Ecological study to estimate the prevalence of patients with acid sphingomyelinase deficiency in Spain. PREVASMD study.

Background And Objective: Prevalence studies of acid sphingomyelinase deficiency (ASMD) are scarce and different in Spain. The objective of the present study was to determine the estimated prevalence of patients diagnosed with ASMD (types A/B and B) in Spain.

Material And Methods: PREVASMD was a descriptive, multicenter, and ecological study involving 21 physicians from different specialties (mainly Internal Medicine, Paediatrics and Hematology), of different autonomous communities, with experience in ASMD management.

Real-World Experience with Diroximel Fumarate in Patients with Multiple Sclerosis: A Prospective Multicenter Study.

Background: Current literature and a real-world study suggest that diroximel fumarate (DRF) is safer than dimethyl fumarate (DMF) in the treatment of multiple sclerosis (MS). However, no real-world study to date has significantly addressed the efficacy of this treatment.

Objectives: This study aims to elucidate the safety, tolerability, and efficacy of DRF in a real-world setting, utilizing data from a Spanish national registry of patients commencing DRF therapy post-market introduction.

GI-RADS versus O-RADS in the differential diagnosis of adnexal masses: a systematic review and head-to-head meta-analysis.

Purpose: The aim of this study was to compare the diagnostic performance of the Gynecology Imaging Reporting and Data System (GI-RADS) and Ovarian-Adnexal Reporting and Data System (O-RADS) ultrasound (US) classification systems and assess their capacity to stratify the risk of malignancy in adnexal masses (AMs).

Methods: A comprehensive search of MEDLINE (PubMed), Scopus, Web of Science, and Google Scholar was conducted to identify articles published between January 2020 and August 2023. The quality of the studies, the risk of bias, and concerns regarding applicability were assessed using QUADAS-2.

A newly developed, easy-to-use prehospital drug-derived score compared with three conventional scores: A prospective multicenter study.

Introduction: The use of medications by emergency medical services (EMS) is increasing. Conventional scores are time-consuming and therefore difficult to use in an emergency setting. For early decision-making, an easy-to-use score based on the medications administered by the EMS may have prognostic value.

Basic action protocol for the outpatient management of chronic heart failure of the Spanish Society of Internal Medicine.

Chronic heart failure (CHF) represents a challenge for the healthy system due to its high prevalence, high burden of morbidity and mortality, and high consumption of health resources. To address this problem, it is necessary to develop efficient management strategies that include both hospital care and outpatient care. The primary objective is to stabilize the patient and prevent decompensation, with the consequent improvement in quality of life, reduction in hospital admissions and emergency department care, and, consequently, reduction in healthcare costs.

Prehospital point-of-care medication burden as a predictor of poor related outcomes in unselected acute diseases.

How prehospital medication predicts patient outcomes is unclear. The aim of this work was to unveil the association between medication burden administration in prehospital care and short, mid, and long-term mortality (2, 30, and 365 day) in unselected acute diseases and to assess the potential of the number of medications administered for short, mid, and long-term mortality prediction. A prospective, multicenter, ambulance-based, cohort study was carried out in adults with unselected acute diseases managed by emergency medical services (EMS).

Paraneoplastic glomerulonephritis and kidney infiltration by mantle cell lymphoma: A diagnostic challenge.

Mantle cell lymphoma (MCL) is a rare and aggressive type of lymphoma that can affect the kidneys. The disease can lead to kidney impairment, and glomerulonephritis (GN) is a rare but serious complication of MCL. We report a case of MCL with kidney interstitial infiltration and membranoproliferative glomerulonephritis with focal and segmental glomerulosclerosis.

Antibodies Against ZSCAN1 in Pediatric and Adult Patients With Non-Paraneoplastic ROHHAD Syndrome.

Objectives: To report the association of zinc finger and SCAN domain containing 1 antibodies (ZSCAN1-abs) with rapid-onset obesity, hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome in patients without tumor.

Methods: Patients with symptoms compatible with ROHHAD syndrome but without an associated tumor were selected from our database. Serum and CSF samples were examined for the presence of ZSCAN1-abs by an in-house cell-based assay.

Use of resources in elderly patients consulting the emergency department: analysis of the Emergency Department and Elder Needs Cohort (EDEN-21).

The elderly population frequently consults the emergency department (ED). This population could have greater use of EDs and hospital health resources. The EDEN cohort of patients aged 65 years or older visiting the ED allowed this association to be investigated.

Hereditary angioedema in Spain: medical care and patient journey.

Background: Hereditary angioedema due to C1 inhibitor deficiency (HAE-C1INH) is a genetic rare disease characterized by recurrent, transient and unpredictable episodes of cold, non-pruriginous oedema without associated urticaria. The characteristics of the disease have a considerable impact on the quality of life of patients. The aim of this study was to increase understanding of the patient journey of HAE in Spain.

Clinical impact of remote heart failure management using the multiparameter ICD HeartLogic alert.

Introduction And Objectives: The multiparametric implantable cardioverter-defibrillator HeartLogic index has proven to be a sensitive and timely predictor of impending heart failure (HF) decompensation. We evaluated the impact of a standardized follow-up protocol implemented by nursing staff and based on remote management of alerts.

