Maintaining Family Engagement During Home Visitor Turnover: a Mixed Methods Study of Best Practices.

Evidence-based home visiting services (EBHV) are available in states and localities nationwide through the federally-funded Maternal, Infant, and Early Childhood Home Visiting (MIECHV) program. Nevertheless, the anticipated benefits of EBHV, such as improved child developmental outcomes and increased positive parenting practices, may be undermined by the fact that most families withdraw from services earlier than the model developers planned. Prior studies have linked family attrition with staff turnover. The current study used a mixed methods design to investigate the conditions under which families remained active in the home visiting program after their assigned home visitor resigned. Coincidence Analysis revealed that giving families advance notice (at least 1 month) prior to the home visitors' upcoming resignation or developing a strong positive working alliance with the inheriting home visitor appears to independently make a difference for ongoing family engagement at 3 and 6 months following a staff transition. These findings suggest that emphasizing how staff turnover is managed may mitigate the risk of family withdrawal during these transitions.

Review of the Pathologic Characteristics in Myhre Syndrome: Gain-of-Function Pathogenic Variants in SMAD4 cause a Multisystem Fibroproliferative Response.

Background: Myhre syndrome, caused by pathogenic variants in SMAD4, is characterized by compact body habitus with short stature, distinctive craniofacial appearance, stiff skin, cardiovascular abnormalities (valve stenosis, coarctation, hypoplasia, or stenosis of aorta), effusions of potential spaces (pericardium, pleura, peritoneum), restricted movement of the joints (including thorax), and hearing loss. Lung and airway disease has been reported, but not always well-defined, to include interstitial lung disease, large airway obstruction, and pulmonary arterial hypertension. Excessive fibroproliferation of tissues especially following trauma or surgical instrumentation has been recognized, although these may also present spontaneously. Method: We report the pathologic features of 1 new patient with progressive choanal stenosis, and 22 literature cases, including the expanded history of 5 patients (3 who died). Results: Examination of patient tissues documents cellular fibroproliferation and deposition of excessive extracellular matrix explaining some of the observed clinical features of Myhre syndrome. Conclusion: Excessive fibrosis is noted in multiple tissues, especially heart, lung, and upper and lower airways. Our research provides the first systematic review to provide a knowledge base of gross and pathologic findings in Myhre syndrome.

Lineage Assignment in Acute Leukemia: A Challenging Case in a Pediatric Patient.

We report a case of a 2-year-old girl who was diagnosed with natural killer cell acute lymphoblastic leukemia and treated with an acute lymphoblastic leukemia chemotherapy regimen. Two months posttherapy, the disease relapsed with a myeloid immunophenotype. Complete response was then achieved with acute myeloid leukemia therapy followed by unrelated donor umbilical cord allogenic stem cell transplant. Retrospectively, reanalysis of the diagnostic specimen showed minimal myeloperoxidase expression that was called negative by conventional single parameter linear gating but better appreciated on histogram overlays. This case illustrates that even low levels of myeloperoxidase expression should be considered significant in lineage assignment in acute leukemia.

A mixed methods evaluation of an intervention to prevent perinatal depression among Latina immigrants.

Objective: The effectiveness of a cognitive behavioural intervention to prevent perinatal depression in low-income Latina immigrant pregnant women and mothers receiving WIC services was evaluated in a mixed methods study using a community based observational design.Background: The Mothers and Babies Course is a preventive intervention for perinatal depression that is based on cognitive behavioural theory (CBT). CBT is an evidence-based treatment and preventive intervention for perinatal depression.Method: Phase 1 includes 86 Latinas, predominantly Central American immigrant women at high risk for depression, who self-selected into the Mothers and Babies Course, a six-week Spanish CBT group intervention aimed at teaching women mood regulation skills to prevent the onset of depression. Participants, who were recruited from the Women, Infants, and Children services, completed measures of depression and psychopathology at pre-, 6 weeks, and 3 months post-intervention. Phase 2 includes qualitative interviews with a randomly selected subsample (n = 26) from Phase 1 to understand the mechanisms and impact of participants' experiences with the intervention and study.Results: Results indicated no significant differences in depressive symptoms among participants with varied attendance levels (0 class; 1-3 classes = non-completers; 4-6 classes = completers). None of the participants met diagnostic criteria for major depressive disorder at the final data collection period. Despite the varied attendance, both quantitative and qualitative results indicated that completers and non-completers reported similar experiences in the intervention and benefiting from study participation.Conclusion: Conducting mixed methods research highlights the complexity of understanding who can benefit from preventive interventions.

