A case of pachydermodactyly in a seventeen year old associated with repetitive minor trauma.

A boy presented initially to a Rheumatology clinic with a three year history of asymptomatic swelling of the third to fourth proximal interphalangeal (PIP) joints bilaterally. A presumptive diagnosis of seronegative arthritis was made. Sulfasalazine was commenced without improvement and resulted in mood disturbance.

Ear health and hearing surveillance in girls and women with Turner's syndrome: recommendations from the Turner's Syndrome Support Society.

Background: Turner's syndrome (TS) is a common chromosomal disorder, affecting one in 2000 newborn girls, in which part or all of one X chromosome is missing. Ear and hearing problems are very common in girls and women with TS. The aim of this review was to review the published literature to suggest recommendations for otological health surveillance.

Empirical exploration of brilliance in health care: perceptions of health professionals.

Objective The aim of the present study was to develop a positive organisational scholarship in health care approach to health management, informed by health managers and health professionals' experiences of brilliance in health care delivery. Methods A sample of postgraduate students with professional and/or management experience within a health service was invited to share their experiences of brilliant health services via online discussions and a survey running on the SurveyMonkey platform. A lexical analysis of student contributions was conducted using the individual as the unit of analysis.

Treatment of pulmonary exacerbations in cystic fibrosis - could do better?

This article describes the nature and significance of pulmonary exacerbations in cystic fibrosis (CF). The effectiveness and safety of current exacerbation treatment are explored. The article concludes with a summary of clinical trials (completed and ongoing) which aim to improve the efficacy and safety of exacerbation treatment.

Automated single particle detection and tracking for large microscopy datasets.

Recent advances in optical microscopy have enabled the acquisition of very large datasets from living cells with unprecedented spatial and temporal resolutions. Our ability to process these datasets now plays an essential role in order to understand many biological processes. In this paper, we present an automated particle detection algorithm capable of operating in low signal-to-noise fluorescence microscopy environments and handling large datasets.

Addressing resistance to antibiotics in systematic reviews of antibiotic interventions.

Antibiotics are among the most important interventions in healthcare. Resistance of bacteria to antibiotics threatens the effectiveness of treatment. Systematic reviews of antibiotic treatments often do not address resistance to antibiotics even when data are available in the original studies.

N2S2 and N4S4 precursors to PS2 macrocycles and cyclic amidinium salts.

The cyclo-condensation of 1R,2R-diaminocyclohexane with 2,2'-(ethane-1,2-diyldisulfanediyl)dibenzaldehyde gave the 1 : 1 addition compound chxn-(im)N2S2 in high yield. When the same condensation reaction was performed with 1R,3S-diamino-1,7,7-trimethylcyclopentane as the diamine, the 2 : 2 addition compound tmcp-(im)N4S4 was obtained selectively. Reduction of the diimines gave the saturated analogues chxn-N2S2 (1) and tmcp-N4S4 (3) the former of which could be phosphorylated with PhP(NMe2)2 to give the novel 13-membered macrocycle chxn-PS2, 2.

Submucous cleft palate: outcomes after primary repair with repositioning of the levator muscle in 51 consecutive patients.

I report the incidence of hypernasal resonance, nasal emission, and fistula after intravelar surgery with retropositioning of the levator muscle by a single surgeon in a consecutive series of 51 patients who presented with symptomatic submucous cleft palate. Intravelar veloplasty with repositioning of the levator muscle was highly effective in that 37/51 patients (73%) achieved either normal or mild and inconsistent resonance (p<0.0001), and 39 (77%) normal or mild and inconsistent nasal emissions (p<0.

Diet and Major Renal Outcomes: A Prospective Cohort Study. The NIH-AARP Diet and Health Study.

Background: Chronic kidney disease (CKD) is prevalent and associated with significant morbidity and mortality. Dietary modification may be an approach to reducing CKD.

Design: In this prospective cohort study, we evaluated the association between diet quality, sodium and potassium intakes, and major renal outcomes.

A Common Variant in the Adaptor Mal Regulates Interferon Gamma Signaling.

Humans that are heterozygous for the common S180L polymorphism in the Toll-like receptor (TLR) adaptor Mal (encoded by TIRAP) are protected from a number of infectious diseases, including tuberculosis (TB), whereas those homozygous for the allele are at increased risk. The reason for this difference in susceptibility is not clear. We report that Mal has a TLR-independent role in interferon-gamma (IFN-γ) receptor signaling.

