Thrombocytopenia and Adverse Bleeding Events in Neonatal Lumbar Punctures in the Emergency Department.

Objective: Lumbar punctures are performed as part of a routine evaluation of neonates with fever in the emergency department. Current recommendations regarding performing a lumbar puncture in the presence of thrombocytopenia exist for children with leukemia and other cancers; however, there are no such recommendations for the general neonatal population. This study assesses the frequency of thrombocytopenia and adverse bleeding events in neonates who undergo a lumbar puncture to determine whether a complete blood count, and specifically the platelet count, should be reviewed before performing the lumbar puncture.

Impact of pregnancy planning and preconceptual dietary training on metabolic control and offspring's outcome in phenylketonuria.

To prevent maternal phenylketonuria (PKU) syndrome low phenylalanine concentrations (target range, 120-360 μmol/L) during pregnancy are recommended for women with PKU. We evaluated the feasibility and effectiveness of current recommendations and identified factors influencing maternal metabolic control and children's outcome. Retrospective study of first successfully completed pregnancies of 85 women with PKU from 12 German centers using historical data and interviews with the women.

Preventing maternal phenylketonuria (PKU) syndrome: important factors to achieve good metabolic control throughout pregnancy.

Background: Insufficient metabolic control during pregnancy of mothers with phenylketonuria (PKU) leads to maternal PKU syndrome, a severe embryo-/fetopathy. Since maintaining or reintroducing the strict phenylalanine (Phe) limited diet in adults with PKU is challenging, we evaluated the most important dietary and psychosocial factors to gain and sustain good metabolic control in phenylketonuric women throughout pregnancy by a questionnaire survey with 38 questions concerning therapy feasibility. Among them, the key questions covered 5 essential items of PKU care as follows: General information about maternal PKU, PKU training, diet implementation, individual metabolic care, personal support.

The neurological and neuropsychiatric spectrum of adults with late-treated phenylketonuria.

Introduction: Phenylketonuria (PKU) is a rare, treatable inborn error of metabolism with frequent neurological and neuropsychiatric complications, especially in undiagnosed or insufficiently treated individuals. Given the wide range of clinical presentations and the importance of treatment implications, we here delineate the neurological and neuropsychiatric symptom spectrum in a large cohort of previously unreported adults with late-treated PKU.

Methods: We consecutively evaluated late-treated PKU cases and pooled clinical and paraclinical data, including video-material, from three centers with expertise in complex movement disorders, inborn errors of metabolism and pediatrics.

Development of a practical dietitian road map for the nutritional management of phenylketonuria (PKU) patients on pegvaliase.

Background: The metabolic dietitian/nutritionist (hereafter 'dietitian') plays an essential role in the nutritional management of patients with phenylketonuria (PKU), including those on pegvaliase. Currently, more educational support and clinical experience is needed to ensure that dietitians are prepared to provide optimal nutritional management and counselling of pegvaliase-treated patients.

Methods: Via a face-to-face data-review meeting, followed by a virtual consolidation meeting, a group of expert dietitians and one paediatrician discussed and developed a series of recommendations on the nutritional evaluation and management of patients receiving pegvaliase.

CSD-Grown YGdBaCuO-BaHfO Nanocomposite Films on Ni5W and IBAD Technical Substrates.

Chemical solution deposition (CSD) was used to grow YGdBaCuO-BaHfO (YGBCO-BHO) nanocomposite films containing 12 mol% BHO nanoparticles and various amounts of Gd, , on two kinds of buffered metallic tapes: Ni5W and IBAD. The influence of the rare-earth stoichiometry on structure, morphology and superconducting properties of these films was studied. The growth process was carefully studied in order to find the most appropriate growth conditions for each composition and substrate.

Two-step chromosome segregation in the stalked budding bacterium Hyphomonas neptunium.

Chromosome segregation typically occurs after replication has finished in eukaryotes but during replication in bacteria. Here, we show that the alphaproteobacterium Hyphomonas neptunium, which proliferates by bud formation at the tip of a stalk-like cellular extension, segregates its chromosomes in a unique two-step process. First, the two sister origin regions are targeted to opposite poles of the mother cell, driven by the ParABS partitioning system.

Chemical solution deposition of Y Gd BaCuO -BaHfO nanocomposite films: combined influence of nanoparticles and rare-earth mixing on growth conditions and transport properties.

Y Gd BaCuO -BaHfO (YGBCO-BHO) nanocomposite films containing 12 mol% BHO nanoparticles and different amounts of Gd were prepared by chemical solution deposition following the trifluoroacetic route on SrTiO single crystals in order to study the influence of the rare earth stoichiometry on structure, morphology and superconducting properties of these films. We optimized the growth process for each of several Gd contents of the 220 nm thick YGBCO-BHO films by varying crystallization temperature and oxygen partial pressure. This optimization process led to the conclusion that mixing the rare earths in YGBCO-BHO films leads to wider growth parameter windows compared to YBCO-BHO and GdBCO-BHO films giving larger freedom for selecting the most convenient processing parameters in order to adapt to different substrates or applications which is very important for the industrial production of coated conductors.

Molecular toolbox for genetic manipulation of the stalked budding bacterium Hyphomonas neptunium.

The alphaproteobacterium Hyphomonas neptunium proliferates by a unique budding mechanism in which daughter cells emerge from the end of a stalk-like extension emanating from the mother cell body. Studies of this species so far have been hampered by the lack of a genetic system and of molecular tools allowing the regulated expression of target genes. Based on microarray analyses, this work identifies two H.