Case report: a giant cell-rich gnathic bone lesion in a child with pycnodysostosis.

Pycnodysostosis is a skeletal dysplasia characterized by short stature, generalized osteosclerosis, acro-osteolysis, and recognizable facial features. Oral manifestations are commonly described and include a high-arched palate with dental crowding and malocclusion, hypoplastic enamel, and retained deciduous teeth with impacted permanent teeth, and there is an increased risk of developing osteomyelitis of the jaw. We report here the history of a 9-year-old male with the typical facial and skeletal phenotype of pycnodysostosis but novel oral features. He presented with bilateral progressive facial swelling, which caused functional impairment with chewing and contributed to his severe obstructive sleep apnea (OSA). The severity of his OSA required surgical intervention, and the lesions were resected. Extensive bone remodeling and replacement by fibrous tissue were noted on submucosal dissection, and bilateral subtotal maxillectomies were required. The histopathology of the biopsied lesion was consistent with a giant cell-rich lesion. Genetic testing identified a pathogenic homozygous variant in the CTSK gene, c.953G > A, p. (Cys318Tyr). The proband had a good postsurgical response with sustained improvement in his sleep OSA. We present here the history and clinical characteristics of a patient with typical features of pycnodysostosis and an unusual presentation and histopathology of gnathic bone lesions. This report adds to the body of literature on this rare condition and also highlights the finding of giant cell-rich lesions of the gnathic bones. Giant cell-rich lesions in pycnodysostosis have previously been reported in two cases in the literature. While there is not enough evidence to support a certain association with pycnodysostosis, it is prudent to consider regular oral dental reviews in affected individuals to identify pathology early and avoid such life-threatening complications.

Human papillomavirus vaccination in Africa: An airway perspective.

INTRODUCTION: Recurrent respiratory papillomatosis (RRP) is a chronic condition caused by Human papillomavirus six (HPV-6) and HPV-11 that involves the respiratory tract. Disease severity ranges from mild (hoarseness), through to severe (stridor, respiratory distress and airway emergencies). Africa has the fastest growing and youngest population of all the continents. It also has the greatest burden of cervical cancer. There is an association with infection of the oncogenic HPV strains and the strains responsible for RRP. It is reasonable to conclude that although RRP may be underestimated in low-to-middle-income countries, it poses a considerable health risk to Africa. The primary aim of this project was to assess the suitability of HPV vaccination coverage on the African continent. METHODS: A prospective study was designed to consist of an online survey. It was distributed to 135 African otolaryngologists. Questions focussed on HPV vaccination programmes; whether they were government directed; and their rollout. Information from countries that had multiple otolaryngologists respond to the survey were compared. Additionally, data review and corroboration were performed. RESULTS: There were 58 (43%) participants from 19 countries. Nine countries reported a national vaccination programme (NVP), five used Cervarix; four used quadrivalent Gardasil. Collateral data revealed 18 of 54 countries had NVP in Africa and 26 countries had completed HPV vaccine pilot or demonstration projects. CONCLUSIONS: HPV vaccination in Africa should be urgently re-evaluated to include the HPV-6 and HPV-11 strains that cause JORRP, which have not been recognised during national vaccination programme planning.

National population prevalence of antibodies to SARS-CoV-2 among pregnant women in Scotland during the second wave of the COVID-19 pandemic: a prospective national serosurvey.

OBJECTIVES: This study aimed to determine SARS-CoV-2 seroprevalence among pregnant women in the Scottish population during the second wave of the COVID-19 pandemic. STUDY DESIGN: Prospective national serosurvey. METHODS: We tested 13,428 residual samples retrieved from pregnant women participating in the first trimester combined ultrasound and biochemical screening for fetal trisomy across Scotland for SARS-CoV-2 antibodies over a 6-month period from November 2020 to April 2021. Seroprevalence estimates were adjusted for the sensitivity and specificity of the assays and weighted to reference populations. RESULTS: Seroprevalence rates in the antenatal samples significantly increased from 5.5% (95% confidence interval [CI] 4.7%-6.5%) in the 5-week period up to and including International Organization for Standardization (ISO) Week 51 (w/b Monday 14 December 2020) to 11.3% (95% CI 10.1%-12.6%) in the 5-week period up to and including ISO Week 14 (w/b Monday 5 April 2021). Increasing seroprevalence trends across the second wave were observed among all age groups. CONCLUSIONS: By the end of the second wave of the COVID-19 pandemic, approximately one in 10 women tested around the end of the first trimester of pregnancy had antibodies to SARS-CoV-2, suggesting that the vast majority were still susceptible to COVID-19 as they progressed to the later stages of pregnancy, when risks from infection are elevated for both mother and baby.

