Study protocol: a pragmatic trial reviewing the effectiveness of the TransitionMate mobile application in supporting self-management and transition to adult healthcare services for young people with chronic illnesses.

BACKGROUND: Transition from paediatric to adult heath care services is a challenging time for many adolescents with chronic illnesses and may include deterioration in illness control as a consequence of inadequate self-management skills, poor understanding of their chronic illness and failure to engage with adult services. Successful transfer of health care requires the development of self-management skills and increased autonomy. Mobile technology has been proposed as a modality to assist this process. Evidence is limited and generally restricted to illness specific applications. The TransitionMate app (TMApp) is a generic (non-illness specific) mobile application designed to support young people with chronic illness in their transition from paediatric to adult health care services. The overall aim of the study is to assess the effectiveness of TMApp in improving engagement and retention of adolescents with chronic illness within adult healthcare services, as well as preventing the deterioration in illness control and unplanned hospitalisations. METHODS: The TransitionMate trial is a dual centre, pragmatic, single arm, mixed methods cohort study conducted within two university teaching tertiary paediatric hospitals in Australia. Data collection points are planned at 0, 6, 12 and 18 months. Outcome indicators include: usage of TransitionMate, engagement with adult services, quantitative markers of illness control, and unplanned hospital admissions. Data are collected through telephone interviews with the participants, their primary healthcare providers, electronic medical records and de-identified mobile application analytics. The development of the application involved co-design with recently transitioned young people with a number of chronic illnesses as well as online user experience in younger adolescents. DISCUSSION: The TransitionMate study is the first identified trial of a generic mobile application designed to support adolescents with chronic illnesses during the transition process. Results are expected to provide novel insights into the value of technological tools in the transition space, especially their effectiveness in improving both the transition process and clinical outcomes of adolescents with chronic illnesses. Furthermore, the approach of a pragmatic study design may help identify research methods better designed to overcome inherent challenges in research involving adolescents, transition of care and use of mobile application technology. TRIAL REGISTRATION: Registered retrospectively as of 30/1/2020 with Australian New Zealand Clinical Trials Registry: ACTRN12620000074998 .

Familial hypercholesterolaemia: Experience of a tertiary paediatric lipid clinic.

AIM: To review the experience of a dedicated paediatric multidisciplinary lipid clinic in the management of familial hypercholesterolaemia (FH) by studying the demographics, clinical presentations as well as statin therapy and outcomes. METHODS: Retrospective database review of all patients under 18 years old seen in the lipid clinic at an Australian tertiary paediatric hospital between April 1999 and August 2017. Outcome measures collected included patient demographics, family history, lipid profile, age at treatment commencement, treatment outcomes and complications. RESULTS: One hundred and eight patients (53 males) were seen in the lipid clinic. Eighty-five had low-density lipoprotein cholesterol (LDL-C) levels at or above the 75th percentile for sex prior to treatment. Of these, 75 had a first-degree relative with hypercholesterolaemia and/or early cardiac death. Four patients had clinical manifestations. Thirty-two patients (14 males) were started on statin therapy for likely FH. LDL-C levels reduced by 2.4 mmol/L (1.4 to 2.7) in boys and 1.9 mmol/L (0.8 to 2.8) in girls at 12 month follow-up. Five patients reported side effects requiring adjustment in therapy. Main reasons for not starting statin therapy in eligible patients were parental refusal and/or lost to follow up (77%). CONCLUSION: A dedicated multidisciplinary lipid clinic is helpful for streamlining and monitoring management of paediatric FH. Clinical manifestations of FH are rare in children and may represent more severe form of FH or other lipid disorder. Statin therapy is efficacious and well tolerated but current recommended targets of treatment are difficult to attain. Greater awareness and coordinated services are required to overcome poor family engagement.

Practice recommendations for addressing racism: A content analysis of the counseling psychology literature.