The role of consultative alliance in infant and early childhood mental health consultation: Child, teacher, and classroom outcomes.

Young children's social-emotional development is powerfully shaped by their early environments, which for many includes early childhood education (ECE). Infant and Early Childhood Mental Health Consultation (IECMHC) pairs teachers and infant and early childhood mental health (IECMH) consultants to promote teachers' capacity to foster positive social-emotional development in ECE. Although the outcomes of IECMHC have been well studied, little research has investigated how this model leads to changes for teachers and children. According to theory, the quality of the relationship between teachers and IECMH consultants, termed consultative alliance (CA), is a key mechanism of change. This study analyzed the role of CA on 6-month outcomes of IECMHC in a sample of 316 children, 289 teachers, and 62 IECMH consultants. Results from multilevel models suggested that stronger CA predicted greater improvements in teacher-child closeness and teacher-rated child attachment behaviors. In addition, a strong CA was related to greater improvement in classroom climate, teachers' self-efficacy, and teachers' perceptions of their jobs. This study upholds the centrality of relationship-building and parallel process in mental health consultation, and by advancing understanding of the mechanisms of change for IECMHC may provide salient implications for policy and practice.

El desarrollo socio-emocional de los niños pequeños es formado de manera poderosa por sus tempranos ambientes, los cuales, para muchos incluyen la educación en la temprana niñez (ECE). La Asesoría de Salud Mental del Infante y la Temprana Niñez (IECMHC) empareja maestros y Asesores de la Salud Mental del Infante y la Temprana Niñez (IECMH) para promover la capacidad de los educadores de crear un positivo desarrollo socio-emocional en ECE. A pesar de que los resultados de IECMHC han sido bien estudiados, poca investigación se ha enfocado en cómo este modelo conduce a cambios para educadores y niños. De acuerdo con la teoría, la calidad de la relación entre educadores y Asesores de IECMH, llamada Alianza Consultiva (CA), es un mecanismo clave para el cambio. Este estudio analizó el papel de la Alianza Consultiva en resultados de seis meses de IECMHC en un grupo muestra de 316 niños, 289 educadores y 62 Asesores de IECMH. Los resultados de modelos de multiniveles sugieren que una más fuerte CA predijo mayores mejoras en la cercanía entre educador y niño y las conductas de afectividad del niño evaluadas por el educador. Adicionalmente, una más fuerte CA se relacionó con una mayor mejora en el ambiente del aula de clases, la auto-efectividad de los educadores y las percepciones que los educadores tenían de su trabajo. Este estudio apoya la centralidad de establecer una relación y un proceso paralelo en la asesoría de salud mental, y por medio del avance en la comprensión de los mecanismos de cambio para IECMHC, pudiera proveer implicaciones destacadas para la política y la práctica.

Le développement socio-émotionnel des jeunes enfants est fortement formé par leurs premiers environnements, ce qui pour bien d'entre eux inclut l'enseignement préscolaire. L'approche IECMHC (Infant and Early Childhood Mental Health Consultation soit Consultation en Santé Mentale du Nourrisson et de la Petite Enfance) jumelle des enseignants et des consultants IECHM (Santé Mentale du Nourrisson et de la Petite Enfance) afin de promouvoir la capacité des enseignements à cultiver et à favoriser un développement socio-émotionnel positif dans l'enseignement préscolaire. Bien que les résultats de l'approche IECMHC aient été bien étudiés il existe peu de recherches sur la manière dont ce modèle mène à des changements pour les enseignants et les enfants. Selon la théorie, la qualité de la relation entre les enseignants et les Consultants IECMH, appelée Alliance Consultative (AC), est un mécanisme clé de changement. Cette étude a analysé le rôle de l'Alliance Consultative sur les résultats à six mois de la IECMHC chez un échantillon de 316 enfants, 289 enseignants, et 62 Consultants IECMH. Les résultats de modèles multi-niveaux ont suggéré qu'une AC plus forte prédisait des améliorations plus importantes dans le rapprochement enseignant-enfant et dans les comportements d'attachement de l'enfant évalués par l'enseignant. De plus une AC plus forte était liée à une amélioration plus importante du climat de la salle de classe, de l'auto-efficacité des enseignements et des perceptions que les enseignants se faisaient de leur travail. Cette étude maintient la centralité de la construction de la relation et du processus parallèle dans la consultation de santé mentale. En faisant progresser la compréhension des mécanismes de changement pour la IECMHC elle présente aussi des implications importantes pour les décisions stratégiques et la pratique.

