Residual enzymatic activity as a prognostic factor in patients with Gaucher disease type 1: correlation with Zimran and GAUSS-I index and the severity of bone disease.

Background: Gaucher disease (GD) is an autosomal recessive disorder produced by mutations in the glucocerebrosidase gene (GBA), causing storage of glucosylceramide in reticuloendothelial cells in multiple organs. Traditionally, the prediction of the phenotype based on the genotype has been reported to be limited.

Subjects And Methods: We investigated the correlation between the enzymatic residual activity (ERA) and the phenotype at diagnosis of the disease in 45 GD Spanish patients (44 with type I and 1 with type III GD).

Pterostilbene Decreases the Antioxidant Defenses of Aggressive Cancer Cells In Vivo: A Physiological Glucocorticoids- and Nrf2-Dependent Mechanism.

Aims: Polyphenolic phytochemicals have anticancer properties. However, in mechanistic studies, lack of correlation with the bioavailable concentrations is a critical issue. Some reports had suggested that these molecules downregulate the stress response, which may affect growth and the antioxidant protection of malignant cells.

Improvement of antibiotic therapy and ICU survival in severe non-pneumococcal community-acquired pneumonia: a matched case-control study.

Introduction: We aimed to compare intensive care unit mortality due to non-pneumococcal severe community-acquired pneumonia between the periods 2000-2002 and 2008-2014, and the impact of the improvement in antibiotic strategies on outcomes.

Methods: This was a matched case-control study enrolling 144 patients with non-pneumococcal severe pneumonia: 72 patients from the 2000-2002 database (CAPUCI I group) were paired with 72 from the 2008-2014 period (CAPUCI II group), matched by the following variables: microorganism, shock at admission, invasive mechanical ventilation, immunocompromise, chronic obstructive pulmonary disease, and age over 65 years.

Results: The most frequent microorganism was methicillin-susceptible Staphylococcus aureus (22.

Intestinal Microbiota and Celiac Disease: Cause, Consequence or Co-Evolution?

It is widely recognized that the intestinal microbiota plays a role in the initiation and perpetuation of intestinal inflammation in numerous chronic conditions. Most studies report intestinal dysbiosis in celiac disease (CD) patients, untreated and treated with a gluten-free diet (GFD), compared to healthy controls. CD patients with gastrointestinal symptoms are also known to have a different microbiota compared to patients with dermatitis herpetiformis and controls, suggesting that the microbiota is involved in disease manifestation.

The Concept and the Epidemiology of Diabetic Nephropathy Have Changed in Recent Years.

Diabetes Mellitus (DM) is a growing worldwide epidemic. It was estimated that more than 366 million people would be affected. DM has spread its presence over the world due to lifestyle changes, increasing obesity and ethnicities, among others.

Short Sleep Duration Is Related to Emerging Cardiovascular Risk Factors in Obese Children.

Objective: The aim of the present study was to evaluate the influence of sleep duration on cardiovascular risk factors in obese children.

Methods: Cross-sectional analysis of 90 obese children ages 7 to 16 years. Anthropometric and clinical evaluation with specification of dietary and lifestyle habits was carried out during an office visit.

Impact of nutritional status on the pharmacokinetics of erlotinib in rats.

Malnourishment is a complex condition in which physiopathological changes take place in multiple systems as a result of energy, protein and nutrient deficiency. The purpose of this study was to evaluate, using an experimental animal model, the impact of nutritional status on the pharmacokinetic profile of erlotinib, a reversible, highly selective, human epidermal growth factor receptor (HER1/EGFR) tyrosine kinase inhibitor. Two groups of rats -WN (well-nourished) and UN (undernourished) - were fed with different diets for 23-26 days.

Left ventricular diastolic function and cardiometabolic factors in obese normotensive children.

Background And Aim: Left ventricular (LV) hypertrophy and diastolic function have been found to be associated with obesity and hypertension in adults. However, there are scarce data about the association of obesity itself to cardiac alteration in children. The aim of this study was to detect early changes in LV structure and function in obese children and whether they are associated with the biomarkers of metabolic risk and endothelial activation.

