Inequities in access to pediatric epilepsy surgery: a bioethical framework.

Epilepsy is a common childhood condition associated with a considerable medical and psychosocial burden. Children in whom medical treatment fails to reduce seizure burden represent an especially vulnerable patient population because prolonged, uncontrolled seizures are associated with poor developmental and neurocognitive outcomes. Surgical treatment in the form of cortical resection, functional disconnection, or neuromodulation may alleviate or significantly reduce the disease burden for a subset of these patients.

Case report - An acute presentation of lymphangiomatosis.

Introduction: Lymphangioma is an uncommon malformation of lymphatic system. Multiple colonic lymphangioma named as lymphangiomatosis is considered an extremely rare disease.

Presentation Of Case: We report a case of colonic lymphangiomatosis presenting as recurrent right iliac fossa pain in a young female treated surgically.

[Auditory and vestibular findings in Fabry disease: a study of 25 patients].

Purpose: Fabry disease (FD, OMIM 301500) is an X-linked lysosomal storage disorder due to deficient activity of the enzyme alpha-galactosidase A. Males and females exhibit severe organ involvement. The high incidence of otological symptoms was recently reported.

FLNA genomic rearrangements cause periventricular nodular heterotopia.

Objective: To identify copy number variant (CNV) causes of periventricular nodular heterotopia (PNH) in patients for whom FLNA sequencing is negative.

Methods: Screening of 35 patients from 33 pedigrees on an Affymetrix 6.0 microarray led to the identification of one individual bearing a CNV that disrupted FLNA.

Regiospecific β-lactam ring-opening/recyclization reactions of N-aryl-3-spirocyclic-β-lactams catalyzed by a Lewis-Brønsted acids combined superacid catalyst system: a new entry to 3-spirocyclicquinolin-4(1H)-ones.

The regiospecific β-lactam ring-opening/recyclization reaction of N-aryl-3-spirocyclic-β-lactams, made by the one-pot cyclization reaction of acetoacetanilides, has been achieved for the first time using a Lewis-Brønsted acids combined superacid catalyst system, thus providing an efficient entry to 3-spirocyclicquinolin-4(1H)-ones. A mechanism involving superacid-catalysis was proposed.

Effect of packaging and storage time on survival of Listeria monocytogenes on kippered beef steak and turkey tenders.

Unlabelled: The objective of our study was to determine effect of packaging method and storage time on reducing Listeria monocytogenes in shelf-stable meat snacks. Commercially available kippered beef steak strips and turkey tenders were dipped into a 5-strain L. monocytogenes cocktail, and dried at 23 °C until a water activity of 0.

Copper-mediated chelation-assisted ortho nitration of (hetero)arenes.

A novel copper-mediated chelation-assisted ortho C-H nitration of (hetero)arenes has been developed for the first time, which used dioxygen as terminal oxidant and 1,2,3-TCP as solvent, leading to the synthesis of nitroaromatics with excellent regioselectivity and in good yields. Mechanistic investigations indicate a mechanism involving a four-centered transition state, with simultaneous cleavage of an ortho C-H bond and a N-O bond of the nitrate anion on the 2-arylpyridine-coordinated copper(II) complex.

Treatment of a traumatic carotid-cavernous fistula by the sole use of a flow diverting stent.

Direct caroticocavernous fistula (CCF) has traditionally been treated by detachable balloon placement within the affected cavernous sinus. We describe a case of a direct CCF treated solely with flow-diverting stents. These novel devices may offer a simpler and potentially safer vessel-sparing option in this rare condition.

Measurement of serum anti-Müllerian hormone concentration in female dogs and cats before and after ovariohysterectomy.

Anti-Müllerian hormone (AMH), or Müllerian inhibitory substance, is a hormone that is best known for its production by fetal testes in mammals and as the inhibitor of Müllerian (paramesonephric) duct development in males. However, following the development of the Müllerian ducts into the oviduct, uterus, and upper vagina in female mammals, the ovaries produce AMH, which can be found in measureable amounts within the peripheral circulation, especially in adults. The ovaries appear to be the sole source of AMH in the circulation; therefore, it may be a useful marker in clinically relevant situations when an assessment of the presence or absence of ovaries or ovarian remnants in dogs and cats is important.

[Pharyngeal fibrovascular polyp].

The giant fibrovascular polyp of the hypopharynx is a rare and benign tumor. We report one case in a 46-year-old man. This lesion, usually unique, affects predominantly men with an average age of 53 years.

Proton coupled electron transfer and redox-active tyrosine Z in the photosynthetic oxygen-evolving complex.

Proton coupled electron transfer (PCET) reactions play an essential role in many enzymatic processes. In PCET, redox-active tyrosines may be involved as intermediates when the oxidized phenolic side chain deprotonates. Photosystem II (PSII) is an excellent framework for studying PCET reactions, because it contains two redox-active tyrosines, YD and YZ, with different roles in catalysis.

Human mutations in NDE1 cause extreme microcephaly with lissencephaly [corrected].

