Addressing Emerging Needs Through the COVID-19 and Children With Medical Complexity ECHO.

The COVID-19 and Children with Medical Complexity (CMC) Extension for Community Healthcare Outcomes (ECHO) was developed as a "just in time" learning format to respond to the vast number of challenges faced by health care clinicians, public health professionals, and families/caregivers of CMC during the coronavirus disease 2019 (COVID-19) pandemic. A unique aspect of the ECHO was the meaningful integration of family leaders who participated as faculty, learners, case study presenters, and guest lecturers. Another distinguishing feature of this ECHO was its inclusion within a Collaborative Improvement and Innovation Network. A mixed methods analysis found that the COVID-19 and CMC ECHO was associated with significant gains in knowledge and confidence in caring for CMC and their families during the COVID-19 pandemic. This article provides an overview of the results of the COVID-19 and CMC ECHO and provides recommendations related to utilizing the ECHO model as a rapid response mechanism for systems improvement, clinical practice improvement, and education during a public health emergency.

Lung Cancer Staging: Imaging and Potential Pitfalls.

Lung cancer is the leading cause of cancer deaths in men and women in the United States. Accurate staging is needed to determine prognosis and devise effective treatment plans. The International Association for the Study of Lung Cancer (IASLC) has made multiple revisions to the tumor, node, metastasis (TNM) staging system used by the Union for International Cancer Control and the American Joint Committee on Cancer to stage lung cancer. The eighth edition of this staging system includes modifications to the T classification with cut points of 1 cm increments in tumor size, grouping of lung cancers associated with partial or complete lung atelectasis or pneumonitis, grouping of tumors with involvement of a main bronchus regardless of distance from the carina, and upstaging of diaphragmatic invasion to T4. The N classification describes the spread to regional lymph nodes and no changes were proposed for TNM-8. In the M classification, metastatic disease is divided into intra- versus extrathoracic metastasis, and single versus multiple metastases. In order to optimize patient outcomes, it is important to understand the nuances of the TNM staging system, the strengths and weaknesses of various imaging modalities used in lung cancer staging, and potential pitfalls in image interpretation.

Approach to Imaging of Mediastinal Masses.

Mediastinal masses present a diagnostic challenge due to their diverse etiologies. Accurate localization and internal characteristics of the mass are the two most important factors to narrow the differential diagnosis or provide a specific diagnosis. The International Thymic Malignancy Interest Group (ITMIG) classification is the standard classification system used to localize mediastinal masses. Computed tomography (CT) and magnetic resonance imaging (MRI) are the two most commonly used imaging modalities for characterization of the mediastinal masses.

Spectrum of Imaging Patterns of Lung Cancer following Radiation Therapy.

Radiation therapy using conventional or newer high-precision dose techniques, including three-dimensional conformal radiotherapy, intensity-modulated radiation therapy, stereotactic body radiation therapy, four-dimensional conformational radiotherapy, and proton therapy, is an important component of treating patients with lung cancer. Knowledge of the radiation technique used and the expected temporal evolution of radiation-induced lung injury, as well as patient-specific parameters such as previous radiotherapy, concurrent chemoradiotherapy, or immunotherapy, is important in image interpretation. This review discusses factors that affect the development and severity of radiation-induced lung injury and its radiological manifestations, as well as the differences between conventional and high-precision dose radiotherapy techniques.

Aberrant p16, p53 and Ki-67 immunohistochemistry staining patterns can distinguish solitary keratoacanthoma from cutaneous squamous cell carcinoma.