Developing a Model to Address Mental Health, Substance Use, and Intimate Partner Violence Among Home Visiting Clients.

Perinatal women enrolled in home visiting (HV) programs exhibit high rates of depression, substance use, and intimate partner violence (IPV). While HV programs have increasingly screened for these psychosocial risks, initiation and uptake of community-based services to address these risks remain challenging. This project used a community-engaged research approach to engage key HV stakeholders in developing the screening, referral, and individualized prevention and treatment (SCRIPT) model. We highlight how a group of key HV stakeholders-the SCRIPT Advisory Panel-collaborated with academic researchers to develop the SCRIPT model by reviewing literature on HV programs' response to psychosocial risk factors and qualitative data obtained from mental health, substance use, and IPV service providers to whom HV programs referred clients. SCRIPT focuses on (a) screening for psychosocial risks, (b) developing partnerships with outside agencies to address these risk factors, and (c) establishing concrete and systematic processes for client referral and monitoring with outside agencies. SCRIPT provides a structured model for HV programs to systematically identify clients for key psychosocial risks and structure their referral and monitoring process when working with social service agencies. Future work should examine the impact of SCRIPT on mental health, substance use, and IPV service access and use by a vulnerable population.

Expulsion prevention: Framework for the role of infant and early childhood mental health consultation in addressing implicit biases.

Young children of color-especially boys-are at disproportionate risk for suspension and expulsion from early care and education settings. Infant and Early Childhood Mental Health Consultation (IECMHC) is an approach associated with lower than expected expulsion rates, but the mechanisms through which it may influence preschool expulsion are unknown. This paper reflects on the parallels between IECMHC and interventions created to reduce implicit bias. Based on interviews with leaders in IECMHC practice, implementation, and evaluation, a theoretical framework was created to articulate how IECMHC is hypothesized to affect expulsion by first reducing the influence of implicit bias on disciplinary decisions. Implications for practice and research are provided.

Los pequeños niños de raza negra -especialmente los varones- están bajo un riesgo desproporcionado en cuanto a la suspensión y expulsión de lugares donde se les presta cuidado y educación tempranos. La Consulta de Salud Mental en la Infancia y la Temprana Niñez (IECMHC) es un acercamiento asociado con niveles más bajos de expulsión que los que se esperan, pero se desconocen los mecanismos a través de los cuales la misma pudiera influir en la expulsión prescolar. Este artículo reflexiona sobre los paralelos entre IECMHC y las intervenciones creadas para reducir los prejuicios implícitos. Con base en entrevistas con los líderes en la práctica, implementación y evaluación de IECMHC, se creó un marco de trabajo para articular cómo IECMHC se plantea como hipótesis para afectar la expulsión por medio de reducir primero la influencia de los implícitos prejuicios sobre las decisiones disciplinarias. Se aportan las implicaciones para la práctica y la investigación.

Les jeunes enfants de couleur - surtout les garçons - sont à risque disproportionné de suspension et d'expulsion d'établissements d'éducation et de crèches. La Consultation de Santé Mentale du Nourrisson et de la Petite Enfance (abrégé en anglais ECMHC) est une approche liée à des taux d'expulsion moins élevés que les taux d'expulsion auxquels on s'attend, mais les mécanismes au travers desquels elle pourrait influencer l'expulsion des crèches sont méconnus. Cet article porte sur les parallèles entre la IECHMH et les interventions créées afin de réduire les préjugés implicites. Basé sur des entretiens avec des praticiens de la pratique de l'IECMHC, sa mise en place et son évaluation, une structure théorique a été créée afin d'articuler la manière dont on suppose que l'IECHHC affecte l'expulsion en réduisant d'abord l'influence des préjugés implicites sur les décisions disciplinaires. Des implications pour la pratique et la recherche sont présentées.