The Mars 2020 Engineering Cameras and Microphone on the Perseverance Rover: A Next-Generation Imaging System for Mars Exploration.

The Mars 2020 Perseverance rover is equipped with a next-generation engineering camera imaging system that represents an upgrade over previous Mars rover missions. These upgrades will improve the operational capabilities of the rover with an emphasis on drive planning, robotic arm operation, instrument operations, sample caching activities, and documentation of key events during entry, descent, and landing (EDL). There are a total of 16 cameras in the Perseverance engineering imaging system, including 9 cameras for surface operations and 7 cameras for EDL documentation. There are 3 types of cameras designed for surface operations: Navigation cameras (Navcams, quantity 2), Hazard Avoidance Cameras (Hazcams, quantity 6), and Cachecam (quantity 1). The Navcams will acquire color stereo images of the surface with a 96 ∘ × 73 ∘ field of view at 0.33 mrad/pixel. The Hazcams will acquire color stereo images of the surface with a 136 ∘ × 102 ∘ at 0.46 mrad/pixel. The Cachecam, a new camera type, will acquire images of Martian material inside the sample tubes during caching operations at a spatial scale of 12.5 microns/pixel. There are 5 types of EDL documentation cameras: The Parachute Uplook Cameras (PUCs, quantity 3), the Descent stage Downlook Camera (DDC, quantity 1), the Rover Uplook Camera (RUC, quantity 1), the Rover Descent Camera (RDC, quantity 1), and the Lander Vision System (LVS) Camera (LCAM, quantity 1). The PUCs are mounted on the parachute support structure and will acquire video of the parachute deployment event as part of a system to characterize parachute performance. The DDC is attached to the descent stage and pointed downward, it will characterize vehicle dynamics by capturing video of the rover as it descends from the skycrane. The rover-mounted RUC, attached to the rover and looking upward, will capture similar video of the skycrane from the vantage point of the rover and will also acquire video of the descent stage flyaway event. The RDC, attached to the rover and looking downward, will document plume dynamics by imaging the Martian surface before, during, and after rover touchdown. The LCAM, mounted to the bottom of the rover chassis and pointed downward, will acquire 90 ∘ × 90 ∘ FOV images during the parachute descent phase of EDL as input to an onboard map localization by the Lander Vision System (LVS). The rover also carries a microphone, mounted externally on the rover chassis, to capture acoustic signatures during and after EDL. The Perseverance rover launched from Earth on July 30th, 2020, and touchdown on Mars is scheduled for February 18th, 2021.

Does thyroid-sparing total laryngectomy decrease the risk of hypothyroidism?

BACKGROUND: Thyroid lobectomy is recommended with total laryngectomy for laryngeal cancer in the National Comprehensive Cancer Network ('NCCN') guidelines. However, it is associated with a 32-89 per cent risk of hypothyroidism, with or without adjuvant radiotherapy. OBJECTIVE: The study aimed to determine whether preserving the whole thyroid, compared to a single lobe, does indeed significantly lower the incidence of hypothyroidism in the setting of total laryngectomy. METHOD: A retrospective study was conducted at Groote Schuur Hospital in Cape Town, South Africa. RESULTS: Eighty-four patients met the inclusion criteria. The overall incidence of hypothyroidism was 45.2 per cent. The incidence of hypothyroidism was significantly reduced in patients who underwent thyroid-sparing total laryngectomy compared to hemithyroidectomy (p = 0.037). Adjuvant radiotherapy was associated with a higher incidence of hypothyroidism (p = 0.001). CONCLUSION: Thyroid-preserving laryngectomy should be advocated in carefully selected patients with advanced laryngeal carcinoma, as it reduces the incidence of hypothyroidism.

Reintroduction of elective paediatric otolaryngology procedures in South Africa during the COVID-19 pandemic.

