Comparing 3D imaging devices for the measurement of cutaneous neurofibromas in patients with Neurofibromatosis Type 1.

Background: Cutaneous neurofibromas (cNFs) are a major cause of disfigurement in patients with Neurofibromatosis Type 1 (NF1). However, clinical trials investigating cNF treatments lack standardised outcome measures to objectively evaluate changes in cNF size and appearance. 3D imaging has been proposed as an objective standardised outcome measure however various systems exist with different features that affect useability in clinical settings.

Protocol for a hybrid effectiveness-implementation clinical trial evaluating video-assisted electronic consent vs standard consent for patients initiating and continuing haemodialysis in Australia (eConsent HD).

Introduction: Communicating complex information about haemodialysis (HD) and ensuring it is well understood remains a challenge for clinicians. Informed consent is a high-impact checkpoint in augmenting patients' decision awareness and engagement prior to HD. The aims of this study are to (1) develop a digital information interface to better equip patients in the decision-making process to undergo HD; (2) evaluate the effectiveness of the co-designed digital information interface to improve patient outcomes; and (3) evaluate an implementation strategy.

The impact of cutaneous neurofibromas on quality of life and mental health in neurofibromatosis type 1.

The skin manifestations of neurofibromatosis 1 significantly reduce health-related quality-of-life. However, data on the utility of existing surveys in capturing neurofibromatosis 1 skin treatment outcomes are lacking. This quantitative study examined the relationship between clinician-rated severity and visibility and patient-rated itch and quality-of-life (QoL) to (1) establish baseline levels of skin- and condition-specific-related QoL, itch, depression and anxiety; (2) identify patient concerns to inform the development and evaluation of skin interventions; and (3) compare the sensitivity of different QoL measures.

The efficacy and safety of deucravacitinib compared to methotrexate, in patients with vulvar lichen planus who have failed topical therapy with potent corticosteroids: a study protocol for a single-centre double-blinded randomised controlled trial.

Background: Vulvar lichen planus (VLP) is a chronic vulvar dermatosis that is difficult to treat and can severely impair quality of life in the absence of adequate treatment. There is a lack of high-quality evidence to direct therapy for VLP. This randomised controlled trial will be the first double-blinded study comparing systemic treatments in VLP and aims to investigate the safety and efficacy of deucravacitinib compared to methotrexate, in patients with VLP who have failed treatment with potent topical corticosteroids.

A cross-sectional study of dermatological conditions in rural and urban Timor-Leste.

Background: There is limited information about the type of skin disease in Timor-Leste. In order to determine the type and magnitude of skin disease in Timor-Leste, we conducted a cross-sectional point prevalence study of 271 patients from rural and urban Timor-Leste. The aim of the study was to estimate the magnitude and burden of dermatological disease.

A randomized controlled trial comparing video-assisted informed consent with standard consent for Mohs micrographic surgery.

Background: There is a need for improvement in informed medical consent to address the lack of standardization and to increase patient engagement.

Objective: To investigate the use of a video to aid informed consent for Mohs micrographic surgery and evaluate patient understanding, satisfaction, anxiety, and time savings relative to verbal consent.

Methods: A 2-armed randomized controlled trial involving 102 patients compared video-assisted consent with a control group who underwent consent in the standard verbal manner.

Society of Dermatology Hospitalists supportive care guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening conditions with high morbidity and mortality. Supportive care management of SJS/TEN is highly variable. A systematic review of the literature was performed by dermatologists, ophthalmologists, intensivists, and gynecologists with expertise in SJS/TEN to generate statements for supportive care guideline development.

Vulvar quality of life index (VQLI) - A simple tool to measure quality of life in patients with vulvar disease.

Background/objectives: Vulvar disease leads to significant disease burden and reduced quality of life (QOL). However, vulvar disease-specific instruments to measure QOL are lacking. We developed the Vulvar Quality of Life Index (VQLI) to comprehensively and reproducibly assess symptomatic, psychosexual and physical aspects of vulvar disease.