An Interprofessional Approach to Teaching Advocacy Skills: Lessons from an Academic Medical-Legal Partnership.

Medical students and educators recognize that preparing the next generation of health leaders to address seemingly intractable problems like health disparities should include advocacy training. Opportunities to acquire the knowledge and skills needed to effectively advocate at the policy level to promote systems-, community-, and population-level solutions are a critical component of such training. But formal advocacy training programs that develop and measure such skills are scarce. Even less common are interprofessional advocacy training programs that include legal and policy experts to help medical students learn such skills. This 2016-2017 pilot study started with a legislative advocacy training program for preclinical medical students that was designed to prepare them to meet with Capitol Hill representatives about a health justice issue. The pilot assessed the impact of adding an interprofessional education (IPE) dimension to the program, which in this case involved engaging law faculty and students to help the medical students understand and navigate the federal legislative process and prepare for their meetings. Results from the pilot suggest that adding law and policy experts to advocacy-focused training programs can improve medical students' advocacy knowledge and skills and increase their professional identity as advocates.

Adaptation of an evidence-based postpartum depression intervention: feasibility and acceptability of mothers and babies 1-on-1.

BACKGROUND: Mothers and Babies (MB) is a cognitive-behavioral intervention with demonstrated efficacy in reducing depressive symptoms and preventing depressive episodes among perinatal women when delivered in a group format by mental health professionals. Study aims were to describe the adaptation of MB into a 1-on-1 modality (MB 1-on-1) and provide data on the adapted intervention's acceptability and feasibility. METHODS: Seventy-five home visitors trained on MB 1-on-1 delivered the 15-session intervention to 1-2 clients. Client acceptability data assessed intervention enjoyment, comprehension, and usefulness. Home visitor feasibility and acceptability data measured amount of intervention material delivered, client comprehension, and client engagement. RESULTS: Home visitors were all female with 8.8 years of experience on average. 117 clients completed acceptability surveys. Average client age was 21.9 years and 41% were pregnant. Home visitors completely covered 87.9% of sessions and reported clients totally understood MB material 82.5% of the time across sessions, although variability was found in comprehension across modules. 82.0% of clients found MB 1-on-1 enjoyable and 91.6% said they totally understood sessions, when averaged across sessions. Clients enjoyed content on noticing one's mood and pleasant activities. Implementation challenges were client engagement, facilitating completion of personal projects, and difficulty shifting between didactic and interactive activities. CONCLUSIONS: Clients found MB 1-on-1 to be enjoyable, easily understood, and useful. Home visitors reported excellent implementation fidelity and felt clients understood MB material. A refined 12-session version of MB 1-on-1 should be examined for its effectiveness in reducing depressive symptoms, given encouraging feasibility and acceptability data.

Low-Level Symptoms of Depression in Mothers of Young Children are Associated with Behavior Problems in Middle Childhood.

OBJECTIVES: Examine the association between mothers' low- and high-level depressive symptoms in early childhood and children's behavior problems in middle childhood. METHODS: We used data from 1844 families in a multi-site, longitudinal study beginning when children were 14 months and continuing to age 11 years. Children's internalizing and externalizing behavior problems at age 11 were assessed using the child behavior checklist for ages 6-18. Mothers' scores on the Center for Epidemiological Studies-Depression Scale when children were 14 months were used to classify them into three groups: 'no depressive symptoms', 'low-level depressive symptoms (below the clinical cut-off)' and 'clinically significant depressive symptoms (above the clinical cut-off).' RESULTS: Mothers were racially/ethnically diverse, including Caucasian (38.9 %), African-American (34.4 %), Hispanic (21.6 %), or other (5.1 %). More than one-third (39.2 %) were teenaged mothers, and 46.0 % did not complete high school. Of the 1844 families, 1172 had age 11 child outcome data. Logistic regression analyses controlling for family demographics revealed a significant association between early maternal depressive symptoms and later child behavioral outcomes. Both low level and clinically significant symptoms were associated with internalizing and externalizing behavior problems. For example, children whose mothers had low-level depressive symptoms were twice as likely to have clinically elevated internalizing problems compared with children whose mothers never had symptoms of depression. CONCLUSIONS: Children whose mothers experience low-level depressive symptoms early in their development have increased risk for later behavioral problems, suggesting a possible need for new screening and intervention strategies for mothers with lower than clinically elevated symptoms.