Cancelling elective clinical consultations and surgical procedures was instrumental in assisting hospitals prepare for the COVID-19 crisis. Essential bed space was made available, and it allowed mobilisation of health workers and enforced social distancing. A shift in patient-centred ethics to public health ethics was required to provide a utilitarian approach to the crisis. However, at some point, clinicians need to start becoming patient centred again, and this needs to happen within the utilitarian framework. Children only account for 1 - 5% of confirmed COVID-19 cases, and they present with a much milder disease spectrum than adults. Consequently, paediatric units may be at the forefront of implementing reintroduction of patient-centred elective clinical and surgical procedures. The following recommendations provide a framework to do this in a way that minimises risk to patients and clinicians. They are the first paediatric guidelines in the literature to propose a strategy to reintroduce elective surgical procedures.

A nationwide survey of pharmacy students' knowledge and perceptions regarding medical cannabis.

OBJECTIVE: The primary objective of this study was to compare knowledge and perceptions of medical cannabis (MC) between pharmacy students who attended schools where MC was legal and illegal. DESIGN: A nationwide anonymous survey regarding MC was distributed to pharmacy students. The survey consisted of the following sections: (1) demographics and personal factors, (2) knowledge assessment of indications and adverse effects of MC, and (3) perceptions assessment. SETTING AND PARTICIPANTS: Equal numbers of pharmacy schools were selected and sent surveys based on geographical regions, private versus public universities, and the state's legal status of MC for first through fourth-year pharmacy students to complete. OUTCOME MEASURES: Student knowledge of MC indications and adverse effects were treated as quizzes. Median percentage correct and whether students passed the quizzes (≥ 70%) were evaluated based on the state's legal status. Students' perceptions were similarly compared. RESULTS: A total of 629 students accessed the survey. Most students who completed the survey attended pharmacy schools where MC was illegal (55%), were female (68%), and had personally tried cannabis (53%). Overall, 91% of students believed that MC should be legalized nationally. A low number of students correctly identify approved indications with 14.8% of students in states with MC legalization scoring greater than 70% compared with 15.9% of students in states without MC legalization (P = 0.748). Similar findings were seen in the side effects quiz with only 6.1% of students in states with legal MC scoring greater than 70% compared with 7.4% of students where MC is illegal (P = 0.569) There were minimal differences in students' perceptions of MC based on the states' legal status. CONCLUSION: Our study continues to highlight that pharmacy students lack knowledge and preparation to counsel and educate on MC. With increasing state legalizations, pharmacy schools need to address this knowledge gap to ensure optimal patient care.

Reintroduction of elective paediatric otolaryngology procedures in South Africa during the COVID-19 pandemic

Cancelling elective clinical consultations and surgical procedures was instrumental in assisting hospitals prepare for the COVID-19 crisis. Essential bed space was made available, and it allowed mobilisation of health workers and enforced social distancing. A shift in patient-centred ethics to public health ethics was required to provide a utilitarian approach to the crisis. However, at some point, clinicians need to start becoming patient centred again, and this needs to happen within the utilitarian framework. Children only account for 1 - 5% of confirmed COVID-19 cases, and they present with a much milder disease spectrum than adults. Consequently, paediatric units may be at the forefront of implementing reintroduction of patient-centred elective clinical and surgical procedures. The following recommendations provide a framework to do this in a way that minimises risk to patients and clinicians. They are the first paediatric guidelines in the literature to propose a strategy to reintroduce elective surgical procedures

Quality of life after total laryngectomy: evaluating the effect of socioeconomic status.

OBJECTIVE: Total laryngectomy is considered the primary treatment modality for advanced laryngeal carcinoma. This study assessed the quality of life in patients after total laryngectomy, and ascertained whether quality of life is affected by socioeconomic status. METHOD: Forty-seven patients (20 state- and 27 private-sector) who underwent total laryngectomy between 1998 and 2014 responded to the University of Washington Quality of Life Questionnaire, the Voice-Related Quality of Life Questionnaire and the Brief Illness Perception Questionnaire. RESULTS: Significant differences were found in socioeconomic status between state- and private-sector patients (p < 0.001). There was no significant difference in overall quality of life between groups (p = 0.210). State-sector patients scored significantly higher Voice-Related Quality of Life Questionnaire scores (p = 0.043). Perception of illness did not differ significantly between groups. CONCLUSION: Overall quality of life after total laryngectomy appears to be similar in patients from different socioeconomic backgrounds. However, patients from lower socioeconomic circumstances have better voice-related quality of life. The results illustrate the importance of including socioeconomic status when reporting voice outcomes in total laryngectomy patients.

Use of a Mindfulness Smartphone App in an Advanced Psychiatry Elective for Pharmacy Students.

