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Coloboma, congenital heart disease, ichthyosiform dermatosis, intellectual disability, conductive hearing loss, and epilepsy (CHIME) syndrome is a rare autosomal recessive neuroectodermal disorder caused by PIGL gene mutations. There is emerging literature to support the use of interleukin-17 (IL-17) antagonists in the treatment of certain ichthyosiform dermatoses. Here, we report a case of severe ichthyosiform dermatosis in a child with CHIME syndrome who was recalcitrant to multiple topical medications and dupilumab. This is the first reported case of successful treatment of congenital ichthyosiform dermatosis in a CHIME syndrome patient with ixekizumab, an IL-17A antagonist., (© 2024 Wiley Periodicals LLC.)

Recessive dystrophic epidermolysis bullosa (RDEB) is a rare inherited skin disease characterized by defects in type VII collagen leading to a range of fibrotic pathologies resulting from skin fragility, aberrant wound healing, and altered dermal fibroblast physiology. Using a novel in vitro model of fibrosis based on endogenously produced extracellular matrix, we screened an FDA-approved compound library and identified antivirals as a class of drug not previously associated with anti-fibrotic action. Preclinical validation of our lead hit, daclatasvir, in a mouse model of RDEB demonstrated significant improvement in fibrosis as well as overall quality of life with increased survival, weight gain and activity, and a decrease in pruritus-induced hair loss. Immunohistochemical assessment of daclatasvir-treated RDEB mouse skin showed a reduction in fibrotic markers, which was supported by in vitro data demonstrating TGFβ pathway targeting and a reduction of total collagen retained in the extracellular matrix. Our data support the clinical development of antivirals for the treatment of patients with RDEB and potentially other fibrotic diseases., (© 2024. The Author(s).)

Leprosy (Hansen's disease) is caused by infection with bacilli of the Mycobacterium leprae complex. It is considered an exotic and rare diagnosis in Missouri. Past leprosy patients diagnosed locally have typically acquired it in areas of the world where leprosy is endemic. However, a recent case in a native Missourian that appears to be locally acquired suggests that leprosy may now be endemic in Missouri, possibly due to the expanded range of its zoonotic vector, the nine-banded armadillo. Health care providers in Missouri should be aware of how leprosy manifests and suspected cases referred to centers such as ours for evaluation and early institution of appropriate treatment., (Copyright 2023 by the Missouri State Medical Association.)

A number of chronic skin diseases, such as vitiligo and alopecia areata, are historically resistant to or respond poorly to treatment. Additionally, disorders such as atopic dermatitis and psoriasis have subtypes that are inadequately treated by current medications. Lastly, in the field of dermatology there are a number of conditions, some genetic (such as Darier's disease and Hailey-Hailey disease) and others caused by aberrant inflammatory responses (macrophage-driven conditions such as sarcoidosis and autoimmune conditions such as localized scleroderma) where effective treatments have been limited to date. A new class of anti-inflammatory medications that inhibit the Janus Kinase-Signal transducer and activator of transcription pathway (JAK-STAT) show great promise in providing new and effective treatment of these formerly recalcitrant conditions. This brief review will cover inhibitors of the JAK-STAT pathway (JAK inhibitors) currently approved for use in treating dermatologic diseases including several very recently approved medications. It will also touch on additional conditions under study or where early reports of efficacy are promising., (Copyright 2023 by the Missouri State Medical Association.)

The World Health Organization (WHO) recommends multidrug therapy (MDT) for the treatment of paucibacillary and multibacillary forms of leprosy, also known as Hansen's disease (HD). MDT combinations of dapsone, rifampin, and clofazimine have reduced the prevalence of the disease but are not without adverse effects impacting regimen adherence. Hence, an urgent need exists to consider alternative MDT regimens with an improved safety profile that promotes treatment adherence. Herein, we described a case series of 10 patients with HD (nine patients with multibacillary leprosy and one with pure neural leprosy) treated with monthly rifampin, moxifloxacin, and minocycline (RMM). The United States National Hansen's Disease Program (NHDP) diagnosed and treated patients across US institutions. All patients received a regimen of 12-24 months of RMM. We reviewed the clinical outcomes, adherence, rate of completion, and adverse events of patients treated with monthly RMM from January 2019 to August 2022. Nine patients had multibacillary leprosy, with some having type-2 reactions. One patient had pure neural leprosy with a reversal reaction. In this case series, we identified that all patients completed the RMM regimen without treatment interruptions. None of the patients experienced any skin hyperpigmentation or any significant side effects. All patients tolerated the monthly RMM regimen with rapid improvement of skin lesions and without logistic hurdles. Based on previous clinical evidence and the results of this case series, the NHDP and other programs should consider the RMM regimen as first-line therapy., Competing Interests: The authors declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Dr. Henao-Martínez reported receiving a K12-clinical trial award as a co-principal investigator for the Expanded Access IND Program (EAP) to provide the Yellow Fever vaccine (Stamaril) to persons in the United States outside the submitted work. The Editor-in-Chief of Therapeutic Advances in Infectious Disease is an author of this paper, therefore, the peer review process was managed by alternative members of the Board and the submitting Editor had no involvement in the decision-making process. No other disclosures were reported., (© The Author(s), 2022.)

