Infantile fibrosarcoma (IFS) is a rare pediatric cancer typically presenting in the first 2 years of life. Surgical resection usually curative and chemotherapy active against gross residual disease. However, when recurrences occur, therapeutic options are limited. We report case refractory IFS with constitutive activation tropomyosin-related kinase (TRK) signaling pathway from an ETS variant gene 6-neurotrophin 3 receptor (ETV6-NTRK3) fusion. The patient enrolled Phase 1 trial LOXO-101,...

Objectives/Hypothesis: To compare efficacy and outcome of daily saline irrigation versus saline/gentamicin for treating chronic rhinosinusitis (CRS). Study Design: Prospective, randomized, double-blinded study. Methods: Forty children diagnosed with CRS were enrolled. Patients randomized to once-daily or 6 weeks. Treatment outcomes measured using 1) Lund-Mackay scoring system pre- post-treatment computer tomography (CT); 2) Sinonasal Quality-of-Life Survey (SN-5) completed at baseline, after...

Exposure to fine particulate matter (PM2.5) during wildfire seasons has been associated with adverse health outcomes. Previous studies have focused on daily exposure, but PM2.5 levels in smoke events can vary considerably within 1 d.We aimed assess the immediate and lagged relationship between sub-daily exposure acute outcomes British Columbia.We used a time-stratified case-crossover study design evaluate association modeled hourly ambulance dispatches from 2010 2015. Distributed lag...

A 13-year retrospective study was undertaken to determine the incidence of posttonsillectomy hemorrhage, evaluate potential risk factors, and assess efficacy safety ambulatory tonsillectomy.From January 1985 December 1997, 4662 patients underwent tonsillectomy at our institution. Ninety with bleeding were identified. For each patient bleeding, 2 nonbleeding control subjects selected matched by age sex factors.Age only factor found be statistically significant among group. The highest (3.61%)...

Abstract Objectives To review pseudotumor of infancy (POI) and congenital muscular torticollis (CMT) to suggest an algorithm for treatment. Study Design Retrospective cases from 1962 1998 at a tertiary care center. Methods Patients included in this study were 81 boys 89 girls who had diagnosis POI (n = 38) or CMT 132) before 24 months age. Results For all patients, the mean age was 4 months; 54.1% left side neck affected, over 90% head tilt, 2.4% feeding difficulty as result disorder....

To determine changes in behavior and sleep children before after adenotonsillectomy for sleep-disordered breathing (SDB) using the validated Pediatric Sleep Questionnaire (PSQ) Conners' Parent Rating Scale-Revised Short Form (CPRS-RS).Prospective, nonrandomized study.Ambulatory surgery center affiliated with an academic medical center.A total of 117 consecutive (61 boys 56 girls) (mean [SD] age, 6.5 [3.1] years) who were clinically diagnosed as having SDB had undergone adenotonsillectomy....

Abstract This is the first scientific report of hereditary hemorrhagic telangiectasia (HHT) epistaxis treatment by intranasal spraying vascular endothelial growth factor (VEGF) inhibitor bevacizumab (Avastin). Epistaxis in patients with HHT a morbid, mortal condition that difficult and unpleasant to manage. Nasal modulated VEGF, which elevated patients. Bevacizumab VEGF diminishes when administered intravenously or injected locally, as reported here sprayed topically onto nasal mucosa....

The diagnosis of vocal fold paralysis in an infant is a devastating finding that may require permanent tracheotomy. incidence congenital unknown, but it thought to be more common infants with anatomic anomalies the aero-digestive system. Vocal after surgical repair esophageal atresia and tracheoesophageal fistula rare often diagnosed multiple failed extubations. Currently do not routinely undergo documentation motion prior repair. We report here on our experience this complication. A...

Large amounts of waste in hospitals are generated the operating rooms from disposable surgical supplies. Tonsillectomy/adenotonsillectomy (T&A) cases use many supplies that not recyclable. It is critical to reduce waste, as such directly affects environment and increases health care costs. The authors noticed a difference between number items prepared, available, but almost never used, for each tonsillectomy case children’s hospital setting university ambulatory surgery center setting....

