A clinical antiracism curriculum for third-year medical students to bring antiracist principles to the bedside.

As medical educators, we have a responsibility to ensure our trainees are exposed to curricula dedicated to Diversity, Equity, and Inclusion (DEI), as illustrated by the Association of American Medical Colleges' recently released DEI Competencies Across the Curriculum. We designed and implemented a curriculum, Social Justice Rounds (SJR), that incorporates teaching on these topics directly into inpatient clinical work. SJR are brief team-based discussions facilitated by Pediatric Hospital Medicine faculty that focus on racism in medicine and other forms of discrimination experienced by patients and the effect it has on their interaction with the healthcare system. Medical students rotating through the Pediatrics Clerkship completed optional pre- and postclerkship surveys, which revealed statistically significant increases in students' frequency and comfort with conversations regarding DEI topics, both with the medical team and with patients. We believe that SJR provides a framework by which educators across specialties and institutions can provide trainees with foundational DEI skills.

Healthy transition: Roadmap for young adults with Down syndrome to adulthood.

Healthcare transition is the purposeful and planned process for preparing young adults with Down syndrome for an adult oriented healthcare system. Significant gaps of a delayed, incomplete, siloed and decentered transition can be avoided when transition is approached in a longitudinal and holistic manner. Young adults with Down syndrome are specifically vulnerable to these gaps as the combination of intellectual differences and healthcare complexity leads to the need for a process that allows for appropriate preparation to develop the skills and process for an appropriate. To establish a successful transition care plan, the six core elements of policy, tracking, readiness, planning, transfer of care, and complete transition will compose the scaffolding of the transition process and address these gaps in care. A comprehensive tool kit including policy statements, healthcare transition tracking forms, the TRAQ tool, and template portable medical summaries will operationalize those elements and counteract any gaps in the transition process.

Operative Surveillance of Airway Hemangiomas in PHACE Syndrome.

OBJECTIVE: PHACE is a rare syndrome that can present with airway hemangiomas. Management for these patients is variable and the utilization of operative endoscopic airway evaluation has not been described. The objectives of this study were to identify the incidence of airway symptoms in patients being evaluated for PHACE syndrome and determine the utility of operative endoscopy. METHODS: An IRB-approved retrospective cohort study was conducted on consecutive pediatric patients with head and neck infantile hemangioma (IH) evaluated in a multi-disciplinary vascular anomalies center between 2013 and 2019. Patients were included if they were being worked up for PHACE syndrome and had an otolaryngology evaluation. Demographics, clinical, and surgical variables were collected. RESULTS: There were 317 patients with head and neck IH. Thirty-six patients met inclusion criteria. The majority of patients were female (31/36; 86.1%) and less than half of the patients (15/36; 41.7%) were eventually diagnosed with PHACE syndrome. Median age at presentation was 2 months (range 0-82 months). A total of 28/36 (77.8%) of patients were managed with propranolol. The majority of the patients presented without aerodigestive symptoms; however, 16/36 (44.4%) of patients presented with symptoms such as stridor, hoarseness, and dysphagia. A total of 20/36 (55.6%) of patients underwent operative endoscopy. A total of 8/20 (40.0%) of patients who underwent operative endoscopy had operative intervention. Of the entire cohort, only 2/15 (13.3%) patients diagnosed with PHACE were found to have a subglottic hemangioma. Both patients presented with stridor. CONCLUSION: Operative endoscopy remains useful in the workup of PHACE syndrome to identify subglottic hemangiomas, however there may be relatively low yield in asymptomatic patients. In office flexible laryngoscopy may be a less invasive means to examine the subglottic region. A multi-center prospective study would be necessary to evaluate incidence of subglottic hemangiomas in asymptomatic patients evaluated for PHACE.

Cardiometabolic profiles in children and adults with overweight and obesity and down syndrome.