Keratoacanthoma (KA) is widely considered a benign, usually self-resolving, neoplasm distinct from cutaneous squamous cell carcinoma (cSCC), while some consider KA to be indistinguishable from cSCC. Published studies indicate utility for p16, p53, Ki-67 immunostaining and elastic van Gieson (EVG) in the assessment of KA and cSCC. We compared clinical features and staining patterns for p16, p53, Ki-67 and EVG in fully excised KA, cSCC with KA-like features (cSCC-KAL) and other cSCC (cSCC-OTHER). Significant differences between KA, cSCC-KAL and cSCC-OTHER were found for head and neck location (20%, 86%, 84%), and duration <5 months (95%, 63%, 36%). KA shows both a mosaic pattern for p16 (>25-90% of neoplasm area) and peripheral graded pattern for p53 (up to 50% moderate and strong nuclear staining) in 92% compared with 0% of cSCC-KAL and 0% of cSCC-OTHER. In contrast, a highly aberrant pattern (usually null) for one or both p16 and p53, was present in 0% of KA, 83.8% of cSCC-KAL and 90.9% of cSCC-OTHER. Abnormal distribution of Ki-67 beyond the peripheral 1-3 cells was uncommon in KA (4.2%) and common in cSCC-KAL (67.6%) and cSCC-OTHER (88.4%). Moderate to striking entrapment of elastic and collagen fibres was present in the majority of KA (84%), cSCC-KAL (81%) and cSCC-OTHER (65%). KA are clinically distinct neoplasms typically of short duration occurring preferentially outside the head and neck and generally lacking aberrations of p16, p53 and Ki-67, compared with cSCC that have high rates of aberrant or highly aberrant p16, p53 and Ki-67, but EVG lacked specificity.

Review of outpatient procedural sedation clinics in a tertiary paediatric hospital in South Australia.

AIM: Procedures, such as immunisation and venepuncture, can be distressing for paediatric patients, especially those with needle phobia and neurodevelopmental disorders. Procedural sedation helps provide access to equitable health care in this population. The aim of this study was to evaluate the pilot outpatient procedural sedation clinics at the Women's and Children's Hospital and the impact on patient care and outcomes. METHODS: A prospective review was undertaken between July 2021 and May 2022 on all patients who attended the procedural sedation clinics at the Women's and Children's Hospital. These clinics were the COVID Specialist Immunisation Sedation Clinic (SISC) and Paediatric Sedation Clinic (PSC). RESULTS: There were 182 visits in a total of 110 children with a 92% primary procedure success rate. Sixty-three per cent of patients had neurodevelopmental disorders with autism spectrum disorder being most common. There was a significant reduction in anxiety scores pre- and post-sedation and a reduction in anxiety scores if patients were to return without the use of sedation. CONCLUSIONS: Outpatient procedural sedation is beneficial for a specific cohort of the paediatric population. This can also have a significant positive impact on patient care and potentially, long-term outcomes.

Inborn Errors of Immunity in Children With Invasive Pneumococcal Disease: A Multicenter Prospective Study.

BACKGROUND: In settings with universal conjugate pneumococcal vaccination, invasive pneumococcal disease (IPD) can be a marker of an underlying inborn error of immunity. The aim of this study was to determine the prevalence and characterize the types of immunodeficiencies in children presenting with IPD. METHODS: Multicenter prospective audit following the introduction of routinely recommended immunological screening in children presenting with IPD. The minimum immunological evaluation comprised a full blood examination and film, serum immunoglobulins (IgG, IgA and IgM), complement levels and function. Included participants were children in whom Streptococcus pneumoniae was isolated from a normally sterile site (cerebrospinal fluid, pleura, peritoneum and synovium). If isolated from blood, features of sepsis needed to be present. Children with predisposing factors for IPD (nephrotic syndrome, anatomical defect or malignancy) were excluded. RESULTS: Overall, there were 379 episodes of IPD of which 313 (83%) were eligible for inclusion and 143/313 (46%) had an immunologic evaluation. Of these, 17/143 (12%) were diagnosed with a clinically significant abnormality: hypogammaglobulinemia (n = 4), IgA deficiency (n = 3), common variable immunodeficiency (n = 2), asplenia (n = 2), specific antibody deficiency (n = 2), incontinentia pigmenti with immunologic dysfunction (n = 1), alternative complement deficiency (n = 1), complement factor H deficiency (n = 1) and congenital disorder of glycosylation (n = 1). The number needed to investigate to identify 1 child presenting with IPD with an immunologic abnormality was 7 for children under 2 years and 9 for those 2 years old and over. CONCLUSIONS: This study supports the routine immune evaluation of children presenting with IPD of any age, with consideration of referral to a pediatric immunologist.