Although racism persists as a significant public health issue that adversely impacts the mental health of people of color (U.S. Department of Health and Human Service, 2001), there has been very little systematic guidance for mental health professionals to address racism through practice (S. Harrell, 2000). Therefore, we conducted a content analysis of the peer reviewed counseling psychology literature-the first of its kind-to provide a summary and critique of the extant practice recommendations and facilitate the development and enhancement of practice efforts aimed at addressing racism. We reviewed racism-related articles published in the Journal of Counseling Psychology, The Counseling Psychologist, and Counseling Psychology Quarterly and identified 73 relevant articles, of which 51 provided practice recommendations. Based on our review of this literature, we identified eight general categories of recommendations for addressing racism: psychoeducation, validation, self-awareness and critical consciousness, critical examination of privilege and racial attitudes, culturally responsive social support, developing positive identity, externalize/minimize self-blame, and outreach and advocacy. We found that most recommendations within each category were at the individual level with far fewer at the group and systemic level. A critique of recommendations is provided along with suggestions for developing and bolstering practice, research, and consultation efforts aimed at addressing racism. (PsycINFO Database Record (c) 2018 APA, all rights reserved).

The intersectionality framework and identity intersections in the Journal of Counseling Psychology and The Counseling Psychologist: A content analysis.

The framework of intersectionality is a powerful analytical tool for making sense of how interlocking systems of privilege and oppression are experienced by individuals and groups. Despite the long history of the concept, intersectionality has only recently gained attention in psychology. We conducted a content analysis to assess counseling psychology's engagement with an intersectional perspective. All articles published in the Journal of Counseling Psychology (n = 4,800) and The Counseling Psychologist (n = 1,915) from their first issues until July 2016 were reviewed to identify conceptual and empirical work focused on intersectionality. A total of 40 articles were identified and examined for themes. Limitations and future directions are discussed. (PsycINFO Database Record

Disposition to pediatric intensive care unit post supraglottoplasty repair: a systematic review.

BACKGROUND: Patients undergoing supraglottoplasty are often routinely admitted post-operatively to the pediatric intensive care unit (PICU) due to rare but potentially fatal complications such as airway compromise. A systematic review was performed to determine the rate of post-operative PICU-level respiratory support required by pediatric patients following supraglottoplasty, to identify risk factors for patients who may benefit from post-operative PICU admission and limit unnecessary use of intensivist resources. REVIEW METHODS: Key search terms 'supraglottoplasty' OR 'supraglottoplasties' were queried on three databases: CINHAL, Medline and Embase. Inclusion criteria were pediatric patients under 18 years of age who underwent a supraglottoplasty procedure with either an admission to PICU or requirement for PICU-level respiratory support. Risk of bias was assessed by two independent reviewers using QUADAS-2. Findings were critically appraised by three independent reviewers and pooled proportions of criteria meeting PICU admission were calculated for meta-analysis. RESULTS: Nine studies met inclusion criteria, totaling 922 patients. Age at time of surgery ranged from 19 days to 15.7 years with mean age of 5.65 months. A weighted pooled estimate suggested that 19% (95% CI 14-24%) of patients who underwent supraglottoplasty required PICU-admission. The included studies revealed several patient and surgical factors have been linked to postoperative respiratory issues requiring PICU admission, including: neurological disease, perioperative oxygen saturation < 95%, prolonged surgical time and age < 2 months. CONCLUSIONS: This study found that the majority of supraglottoplasty patients do not require significant postoperative respiratory support and suggests that routine PICU admission of these patients may be avoided by careful patient selection. Given the wide heterogeneity of outcome measures, further studies are needed to determine the ideal PICU admission criteria following supraglottoplasty.

Structural Integrity of Absorbable Gelatin Sponges for Middle Ear Packing in Otologic Surgery.

