RIMASaud Institutional Repository of the Regional Health System
Not a member yet
61753 research outputs found
Sort by
Vascular anomalies in childhood. Review and update
No full textVascular anomalies are changes in vascularization that usually appear in the foetal stage, at birth or in early childhood. They can cause chronic pain, motor impairment, cosmetic changes or coagulopathy and may be fatal in some cases, but in every case they have a negative impact on the quality of life of the child and the family. Up to 150 different subtypes have been described. They can involve arteries, capillaries, veins, lymphatic vessels or a combination thereof. They may be associated with additional malformations and frequently cause musculoskeletal and soft tissue hypertrophy or hypotrophy. They can develop anywhere in the body, invade any tissue and affect the function of various organs. The prevalence of the different subtypes varies greatly, from 1/20 to 1/1 000 000. Subtypes considered rare diseases (incidenc
Clinical characteristics and prognostic factor in juvenile dermatomyositis: data of the Spanish registry.
No full text20.500.12530/87912Juvenile Dermatomyositis (JDM) is the most common chronic idiopathic inflammatory myopathy in children. The diagnosis is clinical. Baseline laboratory and complementary studies trace the phenotype of these patients. The objective of this study was to describe epidemiological, clinical and laboratory characteristics at diagnosis of JDM patients included in the Spanish JDM registry, as well as to identify prognostic factors on these patients. We retrospectively reviewed clinical features, laboratory tests, and complementary studies at diagnosis of JDM patients included on the Spanish JDM registry. These data were analyzed to assess whether there was a relationship with the development of complications and time to disease inactivity. One hundred and sixteen patients from 17 Spanish paediatric rheumatology centres were included, 76 girls (65%). Median age at diagnosis was 7.3 years (Interquartile range (IQR) 4.5-10.2). All patients had pathognomonic skin lesions at the beginning of the disease. Muscle weakness was present in 86.2%. Median Childhood Muscle Assessment Scale was 34 (IQR 22-47). Twelve patients (34%) had dysphagia and 3,5% dysphonia. Anti-p155 was the most frequently detected myositis specific antibody, followed by anti-MDA5. Twenty-nine patients developed calcinosis and 4 presented with macrophage activation syndrome. 70% reached inactivity in a median time of 8.9 months (IQR 4.5-34.8). 41% relapsed after a median time of 14.4 months (IQR 8.6-22.8) of inactivity. Shorter time to treatment was associated with better prognosis (Hazard ratio (HR) = 0.95 per month of evolution, p = 0.02). Heliotrope rash at diagnosis correlates with higher risk of development complications. We describe heliotrope rash as a risk factor for developing complications in our cohort of JDM patients, an easy-to-evaluate clinical sign that could help us to identify the group of patients we should monitor closely for this complication
Role of recombinant factor VIIa in the clinical management of severe postpartum hemorrhage: consensus among European experts.
No full text20.500.12530/87857There have been significant advances in the medical management of severe postpartum hemorrhage (sPPH) over recent decades, which is reflected in numerous published guidelines. To date, many of the currently available national and international guidelines recommend recombinant factor VIIa (rFVIIa) to be used only at a very late stage in the course of sPPH, as a "last resort", before or after hysterectomy. Based on new safety data, rFVIIa has recently been approved by the European Medicines Agency (EMA) and Swissmedic for use in sPPH, if uterotonics are insufficient to achieve hemostasis, which in fact is significantly earlier in the course of postpartum hemorrhage (PPH). We therefore aimed to develop expert consensus guidance as a step toward standardizing care with the use of rFVIIa for clinicians managing women experiencing life-threatening sPPH. The consensus process consisted of one face-to-face meeting with a group of nine experts, including eight obstetrician-gynecologists and a hematologist highly experienced in sPPH care in tertiary care perinatal centers. The panel was representative of multidisciplinary expertise in the European obstetrics community and provided consensus opinion in answer to pre-defined questions around clinical practice with rFVIIa in the management of sPPH. Recommendations have been based on current national and international guidelines, extensive clinical experience, and consensus opinion, as well as the availability of efficacy and new safety data. The expert panel developed 17 consensus statements in response to the 13 pre-defined questions on the use of rFVIIa in the management of sPPH including: available efficacy and safety data and the need for interdisciplinary expertise between obstetricians, anesthesiologists, and hematologists in the management of sPPH. Based on novel data, the experts recommend: (1) earlier administration of rFVIIa in patients with sPPH who do not respond to uterotonic administration to optimize the efficacy of rFVIIa; (2) the importance of hematological parameter prerequisites prior to the administration of rFVIIa to maximize efficacy; and (3) continued evaluation or initiation of further invasive procedures according to standard practice. Furthermore, recommendations on the timing of rFVIIa treatment within the sPPH management algorithm are outlined in a range of specified clinical scenarios and settings, including vaginal delivery, cesarean section, and smaller birthing units before transfer to a tertiary care center. The panel agreed that according to available, and new data, as well as real-world experience, there is no evidence that the use of rFVIIa in patients with sPPH increases the risk of thromboembolism. The authors acknowledge that there is still limited clinical effectiveness data, as well as pharmacoeconomic data, on the use of rFVIIa in sPPH, and recommend further clinical trials and efficacy investigation. This expert panel provides consensus guidance based on recently available data, clinical experience, and expert opinion, augmented by the recent approval of rFVIIa for use in sPPH by the EMA. These consensus statements are intended to support clinical care for sPPH and may help to provide the impetus and a starting point for updates to existing clinical practice guidelines
[Translated article] Therapeutic Drug Monitoring of antibiotic and antifungical drugs in paediatric and newborn patients. Consensus Guidelines of the Spanish Society of Hospital Pharmacy (SEFH) and the Spanish Society of Paediatric Infectious Diseases (SEIP).