In this study, a protocol was developed to identify reduction-oxidation (redox) transition zones in an effort to exploit natural source zone depletion processes. A sediment core with a total length of 18-m was collected from a site with historical contamination that includes chlorinated benzenes where the redox condition was preserved. In the four redox transition zones investigated, reactive iron coatings are characterized with a suite of analyses under anaerobic conditions. To distinguish surface coating mineralogy, X-ray diffraction, X-ray fluorescence, and field-emission scanning electron microscopy with an energy dispersive X-ray analyzer were applied along with a six-step sequential extraction process. The cycling of Fe and S, as an important contribution and indicator of ongoing natural attenuation processes for constituents of concern (COC), was delineated by using data from multiple and complementary analyses for isolating and identifying iron phases. Along with groundwater chemistry, contaminant concentrations, and microbial genera, attenuation of COCs is expected to be active and sustainable in redox transition zones, where there is an abundance of reactive iron mineral coatings cycling through biogeochemical reactions. Reactions in other redox transition zones may be limited where iron mineral coatings are not dominant., (Copyright © 2021 Elsevier B.V. All rights reserved.)

This study focused on comparing iron mineral coatings found in contaminated sediments from a cryogenic (Cryo Core) core versus an Anaerobic Core (collected under oxygen-free and ambient conditions). After thawing the Cryo Core in an oxygen-free glovebox, a suite of analyses was applied on sediments from both cores: pH, redox potential, X-ray fluorescence (XRF), X-ray diffraction (XRD), and field-emission scanning electron microscopy (FESEM) with energy dispersive X-ray analyzer (EDX). Among the iron minerals identified, crystalline pyrite was found throughout the Cyro Core sediment samples, which is in contrast to that observed for the Anaerobic Core. Moreover, mackinawite and greigite that were ubiquitous in the Anaerobic Core were not observed in Cryo Core samples. To better understand why the metastable minerals were not present, a freeze/thaw process was simulated on Anaerobic Core samples using a liquid‑nitrogen quench with surface coatings characterized by FESEM/EDX. In these quenched samples, mackinawite was no longer observed, and in its place was pyrite. In addition, both greigite and pyrite were found to be unique morphologically after quenching. Dissolution and re-precipitation of iron sulfide coatings during the freeze/thaw process appears to affect the geochemistry of the pore water through two main mechanisms of freeze-concentration and freezing potential., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Children are known to disproportionately bear the health impacts of climate change, particularly children living in impoverished areas. Owing to their developing physiology and immature metabolism, distinct exposure behaviors, and reliance on adults for care and protection, children are uniquely susceptible to the adverse effects of our warming planet. Herein, we summarize the known impacts of climate change on pediatric skin health, including its effects on atopic dermatitis, vector-borne and other infectious diseases, nutritional deficiencies, and psychodermatoses., (© 2020 Published by Elsevier Inc. on behalf of Women's Dermatologic Society.)

The hereditary nature of some forms of cancer was recognized long ago. Over time, recognition of associated findings led to the delineation of numerous hereditary cancer syndromes. Many of these syndromes also have cutaneous manifestations, the recognition of which can lead to their early identification. Recognition of these syndromes allows vigilant surveillance and preemptive treatment, which can dramatically impact the risks of morbidity and mortality for affected patients. The rise of rapid and accurate genetic testing now allows the early identification of asymptomatic at risk family members so that monitoring can be initiated as early as possible. The dermatologist plays a critical role in early identification of these syndromes and, in many cases, their treatment. This review summarizes many known hereditary cancer syndromes with cutaneous findings, their etiology, identification, evaluation, and management. Importantly, this is an ever evolving topic and new findings and syndromes will continue to be recognized. The dermatologist must be always alert to ensure they are detected., (Copyright © 2020 Elsevier Inc. All rights reserved.)

In this article, we describe three life-changing patient cases demonstrating high-quality and timely care they received in their communities, thanks to the Show-Me ECHO project. Early autism diagnosis, a potentially deadly tumor manifesting as a benign-looking rash, a recalcitrant case of hepatitis C: rural and underserved Missourians now have access to state-of-the-art care through their local providers receiving interdisciplinary telementoring on evidence based practices., (Copyright 2020 by the Missouri State Medical Association.)

Cutaneous adverse drug reactions (ADRs) are commonly seen in the pediatric population in both inpatient and outpatient settings and are important to identify, evaluate, and appropriately manage. Early recognition and proper classification of a cutaneous drug reaction allows the clinician the ability to narrow in on a culprit drug and determine whether the medication is safe to continue. This review discusses the clinical presentation, categorization, and management of cutaneous ADRs in the pediatric population. [Pediatr Ann. 2020;49(3):e132-e139.]., (Copyright 2020, SLACK Incorporated.)