Paying Family Medical Caregivers for Children's Home Healthcare in Colorado: A Working Medicaid Model.

OBJECTIVE: To compare the characteristics and healthcare use of children with medical complexity who receive paid certified nursing assistant (CNA) care by a family member (family CNA) and by a traditional nonfamily member (nonfamily CNA). STUDY DESIGN: This was retrospective cohort study of children who received CNA care through Colorado's Medicaid paid family caregiving program between 2017 and 2019 by a home healthcare agency. We compared patient characteristics between the family CNA and nonfamily CNA groups. A multivariable Poisson regression model was used to compare hospitalization rates (days in the hospital per year), adjusting for patient age patient sex, nursing care, and complex chronic condition. RESULTS: Of 861 patients, 79% (n = 680) received family CNA care and 21% (n = 181) received nonfamily CNA care. Patient demographics and hospitalization did not differ between the groups, although patients who had family CNAs were less likely to receive additional nursing-level care (42% vs 60%, P < .01). Family and nonfamily CNA caregivers had similar characteristics, except that family CNA caregivers had substantially better 3-year retention (82% vs 9%, P < .01) despite lower average hourly pay ($14.60 vs $17.60 per hour, P < .01). Hospitalizations were rare (<10% of patients). In the adjusted model, patients who received family CNA care experienced 1 more hospitalized day per year, compared with patients who received nonfamily CNA care (P < .001). CONCLUSIONS: Paid family caregivers provided CAN-level care to children with medical complexity with a greater employee retention compared with nonfamily CNA caregivers, with marginally different hospitalization rates using a family-centered approach. This model may help address workforce shortages while also providing income to family caregivers.

Alzheimer's-Associated Upregulation of Mitochondria-Associated ER Membranes After Traumatic Brain Injury.

Traumatic brain injury (TBI) can lead to neurodegenerative diseases such as Alzheimer's disease (AD) through mechanisms that remain incompletely characterized. Similar to AD, TBI models present with cellular metabolic alterations and modulated cleavage of amyloid precursor protein (APP). Specifically, AD and TBI tissues display increases in amyloid-ß as well as its precursor, the APP C-terminal fragment of 99 a.a. (C99). Our recent data in cell models of AD indicate that C99, due to its affinity for cholesterol, induces the formation of transient lipid raft domains in the ER known as mitochondria-associated endoplasmic reticulum (ER) membranes ("MAM" domains). The formation of these domains recruits and activates specific lipid metabolic enzymes that regulate cellular cholesterol trafficking and sphingolipid turnover. Increased C99 levels in AD cell models promote MAM formation and significantly modulate cellular lipid homeostasis. Here, these phenotypes were recapitulated in the controlled cortical impact (CCI) model of TBI in adult mice. Specifically, the injured cortex and hippocampus displayed significant increases in C99 and MAM activity, as measured by phospholipid synthesis, sphingomyelinase activity and cholesterol turnover. In addition, our cell type-specific lipidomics analyses revealed significant changes in microglial lipid composition that are consistent with the observed alterations in MAM-resident enzymes. Altogether, we propose that alterations in the regulation of MAM and relevant lipid metabolic pathways could contribute to the epidemiological connection between TBI and AD.

Offshore detention: cross-sectional analysis of the health of children and young people seeking asylum in Australia.