No full text20.500.12530/87858Therapeutic monitoring of antibiotics and antifungals based on pharmacokinetic and pharmacodynamic (PK/PD) parameters is a strategy increasingly used for the optimization of therapy to improve efficacy, reduce the occurrence of toxicities, and prevent the selection of antimicrobial resistance, particularly in vulnerable patients including neonates and the critical or immunocompromised paediatric host. In neonates and children, infections account for a high percentage of hospital admissions, and anti-infectives are the most used drugs. However, paediatric PK/PD studies and the evidence regarding the efficacy and safety of some newly marketed antibiotics and antifungals-usually used off-label in paediatrics-to determine the optimal drug dosage regimens are limited. It is widely known that this population presents important differences in the PK parameters (especially in drug clearance and volume of distribution) in comparison with adults that may alter antimicrobial exposure and, therefore, compromise treatment success. In addition, paediatric patients are more susceptible to potential adverse drug effects and they need closer monitoring. The aim of this document, developed jointly by the Spanish Society of Hospital Pharmacy and the Spanish Society of Paediatric Infectious Diseases, is to describe the available evidence on the indications for therapeutic drug monitoring (TDM) of antibiotics and antifungals in newborn and paediatric patients, and to provide practical recommendations for TDM in routine clinical practice to optimise their dosing, efficacy and safety. Of antibiotics and antifungals in the paediatric population
Practical Guidelines by the Andalusian Group for Nutrition Reflection and Investigation (GARIN) on Nutritional Management of Patients with Chronic Obstructive Pulmonary Disease: A Review.
No full textMalnutrition is common in chronic obstructive pulmonary disease (COPD) patients and is associated with worse lung function and greater severity. This review by the Andalusian Group for Nutrition Reflection and Investigation (GARIN) addresses the nutritional management of adult COPD patients, focusing on Morphofunctional Nutritional Assessment and intervention in clinical practice. A systematic literature search was performed using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) methodology, followed by critical appraisal based on Scottish Intercollegiate Guidelines Network (SIGN) guidelines. Recommendations were graded according to the European Society for Clinical Nutrition and Metabolism (ESPEN) system. The results were discussed among GARIN members, with consensus determined using a Likert scale. A total of 24 recommendations were made: 2(A), 6(B), 2(O), and 14(GPP). Consensus exceeded 90% for 17 recommendations and was 75-90% for 7. The care of COPD patients is approached from a nutritional perspective, emphasizing nutritional screening, morphofunctional assessment, and food intake in early disease stages. Nutritional interventions include dietary advice, recommendations on food group intake, and the impact of specialized nutritional treatment, particularly oral nutritional supplements. Other critical aspects, such as physical activity and quality of life, are also analyzed. These recommendations provide practical guidance for managing COPD patients nutritionally in clinical practice
Surgical marker navigation system in breast conserving surgery and excision of non-palpable lesions: first Spanish single-center experience.
