Measuring the attitudes of novice drivers with autism spectrum disorder as an indication of apprehensive driving: Going beyond basic abilities.

For some individuals with autism spectrum disorder, driving apprehension may interfere with the acquisition and application of driving privileges. The Driving Attitude Scale Parent-Report provides an indication of novice drivers' positive and negative attitudes toward driving. Responses were compared for parents of 66 autism spectrum disorder and 166 neuro-typical novice drivers.

Functional Outcome After Resection of Von Hippel-Lindau Disease-Associated Cauda Equina Hemangioblastomas: An Observational Cohort Study.

Background: Cauda equina hemangioblastomas in von Hippel-Lindau (VHL) disease can cause significant neurological signs and symptoms. Despite their associated morbidity, the management of these tumors remains incompletely defined.

Objective: To determine optimal management, we analyzed the functional outcomes after resection of these tumors.

Specific allergen profiles of peanut foods and diagnostic or therapeutic allergenic products.

Background: Generic immunoassays for peanut cannot discriminate between allergen levels in peanut-derived food products or therapeutics. Clinical trials of oral immunotherapy (OIT) are strengthened by using standardized peanut preparations with defined doses of major allergens.

Objective: This article describes measurement of Ara h 1, Ara h 2, and Ara h 6 in peanut foods and in peanut flour extracts used for allergy diagnosis and OIT.

Can Youth with Autism Spectrum Disorder Use Virtual Reality Driving Simulation Training to Evaluate and Improve Driving Performance? An Exploratory Study.

Investigate how novice drivers with autism spectrum disorder (ASD) differ from experienced drivers and whether virtual reality driving simulation training (VRDST) improves ASD driving performance. 51 novice ASD drivers (mean age 17.96 years, 78% male) were randomized to routine training (RT) or one of three types of VRDST (8-12 sessions).

Prospective study of giant paraesophageal hernia repair with 1-year follow-up.

Objective: Evaluating giant paraesophageal hernia (GPEH) repair requires long-term follow-up. GPEH repair can have associated high recurrence rates, yet this incidence depends on how recurrence is defined. Our objective was to prospectively evaluate patients undergoing GPEH repair with 1-year follow-up.

Three-column osteotomy for correction of cervical and cervicothoracic deformities: alignment changes and early complications in a multicenter prospective series of 23 patients.

Purpose: Three-column osteotomy (3CO), including pedicle subtraction osteotomy (PSO) and vertebral column resection (VCR), can provide powerful alignment correction for adult cervical deformity (ACD). Our objective was to assess alignment changes and early complications associated with 3CO for ACD.

Methods: ACD patients treated with 3CO with minimum 90-day follow-up were identified from a prospectively collected multicenter ACD database.

Oral ghrelin receptor agonist MK-0677 increases serum insulin-like growth factor 1 in hemodialysis patients: a randomized blinded study.

Background: Protein-energy wasting (PEW) in end-stage renal disease (ESRD) patients is associated with increased morbidity and mortality, but options for treatment are limited. Growth hormone (GH) increases insulin-like growth factor 1 (IGF-1), with improved nutritional parameters, but must be given subcutaneously and does not provide normal GH secretion patterns. MK-0677, an oral ghrelin receptor agonist (GRA), maintains normal GH secretion and increases lean body mass in normal subjects; it has not been studied in dialysis patients, an essential step in assessing efficacy and safety prior to clinical trials.

A Randomized Phase II Neoadjuvant Study of Cisplatin, Paclitaxel With or Without Everolimus in Patients with Stage II/III Triple-Negative Breast Cancer (TNBC): Responses and Long-term Outcome Correlated with Increased Frequency of DNA Damage Response Gene Mutations, TNBC Subtype, AR Status, and Ki67.

Because of inherent disease heterogeneity, targeted therapies have eluded triple-negative breast cancer (TNBC), and biomarkers predictive of treatment response have not yet been identified. This study was designed to determine whether the mTOR inhibitor everolimus with cisplatin and paclitaxel would provide synergistic antitumor effects in TNBC. Patients with stage II/III TNBC were enrolled in a randomized phase II trial of preoperative weekly cisplatin, paclitaxel and daily everolimus or placebo for 12 weeks, until definitive surgery.

