Osteosarcoma emboli presenting as chronic thromboembolic pulmonary hypertension in a child.

A 13-year-old girl presented with hypoxemia during adjuvant chemotherapy for an osteosarcoma of the left distal femur. She underwent an amputation complicated by a post-operative pulmonary embolism (PE). Three months post-operatively, she was admitted to hospital with severe hypoxemia and diagnosed with pulmonary hypertension on echocardiogram in the context of extensive bilateral PE on computed tomography. She was planned for elective pulmonary thromboendarterectomy, but rapidly deteriorated requiring emergent surgery. At the time of surgery, she was found to have extensive tumor emboli throughout both pulmonary arteries. She recovered well post-operatively but died 2 months later from progressive disease.

Prioritizing a Research Agenda of Transitional Care Interventions for Childhood-Onset Disabilities.

Transitional care interventions have the potential to optimize continuity of care, improve health outcomes and enhance quality of life for adolescents and young adults living with chronic childhood-onset disabilities, including neurodevelopmental disorders, as they transition to adult health and social care services. The paucity of research in this area poses challenges in identifying and implementing interventions for research, evaluation and implementation. The purpose of this project was to advance this research agenda by identifying the transitional care interventions from the scientific literature and prioritize interventions for study. A modified-Delphi approach involving two rounds of online surveys followed by a face-to-face consensus meeting with knowledge users, researchers and clinician experts in transitional care (n = 19) was used. A subsequent virtual meeting concluded the formulation of next steps. Experts rated 16 categories of interventions, derived from a systematic review, on importance, impact, and feasibility. Seven of the 16 interventions categories received a mean score rating of ≥7 (out of 10) on all three rating categories. Participants then rank ordered the reduced list of seven interventions in order of priority and the top four ranked interventions advanced for further discussion at a consensus meeting. Using the Template for Intervention Description and Replication (TIDieR) checklist as a guide, the participants identified that a study of a peer system navigator was worthy of future evaluation. This study highlighted that transitional care interventions are complex and multifaceted. However, the presence of a peer to support system navigation, advocacy and individual and family education was considered the most ideal intervention addressing the current gap in care. Future research, which aims to engage patients and families in a co-design approach, is recommended to further develop this intervention.

Transitional Care Interventions for Youth With Disabilities: A Systematic Review.

CONTEXT: Transition from the pediatric to the adult health care system is a complex process that should include medical, psychosocial, educational, recreational, and vocational considerations. OBJECTIVE: In this systematic review, we aim to synthesize the evidence on transitional care interventions (TCIs) to improve the quality of life (QoL) for adolescents and young adults with childhood-onset disabilities, including neurodevelopmental disorders. DATA SOURCES: Four electronic databases (Medline, Embase, PsycINFO, and Cumulative Index to Nursing and Allied Health Literature) were searched. STUDY SELECTION: In the included studies, researchers examined TCIs for adolescents and young adults (12-24 years of age) with childhood-onset disabilities. Studies were experimental, quasi-experimental, and observational studies published in the last 26 years. DATA EXTRACTION: Two reviewers independently completed study screening, data extraction, and risk-of-bias assessment. RESULTS: Fifty-two studies were included. Five studies reported on QoL, but statistically significant improvements were noted in only 1 of these studies. Significant improvements were also found in secondary outcomes including disability-related knowledge and transitional readiness. TCIs targeted patients, families and/or caregivers, and health care providers and exhibited great heterogeneity in their characteristics and components. LIMITATIONS: Inconsistent reporting on interventions between studies hindered synthesis of the relationships between specific intervention characteristics and outcomes. CONCLUSIONS: Although there is limited evidence on the impact of TCIs on the QoL for youth with childhood-onset disabilities, there is indication that they can be effective in improving patient and provider outcomes. The initiation of transition-focused care at an early age may contribute to improved long-term health outcomes in this population.

Analysis of Extreme Length of Stay Hospitalizations for Children and Youth in a Quaternary Care Hospital.

Length of stay (LOS) is an important issue for many healthcare organizations. In-patients with extreme LOS account for a disproportionately large percentage of hospital costs. Our analysis of over 15,000 pediatric hospital discharges at The Hospital for Sick Children (Toronto, Canada) between 2015 and 2016 revealed that the vast majority of patients with extreme LOS were discharged directly home, with only a minority receiving home-based services. Patients with the greatest LOS were accounted for by primarily four subspecialty services. Although this report outlines an analysis of pediatric in-patients, our findings and implications are relevant for all jurisdictions and populations as many acute care hospitals often "hold" patients with complex, chronic illness as in-patients for extended periods because alternate appropriate services may not exist or be available. Our case study highlights three key areas to improve quality of care for patients with extreme LOS: alternate levels of care, system resources and transitions to home.

Novel Leg Cannula for Venous Decompression in Peripheral Extracorporeal Membrane Oxygenation.

A chimney femoral artery graft for peripheral extracorporeal membrane oxygenation can potentially cause hyperperfusion and subsequent venous congestion in the ipsilateral leg, especially in the context of septic shock and higher flow requirement. This report describes a novel technique to use an additional leg venous cannula to avoid leg congestion as well as to achieve higher total flow.

Development of pontine noradrenergic A5 neurons requires brain-derived neurotrophic factor.

Pontine noradrenergic A5 neurons play a pivotal role in maturation and regulation of the brainstem respiratory rhythm-generating network. Analysis of newborn brain-derived neurotrophic factor (BDNF)-null mice revealed a marked loss of tyrosine hydroxylase-positive A5 neurons compared to wildtype controls that was rescued by null mutation of the proapoptotic gene Bax. In cultures of the A5 region from E12.5 rat embryos, BDNF significantly increased the number and branching of tyrosine hydroxylase-positive neurons. Immunoneutralization of endogenous glial cell line-derived neurotrophic factor partially inhibited the BDNF-dependent increase in the number of tyrosine hydroxylase-positive cells without affecting neurite number. The A5 nucleus is the first brainstem cell group identified at which BDNF is required in vivo for development of neurons critical for cardiorespiratory control.

Delay in diagnosis of Williams syndrome.

Williams syndrome (WS) is a well-known genetic disorder with a variable phenotype. In many cases, physical manifestations are subtle and may not be apparent at an early age, making diagnosis difficult in infants and young children who lack classic manifestations such as supravalvular aortic stenosis and hypercalcemia. Clinical suspicion is essential because the diagnostic genetic finding is not detectable on routine chromosomal analysis. Furthermore, early diagnosis allows for earlier detection and treatment of developmental, behavioral, and medical problems. In an effort to understand how and why individuals with WS are diagnosed, we conducted a survey-based study of parents of WS children. Packets containing a cover letter, consent form, parental survey and preaddressed stamped envelope were distributed to parents of children with WS. The survey included questions concerning initial diagnosis, WS findings present, medical specialists involved, and tests performed. Forty-six completed surveys were returned for analysis. The mean age at diagnosis was 3.66 years (SD 4.13). The mean age at which there were initial concerns was 0.98 year (SD 1.24) resulting in a mean delay in diagnosis of 2.77 years (SD 4.10). In addition, the involvement of a geneticists correlated with earlier diagnosis (2.26 years vs. 5.09 years without geneticist involvement, p = 0.03) and fewer tests ordered (5.2 vs. 8.2 in the nongeneticist group, p = 0.0006). We observed a significant delay in the diagnosis of WS. Of note, the involvement of a geneticist was associated with earlier diagnosis and reduced number of tests.