Individuals with Down syndrome (DS) are at increased risk for being overweight/obese, but the associated cardiometabolic risk (CR) is not clear. Cross-sectional anthropometric and clinical laboratory data from a multi-site, international cohort of individuals with DS were analyzed to determine cardiometabolic risk by reporting observed distributions of cardiometabolic biomarkers in overweight/obese individuals with DS throughout the lifespan. Descriptive statistics and regression analyses by age categories determined the distributive percentiles for cardiometabolic biomarkers and tested for adiposity as a predictor of CR. Across seven DS clinics, data were collected on 240 patients between the ages of 3 and 63 years, with one quarter overweight and three quarters obese among children and nearly all adults being obese. In children and adults, most cardiometabolic biomarker profiles showed distributive values within normal ranges. Blood lipids were positively associated with body mass index (BMI) in children (high density lipid-cholesterol, p = 0.01; low density lipid-cholesterol, p = 0.02). Levels of hs-CRP were elevated in both children and adults, with BMI positively associated with hs-CRP in adults with DS (p = 0.04). Liver enzyme values were positively associated with BMI in children and adults. The data suggest that in contrast to the general population, in individuals with Down syndrome, being overweight and obese does not appear to confer a significantly increased risk for cardiometabolic disease by biomarker profile. Individuals with DS who are overweight/obese appear to have unique cardiometabolic profiles unrelated to adiposity, notable for increased hs-CRP and normal HA1c levels.

Provider Educational Needs and Preferences Regarding Transition from Pediatric to Adult Care in a Pediatric Health System.

INTRODUCTION: There is limited evidence on educational needs and preferences of pediatric health care professionals regarding transition from pediatric to adult health care. METHODS: We distributed an anonymous online survey to physicians and advanced practice providers at a large, free-standing children's hospital and associated primary care network to assess attitudes, practices, and educational preferences about transition. We analyzed data with descriptive statistics, chi-square, and logistic regression. RESULTS: A total of 178 providers responded (20% response rate) across 31 specialties (66% attending physicians, 19% fellows, and 15% advanced practice providers). Less than half (43%) were comfortable in their knowledge of transition, with fellows reporting significantly lower comfort than attendings ( P < .05). In total, 47% reported annually discussing transition with their patients (fellows: 17% vs. attendings: 50%, P < .05). Educational topics of greatest interest included legal (71%) and financial concerns (69%), whereas preferred educational formats included Grand Rounds/lectures (68%) and webinars (63%). Facilitators to transition included lists of local adult providers for transition (89%), parent/family educational resources (88%), and a medical summary template in the electronic health record (85%). DISCUSSION: Major gaps exist in attitudes and practices related to transition. Identified educational strategies may serve as targets to improve the transition process through educational interventions.

Assessment and Diagnosis of Down Syndrome Regression Disorder: International Expert Consensus.

Objective: To develop standardization for nomenclature, diagnostic work up and diagnostic criteria for cases of neurocognitive regression in Down syndrome. Background: There are no consensus criteria for the evaluation or diagnosis of neurocognitive regression in persons with Down syndrome. As such, previously published data on this condition is relegated to smaller case series with heterogenous data sets. Lack of standardized assessment tools has slowed research in this clinical area. Methods: The authors performed a two-round traditional Delphi method survey of an international group of clinicians with experience in treating Down syndrome to develop a standardized approach to clinical care and research in this area. Thirty-eight potential panelists who had either previously published on neurocognitive regression in Down syndrome or were involved in national or international working groups on this condition were invited to participate. In total, 27 panelists (71%) represented nine medical specialties and six different countries reached agreement on preliminary standards in this disease area. Moderators developed a proposed nomenclature, diagnostic work up and diagnostic criteria based on previously published reports of regression in persons with Down syndrome. Results: During the first round of survey, agreement on nomenclature for the condition was reached with 78% of panelists agreeing to use the term Down Syndrome Regression Disorder (DSRD). Agreement on diagnostic work up and diagnostic criteria was not reach on the first round due to low agreement amongst panelists with regards to the need for neurodiagnostic testing. Following incorporation of panelist feedback, diagnostic criteria were agreed upon (96% agreement on neuroimaging, 100% agreement on bloodwork, 88% agreement on lumbar puncture, 100% agreement on urine studies, and 96% agreement on "other" studies) as were diagnostic criteria (96% agreement). Conclusions: The authors present international consensus agreement on the nomenclature, diagnostic work up, and diagnostic criteria for DSRD, providing an initial practical framework that can advance both research and clinical practices for this condition.