Hardwood biochar (pyrolyzed at 700 °C), a potential candidate for Hg removal, has been proposed for use as reactive capping mats along groundwater discharge zones or riverbanks to control release of Hg from contaminated riverbank sediments. Frequent flooding and drainage in fluvial settings can influence the effectiveness of remediation systems in contaminated riverbank sediments and floodplain soils. This study evaluated the effectiveness of Hg removal using hardwood biochar under hydrogeochemical conditions representative of those present within a reactive capping mat installed in a fluvial setting. Two sets of treatment columns, containing 50% v.v biochar and quartz sand, were subjected to 100 weekly wetting/drying cycles that included dry air, water-saturated air, and drainage using leachate derived from two source columns as input solutions: 1. Passing simulated acid rain water through floodplain soil, 2. Passing river water through riverbank sediment. In both treatment columns, >80% of the Hg was retained on the biochar without promoting Hg methylation and the release of other unintended dissolved constituents (including N, P, DOC). Results from solidphase extraction analyses suggest that Hg accumulated near the air/biochar-sand interface (0-2 cm) in the treatment columns at low loadings but was present at greater depths at higher loadings. Results of micro X-ray fluorescence (μ-XRF) mapping and micro X-ray absorption near edge structure (μ-XANES) for the biochar collected at depths 0-2 cm in treatment columns suggest retention of Hg-bearing particles derived from riverbank sediment and floodplain soil within the pore structure of the biochar. Sulfur K-edge XANES analysis of the unused biochar and the biochar after treatment suggest formation of Hg complexes on the biochar surface. These results indicate that hardwood biochar is potentially an effective media for application in reactive mats for controlling Hg discharging from contaminated riverbank sediments., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Context: Human cross-sectional and animal studies have shown an association of the chemical bisphenol A (BPA) with insulin resistance, type 2 diabetes, and other metabolic diseases, but no human experimental study has investigated whether BPA alters insulin/C-peptide secretion., Design: Men and postmenopausal women (without diabetes) were orally administered either the vehicle or a BPA dose of 50 µg/kg body weight, which has been predicted by US regulators (Food and Drug Administration, Environmental Protection Agency) to be the maximum, safe daily oral BPA dose over the lifetime. Insulin response was assessed in two cross-over experiments using an oral glucose tolerance test (OGTT; experiment 1) and a hyperglycemic (HG) clamp (experiment 2). Main outcomes were the percentage change of BPA session measures relative to those of the control session., Results: Serum bioactive BPA after experimental exposure was at levels detected in human biomonitoring studies. In the OGTT, a strong positive correlation was found between hemoglobin A1c(HbA1c) and the percentage change in the insulinogenic index (Spearman = 0.92), an indicator of early-phase insulin response, and the equivalent C-peptide index (Pearson = 0.97). In the HG clamp study, focusing on the later-phase insulin response to a stable level of glucose, several measures of insulin and C-peptide appeared suppressed during the BPA session relative to the control session; the change in insulin maximum concentration (Cmax) was negatively correlated with HbA1c and the Cmax of bioactive serum BPA., Conclusions: This exploratory study suggests that BPA exposure to a dose considered safe by US regulators may alter glucose-stimulated insulin response in humans.

This is a first literature report of a case of uveitis along with severe systemic symptoms following verruca vulgaris treatment with intralesional Candida antigen. We believe the Candida injection was causative.

Epidermodysplasia verruciformis (EV) is an uncommon inherited skin condition with increased vulnerability to widespread infection by certain human papillomavirus types, resulting in extensive verruca plana-like papules coalescing to large confluent plaques. Since the AIDS epidemic starting in the 1980s, an acquired type of EV has been described in patients infected with human immunodeficiency virus. The histopathologic features of EV consist of papillated epidermal hyperplasia with hypergranulosis and a distinct bluish-gray color in the large human papillomavirus-infected keratinocytes in the stratum granulosum. The authors present a case of HIV-associated EV with a unique histopathologic finding of multiple cornoid lamella-like structures. To the authors' knowledge, this finding has not been previously described in the literature.

Importance: Bites from the brown recluse spider (BRS) can cause extreme pain. We propose cytokine release as a cause of the discomfort and a central mechanism through glial cell upregulation to explain measured pain levels and time course., Observations: Twenty-three BRS bites were scored at a probable or documented level clinically, and an enzyme-linked immunosorbent assay was used to confirm the presence of BRS venom. The mean (SD) pain level in these cases 24 hours after the spider bite was severe: 6.74 (2.75) on a scale of 0 to 10. Narcotics may be needed to provide relief in some cases. The difference in pain level by anatomic region was not significant. Escalation observed in 22 of 23 cases, increasing from low/none to extreme within 24 hours, is consistent with a cytokine pain pattern, in which pain increases concomitantly with a temporal increase of inflammatory cytokines., Conclusions and Relevance: These findings in BRS bites support the hypothesis of cytokine release in inflammatory pain. A larger series is needed to confirm the findings reported here. The extreme pain from many BRS bites motivates us to find better prevention and treatment techniques.

We report on the nature and timing of services provided to children with an intellectual disability (ID) identified by a new comprehensive assessment and care planning tool used to evaluate children's needs for Medicaid Personal Care Services (PCS) in Texas. The new assessment procedure resulted from a legal settlement with the advocacy community. Participants in the study were 1,109 children ages 4-20 with an intellectual disability diagnosis who were assessed between January and April of 2010. The need for assistance is higher on Saturday and Sunday, when school services are not available. We report differences in service patterns for children who vary in ID severity. Finally, we consider the implications of our results for policies and programs that serve families with children with an ID.

