Four-month-old with severe PIK3CA-related overgrowth spectrum disorder successfully treated with alpelisb.

PIK3CA-related overgrowth spectrum (PROS) encompasses different clinical entities caused by somatic activating mutations in PIK3CA. Among PROS, CLOVES syndrome represents a severe phenotype with poor survival rate. We present the case of a 4-month-old girl with CLOVES syndrome successfully treated with alpelisib, a PIKC3A inhibitor.

Clinical presentation and outcomes of hospitalized children with COVID-19 in Mexico City.

We describe the demographic, clinical, radiological, and laboratory findings relating them also to the severity and clinical outcome of 129 children (0-18 years) who were admitted to a tertiary care pediatric hospital in Mexico City due to severe acute respiratory syndrome coronavirus 2 infection between April 1, 2020 and March 31, 2021. The infection was confirmed using reverse transcription-polymerase chain reaction Fever (82.2%), tachypnea (72.

Variable mesh optimization applied to fringe pattern demodulation using a Bézier surface.

We present a parametric method to carry out a demodulation process in complex fringe pattern images with either open or closed fringes; this method is based on the parallel demodulation algorithm and introduces a novel way, to the best of our knowledge, to approximate the phase map using the Bezier surface control points. For this study, a recently developed population meta-heuristic called Variable Mesh Optimization is introduced to implement the optimization process. The results of the proposed method improve the phase error and the run time scores in comparison with other global optimization algorithms, which address this type of problem.

Paediatric Emergencies During the COVID-19 Pandemic.

The state of alarm decreed by the Spanish Government, due to the Coronavirus Disease-19 (COVID-19) pandemic, has demanded the lockdown of children and has conditioned a new organization of the Emergency Departments (ED). A pre-triage station and 2 independent circuits were established: and . The ED visits decreased 84,5% from pre-alarm with no increase in the level of urgencies.

Intestinal Pseudo-Obstruction as the Initial Clinical Presentation in Systemic Lupus Erythematosus: A Rare and Severe Disorder.

Intestinal pseudo-obstruction (IPO) is a rarely recognized complication of systemic lupus erythematosus (SLE). We present a 36-year-old African American female, with only known past medical history of anemia, admitted for frequent vomiting, abdominal distension, abdominal pain, diarrhea, and fever that had been ongoing for 5 days. Laboratory results revealed leukopenia and thrombocytopenia.

Impact of a (poly)phenol-rich extract from the brown algae Ascophyllum nodosum on DNA damage and antioxidant activity in an overweight or obese population: a randomized controlled trial.

Background: Epidemiologic evidence suggests that a diet rich in (poly)phenols has beneficial effects on many chronic diseases. Brown seaweed is a rich source of (poly)phenols.

Objective: The aim of this study was to investigate the bioavailability and effect of a brown seaweed (Ascophyllum nodosum) (poly)phenol extract on DNA damage, oxidative stress, and inflammation in vivo.

Influence of SNPs in Genes that Modulate Lung Disease Severity in a Group of Mexican Patients with Cystic Fibrosis.

Background: The variation in cystic fibrosis (CF) lung disease not always is explained by the CFTR genotype, so it has become apparent that modifier genes must play a considerable role in the phenotypic heterogeneity of CF, so we investigated the association of allelic variants in modifier genes that modulate the severity of lung function in a group of Mexican patients diagnosed with CF.

Methods: We included 140 CF patients classified according to lung phenotype and analyzed 17 single nucleotide polymorphisms (SNPs) by TaqMan allelic discrimination.

Results: We demonstrated that patients with GG or GC genotype of the allelic variant rs11003125 (MBL2-550) of the MBL2 gene exhibit most of the lung manifestations at an earlier age; and the rs1042713 allelic variant of ADRB2 gene, showed statistical difference only with the age of first spirometry.

Effect of a grapevine-shoot waste extract on red wine aromatic properties.

Background: The use of a grapevine-shoot extract (VIN) is being studied as an alternative to sulfur dioxide (SO ). VIN stabilizes anthocyanins and preserves polyphenolic compounds, and thus improves chromatic wine properties. In this study, selected aroma compounds (esters, C -norisoprenoids, oxidation and vine-shoot-related compounds), sensory analysis and the olfactometric profile were determined in the wines treated with VIN at two concentrations.

Sulfur free red wines through the use of grapevine shoots: Impact on the wine quality.

Following a preliminary study to determine the possibility of using a grapevine shoot extract (VIN) as a sustainable alternative to sulfur dioxide (SO), in this study, the chromatic features, phenolic composition, and sensory analysis of wines treated with VIN at two concentrations were studied during storage in bottle for the first time. The highest differences were found in phenolic compounds after 12months of storage in bottle. The VIN wines had a low content of free anthocyanins and were high in vinyl-pyranoanthocyanins, and B-type vitisins.