OBJECTIVE: This study investigates the physical properties upon immersion of two gelatin sponges commonly used in otologic surgery. BACKGROUND: Absorbable gelatin sponges are often used in middle ear surgery to achieve hemostasis and, perhaps more importantly, to provide a "scaffolding" to support ossicular chain and/or tympanic membrane reconstructions. Their rate of dissolution may therefore affect the success of tympanic membrane closure. METHODS: An in vitro study was conducted to quantify the material changes of two absorbable gelatin sponges, a standard-density sponge and one with fewer collagen cross-linkages (low-density sponge). Volume loss (%) in 0.9% saline, 0.3% ciprofloxacin, and/or 0.1% dexamethasone as single-agent otic drops in a combination formulation was measured at 15-minute intervals for the first hour and at days 1, 3, and 5 postimmersion. Secondary end points included compressibility, porosity under microscopy, and infrared spectroscopy analysis. RESULTS: The low-density sponge immersed in any of the three otic solutions showed a statistically significant greater volume loss at all time points when compared with the standard-density sponge (27.2% ± 5.4% vs. 15.4% ± 6.0% at 15 minutes and 44.8% ± 5.1% vs. 34.6% ± 2.9% at 5 days, p < 0.001). Interestingly, both sponges immersed in normal saline had lost almost half of their original volume after 15 minutes when compared with samples immersed in an otic solution (48.3% ± 4.6% vs. 21.3% ± 8.3%, respectively, p < 0.001). CONCLUSION: The standard-density sponge immersed in an otic solution of ciprofloxacin, dexamethasone, or a combination formulation best maintained its structural integrity. Ancillary in vivo studies are required to assess the hemostatic properties, surgical outcomes, and middle ear synechiae of the above study conditions. LEVEL OF EVIDENCE: Foundational evidence.

International Pediatric Otolaryngology Group: Consensus guidelines on the diagnosis and management of non-tuberculous mycobacterial cervicofacial lymphadenitis.

INTRODUCTION: Non-tuberculous mycobacterial (NTM) infection commonly manifests as subacute or chronic cervicofacial lymphadenitis in immunocompetent children. The optimal management of this pathology remains controversial. OBJECTIVES: This international consensus guideline aims to understand the practice patterns for NTM cervicofacial lymphadenitis and to address the primary diagnostic and management challenges. METHODS: A modified three-iterative Delphi method was used to establish expert recommendations on the diagnostic considerations, expectant or medical management, and operative considerations. The recommendations herein are derived from current expert consensus and critical review of the literature. SETTING: Multinational, multi-institutional, tertiary pediatric hospitals. RESULTS: Consensus recommendations include diagnostic work-up, goals of treatment and management options including surgery, prolonged antibiotic therapy and observation. CONCLUSION: The recommendations formulated in this International Pediatric Otolaryngology Group (IPOG) consensus statement on the diagnosis and management of patients with NTM lymphadenitis are aimed at improving patient care and promoting future hypothesis generation.

Procedural Sedation in Minor Procedure Rooms for Pediatric Myringotomy and Tympanostomy: A Quality Improvement Initiative.

OBJECTIVE: Lengthy wait times for elective surgery is a widespread health care system conundrum that may increase patient distress and jeopardize health outcomes. The primary aim of this quality improvement project was to reduce the surgical wait time in patients undergoing tympanostomy tube insertion. METHODS: As of January 2018, our tertiary care institution implemented a novel protocol whereby healthy children may undergo tympanostomy tube insertion in a minor procedure room under ketamine sedation administered by pediatric emergency physicians to address lack of both physical and anesthesia staffing resources. A retrospective study of all children undergoing elective tympanostomy tube insertion was conducted between September 1, 2017, and May 8, 2019, to assess wait time to surgery, as well as anesthesia-related and surgical complications. RESULTS: Procedural sedation in minor procedure rooms effectively decreased surgical wait times by 53 days (from 134 to 81 days, P < .001) at 16 months postimplementation. This new protocol was found to be safe and effective for healthy children, with no major surgical or anesthesia-related complications noted in 113 patients having undergone the procedure in the novel setting. DISCUSSION: Although conscious sedation by emergency physicians has been well studied across a variety of surgical procedures, its novel use in pediatric tympanostomy tube insertion requires careful patient selection to enhance accessibility while maintaining anesthetic safety. IMPLICATIONS FOR PRACTICE: This quality improvement project describes a novel combination of processes, using a minor procedure room space and ketamine-based procedural sedation to address surgical wait times in pediatric patients undergoing tympanostomy tube insertion.

Evaluating Strength of Evidence of Pediatric Otolaryngology Research Literature: A 20-Year Review.

