Management of Skin Lesions in Patients with Epidermolysis Bullosa by Topical Treatment: Systematic Review and Meta-Analysis.

Epidermolysis bullosa (EB) is the overarching term for a set of rare inherited skin fragility disorders that result from mutations in at least 20 different genes. Currently, there is no cure for any of the EB subtypes associated with various mutations. Existing therapies primarily focus on alleviating pain and promoting early wound healing to prevent potential complications. Consequently, there is an urgent need for innovative therapeutic approaches. The objective of this research was to assess the efficacy of various topical treatments in patients with EB with the goal of achieving wound healing. A secondary objective was to analyse the efficacy of topical treatments for symptom reduction. A literature search was conducted using scientific databases, including The Cochrane Library, Medline (Pubmed), Web of Science, CINHAL, Embase, and Scopus. The protocol review was registered in PROSPERO (ID: 418790), and inclusion and exclusion criteria were applied, resulting in the selection of 23 articles. Enhanced healing times were observed compared with the control group. No conclusive data have been observed on pain management, infection, pruritus episodes, and cure rates over time. Additionally, evidence indicates significant progress in gene therapies (B-VEC), as well as cell and protein therapies. The dressing group, Oleogel S-10, allantoin and diacerein 1%, were the most represented, followed by fibroblast utilisation. In addition, emerging treatments that improve the patient's innate immunity, such as calcipotriol, are gaining attention. However, more trials are needed to reduce the prevalence of blistering and improve the quality of life of individuals with epidermolysis bullosa.

The Effects of Critical Care Nutrition on Weight Gain in African Penguin (Spheniscus demersus) Chicks.

Nutritional support in malnourished animals is an essential aspect of wildlife rehabilitation; this support is especially relevant when providing lifesaving nutrition to endangered species such as the African penguin (Spheniscus demersus). This study investigated the short-term effects of a commercially available, semi-elemental, critical care diet compared with a hand-made fish formula. Twenty-one African penguin chicks were selected on admission to the Southern African Foundation for the Conservation of Costal Birds in Cape Town, South Africa, in November 2015. Initial assessment included body weight, a full clinical exam, white blood cell count, packed cell volume, and total plasma protein. Ten animals received the commercial critical care diet, whereas a control group of 11 animals were fed hand-made formula for the 2-week study period. All animals were weighed daily and blood sampling was repeated after 14 days. The median weight of both groups increased significantly over 14 days (critical care diet χ2 = 10.1, P = 0.002; control χ2 = 7.4, P = 0.006). The difference was not significant between the groups for start weight (χ2 = 0.1, P = 0.725) or end weight (χ2 = 0, P = 1.000) and was not significantly different in the change over time for either absolute numbers (χ2 = 1.7, P = 0.193) or percent gain (χ2 = 0.8, P = 0.36). The values for packed cell volume, total plasma protein, and white blood cell count increased in all animals after the 14-day study period was complete. On the basis of the results of this study, it was determined that the differing diets led to similar weight gain.

Benefits of Routine Screening for Renal Cell Carcinoma of Native Kidney in Renal Transplant Recipients.

BACKGROUND: Renal-cell carcinoma (RCC) is the most common solid organ cancer in kidney transplantation recipients (KTRs). BACKGROUND: Analyze the incidence, prognosis, and evolution of primitive kidney RCC in KTRs at our institution. MATERIAL AND METHODS: Observational descriptive retrospective study in which all KTRs from January 2000 to December 2022 were included. We performed an annual abdominal ultrasound in all KTRs. Demographic and clinical data were collected. The surgical approach, location, size, histologic type, and tumor grade were analyzed. We assessed the coexistence of risk factors. We reported the appearance of tumors in other locations, changes in immunosuppressants (IS) after the diagnosis, and survival and recurrence rates observed during follow-up. RESULTS: Eighteen RCCs of native kidneys were diagnosed with an incidence in our population of 1.08%. The majority were men (77.8%), with a mean age of 59.9 years. The pathologic analysis revealed 11 clear cell carcinomas, 6 papillary carcinomas, and 1 chromophobe cell carcinoma. The median tumor size was 2.7 cm. TNM stage was T1aN0M0 in 15 cases. Laparoscopy was performed to remove the tumor in most cases. All our patients underwent changes in IS therapy, with conversion to mammalian target of rapamycin inhibitors when possible and reduction of IS in all of them. After a mean follow-up of 78.6 months, survival was 100% without tumor recurrence. Seven of the patients were diagnosed with a new tumor in another location. CONCLUSION: In our experience, annual abdominal ultrasound in KTRs may be an option for the early detection of RCC in native kidneys.

