Childhood-Onset Granulomatosis With Polyangiitis as a Palatal Defect: A Case Report.

Granulomatosis with polyangiitis (GPA) is a necrotizing systemic vasculitis of small and medium-sized vessels with renal and sinopulmonary involvement. Its symptoms include chronic sinusitis, recurrent pneumonia, glomerulonephritis, constitutional symptoms, and skin manifestations with a typical onset in the fourth to sixth decade of life. We present a rare case of GPA in a 16-year-old female who presented with facial numbness and nasal regurgitation via a palatal defect. The patient reported a several-month history of recurrent epistaxis and chronic nasal congestion accompanied by several weeks of night sweats, lower right-sided facial numbness and pain, nasal regurgitation of food and liquids, and a 30-pound weight loss. A physical exam found a 3-cm defect on the right side of her palate. CT of the sinuses showed significant sinonasal destruction and petrous apicitis. GPA was confirmed via pathognomonic chest X-ray findings and biopsy results. The patient was treated with maxillary antrostomy and anterior ethmoidectomy and a follow-up was scheduled to address sequelae of the destructive sinopulmonary lesions. This case report highlights a unique presentation of GPA with an insidious development of autoimmune sinonasal destruction in an adolescent female. This presentation is rare and highlights the importance of considering autoimmune disease in cases of tissue destruction where the etiology is not apparent, even in patients at low risk for autoimmune conditions.

Adolescent and Young Adult (AYA) Oncology, Version 2.2024, NCCN Clinical Practice Guidelines in Oncology.

This selection from the NCCN Guidelines for Adolescent and Young Adult (AYA) Oncology focuses on considerations for the comprehensive care of AYA patients with cancer. Compared with older adults with cancer, AYA patients have unique needs regarding treatment, fertility counseling, psychosocial and behavioral issues, and supportive care services. The complete version of the NCCN Guidelines for Adolescent and Young Adult (AYA) Oncology addresses additional aspects of caring for AYA patients, including risk factors, screening, diagnosis, and survivorship.

Checking IN: Development, Acceptability, and Feasibility of a Pediatric Electronic Distress Screener.

Objective: Chronic illness in children and adolescents is associated with significant stress and risk of psychosocial problems. In busy pediatric clinics, limited time and resources are significant barriers to providing mental health assessment for every child. A brief, real-time self-report measure of psychosocial problems is needed. Methods: An electronic distress screening tool, Checking IN, for ages 8-21 was developed in 3 phases. Phase I used semi-structured cognitive interviews (N = 47) to test the wording of items assessing emotional, physical, social, practical, and spiritual concerns of pediatric patients. Findings informed the development of the final measure and an electronic platform (Phase II). Phase III used semi-structured interviews (N = 134) to assess child, caregiver and researcher perception of the feasibility, acceptability, and barriers of administering Checking IN in the outpatient setting at 4 sites. Results: Most patients and caregivers rated Checking IN as "easy" or "very easy" to complete, "feasible" or "somewhat feasible," and the time to complete the measure as acceptable. Most providers (n = 68) reported Checking IN elicited clinically useful and novel information. Fifty-four percent changed care for their patient based on the results. Conclusions: Checking IN is a versatile and brief distress screener that is acceptable to youth with chronic illness and feasible to administer. The summary report provides immediate clinically meaningful data. Electronic tools like Checking IN can capture a child's current psychosocial wellbeing in a standardized, consistent, and useful way, while allowing for the automation of triaging referrals and psychosocial documentation during outpatient visits.

The Effect of Pediatric Cancer on Identity in Young Adult Survivors: Results from Project REACH.

Purpose: Living through cancer can have a profound effect on identity. Among those treated for adult-onset cancer, identifying as a cancer survivor is common and has been linked to positive mental and physical health outcomes. Although childhood is a critical period for identity formation, comparatively less is known about identity development after pediatric cancers. Methods: One hundred seventy young adults previously treated for pediatric cancer enrolled in Project REACH, a prospective cohort study, and completed self-report measures on effect of cancer on identity (e.g., cancer victim and survivor identity), frequency of thoughts of cancer, health outcomes (i.e., anxiety, depression, and health-related quality of life), and health behaviors (i.e., smoking, sun protection, exercise, and binge drinking). Results: The majority of participants identified as a survivor (n = 146, 85.9%), and survivor identity was linked to significantly higher anxiety (p = 0.01) and worse overall mental health (p = 0.04), but no other health outcomes or behaviors. Only 14 participants (8.2%) endorsed the victim identity, and they were significantly more likely to report binge drinking (p = 0.03) and worse overall mental health (p = 0.02) than nonvictims. Frequency of thoughts of cancer and effect of cancer on identity were also significantly associated with health outcomes (e.g., more frequent thoughts associated with higher anxiety). Conclusions: Our findings suggest that what is known about the relationship between identity and health outcomes after adult-onset cancer cannot be generalized to pediatric cancers, and that clinicians should not assume survivor identity is associated with better health outcomes in this group.

