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Five Key Points • Subglottic stenosis (SGS) is the commonest tracheobronchial manifestation of granulomatosis with polyangiitis (GPA), with early recognition and treatment key to preventing its vasculitic progression and fibrosis. • Previous studies have shown SGS to be the first feature of GPA in 4% of cases. It is not uncommon to see negative biochemical (10% ANCA negative) and negative histological biopsies (77% of head and neck specimens are negative). • Our management strategy emphasized rapid SGS-GPA treatment with limited surgical manipulation of the airway and systemic immunosuppression (IS) to prevent evolution of SGS & concurrent systemic vasculitic relapse. • In our study early multi-disciplinary team involvement to deliver induction IS in the presence of active SGS-GPA led to a procedure free interval (PFI) of 31.3 months. This is a significant increase compared to other published studies. • Nineteen percent (4/21) of the cohort did not require any surgical input following induction IS.

We evaluated current trends of pediatric urology exposure during the 3-year pediatric residency period nationwide. We also evaluated the opinions of urology and pediatric residency program directors regarding the need for additional exposure to pediatric urology.From February to October 2011 we administered 2 concurrent electronic surveys. One set was sent to urology residency program directors and the other was sent to pediatric residency program directors. The surveys consisted of 6 and 12 questions, respectively. The questions were created to best evaluate exposure to pediatric urology from the perspective of each cohort. Response data were analyzed using the chi-square test and case-control methods.Of the 117 accredited urology residency program directors and 190 pediatric residency program directors 51 (43.5%) and 78 (41.1%), respectively, completed the survey. Urology program directors answered favorably by a margin of 66% toward increased involvement with pediatric residents, while 84.6% of pediatric residency directors would like increased exposure to pediatric urology. Furthermore, 87% of pediatric residency directors reported that they do not require residents to have a formal pediatric urology rotation. However, in 65% of pediatric programs residents received some form of didactic education.These results show the desire on the part of urology and pediatric residency program directors for pediatric residents to have greater exposure to pediatric urology, particularly didactic and bedside teaching in the management of pediatric urological disorders. Increasing pediatric resident exposure to pediatric urological pathology and treatment during training would have a positive impact on the subsequent diagnosis and care of pediatric urological conditions.

Multiple reports have shown that echocardiograms are neither cost-effective nor of high diagnostic yield for a number of indications. This study sought to evaluate the impact of indications and provider type on the diagnostic yield of first-time outpatient pediatric echocardiograms. All initial echocardiograms interpreted at our institution from February 2009 to December 2014 were reviewed retrospectively. Positive findings were defined as any abnormality of structure or function. Ordering physicians were grouped as Primary Care, Subspecialist, or Cardiologist. A cost analysis of cardiac consultation versus direct echocardiogram ordering was performed using 2014 Arkansas Medicaid office-based allowables. A total of 7854 echocardiograms had complete data and were included in the study. Median age was 7.2 years (range 2 days to 18.9 years). There were 1179 (15%) abnormal first-time echocardiograms. Diagnostic yields were particularly low for the indications of chest pain (4.9%), syncope (5.3%), and palpitations (9.1%). When ordered by the Cardiology group, echocardiographic yields were increased 35% for all indications (p

Background. Agitation and aggression are common behaviors that often lead to psychiatric emergency center (PEC) admission of nursing home patients with dementia or Alzheimer’s disease. However, few if any data are available that adequately describe characteristics and psychosocial triggers of agitation and aggression leading to transport and admission to a PEC. Methods. A preliminary investigation to explore all possible characteristics and psychosocial predictors of PEC transport and length of stay in men and women nursing home patients with dementia or Alzheimer’s disease was designed. Frequency distributions, chi-square, analyses of variance, and regression analyses were used to analyze the data. Results. One hundred PEC patient charts were reviewed, of which 58 charts were missing information and 42 charts provided evaluable data. Nursing homes located in impoverished areas transported patients to the PEC significantly more often than those in affluent areas. A disconnect between the agitated/aggressive mental state reported by nursing home staff leading to the PEC transport and the calm/cooperative mental status PEC clinicians observed during the admission process was evident. Data from the charts also showed that 74% of patients received off-label antipsychotics rather than FDA-approved medications to treat dementia or Alzheimer’s disease. Conclusions. This is one of the few studies to identify characteristics and psychosocial triggers of PEC use and length of stay in nursing home patients. We also highlight potentially dangerous antipsychotic use in dementia and Alzheimer disease. Thus, our data add to the existing knowledge base regarding PEC utilization, length of stay, and pharmacotherapy in nursing home patients with dementia and Alzheimer’s disease. Given the preliminary nature of this study, however, the results should be interpreted with caution.

Parents rely on pediatricians to monitor their child's development. The American Academy of Pediatrics recommends routine developmental screening with both broadband and autism-specific instruments at specified ages. If broadband screeners can detect autism risk, this might minimize the burden of administering autism-specific screens to all children. The current study examines the ability of the Ages and Stages Questionnaire-Third Edition (ASQ-3) to identify children at risk for autism. We looked at ASQ-3 scores of children who screen positive on the Modified Checklist for Autism in Toddlers-Revised (M-CHAT-R), children who continue to screen positive on the M-CHAT-R Follow-up Interview, and children diagnosed with autism spectrum disorder (ASD).A total of 2848 toddlers, aged 16 to 30 months, were screened with the ASQ-3 and M-CHAT-R across 20 pediatric sites. Children who screened positive on the M-CHAT-R and its follow-up interview were offered a diagnostic evaluation.Using the "monitor and/or fail" cutoff on any domain, the ASQ-3 identified 87% of the children who screened positive on the M-CHAT-R with follow-up and 95% (20/21) of those diagnosed with an ASD. Monitor and/or fail on the Communication domain alone also identified 95% of the diagnosed children.Scores below the "monitor" cutoff on the Communication domain of the ASQ-3 can indicate initial concern requiring autism-specific follow-up. If these results are confirmed with a sample large enough to separately examine toddlers of different ages and different cultural backgrounds, it may be feasible to implement a 2-stage screening strategy, with autism-specific screening reserved for those who are positive on a broadband screen.