Clinical implications of the melanopsin-based non-image-forming visual system.

Since the discovery of the non-image-forming visual system, tremendous research efforts have been dedicated to understanding its mechanisms and functional roles. Original functions associated with the melanopsin system include the photoentrainment of circadian sleep-wake cycles and the pupillary light reflex. Recent findings, however, suggest a much broader involvement of this system in an array of physiologic responses to light.

Dependence of Glomerulonephritis Induction on Novel Intraglomerular Alternatively Activated Bone Marrow-Derived Macrophages and Mac-1 and PD-L1 in Lupus-Prone NZM2328 Mice.

Glomerular damage mediated by glomerulus-infiltrating myeloid-derived cells is a key pathogenic event in lupus nephritis (LN), but the process is poorly understood. Confocal microscopy of kidney sections and flow cytometry analysis of glomerular cells from magnetic bead-purified glomeruli have identified glomerulus-infiltrating leukocyte populations in NZM2328 (NZM) lupus-prone mice with spontaneous chronic glomerulonephritis (GN) and anti-glomerular basement membrane-induced nephritis. The occurrence of a major glomerulus-infiltrating CD11bF4/80I-A macrophage population exhibiting the markers programmed death ligand-1 (PD-L1), Mac-2, and macrophage mannose receptor (CD206) and producing , , , , and mRNA, which are known to be expressed by alternatively activated (M2b) macrophages, correlated with proteinuria status.

Convection-enhanced delivery improves MRI visualization of basal ganglia for stereotactic surgery.

OBJECTIVE Stereotactic deep brain stimulation surgery is most commonly performed while patients are awake. This allows for intraoperative clinical assessment and electrophysiological target verification, thereby promoting favorable outcomes with few side effects. Intraoperative CT and MRI have challenged this concept of clinical treatment validation.

Loss of Quiescence in von Hippel-Lindau Hemangioblastomas is Associated with Erythropoietin Signaling.

von Hippel-Lindau (VHL) patients develop multiple central nervous system hemangioblastomas (HB). Some HBs become symptomatic with exponential growth or cyst formation following long periods of quiescence. Understanding the factors underlying growth in hemangioblastoma may lead to better strategies to arrest or prevent tumor growth.

A Randomized Trial of Focused Ultrasound Thalamotomy for Essential Tremor.

Background: Uncontrolled pilot studies have suggested the efficacy of focused ultrasound thalamotomy with magnetic resonance imaging (MRI) guidance for the treatment of essential tremor.

Methods: We enrolled patients with moderate-to-severe essential tremor that had not responded to at least two trials of medical therapy and randomly assigned them in a 3:1 ratio to undergo unilateral focused ultrasound thalamotomy or a sham procedure. The Clinical Rating Scale for Tremor and the Quality of Life in Essential Tremor Questionnaire were administered at baseline and at 1, 3, 6, and 12 months.

Neisseria gonorrhoeae Evades Calprotectin-Mediated Nutritional Immunity and Survives Neutrophil Extracellular Traps by Production of TdfH.

Neisseria gonorrhoeae successfully overcomes host strategies to limit essential nutrients, termed nutritional immunity, by production of TonB-dependent transporters (TdTs)-outer membrane proteins that facilitate nutrient transport in an energy-dependent manner. Four gonococcal TdTs facilitate utilization of iron or iron chelates from host-derived proteins, including transferrin (TbpA), lactoferrin (LbpA), and hemoglobin (HpuB), in addition to xenosiderophores from other bacteria (FetA). The roles of the remaining four uncharacterized TdTs (TdfF, TdfG, TdfH, and TdfJ) remain elusive.

Spindle Cell Melanoma and Interdigitating Dendritic Cell Sarcoma: Do They Represent the Same Process?

