Transition of care in adolescents with epidermolysis bullosa: The provider perspective.

The characteristics of epidermolysis bullosa (EB) demand higher than average provider support for transition from pediatric to adult care. We administered an online Qualtrics survey to members of the Epidermolysis Bullosa Clinical Research Consortium (EBCRC), a group of providers who care for patients with EB, in order to examine their practices and perspectives on transition of care (TOC) and identify barriers to successful implementation. Sixteen of eighteen medical centers completed the survey. Eighty-eight percent of center representatives expressed concerns about their patients transitioning/transferring from the pediatric to adult-centered care. Thirty-eight percent of providers reported having a formal TOC program in place. Our findings support the desire for formal TOC programs, the need for a team-based approach and, in particular, identification of adult providers to participate in the transition to improve this often challenging time.

How many is too many? A review of the significant numbers in pediatric skin lesions and their recommended evaluation.

Pediatric dermatologists are frequently consulted to evaluate children for cutaneous signs of systemic disorders. Numerical thresholds of significance have been described in the dermatologic literature for various skin findings where the likelihood of an associated extracutaneous abnormality or known genetic syndrome increases significantly. Knowledge of these numerical thresholds facilitates diagnosis and management, which improves clinical outcomes and avoids severe complications. This review highlights the clinical presentation, complications, evaluation, and numerical significance, when applicable, for the following skin findings: infantile hemangiomas, capillary malformations, café-au-lait macules, hypopigmented macules, juvenile xanthogranulomas, pilomatricomas, and angiofibromas.

Comparing long-term outcomes of children treated with new-onset type 2 diabetes in an outpatient versus inpatient setting: A retrospective chart review.

BACKGROUND: Early identification and management of pediatric type 2 diabetes mellitus (T2DM) is crucial for improving long-term outcomes. This study aimed to assess if the severity of T2DM at presentation, inferred by the location of treatment initiation (inpatient or outpatient), influences long-term clinical outcomes. METHODS: A retrospective chart review was conducted on 116 pediatric T2DM patients. Data on treatment initiation location, initial and subsequent glycated hemoglobin (HbA1c) levels, prescribed insulin, and body mass index were collected from electronic medical records. RESULTS: Of the 116 patients, 69 were initially treated in an inpatient setting, and 47 received outpatient treatment. At treatment initiation, the inpatient group had significantly higher HbA1c levels compared to the outpatient group (p < .001), but 3 years after treatment initiation, no significant difference in HbA1c was observed between the two groups (p = .057). Prescribed insulin dosages were higher in the inpatient group at treatment initiation (p < .001) and remained higher after 3 years (p < 0.003) compared to the outpatient group. CONCLUSIONS: Pediatric patients initially treated in an inpatient setting had poorer glycemic control and higher prescribed insulin dosing at baseline. After 3 years, there was no significant difference in HbA1c levels, but patients treated as inpatients continued to have higher prescribed insulin. These findings suggest that the severity of diabetes at initial presentation may affect long-term clinical outcomes in children with T2DM.

Inpatient management of epidermolysis bullosa: Consensus-based hands-on instructions for neonates and postneonates.

BACKGROUND: Epidermolysis bullosa (EB), characterized by skin fragility and blistering, often requires hospitalization. Training for inpatient management of EB is limited, with no unified recommendations available in North America. OBJECTIVE: To develop consensus-derived best practices for hands-on inpatient management of EB in both the neonatal and postneonatal period. METHODS: A modified Delphi method (expert-based input via 2 surveys and a final review) was implemented. Available guidelines from EB Clinical Research Consortium centers were analyzed to determine areas of focus and formulate statements to be voted on by EB Clinical Research Consortium members, experienced EB nurses, and select family members. Study participants evaluated statements using a Likert scale: statements with at least 70% agreement were accepted; statements with 30% or more disagreement were rejected. RESULTS: Ten areas of focus were identified. Delphi participants included 15 dermatologists, 8 nurses, and 6 nonhealth care caregivers. Consensus was established on 103/119 neonatal statements and 105/122 postneonatal statements; no statements were rejected. Most recommendations applied to both age groups. LIMITATIONS: Recommendations may require adjustment based on individual patient's clinical context. CONCLUSION: Using the Delphi method, a consensus-derived resource for hospital-based health care professionals who manage patients with EB has been developed to improve the quality of inpatient care.