Prepulse inhibition deficit as a transdiagnostic process in neuropsychiatric disorders: a systematic review.

BACKGROUND: Psychopathological research is moving from a specific approach towards transdiagnosis through the analysis of processes that appear transversally to multiple pathologies. A phenomenon disrupted in several disorders is prepulse inhibition (PPI) of the startle response, in which startle to an intense sensory stimulus, or pulse, is reduced if a weak stimulus, or prepulse, is previously presented. OBJECTIVE AND METHODS: The present systematic review analyzed the role of PPI deficit as a possible transdiagnostic process for four main groups of neuropsychiatric disorders: (1) trauma-, stress-, and anxiety-related disorders (2) mood-related disorders, (3) neurocognitive disorders, and (4) other disorders such as obsessive-compulsive, tic-related, and substance use disorders. We used Web of Science, PubMed and PsycInfo databases to search for experimental case-control articles that were analyzed both qualitatively and based on their potential risk of bias. A total of 64 studies were included in this systematic review. Protocol was submitted prospectively to PROSPERO 04/30/2022 (CRD42022322031). RESULTS AND CONCLUSION: The results showed a general PPI deficit in the diagnostic groups mentioned, with associated deficits in the dopaminergic neurotransmission system, several areas implied such as the medial prefrontal cortex or the amygdala, and related variables such as cognitive deficits and anxiety symptoms. It can be concluded that the PPI deficit appears across most of the neuropsychiatric disorders examined, and it could be considered as a relevant measure in translational research for the early detection of such disorders.

NbSe2 Nanosheets/Nanorolls Obtained via Fast and Direct Aqueous Electrochemical Exfoliation for High-Capacity Lithium Storage.

Layered transition-metal dichalcogenides (LTMDs) in two-dimensional (2D) form are attractive for electrochemical energy storage, but research efforts in this realm have so far largely focused on the best-known members of such a family of materials, mainly MoS2, MoSe2, and WS2. To exploit the potential of further, currently less-studied 2D LTMDs, targeted methods for their production, preferably by cost-effective and sustainable means, as well as control over their nanomorphology, are highly desirable. Here, we report a quick and straightforward route for the preparation of 2D NbSe2 and other metallic 2D LTMDs that relies on delaminating their bulk parent solid under aqueous cathodic conditions. Unlike typical electrochemical exfoliation methods for 2D materials, which generally require an additional processing step (e.g., sonication) to complete delamination, the present electrolytic strategy yielded directly exfoliated nano-objects in a very short time (1-2 min) and with significant yields (∼16 wt %). Moreover, the dominant morphology of the exfoliated 2D NbSe2 products could be tuned between rolled-up nanosheets (nanorolls) and unfolded nanosheets, depending on the solvent where the nano-objects were dispersed (water or isopropanol). This rather unusual delamination behavior of NbSe2 was explored and concluded to occur via a redox mechanism that involves some degree of hydrolytic oxidation of the material triggered by the cathodic treatment. The delamination strategy could be extended to other metallic LTMDs, such as NbS2 and VSe2. When tested toward electrochemical lithium storage, electrodes based on the exfoliated NbSe2 products delivered very high capacity values, up to 750-800 mA h g-1 at 0.5 A g-1, where the positive effect of the nanoroll morphology, associated to increased accessibility of the lithium storage sites, was made apparent. Overall, these results are expected to expand the availability of fit-for-purpose 2D LTMDs by resorting to simple and expeditious production strategies of low environmental impact.

Dermatomyositis unleashed by immune checkpoint inhibitors. Three additional cases and a review of the literature.