To determine the incidence of vocal cord (VC) paralysis and dysphagia after aortic arch reconstruction, including Norwood procedure.Tertiary children's hospital.Retrospective cohort.Database/chart review neonates requiring or surgery between January 2005 December 2012. Demographics, postoperative VC function, dysphagia, need for gastrostomy tube and/or tracheotomy, long-term follow-up were reviewed.One hundred fifty-one consecutive subjects (96 Norwood, 55 arch) reviewed. Median age at...

Abstract Hypothesis Pharyngeal inflammatory pain is reduced by a single dose of dexamethasone. Study Design Prospective, randomized, double‐blinded, placebo‐controlled study. Methods From August 1998 to July 2000, total 118 patients were enrolled. We compared placebo (n = 37), 10‐mg intramuscular injection dexamethasone 39), and oral 42). All given antibiotics had bacterial throat cultures. Results Complete telephone follow‐up 12 hours after treatment was available in 111 patients, 24‐hour...

Chronic rhinosinusitis(CRS) results in significant morbidity and health care expenditure. Safety efficacy of nasal irrigation use the treatment pediatric CRS have been demonstrated, but long-term outcomes are unknown. We reviewed characteristics after 6 weeks once daily based on computed tomography (CT) scans, summarized parental reports subsequent for recurring symptomsRetrospective cohort study cross-sectional survey.Review survey 144 patients diagnosed between July 2003 January 2012.One...

To evaluate the experience of a tertiary care children's hospital in diagnosis, treatment, and long-term outcomes patients with primary head neck Langerhans' cell histiocytosis.A retrospective study who presented histiocytosis that primarily affected sites between January 1, 1986, December 31, 2004, at Children's Mercy Hospital.Twenty-two were diagnosed treated for LCH our during this time period. Seventeen (77%) had involvement; 14 (64%) these LCH. Lesions are complicated involve multiple...

Objective To report treatment outcomes of nontuberculous mycobacteria (NTM) cervicofacial lymphadenitis. Study Design A retrospective study from 1995 to 2006. Subjects and Methods Fifty‐one patients (mean age, 30.3 months). Results All but one patient presented with lymphadenopathy. The mean time symptoms diagnosis was 42.3 days. Tuberculin skin testing (TST) performed on 21 51 positive 14 patients. Imaging ordered in 23; 19 had CT scans. Pathology confirmed caseating granuloma 45 (88%);...

Chronic nasal congestion often persists in children despite empirical treatment using intranasal corticosteroids, systemic antihistamines, and/or leukotriene receptor antagonists. Symptoms are reported even with negative results of skin or blood allergy testing. Inferior turbinoplasty has been effective adults and children, but outfracture inferior turbinates is rarely reported, as use validated quality-of-life measures to quantify improvements after intervention. Effective these 2...

Emergence delirium (ED) is a state of aggressive agitation that can occur temporarily in the process emerging from anesthesia children exposed to volatile or intravenous anesthetics. typically assessed using published and validated Pediatric Delirium (PAED) Scale. Due some variation properties between sevoflurane desflurane for maintenance after standard induction, we designed prospective study examine potential differences emergence behavior incidence ED undergoing elective ear-nose-throat...

To characterize peripheral eosinophil migration in patients with chronic rhinosinusitis the presence of nasal mucin and tissue extracts.Prospective, controlled, ex-vivo.Peripheral blood eosinophils, mucin, were harvested at time sinus surgery 10 patients, as well obtained healthy control subjects. Extracts prepared from tissue. A modified Boyden chamber was used to study both subjects extracts.Patients all demonstrated a concentration-dependent increased eosinophils extracts. The percentage...

To identify risk factors predisposing to postextubation complications and the incidence of subglottic stenosis following endotracheal intubation for bronchiolitis.A review 144 consecutive infants children intubated bronchiolitis between 2000 2005 at a regional children's hospital.The mean age diagnosis was 6.4 months. Follow-up data were available in 93 patients (64.6%), average length follow-up 9.3 One hundred three (71.5%) had positive RSV detection. Average duration 5.5 days. Twenty-six...

The objective of this study was to review our pediatric emergency department's (ED's) utilization computed tomography (CT) in the diagnosis peritonsillar abscess (PTA) and treatment outcomes.This used case series with chart review.From January 2007 2009, 148 patients were seen ED for possible PTA. Mean age at presentation 11.8 years (range, 10 months 18 years); 81 (54.7%) females. Computed ordered 96 (64.9%) patients, which 73 (49.3%) confirmed have who underwent CT younger when compared...