OBJECTIVE: To describe the health and well-being of children and young people (CYP) seeking asylum subjected to Australia's immigration policy of indefinite mandatory detention on Nauru. DESIGN: Cross-sectional analysis of a cohort of CYP seeking asylum. SETTING: Australian paediatric clinicians from 10 health services completed detailed health assessments around the time of transfer from Nauru, mostly to Australia. PARTICIPANTS: Sixty-two CYP who were ≤18 years on entry into offshore immigration detention on Nauru between 2013 and 2019. Mean age at health assessment was 9 years. MAIN MEASURES: Health outcomes were categorised as physical, mental or neurodevelopmental concerns/conditions. Risk and protective factor data were collected using the adverse childhood experiences and refugee-specific adverse childhood experiences tools. RESULTS: Over half of the CYP (n=32, 52%) were held on Nauru for ≥4 years. The vast majority of CYP had physical health (n=55, 89%) and mental health (n=49, 79%) concerns including self-harm or suicidal ideation/attempt (n=28, 45%). Mental health concerns were more likely in CYP who were school-aged (p=0.001), had been held on Nauru for ≥1 year (p=0.01); originated from the Eastern Mediterranean region (p<0.05); witnessed trauma (p<0.05) or had exposure to ≥4 refugee-specific adverse childhood experiences (p<0.05). Neurodevelopmental concerns were seen in eight children (13%). CONCLUSIONS: This study highlights the almost universal physical and mental health difficulties in a sample of CYP who experienced forced migration and were subjected to Australia's offshore immigration detention policy. Immigration detention in recipient countries, a known adverse childhood experience, may contribute to or exacerbate harmful outcomes in CYP seeking asylum.

Clinical Phenotypes of Patients With Systemic Sclerosis With Distinct Molecular Signatures in Skin.

OBJECTIVE: Systemic sclerosis (SSc) patients are classified according to degree of skin fibrosis (limited and diffuse cutaneous [lc and dc]) and serum autoantibodies. We undertook the present multicenter study to determine whether intrinsic subset (IS) classification based upon skin gene expression yields additional valuable clinical information. METHODS: SSc patients and healthy participants (HPs) were classified into Normal-like, Limited, Fibroproliferative, and Inflammatory ISs using a previously trained classifier. Clinical data were obtained (serum autoantibodies, pulmonary function testing, modified Rodnan skin thickness scores [mRSS], and high-resolution chest computed tomography [HRCT]). Statistical analyses were performed to compare patients classified by IS, traditional cutaneous classification, and serum autoantibodies. RESULTS: A total of 223 participants (165 SSc [115 dcSSc and 50 lcSSc] and 58 HPs) were classified. Inflammatory IS patients had higher mRSS (22.1 ± 9.9; P < 0.001) than other ISs and dcSSc patients (19.4 ± 9.4; P = 0.05) despite similar disease duration (median [interquartile range] months 14.9 [19.9] vs. 18.4 [31.6]; P = 0.48). In multivariable modeling, no significant association between mRSS and RNA polymerase III (P = 0.07) or anti-topoisomerase I (Scl-70) (P = 0.09) was found. Radiographic interstitial lung disease (ILD) was more prevalent in Fibroproliferative IS compared with other ISs (91%; P = 0.04) with similar prevalence between lcSSc and dcSSc (67% vs. 76%; P = 0.73). Positive Scl-70 antibody was the strongest ILD predictor (P < 0.001). Interestingly, all lcSSc/Fibroproliferative patients demonstrated radiographic ILD. CONCLUSIONS: Classification by IS identifies patients with distinct clinical phenotypes versus traditional cutaneous or autoantibody classification. IS classification identifies subgroups of SSc patients with more radiographic ILD (Fibroproliferative), higher mRSS (Inflammatory), and milder phenotype (Normal-like) and may provide additional clinically useful information to current SSc classification systems.

A Framework for Harmonization of Radiomics Data for Multicenter Studies and Clinical Trials.