OBJECTIVES/HYPOTHESIS: Quantity and quality of Otolaryngology-Head and Neck Surgery (OTL-HNS) research are increasing, yet patterns within Pediatric OTL-HNS publications are unknown. This study examines trends in the level of evidence of pediatric OTL-HNS articles over a 20-year period to quantify the growth and characterize contributing factors. STUDY DESIGN: Review article. METHODS: A retrospective review was conducted on 12 peer-reviewed OTL-HNS journals at three time-points: 1996, 2006, and 2016. Pediatric-specific OTL-HNS journals were selected; all were among the top 10 highest impact factor journals, with one pediatric-specific and one Canadian journal. Publication details, author characteristics, and study focus were collected. Papers were classified based on the Oxford Centre for Evidence-Based Medicine Levels of Evidence by two independent reviewers. RESULTS: Of the 1,733 articles reviewed, 727 met inclusion criteria. A greater absolute number of pediatric OTL-HNS articles were published over the years studied: from 95 in 1996 to 359 in 2016 (P < .001). As well, the absolute number of high-quality studies has increased over the study period, from 28 articles in 1996 to 100 articles in 2016. However, the relative percentage of high-quality papers remained stable between 27.9% and 32.2% with an average of 29.7% (P = .89). Higher impact factor journals did not tend to publish higher-quality pediatric OTL-HNS articles (P = .48). CONCLUSIONS: Over the past 20 years, there is no appreciable improvement in the proportion of high-quality publications in pediatric OTL-HNS; however, there is an overall greater number of high-quality papers within OTL-HNS literature. These findings likely relate to challenges of research within pediatric surgical specialties. LEVEL OF EVIDENCE: NA Laryngoscope, 132:1869-1876, 2022.

Carbon dioxide laser versus cold-steel supraglottoplasty: A comparison of post-operative outcomes.

OBJECTIVE: Supraglottoplasty is the mainstay of surgical treatment for laryngomalacia, and is commonly performed via two methods: cold steel or carbon dioxide (CO2) laser. The degree of post-operative monitoring following supraglottoplasty varies, both within and between institutions. The aim of this study was to compare the post-operative monitoring and interventions required by patients undergoing cold-steel versus CO2 laser supraglottoplasty. DESIGN: Retrospective cohort of pediatric patients (age < 18 years) undergoing supraglottoplasty at a tertiary care pediatric hospital. The primary exposure was the surgical instrument(s) used during supraglottoplasty. The primary outcome was prolonged intensive care unit (ICU)-stay (defined as >24 h). RESULTS: 155 cases were eligible for inclusion. Fifty-eight (37.4%) patients had a comorbid condition. Common indications for surgery included feeding difficulty (56.1%), severe respiratory distress (33.5%), and obstructive sleep apnea (25.2%). CO2 laser was employed in 49 cases and cold-steel in 106 cases. Prolonged ICU-stay (>24 h) was observed in 14 CO2 laser cases (28.6%) and 11 cold-steel cases (10.4%) (adjusted OR 3.42; 95% CI 1.43, 8.33). CO2 laser cases were more likely to require post-operative intubation, non-invasive positive pressure ventilation, and nebulized racemic epinephrine. Concomitant neurological condition was associated with an increased risk of prolonged ICU-stay, while extent of surgery and age were not. CONCLUSIONS: CO2 laser supraglottoplasty is associated with an increased risk of prolonged ICU-stay and need for ICU-level airway intervention, compared to the cold-steel technique. While this association should not be misconstrued as a causal relationship, the current study demonstrates that specific surgical factors may influence the patient monitoring requirements following supraglottoplasty, particularly the choice of instrument and the extent of surgery.

A novel computer algorithm improves antibody epitope prediction using affinity-selected mimotopes: a case study using monoclonal antibodies against the West Nile virus E protein.