Effect of co-cultivation of white and brown rot species on basidiome production, lignocelluloytic enzyme activity and dye decolourisation.

The aim of this work was studying the impact of co-cultivating two mushroom species: a white (Pleurotus albidus CLA 45) and a brown rot one (Laetiporus sulphureus BAFC 205) in substrates based on poplar or pine sawdust, on their lignocellulolytic enzyme production, yield values and basidiomes properties. Laetiporus sulphureus only developed basidiome primordia, but P. albidus monoculture and co-culture in pine sawdust achieved biological efficiencies of up to 50-55 %. Co-cultivation on diverse substrates rendered varied enzyme titers. Laccase and Manganese peroxidase titers were highest in pine co-culture and P. albidus poplar monoculture, respectively. Enzymatic extracts obtained from spent poplar substrate of dual cultures displayed potential for treating non-sterile textile-coloured effluents, achieving 35 % decolourisation after 120 h. The knowledge available on the effects of co-culture of white and brown rot fungi is still limited. This study represents an initial exploration of the interaction between them within intensive cultivation conditions.

An investigation of vascular anomalies centers' transition of care practices.

This study aims to examine transition of care (TOC) practices of multidisciplinary vascular anomalies centers (VACs). Thirty-seven of 71 VAC leaders to whom the survey was sent completed the questionnaire. TOC and transfer practices varied with only 16% of VACs having TOC programs. The most frequently cited barriers to developing a TOC program were lack of resources and difficulty finding expert adult providers.

Characterization of wound microbes in epidermolysis bullosa: A focus on Pseudomonas aeruginosa.

The most common bacteria isolated from wound cultures in patients recorded in the Epidermolysis Bullosa Clinical Characterization and Outcomes Database (EBCCOD) are Staphylococcus aureus and Pseudomonas aeruginosa. Given the prevalence of P. aeruginosa in this patient population and prior research implicating P. aeruginosa's potential role in carcinogenesis, we sought to further analyze patients with recorded wound cultures positive for Pseudomonas aeruginosa in the EBCCOD. We provide a descriptive analysis of this subset of patients and highlight potential avenues for future longitudinal studies that may have significant implications in our wound care management for patients with epidermolysis bullosa.

Cierre de mordida abierta con alineadores / Open bite closure with aligners

Los avances tecnológicos referidos a los alineadores nos demuestran que pueden ser utilizados tanto en tratamientos simples como en maloclusiones complejas, como en este caso de una mordida abierta. En este artículo presentamos un caso clínico de una paciente de 36 años, quien concurre a la consulta expresando no estar conforme con su estética y con el funcionamiento de su boca.

Protocolo biodigital aplicado al tratamiento de Ortodoncia con alineadores: caso clínico / Biodigital protocol applied to orthodontic treatment with aligners: a clinical case

En la actualidad, contamos con softwares de diseño que nos permiten planificaciones precisas y eficientes de nuestros tratamientos; por lo tanto, nuestros diagnósticos deben estar a la altura. En este artículo presentaremos un caso clínico que se ha desarrollado en el marco de la Diplomatura en Ortodoncia con Alineadores de la Sociedad Argentina Ortodoncia (SAO), donde trabajamos cada pieza dentaria de manera independiente, mediante la técnica de Ortodoncia con alineadores. Teniendo en cuenta que contamos con un stock óseo reducido, utilizamos como aliado un gran recurso: la tomografía computada de haz cónico (CBCT). Esta nos garantiza trabajar con seguridad en casos donde las limitaciones óseas nos dificultan los movimientos dentarios.

We currently find design softwares that allows us to plan our treatments accurately and efficiently; therefore, our diagnoses must be up to par. In this article we will present a clinical case that has been developed within the framework of the Subspeciality in Orthodontics with Aligners Argentine Society of Orthodontics (SAO), where we work, through the orthodontic technique with aligners, each tooth independently. Considering that we have a reduced bone stock, we use as an ally a great resource, the cone beam computed tomography (CBCT), which guarantees us to work safely in these cases where bone limitations hinder tooth movements.

Brain structural abnormalities of the associative striatum in adolescents and young adults at genetic high-risk of schizophrenia: Implications for illness endophenotypes.

