Special care dentistry for patients diagnosed with sickle cell disease: an update for dentists.

Sickle cell disease (SCD) is a relatively common genetic disorder. Patients diagnosed with SCD may encounter barriers to dental care. Consequently, dental care providers should update their knowledge regarding the management of patients with SCD to reduce the possibility of triggering sickling events and potential damage to the body. The purpose of this article is to discuss the oral and dental management of patients with SCD and to clarify the risk factors that can lead to the sickling of the red blood cells during dental care. Adherence to clinical guidelines for preventive dentistry, effective pain and anxiety control, and stress reduction is crucial. Conscious sedation techniques, such as inhalation sedation with nitrous oxide and oxygen, can help to reduce episodes of stress and the potential for sickling. For patients with SCD, intravenous sedation should be provided only in a secondary care setting by a suitably experienced specialist in dental sedation.

Application of the Mental Capacity Act (2005) and Consenting for Treatment under Concious Sedation.

The Mental Capacity Act 2005 (MCA) is one piece of legislation with which all clinicians should be familiar. As treating clinicians, a capacity assessment is carried out each time a patient is seen, in order to obtain informed consent. When clinicians question the capacity of their patients, they should ensure steps are taken to provide relevant adjuncts to aid understanding, from visual aids to alternative time and environment. If a patient is deemed to lack capacity, it should be determined if this is temporary, fluctuating or permanent, as well as the severity. For those who lack capacity a best interest assessment should be undertaken with all relevant parties involved, with the overall decision being made in the patient's best interest and in the least restrictive manner. Considerations of sedation technique, clinical holding, or any other procedures should be included in the consent form, with periodic review of the best interest decision as a patient's circumstances may change over time.

Periodontal Treatment in Patients with Learning Disabilities Part 2: Professional Mechanical Intervention.

The first part of this two part series discussed the potential barriers and risk factors that may lead to an increased incidence and severity of periodontal disease amongst patients with learning disabilities. Additionally, preventive strategies and tools that can be used by general dental practitioners, oral health promotion teams as well as specialists within the field to control and prevent disease progression were explored. To prevent periodontal disease progression and attain optimal periodontal health, a combination of prevention and professional mechanical instrumentation is usually required. The second part of the series concentrates on the role of the dental professional in implementing professional mechanical instrumentation to attempt to reduce the burden of disease further in this patient group. Clinical relevance: Although research continues into which professional techniques for instrumentation are the most successful amongst patients with periodontal disease, very little data specifically explore the needs of patients with learning disabilities, despite their high unmet needs. This paper aims to report on any available data present to produce suggestions for care.

Obstructive jaundice secondary to Morgagni hernia in an infant with Fontan circulation.

The vast majority of congenital diaphragmatic cases present with severe respiratory distress in the immediate neonatal period. Obstructive jaundice secondary to CDH is a rare presenting symptom, previously unreported with Morgagni type hernias which comprise 2% of all CDHs. Although the safety of minimally invasive repair for CDH has been shown previously, there has been little data on its safety for patients with complex congenital cardiac disease. In this case report, we present an 18-month old infant with Fontan circulation presenting with jaundice secondary to Morgagni hernia who ultimately underwent laparoscopic repair. A brief discussion of the safety of laparoscopic surgery in patients post-Fontan is included.

The p-EVES study design and methodology: a randomised controlled trial to compare portable electronic vision enhancement systems (p-EVES) to optical magnifiers for near vision activities in visual impairment.