Background: Medicaid Personal Care Services (PCS) help families meet children's needs for assistance with functional tasks. However, PCS may have other effects on a child's well-being, but research has not yet established the existence of such effects., Objectives: To investigate the relationship between the number of PCS hours a child receives with subsequent visits to physicians for evaluation and management (E&M) services., Methods: Assessment data for 2058 CSHCN receiving PCS were collected in 2008 and 2009. Assessment data were matched with Medicaid claims data for the period of 1 year after the assessment. Zero-inflated negative binomial and generalized linear multivariate regression models were used in the analyses. These models included patient demographics, health status, household resources, and use of other medical services., Results: For every 10 additional PCS hours authorized for a child, the odds of having an E&M physician visit in the next year were reduced by 25%. However, the number of PCS hours did not have a significant effect on the number of visits by those children who did have a subsequent E&M visit. A variety of demographic and health status measures also affect physician use., Conclusions: Medicaid PCS for CSHCN may be associated with reduced physician usage because of benefits realized by continuity of care, the early identification of potential health threats, or family and patient education. PCS services may contribute to a child's well-being by providing continuous relationships with the care team that promote good chronic disease management, education, and support for the family., (Copyright © 2013 Elsevier Inc. All rights reserved.)

The Canadian dairy sector is a major industry with about 1 million cows. This industry emits about 20% of the total greenhouse gas (GHG) emissions from the main livestock sectors (beef, dairy, swine, and poultry). In 2006, the Canadian dairy herd produced about 7.7 Mt of raw milk, resulting in about 4.4 Mt of dairy products (notably 64% fluid milk and 12% cheese). An integrated cradle-to-gate model (field to processing plant) has been developed to estimate the carbon footprint (CF) of 11 Canadian dairy products. The on-farm part of the model is the Unified Livestock Industry and Crop Emissions Estimation System (ULICEES). It considers all GHG emissions associated with livestock production but, for this study, it was run for the dairy sector specifically. Off-farm GHG emissions were estimated using the Canadian Food Carbon Footprint calculator, (cafoo)(2)-milk. It considers GHG emissions from the farm gate to the exit gate of the processing plants. The CF of the raw milk has been found lower in western provinces [0.93 kg of CO2 equivalents (CO2e)/L of milk] than in eastern provinces (1.12 kg of CO2e/L of milk) because of differences in climate conditions and dairy herd management. Most of the CF estimates of dairy products ranged between 1 and 3 kg of CO2e/kg of product. Three products were, however, significantly higher: cheese (5.3 kg of CO2e/kg), butter (7.3 kg of CO2e/kg), and milk powder (10.1 kg of CO2e/kg). The CF results depend on the milk volume needed, the co-product allocation process (based on milk solids content), and the amount of energy used to manufacture each product. The GHG emissions per kilogram of protein ranged from 13 to 40 kg of CO2e. Two products had higher values: cream and sour cream, at 83 and 78 kg of CO2e/kg, respectively. Finally, the highest CF value was for butter, at about 730 kg of CO2e/kg. This extremely high value is due to the fact that the intensity indicator per kilogram of product is high and that butter is almost exclusively fat. Protein content is often used to compare the CF of products; however, this study demonstrates that the use of a common food component is not suitable as a comparison unit in some cases. Functionality has to be considered too, but it might be insufficient for food product labeling because different reporting units (adapted to a specific food product) will be used, and the resulting confusion could lead consumers to lose confidence in such labeling. Therefore, simple units might not be ideal and a more comprehensive approach will likely have to be developed., (Copyright © 2013 American Dairy Science Association. Published by Elsevier Inc. All rights reserved.)

New technologies are accelerating the pace at which genetic defects leading to inherited skin disease are elucidated. Translation of these genetic discoveries into new therapies for patients with inherited skin diseases has not been as rapid but the pace is now accelerating. This article summarizes recent findings in genetic skin diseases, the scope of advances being made, the role of new DNA analysis technologies in these discoveries, as well as highlighting some examples of how an understanding of the genetic cause of inherited skin diseases can lead to therapeutic interventions for patients., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Many organ systems undergo significant and rapid changes during the transition from an intrauterine to an extrauterine environment, especially those which serve as interfaces between the infant and the external environment. Historically the skin care methods employed during and after this period of rapid physiologic change have been derived from individual anecdotal experience or cultural tradition, rather than evidence-based or pathomechanistically derived data. While research in this area has historically been limited, it is increasing in scope and volume, and recent work has shed light on the changes experienced by the cutaneous organ during this period of transition. This increased understanding has driven new recommendations in skin care protocols for newborn infants and neonates., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Mendelian disorders of cornification (ichthyosis; MeDOC) often present in the neonatal period with little warning to providers or parents. This report reviews the majority of ichthyoses with congenital findings. The neonatal presentation of many MeDOC often differs from the later phenotype because of the changes in the skin that occur with transition from an intrauterine to extrauterine environment. While differentiation of ichthyosis subtypes in the neonatal period is difficult, there are certain phenotypic groups within which these neonates fall, recognition of which can guide initial work up and treatment. For this report, these are categorized as: exuberant vernix; collodion baby/harlequin ichthyosis (HI); ichthyosiform erythroderma; blistering; and normal skin/xerosis phenotypes., (© 2013 Wiley Periodicals, Inc.)