Methods: The algorithm was activated in HF patients at 19 Spanish centers.

Short-term outcomes of a multicentre prospective study using a "visible" polyvinylidene fluoride onlay mesh for the prevention of midline incisional hernia.

Introduction: Prophylactic meshes in high-risk patients prevent incisional hernias, although there are still some concerns about the best layer to place them in, the type of fixation, the mesh material, the significance of the level of contamination, and surgical complications. We aimed to provide answers to these questions and information about how the implanted material behaves based on its visibility under magnetic resonance imaging (MRI).

Method: This is a prospective multicentre observational cohort study.

Effects of the COVID-19 pandemic on gestational diabetes in Castilla-La Mancha (Spain).

Subject-matter: To assess the effect of the 2019 coronavirus (COVID-19) pandemic on gestational diabetes (GDM).

Material And Methods: In this retrospective, multicentre, non-interventional study carried out in Castilla-La Mancha, Spain, we compared 663 women with GDM exposed to the pandemic (pandemic group), with 622 women with GDM seen one year earlier (pre-pandemic group). The primary endpoint was a Large for Gestational Age (LGA) newborn as an indicator of poor GDM control.

Gender Differences among Nurses in Managing Arterial Puncture-Related Pain: A Multicenter Cross-Sectional Study.

There is evidence that healthcare can be executed differentially depending on the gender of patients, researchers, and clinicians. The aim was to analyze the possible existence of nursing gender differences in pain management produced by arterial puncture for blood gas analysis. A cross-sectional, multicenter study designed was conducted in Castilla-la Mancha (Spain).

Effectiveness of Body Weight-Supported Gait Training on Gait and Balance for Motor-Incomplete Spinal Cord Injuries: A Systematic Review with Meta-Analysis.

Objective: This review aims to analyse the effectiveness of body weight-supported gait training for improving gait and balance in patients with motor-incomplete spinal cord injuries.

Method: Relevant articles were systematically searched in electronic databases to identify randomised controlled trials of body weight-supported gait training (either with methods of robotic, manual, and functional electrical stimulation assistance) versus conventional physical therapy or no intervention. Subjects were >16 years-old with motor-incomplete spinal cord injury (AIS C or D).

ROHHAD syndrome spectrum in an adult: a possible new variant.

https://bit.ly/49AN3Vv.

National observational study about the surgical treatment of anal fistula: Does the kind of hospital modify the results?

Introduction: Performing the surgical procedure in a high-volume center has been seen to be important for some surgical procedures. However, this issue has not been studied for patients with an anal fistula (AF).

Material And Methods: A retrospective multicentric study was performed including the patients who underwent AF surgery in 2019 in 56 Spanish hospitals.

Impact of First Wave of COVID-19 Pandemic on Mortality at Emergency Department in Older Patients with COVID and Non-COVID Diagnoses.

Introduction: Mortality in emergency departments (EDs) is not well known. This study aimed to assess the impact of the first-wave pandemic on deaths accounted in the ED of older patients with COVID and non-COVID diseases.

Methods: We used data from the Emergency Department and Elderly Needs (EDEN) cohort (pre-COVID period) and from the EDEN-COVID cohort (COVID period) that included all patients ≥65 years seen in 52 Spanish EDs from April 1 to 7, 2019, and March 30 to April 5, 2020, respectively.

The Relationship between General Movements and Risk Factors in Moderate-Late Preterm Infants: A Prospective Cohort Study.

Introduction: Moderate-late preterm infants constitute the largest segment of preterm births globally. While previously considered to have a low neurological risk, recent research has uncovered an elevated incidence of neurodevelopmental conditions in this group. This study aimed to assess the relationship between the general movement assessment and birth-related risk factor-based tools in moderate-late preterm infants.

Waldenström's Macroglobulinemia and Cryoglobulinemic Glomerulonephritis: An Unusual Case of Monoclonal Gammopathy of Renal Significance.

Cryoglobulins are immunoglobulins that precipitate at temperatures below 37 °C and dissolve upon reheating. They can induce small-vessel vasculitis with renal involvement. Cryoglobulinemic glomerulonephritis is a rare manifestation that occurs in patients with monoclonal gammopathy, specifically Waldenström's macroglobulinemia.

A Randomized Trial of Dolutegravir Plus Darunavir/Cobicistat as a Switch Strategy in HIV-1-Infected Patients With Resistance to at Least 2 Antiretroviral Classes.

Background: Suppressed patients with drug-resistant HIV-1 require effective and simple antiretroviral therapy to maintain treatment adherence and viral suppression.

Methods: This randomized, open-label, noninferiority, multicenter pilot study involved HIV-infected adults who met the following criteria: confirmed HIV-1 RNA <50 copies/mL for ≥6 months preceding the study randomization, treatment with at least 3 antiretroviral drugs, and a history of drug resistance mutations against at least 2 antiretroviral classes but remaining fully susceptible to darunavir (DRV) and integrase inhibitors. Participants were randomized 1:1 to switch to dolutegravir (DTG; 50 mg once per day) plus DRV boosted with cobicistat (DRV/c; 800/150 mg once per day; 2D group) or continue with their baseline regimen (standard-of-care [SOC] group).