PURPOSE: To describe the study design and methodology for the p-EVES study, a trial designed to determine the effectiveness, cost-effectiveness and acceptability of portable Electronic Vision Enhancement System (p-EVES) devices and conventional optical low vision aids (LVAs) for near tasks in people with low vision. METHODS: The p-EVES study is a prospective two-arm randomised cross-over trial to test the hypothesis that, in comparison to optical LVAs, p-EVES can be: used for longer duration; used for a wider range of tasks than a single optical LVA and/or enable users to do tasks that they were not able to do with optical LVAs; allow faster performance of instrumental activities of daily living; and allow faster reading. A total of 100 adult participants with visual impairment are currently being recruited from Manchester Royal Eye Hospital and randomised into either Group 1 (receiving the two interventions A and B in the order AB), or Group 2 (receiving the two interventions in the order BA). Intervention A is a 2-month period with conventional optical LVAs and a p-EVES device, and intervention B is a 2-month period with conventional optical LVAs only. RESULTS: The study adopts a mixed methods approach encompassing a broad range of outcome measures. The results will be obtained from the following primary outcome measures: Manchester Low Vision Questionnaire, capturing device 'usage' data (which devices are used, number of times, for what purposes, and for how long) and the MNRead test, measuring threshold print size, critical print size, and acuity reserve in addition to reading speed at high (≈90%) contrast. Results will also be obtained from a series of secondary outcome measures which include: assessment of timed instrumental activities of daily living and a 'near vision' visual functioning questionnaire. A companion qualitative study will permit comparison of results on how, where, and under what circumstances, p-EVES devices and LVAs are used in daily life. A health economic evaluation will provide results on: the incremental cost-effectiveness of p-EVES compared to optical magnifiers; cost-effectiveness; and cost-utility. CONCLUSIONS: The evidence base in low vision rehabilitation is modest and further high quality clinical trials are required to inform decisions on healthcare provision. The p-EVES study findings are anticipated to contribute to this broader evidence requirement, with the methodological issues evident here being relevant to other trials within the field.

Variability in compounding of oral liquids for pediatric patients: a patient safety concern.

OBJECTIVE: To determine the degree in variation of oral liquid pediatric compounding practices in Michigan pharmacies. DESIGN: Cross-sectional survey study. SETTING: All types of inpatient and outpatient pharmacies across the state of Michigan, excluding nuclear pharmacies and long-term care facilities. PARTICIPANTS: 244 Michigan pharmacies. INTERVENTION: An online survey tool was used to assess the current compounding practices of 147 oral liquid pediatric medications. The survey was e-mailed or faxed to hospitals, chain pharmacies, and independent pharmacies. Pharmacists were also mailed a follow-up postcard, and the Michigan Pharmacists Association publicized the project through its journal and annual meeting. MAIN OUTCOME MEASURES: Pharmacy demographics; number of compounding pharmacies; number of medications compounded; awareness of compounding errors; results of compounding errors; and number of concentrations compounded per medication. RESULTS: The majority of respondents were from outpatient pharmacies, but inpatient and other types of pharmacies were also represented. The majority of participating pharmacies compound fewer than five oral liquid medications per week. Awareness of errors was low overall, with no errors believed to result in permanent harm or death. The number of concentrations compounded per medication ranged from 1 to 9, with the majority of pharmacies compounding more than 3 concentrations per medication. CONCLUSION: There is a considerable degree of variation in current oral pediatric liquid compounding practices in Michigan pharmacies. This variability poses a significant risk to patient safety.

A systematic approach to improving medication safety in a pediatric intensive care unit.

Safety and quality improvement are major issues in children's hospitals. Improving pediatric medication safety often takes on a larger role in pediatric units than in adult units due to the larger size differences and dose ranges found in a pediatric intensive care unit. This article reviews the literature and our own experience at the CS Mott Children's Hospital, University of Michigan, to improve medication safety. The issues identified include (1) an effective pediatric medication safety governance structure within a larger hospital, (2) practice standardization strategies for physicians, nurses, and pharmacists, (3) use of pharmacy technicians as unit medication managers, which reduces medication costs and decreases nursing time spent hunting for medications, and (4) methods to improve the safety culture in a pediatric intensive care unit.

Are Special Care Dentistry Services Prepared for a Global Disruption in Healthcare? A Call for a Wider Promotion of Dental Conscious Sedation Training.