Psychosexual Care of Adolescent and Young Adult (AYA) Cancer Survivors.

Adolescent and young adult (AYA) survivors of cancer have diverse psychosocial and medical needs, including those related to fertility and sexual health. Much of the focus of care around issues such as fertility and sexual health tends to be filtered through a biomedical lens. However, it is essential that health care providers assess and support AYA survivors using a biopsychosocial and contextual framework to ensure the most comprehensive and accurate understanding of AYA survivor needs, especially those related to psychosexual health. A trusting relationship between the multi-disciplinary medical team and the AYA survivor that allows for open discussion about the physical and psychosocial components of sexual health is key to providing best care and outcomes.

Hospitalization for Suicide Ideation or Attempt: 2008-2015.

OBJECTIVES: Suicide ideation (SI) and suicide attempts (SAs) have been reported as increasing among US children over the last decade. We examined trends in emergency and inpatient encounters for SI and SA at US children's hospitals from 2008 to 2015. METHODS: We used retrospective analysis of administrative billing data from the Pediatric Health Information System database. RESULTS: There were 115 856 SI and SA encounters during the study period. Annual percentage of all visits for SI and SA almost doubled, increasing from 0.66% in 2008 to 1.82% in 2015 (average annual increase 0.16 percentage points [95% confidence intervals (CIs) 0.15 to 0.17]). Significant increases were noted in all age groups but were higher in adolescents 15 to 17 years old (average annual increase 0.27 percentage points [95% CI 0.23 to 0.30]) and adolescents 12 to 14 years old (average annual increase 0.25 percentage points [95% CI 0.21 to 0.27]). Increases were noted in girls (average annual increase 0.14 percentage points [95% CI 0.13 to 0.15]) and boys (average annual increase 0.10 percentage points [95% CI 0.09 to 0.11]), but were higher for girls. Seasonal variation was also observed, with the lowest percentage of cases occurring during the summer and the highest during spring and fall. CONCLUSIONS: Encounters for SI and SA at US children's hospitals increased steadily from 2008 to 2015 and accounted for an increasing percentage of all hospital encounters. Increases were noted across all age groups, with consistent seasonal patterns that persisted over the study period. The growing impact of pediatric mental health disorders has important implications for children's hospitals and health care delivery systems.

Recall of Fertility Discussion by Adolescent Female Cancer Patients: A Survey-Based Pilot Study.

Many adolescent female cancer patients will survive into their reproductive years. Pediatric oncologists are advised to discuss oncofertility during treatment planning. In this pilot study, 19 adolescent females completed a retrospective survey assessing recall of a fertility discussion, satisfaction with fertility knowledge, and multiple factors that may influence recall, including parental involvement in decision-making. Eleven respondents (58%) remembered a discussion about infertility risk and 9 (47%) about fertility preservation. Most who recalled a discussion were satisfied with their fertility knowledge (10/11, 90.9%). In this study, we validated the feasibility of survey administration and identified trends in oncofertility counseling at our center.

Establishing psychosocial palliative care standards for children and adolescents with cancer and their families: An integrative review.

BACKGROUND: Despite standardization in disease assessments and curative interventions for childhood cancer, palliative assessments and psychosocial interventions remain diverse and disparate. AIM: Identify current approaches to palliative care in the pediatric oncology setting to inform development of comprehensive psychosocial palliative care standards for pediatric and adolescent patients with cancer and their families. Analyze barriers to implementation and enabling factors. DESIGN: Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines framed the search strategy and reporting. Data analysis followed integrative review methodology. DATA SOURCES: Four databases were searched in May 2014 with date restrictions from 2000 to 2014: PubMed, Cochrane, PsycINFO, and Scopus. A total of 182 studies were included for synthesis. Types of studies included randomized and non-randomized trials with or without comparison groups, qualitative research, prior reviews, expert opinion, and consensus report. RESULTS: Integration of patient, parent, and clinician perspectives on end-of-life needs as gathered from primary manuscripts (using NVivo coding for first-order constructs) revealed mutual themes across stakeholders: holding to hope, communicating honestly, striving for relief from symptom burden, and caring for one another. Integration of themes from primary author palliative care outcome reports (second-order constructs) revealed the following shared priorities in cancer settings: care access; cost analysis; social support to include primary caregiver support, sibling care, bereavement outreach; symptom assessment and interventions to include both physical and psychological symptoms; communication approaches to include decision-making; and overall care quality. CONCLUSION: The study team coordinated landmark psychosocial palliative care papers into an informed conceptual model (third-order construct) for approaching pediatric palliative care and psychosocial support in oncology settings.