To assess tradeoffs between environmental sustainability and changes in food production on agricultural land in Canada the Unified Livestock Industry and Crop Emissions Estimation System (ULICEES) was developed. It incorporates four livestock specific GHG assessments in a single model. To demonstrate the application of ULICEES, 10% of beef cattle protein production was assumed to be displaced with an equivalent amount of pork protein. Without accounting for the loss of soil carbon, this 10% shift reduced GHG emissions by 2.5 TgCO₂e y(-1). The payback period was defined as the number of years required for a GHG reduction to equal soil carbon lost from the associated land use shift. A payback period that is shorter than 40 years represents a net long term decrease in GHG emissions. Displacing beef cattle with hogs resulted in a surplus area of forage. When this residual land was left in ungrazed perennial forage, the payback periods were less than 4 years and when it was reseeded to annual crops, they were equal to or less than 40 years. They were generally greater than 40 years when this land was used to raise cattle. Agricultural GHG mitigation policies will inevitably involve a trade-off between production, land use and GHG emission reduction. ULICEES is a model that can objectively assess these trade-offs for Canadian agriculture.

A significant seasonal correlation was recently shown for brown recluse spider activity. Vetter (2011) observed brown recluse spiders were submitted by the general public predominantly during April-October. For patients with suspected brown recluse spider bites (BRSB), we have observed the same seasonality. Among 45 cases with features consistent of a BRSB, 43 (95.6%) occurred during April-October. Both the Vetter study and our study serve to demonstrate seasonal activity for brown recluse spiders., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Background: To test the validity and reliability of scales intended to measure activity limitations faced by children with chronic illnesses living in the community. The scales were based on information provided by caregivers to service program personnel almost exclusively trained as social workers. The items used to measure activity limitations were interRAI items supplemented so that they were more applicable to activity limitations in children with chronic illnesses. In addition, these analyses may shed light on the possibility of gathering functional information that can span the life course as well as spanning different care settings., Methods: Analyses included testing the internal consistency, predictive, concurrent, discriminant and construct validity of two activity limitation scales. The scales were developed using assessment data gathered in the United States of America (USA) from over 2,700 assessments of children aged 4 to 20 receiving Medicaid Early and Periodic Screening, Diagnostic and Treatment (EPSDT) services, specifically Personal Care Services to assist children in overcoming activity limitations. The Medicaid program in the USA pays for health care services provided to children in low-income households. Data were collected in a single, large state in the southwestern USA in late 2008 and early 2009. A similar sample of children was assessed in 2010, and the analyses were replicated using this sample., Results: The two scales exhibited excellent internal consistency. Evidence on the concurrent, predictive, discriminant, and construct validity of the proposed scales was strong. Quite importantly, scale scores were not correlated with (confounded with) a child's developmental stage or age. The results for these scales and items were consistent across the two independent samples., Conclusions: Unpaid caregivers, usually parents, can provide assessors lacking either medical or nursing training with reliable and valid information on the activity limitations of children. One can summarize these data in scales that are both internally consistent and valid. Researchers and clinicians can use supplemented interRAI items to provide guidance for professionals and programs serving children, as well as older persons. This research emphasizes the importance of developing medical information systems that allow one to integrate information not only across care settings but also across an individual's life course.

Objective: To investigate the relationship between Medicaid Personal Care Services (PCS) and caregivers' reports of activity (activities of daily living [ADL]) limitations for children with chronic health problems., Data Sources/study Setting: Primary data collected in 2008 and 2009. A state Medicaid program was the setting. The focus was children receiving Medicaid PCS., Data Collection: Medicaid case managers assessed children to determine their need for PCS, using information provided by the child or informal caregivers. Two thousand seven hundred assessments were provided to researchers directly from case managers., Principal Findings: Medical conditions and impairments explained 58 percent of the variance in the child's activity limitations. Activity limitations and problem behaviors explained 28 percent of the variance in PCS hours authorized. Which case manager completed the assessment also played a substantial role in determining hours of care., Conclusions: Caregivers' reports of the severity of a child's activity limitations effectively summarize the effects of conditions and impairments on the child's ADL performance and have a significant impact on the level of services provided. Assessors often respond differently to children's characteristics and circumstances as they move from assessment to decisions concerning care provision. Our results imply that the provision of appropriate services may be enhanced when both case managers and caregivers play an active role in decisions concerning care provision., (© Health Research and Educational Trust.)

