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.

Biomarkers of response to ocrelizumab in relapsing-remitting multiple sclerosis.

Objective: To ascertain the changes of serum neurofilament light chain (sNfL) and glial fibrillary acidic protein (sGFAP) values in relapsing-remitting multiple sclerosis (RRMS) patients treated with ocrelizumab and their association with treatment response.

Methods: Multicenter prospective study including 115 RRMS patients initiating ocrelizumab treatment between February 2020 and March 2022 followed during a year. Serum samples were collected at baseline and every 3 months to measure sNfL and sGFAP levels using single-molecule array (SIMOA) technology.

Impact of pharmaceutical care on hospital readmissions for heart failure: a randomised trial.

Objectives: To evaluate the impact of pharmaceutical care on the number of readmissions and visits to the emergency department due to heart failure 30 days after hospital discharge, based on a programme of continuous pharmaceutical care throughout the care process, and to assess the differences between the control and intervention groups at 90 days after discharge (number of readmissions and visits to the emergency department, time from discharge to new readmission or visit to the emergency department).

Methods: A single-centre experimental longitudinal prospective open and parallel-group study with balanced randomisation (1:1) was carried out in a tertiary hospital in Spain. Patients with a diagnosis of primary or decompensated heart failure admitted to the Cardiology Service or the Heart Failure and Vascular Risk Unit were recruited between March 2019 and November 2021 and randomly assigned, using a randomised block model, to the control (standard care) or intervention (continuing care model) groups.

Introduction to iodinated contrasts: Properties, intravenous administration and distribution throughout the body.

Iodinated contrast media enable greater attenuation of vascular and visceral structures in radiological studies and are widely used due to their high degree of safety, easy administration, acceptable level of tolerance, wide distribution and rapid excretion, qualities determined by their composition. Automated power injectors are used to administer contrast media. Once injected, contrast is distributed throughout the body, remaining in the intravascular space during its first passage before reaching the organs and spreading into the extracellular interstitial space, followed by a recirculation phenomenon.

Multi-energy CT and iodinated contrast.

Spectral CT acquires images with the emission or detection of two separate energy spectra. This enables material decomposition due to the photoelectric effect (prevalent in low-energy photons) and Compton scattering (prevalent in high-energy photons). Iodine and other materials with high atomic numbers appear more hyperdense on low-energy monoenergetic images because of the direct relation between the photoelectric effect and the Z value.

Polymorphisms of the Vitamin D Binding Protein (VDBP) and Free Vitamin D in Patients with Cystic Fibrosis.

Objectives/background: Vitamin D-binding protein (VDBP) and free vitamin D are new markers that are being studied as a possible markers of vitamin D status. The main aim of our study was to analyze the VDBP genotype and quantify the levels of free vitamin D in a sample of cystic fibrosis (CF) patients.

Methods: We conducted a multicenter, cross-sectional, and prospective study including patients with CF and exocrine pancreatic insufficiency who were clinically stable.

Preclinical gout is common in the patient with stage 3-5 chronic kidney disease. Relevance of musculoskeletal ultrasound.

Background: One in 10 patients with hyperuricemia may develop gout over time, with urate deposition sometimes asymptomatic. Recent reviews and guidelines support ultrasound (US) to assess asymptomatic hyperuricemic (AH) patients to detect gout lesions, showing double contour (DC) and tophus the highest specificities and positive predictive values. Hyperuricemia and gout are common in chronic kidney disease (CKD), especially with glomerular filtration rate (GFR) <60, and both are associated with worse prognosis, although treatment of AH in CKD is not yet recommended in all guidelines.

Sustained Drug-Free Remission in Giant Cell Arteritis.

Objetive: To evaluate the frequency and timing of sustained drug-free remission (SDFR) in patients with giant cell arteritis (GCA) and to identify potential predictive factors of this outcome.

Methods: Retrospective review of all patients included in the large Spanish multicentre registry for GCA (ARTESER) with at least two years of follow-up. SDFR was defined as the absence of typical signs, symptoms, or other features of active GCA for ≥12 months after discontinuation of treatment.

