Exploring the Content Validity of the Unified Wilson Disease Rating Scale: Insights from Qualitative Research.

Introduction: Wilson disease (WD) is a rare metabolic disorder of impaired copper transport manifesting in hepatic, neurological, and psychiatric symptoms. To evaluate the clinical symptoms of WD in clinical trials, a group of clinicians created the Unified Wilson Disease Rating Scale (UWDRS). Content validity of this scale has not been established.

Variation in Endoscopic Activity Assessment and Endoscopy Score Validation in Adults With Eosinophilic Esophagitis.

Background & Aims: Eosinophilic esophagitis (EoE) is assessed endoscopically (endoscopic activity), based on grades of edema, rings, exudates, furrows, and strictures (EREFS). We examined variations in endoscopic assessments of severity, developed and validated 3 EREFS-based scoring systems, and assessed responsiveness of these systems using data from a randomized placebo-controlled trial of patients with EoE.

Methods: For the development set, 5 gastroenterologists reviewed EREFS findings from 266 adults with EoE and provided endoscopist global assessment scores (EndoGA, scale of 0 to 10); variation (ΔEndoGA) was assessed using linear regression.

Tat controls transcriptional persistence of unintegrated HIV genome in primary human macrophages.

In HIV infected macrophages, a large population of viral genomes persists as the unintegrated form (uDNA) that is transcriptionally active. However, how this transcriptional activity is controlled remains unclear. In this report, we investigated whether Tat, the viral transactivator of transcription, is involved in uDNA transcription.

The steady-state response of the cerebral cortex to the beat of music reflects both the comprehension of music and attention.

The brain's analyses of speech and music share a range of neural resources and mechanisms. Music displays a temporal structure of complexity similar to that of speech, unfolds over comparable timescales, and elicits cognitive demands in tasks involving comprehension and attention. During speech processing, synchronized neural activity of the cerebral cortex in the delta and theta frequency bands tracks the envelope of a speech signal, and this neural activity is modulated by high-level cortical functions such as speech comprehension and attention.

A clinicopathologic study of diencephalic pediatric low-grade gliomas with BRAF V600 mutation.

Among brain tumors, the BRAF (V600E) mutation is frequently associated with pleomorphic xanthoastrocytomas (PXAs) and gangliogliomas (GGs). This oncogenic mutation is also detected in ~5 % of other pediatric low-grade gliomas (LGGs) including pilocytic astrocytomas (PAs) and diffuse astrocytomas. In the current multi-institutional study of 56 non-PXA/non-GG diencephalic pediatric LGGs, the BRAF (V600) mutation rate is 36 %.

BRCA1 functions as a novel transcriptional cofactor in HIV-1 infection.

Background: Viruses have naturally evolved elegant strategies to manipulate the host's cellular machinery, including ways to hijack cellular DNA repair proteins to aid in their own replication. Retroviruses induce DNA damage through integration of their genome into host DNA. DNA damage signaling proteins including ATR, ATM and BRCA1 contribute to multiple steps in the HIV-1 life cycle, including integration and Vpr-induced G2/M arrest.

HIV-1 triggers WAVE2 phosphorylation in primary CD4 T cells and macrophages, mediating Arp2/3-dependent nuclear migration.

The human immunodeficiency virus type 1 (HIV-1) initiates receptor signaling and early actin dynamics during viral entry. This process is required for viral infection of primary targets such as resting CD4 T cells. WAVE2 is a component of a multiprotein complex linking receptor signaling to dynamic remodeling of the actin cytoskeleton.

Development of a mild traumatic brain injury-specific vision screening protocol: a Delphi study.

Although traumatic brain injury (TBI) can happen to anyone at any time, the wars in Iraq and Afghanistan have brought it renewed attention. Fortunately, most cases of TBI from the recent conflicts are mild TBI (mTBI). Still, many physical, psychological, and social problems are associated with mTBI.

A potential role for adjunctive vitamin D therapy in the management of weight gain and metabolic side effects of second-generation antipsychotics.

Second-generation antipsychotic (SGA) medications introduced about 20 years ago are increasingly used to treat psychiatric illnesses in children and adolescents. There has been a five-fold increase in the use of these medications in U.S.

A systematic review of the Diagnostic and Statistical Manual diagnostic criteria for nicotine dependence.