Subclinical hypothyroidism and cardiovascular risk.

Background: Subclinical hypothyroidism (SCH) has been suggested to be associated with increased cardiovascular risk by different mechanisms. Several cardiovascular risk factors have been analysed, but yielded controversial results.

Objectives: We aimed to analyse whether there are differences in several cardiovascular risk markers, such as lipids, inflammatory parameters: plasma viscosity (PV), fibrinogen and C reactive protein (CRP); homocysteine (Hcy) and red blood cell distribution width (RDW), when comparing SCH and controls.

Association of metabolic syndrome and its components with hyperuricemia in a Mediterranean population.

Several studies have found an association between hyperuricemia and metabolic syndrome (MS), although there are discrepancies as to which MS components play a pivotal role in this association. We aimed to investigate the association between serum uric acid (SUA) levels and MS in a Mediterranean population (eastern Spain). We performed a case-control study of 71 patients with MS and 122 healthy controls.

Enhanced reduction in oxidative stress and altered glutathione and thioredoxin system response to unsaturated fatty acid load in familial hypercholesterolemia.

Objectives: Familial hypercholesterolemia (FH) is characterized by increased oxidative stress (OS) levels. In the postprandial state, lipids and lipoproteins modulate OS status through their impact on pro-oxidant and antioxidant mechanisms. The objective of this study was to evaluate in patients with FH the response to an unsaturated oral fat load test (OFLT) by analyzing the mRNA levels of genes involved in the glutathione and thioredoxin antioxidant systems.

Resistin: insulin resistance to malignancy.

Adipose tissue is recognized as an endocrine organ that secretes bioactive substances known as adipokines. Excess adipose tissue and adipose tissue dysfunction lead to dysregulated adipokine production that can contribute to the development of obesity-related co-morbidities. Among the various adipokines, resistin, which was initially considered as a determinant of the emergence of insulin resistance in obesity, has appeared as an important link between obesity and inflammatory processes.

Association of erythrocyte deformability with red blood cell distribution width in metabolic diseases and thalassemia trait.

Increased red blood distribution width (RDW) in anemia is related to disturbances in the cellular surface/volume ratio, usually accompanied by morphological alterations, while it has been shown in inflammatory diseases that the activity of pro-inflammatory cytokines disturbing erythropoiesis increases RDW. Recently it has been reported that higher RDW is related with decreased erythrocyte deformability, and that it could be related with the association of RDW and increased risk of cardiovascular diseases. In order to analyze the influence of morphological alterations and proinflammatory status on the relationship between RDW and erythrocyte deformability, we analyzed erythrocyte deformability along with RDW and other hematological and biochemical parameters in 36 α-thalassemia, 20 β-thalassemia, 20 δβ-thalassemia trait carriers, 61 metabolic syndrome patients and 76 morbidly obese patients.

Red blood cell distribution width is not related with inflammatory parameters in morbidly obese patients.

Objective: Red blood cell distribution width (RDW) is a hematological parameter that has been studied in several clinical settings and has been found to be related to both anemia and inflammatory status. As obesity is related to increased inflammatory pattern, we aimed to analyze the RDW in this setting.

Methods: We determined hematological and inflammatory parameters in morbidly obese patients before bariatric surgery (n=142) and normo-weight controls (n=144).

Development and application to clinical practice of a validated HPLC method for the analysis of β-glucocerebrosidase in Gaucher disease.

The main objective of our study is to develop a simple, fast and reliable method for measuring β-glucocerebrosidase activity in Gaucher patients leukocytes in clinical practice. This measurement may be a useful marker to drive dose selection and early clinical decision making of enzyme replacement therapy. We measure the enzyme activity by high-performance liquid chromatography with ultraviolet detection and 4-nitrophenyl-β-d-glucopyranoside as substrate.

Decrease in mortality in severe community-acquired pneumococcal pneumonia: impact of improving antibiotic strategies (2000-2013).

Objective: The objective of the present study was to compare antibiotic prescribing practices and survival in the ICU for patients with pneumococcal severe community-acquired pneumonia (SCAP) between 2000 and 2013.