Understanding antibody function is often enhanced by knowledge of the specific binding epitope. Here, we describe a computer algorithm that permits epitope prediction based on a collection of random peptide epitopes (mimotopes) isolated by antibody affinity purification. We applied this methodology to the prediction of epitopes for five monoclonal antibodies against the West Nile virus (WNV) E protein, two of which exhibit therapeutic activity in vivo. This strategy was validated by comparison of our results with existing F(ab)-E protein crystal structures and mutational analysis by yeast surface display. We demonstrate that by combining the results of the mimotope method with our data from mutational analysis, epitopes could be predicted with greater certainty. The two methods displayed great complementarity as the mutational analysis facilitated epitope prediction when the results with the mimotope method were equivocal and the mimotope method revealed a broader number of residues within the epitope than the mutational analysis. Our results demonstrate that the combination of these two prediction strategies provides a robust platform for epitope characterization.

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Diagnosis, pre-operative, operative and post-operative pediatric choanal atresia care.

OBJECTIVE: To provide recommendations to otolaryngologists and allied physicians for the comprehensive management of young infants who present with signs or symptoms of choanal atresia. METHODS: A two-iterative delphi method questionnaire was used to establish expert recommendations by the members of the International Otolaryngology Group (IPOG), on the diagnostic, intra-operative, post-operative and revision surgery considerations. RESULTS: Twenty-eight members completed the survey, in 22 tertiary-care center departments representing 8 countries. The main consensual recommendations were: nasal endoscopy or fiberscopy and CT imaging are recommended for diagnosis; unilateral choanal atresia repair should be delayed after at least age 6 months whenever possible; transnasal endoscopic repair is the preferred technique; long term follow-up is recommended (minimum one year) using nasal nasofiberscopy or rigid endoscopy, without systematic imaging. CONCLUSION: Choanal atresia care consensus recommendations are aimed at improving patient-centered care in neonates, infants and children with choanal atresia.

Revision cochlear implant surgery in children: Surgical and audiological outcomes.

OBJECTIVES/HYPOTHESIS: To determine the incidence of cochlear implant failure and to examine surgical and audiological outcomes. STUDY DESIGN: Retrospective review, case series. METHODS: This study sought indications for revision surgery, surgical findings, and outcomes, and audiological outcomes in pediatric cochlear implant patients. Pre- and postcochlear reimplantation word recognition performance was analyzed using a modified version of the Pediatric Ranked Order Speech Perception (PROSPER) score. RESULTS: Over a 20-year period, a total of 868 cochlear implants were performed in 578 patients. The overall institutional reimplant rate was 5.9%. The indications for explantation were hard failure (30), soft failure (23), and medical/surgical indication (13). A significant portion of devices belonged to vendor recalled batches (15) or were damaged by head trauma (eight). Full electrode insertion was achieved in all 62 reimplantations. Post-reimplantation Boston Children's Hospital modified PROSER scores were either stable or improved compared to pre-reimplantation scores. CONCLUSIONS: The need for cochlear implant revision/reimplantation is infrequent, but the rate is not inconsequential. Hard and soft device failures account for the majority of reimplants. Surgical complications during reimplantation is low, and post-reimplantation audiological performance is excellent. LEVEL OF EVIDENCE: 4. Laryngoscope, 2619-2624, 2018.

Recombinant nucleases CEL I from celery and SP I from spinach for mutation detection.

BACKGROUND: The detection of unknown mutations is important in research and medicine. For this purpose, a mismatch-specific endonuclease CEL I from celery has been established as a useful tool in high throughput projects. Previously, CEL I-like activities were described only in a variety of plants and could not be expressed in an active form in bacteria. RESULTS: We describe expression of active recombinant plant mismatch endonucleases and modification of their activities. We also report the cloning of a CEL I ortholog from Spinacia oleracea (spinach) which we termed SP I nuclease. Active CEL I and SP I nucleases were expressed as C-terminal hexahistidine fusions and affinity purified from the cell culture media. Both recombinant enzymes were active in mutation detection in BRCA1 gene of patient-derived DNA. Native SP nuclease purified from spinach is unable to incise at single-nucleotide substitutions and loops containing a guanine nucleotide, but the recombinant SP I nuclease can cut at these sites. CONCLUSION: The insect cell-expressed CEL I orthologs may not be identical to their native counterparts purified from plant tissues. The present expression system should facilitate further development of CEL I-based mutation detection technologies.

