Gitelman syndrome disclosed by calcium pyrophosphate deposition disease: Early diagnosis by ultrasonographic study

Gitelman's syndrome is a rare autosomal-recessive tubular disorder characterized by hypomagnesemia and hypocalciuria associated to hypokalemia. The clinical spectrum is wide and usually characterized by chronic fatigue, cramps, muscle weakness and paresthesiae. We describe a case of a 43 year-old male patient with early onset of knee arthritis and no other symptoms. Ultrasound revealed diffuse and confluent hyperechoic deposits in cartilage, fibrocartilage of the menisci and synovium and calcium pyrophosphate crystals were observed in the synovial fluid of the knee. The concomitant presence of hypomagnesemia, hypocalciuria and hypokalemia made clear the diagnosis of Gitelman's syndrome associated with chondrocalcinosis

Documentation of adherence to infection prevention best practice in patient records: a mixed-methods investigation.

BACKGROUND Healthcare-associated infections remain a preventable cause of patient harm in healthcare. Full documentation of adherence to evidence-based best practices for each patient can support monitoring and promotion of infection prevention measures. Thus, we reviewed the extent, nature, and determinants of the documentation of infection prevention (IP) standards in patients with HAI. METHODS We reviewed electronic patient records (EMRs) of patients included in four annual point-prevalence studies 2013-2016 who developed a device- or procedure-related HAI (surgical site infection (SSI), catheter-associated urinary tract infection (CAUTI), ventilator-associated infection (VAP), catheter-related bloodstream infection (CRBSI)). We examined the documentation quality of mandatory preventive measures published as institutional IP standards. Additionally, we undertook semi-structured interviews with healthcare providers and a two-step inductive (grounded theory) and deductive (Theory of Planned Behaviour) content analysis. RESULTS Of overall 2972 surveyed patients, 249 (8.4%) patients developed 272 healthcare-associated infections. Of these, 116 patients met the inclusion criteria, classified as patients with SSI, CAUTI, VAP, CRBSI in 78 (67%), 21 (18%), 10 (9%), 7 (6%), cases, respectively. We found documentation of IP measures in EMRs in 432/1308 (33%) cases. Documentation of execution existed in the study patients' EMRs for SSI, CAUTI, VAP, CRBSI, and overall, in 261/931 (28%), 27/104 (26%), 46/122 (38%), 26/151 (17%), and 360/1308 (28%) cases, respectively, and documentation of non-execution in 67/931 (7%), 2/104 (2%), 0/122 (0%), 3/151 (2%), and 72/1308 (6%) cases, respectively. Healthcare provider attitudes, subjective norms, and perceived behavioural control indicated reluctance to document IP standards. CONCLUSIONS EMRs rarely included conclusive data about adherence to IP standards. Documentation had to be established indirectly through data captured for other reasons. Mandatory institutional documentation protocols or technically automated documentation may be necessary to address such shortcomings in patient safety documentation

Inaugurating a Dutch Napoleon? Conservative criticism of the 1815 constitution of the United Kingdom of The Netherlands

International audienc

CORRECTION

Idiopathic pulmonary fibrosis (IPF) is a progressive scarring disease of the lung with feweffective therapeutic options. Structural remodelling of the extracellular matrix [i.e. collagen cross-linkingmediated by the lysyl oxidase (LO) family of enzymes (LOX, LOXL1-4)] might contribute to disease pathogenesis and represent a therapeutic target. This study aimed to further our understanding of the mechanisms by which LO inhibitors might improve lung fibrosis. Lung tissues from IPF and non-IPF subjects were examined for collagen structure (second harmonic generation imaging) and LO gene (microarray analysis) and protein (immunohistochemistry and western blotting) levels. Functional effects (collagen structure and tissue stiffness using atomic force microscopy) of LO inhibitors on collagen remodelling were examined in two models, collagen hydrogels and decellularized human lung matrices. LOXL1/LOXL2 gene expression and protein levels were increased in IPF versus non-IPF. Increased collagen fibril thickness in IPF versus non-IPF lung tissues correlated with increased LOXL1/LOXL2, and decreased LOX, protein expression. beta-Aminoproprionitrile (beta-APN; pan-LO inhibitor) but not Compound A (LOXL2-specific inhibitor) interfered with transforming growth factor-beta-induced collagen remodelling in both models. The beta-APN treatment group was tested further, and beta-APN was found to interfere with stiffening in the decellularized matrix model. LOXL1 activity might drive collagen remodelling in IPF lungs. The interrelationship between collagen structural remodelling and LOs is disrupted in IPF lungs. Inhibition of LO activity alleviates fibrosis by limiting fibrillar collagen cross-linking, thereby potentially impeding the formation of a pathological microenvironment in IPF

Cinnamic Acid Derivate From the Bark of Lerchea bracteata Val.