Increased hepatic stiffness as consequence of high hepatic afterload in the Fontan circulation: a vascular Doppler and elastography study.

UNLABELLED: Hepatic dysfunction is a recognized complication after Fontan palliation of congenital heart disease. We sought to quantitatively measure hepatic stiffness and vascular Doppler indices using ultrasound (US) and shear wave elastography (SWE) in a Fontan cohort. Subjects were prospectively recruited for echocardiography and real-time hepatic duplex US with SWE for hepatic stiffness (kPa). Doppler peak velocities, velocity time integral, resistive, pulsatility, acceleration indices (RI, PI, AI), and flow volume were measured in celiac artery, superior mesenteric artery, and main portal vein (MPV). A subset underwent cardiac catheterizations with liver biopsy. Correlations were explored between SWE, duplex, hemodynamic, and histopathologic data. In all, 106 subjects were studied including 41 patients with Fontan physiology (age 13.8 ± 6 years, weight 45.4 ± 23 kg) and 65 controls (age 15.0 ± 8.4 years, weight 47.9 ± 22 kg). Patients with Fontan physiology had significantly higher hepatic stiffness (15.6 versus 5.5 kPa, P < 0.0001), higher celiac RI (0.78 versus 0.73, P = 0.04) superior mesenteric artery RI (0.89 versus 0.84, P = 0.005), and celiac PI (1.87 versus 1.6, P = 0.034); while MPV flow volume (287 versus 420 mL/min in controls, P = 0.007) and SMA AI (829 versus 1100, P = 0.002) were lower. Significant correlation was seen for stiffness with ventricular end-diastolic pressure (P = 0.001) and pulmonary artery wedge pressure (P = 0.009). Greater stiffness correlated with greater degrees of histopathologic fibrosis. No significant change was seen in stiffness or other duplex indices with age, gender, time since Fontan, or ventricular morphology. CONCLUSION: Elevated hepatic afterload in Fontan, manifested by high ventricular end-diastolic pressures and pulmonary arterial wedge pressures, is associated with remarkably increased hepatic stiffness, abnormal vascular flow patterns, and fibrotic histologic changes. The MPV is dilated and carries decreased flow volume, while the celiac and superior mesenteric arterial RI is increased. SWE is feasible in this population and shows promise as a means for predicting disease severity on liver biopsy.

Myhre syndrome: Clinical features and restrictive cardiopulmonary complications.

Myhre syndrome, a connective tissue disorder characterized by deafness, restricted joint movement, compact body habitus, and distinctive craniofacial and skeletal features, is caused by heterozygous mutations in SMAD4. Cardiac manifestations reported to date have included patent ductus arteriosus, septal defects, aortic coarctation and pericarditis. We present five previously unreported patients with Myhre syndrome. Despite varied clinical phenotypes all had significant cardiac and/or pulmonary pathology and abnormal wound healing. Included herein is the first report of cardiac transplantation in patients with Myhre syndrome. A progressive and markedly abnormal fibroproliferative response to surgical intervention is a newly delineated complication that occurred in all patients and contributes to our understanding of the natural history of this disorder. We recommend routine cardiopulmonary surveillance for patients with Myhre syndrome. Surgical intervention should be approached with extreme caution and with as little invasion as possible as the propensity to develop fibrosis/scar tissue is dramatic and can cause significant morbidity and mortality.

Preventing Perinatal Depression in High Risk Women: Moving the Mothers and Babies Course from Clinical Trials to Community Implementation.

A growing research literature highlights the public health need for preventive interventions to reduce symptoms and incidence of perinatal depression among vulnerable populations. The Mothers and Babies (MB) course is a cognitive-behavioral intervention designed to teach mood regulation skills to English- and Spanish-speaking low-income women at high risk for perinatal depression. We describe the development of the MB course and evaluate the extent to which research findings support efficacy, effectiveness, and dissemination based on the Society for Prevention Research Standards Committee's standards of evidence. Our review of research and implementation activities suggests that the MB intervention demonstrates promising evidence for efficacy in reducing depressive symptoms; empirical support for prevention of major depressive episodes is still preliminary. Work is in progress to evaluate program effectiveness and prepare for broad dissemination and implementation. The MB course shows promise as an intervention for low-income women at risk for perinatal mood issues. Spanish and English intervention materials have been developed that can be delivered in different settings (hospitals, home visiting), in different dosages (6, 8, or 12 sessions), and via different modalities (group, individual). Evaluating the MB course against current standards is intended to inform other prevention intervention development research.