Unexplained regression in Down syndrome: Management of 51 patients in an international patient database.

Research to guide clinicians in the management of the devastating regression which can affect adolescents and young adults with Down syndrome is limited. A multi-site, international, longitudinal cohort of individuals with a clinical diagnosis of Unexplained Regression in Down syndrome (URDS) was collated through seven Down syndrome clinics. Tiered medical evaluation, a 28-item core symptom list, and interim management are described naturalistically. Improvement-defined by the percentage of baseline function on a Parent-reported Functional Score, overall improvement in symptoms on a Clinician-administered Functional Assessment, or report of management type being associated with improvement-was analyzed. Improvement rates using ECT, IVIG, and others were compared. Across seven clinics, 51 patients with URDS had regression at age 17.6 years, on average, and showed an average 14.1 out of 28 symptoms. Longitudinal improvement in function was achieved in many patients and the medical management, types of treatment, and their impact on function are described. Management with intravenous immunoglobulin (IVIG) was significantly associated with higher rate of improvement in symptoms at the next visit (p = 0.001). Our longitudinal data demonstrates that URDS is treatable, with various forms of clinical management and has a variable course. The data suggests that IVIG may be an effective treatment in some individuals. Our description of the management approaches used in this cohort lays the groundwork for future research, such as development of standardized objective outcome measure and creation of a clinical practice guideline for URDS.

Non-involuting congenital hemangioma with delayed hypertrophy: A case series.

BACKGROUND: Noninvoluting congenital hemangiomas (NICH) are rare and poorly understood vascular tumors that are present at birth, characterized by lack of growth after birth and lack of involution. We report uncharacteristic cases of NICH hypertrophy occurring later in life. METHODS: This is a case series describing the clinical presentation, management, and histologic characteristics of two cases of NICH hypertrophy. RESULTS: Two patients with a NICH of the scalp experienced lesion hypertrophy in teenage or early adult life. Case 1 is a 14-year-old female who presented with a flat left parietal scalp lesion that at first grew slowly with the patient; however, over the span of months grew substantially resulting in an exophytic lesion. The patient had the lesion surgically excised. Case 2 is a 26-year-old female with NICH of left occipital scalp and posterior neck who noted new nodules on the inferior border of the lesion. MRA/MRI showed extension into the occipital calvarium, level V of the neck, and paraspinal musculature. The patient elected to observe given the extent of the lesion and her minimal symptoms. CONCLUSION: Although postnatal growth of NICH have been described, cases usually occur during the pre-adolescent period where growth is usually proportional to overall growth of the patient. This study describes two cases of rapid onset NICH hypertrophy occurring later in life. Knowledge of the potential for delayed hypertrophy may lead families to seek earlier intervention or opt for more definitive interventions. Additionally, recognition of these variable distinctions will contribute to a better understanding of CH and its various subtypes.

Design and rationale of a clinical trial to increase cardiomyocyte division in infants with tetralogy of Fallot.

BACKGROUND: Patients with Tetralogy of Fallot with pulmonary stenosis (ToF/PS), the most common form of cyanotic congenital heart disease (CHD), develop adverse right ventricular (RV) remodeling, leading to late heart failure and arrhythmia. We recently demonstrated that overactive ß-adrenergic receptor signaling inhibits cardiomyocyte division in ToF/PS infants, providing a conceptual basis for the hypothesis that treatment with the ß-adrenergic receptor blocker, propranolol, early in life would increase cardiomyocyte division. No data are available in ToF/PS infants on the efficacy of propranolol as a possible novel therapeutic option to increase cardiomyocyte division and potentially reduce adverse RV remodeling. METHODS: Using a randomized, double-blind, placebo-controlled trial, we will evaluate the effect of propranolol administration on reactivating cardiomyocyte proliferation to prevent adverse RV remodeling in 40 infants with ToF/PS. Propranolol administration (1 mg/kg po QID) will begin at 1 month of age and last until surgical repair. The primary endpoint is cardiomyocyte division, quantified after 15N-thymidine administration with Multi-isotope Imaging Mass Spectrometry (MIMS) analysis of resected myocardial specimens. The secondary endpoints are changes in RV myocardial and cardiomyocyte hypertrophy. CONCLUSION: This trial will be the first study in humans to assess whether cardiomyocyte proliferation can be pharmacologically increased. If successful, the results could introduce a paradigm shift in the management of patients with ToF/PS from a purely surgical approach, to synergistic medical and surgical management. It will provide the basis for future multi-center randomized controlled trials of propranolol administration in infants with ToF/PS and other types of CHD with RV hypertension. CLINICAL TRIAL REGISTRATION: The trial protocol was registered at clinicaltrials.gov (NCT04713657).