OBJECTIVES: Immune checkpoint inhibitors (ICI) have revolutionized the treatment of several locally advanced and metastatic tumors. They enhance the effector function of the immune system, consequently leading to different immune-related adverse events. The aim of the present study was to describe three cases of dermatomyositis (DM) triggered by ICI diagnosed at our institution and to perform a review of the literature. METHODS: We performed a retrospective clinical, laboratory, and pathological evaluation of three cases of DM triggered by ICI belonging to a cohort of 187 DM patients from the Clinic Hospital Muscle Research Group of Barcelona from January 2009 to July 2022. Moreover, we undertook a narrative review of the literature from January 1990 to June 2022. RESULTS: Cases from our institution were triggered by avelumab, an anti-PD-1 ligand (PD-L1), nivolumab, and pembrolizumab, both anti-programmed death-1 (PD-1). One of these patients had locally advanced melanoma, and two had urothelial carcinoma. The severity and response to treatment were heterogeneous among the different cases. All were positive at high titers for anti-TIF1γ autoantibodies; in one of them, serum before the onset of ICI was available, and anti-TIF1γ autoantibodies were already present. RNA expression of IFNB1, IFNG and genes stimulated by these cytokines were markedly elevated in these patients. CONCLUSIONS: In conclusion, data from our patients and the narrative review suggest that early positivity to anti-TIF1γ unleashed by ICI may play a role in the development of full-blown DM, at least in some cases.

Real-world safety and effectiveness of cenobamate in patients with focal onset seizures: Outcomes from an Expanded Access Program.

OBJECTIVE: This study investigated early, real-world outcomes with cenobamate (CNB) in a large series of patients with highly drug-resistant epilepsy within a Spanish Expanded Access Program (EAP). METHOD: This was a multicenter, retrospective, observational study in 14 hospitals. Inclusion criteria were age ≥18 years, focal seizures, and EAP authorization. Data were sourced from patient clinical records. Primary effectiveness endpoints included reductions (100%, ≥90%, ≥75%, and ≥50%) or worsening in seizure frequency at 3-, 6-, and 12-month visits and at the last visit. Safety endpoints included rates of adverse events (AEs) and AEs leading to discontinuation. RESULTS: The study included 170 patients. At baseline, median epilepsy duration was 26 years and median number of seizures/month was 11.3. The median number of prior antiseizure medications (ASMs) and concomitant ASMs were 12 and 3, respectively. Mean CNB dosages/day were 176 mg, 200 mg, and 250 mg at 3, 6, and 12 months. Retention rates were 98.2%, 94.5%, and 87% at 3, 6, and 12 months. At last available visit, the rate of seizure freedom was 13.3%; ≥90%, ≥75%, and ≥50% responder rates were 27.9%, 45.5%, and 63%, respectively. There was a significant reduction in the number of seizures per month (mean: 44.6%; median: 66.7%) between baseline and the last visit (P < 0.001). Responses were maintained regardless of the number of prior or concomitant ASMs. The number of concomitant ASMs was reduced in 44.7% of patients. The cumulative percentage of patients with AEs and AEs leading to discontinuation were 68.2% and 3.5% at 3 months, 74.1% and 4.1% at 6 months, and 74.1% and 4.1% at 12 months. The most frequent AEs were somnolence and dizziness. SIGNIFICANCE: In this highly refractory population, CNB showed a high response regardless of prior and concomitant ASMs. AEs were frequent but mostly mild-to-moderate, and few led to discontinuation.

Temperature-Dependent Anisotropic Refractive Index in ß-Ga2O3: Application in Interferometric Thermometers.