BACKGROUND: There is a mental health crisis among US University students. Evidence supporting techniques to help students manage the stress are critically needed. Mindfulness meditation is one such strategy which may be helpful for pharmacy students. INNOVATION: Third-year pharmacy students enrolled in an Advanced Psychiatry elective course were invited to participate in the study. Students were given a 10-day assignment to participate in 10 free mindfulness sessions offered by the smart phone app, Headspace®. The Mindful Attention Awareness Scale (MAAS) was completed before and after the assignment. Student qualitative feedback was also obtained after utilization of the app. FINDINGS: Seventy-six percent of students completed the mindfulness sessions daily. A small, non-statistically significant change in MAAS Total Score was observed in the students after use of the mindfulness app. The majority of students would recommend the mindfulness app to their patients, as well as 1st year pharmacy students. The students also reported that the assignment aided in their education about non-pharmacologic treatment options for depression and anxiety. CONCLUSION: This pilot study provides preliminary evidence for the further investigation of mindfulness meditation smartphone apps in pharmacy students both as a tool to aid in managing the stress of pharmacy school and as a teaching tool.

Radiological differences between HIV-positive and HIV-negative children with cholesteatoma.

INTRODUCTION: HIV-positive children are possibly more prone to developing cholesteatoma. Chronic inflammation of the middle ear cleft may be more common in patients with HIV and this may predispose HIV-positive children to developing cholesteatoma. There are no studies that describe the radiological morphology of the middle ear cleft in HIV-positive compared to HIV-negative children with cholesteatoma. OBJECTIVES: Compare the radiological differences of the middle ear cleft in HIV-positive and HIV-negative children with cholesteatoma. METHODS: A retrospective, cross-sectional, observational analytical review of patients with cholesteatoma at our institute over a 6 year period. RESULTS: Forty patients were included in the study, 11 of whom had bilateral cholesteatoma and therefore 51 ears were eligible for our evaluation. HIV-positive patients had smaller (p=0.02) mastoid air cell systems (MACS). Forty percent of HIV-positive patients had sclerotic mastoids, whereas the rate was 3% in HIV-negative ears (p<0.02). Eighty-two percent of the HIV-positive patients had bilateral cholesteatoma compared to 7% of the control group (p<0.02). There was no difference between the 2 groups with regards to opacification of the middle ear cleft, bony erosion of middle ear structures, Eustachian tube obstruction or soft tissue occlusion of the post-nasal space. CONCLUSION: HIV-positive paediatric patients with cholesteatoma are more likely to have smaller, sclerotic mastoids compared to HIV-negative patients. They are significantly more likely to have bilateral cholesteatoma. This may have implications in terms of surveillance of HIV-positive children, as well as, an approach to management, recurrence and follow-up. HIV infection should be flagged as a risk factor for developing cholesteatoma.

Oral cancer radiotherapy affects enamel microhardness and associated indentation pattern morphology.

OBJECTIVES: The aim of this study is to determine the effects of in vitro and in vivo high-dose radiotherapy on microhardness and associated indentation pattern morphology of enamel. MATERIALS AND METHODS: The inner, middle, and outer microhardness of enamel was evaluated using three experimental groups: control (non-radiated); in vitro irradiated; in vivo irradiated. In vitro specimens were exposed to simulated radiotherapy, and in vivo specimens were extracted teeth from oral cancer patients previously treated with radiotherapy. Indentations were measured via SEM images to calculate microhardness values and to assess the mechanomorphological properties of enamel before and after radiotherapy. RESULTS: Middle and outer regions of enamel demonstrated a significant decrease in microhardness after in vitro and in vivo irradiation compared to the control group (p < 0.05). Two indentation patterns were observed: pattern A-presence of microcracks around indent periphery, which represents local dissipation of deformation energy; pattern B-clean, sharp indents. The percentage of clean microindentation patterns, compared to controls, was significantly higher following in vitro and in vivo irradiation in all enamel regions. The highest percentage of clean microindentations (65%) was observed in the in vivo irradiated group in the inner region of enamel near the dentin-enamel junction. CONCLUSIONS: For the first time, this study shows that in vitro and in vivo irradiation alters enamel microhardness. Likewise, the indentation pattern differences suggest that enamel may become more brittle following in vitro and in vivo irradiation. CLINICAL RELEVANCE: The mechanomorphological property changes of enamel following radiation may be a contributory component of pathologic enamel delamination following oral cancer radiotherapy.

The Structured Assessment of Violence Risk in Adults with Intellectual Disability: A Systematic Review.