Intranodal spindle cell lesions on biopsy are problematic for a surgical pathologist, often requiring an extensive immunohistochemical evaluation with variable and frequently unsatisfactory results. In the absence of a history of malignancy, the differential diagnosis of a spindle cell tumor must include both a primary nodal proliferation and a metastatic process. Particularly challenging are those lesions that share morphologic and immunohistochemical features; spindle cell melanomas (SCM) and interdigitating dendritic cell sarcomas (IDCS) belong to this category.

Descending Aortic Translocation for Relief of Distal Tracheal and Proximal Bronchial Compression.

Background: A descending thoracic aorta that traverses the midline is an uncommon cause of airway compression affecting the distal trachea and proximal main bronchi. Posterior aortopexy has had inconsistent results.

Methods: A retrospective review determined that, since 2004, 5 children have undergone descending aortic translocation at Texas Children's Hospital.

Patency of the posterior communicating artery following treatment with the Pipeline Embolization Device.

OBJECTIVE The Pipeline Embolization Device (PED) has become an effective treatment strategy for some cerebral aneurysms. Concerns regarding the patency of branch arteries have been raised. The objective of this study was to assess the patency of the posterior communicating artery (PCoA) following treatment of PCoA aneurysms using the PED.

The Contemporary Role of Stereotactic Radiosurgery in the Treatment of Meningiomas.

Meningiomas are among the most common intracranial tumors in adults. The mainstay of treatment has been extirpation. Stereotactic radiosurgery (SRS) is an important option in the management of inaccessible, recurrent, or residual benign meningiomas.

The Challenges of Applying Massively Parallel Sequencing to Newborn Screening for Cystic Fibrosis.

This Commentary highlights the article by Lefterova et al that describes newborn screening of cystic fibrosis using massively parallel sequencing.

An oncogenic MYB feedback loop drives alternate cell fates in adenoid cystic carcinoma.

Translocation events are frequent in cancer and may create chimeric fusions or 'regulatory rearrangements' that drive oncogene overexpression. Here we identify super-enhancer translocations that drive overexpression of the oncogenic transcription factor MYB as a recurrent theme in adenoid cystic carcinoma (ACC). Whole-genome sequencing data and chromatin maps highlight distinct chromosomal rearrangements that juxtapose super-enhancers to the MYB locus.

Human GRK4γ142V Variant Promotes Angiotensin II Type I Receptor-Mediated Hypertension via Renal Histone Deacetylase Type 1 Inhibition.

The influence of a single gene on the pathogenesis of essential hypertension may be difficult to ascertain, unless the gene interacts with other genes that are germane to blood pressure regulation. G-protein-coupled receptor kinase type 4 (GRK4) is one such gene. We have reported that the expression of its variant hGRK4γ(142V) in mice results in hypertension because of impaired dopamine D1 receptor.

Natural and surgical history of Chiari malformation Type I in the pediatric population.

OBJECT The natural and surgical history of Chiari malformation Type I (CM-I) in pediatric patients is currently not well described. In this study the authors discuss the clinical and radiological presentation and outcomes in a large cohort of pediatric CM-I patients treated with either conservative or surgical management. METHODS The authors retrospectively reviewed 95 cases involving pediatric patients with CM-I who presented between 2004 and 2013.

Managing gastroesophageal reflux disease - comparative efficacy and outcomes of dexlansoprazole MR.

The management of gastroesophageal reflux disease (GERD) has been revolutionized with the development of proton pump inhibitors (PPIs). Unfortunately, due to the inherent pharmacokinetic and pharmacodynamic profiles of conventional PPIs, many patients continue to suffer from symptoms related to GERD despite appropriate use of PPIs. Dexlansoprazole MR is a PPI with a unique dual delayed-release delivery system that has been designed to address the unmet needs in GERD management.

Biological and clinical impact of hemangioblastoma-associated peritumoral cysts in von Hippel-Lindau disease.

Objective: Peritumoral cysts are frequently associated with CNS hemangioblastomas and often underlie neurological morbidity and mortality. To determine their natural history and clinical impact, the authors prospectively analyzed hemangioblastoma-associated peritumoral cysts in patients with von Hippel-Lindau (VHL) disease.

Methods: Patients with VHL disease who had 2 or more years of follow-up and who were enrolled in a prospective study at the National Institutes of Health were included.