Six-month outcomes from a randomized controlled trial to prevent perinatal depression in low-income home visiting clients.

Perinatal depression (PD) has negative consequences for mothers and children and is more prevalent among women of low socioeconomic status. Home visitation programs serve low-income pregnant women at risk for PD. This study tested the efficacy of a group-based cognitive behavioral intervention (Mothers and Babies Course; MB) in reducing depressive symptoms and preventing the onset of perinatal depression among low-income women enrolled in home visitation. A randomized controlled trial was conducted. Seventy-eight women who were pregnant or had a child less than 6 months of age and who were assessed as at risk for PD were randomized to the MB intervention or usual home visiting services. Depressive symptoms were assessed at baseline and 1-week, 3- and 6-months post-intervention; depressive episodes were assessed with a clinical interview at the 6-month follow-up. Depressive symptoms declined at a significantly greater rate for intervention participants than usual care participants between baseline and 1-week, 3 and 6 months post-intervention. At the 6-month follow-up, 15 % of women who received the MB intervention had experienced a major depressive episode as compared with 32 % of women receiving usual care. Integrating mental health interventions into home visitation appears to be a promising approach for preventing PD. Cognitive behavioral techniques can be effective in preventing depression in perinatal populations and treating it.

The latent symptom structure of the Beck depression inventory: second edition in Latina pregnant women.

Pregnancy represents a unique period of time when women are at an increased risk of developing depression. Although the Beck Depression Inventory-Second Edition (BDI-II) is one of the most widely used self-report measures of depression symptomology, its psychometric properties and underlying factor structures have not been determined for antenatal women and among Latinas. The current study evaluated the latent symptom structure of the BDI-II in a community-based sample of Latina pregnant women (N = 217) identified to be at high risk for depression. Exploratory factor analyses were used to identify underlying salient individual item loadings for two- and three-factor models. Confirmatory factor analyses then examined several different indices to determine the best model fit. Examination of exploratory and confirmatory factor analyses supports a three-factor oblique structure of the BDI-II composed of Cognitive-Affective, Somatic, and Pregnancy Symptoms. The three-factor model provides clinicians with the ability to target specific constellations of depressive symptoms instead of relying on the BDI-II total score that represents the overall severity of depression in this population.

A systematic review of perinatal depression interventions for adolescent mothers.

Poor, adolescent, racial/ethnic minority women are at great risk for developing perinatal depression. However, little research has been conducted evaluating interventions for this population. We conducted a systematic review of preventive and treatment interventions for perinatal depression tested with adolescents, with a focus on low income, minority populations. Nine research-based articles (including one that reported on two studies) were reviewed systematically, and quality ratings were assigned based on a validated measure assessing randomization, double-blinding, and reporting of participant withdrawals. Two treatment studies were identified, both of which were successful in reducing depression. Eight prevention studies were located, of which four were more efficacious than control conditions in preventing depression. Studies sampled mostly minority, low socioeconomic status adolescents. No consistent characteristics across efficacious interventions could be identified. This review underscores the need for researchers to further investigate and build an evidence base.

Integration of Medical Legal Services into a Hospital-Based Violence Intervention Program: A Survey and Interview-Based Provider Needs Assessment.

BACKGROUND: Violent injury among trauma surgery patients is strongly associated with exposure to harmful social determinants of health and negative long-term health outcomes. Medical-legal partnerships in other settings successfully provide patients with legal services to address similar health-harming legal needs and may offer a promising model for the care of violently-injured patients. STUDY DESIGN: An electronic survey tool was distributed to clinicians and staff affiliated with the hospital-based violence intervention program at a single urban level one trauma center. Semi-structured follow up interviews were conducted with participants, and interviews were coded using thematic analysis and grounded theory. RESULTS: Participants reported many health-harming legal needs among their violently injured patients. The most commonly-identified needs were: health insurance denials (62.5%); difficulty accessing crime victims compensation funds (56.3%); trouble accessing official documents (50%); and problems with non-SSDI public benefits (50%). Participants reported inconsistent methods for learning about and responding to patients' health-harming legal needs. The most common barriers to addressing these needs included: lack of awareness that a lawyer could help with the issue (68.8%); prioritization of other needs (68.8%); previous negative legal experiences (62.5%); and cost (62.5%). Identified needs encompass issues traditionally-addressed by MLPs as well as more novel challenges faced by violent injury survivors. CONCLUSION: This survey and interview-based study identifies complex health-harming legal needs present among violently-injured trauma surgery patients. Medical-legal partnerships specially-designed for the setting of violent injury appear well-suited to meet these needs, potentially reducing risk of violent re-injury, long-term negative health outcomes, and healthcare system costs. LEVEL OF EVIDENCE: Level IV / Prognostic and Epidemiological.