PURPOSE: Variability in computed tomography images intrinsic to individual scanners limits the application of radiomics in clinical and research settings. The development of reproducible and generalizable radiomics-based models to assess lesions requires harmonization of data. The purpose of this study was to develop, test, and analyze the efficacy of a radiomics data harmonization model. MATERIALS AND METHODS: Radiomic features from biopsy-proven untreated hepatic metastasis (N = 380) acquired from 167 unique patients with pancreatic, colon, and breast cancers were analyzed. Radiomic features from volume-match 551 samples of normal liver tissue and 188 hepatic cysts were included as references. A novel linear mixed effect model was used to identify effects associated with lesion size, tissue type, and scanner model. Six separate machine learning models were then used to test the effectiveness of radiomic feature harmonization using multivariate analysis. RESULTS: Proposed model identifies and removes scanner-associated effects while preserving cancer-specific functional dependence of radiomic features on the tumor size. Data harmonization improves the performance of classification models by reducing the scanner-associated variability. For example, the multiclass logistic regression model, LogitBoost, demonstrated the improvement in sensitivity in the range from 15% to 40% for each type of liver metastasis, whereas the overall model accuracy and the kappa coefficient increased by 5% and 8% accordingly. CONCLUSION: The model removed scanner-associated effects while preserving cancer-specific functional dependence of radiomic features.

The Role of Care Coordinators for Children with Respiratory Technologies and Home Nursing.

Background: Children with respiratory technologies, particularly those with mechanical ventilation, represent a growing population that require complex home nursing, medical equipment, outpatient medical and habilitative supports to live and thrive in their community. Care coordination is essential to support these children and their families to navigate and integrate key community-based health and educational services, however, care is often fragmented and care coordination needs unmet. Therefore, to fully support children with respiratory technologies, it is critical to understand the role of care coordinators (CCs) and how to sustain this workforce. The aim of this article is to describe CCs' perspective on (1) their role in supporting families in a home care program for children with respiratory technologies and home nursing, and (2) the core components of recruiting into and sustaining the CC workforce. Methods: Semistructured interviews were conducted with 15 CC from the Division of Specialized Care for Children (DSCC) Home Care program for children with technology dependence and home nursing in Illinois. Two independent coders utilized a modified template approach and discussed to agreement to analyze transcripts. Results: CC averaged 6.6 years of CC experience; the majority had social work or nursing backgrounds. CCs' job satisfaction was derived from their role supporting hospital discharge, seeing children improve over time, and navigating challenges with families. CCs enjoyed working in a collaborative environment where they could draw from their colleagues' experience to solve problems. Job dissatisfaction and job turnover stemmed from difficult family interactions, high caseloads, and redundant and time-intensive administrative tasks, which interfered with family engagement. Conclusions: CCs for children with respiratory technologies require diverse skills, but interdisciplinary teams enable collaborative support of families. Seeing children thrive can sustain the workforce, however, CCs report challenges due to high caseloads and administrative tasks, which impede direct family involvement.

Health Equity for Children and Youth With Special Health Care Needs: A Vision for the Future.

Health equity is a key pillar in supporting a future in which CYSHCN enjoy a full life and thrive, as envisioned by experts and community partners who gathered in 2019 and 2020 to develop the Blueprint for Change: Guiding Principles for a System of Services for Children and Youth With Special Health Care Needs and Their Families. However, a variety of contextual factors impact health outcomes across the life course and intergenerationally and must be addressed to achieve this goal. For example, poverty and discrimination, including by some health care professionals and systems, are important, modifiable root causes of poor health outcomes. There are numerous barriers to achieving health equity, including political will, lack of resources, insufficient training, and limited cross-sector collaborations. Political, cultural, societal, and environmental interventions are necessary to eliminate health disparities and achieve health equity. The entities that serve CYSHCN should be equitably designed and implemented to improve health outcomes and address health disparities. Many entities that serve CYSHCN are taking positive steps through workforce development, policy changes, community engagement, and other means. The purpose of this article is to frame health equity for CYSHCN, detail their health disparities, review barriers to health equity, provide examples of strategies to advance health equity for them, and describe a path toward the future in which all CYSHCN have a fair and just opportunity to be as healthy as possible.