Methods: This was a matched case-control study of two prospectively recorded cohorts in Europe. Eighty patients from the Community-Acquired Pneumonia en la Unidad de Cuidados Intensivos (CAPUCI) II study (case group) were matched with 80 patients from CAPUCI I (control group) based on the following: shock at admission, need of mechanical ventilation, COPD, immunosuppression, and age.

Plasma resistin levels are associated with homocysteine, endothelial activation, and nitrosative stress in obese youths.

Objective: To evaluate whether serum resistin levels are related to cardiovascular risk in obese children.

Design And Methods: Cross-sectional study of 110 children (40 normal weight and 70 severely obese). Clinical and biochemical parameters, including lipid profile, fasting glucose and insulin, and homocysteine, were determined.

Dried apples enriched with mandarin juice by vacuum impregnation improve antioxidant capacity and decrease inflammation in obese children.

Background: A favorable effect over development of degenerative diseases is derived of an adecuate intake of fruit and vegetables, mainly due to their antioxidant compounds

Objectives: The goal of this study was to test the effect in vivo over oxidant status and inflammation in obese children of a novel food product made of dried apples enriched with mandarin juice by vacuum impregnation.

Methods: A four-week intervention study was conducted in 41 obese children (> 2 standard deviation score-body mass index). Participants were instructed to follow their usual diet supplemented with 40 g/day of the developed product.

Dried apple enriched with mandarin juice counteracts tamoxifen-induced oxidative stress in rats.

The effect of a product made of dehydrated apples enriched with mandarin juice by vacuum impregnation on markers of oxidative stress (plasma antioxidant capacity, carbonyl groups (CGs), 8-hydroxydeoxyguanosine (8OHdG) and α-tocopherol) was tested in rats. Six groups of animals were studied: one group was fed a standard diet; two groups were supplemented with dehydrated apple either impregnated or not with mandarin juice throughout 28 days; and three groups (one unsupplemented and two supplemented) were additionally treated with tamoxifen (TAM) for 21 days used for induction of oxidative stress. The rats treated with TAM showed an increase in aminotransferases, CGs and 8OHdG.

Retinol-binding protein 4 levels are associated with measures of liver and renal function and oxidant/antioxidant status in obese children.

Serum retinol-binding protein 4 (RBP4) has been proposed as a metabolic risk factor in obesity. We found that RBP4 levels also were associated with liver enzymes and cystatin C. Oxidant stress is a significant feature in obese children with greater values of RBP4 that can mediate the development of comorbidities.

Diet supplementation during early lactation with non-alcoholic beer increases the antioxidant properties of breastmilk and decreases the oxidative damage in breastfeeding mothers.

Background: After delivery and birth, mothers and neonates are exposed to oxidative stress. We tested whether supplementing the diet of breastfeeding mothers with non-alcoholic beer, a product rich in antioxidants, could improve their oxidative status and the antioxidant content of their milk. A prospective trial begun on Day 2 postpartum was conducted in mother-infant dyads.

Psoriasis and hemorheology. Influence of the metabolic syndrome.

Psoriasis is a systemic inflammatory disorder with increased cardiovascular risk which has been partly attributed to the increased prevalence of the metabolic syndrome (MS). However, the contribution of rheological alterations to cardiovascular risk has been scarcely investigated. In 91 psoriasis patients and in 101 healthy volunteers, we determined the rheological profile (fibrinogen, blood viscosity and erythrocyte aggregation), along with lipidic and inflammatory parameters.

SEOM clinical guidelines for myeloid growth factors.

Neutropenia induced by chemotherapy (CT) is an infection risk factor associated to greater morbidity/mortality and dose-limiting toxicity that on many occasions requires a reduction of the dose of cytostatics or a delay in the administration of treatment. This may have a negative effect on the patient's quality of life and even diminish the efficacy of the treatment, especially when the intention is to cure or prolong survival. Management of treatment or prophylaxis of grade 3-4 neutropenia and febrile neutropenia with myeloid growth factors (CSF) varies very much in clinical practice, both in the time of starting treatment and the types of patients it is given to.