Synthesis of 1,2-trans-2-acetamido-2-deoxyhomoiminosugars.

The first synthesis of 1,2-trans-homoiminosugars devised as mimics of β-D-GlcNAc and α-D-ManNAc is described. Key steps include a regioselective azidolysis of a cyclic sulfite and a β-amino alcohol skeletal rearrangement applied to a polyhydroxylated azepane. The β-D-GlcNAc derivative has been coupled to serine to deliver an iminosugar C-amino acid.

Observation of e+e- → π+π-π(0)(χbJ) and Search for X(b) → ωϒ(1S) at sqrt[s] = 10.867 GeV.

The e(+)e(-) → π(+)π(-)π(0)χ(bJ) (J = 0,1,2) processes are studied using a 118 fb(-1) data sample acquired with the Belle detector at a center-of-mass energy of 10.867 GeV. Unambiguous π(+)π(-)π(0)χ(bJ) (J = 1,2), ωχ(b1) signals are observed, and indication for ωχ(b2) is seen, both for the first time, and the corresponding cross section measurements are presented.

N- and C-alkylation of seven-membered iminosugars generates potent glucocerebrosidase inhibitors and F508del-CFTR correctors.

The glycosidase inhibitory properties of synthetic C-alkyl and N-alkyl six-membered iminosugars have been extensively studied leading to therapeutic candidates. The related seven-membered iminocyclitols have been less examined despite the report of promising structures. Using an in house ring enlargement/C-alkylation as well as cross-metathesis methodologies as the key steps, we have undertaken the synthesis and biological evaluation of a library of fourteen 2C- and eight N-alkyl tetrahydroxylated azepanes starting from an easily available glucopyranose-derived azidolactol.

Measurement of the branching fraction B(Λc+ → pK-π+).

We present the first model-independent measurement of the absolute branching fraction of the Λ(c)(+) → pK(-)π(+) decay using a data sample of 978 fb(-1) collected with the Belle detector at the KEKB asymmetric-energy e(+)e(-) collider. The number of Λ(c)(+) baryons is determined by reconstructing the recoiling D((*)-) pπ(+) system in events of the type e(+)e(-) → D((*)-) pπ(+)Λ(c)(+). The branching fraction is measured to be B(Λ(c)(+) → pK(-)π(+)) = (6.

Implantation of total artificial heart in congenital heart disease.

In patients with end-stage heart failure (HF), a total artificial heart (TAH) may be implanted as a bridge to cardiac transplant. However, in congenital heart disease (CHD), the malformed heart presents a challenge to TAH implantation. In the case presented here, a 17 year-old patient with congenital transposition of the great arteries (CCTGA) experienced progressively worsening HF due to his congenital condition.

Synthesis and biological evaluation of α-1-C-4'-arylbutyl-L-arabinoiminofuranoses, a new class of α-glucosidase inhibitors.

A series of α-1-C-4'-arylbutyl-L-arabinoiminofuranoses 3 with functional groups attached to the phenyl ring, which are potential α-glycosidase inhibitors, was designed and synthesized by using a Negishi cross-coupling reaction as the key reaction. Arylbutyl derivatives 3a-e showed potent inhibitory activities against intestinal maltase. Among them, difluorophenylbutyl derivative 3e showed good inhibition activities against intestinal isomaltase and sucrase as compared to those of 1 and commercial drugs.

Aortic arch advancement for aortic coarctation and hypoplastic aortic arch in neonates and infants.

Background: The optimal treatment for infants with aortic coarctation and hypoplastic aortic arch is controversial. The goal of this study was to report the short-term and mid-term outcomes of aortic arch advancement (AAA) in infants with hypoplastic aortic arch.

Methods: All infants who underwent AAA at our institution from 1995 to 2012 were included.

Synthesis of the enantiomers of XYLNAc and LYXNAc: comparison of β-N-acetylhexosaminidase inhibition by the 8 stereoisomers of 2-N-acetylamino-1,2,4-trideoxy-1,4-iminopentitols.

The enantiomers of XYLNAc (2-N-acetylamino-1,2,4-trideoxy-1,4-iminoxylitol) are prepared from the enantiomers of glucuronolactone; the synthesis of the enantiomers of LYXNAc (2-N-acetylamino-1,2,4-trideoxy-1,4-iminolyxitol) from an L-arabinono-δ-lactone and a D-ribono-δ-lactone is reported. A comparison is made of the inhibition of β-N-acetylhexosaminidases (HexNAcases) and α-N-acetylgalactosaminidase (α-GalNAcase) by 8 stereoisomeric 2-N-acetylamino-1,2,4-trideoxy-1,4-iminopentitols; their N-benzyl derivatives are better inhibitors than the parent compounds. Both XYLNAc and LABNAc are potent inhibitors against HexNAcases.

[Usefulness of one point measurement method of pediatric dose and UV spectrophotometry for filterability test of in-line filter].

The adsorption of Bevacizumab, Trastuzumab, Rituximab, Nedaplatin, Vincristine sulfate, Nogitecan hydrochloride, Actinomycin D and Ramosetron hydrochloride to 0.2 μm endotoxin-retentive in-line filters was evaluated with pediatric doses by UV spectrophotometry. The results indicated that some drug adsorption was shown with Nogitecan hydrochloride, Actinomycin D and Ramosetron hydrochloride, and good recovery was shown with the other five drugs.

Nature of the underlying heart disease affects survival in pediatric patients undergoing extracorporeal cardiopulmonary resuscitation.

