint hypermobility, bilateral congenital hip dislocation, skin hyperextensibility, muscle hypotonia, and mild dysmorphic features. It is an autosomal dominant connective tissue disease causing defects in collagen, associated with two genes, COL1A1 or COL1A2. Only about 42 cases have been published worldwide. Treatment is currently symptomatic and focuses on increasing the quality of life of these patients, as there is no curative treatment. The main objective of the review was to update information on Ehlers–Danlos syndrome type arthrochalasia from scientific publications. The review report was carried out in accordance with the criteria of the Preferred Reporting Items for Systematic reviews and MetaAnalyses (PRISMA) review protocol, by searching Orphanet, OMIM, PubMed, and Scopus, as well as free sources. A total of 20 articles were analyzed, which, after analysis, provide an updated report that aims to establish a solid starting point for future lines of research.

Introduction: Healthy lifestyle habits formed in childhood provide the foundation for a healthy adult life; therefore, it is important to encourage healthy habits and to correct poor habits from an early age. Aim: In this study, we aim to evaluate the influence of educational intervention in order to modify postural hygiene habits in school-age children. Materials and methods: We randomly selected three public primary education schools in the Galician provinces of Orense and Pontevedra based on stratified multistage sampling. A sample of 479 students was obtained, representing 2% of all 6- to 12-year-old children registered during the 2015/2016 academic year in those provinces. Results: Following the intervention, the students’ postural hygiene improved. We found statistically significant differences regarding a reduction in the number of hours spent watching television (0.531 h/day); the way school books and supplies were carried, with an increase in the use of backpacks with wheels (from 58.5% to 64.1%); and an improvement in postural hygiene when watching television, with an increase from 63.7% to 80.8% of those surveyed opting to watch television whilst seated on a chair instead of lying down. Conclusions: Educational intervention by inculcating healthy postural hygiene habits in children at an early age can improve and correct unhealthy behaviours.

Pregnancy is a vital event in a woman’s life that involves not only important physical changes, but also psychological changes [...]

In healthcare practice, there may be critically injured patients in whom catheterisation of a peripheral venous access is not possible. In these cases, intraosseous access may be the preferred technique, using an intraosseous vascular access device (IOVA). Such devices can be used for infusion or administration of drugs in the same way as other catheterisations, which improves emergency care times, as it is a procedure that can be performed in seconds to a minute. The aim of this study was to analyse the level of knowledge of nursing staff working in emergency departments regarding the management of the intraosseous vascular access devices. To this end, a cross-sectional online study was carried out using an anonymous questionnaire administered to all professionals working in emergency and critical care units (ECCUs) in Granada district (Spain). The results show that 60% of the participants believe that with the knowledge they have, they would not be able to perform intraosseous vascular access, and 74% of the participants believe that the low use of this device is due to insufficient training. The obtained results suggest that the intraosseous access route, although it is a safe and quick way of achieving venous access in critical situations, is considered a secondary form of access because the knowledge of emergency and critical care professionals is insufficient, given the totality of the participants demanding more training in the management of intraosseous access devices. Therefore, the implementation of theoretical/practical training programmes related to intraosseous access (IO) could promote the continuous training of nurses working in ECCUs, in addition to improving the quality of care in emergency and critical care situations.

Rare diseases are a subject of great scientific and health interest that has been on the rise in recent years [...]

Therapeutic hypothermia is a treatment used for patients who have suffered cardiorespiratory arrest and remain conscious after the recovery of spontaneous circulation. However, its effectiveness is controversial. The objective of this systematic review is to summarize the scientific evidence available about the effect of therapeutic hypothermia on neurological status and survival in this type of patients. Methodology: A primary search in CINAHL, CUIDEN, Pubmed, Web of Science, and Scopus databases was carried out. Randomized clinical trials (RCT) published from 2016 to 2020 were selected. Results: 17 studies were selected for inclusion and most relevant data were extracted. Methodological quality was assessed by the RoB tool. Conclusions: Although therapeutic hypothermia is a safe technique with few adverse and manageable effects, it has not shown to improve survival rate and neurological status of adult nor pediatric patients. It is possible that its positive effect on neuroprotection could be achieved only by preventing hyperthermia although further investigation is needed.

The recent respiratory virus known as SARS-CoV-2 has caused millions of deaths worldwide, causing great uncertainty due to the lack of a specific treatment, which has been mitigated by the use of various drugs traditionally used against other types of pathologies. Pregnancy presents special physiological conditions that expose the pregnant woman and the foetus to greater risk. Pregnant women are often excluded from trials due to possible risk of toxicity or side effects, resulting in a lack of knowledge about the use of drugs and treatments during pregnancy. The main objective of this review was to compile existing knowledge about currently available drug treatments for COVID-19 in pregnant women. The review report met the criteria of the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) review protocol and was registered with the registration number CRD42021251036. The electronic databases searched were Scopus, PubMed, CINAHL and SciELO. Finally, 22 articles were included, resulting in an analysis of drugs with an acceptable safety profile in the treatment of pregnant women with COVID-19.