Genes disrupted in human microcephaly (meaning "small brain") define key regulators of neural progenitor proliferation and cell-fate specification. In comparison, genes mutated in human lissencephaly (lissos means smooth and cephalos means brain) highlight critical regulators of neuronal migration. Here, we report two families with extreme microcephaly and grossly simplified cortical gyral structure, a condition referred to as microlissencephaly, and show that they carry homozygous frameshift mutations in NDE1, which encodes a multidomain protein that localizes to the centrosome and mitotic spindle poles.

N-formylkynurenine as a marker of high light stress in photosynthesis.

Photosystem II (PSII) is the membrane protein complex that catalyzes the photo-induced oxidation of water at a manganese-calcium active site. Light-dependent damage and repair occur in PSII under conditions of high light stress. The core reaction center complex is composed of the D1, D2, CP43, and CP47 intrinsic polypeptides.

Perturbations of aromatic amino acids are associated with iron cluster assembly in ribonucleotide reductase.

The β2 subunit of class Ia ribonucleotide reductases (RNR) contains an antiferromagnetically coupled μ-oxo bridged diiron cluster and a tyrosyl radical (Y122•). In this study, an ultraviolet resonance Raman (UVRR) difference technique describes the structural changes induced by the assembly of the iron cluster and by the reduction of the tyrosyl radical. Spectral contributions from aromatic amino acids are observed through UV resonance enhancement at 229 nm.

Proton coupled electron transfer and redox active tyrosines in Photosystem II.

In this article, progress in understanding proton coupled electron transfer (PCET) in Photosystem II is reviewed. Changes in acidity/basicity may accompany oxidation/reduction reactions in biological catalysis. Alterations in the proton transfer pathway can then be used to alter the rates of the electron transfer reactions.

Package systems and storage times serve as postlethality controls for Listeria monocytogenes on whole-muscle beef jerky and pork and beef smoked sausage sticks.

To validate how packaging and storage reduces Listeria monocytogenes on whole-muscle beef jerky and smoked pork and beef sausage sticks, four packaging systems (heat sealed [HS] without vacuum, heat sealed with oxygen scavenger, nitrogen flushed with oxygen scavenger [NFOS], and vacuum) and four ambient temperature storage times were evaluated. Commercially available whole-muscle beef jerky and smoked pork and beef sausage sticks were inoculated with a five-strain L. monocytogenes cocktail, packaged, and then stored at 25.

ABET Criterion 3.f: how much curriculum content is enough?

Even after multiple cycles of ABET accreditation, many engineering programs are unsure of how much curriculum content is needed to meet the requirements of ABET's Criterion 3.f (an understanding of professional and ethical responsibility). This study represents the first scholarly attempt to assess the impact of curriculum reform following the introduction of ABET Criterion 3.

I know what you did: The effects of interpersonal deviance on bystanders.

Using social information processing theory, we explore how interpersonally directed deviance affects work group members who observe or are aware of these insidious behaviors. In a field study, we find that indirect knowledge of work group member interpersonal deviance leads to subsequent interpersonal deviance of a focal individual. We also find that when work group cohesion is high, direct observation of deviance is more likely to result in subsequent bystander deviance.

Assessing two-dimensional crystallization trials of small membrane proteins for structural biology studies by electron crystallography.

Electron crystallography has evolved as a method that can be used either alternatively or in combination with three-dimensional crystallization and X-ray crystallography to study structure-function questions of membrane proteins, as well as soluble proteins. Screening for two-dimensional (2D) crystals by transmission electron microscopy (EM) is the critical step in finding, optimizing, and selecting samples for high-resolution data collection by cryo-EM. Here we describe the fundamental steps in identifying both large and ordered, as well as small 2D arrays, that can potentially supply critical information for optimization of crystallization conditions.

Candidate gene sequencing of LHX2, HESX1, and SOX2 in a large schizencephaly cohort.

Schizencephaly is a malformation of cortical development characterized by gray matter-lined clefts in the cerebral cortex and a range of neurological presentations. In some cases, there are features of septo-optic dysplasia concurrently with schizencephaly. The etiologies of both schizencephaly and septo-optic dysplasia are thought to be heterogeneous, but there is evidence that at least some cases have genetic origin.

Control of proton and electron transfer in de novo designed, biomimetic β hairpins.

Tyrosine side chains are involved in proton coupled electron transfer reactions (PCET) in many complex proteins, including photosystem II (PSII) and ribonucleotide reductase. For example, PSII contains two redox-active tyrosines, TyrD (Y160D2) and TyrZ (Y161D1), which have different protein environments, midpoint potentials, and roles in catalysis. TyrD has a midpoint potential lower than that of TyrZ, and its protein environment is distinguished by potential π-cation interactions with arginine residues.

Mutations in WDR62, encoding a centrosome-associated protein, cause microcephaly with simplified gyri and abnormal cortical architecture.

Genes associated with human microcephaly, a condition characterized by a small brain, include critical regulators of proliferation, cell fate and DNA repair. We describe a syndrome of congenital microcephaly and diverse defects in cerebral cortical architecture. Genome-wide linkage analysis in two families identified a 7.