Cutaneous malignancies in Indigenous Peoples of urban Sydney.

Introduction: Despite 3% of Australians identifying as Indigenous, cutaneous malignancies in these patients, including incidence, risk factors and outcomes have not been investigated. This is despite recognition that cancer outcomes in this population are significantly poorer.

Methods: We undertook a retrospective case series of Indigenous Peoples who presented to two urban cancer therapy centres for the management of cutaneous malignancies from 2003 to 2017.

Clonal differences in Staphylococcus aureus bacteraemia-associated mortality.

The bacterium Staphylococcus aureus is a major human pathogen for which the emergence of antibiotic resistance is a global public health concern. Infection severity, and in particular bacteraemia-associated mortality, has been attributed to several host-related factors, such as age and the presence of comorbidities. The role of the bacterium in infection severity is less well understood, as it is complicated by the multifaceted nature of bacterial virulence, which has so far prevented a robust mapping between genotype, phenotype and infection outcome.

Viral exanthems.

Purpose Of Review: Determining the viral cause of a rash presents significant diagnostic challenges. We review contemporary literature on viral exanthems and suggest a structured approach to aid diagnosis.

Recent Findings: Strains responsible for, and the clinical presentation of, enteroviral infections have diverged from classic descriptions.

Impact of infectious diseases consultation on the management of Staphylococcus aureus bacteraemia in children.

Objectives: Infectious diseases consultation (IDC) in adults with Staphylococcus aureus bacteraemia (SAB) has been shown to improve management and outcome. The aim of this study was to evaluate the impact of IDC on the management of SAB in children.

Study Design: Observational cohort study of children with SAB.

Pentoxifylline-induced drug rash with eosinophilia and systemic symptoms (DRESS) in a patient with caffeine intolerance.

Background: Drug rash with eosinophilia and systemic symptoms (DRESS) is a severe drug reaction characterised by rash, eosinophilia and systemic involvement.

Main Observations: We report a case of DRESS induced by pentoxifylline used for the treatment of severe alcoholic hepatitis, in a patient with longstanding caffeine intolerance. A history of intolerance to caffeine and other methylxanthines is listed as a contraindication to the use of pentoxifylline, yet this precaution is not mentioned in alcoholic hepatitis treatment guidelines.

Genetic variants in the promoter of TARDBP in sporadic amyotrophic lateral sclerosis.

All patients with sporadic amyotrophic lateral sclerosis (SALS) have TDP-43 inclusions in their motor neurons, suggesting this protein plays a major role in the disease. Coding mutations in the gene for TDP-43, TARDBP, have been found in only a few patients with SALS. However, the non-coding regulatory regions of TARDBP have not yet been examined in SALS.

A comparison of the lengths of androgen receptor triplet repeats in brain and blood in motor neuron diseases.

Background: Expansions of triplet repeats are found in a number of neurodegenerative conditions, and different tissues in the same person can have varying repeat lengths. In Kennedy disease, motor neuron loss is due to expansion of the CAG repeat length in the androgen receptor gene (AR). We hypothesised that patients with other sporadic motor neuron diseases could have AR expansions that were restricted to CNS tissue.

Are enteroviral receptors different in sporadic motor neuron disease?

Enteroviruses have been suspected to play a part in the pathogenesis of sporadic motor neuron disease (SMND). Intercellular adhesion molecule type-1 (ICAM1) and coxsackie and adenovirus receptor (CAR) act as receptors for a number of enteroviruses. We therefore examined the viral binding domains of ICAM1 and CAR to see if any changes could be found that might predispose to enteroviral infections.

A polymorphism in the poliovirus receptor gene differs in motor neuron disease.

Poliovirus has been implicated in the etiology of sporadic motor neuron disease. DNA polymorphisms in the poliovirus receptor gene (PVR) are associated with persistent poliovirus infection in cell culture. PVR DNA polymorphisms were therefore studied in 110 cases of amyotrophic lateral sclerosis, 30 cases of progressive muscular atrophy (a disorder of lower motor neurons) and 280 normal controls.