Among the types of blood cancers, non-Hodgkin lymphoma is the most common. The usual treatments for this type of cancer can cause heart failure. A descriptive observational study was conducted that included 16 non-Hodgkin lymphoma survivors and 16 healthy controls matched by age and sex. Vagal tone was evaluated in the short term with a three-channel Holter device, and the time and frequency domains were analyzed following a previously accepted methodology to evaluate cardiac autonomic balance. The results of the analysis revealed that the standard deviation of the NN interval (F = 6.25, p = 0.021) and the square root of the mean of the sum of the differences between NN intervals (F = 9.74, p = 0.004) were significantly higher in healthy subjects than in lymphoma survivors. In the heart rate variability (HRV) index, there were no significant differences between the groups (F = 0.03, p = 0.85), nor in the parameters of the frequency domains LF (F = 1.94, p = 0.17), HF (F = 0.35, p = 0.55), and the ratio LF/HF (F = 3.07, p = 0.09). HRV values were lower in non-Hodgkin lymphoma survivors in the first year after treatment, resulting in autonomic imbalance compared to healthy paired subjects., Education Ministry (Program FPU16/01437), Madrid, Spanish Government

We thank our medical translator Megan Berry for her services and comments that contributed to improving the manuscript for publication., Cerebral palsy is described as a group of permanent neuromotor-type disorders caused by non-progressive injuries in the developmental stages of the central nervous system, and which have serious repercussions on the quality of life of affected children due to the physical and psychological damage it entails for them. Today, it is the leading cause of physical disability in childhood. Since there is no cure for this disorder, treatment is based on the improvement of symptoms, which is not always achieved through conventional therapies. For this reason, the need arises to investigate other alternative therapies, such as hippotherapy, to determine the main effects of hippotherapy as a rehabilitation therapy in children with cerebral palsy. The review was performed in accordance with the criteria of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) protocol and was registered under the number CRD42021233003. The databases used were PubMed, Dialnet and the web browser Google Scholar. After applying the inclusion criteria, we included 11 articles. As a conclusion, we found that hippotherapy provides benefits at physical, psychological, cognitive and social levels in children with cerebral palsy, and thus it should be considered as a complementary rehabilitation therapy to conventional treatments.