An accurate knowledge of the optical properties of ß-Ga2O3 is key to developing the full potential of this oxide for photonics applications. In particular, the dependence of these properties on temperature is still being studied. Optical micro- and nanocavities are promising for a wide range of applications. They can be created within microwires and nanowires via distributed Bragg reflectors (DBR), i.e., periodic patterns of the refractive index in dielectric materials, acting as tunable mirrors. In this work, the effect of temperature on the anisotropic refractive index of ß-Ga2O3n(λ,T) was analyzed with ellipsometry in a bulk crystal, and temperature-dependent dispersion relations were obtained, with them being fitted to Sellmeier formalism in the visible range. Micro-photoluminescence (µ-PL) spectroscopy of microcavities that developed within Cr-doped ß-Ga2O3 nanowires shows the characteristic thermal shift of red-infrared Fabry-Perot optical resonances when excited with different laser powers. The origin of this shift is mainly related to the variation in the temperature of the refractive index. A comparison of these two experimental results was performed by finite-difference time-domain (FDTD) simulations, considering the exact morphology of the wires and the temperature-dependent, anisotropic refractive index. The shifts caused by temperature variations observed by µ-PL are similar, though slightly larger than those obtained with FDTD when implementing the n(λ,T) obtained with ellipsometry. The thermo-optic coefficient was calculated.

High-risk mucosal human papillomavirus-associated squamous cell carcinoma of the fingers presents distinctive clinicopathological characteristics and outcomes: a bicentric retrospective study.

BACKGROUND: High-risk mucosal human papillomavirus (HR-HPV) seems to play a role in cutaneous squamous cell carcinoma (cSCC), particularly in nail tumours, where genitodigital transmission has been suggested. The role of HR-HPV in nonungual cSCC of the finger needs to be clarified. AIM: To evaluate the prevalence, clinicopathological characteristics, surrogates and outcomes of HR-HPV in cSCC of the finger. METHODS: This was an observational bicentric study including patients with an excised in situ or invasive cSCC located on the finger. Differences in HR-HPV and non-HR-HPV tumours were evaluated. RESULTS: Forty-five patients (45 tumours) were included. HR-HPV was detected in 33% of cases (22% HPV type 16). The mean age was lower in patients with HR-HPV than in those with non-HR-HPV (62·4 vs. 81·1 years, P = 0·001). HR-HPV tumours were smaller (10 mm vs. 15 mm, P = 0·07) and more frequently intraepidermal (60% vs. 20%, P = 0·004). The absence of elastosis (P = 0·030) and inflammation (P = 0·026) and the presence of basaloid morphology (P = 0·003) were surrogates of HR-HPV detection. Mean p16 positivity was 61% in HR-HPV and 36% in non-HR-HPV tumours (P = 0·061). Recurrence after surgery was more common in HR-HPV tumours (58% vs. 34%), although this was not statistically significant. HR-HPV was detected in 27% of the nonungual tumours. CONCLUSION: HR-HPV-associated cSCC of the finger appears in younger patients, is smaller and is less infiltrative than non-HR-HPV tumours. The presence of a basaloid morphology and the absence of elastosis and inflammation could be used as markers for HR-HPV detection. The high prevalence of HR-HPV in nonungual cSCC suggests its aetiopathogenic role in these tumours.

Photoprotection for people with skin of colour: needs and strategies.

Skin of colour or pigmented skin has unique characteristics: it has a higher eumelanin-to-pheomelanin ratio, more mature melanosomes, an increased amount of melanin distributed in the upper layers of the epidermis, and more efficient DNA repair compared with lighter skin. However, individuals with skin of colour are at a significant risk of skin damage caused by ultraviolet radiation, including the development of photodermatoses and photoageing changes such as uneven skin tone, and are predisposed to pigmentary disorders. In fact, one of the most common conditions leading to dermatology consultations by patients with skin of colour is photoexacerbated pigmentary disorders. Unfortunately, individuals with skin of colour may be less prone to engage in photoprotective measures, including the use of sunscreens. Physicians are also less likely to prescribe sunscreens for them. There is thus a clear need for better education on photodamage and for more efficient and suitable photoprotection in populations with skin of colour. However, this need has thus far only partially been met, and the development of sunscreen products designed to provide optimal photoprotection for people with skin of colour remains a challenge. Targeted sunscreens for individuals with skin of colour require optimal cosmetic appeal (leaving no white residue and not disrupting skin tone). They should include broad-spectrum [ultraviolet (UV)B/UVA] protection with high sun protection factor, as well as protection against long-wave UVA (UVA1) and visible light, as these wavelengths are capable of inducing or augmenting pigmentary disorders. They may also contain depigmenting agents for patients with pigmentary disorders.