Investigation of chemical constituen of the bark of Lerchea bracteata Val. has been carried out. A 3,4-dihyroxymethylcinnamic acid has been isolated and charactirezed from ethyl acetate fraction by chromatography methods and follewed by recrystalization, obtained as yellowish crystalline and melted at 162-163 oC. The structure of this compound was elucidated from its spectroscopic datas: UV, IR, H1 and C13 NMR spectra, 2D NMR (COSY, HMBC/HSQC) and as well as mass spectra. Keywords: Lerchea bracteata Val, Rubiaceae, 3,4-dihyroxymethylcinnamic aci

Hydrogen and Carbon Monoxide-Utilizing Kyrpidia spormannii Species From Pantelleria Island, Italy

Volcanic and geothermal areas are hot and often acidic environments that emit geothermal gasses, including H2, CO and CO2. Geothermal gasses mix with air, creating conditions where thermoacidophilic aerobic H2- and CO-oxidizing microorganisms could thrive. Here, we describe the isolation of two Kyrpidia spormannii strains, which can grow autotrophically by oxidizing H2 and CO with oxygen. These strains, FAVT5 and COOX1, were isolated from the geothermal soils of the Favara Grande on Pantelleria Island, Italy. Extended physiology studies were performed with K. spormannii FAVT5, and showed that this strain grows optimally at 55\ub0C and pH 5.0. The highest growth rate is obtained using H2 as energy source (\u3bcmax 0.19 \ub1 0.02 h\u20131, doubling time 3.6 h). K. spormannii FAVT5 can additionally grow on a variety of organic substrates, including some alcohols, volatile fatty acids and amino acids. The genome of each strain encodes for two O2-tolerant hydrogenases belonging to [NiFe] group 2a hydrogenases and transcriptome studies using K. spormannii FAVT5 showed that both hydrogenases are expressed under H2 limiting conditions. So far no Firmicutes except K. spormannii FAVT5 have been reported to exhibit a high affinity for H2, with a Ks of 327 \ub1 24 nM. The genomes of each strain encode for one putative CO dehydrogenase, belonging to Form II aerobic CO dehydrogenases. The genomic potential and physiological properties of these Kyrpidia strains seem to be quite well adapted to thrive in the harsh environmental volcanic conditions

Sex-specific behavioral and neurogenic responses to cocaine in mice lacking and blocking dopamine D1 or dopamine D2 receptors

Adult neurogenesis in rodents is modulated by dopaminergic signaling and inhibited by cocaine. However, the sex-specific role of dopamine D1 and D2 receptors (D1R, D2R) in the deleterious effect of cocaine on adult neurogenesis has not been described yet. Here, we explored sex differences in (a) cell proliferation (5′-bromo-2′-deoxyuridine [BrdU]), (b) neural precursor (nestin), (c) neuronal phenotype (BrdU/β3-tubulin), and (d) neuronal maturity (NeuN) in the subventricular zone (SVZ) of the lateral ventricles and striatum of mice with genetic deletion (D1, D2) or pharmacological blockage (SCH23390: 0.1 mg/kg/day/5 days; Raclopride: 0.3 mg/kg/day/5 days) of D1R and D2R, and treated (10 mg/kg/day/5 days) and then challenged (5 mg/kg, 48 hr later) with cocaine. Results indicated that hyperactivity responses to cocaine were absent in D1 mice and reduced in SCH23390-treated mice. Activity responses to cocaine were reduced in D2 males, but absent in D2 females and increased in Raclopride-treated females. D1R deletion blocked the deleterious effect of cocaine on SVZ cell proliferation in males. Cocaine-exposed D1 males also had reduced neuronal phenotype of SVZ newborn cells and increased striatal neuronal maturity. D2 mice had lower proliferative and neural precursor responses. Cocaine in D2 females or coadministered with Raclopride in wild-type females improved SVZ cell proliferation, an effect that positively correlated with plasma brain-derived neurotrophic factor (BDNF) concentrations. In conclusion, the sex-specific D1R and D2R signaling on SVZ cell proliferation, neural progenitor and neuronal maturity is differentially perturbed by cocaine, and BDNF may be required to link D2R to neuroplasticity in cocaine addiction in females.Consejería de Salud, Junta de Andalucía, Grant/Award Number: C1-0049-2019; Instituto de Salud Carlos III, Grant/Award Numbers: CP19/00068, CPII17/00024, CPII19/00022, CPII19/00031, PI19/01577, PI19/00886, PI17/02026, RD16/0017/0001; Ministerio de Sanidad, Servicios Sociales e Igualdad, Grant/Award Numbers: PND2017/043, PND2018/033, PND2018/044, PND2019/04

The DNA-binding domain of the Chd1 chromatin-remodelling enzyme contains SANT and SLIDE domains

The ATP-dependent chromatin-remodelling enzyme Chd1 is a 168-kDa protein consisting of a double chromodomain, Snf2-related ATPase domain, and a C-terminal DNA-binding domain. Here, we show the DNA-binding domain is required for Saccharomyces cerevisiae Chd1 to bind and remodel nucleosomes. The crystal structure of this domain reveals the presence of structural homology to SANT and SLIDE domains previously identified in ISWI remodelling enzymes. The presence of these domains in ISWI and Chd1 chromatin-remodelling enzymes may provide a means of efficiently harnessing the action of the Snf2-related ATPase domain for the purpose of nucleosome spacing and provide an explanation for partial redundancy between these proteins. Site directed mutagenesis was used to identify residues important for DNA binding and generate a model describing the interaction of this domain with DNA. Through inclusion of Chd1 sequences in homology searches SLIDE domains were identified in CHD6–9 proteins. Point mutations to conserved amino acids within the human CHD7 SLIDE domain have been identified in patients with CHARGE syndrome