Recently, calls for prompt and smart reform of dental education and postgraduate training have been made, reflecting the current global healthcare needs and addressing the most common problems faced by dental care providers. Objectives: Herewith, we propose the enhancement of multilevel dental training in dental conscious sedation (DCS), in order to meet the increasing demands associated with current and post-pandemic times. The temporary suspension of general anaesthesia and hospital-based sedation provision in response to coronavirus disease 2019 (COVID-19) revealed the urgent need for more efficient utilization of a variety of forms of DCS. Whilst the global spread of Severe Acute Respiratory Syndrome novel coronavirus (SARS-CoV-2) has particularly challenged dental sedation teams in community services, the appropriate preparation for similar disruptions in future should be undertaken proactively. In response, dental schools and commissioners are obliged to implement innovations in teaching, with the development of new programs supporting trainer-trainee interactions and focusing on practical sedation skills. Conclusions: The joint efforts of educators, healthcare providers, and commissioners, as well as adequate and robust DCS training utilizing a variety of teaching methods, would allow our profession to face the growing demand for pain and anxiety control measures in light of the current situation, which may increase even further over time. Decision makers are urged to consider making training in DCS more accessible, meeting current healthcare demands, and equally providing essential support for the special dental care sector.

The influence of manual dexterity on reading speed with a hand-held magnifier.

PURPOSE: To determine whether reading speed with a hand-held magnifier is influenced by age-related manual dexterity decline. METHODS: Two groups of normally sighted individuals who were inexperienced in the use of a magnifier were recruited. The older group comprised 15 subjects 60 to 85 years of age, and the younger group consisted of 12 subjects 18 to 30 years of age. Subjects' reading speeds were measured under three conditions: (1) reading 12-point print without a magnifier, (2) reading 3-point print by moving a hand-held magnifier across the page, and (3) using the same print and magnifier, but moving the text under the stationary magnifier. Manual dexterity was assessed using the Jebsen-Taylor hand-function test, upper arm motor coordination with the finger-nose test, and grip strength using the Jamar dynamometer. RESULTS: No significant difference was found between the two age groups in the mean reading speed for unmagnified text. Reading speed with a magnifier was significantly reduced in the older group, but not in the younger group. In both groups, no significant difference was found between moving the magnifier and moving the text during magnifier reading. Grip strength did not differ between the groups. The Jebsen-Taylor and finger-nose tests, however, showed poorer manual dexterity in the older group, and these correlated with the reading speed with the magnifier. CONCLUSIONS: Manual dexterity should be considered as a possible prognostic factor for successful use of a hand-held low-vision aid. It is not known whether the deficit can be overcome with suitable training.

A new measure for the assessment of visual awareness in individuals with tunnel vision.

BACKGROUND: Individuals with a restricted peripheral visual field or tunnel vision (TV) have problems moving about and avoiding obstacles. Some individuals adapt better than others and some use assistive optical aids, so measurement of the visual field is not sufficient to describe their performance. In the present study, we developed a new clinical test called the 'Assessment of Visual Awareness (AVA)', which can be used to measure detection of peripheral targets. METHODS: The participants were 20 patients with TV due to retinitis pigmentosa (PTV) and 50 normally sighted participants with simulated tunnel vision (STV) using goggles. In the AVA test, detection times were measured, when subjects searched for 24 individually presented, one degree targets, randomly positioned in a 60 degrees noise background. Head and eye movements were allowed and the presentation time was unlimited. The test validity was investigated by correlating the detection times with the 'percentage of preferred walking speed' (PPWS) and the 'number of collisions' on an indoor mobility course. RESULTS: In PTV and STV, the detection times had significant negative correlation with the field of view. The detection times had significant positive relations with target location. In the STV, the detection time was significantly negatively correlated with the PPWS and significantly positively correlated with the collisions score on the indoor mobility course. In the PTV, the relationship was not statistically significant. No significant difference in performance of STV was found when repeating the test one to two weeks later. CONCLUSION: The proposed AVA test was sensitive to the field of view and target location. The test is unique in design, quick, simple to deliver and both repeatable and valid. It could be a valuable tool to test different rehabilitation strategies in patients with TV.

Standardization of compounded oral liquids for pediatric patients in Michigan.