Sleep disordered breathing in Bardet-Biedl Syndrome.

INTRODUCTION: Bardet-Biedl Syndrome (BBS) is an autosomal recessive ciliopathy, and obesity is among its defining characteristics. Consequently, the incidence of sleep disordered breathing (SDB) in this population is expected to be high. Due to its relative rarity, the nature of SDB in this population is poorly described. The objective of this study was to review a single institutional experience in the assessment and management of SDB in patients with BBS. METHODS: Retrospective chart review of tertiary care, academic pediatric hospital. RESULTS: 20 patients with BBS were evaluated over a 25-year period. Median age at initial consultation was 69 months; half of these patients were referred before the diagnosis of BBS was made. Eighteen of twenty patients had symptoms of sleep-disordered breathing. Median follow-up duration was 17.5 months. A wide range of polysomnographic outcomes was observed, including obstructive apnea-hypopnea indexes of 0-195 events/hour. Patients were managed with adenotonsillectomy and/or non-invasive positive pressure ventilation. CONCLUSIONS: SDB is commonly seen in BBS. These patients should be routinely screened for OSA and if present, a polysomnogram should be obtained. Based on patient characteristics, the failure rate of primary surgical intervention, namely adenotonsillectomy, is expected to be high. Further investigation into the role of ancillary diagnostic testing is still needed.

Light-emitting diode aspiration: Distinct radiographic features and approach to management.

Foreign body aspiration is a potentially life threatening problem. The successful removal of airway foreign bodies is dependent on positively identifying the object and developing a solution to its extraction prior to attempts at retrieval. Thus, pre-operative radiographic evaluation is essential in the diagnosis and management of foreign body aspiration. The current case report describes the unique challenges in the evaluation and management of an unusual foreign body within the airway. The distinctive radiographic appearance of this foreign body allows it to be easily identified pre-operatively, and this may decrease the likelihood of operative complications and patient morbidity.

International Pediatric Otolaryngology Group: Consensus guidelines on the diagnosis and management of type I laryngeal clefts.

INTRODUCTION: The diagnosis and management of type I laryngeal clefts can be controversial and varies across centers and surgeons. Using existing peer-reviewed literature to develop an expert-based consensus will help guide physicians in the treatment of these patients as well as develop research hypotheses to further study this condition. OBJECTIVE: To provide recommendations for the diagnosis and management of type I laryngeal clefts. METHODS: Determination of current expert- and literature-based recommendations, via a survey of the International Pediatric Otolaryngology Group, using a modified Delphi method. SETTING: Multinational, multi-institutional, tertiary pediatric hospitals. RESULTS: Consensus recommendations include diagnostic workup, medical management, pre-operative, intra-operative and post-operative considerations for type I laryngeal clefts. CONCLUSIONS: This guide on the diagnosis and management of patients with type I laryngeal clefts is aimed at improving patient care and promoting future hypothesis generation and research to validate the recommendations made here.

Bilateral Vocal Cord Paralysis and Cervicolumbar Radiculopathy as the Presenting Paraneoplastic Manifestations of Small Cell Lung Cancer: A Case Report and Literature Review.

Introduction. Bilateral vocal cord paralysis (BVCP) is a potential medical emergency. The Otolaryngologist plays a crucial role in the diagnosis and management of BVCP and must consider a broad differential diagnosis. We present a rare case of BVCP secondary to anti-Hu paraneoplastic syndrome. Case Presentation. A 58-year-old female presented to an Otolaryngology clinic with a history of progressive hoarseness and dysphagia. Flexible nasolaryngoscopy demonstrated BVCP. Cross-sectional imaging of the brain and vagus nerves was negative. An antiparaneoplastic antibody panel was positive for anti-Hu antibodies. This led to an endobronchial biopsy of a paratracheal lymph node, which confirmed the diagnosis of small cell lung cancer. Conclusion. Paraneoplastic neuropathy is a rare cause of BVCP and should be considered when more common pathologies are ruled out. This is the second reported case of BVCP as a presenting symptom of paraneoplastic syndrome secondary to small cell lung cancer.