The influence of pre-fermentative maceration and ageing factors on ester profile and marker determination of Pedro Ximenez sparkling wines.

The influence of pre-fermentative maceration and ageing factors on the ester profiles of Pedro Ximenez sparkling wines was evaluated. The pre-fermentative maceration consisted of the skin-maceration of musts at 10°C for 6h. The sparkling wines were produced following the Champenoise method.

Quantitative Profiling of Ester Compounds Using HS-SPME-GC-MS and Chemometrics for Assessing Volatile Markers of the Second Fermentation in Bottle.

A quantitative approach using HS-SPME-GC-MS was performed to investigate the ester changes related to the second fermentation in bottle. The contribution of the type of base wine to the final wine style is detailed. Furthermore, a discriminant model was developed based on ester changes according to the second fermentation (with 100% sensitivity and specificity values).

Assessing a traceability technique in fresh oranges (Citrus sinensis L. Osbeck) with an HS-SPME-GC-MS method. Towards a volatile characterisation of organic oranges.

A targeted approach using HS-SPME-GC-MS was performed to compare flavour compounds of 'Navelina' and 'Salustiana' orange cultivars from organic and conventional management systems. Both varieties of conventional oranges showed higher content of ester compounds. On the other hand, higher content of some compounds related with the geranyl-diphosphate pathway (neryl and geranyl acetates) and some terpenoids were found in the organic samples.

Effect of Organic and Conventional Management on Bio-Functional Quality of Thirteen Plum Cultivars (Prunus salicina Lindl.).

In this study, thirteen Japanese plum cultivars (Prunus salicina Lindl.) grown under conventional and organic conditions were compared to evaluate the influence of the culture system on bioactive compounds. Their organic acids content (malic, citric, tartaric, succinic, shikimic, ascorbic and fumaric acid), total polyphenols, total anthocyanins, total carotenoids and antioxidant capacity (FRAP, ABTS) were evaluated.

Effects of salinity and nitrogen supply on the quality and health-related compounds of strawberry fruits (Fragaria × ananassa cv. Primoris).

Background: Different nitrogen inputs and/or development under adverse water conditions (water stress/low quality and/or high salinity/electrical conductivity), such as those prevailing in Almeria (Mediterranean coast, south-east Spain), may affect overall fruit and vegetable quality. This study evaluated the influence of salinity and nitrogen reduction in hydroponic nutrient solution on strawberry fruit quality and nutritional compounds (Fragaria × ananassa Duch., cv.

Differential dynamic and structural behavior of lipid-cholesterol domains in model membranes.

Changes in the cholesterol (Chol) content of biological membranes are known to alter the physicochemical properties of the lipid lamella and consequently the function of membrane-associated enzymes. To characterize these changes, we used steady-state and time resolved fluorescence spectroscopy and two photon-excitation microscopy techniques. The membrane systems were chosen according to the techniques that were used: large unilamellar vesicles (LUVs) for cuvette and giant unilamellar vesicles (GUVs) for microscopy measurements; they were prepared from dipalmitoyl phosphatidylcholine (DPPC) and dioctadecyl phosphatidylcholine (DOPC) in mixtures that are well known to form lipid domains.

Tumor necrosis factor-alpha is a common genetic risk factor for asthma, juvenile rheumatoid arthritis, and systemic lupus erythematosus in a Mexican pediatric population.

There is a great deal of evidence that points to the association of the tumor necrosis factor-alpha (TNF-alpha) gene as a common genetic factor in the pathogenesis of diseases that are caused by inflammatory and/or autoimmune etiologies. Two single nucleotide polymorphisms (SNPs) identified in the TNF-alpha promoter region have been associated with disease susceptibility and severity. We investigated whether -308G/A and -238G/A TNF-alpha polymorphisms were associated with asthma, systemic lupus erythematosus (SLE), and juvenile rheumatoid arthritis (JRA) in a pediatric Mexican population.

The antiepileptic drug diphenylhydantoin affects the structure of the human erythrocyte membrane.

Phenytoin (diphenylhydantoin) is an antiepileptic agent effective against all types of partial and tonic-clonic seizures. Phenytoin limits the repetitive firing of action potentials evoked by a sustained depolarization of mouse spinal cord neurons maintained in vitro. This effect is mediated by a slowing of the rate of recovery of voltage activated Na+ channels from inactivation.

Efficacy and tolerability of salmeterol/fluticasone propionate versus montelukast in childhood asthma: A prospective, randomized, double-blind, double-dummy, parallel-group study.