Repeat tympanostomy tubes in children with Down syndrome.

OBJECTIVES: Children with Down syndrome (DS) have a higher incidence of tympanostomy tube insertion (TTI) than children in the general population. As there were no studies investigating factors that are associated with multiple TTIs in children with DS, we sought to determine what factors increase or decrease the likelihood of repeat TTI in children with DS. METHODS: A retrospective case-control study was performed on consecutive children with DS from 2007 to 2018 with first TTI at a large tertiary children's hospital and follow-up duration at least 27 months since first TTI. RESULTS: 277 patients met the inclusion criteria. Repeat TTI rate was 61.4%. Having an indication of chronic otitis media with effusion (COME) at first TTI was an adjusted risk factor for increased rate of repeat TTI (OR: 2.01, 95%CI: 1.15-3.51, p = .014), while being older at first TTI was an adjusted protective factor for decreased rate of repeat TTI (OR: 0.84, 95%CI: 0.74-0.95, p = .004). Adenotonsillectomy at or before first TTI was not an adjusted protective factor for decreased rate of repeat TTI (OR: 0.915, 95%CI: 0.448-1.872, p = .809) and bilateral intra-operative fluid was not an adjusted risk factor for repeat TTI (OR: 1.97, 95%CI: 0.99-3.90, p = .054). CONCLUSION: Children with DS were more likely to undergo repeat TTI if they were of younger age and if the indication for surgery was COME. The repeat TTI rate for children with DS was high at 61.4%. Prospective studies are warranted to more precisely investigate factors associated with repeat TTIs in this unique patient population.

Restrictive Eating Disorders: Accelerating Treatment Outcomes in the Medical Hospital.

Pediatric patients with anorexia nervosa and atypical anorexia nervosa may present to hospitals with significant vital sign instability or serum laboratory abnormalities necessitating inpatient medical hospitalization. These patients require specialized care, numerous resources, and interdisciplinary collaboration during what can be a protracted admission. Recent evidence informs areas in which care can be accelerated, and published protocols from major children's hospitals are helpful roadmaps to creating a streamlined hospitalization. In our narrative review, we focused on 3 key areas: (1) implementation of a rapid nutritional rehabilitation program; (2) assessment and management of the refeeding syndrome; and (3) early integration of psychoeducation and therapeutic interventions during inpatient hospitalization. A practical review of the literature in these 3 areas will give concrete, actionable information to pediatric hospitalists as they care for young people with restrictive eating disorders.

Health care transition for individuals with Down syndrome: A needs assessment.

Transition to adulthood is a multifaceted process that requires integration of numerous domains within a young person's life, including their health care. For children with special health care needs, the transition process can be markedly more difficult to navigate. This is especially true for children with Down syndrome (DS) who receive fewer transition planning services. The aim of this needs assessment was to identify current trends, potential gaps, and areas for targeted intervention within the current transition landscape for individuals with DS. We utilized DS-Connect, a National Institutes of Health (NIH) funded family/self-advocate survey repository, as a platform to recruit respondents to the "Transition to Adulthood" survey. Sixty-five respondents (64 parents and 1 caregiver) completed the survey. Responses indicated that 42% of respondents reported comfort in the transition process, but 5% or less reported completing one of the core transition outcomes: transition readiness assessment, portable medical summary, or written transition plan. These findings translated across other domains of respondents' lives. While many individuals with DS and their caregivers are aware of the transition process, there appears to be a disconnect between the introduction of transition concepts and an actualization of transition outcomes necessary for success.

Peri-procedural Anticoagulation in Patients with Head and Neck Versus Extremity Venous Malformations.