PURPOSE: The development, dissemination, and adoption of standard concentrations for compounded oral liquids for pediatric patients in Michigan are described. SUMMARY: A baseline assessment of current practices in Michigan revealed significant variations in the concentrations of commonly used oral liquid medicines for pediatric patients. A statewide collaborative initiative in Michigan was created to standardize the concentrations of compounded oral liquids for pediatric patients. Standard concentrations were proposed and adopted by key stakeholders. These standards were then disseminated across the state to prescribers and pharmacists, with encouragement to voluntarily adopt the standards as a patient safety measure. A follow-up survey was conducted to evaluate adoption of the standards. A total of 263 pharmacists responded to the survey. Standardization of the concentrations of compounded oral liquids in Michigan was welcomed by most pharmacies and is perceived to have reduced the risk for errors at transitions of care for children receiving compounded oral liquids. Awareness of the standardization initiative was acknowledged by 77% of survey respondents, and adoption of the standards was observed to some degree by 57% of survey respondents. In addition, 70% of survey respondents agreed or strongly agreed that adoption of the standards has improved patient safety in Michigan. CONCLUSION: Standard drug concentrations for compounded oral liquids were developed for pediatric patients in Michigan. A survey after dissemination of the recommended standards confirmed general awareness of the initiative and adoption of the standards by a substantial proportion of respondents. Most respondents indicated a belief that creation of the standards improved patient safety.

Inverse agonist activity of agouti and agouti-related protein.

Agouti and agouti-related protein (AgRP) are endogenous antagonists of the melanocortin receptors (MCxR). Previous data showed that recombinant full-length agouti and a synthetic fragment of AgRP, AgRP (83-132), are inverse agonists at the MC1R and MC4R, respectively. This study demonstrates the smaller analogs AgRP (87-120) and ASIP [90-132 (L89Y)], and short peptides Yc[CRFFNAFC]Y and Qc[CRFFRSAC]S are also MC4R inverse agonists. Furthermore, the relative affinity of the series of MC4R ligands for displacement of radiolabeled antagonist 125I-AgRP (86-132) versus radiolabeled agonist 125I-NDP-MSH did not correlate with ligand efficacy, which is more consistent with an induced-fit model than a simple two-state model of MC4R activation. These data shed new light on the determinants and mechanism of inverse agonism at the MC4R.

How effective is low vision service provision? A systematic review.

Visual impairment is a large and growing socioeconomic problem. Good evidence on rehabilitation outcomes is required to guide service development and improve the lives of people with sight loss. Of the 478 potentially relevant articles identified, only 58 studies met our liberal inclusion criteria, and of these only 7 were randomized controlled trials. Although the literature is sufficient to confirm that rehabilitation services result in improved clinical and functional ability outcomes, the effects on mood, vision-related quality of life (QoL) and health-related QoL are less clear. There are some good data on the performance of particular types of intervention, but almost no useful data about outcomes in children, those of working age, and other groups. There were no reports on cost effectiveness. Overall, the number of well-designed and adequately reported studies is pitifully small; visual rehabilitation research needs higher quality research. We highlight study design and reporting considerations and suggest a future research agenda.

Pediatric disorders of the stomach.

Disorders of the stomach represent a significant portion of the practice of pediatric gastroenterology. Controversy still exists in the appropriate management of children with abdominal pain and vomiting and large gaps remain in our understanding of the physiology and pathophysiology of the stomach in children. Nevertheless, we have made significant progress in understanding Helicobacter pylori infection and gastric motility in the pediatric population.

Gastrin biosynthesis in canine G cells.

Gastrin requires extensive posttranslational processing for full biological activity. It is presumed that progastrin is cleaved at pairs of basic amino acids by a prohormone convertase to form a glycine-extended intermediate (G-Gly) that serves as a substrate for peptidyl-glycine alpha-amidating monooxygenase (PAM), resulting in COOH-terminally amidated gastrin. To confirm the nature of progastrin processing in a primary cell line, we performed [(35)S]methionine-labeled pulse-chase biosynthetic experiments in canine antral G cells. Radiolabeled progastrin reached a peak earlier than observed for G-Gly or amidated gastrin. G-Gly radioactivity accumulated in G cells and preceded the appearance of radioactivity in amidated gastrin. The conversion of G-Gly to amidated gastrin was enhanced by the PAM cofactor ascorbic acid. To determine whether one member of the prohormone convertase family (PC2) was responsible for progastrin cleavage, G cells were incubated with PC2 antisense oligonucleotide probes. Cells treated with antisense probes had reduced PC2 expression, an accumulation of radiolabeled progastrin, and a delay in the formation of amidated gastrin. Progastrin in antral G cells is cleaved via PC2 to form G-Gly that is converted to amidated gastrin via the actions of PAM.