Demystifying the CT Radiation Dose Sheet.

The radiation dose sheet generated by the CT scanner is a form that displays important information about an examination. It functions as a road map for the examination, detailing what CT examinations were performed and what parameters were used to perform them. One essential element of the radiation dose sheet, the volume CT dose index (CTDIvol), is a commonly used radiation dose index that is displayed on most CT scanners. The CTDIvol is used for quality control and is helpful for comparing the radiation output among different protocols and different scanners. The dose-length product (DLP) is a radiation dose index that builds on the CTDIvol by incorporation of the scan length. The DLP is combined with a conversion coefficient and used to determine the effective dose from the CT examination. Determining the effective dose is a way to estimate the whole-body radiation dose, even if the CT examination is confined to a smaller part of the body. In addition to these values, other data about the study from the CT scanner manufacturer, including the tube voltage and tube current-time product, usually are displayed on CT scanners. These values are major determinants of the image quality and radiation dose. The radiation dose sheet is a useful tool for radiologists, technologists, and physicists, allowing them to comprehend the technical details of a CT examination. The authors describe the components of the radiation dose sheet, the relationships of these components with one another, and the contributions of these components to the radiation dose. ©RSNA, 2022.

Understanding Caregiving and Caregivers: Supporting Children and Youth With Special Health Care Needs at Home.

Caregiving encompasses the nurturing, tasks, resources, and services that meet the day-to-day needs of children and youth with special health care needs (CYSHCN) at home. Many gaps exist in the strategies currently offered by the health care system to meet the caregiving needs of CYSHCN. The work of family caregivers of CYSHCN is known to be extensive, but it is so poorly understood that it has been described as "invisible". This invisibility leads to poor communication and gaps in understanding between professional health care providers and family caregivers. To address these gaps, health care researchers must work with family caregivers to incorporate their expertise on caregiving and create meaningful and sustainable research partnerships. A growing body of research is attempting to remedy the problem of caregiving invisibility and lay better foundations for successful integration between health care settings, family caregiving, professional caregiving, and community supports for families of CYSHCN. We identify high-priority gaps in CYSHCN caregiving research and propose research questions that are designed to accelerate growth in evidence-based understanding of the work of family caregivers of CYSHCN and how best to support them.

Alcohol induced hepatic retinoid depletion is associated with the induction of multiple retinoid catabolizing cytochrome P450 enzymes.

Chronic alcohol consumption leads to a spectrum of liver disease that is associated with significant global mortality and morbidity. Alcohol is known to deplete hepatic vitamin A content, which has been linked to the pathogenesis of alcoholic liver disease. It has been suggested that induction of Cytochrome P450 2E1 (CYP2E1) contributes to alcohol-induced hepatic vitamin A depletion, but the possible contributions of other retinoid-catabolizing CYPs have not been well studied. The main objective of this study was to better understand alcohol-induced hepatic vitamin A depletion and test the hypothesis that alcohol-induced depletion of hepatic vitamin A is due to CYP-mediated oxidative catabolism. This hypothesis was tested in a mouse model of chronic alcohol consumption, including wild type and Cyp2e1 -/- mice. Our results show that chronic alcohol consumption is associated with decreased levels of hepatic retinol, retinyl esters, and retinoic acid. Moreover, the depletion of hepatic retinoid is associated with the induction of multiple retinoid catabolizing CYPs, including CYP26A1, and CYP26B1 in alcohol fed wild type mice. In Cyp2e1 -/- mice, alcohol-induced retinol decline is blunted but retinyl esters undergo a change in their acyl composition and decline upon alcohol exposure like WT mice. In conclusion, the alcohol induced decline in hepatic vitamin A content is associated with increased expression of multiple retinoid-catabolizing CYPs, including the retinoic acid specific hydroxylases CYP26A1 and CYP26B1.