The research group behind this project wishes to thank the following associations for their collaboration in the distribution of the study questionnaire: Asogaf–Asociación Granadina de la Ataxia de Friedreich. Asociación de Enfermos Musculares de Granada. AGRADA Asociación de Ataxias de Granada. Asociación española de afectados por Epilepsia Grave. Phelan Mcdermid. 22q13. Asociación Síndrome Shy Drager–Atrofia Multisistémica. Adisen–Asociación Nacional de Addison y Otras Enfermedades Endocrinas. ASENCO, Asociación de Enfermedades Neuromusculares de Córdoba. Asociación 22 q Andalucía. Asociación Andaluza de Esclerosis Lateral Amiotrófica. Asociación Andaluza de Fibrosis Quística. Asociación Andaluza de Hemofilia. Asociación Cordobesa para la Investigación de Corea de Huntington. Asociación de Enfermedades Neuromusculares de Andalucía. Asociación de Enfermos con Neurodegeneración por Acumulación Cerebral de Hierro. Asociación de Familiares y Afectados Síndrome de Coffin-Lowry España. Asociación de Retinosis Pigmentaria de Andalucía. Asociación del Síndrome de Prader Willi de Andalucía. Asociación Española de Enfermos por Pseudoxantoma Elástico. Asociación Española de Familiares y Enfermos de Wilson. Asociación Española para la Investigación y Ayuda al Síndrome de Wolfram. Asociación Síndrome de Turner de Andalucía. Asociación Sindrome de Williams de Andalucía. Asociación Valverdeña de la Enfermedad de Andrade. DEBRA Piel de Mariposa (Asociación de Epidermólisis Bullosa de España). Federación Andaluza de Asociaciones de Ataxias. Asociación de Atrofia del Nervio Óptico de Léber. Acción y Cura para Tay Sachs. ACTAYS. Asociación 11q España. Asociación AMC–Artrogriposis Múltiple Congénita–España. Asociación de Afectados de Neurofibromatosis. Asociación de Afectados Síndrome Landau –Kleffner. Asociación de Apoyo e Investigación del Síndrome de 5p-. Asociación de Corea de Huntington Española. Asociación de Enfermedades Raras de Alcalá de Henares. Asociación de Enfermos de Patología Mitocondrial. Asociacion de Familias con Perthes. Asociación Española de Porfiria. Asociación Española del Síndrome de Beckwith– Wiedemann. Asociación Española del Síndrome de Joubert. Asociación Huesos de Cristal de España. Asociación Nacional Síndrome de Apert y otras Craneosinostosis Sindrómicas. Asociación de Enfermedades Raras D’Genes. FEGEREC–Federación Gallega de enfermedades raras. Asociación Chiari y Siringomielia del Principado de Asturias (CHySPA). Asociación Asturiana contra la Fibrosis Quística (F.Q.). Asociación Ataxias de Extremadura. Asociación de Retinosis Pigmentaria Extremeña. Asociación ELA Extremadura. Asociación Española de Cavernomas. Asociación Española de Lucha contra las Hemoglobinopatias y Talasemias. Asociación Española de Stickler. Asociación Extremeña contra la Fibrosis Quística. Asociación Extremeña de Enfermos de Huntington. Asociación La Vida con Hernia Diafragmática Congénita. Asociación PrincesaRett. Asociación Síndrome X Frágil de Extremadura. Alba, Asociación de Ayuda a Personas con Albinismo. Asociación “Síndrome de Microdeleción 5q14.3–MEF2C”. Asociación ADIBI. Asociación de Discapacitados y Enfermedades Raras de IBI. Asociación Afectados CDKL5. Asociación Conquistando Escalones. Asociación de Enfermedades Minoritarias de la Comunidad Valenciana. Asociación de Enfermedades Raras de Benidorm y Comarca. Asociación de enfermedades raras y discapacidad Sense Barreres de Petrer. Asociación de enfermos de FMF y síndromes autoinflamatorios de España. Asociación de Esclerodermia Castellón. Asociación de Fibrosis Quística de la Comunidad Valenciana. Asociación de lucha contra la Distonía Mioclónica de Valencia. Asociación de Microsomia Hemifacial. Asociación de Padres de Niños y Jóvenes con Enfermedades Reumáticas de la Comunidad Valenciana. Asociación CADASIL España. Asociación Catalana de Espina Bífida e Hidrocefalia. Asociación Duchenne Somriures Valents. Asociación Española de Enfermos del Síndrome de Camurati–Engelmann. Asociación Española del Síndrome de Cornelia de Lange. Asociación Microtia España. Asociación Síndrome STXBP1. Associació Catalana de la Síndrome de Rett. Associació Catalana de Malaltia de Huntington., Scientific knowledge on depression and anxiety in patients with rare diseases during the COVID-19 pandemic is scarce; however, it is essential to perform comprehensive management of these patients. The aim of this study was to research how the situation caused by the SARS-CoV-2 pandemic has influenced the lives of patients with rare diseases regarding depression and anxiety. This Spanish study considered a heterogeneous population sample of 86 patients with confirmed diagnosis of different rare diseases. Participants took part in a cross-sectional online study by completing specific questionnaires on the study topic. Depression was measured using the Patient Health Questionnaire (PHQ-9), and the General Anxiety Disorder Scale (GAD-7) was used for evaluating anxiety. Data collection through an online questionnaire allowed for a greater population scope and therefore the inclusion patients of other nationalities in the study sample. Finally, as a general result, this study found that, in the face of the pandemic, anxiety and depression remained at a higher level in this group than in the general population, making these patients a vulnerable population group.

Hajdu–Cheney syndrome (HCS) is a rare genetic disease that causes acroosteolysis and generalized osteoporosis, accompanied by a series of developmental skeletal disorders and multiple clinical and radiological manifestations. It has an autosomal dominant inheritance, although there are several sporadic non-hereditary cases.

Hajdu–Cheney syndrome (HCS) is a rare genetic disease that causes acroosteolysis and generalized osteoporosis, accompanied by a series of developmental skeletal disorders and multiple clinical and radiological manifestations. It has an autosomal dominant inheritance, although there are several sporadic non-hereditary cases. The gene that has been associated with Hajdu-Cheney syndrome is NOTCH2. The described phenotype and clinical signs and symptoms are many, varied, and evolve over time. As few as 50 cases of this disease, for which there is currently no curative treatment, have been reported to date. The main objective of this systematic review was to evaluate the results obtained in research regarding Hajdu–Cheney Syndrome. The findings are reported in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines and were registered on the web PROSPERO under the registration number CRD42020164377. A bibliographic search was carried out using the online databases Orphanet, PubMed, and Scielo; articles from other open access sources were also considered. Finally, 76 articles were included, and after their analysis, we have obtained a series of hypotheses as results that will support further studies on this matter.