OBJECTIVE: (1) Review a multidisciplinary vascular anomalies center's practice regarding periprocedural anticoagulation for venous malformations (VM) and the associated risk of thromboembolic and disseminated intravascular coagulation (DIC) events. (2) Compare the risk of thromboembolic events and DIC post-procedure between head and neck (H&N) and extremity VM patients. METHODS: An Institutional Review Board (IRB)-approved, retrospective chart review was performed on 120 VM patients. A thromboembolic event was defined as a thrombus formation post-sclerotherapy or post-surgery within 2 months in a distant or local venous structure not directly addressed by the procedure. RESULTS: There were 39 cases involving the H&N and 81 cases based at the extremities. There were eight cases of post-procedure thrombus formation within the extremity VM group (8/71; 11.3%) as opposed to 0 cases in the H&N group (OR: 0, 95% CI .00-.09), p = .049. There was no difference in incidence of post-procedure thromboembolic events between those with elevated D-dimer (H&N: 0%, extremity: 22.7%, 5/22) and normal D-dimer values (H&N: 0%, extremity: 6.3% [1/16], P = .370). There was no difference in incidence of post-procedure thromboembolic events between those who received periprocedural anticoagulation (H&N: 0%, extremity: 21%, 4/19) and those who did not (H&N: 0%, extremity: 8.2%, 4/49), (Extremity: OR: 3.00, .67-13.50, P = .206). CONCLUSION: Post-procedure thromboembolism is rare in the treatment of venous malformations, especially in the head and neck subsite. Regardless of anticoagulation use, there were no thromboembolic events for H&N VM patients. Such events are rare, and the odds may approach zero, especially with small sample size. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:1163-1167, 2021.

Learning by Doing: Design and Evaluation of a Quality Improvement Curriculum for Pediatric Hospitalists.

Quality improvement (QI) is a core competency for Pediatric Hospital Medicine (PHM) and required for maintenance of certification, but many hospitalists lack QI training. This project set out to increase a PHM faculty's QI knowledge and comfort participating in QI projects, while concurrently applying the skills learned to a QI project in the hospital. METHODS: We designed a 4-session curriculum utilizing principles of adult learning. Faculty immediately applied learned concepts to a QI project to increase the percentage of patients who were seen by an attending and billed for on the same day as admission to the PHM service. Attitudinal data and scores on the validated Quality Improvement Knowledge Application Tool- Revised knowledge assessment were compared precurriculum and postcurriculum. A manifest content analysis was carried out for qualitative questions. RESULTS: Twenty faculty (83%) completed the preassessment; 15 (63%) completed the postassessment. Respondents showed statistically significant improvements in their perceived ability to participate in QI projects and their Quality Improvement Knowledge Application Tool- Revised scores. The group completed a QI project that increased revenue for the division. Faculty appreciated that the curriculum was applied to a real QI project and felt they would use the new skills in their daily practice. DISCUSSION: This curricular model based on adult learning theory, with immediate application to a real QI project, conclusively showed attitudinal, knowledge-based, and hospital system-level improvements, and was well received by faculty.

Contrast-Enhanced Ultrasound of Congenital and Infantile Hemangiomas: Preliminary Results From a Case Series.

OBJECTIVE. The contrast-enhanced ultrasound (CEUS) imaging features of hepatic vascular tumors in infants, including infantile hemangioma (IH) and congenital hemangioma (CH), are not well reported. Frequent inaccurate use of lesion terminology in the literature has created diagnostic confusion. The purpose of this study is to describe the CEUS features of IH and CH. MATERIALS AND METHODS. Ten patients, ranging in age from 8 days to 16 months, with hepatic vascular tumors were included for retrospective analysis. Gray-scale ultrasound, color Doppler ultrasound, and CEUS features were reviewed, and interobserver kappa coefficients were calculated. Final diagnoses were clinically determined by a pediatrician with expertise in vascular anomalies except in one patient who underwent surgical excision. RESULTS. Of the 10 patients, five had CHs and five had IHs. All 10 lesions were hyperenhancing in the early arterial phase. In the portal venous phase, four of five (80%) CHs showed hyperenhancement relative to normal liver parenchyma, whereas four of five (80%) IHs showed isoenhancement. In the late phase, washout of contrast material was seen in three of five (60%) IHs, whereas one IH remained isoenhancing and one IH was hyperenhancing. None of the CHs showed late washout. Interobserver kappa coefficients for CEUS features ranged from 0.60 to 1.00. CONCLUSION. Except for the CEUS feature portal venous phase enhancement (κ = 0.60), good to excellent (κ = 0.74-1.00) agreement about CEUS features of IHs and CHs was observed. A significant proportion of IHs (60%) showed washout at delayed phase imaging, which has also been reported with malignancies. Recognition of the overlap in imaging appearance of these two entities is vital to preventing misdiagnosis of malignancy.