OBJECTIVE: Dysfunction in cortico-striatal circuitry represents a core component of the pathophysiology in schizophrenia (SZ) but its potential as a candidate endophenotype of the illness is often confounded by neuroleptic medication. METHODS: Accordingly, 26 adolescent and young adult participants at genetic high-risk for schizophrenia, but who were asymptomatic and neuroleptic naïve, and 28 age-matched controls underwent 1.5T structural magnetic resonance imaging of the striatum, manually parcellated into limbic (LST), associative (AST), and sensorimotor (SMST) functional subregions. RESULTS: In relation to their age peers, participants at genetic high-risk for schizophrenia showed overall lower striatal gray matter volume with their most pronounced loss, bilaterally in the AST, but not the LST or SMST. Neuropsychological testing revealed reduced executive functioning for genetically at-risk participants, although the groups did not differ significantly in overall intelligence or oral reading. For controls but not for at-genetic high-risk participants, stronger executive functioning correlated with increased bilateral AST volume. CONCLUSIONS: Reduced bilateral AST volume in genetic high-risk adolescents and young adults, accompanied by heritable loss of higher cognitive brain-behavior relationships, might serve as a useful endophenotype of SZ.

Network analysis of mild traumatic brain injury, persistent neurobehavioral and psychiatric symptoms, and functional disability among recent-era United States veterans.

Recent-era U.S. veterans are clinically complex, with a high prevalence of co-occurring mild traumatic brain injury (mTBI), psychiatric conditions, and behavioral dysfunction. The current study examined the direct and indirect associations between mTBI and persistent neurobehavioral, psychiatric, and functional disability symptoms among recent-era U.S. veterans and service members (n = 648). We evaluated the postconcussive syndrome (PCS) potential causal model with two network analysis modeling approaches. Separate analyses were conducted for military mTBI and lifetime mTBI. An exploratory factor analysis was conducted to limit topological overlap in the network analysis. The most influential symptoms (i.e., the unique variables most strongly associated with the rest of the network) in the military mTBI network were behavioral disengagement, expected influence (EI) = 1.10; cognitive difficulties, EI = 1.08; agitation/irritability, EI = 1.05; and PTSD-related reexperiencing and avoidance symptoms, EI = 0.98. After accounting for other symptoms, mTBI was only minimally informative, EI = 0.34. Additionally, military mTBI did not moderate the association between symptoms or the overall connectivity of the network. The results for lifetime mTBI were consistent with those for military mTBI. The present analyses identified a variety of behavioral, cognitive, and emotional symptoms that play an important role in understanding comorbidity and daily functioning among recent-era U.S. veterans. Associations between cumulative mTBI that occurred in civilian or military settings were indirect and relatively small in magnitude. The current results add to a growing literature raising doubts about the PCS model.

Lumps and bumps: What not to miss.

The evaluation of pediatric patients with subcutaneous nodules remains a diagnostic challenge. Pediatric dermatologists are regularly confronted with patients who have a nonspecific nodule. Though most masses that require evaluation are ultimately benign, the possibility of a more aggressive process, including borderline or malignant neoplasms, underscores the pivotal role of the pediatric dermatologist in recognizing these lesions. The aim of this review is to provide an overview of lumps and bumps that are important to recognize to prevent delay in diagnosis or treatment of a serious underlying condition. Clinical clues that may lead the pediatric dermatologist to have a higher index of suspicion for more aggressive lesions are reviewed. Suggestions for evaluation and workup, as well as tips for the difficult to discern lesion, are proposed.

Impact of SARS-CoV2 on youth onset type 2 diabetes new diagnoses and severity.