BACKGROUND: While structured professional judgement approaches to assessing and managing the risk of violence have been extensively examined in mental health/forensic settings, the application of the findings to people with an intellectual disability is less extensively researched and reviewed. This review aimed to assess whether risk assessment tools have adequate predictive validity for violence in adults with an intellectual disability. METHODS: Standard systematic review methodology was used to identify and synthesize appropriate studies. RESULTS: A total of 14 studies were identified as meeting the inclusion criteria. These studies assessed the predictive validity of 18 different risk assessment tools, mainly in forensic settings. All studies concluded that the tools assessed were successful in predicting violence. Studies were generally of a high quality. CONCLUSIONS: There is good quality evidence that risk assessment tools are valid for people with intellectual disability who offend but further research is required to validate tools for use with people with intellectual disability who offend.

Genetic assessment of the effects of streamscape succession on coho salmon Oncorhynchus kisutch colonization in recently deglaciated streams.

Measures of genetic diversity within and among populations and historical geomorphological data on stream landscapes were used in model simulations based on approximate Bayesian computation (ABC) to examine hypotheses of the relative importance of stream features (geomorphology and age) associated with colonization events and gene flow for coho salmon Oncorhynchus kisutch breeding in recently deglaciated streams (50-240 years b.p.) in Glacier Bay National Park (GBNP), Alaska. Population estimates of genetic diversity including heterozygosity and allelic richness declined significantly and monotonically from the oldest and largest to youngest and smallest GBNP streams. Interpopulation variance in allele frequency increased with increasing distance between streams (r = 0·435, P < 0·01) and was inversely related to stream age (r = -0·281, P < 0·01). The most supported model of colonization involved ongoing or recent (<10 generations before sampling) colonization originating from large populations outside Glacier Bay proper into all other GBNP streams sampled. Results here show that sustained gene flow from large source populations is important to recently established O. kisutch metapopulations. Studies that document how genetic and demographic characteristics of newly founded populations vary associated with successional changes in stream habitat are of particular importance to and have significant implications for, restoration of declining or repatriation of extirpated populations in other regions of the species' native range.

Re-calculating! Navigating through the osteosarcoma treatment roadblock.

The survival rates for patients with osteosarcoma have remained almost static for the past three decades. Current standard of care therapy includes chemotherapies such as doxorubicin, cisplatin, and methotrexate along with complete surgical resection and surgery with or without ifosfamide and etoposide for relapse, though outcomes are hoped to be improved through clinical trials. Additionally, increased understanding of the genetics, signaling pathways and microenvironmental factors driving the disease have led to the identification of promising agents and potential paths towards translation of an exciting array of novel targeted therapies. Here, we review the mechanism of action of these emerging therapies and how, with clinical translation, they can potentially improve the survival rates for osteosarcoma patients in the near future.

Management of pediatric cholesteatoma based on presentations, complications, and outcomes.

OBJECTIVES: To highlight important aspects and paradigms in the management of paediatric cholesteatoma in a developing world setting. METHODS: A retrospective audit was conducted of paediatric cholesteatomas that underwent tympanomastoid surgery between 2008 and 2012 at the Red Cross War Memorial Children's Hospital in Cape Town. The following was audited: initial presentation; cholesteatoma complications; types of surgery, intraoperative findings and outcomes of surgery in terms of hearing, otorrhoea and recidivism; and the reliability of follow-up and how this might influence the type of surgery. RESULTS: Fifty-seven children aged 2-13 years with 61 cholesteatomas (4 bilateral) were reviewed. Fifty-five mastoidectomies were done; 11% presented with complicated cholesteatoma. Referrals from primary care were significantly delayed (>6 months) in 76%. Canal wall down surgery was done in 71%. Forty-five percent had improved hearing (within 15dB of better hearing ear) and a further 15% had no or only mild hearing loss. Ossicular chain involvement and ossicles encased in inflammatory tissue were associated with poorer hearing outcomes. Sixty-four percent of ears remained dry. Forty-five percent of the canal wall up, and 23% of canal wall down mastoidectomies had recidivism. Twenty-six percent of patients were lost to follow-up. CONCLUSIONS: Children are likely to present with advanced cholesteatoma with ossicular chain involvement. The children present with high rates of complications, poor pre-operative hearing and have high recurrence rates post-surgery. Referral from primary health care is delayed. Canal wall down procedures may be appropriate in a setting where patient follow-up is unreliable and access to operating theatre is limited.