Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin.

Hemophagocytic lymphohistiocytosis (HLH) is a rare genetic hyperinflammatory syndrome that occurs early in life. Macrophage activation syndrome (MAS) usually refers to a secondary form of HLH associated with autoimmunity, although there are other causes of secondary HLH, such as infections and malignancy. In this article, we reviewed the concepts, epidemiology, clinical and laboratory features, diagnosis, differential diagnosis, prognosis, and treatment of HLH and MAS.

Hypertrophic Osteoarthropathy: A Case of an Undifferentiated Polyarthritis.

Hypertrophic osteoarthropathy is a paraneoplastic syndrome and is considered an important secondary cause of rheumatic disease. It typically manifests as tibial and femoral bone pain, with arthralgia or synovitis of adjacent joints also being common findings. Usually, musculoskeletal symptoms accompany the course of the disease, disappearing with treatment of the neoplasm and recurring coincidentally with the tumor relapse.

Prevalence of Sleep Disturbances in Latin American Populations and Its Association with Their Socioeconomic Status-A Systematic Review and a Meta-Analysis.

Background: The worldwide increase in the prevalence and incidence of sleep disturbances represents a major public health issue. Among multiple determinants affecting sleep health, an individual's socioeconomic status (SES) is the most ignored and underestimated throughout the literature. No systematic review on the relation between SES and sleep health has been previously conducted in Latin America.

Lymphangioleiomyomatosis With Atypical Presentation Following Pneumothorax: A Case Report.

Lymphangioleiomyomatosis (LAM) is a rare systemic disease that typically presents like cystic lung disease. High-resolution computed tomography (CT) is the recommended imaging technique, with cysts being the hallmark: typically multiple, well-circumscribed, thin-walled, with a variable diameter (usually <2 cm) and widespread in distribution. The gold standard for diagnosis is a biopsy.

Wolfram Syndrome: A Curious Case of Repetitive Loss of Consciousness.

Wolfram syndrome is a rare, multisystemic, progressive, and autosomal-recessive genetic disease, characterized by diabetes mellitus and diabetes insipidus, optic nerve atrophy, deafness, and other neurological signs. The diagnosis is usually based on history and clinical manifestations but genetic tests are necessary for confirmation. Currently, there are no treatments available to cure or delay disease progression.

Development of a novel electrochemical biosensor based on plastic antibodies for detection of STEAP1 biomarker in cancer.

STEAP1 is a cell surface protein of the STEAP family whose main function focuses on intercellular communication and cell growth. STEAP1 is considered a promising putative biomarker and a candidate target for prostate cancer treatment. For specific and selective detection of STEAP1, a molecularly imprinted polymers (MIP) was developed on a screen-printed electrode (C-SPE) whose surface was modified with a nanocomposite based on carbon nanotubes decorated with dendritic platinum nanoparticles (CNTs- PAH /Pt).

The Impact of an Intensivist-Led Critical Care Transition Program.

Objective: Evaluate the impact of a post-discharge critical care transition program (CTP) on intensive care unit (ICU) readmission, in-hospital mortality, and six-month survival.

Methods: This was a prospective observational, single-center study, with a before-after design, in a critical care department in a tertiary hospital in Northern Portugal. Critically ill patients with ICU stay > 48 h or intermediate care stay >72 h or tracheostomized patients were included in the program.

[Pre-Exposure Prophylaxis for Human Immunodeficiency Virus in the Medical Curricula in Portugal: A Cross-Sectional Analysis].

Introduction: Pre-exposure prophylaxis (PrEP) has gained relevance as a method of prevention for HIV in certain people and settings. Following the publication of the guideline on PrEP prescribing in Portugal, we aimed to assess the knowledge of Portuguese Medical Students about PrEP.

Material And Methods: An online survey was sent to Medical students of Portuguese Medical Schools.

Molecular cytogenetic analyses reveal extensive chromosomal rearrangements and novel B chromosomes in Moenkhausia (Teleostei, Characidae).

The cytogenetic characteristics of five fish species of the Moenkhausia are described, based on the analysis of specimens collected in different headwater. All the species analyzed presented 2n=50 chromosomes. The C-banding revealed a similar distribution pattern of heterochromatic blocks in all the species, except Moenkhausia nigromarginata.

The non-monotypic status of the neotropical fish genus Hemiodontichthys (Siluriformes, Loricariidae) evidenced by genetic approaches.

The combination of cytogenetic and molecular data with those traditionally obtained in areas like systematics and taxonomy created interesting perspectives for the analysis of natural populations under different aspects. In this context, this study aimed to evaluate the genetic differentiation among populations of the genus Hemiodontichthys Bleeker, 1862, through combined genetic techniques and included the analysis of populations sampled in the Araguaia River, Guamá River, Madeira River and two populations from the Purus River. Hemiodontichthys samples from the two localities in Purus River were also karyotyped in order to address the degree of chromosomal variation between populations.

Boosting bioethanol production from Eucalyptus wood by whey incorporation.