Objective: To describe the use of extracorporeal membrane oxygenation (ECMO) in acute resuscitation after cardiac arrest in pediatric patients with heart disease, with reference to patient selection and predictors of outcome.

Methods: A retrospective medical record review was performed of all patients aged ≤21 years with heart disease who had undergone ECMO for cardiopulmonary resuscitation (ECPR) at Texas Children's Hospital from January 2005 to December 2012. The most recent Pediatric Overall Performance Category score was determined from the patients' medical records.

Contemporary results of surgical repair of recurrent aortic arch obstruction.

Background: There is a paucity of data on the current outcomes of surgical intervention for recurrent aortic arch obstruction (RAAO) after initial aortic arch repair in children. The goal of this study is to report the long-term results in these patients.

Methods: All patients undergoing surgical intervention for RAAO at Texas Children's Hospital from 1995 to 2012 were included.

Mechanical assist devices in neonates and infants.

Clinical advances in the field of mechanical support have led to additional treatment strategies in adult and, subsequently, pediatric heart failure management. The limited number of device options available to the pediatric population has led to a reallocation of resources in order to broaden the pediatric device armamentarium. Although there are very few device options in pediatrics, there are short-term support options that include the RotaFlow and PediMag.

Observation of D0-D0 mixing in e+e- collisions.

We observe D(0)-D(0) mixing in the decay D(0) → K+π- using a data sample of integrated luminosity 976 fb(-1) collected with the Belle detector at the KEKB e+e- asymmetric-energy collider. We measure the mixing parameters x'(2) = (0.09 ± 0.

Docking and SAR studies of calystegines: binding orientation and influence on pharmacological chaperone effects for Gaucher's disease.

We report on the identification of the required configuration and binding orientation of nor-tropane alkaloid calystegines against β-glucocerebrosidase. Calystegine B2 is a potent competitive inhibitor of human lysosomal β-glucocerebrosidase with Ki value of 3.3 μM.

Spontaneous spatial mapping of learned sequence in chimpanzees: evidence for a SNARC-like effect.

In the last couple of decades, there has been a growing number of reports on space-based representation of numbers and serial order in humans. In the present study, to explore evolutionary origins of such representations, we examined whether our closest evolutionary relatives, chimpanzees, map an acquired sequence onto space in a similar way to humans. The subjects had been trained to perform a number sequence task in which they touched a sequence of "small" to "large" Arabic numerals presented in random locations on the monitor.

Nine of 16 stereoisomeric polyhydroxylated proline amides are potent β-N-acetylhexosaminidase inhibitors.

All 16 stereoisomeric N-methyl 5-(hydroxymethyl)-3,4-dihydroxyproline amides have been synthesized from lactones accessible from the enantiomers of glucuronolactone. Nine stereoisomers, including all eight with a (3R)-hydroxyl configuration, are low to submicromolar inhibitors of β-N-acetylhexosaminidases. A structural correlation between the proline amides is found with the ADMDP-acetamide analogues bearing an acetamidomethylpyrrolidine motif.

Iteamine, the first alkaloid isolated from Itea virginica L. inflorescence.

Iteamine, o-aminobenzyl β-D-glucopyranoside, is the first alkaloid to be isolated from Itea virginica. Itea is the sole plant source of D-psicose, a rare sugar likely to be a major dietary supplement. The structure of iteamine was established by NMR and confirmed by total synthesis.

Measurement of the τ-lepton lifetime at Belle.

The lifetime of the τ lepton is measured using the process e+ e- → τ+ τ- , where both τ leptons decay to 3πν(τ). The result for the mean lifetime, based on 711  fb(-1) of data collected with the Belle detector at the ϒ(4S) resonance and 60  MeV below, is τ=(290.17±0.

Pediatric ventricular assist device use as a bridge to transplantation does not affect long-term quality of life.

Objective: The present study sought to determine the long-term quality of life (QOL) of children who required long-term ventricular assist device (VAD) support as a bridge to transplantation (BTT) compared with children who underwent heart transplantation without VAD support. Currently, 20% of children undergoing heart transplantation have required a VAD as a BTT. Few data have been published assessing how children requiring a VAD as a BTT will fair in terms of their long-term QOL.

Effects of eugenol-reduced clove extract on glycogen phosphorylase b and the development of diabetes in db/db mice.

We found that the 50% aqueous EtOH extract of clove (Syzygium aromaticum) had potent dose-dependent inhibitory activity toward glycogen phosphorylase b and glucagon-stimulated glucose production in primary rat hepatocytes. Among the components, eugeniin inhibited glycogen phosphorylase b and glucagon-stimulated glucose production in primary rat hepatocytes, with IC50 values of 0.14 and 4.

Conceptual metaphorical mapping in chimpanzees (Pan troglodytes).

Conceptual metaphors are linguistic constructions. Such a metaphor is humans' mental representation of social rank as a pyramidal-like structure. High-ranked individuals are represented in higher positions than low-ranked individuals.

Glucosylceramide mimics: highly potent GCase inhibitors and selective pharmacological chaperones for mutations associated with types 1 and 2 Gaucher disease.

A series of iminoxylitol derivatives carrying a C-linked di-O-acyl or di-O-alkyl glyceryl substituent were prepared and characterized. All of these compounds, which were designed as glucosylceramide (GlcCer) mimics, were nanomolar inhibitors of lysosomal β-glucosidase (glucocerebrosidase, GCase). Two of these pseudoglycolipids were further evaluated for their ability to enhance the activity of mutant GCase in human Gaucher cells.