Development and Evaluation of Aquatic and Terrestrial Food Web Bioaccumulation Models for Per- and Polyfluoroalkyl Substances.

There is a need for reliable models to predict the food web bioaccumulation and assess ecological and human health risks of per- and polyfluoroalkyl substances (PFAS). This present study presents (i) the development of novel mechanistic aquatic and terrestrial food web bioaccumulation models for PFAS and (ii) an evaluation of model performance using available laboratory and field data. Model predictions of laboratory-measured bioconcentration factors and field-based bioaccumulation factors of PFAS in fish were in good agreement with observed data as measured by the mean model bias (MB), representing systematic over- or under-estimation and the standard deviation of the MB, representing general uncertainty.

Sensorimotor Features and Daily Living Skills in Autistic Children With and Without ADHD.

Attention-deficit/hyperactivity disorder (ADHD) commonly co-occurs in autistic children. However, additional research is needed to explore the differences in motor skills and sensory features in autistic children with and without ADHD, as well as the impacts of these factors on daily living skills (DLS). This observational study sought to fill this gap with 67 autistic children (6.

A case of STING-associated vasculopathy with onset in infancy with novel STING1 variant.

STING-associated vasculopathy with onset in infancy (SAVI) is a rare, monogenic interferonopathy caused by gain-of-function variants in STING1 (TMEM173) characterized by systemic inflammation, cutaneous vasculopathy, and interstitial lung disease. We report a case of SAVI attributed to a novel STING1 p.R284T variant who demonstrated characteristic cutaneous features including telangiectasias, livedo and acrocyanotic changes on face and extremities, as well as saddle nose deformity, failure to thrive, inflammatory arthritis and notable lack of pulmonary disease or autoantibody positivity.

Multicenter Study of Long-Term Outcomes and Quality of Life in PHACE Syndrome after Age 10.

Objective: To characterize long-term outcomes of PHACE syndrome.

Study Design: Multicenter study with cross-sectional interviews and chart review of individuals with definite PHACE syndrome ≥10 years of age. Data from charts were collected across multiple PHACE-related topics.

Economic hardship and adolescent behavioral outcomes: Within- and between-family associations.

Understanding how youth perceive household economic hardship and how it relates to their behavior is vital given associations between hardship and behavioral development. Yet, most studies ignore youth's own perceptions of economic hardship, instead relying solely on caregiver reports. Moreover, the literature has tended to treat economic hardship as a stable force over time, rather than a volatile one that varies month-to-month.

Risk factors and outcomes of melanoma in children and adolescents: A retrospective multicenter study.

Background: Pediatric melanoma presents with distinct clinical features compared to adult disease.

Objective: Characterize risk factors and negative outcomes in pediatric melanoma.

Methods: Multicenter retrospective study of patients under 20 years diagnosed with melanoma between January 1, 1995 and June 30, 2015 from 11 academic medical centers.

Next generation sequencing aids diagnosis and management in a case of encephalocraniocutaneous lipomatosis.

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous disorder caused by somatic FGFR1 and KRAS variants. It shares significant phenotypic overlap with several closely related disorders caused by mutations in the RAS-MAPK pathway (mosaic RASopathies). We report a diagnostically challenging case of ECCL in which next-generation sequencing of affected tissue identified a pathologic FGFR1 p.

Positive parenting moderates associations between childhood stress and corticolimbic structure.

Childhood stress has a deleterious impact on youth behavior and brain development. Resilience factors such as positive parenting (e.g.

Impact of Socioeconomic Status on Amygdala and Hippocampus Subdivisions in Children and Adolescents.

Socioeconomic status (SES) in childhood can impact behavioral and brain development. Past work has consistently focused on the amygdala and hippocampus, two brain areas critical for emotion and behavioral responding. While there are SES differences in amygdala and hippocampal volumes, there are many unanswered questions in this domain connected to neurobiological specificity, and for whom these effects may be more pronounced.