The Diagnostic and Statistical Manual diagnostic criteria for nicotine dependence (DSM-ND) are based on the proposition that dependence is a syndrome that can be diagnosed only when a minimum of 3 of the 7 proscribed features are present. The DSM-ND criteria are an accepted research measure, but the validity of these criteria has not been subjected to a systematic evaluation. To systematically review evidence of validity and reliability for the DSM-ND criteria, a literature search was conducted of 16 national and international databases.

Addressing tobacco use through organizational change: a case study of an addiction treatment organization.

Compared to the general population, persons entering addiction treatment are three to four times more likely to be tobacco dependent and even addiction treatment staff members are two to three times more likely to be tobacco dependent. In these settings, tobacco use continues to be the norm; however addiction treatment programs are increasingly aware of the need to assess for and treat tobacco dependence. The problem is a cultural issue that is so ingrained that assumptions about tobacco use and dependence in addiction treatment are rarely questioned.

Public speaking maxim No. 101. The corpse is more comfortable than the eulogizer at a funeral.

Making a presentation can be a grueling experience. But imagine making one without any preparation--as a job candidate, no less.

Everything I needed to know about medical management I learned in acting school.

Some people are doctors. Some people play them on TV. Brian Meltzer could probably do both.

Acetaminophen (Tylenol): Johnson & Johnson and consumer safety.

Considers, against backdrop of Johnson & Johnson's handling of the Tylenol poisonings of the 1980s, Johnson & Johnson's response to product safety concerns raised about acetaminophen by studies suggesting links to hepatotoxicity and end-stage renal disease.

Psychotic episodes in an elderly woman with an anticentromere-positive scleroderma variant, IgA deficiency, and hypothyroidism.

A 66-year-old woman presented with a recurrent psychotic disorder with central manifestations that initially suggested a delusional disorder. Peripheral stiffness was at first attributed to a dystonic reaction secondary to neuroleptic treatment. The atypical presentation led to suspicion of collagen vascular disease.

Emergence and human conduct.

Emergence, the occurrence of novel, unpredictable human behavior, is an inherent aspect of human experience on both the individual and collective levels. In this article, we considered the ubiquitous nature of emergent events, indicated some of the ways such events manifest themselves, analyzed the personal and interpersonal processes that give rise to them, and suggested some of their implications for psychology and the other social sciences. Given the inescapable presence of emergence in human conduct, a redefinition of the goals and scope of social science may be desirable.

Uterine leiomyosarcoma with massive necrosis diagnosed during gonadotropin-releasing hormone analog therapy for presumed uterine fibroid.

Recurrence of heavy vaginal bleeding and massive necrosis of a uterine leiomyosarcoma are reported in a 41-year-old female who was being treated with GnRH-a for a presumed uterine fibroid. The pathogenic mechanisms of such an event are reviewed and discussed in light of the available literature on the subject of GnRH-a and the treatment of uterine smooth muscle neoplasms.

Arterial blood pressure response in conscious rabbits to administration of C-terminal fragment of substance P into the cerebral ventricles, intravenously and intragastrically.

The blood pressure was measured in the common carotid artery in a cutaneous bridge in conscious rabbits. The synthetic hexapeptide SP6-11 of C-terminal fragment of Arg-Pro-Lys-Pro-Gln-Gln-Phe-Phe-Gly-Leu-Met-NH2 (Substance P) was administered to animals accustomed to the experimental environment in three separate trials: into the cerebral ventricles, intravenously and intragastrically. Introduction of 2 nmol/kg hexapeptide into the cerebral ventricles via a chronically implanted cannula in the lateral ventricle caused an increase in the blood pressure, reaching a peak 10 min after administration.

Action pattern of human pancreatic and salivary alpha-amylase on 1,4-alpha-D-nitrophenylmaltooligosaccharides. 1,4-alpha-D-nitrophenylmaltooligosaccharides as substrates of alpha-amylse, I.

High performance liquid chromatography (HPLC) was used to monitor the purity of the substrates and to establish the patterns of hydrolysis of ortho- and para-nitrophenylmaltooligosaccharides (2-7 glucose residues) catalysed by human pancreatic and salivary alpha-amylase. Separation of the reaction products from the remaining substrate was performed on a TSK-G-2000 PW or a RP18 column. By measuring the quantitative distribution of products, and assuming a 5-subsite model for the active site of alpha-amylase, differential activities for the hydrolysis of the different glycosidic bonds in the 2 series of substrates were deduced.