DRYAD and ADH: Further comments on explaining age-related differences in memory.

Recently, Smyth and Naveh-Benjamin (2016) questioned some of the main assumptions/hypotheses of DRYAD (or density of representations yields age-related deficits), a global-deficit model of aging and memory judgments (Benjamin, 2010; Benjamin et al., 2012). Smyth and Naveh-Benjamin (2016) provided empirical evidence that seems incompatible with DRYAD, but that fits the associative deficit hypothesis (ADH; Naveh-Benjamin, 2000), 1 specific-deficit theoretical view.

A phase 3, multi-center, multinational, randomized, double-blind, placebo-controlled study to evaluate the efficacy and safety of levofloxacin inhalation solution (APT-1026) in stable cystic fibrosis patients.

Rationale: For patients with cystic fibrosis (CF), the use of inhaled antibiotics has become standard of care to suppress chronic Pseudomonas airways infection. There are limited antibiotic options formulated and approved for inhaled use and antibiotic efficacies attenuate over time, making additional inhaled antibiotic classes desirable. APT-1026 (levofloxacin inhalation solution, LIS) is a fluoroquinolone in development for management of chronic P.

Did the use of the Guideliner V2(TM) guide catheter extension increase complications? A review of the incidence of complications related to the use of the V2 catheter, the influence of right brachiocephalic arterial anatomy and the redesign of the V3(TM) Guideliner and clinical outcomes.

Objective: We sought to investigate the incidence of complications associated with V2 Guideliner, understand the mechanisms and evaluate the impact of alterations made to the V3 Guideliner.

Methods: Retrospective analysis of consecutive cases employing V2 Guideliner from two university teaching hospitals. Complications were identified, analysed and classified into major versus minor ones.

Evaluating the Psychometric Properties of the Conners Adult ADHD Rating Scales.

Characterized by significant work-related impairments, difficulties with relationships, and higher rates of criminal behavior and substance abuse, ADHD is now understood as a disability that can persist throughout a lifetime. Differing opinions remain, however, regarding adult symptoms of ADHD, particularly with regard to gender differences. These issues add to the challenges of constructing a reliable and valid measure of ADHD for use with adults in research and practice.

Bronchoscopy-guided antimicrobial therapy for cystic fibrosis.

Background: Early diagnosis and treatment of lower respiratory tract infections are the mainstay of management of lung disease in cystic fibrosis. When sputum samples are unavailable, treatment relies mainly on cultures from oropharyngeal specimens; however, there are concerns regarding the sensitivity of these to identify lower respiratory organisms.Bronchoscopy and related procedures (including bronchoalveolar lavage) though invasive, allow the collection of lower respiratory specimens from non-sputum producers.

Glutamine supplementation in cystic fibrosis: A randomized placebo-controlled trial.

Rationale: Pulmonary infection and malnutrition in cystic fibrosis are associated with decreased survival. Glutamine has a possible anti-microbial effect, with a specific impact against Pseudomonas aeruginosa. We aimed to test the hypothesis that oral glutamine supplementation (21 g/day) for 8 weeks in adults with cystic fibrosis would decrease pulmonary inflammation and improve clinical status.

US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis: executive summary.

Non-tuberculous mycobacteria (NTM) are ubiquitous environmental organisms that can cause chronic pulmonary infection, particularly in individuals with pre-existing inflammatory lung disease, such as cystic fibrosis (CF). Pulmonary disease (PD) caused by NTM has emerged as a major threat to the health of individuals with CF, but remains difficult to diagnose and problematic to treat. In response to this challenge, the US Cystic Fibrosis Foundation (CFF) and the European Cystic Fibrosis Society (ECFS) convened a panel of 19 experts to develop consensus recommendations for the screening, investigation, diagnosis and management of NTM-PD in individuals with CF.

US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis.

Non-tuberculous mycobacteria (NTM) are ubiquitous environmental organisms that can cause chronic pulmonary infection, particularly in individuals with pre-existing inflammatory lung disease such as cystic fibrosis (CF). Pulmonary disease caused by NTM has emerged as a major threat to the health of individuals with CF but remains difficult to diagnose and problematic to treat. In response to this challenge, the US Cystic Fibrosis Foundation (CFF) and the European Cystic Fibrosis Society (ECFS) convened an expert panel of specialists to develop consensus recommendations for the screening, investigation, diagnosis and management of NTM pulmonary disease in individuals with CF.