Objectives: This research investigated the relationship between a child's reported intellectual disability (ID) level and caregivers' reports of the child's health status to predict Medicaid Personal Care Services (PCS) hours authorized for that child. We also investigated how activity limitations in the home varied with the level of ID., Design: The sample included 1,108 community-residing children with a reported level of ID in the Texas Medicaid system and who were assessed for the PCS program. All data were collected with the Personal Care Assessment Form (PCAF), an instrument developed by the authors for evaluating children's PCS needs. Case managers completed the PCAF in the child's home with the child and primary caregivers present. Structural equation modeling (SEM) was used to test a model reflecting the role of ID and other characteristics of the child in determining the number of PCS hours authorized. Additional analyses revealed the degree to which variation among the case managers affected the number of hours authorized., Results: ID level and other individual characteristics had a significant effect on reports of a child's activity limitations (R2 = .67), which in turn affected the hours of PCS authorized (R2 = .27). We found no significant direct relationship between ID level and PCS hours: ID level had an indirect relationship on PCS hours through activity limitations. When the variance in hours authorized was decomposed, individual characteristics accounted for 20% of the variance and case managers accounted for 14%., Conclusions: Assessments of caregiver and child strengths and limitations in the home are critical in the allocation of Medicaid home-based services, above and beyond the information conveyed by demographic and diagnostic data. Implications for home-based assessments of functional limitations and needs for family caregivers and their children with ID are discussed.

Purpose: Meeting the personal care challenges of children with special health care needs (CSHCN) living in the community demands a reliable assessment of their abilities to perform activities of daily living (ADLs). This research investigates factors affecting the inter-rater reliability of functional assessments of CSHCN conducted in the home., Methods: Dual-standardised assessments were conducted with 236 community-dwelling CSHCN seeking or receiving Medicaid Personal Care Services (PCS) in a single state in USA., Results: Analyses revealed that assessments of CSHCN with the greatest or least amount of activity limitations exhibited the greatest agreement. The greatest disagreement occurred when assessors faced children with moderate to moderately severe activity limitations. Specific ADLs, where the greatest and least agreements occurred, varied by the level of the child's overall activity limitation., Conclusions: These results imply that the most serious challenges to the reliability of home-based assessment of ADLs among CSHCN occur in cases of children with moderate or moderately severe activity limitations.

Background: Like most caregivers, informal caregivers for children (typically parents) with special needs supply the majority of the care provided. Formal care is sometimes needed to supplement informal caregivers' efforts. For those interested in children with special needs, there is a paucity of research on those factors affecting the amount of formal care that caregivers' request., Objectives/hypotheses: This research investigates factors affecting the amount of Medicaid personal care services (PCS) requested by primary caregivers for children with special needs. The research focuses especially on the roles played by the child's functional status, intellectual abilities, and behaviors in determining the level of assistance requested by caregivers., Methods: The data used in this research are cross-sectional information on 262 children with special needs who were Medicaid recipients in a single southwestern state. These data were collected in 2007 by master's trained social workers or registered nurses using a standardized assessment instrument., Results: The results indicate that a child's ability to independently perform activities of daily living (ADLs) tasks plays the major role in determining caregivers' perceptions of the need for PCS. The severity of a child's intellectual disability, like some other factors investigated, has an effect on caregivers' perceptions, but it is an indirect effect that operates through the level of the child's ADL impairment. A child's age and behaviors have direct effects on caregivers' perceptions of need, as does the presence of barriers to the caregiver providing care., Discussion: Much of the research on children with special needs has emphasized the importance of the child's medical or behavioral diagnoses. Little attention has been given to modeling caregivers' perceptions of children's needs. This analysis of caregivers' requests for formal PCS brings to the forefront the role of ADL or functional status in this process. Many factors that one would expect to directly affect caregivers' perceptions of need, instead, have indirect effects filtered through their impact on the child's functional status., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Teledermatology studies have examined diagnostic concordance between live-interactive (LI) and in-person examinations (IP); and between store-and-forward (SF) and IP examinations. However, no studies have looked simultaneously across all three care delivery modalities, and few have measured management concordance and diagnostic confidence of the dermatologist. The purpose of this study was to compare LI and SF modalities with IP with respect to diagnostic and management concordance and to compare physician diagnostic confidence across the three modalities. Four dermatologists, in random rotation among all three care modalities, examined 110 new patients. Confidence was rated on a Likert scale from 1 to 5 (5 = total confidence; 1 = no confidence). Identical diagnoses were given to the patient by examiners from all three examination modalities in 70/110 patients (64%). More identical diagnoses were given for IP and LI examinations than for IP and SF examinations (80% vs. 73%); however, the difference was not statistically significant (p = 0.13). The highest self-reported confidence rating was given for 87% of IP examinations, 59% for LI, and 54% for SF. Diagnostic confidence ratings for SF and LI were not significantly different from each other (p = 0.50); however, diagnostic confidence ratings for LI and SF were both statistically lower than IP (p < 0.0001). Dermatologists were more confident with IP examination than either form of teledermatology. The percent of diagnostic and management agreement among IP, LI, and SF modalities was high.

Demand for pediatric dermatologic care far exceeds the supply of pediatric dermatologists. Teledermatology has been proposed as a solution to improve access to care, however, data regarding teledermatology use for pediatric patients are lacking. Surveys assessing use and experience with teledermatology were administered to pediatric dermatologists attending the 2006 Society for Pediatric Dermatology Annual Meeting and to programs offering teledermatology identified in the American Telemedicine Association's 2003 Teledermatology Survey. Over half of the 76 pediatric dermatologists responding to the survey had previous experience with teledermatology and nearly half currently use some form of teledermatology. For most, this represents nonreimbursed, store-and-forward consultations. Respondents noted that teledermatology allows more accurate triage of dermatology patients, decreases travel and outpatient clinic visits, and provides an avenue for ongoing support and education for primary care physicians. While difficulties exist, overcoming obstacles to the use of pediatric teledermatology and reimbursement for such services could improve access to expert pediatric dermatologic care.