The mixture of Eucalyptus globulus wood (EGW) and cheese whey powder (CWP) was proposed for intensification of simultaneous saccharification and fermentation (SSF) at high temperature and solid loadings using the industrial Saccharomyces cerevisiae Ethanol Red® strain. High ethanol concentration (93 g/L), corresponding to 94% ethanol yield, was obtained at 35 °C from 37% of solid mixture using cellulase and β-galactosidase enzymes (24.2 FPU/g and 20.

Improving succinic acid production by Actinobacillus succinogenes from raw industrial carob pods.

Carob pods are an inexpensive by-product of locust bean gum industry that can be used as renewable feedstock for bio-based succinic acid. Here, for the first time, unprocessed raw carob pods were used to extract a highly enriched sugar solution, afterwards used as substrate to produce succinic acid using Actinobacillus succinogenes. Batch fermentations containing 30g/L sugars resulted in a production rate of 1.

Carob pod water extracts as feedstock for succinic acid production by Actinobacillus succinogenes 130Z.

Carob pods are a by-product of locust bean gum industry containing more than 50% (w/w) sucrose, glucose and fructose. In this work, carob pod water extracts were used, for the first time, for succinic acid production by Actinobacillus succinogenes 130Z. Kinetic studies of glucose, fructose and sucrose consumption as individual carbon sources till 30g/L showed no inhibition on cell growth, sugar consumption and SA production rates.

Acute renal failure as a form of presentation of sarcoidosis in a young adult: a case report.

Introduction: Sarcoidosis is a systemic granulomatous disease. Renal involvement is a rare initial presentation of this disease. Few articles on renal involvement as an initial presentation of sarcoidosis have been published in the literature.

Symptoms other than pain in palliative care in Portugal.

Objective: To study the burden of patients' symptoms other than pain and their treatment by Portuguese palliative care teams.

Methods: Of the 21 Portuguese palliative care teams identified, 10 accepted to participate. Data from all patients observed on the 18th week of 2011 were collected.

Succinic acid production from glycerol by Actinobacillus succinogenes using dimethylsulfoxide as electron acceptor.

Glycerol, a highly abundant byproduct of the biodiesel industry, constitutes today a cheap feedstock for biobased succinic acid (SA) production. Actinobacillus succinogenes is one of the best SA producers. However, glycerol consumption by this biocatalyst is limited because of a redox imbalance during cell growth.

A cross-sectional survey of pain in palliative care in Portugal.

Objective: The purpose of this work is to study the prevalence, intensity, and treatment of pain in Portuguese palliative care teams.

Methods: Twenty-one palliative care teams were invited to participate in a cross-sectional survey. Ten of these accepted and were included in the study.

A cross-sectional survey of the activity of palliative care teams in Portugal.

Of the 21 Portuguese teams identified, 10 accepted to participate in the study. A total of 164 patients were included with a median of 15.5 per team (4-32).

An approach of hypertension prevalence in a sample of 5381 Portuguese children and adolescents. The AVELEIRA registry. "Hypertension in children".

Introduction: The distribution of blood pressure (BP) in children and adolescents remains relatively unknown. Thus, it is imperative to ascertain BP distribution at a young age, which constitutes the main objective of this registry.

Methods: Cross-sectional assessment of BP in 5381 Portuguese children and adolescents, mean age 12.

Blood pressure distribution of a sample of healthy Portuguese children and adolescents: the AVELEIRA registry.

Introduction: The distribution of blood pressure in Portuguese children and adolescents remains relatively unknown. Given the prevalence of hypertension in Portugal and the epidemiological trends for most cardiovascular diseases, it is important to ascertain blood pressure distribution at young ages, which constitutes the main objective of this registry.

Methods: A sample of 1618 children and adolescents from the Central region of Portugal was included in this survey.

Usefulness of the ankle-brachial index in assessing vascular function in normal individuals.

Introduction: The ankle-brachial index (ABI) has been widely used in the diagnosis of established obstructive peripheral artery disease. However, its applicability in the earlier stages of vascular dysfunction lacks experimental demonstration. Our aim was thus to evaluate the potential of this indicator in the study of vascular function in a normal population.

Guarana (Paullinia cupana var. sorbilis), an anciently consumed stimulant from the Amazon rain forest: the seeded-fruit transcriptome.

Guarana (Paullinia cupana var. sorbilis) is a plant native to the central Amazon basin. Roasted seed extracts have been used as medicinal beverages since pre-Colombian times, due to their reputation as stimulants, aphrodisiacs, tonics, as well as protectors of the gastrointestinal tract.

Diversity of microfungi in the phylloplane of plants growing in a Mediterranean ecosystem.

Mediterranean ecosystems have not been investigated as natural habitats for microorganisms in general, and microfungi in particular. Plants harvested in "Serra da Arrábida" (38 degrees 27' N, 9 degrees 02' W), a Mediterranean ecosystem in Portugal, were analyzed for the filamentous microfungi inhabiting their surface. Two field locations with distinct climatic characteristics were studied: 'Fonte do Veado' (38 degrees 28'50" N, 9 degrees 0'17" W; 300 m elevation) located on the northern slope, and 'Mata do Solitário' (38 degrees 27'55" N, 8 degrees 59'35" W; 50 m elevation), on the southern slope.