Designing and Developing a Medical-Legal Partnership to Address Cancer Patients' Health-Harming Legal Needs.

The Georgetown University's Cancer Legal Assistance and Well-being Project launched in 2020 as a medical-legal partnership that works with health care providers at a Washington, D.C. safety-net hospital to treat the health-harming legal needs of historically and intentionally marginalized patients with cancer.

Molecular and clinical characterization of the myopathic form of mitochondrial DNA depletion syndrome caused by mutations in the thymidine kinase (TK2) gene.

Mitochondrial DNA (mtDNA) depletion syndromes (MDSs) are a clinically and molecularly heterogeneous group of mitochondrial cytopathies characterized by severe mtDNA copy number reduction in affected tissues. Clinically, MDSs are mainly categorized as myopathic, encephalomyopathic, hepatocerebral, or multi-systemic forms. To date, the myopathic form of MDS is mainly caused by mutations in the TK2 gene, which encodes thymidine kinase 2, the first and rate limiting step enzyme in the phosphorylation of pyrimidine nucleosides. We analyzed 9 unrelated families with 11 affected subjects exhibiting the myopathic form of MDS, by sequencing the TK2 gene. Twelve mutations including 4 novel mutations were detected in 9 families. Skeletal muscle specimens were available from 7 out of 11 subjects. Respiratory chain enzymatic activities in skeletal muscle were measured in 6 subjects, and enzymatic activities were reduced in 3 subjects. Quantitative analysis of mtDNA content in skeletal muscle was performed in 5 subjects, and marked mtDNA content reduction was observed in each. In addition, we outline the molecular and clinical characteristics of this syndrome in a total of 52 patients including those previously reported, and a total of 36 TK2 mutations are summarized. Clinically, hypotonia and proximal muscle weakness are the major phenotypes present in all subjects. In summary, our study expands the molecular and clinical spectrum associated with TK2 deficiency.

Hepatic fibrosis persists and progresses despite biochemical improvement in children treated with intravenous fish oil emulsion.

OBJECTIVES: Intestinal failure-associated liver disease (IFALD) is a multifactorial process, which can culminate in cirrhosis and need for transplantation. Fish oil-based lipid emulsions (FOE) reportedly reverse hyperbilirubinemia, but there are little data on their effect on the histopathology of IFALD. METHODS: We blindly examined sequential liver biopsy data on 6 children receiving FOE, with scoring of cholestasis, inflammation, fibrosis, and ductal proliferation based on standardized systems. This information was correlated with biochemical and clinical data to determine any possible relations between biologic and histologic improvement. RESULTS: The median gestational age was 35 weeks, median birth weight 2064 g, and common most reason for intestinal loss was gastroschisis (5/6 children). Median intestinal length was 26 cm beyond the ligament of Treitz and most children had roughly 2 of 3 of their colonic length. It was observed that although hyperbilirubinemia reversed and hepatic synthetic function was preserved across timepoints, fibrosis was persistent in 2 cases, progressive in 3 cases, and regressed in only 1. It remained severe (grade 2 or higher) in 5 of 6 children at last biopsy. Histologic findings of cholestasis improved in all patients and inflammation improved in 5 of 6 children. There were mixed effects on ductal proliferation and steatosis. CONCLUSIONS: In children treated with FOE, reversal of hyperbilirubinemia is not reflected by a similar histologic regression of fibrosis at the timepoints studied. Children with IFALD should have active ongoing treatment and be considered for early referral to an Intestinal Failure Program even with a normalized bilirubin.