Neutrophilic panniculitis is rare and is classified as a panniculitic member of the neutrophilic dermatoses spectrum. In affected patients, an underlying systemic disease, such as myelodysplasia, is often present. We describe an infant with juvenile rheumatoid arthritis who developed neutrophilic panniculitis. Neutrophilic panniculitis clinically mimics other panniculitides and biopsy specimen can be diagnostic. Identification of this entity can aid the diagnosis of the underlying systemic process.

In this case presentation recognition of multiple triangular lunula unguis led to the diagnosis of the rare nail-patella syndrome (NPS). A single triangular lunula may be traumatic in origin, but multiple, with apex pointing toward the free edge of the nail, must be considered part of the NPS especially when combined with absence of the distal interphalangeal joint creases.

Purpose of Review: The delineation of syndromes carrying a predisposition to malignancy has led to great insights into the molecular biology of malignancy. Many such syndromes have cutaneous findings which can precede the development of neoplasia. Early recognition of the cutaneous stigmata of the genodermatoses with malignant potential can lead to early diagnosis and initiation of proper screening and treatment when indicated., Recent Findings: This article reviews 'classic' genodermatoses with malignant potential and highlights recent recommendations for screening and treatment. Additionally more recently delineated syndromes and their cutaneous findings are discussed., Summary: Certain inherited syndromes with a risk of neoplasia exhibit characteristic cutaneous findings. Recognition of these findings by the astute practitioner can lead to early intervention which can impact the course of these rare diseases.

Cutaneous mosaicism is a poorly understood but relatively common dermatological finding. This paper reviews basic concepts of mosaicism as well as common and rare mosaic skin disorders.

The practice of teledermatology has grown considerably in rural and frontier areas where access barriers exist. Live-interactive and store-and-forward modalities are used, either alone or in combination (hybrid model). Research has shown that the diagnoses made and management recommended during teledermatology interactions are in concordance with in-person diagnosis and treatment. Many patients worldwide have limited access to expert dermatological care and the current technology is increasingly offering access for these patients.

All athletes have an increased risk of cutaneous infections. In both team and individual sports the skin is exposed to a range of infectious organisms. While effective therapies exist, preventive measures can decrease outbreaks of most sports-related dermatologic infections. This article highlights common dermatologic infections of the athlete, their prevention and treatment.

Many viral infections exhibit cutaneous lesions. Recognition of the exanthems associated with these infections and the broader clinical scenarios in which they occur can lead to more rapid diagnosis and appropriate treatment for affected patients.

Macroscopic sorption studies indicated that Pb sorption capacity was independent of pH over the pH range 5-7, while sorption as a function of reaction time up to two weeks for systems with no bulk precipitate phases showed continuous Pb uptake on dolomite. This could be due to diffusion of Pb into the micropores of dolomite as well as an increase in surface sites caused by particle size reduction during suspension mixing. Normalized XANES spectra for systems undersaturated with respect to Pb carbonate precipitates resembled the spectrum of Pb4(OH)4(4+), suggesting that Pb is mainly coordinated to dolomite as an inner-sphere surface complex. On the other hand, the XANES spectrum for 10(-3) M Pb at 1 atm CO2(g) in a 2 M Mg(NO3)2 background electrolyte solution resembled that of cerussite, while a sample at 5 x 10(-4) M Pb in equilibrium with air and 2 M Mg(NO3)2 resembled that of hydrocerussite. EXAFS analyses of sorption samples in chloride solutions showed that there were only first-shell contributions under 1 atm CO2(g), while higher shell contributions from Ca/Mg were seen at 10(-3.42) atm CO2(g). On the other hand, EXAFS samples prepared in nitrate solutions showed noticeable differences in speciation under different reaction conditions-from outer-sphere surface complexes at low Pb concentrations and pH, to inner-sphere surface complexes at moderate Pb concentrations and neutral pH, to the formation of Pb carbonate precipitates at the highest Pb loadings.

Accurate sorption modeling is critical for environmental risk assessment and development of sound remedial technologies. Adsorption to iron oxide phases is one of the important sorption processes regulating the bioavailability and toxicity of metal ions in natural systems. In this study, we used spectroscopically derived bidentate surface species to constrain surface complexation modeling in addressing Ni(ll) and Zn(ll) adsorption and competition on goethite surfaces. The 2-pK(a) triple layer model successfully predicted adsorption in single adsorbate systems. The curvature in adsorption isotherms was accurately depicted using two types of sites: high affinity and low affinity, and mononuclear bidentate surface complexes. A constrained set of parameters was found for each metal (log K(L) = -6.63 and log K(H) = -2.45 for Ni, log K(L) = -3.92 and log K(H) = 2.14 for Zn) that successfully described adsorption over a large range of experimental conditions, covering 6 to 7 orders of magnitude in concentration, ionic strength from 10(-3) to 10(-2), and environmentally relevant pH range between 4 and 6.5. Adsorption competition was predicted using the bidentate surface species with parameters calibrated using single adsorbate data.