Unexplained regression in Down syndrome: 35 cases from an international Down syndrome database.

PURPOSE: An entity of regression in Down syndrome (DS) exists that affects adolescents and young adults and differs from autism spectrum disorder and Alzheimer disease. METHODS: Since 2017, an international consortium of DS clinics assembled a database of patients with unexplained regression and age- and sex-matched controls. Standardized data on clinical symptoms and tiered medical evaluations were collected. Elements of the proposed definition of unexplained regression in DS were analyzed by paired comparisons between regression cases and matched controls. RESULTS: We identified 35 patients with DS and unexplained regression, with a mean age at regression of 17.5 years. Diagnostic features differed substantially between regression cases and matched controls (p < 0.001 for all but externalizing behaviors). Patients with regression had four times as many mental health concerns (p < 0.001), six times as many stressors (p < 0.001), and seven times as many depressive symptoms (p < 0.001). Tiered medical evaluation most often identified abnormalities in vitamin D 25-OH levels, polysomnograms, thyroid peroxidase antibodies, and celiac screens. Analysis of the subset of patients with nondiagnostic medical evaluations reinforced the proposed definition. CONCLUSIONS: Our case-control evidence supports a proposed definition of unexplained regression in Down syndrome. Establishing this clinical definition supports future research and investigation of an underlying mechanism.

Subglottic hemangioma: Understanding the association with facial segmental hemangioma in a beard distribution.

OBJECTIVE: A subglottic hemangioma (SGH) is a benign tumor of infancy that can cause severe obstruction of the airway. Infantile hemangiomas, in general, are the most common head and neck tumor in children, affecting 4-5% of the pediatric population. This retrospective cohort study characterizes subglottic infantile hemangiomas at a single vascular anomaly center over a 5-year period (2013-2017) during the era of propranolol treatment. METHODS: Queried the Vascular Anomaly Database at Children's Hospital of Pittsburgh for all infantile hemangioma(s) and then identified case of subglottic hemangiomas. Characterized key features of presentation, natural history and management for subglottic hemangiomas. A secondary differentiation focused on differences between subglottic hemangiomas associated with Beard Distribution (BD) vs not (NBD). RESULTS: Analysis of 761 cases of infantile hemangiomas demonstrated only 13 patients with subglottic hemangiomas (1.7%). Of those 13 patients, only 4 patients (30%) had BD while 2 patients (15%) had other cutaneous hemangiomas and 7 patients (55%) had no cutaneous hemangiomas. Secondarily, a total of 31 case of beard distribution cutaneous hemangiomas with 11 patients having oropharyngeal involvement (35%) but only 4 patients with subglottic hemangiomas (13%). Interestingly, 2 of the 4 BD patients had treatment failure on propranolol and required second line treatment with steroids or surgical excision while only 1 of 9 NBD patients failed propranolol treatment. As well the same 2 BD patients which failed propranolol also had PHACES syndrome. CONCLUSION: Subglottic hemangiomas are a rare presentation of infantile hemangiomas but with significant morbidity. While the classic teaching that a segmental beard distribution hemangioma raises concern for a subglottic hemangioma, this cohort indicates subglottic hemangiomas occur in a NBD presentation (1.3%), and demonstrated only an approximate 10% incidence rate with a beard distribution. But more importantly, this study raises the question that beard distribution in setting of PHACES syndrome may herald a more recalcitrant and complicated natural history for a subglottic hemangioma. This is of significant concern as risk for CVA in setting of PHACES is highest with use of steroid treatment. None of our patients had high risk extra or intra cranial vascular arterial anomalies and no CVA were noted.