Palliative Care as a Standard of Care in Pediatric Oncology.

The study team conducted a systematic review of pediatric and adolescent palliative cancer care literature from 1995 to 2015 using four databases to inform development of a palliative care psychosocial standard. A total of 209 papers were reviewed with inclusion of 73 papers for final synthesis. Revealed topics of urgent consideration include the following: symptom assessment and intervention, direct patient report, effective communication, and shared decision-making. Standardization of palliative care assessments and interventions in pediatric oncology has the potential to foster improved quality of care across the cancer trajectory for children and adolescents with cancer and their family members.

Surgeons' views of health technology assessment in Australia: online pilot survey.

INTRODUCTION: Many governments have introduced health technology assessment (HTA) as an important tool to manage the uptake and use of health-related technologies efficiently. Although surgeons play a central role in the uptake and diffusion of new technologies, little is known about their opinion and understanding of the HTA role and process. METHODS: A cross-sectional pilot study was conducted using an online questionnaire which was distributed to Fellows of the Royal Australasian College of Surgeons over a 4-week period. Information was sought about knowledge and views of the HTA process. Descriptive statistics were used to summarize the data, frequencies, and proportions were calculated. RESULTS: Sixty-two surgeons completed the survey; of these, 55 percent reported their primary work place as a public hospital. Twenty-four percent of the participants reported that they had never heard of the HTA agency and 60 percent reported that surgical procedures are most likely to be introduced in the Australian healthcare system at the public hospital level (which is beyond the HTA's scope and dealt with at a state level). However, 61 percent considered that decisions about funding and adoption of new technologies should take place at the national level. CONCLUSIONS: This survey provides some evidence that many surgeons remain unaware of the federal government's HTA process but still value evidence-based information. In order for HTA to be an effective aid to rational adoption of health-related technologies, there is a need for an evidence-based approach that is integrated and is accepted and understood by the medical professions.

Introduction and uptake of new medical technologies in the Australian health care system: a qualitative study.

OBJECTIVE: The aim of this study was to explore the views and perceptions of stakeholders about the current national health technology assessment process conducted by the Medical Services Advisory Committee (MSAC) and its role in the uptake and diffusion of new medical technologies in Australia. METHODS: Data collection occurred over a nine month period (August 2008-April 2009). Twenty in-depth, semi-structured interviews were conducted with individuals from four stakeholders groups: (i) MSAC members and evaluators, (ii) academic and health technology assessment experts, (iii) medical industry representatives and (iv) medical specialists. Interviews were digitally recorded, transcribed verbatim and coded using a constant comparative method. RESULTS: Respondents expressed a consensus opinion that the MSAC process is generally fair and transparent, and has been increasingly so over time. The process was described as "flexible" and "intuitive" yet also "idiosyncratic" due to the nature of the technologies being appraised. Approval by MSAC was generally reported to be increasingly important once a technology becomes more widely used. While successful MSAC approval was felt to be important for widespread distribution of a new technology, it was viewed more as a "facilitator of the uptake of new technologies" as opposed to a primary "driver" of technology uptake. Instead, other factors were identified as providing the actual impetus for the uptake of new technologies, with MSAC approval and reimbursement eventually helping facilitate more widespread diffusion. CONCLUSIONS: MSAC's decision making process is perceived as fair but with room for improvement. Its role in the uptake and diffusion of new medical technologies in Australia is limited. MSAC does not act as a barrier to significant market penetration of new procedures and medical technologies. However reimbursement is a trigger for increased use.

Psychological aspects of hematologic diseases.

This article focused on two hematologic diseases that have received significant attention in the pediatric literature: sickle cell anemia and hemophilia. As the data indicate, these illnesses present lifelong challenges to individuals afflicted. With advances in medical interventions, many children with these hematologic diseases are living longer and with fewer serious complications. Intervention efforts only recently have begun to consider issues related to quality of life and increased psychological adjustment, including active coping strategies by children and their parents and necessary social support for all family members. Theoretical models [54] have highlighted the multiple factors that play a role in illness adjustment and the complex interactions of these factors. Individual, family, and community characteristics are impacted by and, in turn, impact on illness-related characteristics. The notion that illness severity is predictive of psychological adjustment has been discarded in favor of a model that recognizes multiple influences and multiple outcomes. Child coping, parent coping, social support, adaptive functioning, treatment compliance, and illness severity are being considered to better understand and influence overall psychological functioning. The field of pediatric psychology has made tremendous advances in improving knowledge of illness and its impact on development. Even with these advances, however, there remains much to be discovered. To date, pediatric psychology research has focused primarily on individual illness categories, making comparisons across illness types difficult. By considering different illnesses within the same study, characteristics that are crucial to improved adjustment and common across illnesses can be identified. As we continue to work for cures for these debilitating illnesses, our goal remains to improve quality of life for children.