The classic features of lipoid proteinosis - beadlike papules and hoarseness - result from the accumulation of hyaline material in the mucocutaneous dermis. However, the characteristic manifestation in children - erosive, crusted lesions that lead to scarring - is rarely discussed and poorly understood. Lipoid proteinosis results from mutations in extracellular matrix protein 1, but the function of this protein is largely unknown. We performed ultrastructural studies on lesional epidermis, cultured monolayer keratinocytes, and raft keratinocyte cultures from blistering lesions of a child with lipoid proteinosis. All sections showed the dissociation of relatively intact desmosomes from keratinocytes, with desmosomes that were "free-floating" in the intercellular spaces or attached by thin strands to the cell membrane. These changes were present in serial sections of both tissue and cultured keratinocytes, suggesting this observation to be an inherent feature of keratinocytes devoid of extracellular matrix protein 1, rather than an artifact. Although additional patients should be studied, the diminished appearance of the inner dense plaque - the region of attachment of keratin intermediate filaments to desmosomal proteins - provides preliminary evidence that extracellular matrix protein 1 may participate in attaching keratin intermediate filaments to desmosomal region protein(s).

The congenital disorders of glycosylation are a group of rare metabolic disorders with predominantly neurologic findings. Some variants of this disorder also exhibit cutaneous manifestations. We report a patient with a congenital disorder of glycosylation type Ia, the most common form, with emphasis on the cutaneous findings of this type, and summarize the cutaneous findings in the other forms of the disorder.

Raised limb bands are a recently described disorder characterized by skin-colored linear plaques typically located on the extremities of affected children. Various etiologies for their development have been proposed, including amniotic bands. We report a child with extensive raised bands of the trunk and extremities and documented amniotic bands in utero. We propose altering the name of this entity to reflect the wider potential distribution of the lesions. The presence of documented amniotic bands and other sequelae typical for the amniotic band sequence in this patient strongly implicates amniotic bands or related pathology in the formation of acquired raised bands of infancy.

A previous study involving lead(II) [Pb(II)] sorption onto ferrihydrite over a wide range of conditions highlighted the advantages of combining molecular- and macroscopic-scale investigations with surface complexation modeling to predict Pb(II) speciation and partitioning in aqueous systems. In this work, an extensive collection of new macroscopic and spectroscopic data was used to assess the ability of the modified triple-layer model (TLM) to predict single-solute zinc(II) [Zn(II)] sorption onto 2-line ferrihydrite in NaNO(3) solutions as a function of pH, ionic strength, and concentration. Regression of constant-pH isotherm data, together with potentiometric titration and pH edge data, was a much more rigorous test of the modified TLM than fitting pH edge data alone. When coupled with valuable input from spectroscopic analyses, good fits of the isotherm data were obtained with a one-species, one-Zn-sorption-site model using the bidentate-mononuclear surface complex, (triple bond FeO)(2)Zn; however, surprisingly, both the density of Zn(II) sorption sites and the value of the best-fit equilibrium "constant" for the bidentate-mononuclear complex had to be adjusted with pH to adequately fit the isotherm data. Although spectroscopy provided some evidence for multinuclear surface complex formation at surface loadings approaching site saturation at pH >/=6.5, the assumption of a bidentate-mononuclear surface complex provided acceptable fits of the sorption data over the entire range of conditions studied. Regressing edge data in the absence of isotherm and spectroscopic data resulted in a fair number of surface-species/site-type combinations that provided acceptable fits of the edge data, but unacceptable fits of the isotherm data. A linear relationship between logK((triple bond FeO)2Zn) and pH was found, given by logK((triple bond FeO)2Znat1g/l)=2.058 (pH)-6.131. In addition, a surface activity coefficient term was introduced to the model to reduce the ionic strength dependence of sorption. The results of this research and previous work with Pb(II) indicate that the existing thermodynamic framework for the modified TLM is able to reproduce the metal sorption data only over a limited range of conditions. For this reason, much work still needs to be done in fine-tuning the thermodynamic framework and databases for the TLM.

Aims: To determine whether there were temporal or seasonal patterns in the occurrence of chemical incidents reported to the West Midlands Chemical Incident Surveillance System, UK., Methods: A retrospective analysis of reports maintained on a computerised database was carried out for information received from January 1997 to December 2001., Results: Annual numbers of events increased significantly from 77 (7.5%) in 1997 to 282 (27.4%) in 2001. Compared to the average of other seasons, proportionately more incidents occurred in the summer (29.3%). Compared with the average of other days, incidents were more likely to occur on Thursdays (17.1%) and least likely on Saturdays (8.9%). When grouped together on a six hourly basis incidents were most frequent between 12 00 and 17 59 (31.1%) and least frequent between 00 00 and 05 59 (21.2%)., Conclusions: The surveillance system shows that chemical incidents do not occur randomly but have marked temporal and seasonal variation. These results have implications for service provision, training, and the development of preventive strategies.