Background The purpose of this study was to assess swallowing outcomes in a cohort of patients with oropharyngeal squamous cell carcinoma (SCC) undergoing nonoperative treatment. Methods We conducted a retrospective study of patients who completed videofluoroscopic swallowing studies (VFSS) after nonoperative treatment of oropharyngeal SCC. All patients received intensity-modulated radiation therapy (IMRT) ± chemotherapy. Swallowing abnormalities were recorded and the Penetration Aspiration Scale (PAS) quantified airway infiltration. Results Posttreatment VFSS (n = 71) occurred at an average of 4.69 months posttreatment. Abnormal PAS was noted in 45% of swallow studies. Swallowing decompensations included reduced pharyngeal constriction (75%), epiglottic tilt (70%), cricopharyngeal opening (42%), and hyoid excursion (42%). The only variable independently associated with abnormal PAS was pretreatment swallowing difficulty (odds ratio [OR] = 4.02; p = .009). Conclusion This study demonstrates that patients undergoing nonsurgical treatment for oropharyngeal SCC are at risk for posttreatment dysphagia. This suggests a need for dysphagia evaluation/management and refinement of interventions to minimize dysphagia. © 2014 Wiley Periodicals, Inc. Head Neck, 36: 1392–1397, 2014

Vicki A Nejtek, Sarah Hardy, Scott WinterUniversity of North Texas Health Science Center, Fort Worth, TX, USAAbstract: The leading cause of suicide ideation, attempts, and completion in adolescents is persistent and unresolved parental conflict. National statistics show extremely high rates of childhood neglect and abuse are perpetrated most often by single mothers. Psychiatric disorders arising from maternal–child dysfunction are well-documented. However, resources to prevent offspring victimization are lacking. Here, we report maternal neglect of a 15-year-old male brought to the psychiatric emergency room for suicidal ideation. An inpatient treatment plan including pharmacotherapy, family therapy and psychological testing was initiated. The patient’s mother failed to attend clinic appointments or family therapy sessions. Clinician attempts to engage the mother in the treatment plan was met with verbal assaults, aggression, and threatening behavior. The patient decompensated in relation to the mother’s actions. Child Protective Services were contacted and a follow-up assessment with the patient and mother is pending. Psychiatric treatment of the mother may be a necessary intervention and prevention regimen for both the adolescent and the mother. Without consistent Child Protective Services oversight, medical and psychosocial follow-up, the prognosis and quality of life for this adolescent is considered very poor. Stringent mental health law and institutional policies are needed to adequately intercede and protect adolescents with mental illness.Keywords: adolescent, suicide, maternal treatment noncompliance, maternal neglect

Apo A-1 Milano, an Arg173 to Cys point mutation of apo A-1, has demonstrated marked efficacy in the reduction of atherosclerotic lesions in animal models. A recent human trial testing recombinant apo A-1Milano infusions has similarly shown significant and rapid reduction in coronary atheroma volumes. In this project, we tested the utility of rAAV vectors encoding apo A-1 Milano for gene therapy of atherosclerosis in vivo following either intra-muscular injection or transplantation of rAAV-transduced bone marrow cells. ApoE-/- mice were injected with approximately 1012 vector genomes/kg at 6–8 weeks of age and placed on a high fat diet two weeks later. No major differences in body weight were observed between the treatment groups. 20–24 weeks after injection, the mice were harvested, aortas were cleaned, fixed, mounted, stained with oil red O and atherosclerotic plaque areas quantified. Control untreated mice or mice treated with an irrelevant rAAV vector showed marked atherosclerotic lesion formation. In contrast, the rAAV-2-apo A-1Milano and rAAV-5-apo A-1Milano injected groups showed significant reductions in plaque formation, 58% and 50% respectively, despite a lack of major differences in total plasma cholesterol levels. Since marrow-derived monocytes and macrophages play a pivotal role in atherogenesis, we tested the hypothesis that transplantation of rAAV apo A-1 Milano-transduced marrow cells would also result in a decline in plaque formation, possibly due to the localization of transduced macrophages within atherosclerotic lesions. Lethally irradiated Apo E-/- mice were transplanted with rAAV transduced cells, and high fat diet was initiated two weeks later. Twenty two to 24 weeks after transplantation, the mice were euthanized, and aortas were analyzed for atherosclerotic plaques. Untransplanted mice, and mice transplanted with untransduced and control rAAV transduced marrow served as negative controls. Wild type B6 to Apo E-/- transplants served as positive controls for plaque reduction. Results revealed a 41% and 47% reduction in atheroma formation following transplantation with cells transduced with rAAV-2 and rAAV5 vectors encoding apo A-1 Milano, respectively. These results suggest that either intramuscular injection of rAAV Apo A-1 Milano or transplantation of rAAV transduced marrow cells results in significant long-term inhibition of atherogenesis following a single treatment with an rAAV vector.