Porto biomedical journal最新文献

Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a heterogeneous group of rare diseases characterized by necrotizing inflammation predominantly of small vessels and the presence of these circulating antibodies. AAV comprises three important diseases, namely granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis, which affect multiple organ systems, significantly affecting patients' quality of life and survival. The diagnosis is established according to the clinical manifestations, detectable ANCA, and histopathology findings. Primary treatment strategies are adapted to the severity of the disease and based on immunosuppression with corticosteroids and cyclophosphamide, with increasing adoption of new, less toxic agents aimed at sustained remission of the disease, such as rituximab, methotrexate, and mycophenolate mofetil. Several international medical organizations have proposed recommendations for diagnosing and managing these diseases to standardize the procedures. In this study, we provide an up-to-date European perspective on AAV management, compiling current and relevant information regarding its epidemiology, symptoms, diagnosis, treatment strategies, and prognosis.

Background: Health care workers (HCW) are presumably exposed to a higher risk of infection by SARS-CoV-2 and could possibly represent a source of transmission to susceptible patients. Thus, characterization of SARS-CoV-2 infection among HCW is necessary to better understand the determinants of viral transmission and properly implement strategies to prevent dissemination and protect HCW and vulnerable patients. The aim of this study was to estimate the seroprevalence of antibodies against SARS-CoV-2 in a Portuguese tertiary hospital, in the period of July 2020 to March 2021, before the generalized use of the SARS-CoV-2 vaccine, characterize its evolution over time, and identify risk factors associated with seroconversion.

Methods: HCW were approached to collect serum samples for qualitative SARS-CoV-2 antibody testing and completion of an online survey capturing demographics, previous symptoms, and details of health care and community exposure. Odds ratio with bivariable and multivariable logistic regression was used to assess characteristics associated with seroprevalence.

Results: One thousand HCW were included for analysis. Two hundred nineteen HCW (22%) were seropositive for immunoglobulin G against SARS-CoV-2, and 166 (17%) were seropositive for immunoglobulin M, most of whom reported a previous diagnosis of SARS-CoV-2 infection. The risk factors associated with seroconversion included a previous COVID-19 diagnosis, contact with patients, occupational contact with colleagues, and outside contact. However, in a multivariate logistic regression analysis, only a previous diagnosis and outside contact were associated with seroconversion. Seropositivity decreased over time, especially 28 weeks after infection.

Conclusion: HCWs have a high seroprevalence for SARS-CoV-2 infection, probably due to a combination of health care and community exposure. Seropositivity decreases over time, but further studies are needed to better understand our adaptive immune response.

Background: Hemoglobinopathies and thalassemias are widely prevalent autosomal inherited recessive disorders of the structure and synthesis of hemoglobin, respectively. Given the regional heterogeneity of these disorders, this study was undertaken to elucidate the patterns and prevalence of these disorders from this region.

Methods: This was a tertiary-care hospital-based study in southern India over 4 years. Screening for hemoglobin (Hb) disorders was done using Hb high-performance liquid chromatography in patients based on initial screening of complete blood count parameters and for clinically indicated cases.

Results: A normal Hb HPLC pattern was observed in 404 (72.1%) and abnormal in 156 (27.9%) of 560 cases studied. The abnormalities seen were heterozygous β-thalassemia in 73 (46.8%), homozygous β-thalassemia in 19 (12.2%), heterozygous α-thalassemia in 7 (4.5%), HbH disease and heterozygous δβ-thalassemia in 1 (0.6%) each, sickle cell trait in 9 (5.8%), sickle cell anemia in 8 (5.1%), sickle β-thalassemia in 17 (10.9%), HbS+ Hb D-Punjab in 1 (0.6%), heterozygous HbE in 6 (3.8%), homozygous HbE in 2 (1.3%), HbE β-thalassemia in 3 (1.9%), Hb J-Meerut in 1 (0.6%), Hb Kirksey in 4 (2.6%), unknown α-hemoglobinopathy in 2 (1.3%), and Hb Lepore in 2 (1.3%) cases. Most of the patients were from the neighboring districts, and some were referred from other states.

Conclusion: The most common hemoglobin disorders were heterozygous β-thalassemia in 73 cases (46.8%) and sickle hemoglobinopathy in 35 cases (22.4%). A heterogeneous group of hemoglobin disorders, including uncommon α-hemoglobinopathies, was found in the study population, likely due to the referral of patients from various regions.

Light chain amyloidosis (AL) is a complex disorder defined by the extracellular deposition of insoluble amyloid fibrils formed by intact or fragmented immunoglobulin light chains, leading to cell dysfunction, rapid organ deterioration, and, ultimately, death. Although the clinical presentation of AL is directly connected to organ involvement, signs and symptoms of AL are frequently nonspecific, misinterpreted, and late recognized. Thus, an early diagnosis combined with effective therapies to cease disease progression and rescue organ function is essential. The aim of this study was to assess the knowledge and characterize the current clinical practice regarding AL diagnosis and referral among Portuguese physicians. A Delphi-like panel (one round only) with a group of national experts from different medical specialties (cardiology, hematology, internal medicine, nephrology, and neurology) was carried out online, in which 30 statements were classified using a 4-point Likert scale. For each statement, the consensus level was set at 70% for "fully agree/disagree" and the majority level was defined as >70% in agreement or disagreement. Although the results suggest the existence of adequate general knowledge of AL amyloidosis, they also disclosed the necessity to raise awareness for this disease. Overall, this Delphi panel revealed a high lack of consensus regarding the diagnosis and early management of patients with AL among different specialties despite the qualified majority obtained in 26 statements. An optimized strategy for AL early diagnosis, transversal to several medical fields, is urgently needed. Moreover, referral centers with access to diagnostic technology and a network of diverse specialties should be established to foster an early diagnosis and better disease approach to boost the possibility of a better outcome for patients with AL.

The cardiovascular response to exercise has long been a focus of interest. Over a century ago, the first descriptions of electrocardiographic changes occurring during exercise highlighted the possible relevance of this dynamic assessment. In this background, the inception of the Bruce protocol circa 60 years ago allowed for a major leap in this field by providing a standardized framework with which to address this issue, by means of an integrated and structured methodology. Since then, exercise stress testing with electrocardiographic monitoring (ExECG) has become one of the most widely appraised tests in cardiovascular medicine. Notably, past few decades have been profoundly marked by substantial advances in the approach to cardiovascular disease, challenging prior notions concerning both its physiopathology and overall management. Among these, the ever-evolving presentations of cardiovascular disease coupled with the development and implementation of several novel diagnostic modalities (both invasive and noninvasive) has led to a shifting paradigm in the application of ExECG. This technique, however, has continuously shown to be of added value across various momentums of the cardiovascular continuum, as depicted in several contemporary guidelines. This review provides a pragmatical reflexion on the development of ExECG, presenting a comprehensive overview concerning the current role of this modality, its challenges, and its future perspectives.