No full textDue to the establishment of screening mammography for breast cancer detection, the number of non-palpable lesions has increased. Thus, an optimal localization system is mandatory for the excision of non-palpable breast tumors. The aim of the study is to report the feasibility Surgical Marker Navigation (SMN) system Sirius Pintuition® for the excision of non-palpable breast tumors and non-palpable axillary lymph nodes. A retrospective observational study of patients undergoing breast-conserving surgery and lymph node excision guided by SMN between December 2022 and May 2023 was performed. A total of 84 patients underwent excision of non-palpable breast tumors (77; 91.7%) or non-palpable axillary lymph-nodes (7; 8.3%) using SMN. In total, 94 markers were placed, in 74 patients (88.1%) only one marker was placed, whereas in 10 patients (11.9%) two markers were placed to correctly localize the lesion in the operating room. Most markers were placed using ultrasonographic guidance (69; 82.1%). Seventy-seven patients underwent breast-conserving surgery (91.7%) and 7 (8.3%) lymph node excision. In 10 cases (11.9%), the marker was accidentally displaced during surgery due to the use of magnetized instruments, although the specimen could be removed. In sum, all the markers were removed from the patients, although the marker retrieval rate, as we defined it (percentage of patients in whom the initial excised specimen contained the marker divided by the total number of patients), was 88.1%. The use of Sirius Pintuition® SMN for non-palpable breast tumors and non-palpable lymph nodes is feasible, with a retrieval rate of 88.1%
Inflammatory Myofibroblastic Tumor of the Pleura With Lung Metastases in an Elderly Patient.
No full text20.500.12530/8790
Descriptivo de los casos de citomegalovirus congénitos en un hospital terciario entre 2017-2023.
No full text20.500.12530/87855Cytomegalovirus infection (CMV) is the most common congenital infection in developed countries. The aim of our study was to describe the features of the children that have congenital CMV infection at our hospital for the last 6 years. A retrospective descriptive study was designed that included all the children with CMV congenital infection that were diagnosed at tertiary hospital of Madrid Community between 2017 and 2023. Twenty-two children were included. 54.5% have a prenatal diagnosis, 50% of them were in the third trimester, 25% at first trimester and 25% at the second. 22.7% were preterm. CMV was isolated in all the samples with CV more than 1000 copies/ml. When CMV was made in blood, 11/22 (50%) had a high CV. Only one newborn had a high CV at CRL. 44% have affectation at transfontanellar ultrasound evidenced by vasculopathy (62%), intraventricular hemorrhage (IVH) or periventricular calcifications (20%). 68% were asymptomatic, al though 20% had a retarded intrauterine growth (RIG) at birth or clinical features or analytical were objectified (neutropenia, thrombocytopenia, cholestasis). 33% got treatment with val ganciclovir and 33% had sequelae (hearing loss). CMV congenital infection is still a severe public health issue in developed countries. Most of the cases are mild or asymptomatic even though we should have high clinical suspicion with compatible symptoms and consistent maternal history in order to make an early diagnosis and treatment to prevent or reduce sequelae
Single-cell RNA sequencing in endometrial cancer: exploring the epithelial cells and the microenvironment landscape.
No full text20.500.12530/87908Single-cell RNA sequencing (scRNA-seq) technology has emerged as a powerful tool for dissecting cellular heterogeneity and understanding the intricate biology of diseases, including cancer. Endometrial cancer (EC) stands out as the most prevalent gynecological malignancy in Europe and the second most diagnosed worldwide, yet its cellular complexity remains poorly understood. In this review, we explore the contributions of scRNA-seq studies to shed light on the tumor cells and cellular landscape of EC. We discuss the diverse tumoral and microenvironmental populations identified through scRNA-seq, highlighting the implications for understanding disease progression. Furthermore, we address potential limitations inherent in scRNA-seq studies, such as technical biases and sample size constraints, emphasizing the need for larger-scale research encompassing a broader spectrum of EC histological subtypes. Notably, a significant proportion of scRNA-seq analyses have focused on primary endometrioid carcinoma tumors, underscoring the need to incorporate additional histological and aggressive types to comprehensively capture the heterogeneity of EC. By critically evaluating the current state of scRNA-seq research in EC, this review underscores the importance of advancing towards more comprehensive studies to accelerate our understanding of this complex disease
The association between periodontitis and cerebrovascular disease, and dementia. Scientific report of the working group of the Spanish Society of Periodontology and the Spanish Society of Neurology.
No full textThis article reviews the scientific evidence on the relationship between periodontitis and neurological disease, and particularly cerebrovascular disease and dementia. We also issue a series of recommendations regarding the prevention and management of periodontitis and these neurological diseases at dental clinics and neurology units. In response to a series of questions proposed by the SEPA-SEN working group, a literature search was performed, with no restrictions on study design, to identify the most relevant articles on the association between periodontitis and cerebrovascular disease and dementia from the perspectives of epidemiology, treatment, and the biological mechanisms involved in these associations. Periodontitis increases the risk of ischaemic stroke and Alzheimer dementia. Recurrent bacterial infections and increased low-grade systemic inflammation seem to be possible biological mechanisms underlying this association. Limited evidence suggests that various oral health interventions can reduce the future risk of cerebrovascular disease and dementia