Persistence, Effectiveness, and Safety of Upadacitinib in Crohn's Disease and Ulcerative Colitis in Real Life: Results From a Spanish Nationwide Study (Ureal Study).

Introduction: Real-world data on the effectiveness of upadacitinib for inflammatory bowel disease (IBD) are limited. To assess upadacitinib persistence, effectiveness, and safety in a real-world scenario.

Methods: Retrospective multicenter study of patients with IBD who received upadacitinib before 31st December 2022 and at least 12 weeks before the recruitment date.

Impact of SARS-CoV-2 vaccination in patients with vascular liver diseases: Observations from a VALDIG multicenter study.

Background & Aims: Patients with vascular liver diseases (VLD) are at higher risk of both severe courses of COVID-19 disease and thromboembolic events. The impact of SARS-CoV-2 vaccination in patients with VLD has not been described and represents the aim of our study.

Methods: International, multicenter, prospective observational study in patients with VLD analyzing the incidence of COVID-19 infection after vaccination, severity of side effects, occurrence of thromboembolic events and hepatic decompensation.

Ceftaroline for bloodstream infections caused by methicillin-resistant Staphylococcus aureus: a multicentre retrospective cohort study.

Objectives: To evaluate the effectiveness of ceftaroline vs. vancomycin or daptomycin in the treatment of methicillin-resistant Staphylococcus aureus bloodstream infections (BSIs) (MRSA-BSIs).

Methods: This multicentre retrospective study conducted in 15 Spanish hospitals included data from the first MRSA-BSIs of adult patients between January 2019 and December 2022.

Donor-derived cell-free DNA as a new biomarker for cardiac allograft rejection: A prospective study (FreeDNA-CAR).

Background: There is a long-standing need for a noninvasive biomarker that allows monitoring of cardiac allograft rejection, avoiding the need for periodic endomyocardial biopsies (EMB).

Methods: Multicenter, observational, prospective study, performed between 2019 and 2023 (NCT04973943). All patients underwent 7 per-protocol surveillance EMB during the first postheart transplantation year.

Predictors and implications of renal injury after CD19 chimeric antigen receptor T-cell therapy.

Chimeric Antigen Receptor (CAR) T cells targeting CD19 induce durable remissions in patients with relapsed or refractory non-Hodgkin lymphoma (NHL), but many patients experience treatmentrelated toxicity. Cytokine release syndrome and immune effector cell-associated neurologic syndrome are extensively characterized. However, limited data exist on the burden, predictors, and implications of acute kidney injury (AKI) after CAR T cell therapy.

Burden of incidental cerebral aneurysms on lifestyle and quality of life: a survey of patients in expectant management (the SPICE Study).

Background: The increasing availability of neuroimaging tests has led to a rise in the identification of incidental unruptured intracranial aneurysms (UIAs). Their management is under debate, with no consensus on their follow-up strategy, which can cause anxiety in patients. Our aim is to evaluate the impact of diagnosis and imaging follow-up on daily activities and quality of life.

Anthracyclines-induced cardiotoxicity in patients with early breast cancer carrying germline BRCA1/2 mutation: the BRCAN study.

Background: BRCA1/2 genes play a critical role in genome stability and DNA repair. In animal models, loss of cardiomyocyte-specific BRCA1/2 is associated with DNA damage, apoptosis, cardiac dysfunction, and mortality following anthracycline exposure. However, whether these preclinical findings translate to humans remains unclear.

[Translated article] Trichotillomania Treatment Update.

Trichotillomania (TTM) is an obsessive-compulsive disorder in which affected individuals recurrently pull-out hair from any region of the body, causing hair loss or alopecia. The management of TTM is a therapeutic challenge for dermatologists and consists of a combination of pharmacological and non-pharmacological alternatives. Cognitive-behavioral therapy has successfully been used to treat TTM.

Acne Keloidalis Nuchae: A Therapeutic Challenge for Dermatologists.

Acne keloidalis nuchae (AKN) is a chronic inflammatory dermatosis of the scalp that causes scarring alopecia. The etiology of AKN has been associated with an immune response vs. follicular antigens.