INTRODUCTION: Initial reports show an increase in youth onset type 2 diabetes during the COVID-19 pandemic. We aim to expand on existing evidence by analyzing trends over a longer period. OBJECTIVES: Our study aims to describe change in the amount, severity, and demographics of youth onset type 2 diabetes diagnoses during the COVID-19 pandemic compared to the five years before. METHODS: We performed a retrospective cross-sectional review of youth (age ≤ 21) diagnosed with type 2 diabetes during the COVID-19 pandemic (1 May 2020-30 April 2021) and the five years before (1 May 2015-30 April 2020) at a tertiary care center. Children were identified by International Classification of Diseases codes. Charts were reviewed to confirm diagnosis. Chi-square, t tests, and Fisher's exact tests were used for analyses. RESULTS: In the prepandemic era annual diagnoses of type 2 diabetes ranged from 41-69 (mean = 54.2), whereas during the pandemic period 159 children were diagnosed, an increase of 293%. The increase resulted in a higher incidence rate ratio during the pandemic than before, 2.77 versus 1.07 (p = .006). New diagnoses increased most, by 490%, in Non-Hispanic Black patients. The average HbA1c at presentation was higher during the pandemic (9.5% ± 2.6) (79.9 mmol/mol ± 28.2) than before (8.7%±2.1) (72.1 mmol/mol ± 23.1) (p = .003). Of those diagnosed during the pandemic, 59% were tested for COVID-19 and three tested positive. CONCLUSIONS: New diagnoses of type 2 diabetes increased during the pandemic, most notably in Non-Hispanic Black youth. There was not a significant correlation found with clinical or biochemical COVID-19 infection in those tested.

Analysis of existing "how to" manuals for the care of inpatients with epidermolysis bullosa.

"How to" skin care manuals assist health care professionals caring for hospitalized patients with epidermolysis bullosa and other disorders. Manuals created by Epidermolysis Bullosa Clinical Research Consortium sites were collected and analyzed. Analysis of manuals revealed variable content. Creating a unified, optimized "how" to manual would benefit providers and patients.

Lignocellulosic residues as supports for enzyme immobilization, and biocatalysts with potential applications.

Growing demand for agricultural production means a higher quantity of residues produced. The reuse and recycling of agro-industrial wastes reduce worldwide greenhouse emissions. New opportunities are derived from this kind of residuals in the biotechnological field generating valuable products in growing sectors such as transportation, bioenergy, food, and feedstock. The use of natural macromolecules towards biocatalysts offers numerous advantages over free enzymes and friendliness with the environment. Enzyme immobilization improves enzyme properties (stability and reusability), and three types of supports are discussed: inorganic, organic, and hybrid. Several examples of agro-industrial wastes such as coconut wastes, rice husks, corn residues and brewers spent grains (BSG), their properties and potential as supports for enzyme immobilization are described in this work. Before the immobilization, biological and non-biological pretreatments could be performed to enhance the waste potential as a carrier. Additionally, immobilization methods such as covalent binding, adsorption, cross-linking and entrapment are compared to provide high efficiency. Enzymes and biocatalysts for industrial applications offer advantages over traditional chemical processes with respect to sustainability and process efficiency in food, energy, and bioremediation fields. The wastes reviewed in this work demonstrated a high affinity for lipases and laccases and might be used in biodiesel production and textile wastewater treatment, among other applications.

Review of Speech Outcomes in Cochlear Implant Recipients at a Nascent Cochlear Implant Program.

INTRODUCTION: The use of cochlear implantation to rehabilitate moderate to profound sensorineural hearing loss has become more widespread; however, the adult utilization rate of cochlear implant candidates is still very less. The study aims to examine the percentage of adult patients in a heterogeneous group of cochlear implant recipients at a nascent cochlear implant program who demonstrate improvements in speech outcomes. METHODS: Speech outcome scores were assessed preoperatively and postoperatively at three, six, and 12-month intervals using consonant-nucleus-consonant (CNC) words and AzBio sentences in quiet. Mean speech outcome scores at each time point and binomial distribution tables with 95% CI were used to assess individual improvement in speech understanding. RESULTS: 45 patients underwent a total of 49 cochlear implantation surgeries. The mean age at surgery was 62 years. The mean preoperative CNC score in the ear to be implanted was 18%±18, while the mean postoperative CNC score at three, six, and 12 months was 35%±21, 44%±23, and 45%±25, respectively. The mean preoperative AzBio score in the ear to be implanted was 22%±26 while the mean postoperative AzBio score at three, six, and 12 months was 50%±29, 56%±27, and 63%±26, respectively. Of the implantations, 74% (32 of 43) and 69% (22 of 32) showed significant improvement at six months or one year using AzBio and CNC binomial distribution tables, respectively. CONCLUSIONS: Findings demonstrate significant improvements in speech perception following cochlear implantation for patients not benefiting from hearing aid aural rehabilitation. The study provides realistic expectations for new and emerging programs hoping to demonstrate cochlear implant utility for improving patients' speech outcomes.