Background: Asthma control remains suboptimal in adults and children worldwide. Inhaled salmeterol/fluticasone propionate combination (SFC) and oral montelukast (MON) are 2 treatments available for childhood asthma.

Objective: This study, the PEdiatric Asthma Control Evaluation (PEACE), investigated the efficacy and tolerability of SFC compared with MON for the control of persistent asthma in children.

Modulation of reconstituted pig kidney Na+/K(+)-ATPase activity by cholesterol in endogenous lipid vesicles: role of lipid domains.

Diverse experimental and theoretical evidence suggests that plasma membranes contain cholesterol-induced segregated domains that could play a key role in the modulation of membrane functions, including intrinsic enzyme activity. To gain insight into the role of cholesterol, we reconstituted pig kidney Na+/K+-ATPase into unilamellar vesicles of endogenous lipids mimicking the natural membrane and addressed the question of how modification of the cholesterol content could affect the ATPase activity via changes in the membrane lipid phase and in the protein structure and dynamics. We used steady-state and time-resolved fluorescence spectroscopy with the lipid phase probes DPH and Laurdan and the protein probe fluorescein and also used infrared spectroscopy using attenuated total reflectance.

A study of the perturbation effects of the local anesthetic procaine on human erythrocyte and model membranes and of modifications of the sodium transport in toad skin.

The interaction of the local anesthetic procaine with human erythrocytes, isolated unsealed human erythrocyte membranes (IUM), isolated toad skins, and molecular models is described. The latter consisted of phospholipid multilayers built-up of dimyristoylphosphatidylcholine (DMPC) and of dimyristoylphosphatidylethanolamine (DMPE), representatives of phospholipid classes located in the outer and inner monolayers of the human erythrocyte membrane, respectively. Optical and scanning electron microscopy of human erythrocytes revealed that procaine induced the formation of stomatocytes.

Effects of the local anesthetic benzocaine on the human erythrocyte membrane and molecular models.

The interaction of the local anesthetic benzocaine with the human erythrocyte membrane and molecular models is described. The latter consisted of isolated unsealed human erythrocyte membranes (IUM), large unilamellar vesicles (LUV) of dimyristoylphospatidylcholine (DMPC), and phospholipid multilayers of DMPC and dimyristoylphospatidyletanolamine (DMPE), representatives of phospholipid classes located in the outer and inner monolayers of the human erythrocyte membrane, respectively. Optical and scanning electron microscopy of human erythrocytes revealed that benzocaine induced the formation of echinocytes.

Structural effects of the local anesthetic bupivacaine hydrochloride on the human erythrocyte membrane and molecular models.

The interaction of the local anesthetic bupivacaine with the human erythrocyte membrane and molecular models is described. The latter consisted of isolated unsealed human erythrocyte membranes (IUM), large unilamellar vesicles (LUV) of dimyristoylphosphatidylcholine (DMPC), and phospholipid multilayers built-up of DMPC and dimyristoylphosphatidylethanolamine (DMPE), representatives of phospholipid classes located in the outer and inner monolayers of the human erythrocyte membrane, respectively. Optical and scanning electron microscopy revealed that bupivacaine induced erythrocyte spheroechinocytosis.

The local anesthetic proparacaine modifies sodium transport in toad skin and perturbs the structures of model and cell membranes.

Experimental results indicate a significant decrease in the potential difference (PD) and in the short-circuit current (Isc) after the application of proparacaine to isolated toad skin, which may reflect an inhibition of the active transport of ions. This finding was explained on the basis of the results obtained from membrane models incubated with proparacaine. These consisted of human erythrocytes, isolated unsealed human erythrocyte membranes (IUM), phospholipid multilayers built-up of dimyristoylphosphatidylcholine (DMPC) and dimyristoylphosphatidylethanolamine (DMPE), representatives of phospholipid classes located in the outer and inner monolayers of the human erythrocyte membrane, respectively, and in large unilamellar vesicles (LUV) of DMPC X-ray diffraction showed that proparacaine interaction with DMPC and DMPE bilayers perturbed both structures, especially DMPC.

Modulo 2pi fringe orientation angle estimation by phase unwrapping with a regularized phase tracking algorithm.

The fringe orientation angle provides useful information for many fringe-pattern-processing techniques. From a single normalized fringe pattern (background suppressed and modulation normalized), the fringe orientation angle can be obtained by computing the irradiance gradient and performing a further arctangent computation. Because of the 180 degrees ambiguity of the fringe direction, the orientation angle computed from the gradient of a single fringe pattern can be determined only modulo pi.