Botulinum toxin for refractory Raynaud's phenomenon: A "how to" guide for pediatric patients.

Raynaud's phenomenon describes symptoms caused by digital vascular spasm and is classically induced by cold exposure. Severe cases can result in ulceration, necrosis, and digital autoamputation. When standard and adjunctive medical therapies fail or are contraindicated, botulinum toxin A (BTX-A) is an effective treatment option that can be added to existing regimens and should be considered before utilizing rescue therapies associated with higher risk and often higher cost.

Perioperative Outcomes of Immersive Virtual Reality as Adjunct Anesthesia in Primary Total Hip and Knee Arthroplasty.

Background: Immersive virtual reality (IVR) is utilized as an adjunct to anesthesia to distract patients from their intraoperative environment, thereby potentially reducing sedative and narcotic medication usage. This study evaluated intraoperative and acute postoperative results of patients undergoing primary total hip (THA) and total knee arthroplasty (TKA) with and without IVR.

Methods: Utilizing IVR as an adjunct to spinal anesthesia, 18 primary THAs (n = 8) and TKAs (n = 10) were performed.

From Genotype to Phenotype-A Review of Kabuki Syndrome.

Kabuki syndrome (KS) is a rare neuro-developmental disorder caused by variants in genes of histone modification, including and . This review assesses our current understanding of KS, which was originally named Niikawa-Kuroki syndrome, and aims to guide surveillance and medical care of affected individuals as well as identify gaps in knowledge and unmet patient needs. Ovid MEDLINE and EMBASE databases were searched from 1981 to 2021 to identify reports related to genotype and systems-based phenotype characterization of KS.

Dynamic evolution of a scalp congenital melanocytic nevus with poliosis and cutis verticis gyrata.

Cutis verticis gyrata (CVG), characterized by cerebriform overgrowth of the scalp, is rarely observed in congenital melanocytic nevi (CMN). We describe a 13-year-old male with autism and a large CMN of the scalp with numerous satellite nevi whose scalp nevus exhibited evolution with poliosis and CVG. Given the potential association of CVG (independent of CMN) with seizures, neuropsychiatric, and ophthalmologic disorders, and nevus-associated CVG (cerebriform intradermal nevus) with melanoma, multidisciplinary evaluation of CMN patients with CVG is important to guide management and treatment.

Paraneoplastic pemphigus associated with Castleman disease: A multicenter case series.

Introduction: Paraneoplastic pemphigus (PNP) is a rare, often fatal, autoimmune blistering disease of the skin and mucous membranes. In children, PNP is frequently associated with Castleman disease (CD). This series describes five cases of PNP associated with CD.

Pediatric Lichen Myxedematosus: A Diagnostic and Management Challenge.

Localized lichen myxedematosus (LM) is a rare, idiopathic mucinosis characterized by dermal mucin deposition and variable fibroblast proliferation. Nodular lichen myxedematosus, a clinicopathologic subtype of localized LM, is exceedingly rare in pediatric patients with only three prior cases reported. Understanding of LM in pediatric patients is limited by the rarity of the disease, and diagnosis is complicated by overlapping clinical and histopathologic features.

Access to pediatric dermatology.

Purpose Of Review: To identify factors that impact accessibility to pediatric dermatology and review healthcare delivery models that improve access and address these barriers.

Recent Findings: Up to one-third of pediatric primary care visits include a skin-related problem, yet pediatric dermatology subspecialist services are highly inaccessible. Workforce shortages and geographic, sociocultural, and economic barriers perpetuate inaccessibility.

A food web bioaccumulation model for the accumulation of per- and polyfluoroalkyl substances (PFAS) in fish: how important is renal elimination?

Per- and polyfluoroalkyl substances (PFAS) are a large class of highly fluorinated anthropogenic chemicals. Some PFAS bioaccumulate in aquatic food webs, thereby posing risks for seafood consumers. Existing models for persistent organic pollutants (POPs) perform poorly for ionizable PFAS.