Optimising Health-Related Quality of Life in Children With Osteogenesis Imperfecta.

Osteogenesis Imperfecta is a rare, hereditary bone condition with an incidence of 1/15,000-20,000. Symptoms include bone fragility, long bone deformity, scoliosis, hypermobility, alongside secondary features such as short stature, basilar invagination, pulmonary and cardiac complications, hearing loss, dentinogenesis imperfecta and malocclusion. Osteogenesis Imperfecta can have a large impact on the child and their family; this impact starts immediately after diagnosis. Fractures, pain, immobility, hospital admissions and the need for equipment and adaptations all influence the health-related quality of life of the individual and their family. This narrative review article aims to examine the impact the diagnosis and management of osteogenesis imperfecta has on the health-related quality of life of a child. It will touch on the effect this may have on the quality of life of their wider family and friends and identify strategies to optimise health-related quality of life in this population. Optimising health-related quality of life in children with Osteogenesis Imperfecta is often a complicated, multifaceted journey that involves the child, their extended family, school, extracurricular staff and numerous health professionals.

Protective mechanism of dried blood spheroids: stabilization of labile analytes in whole blood, plasma, and serum.

Three-dimensional (3D) dried blood spheroids form when whole blood is deposited onto hydrophobic paper and allowed to dry in ambient air. The adsorbed 3D dried blood spheroid present at the surface of the hydrophobic paper is observed to offer enhanced stability for labile analytes that would otherwise degrade if stored in the traditional two-dimensional (2D) dried blood spot method. The protective mechanism for the dried blood spheroid microsampling platform was studied using scanning electron microscopy (SEM), which revealed the presence of a passivation thin film at the surface of the spheroid that serves to stabilize the interior of the spheroid against environmental stressors. Through time-course experiments based on sequential SEM analyses, we discovered that the surface protective thin film forms through the self-assembly of red blood cells following the evaporation of water from the blood sample. The bridging mechanism of red blood cell aggregation is evident in our experiments, which leads to the distinct rouleau conformation of stacked red blood cells in less than 60 min after creating the blood spheroid. The stack of self-assembled red blood cells at the exterior of the spheroid subsequently lyse to afford the surface protective layer detected to be approximately 30 µm in thickness after three weeks of storage in ambient air. We applied this mechanistic insight to plasma and serum to enhance stability when stored under ambient conditions. In addition to physical characterization of these thin biofilms, we also used paper spray (PS) mass spectrometry (MS) to examine chemical changes that occur in the stored biofluid. For example, we present stability data for cocaine spiked in whole blood, plasma, and serum when stored under ambient conditions on hydrophilic and hydrophobic paper substrates.

Determining Surface Energy of Porous Substrates by Spray Ionization.

We have developed a new spray-based method for characterizing surface energies of planar, porous substrates. Distinct spray modes (electrospray versus electrostatic spray), from the porous substrates, occur in the presence of an applied DC potential after wetting with solvents of different surface tension. The ion current resulting from the spray process is maximized when the surface energy of the porous substrate approaches the surface tension of the wetting solvent. By monitoring the selected ion current (e.g., benzoylecgonine, m/z 290 → 168) with a mass spectrometer or the total ion current with an ammeter, we determined the solvent surface tension yielding the maximum ion current to indicate the surface energy of the solid. Detailed evaluations using polymeric substrates of known surface energies enabled effective calibration of the approach that resulted in the correct estimation of the surface energy of hydrophobic paper substrates prepared by gas-phase silanization. A three-parameter empirical model suggests that the experimentally observed ion current profile is governed by differential partitioning of analyte controlled by the interfacial forces between the wetting solvent and the porous substrate.

High Engagement in Care in a Pediatric Medical Home for Children Impacted By Parental Substance Use.

The opioid epidemic has heavily affected adults of childbearing age, leading to thousands of children impacted by parental substance use. Few programs provide longitudinal support to these children. This article describes an innovative pediatric medical home model for substance-impacted children and their families, at an urban safety-net hospital. The team-based program directly serves children, and also devotes significant resources to parental health and recovery. In the program's first 3 years, 95% of enrollees were engaged in care, meeting the American Academy of Pediatrics' recommended periodicity schedule for preventive health visits. On-time receipt of childhood vaccines ranged from 95% (pneumococcal conjugate) to 100% (human papilloma virus). The program's high engagement in care shows promise in engaging vulnerable families over time. Future work should explore how to engage children from more diverse backgrounds, and should examine whether the model impacts other indicators of health and well-being for children impacted by parental substance use.

Dermoid Inclusion Cyst Following Percutaneous Needle Fasciotomy: A Novel Complication.

We present the case of a fit and well 62-year-old male with Dupuytren's disease in the right hand who underwent percutaneous needle fasciotomy (PNF) for a moderate flexion contracture of the right little finger. 18 months later he developed a pain-free soft tissue swelling at the distal previous needling site. A fasciectomy procedure identified a cyst within the pre-tendinous cord, which was confirmed as a dermoid inclusion cyst on histological analysis. Dermoid inclusion cysts may occur in the hands at the site of penetrating trauma but we are unaware of any report of an inclusion cyst at the site of PNF surgery. We present this unique case of a dermoid inclusion cyst following percutaneous needle fasciotomy as a novel complication.

Towards standardization of training and practice of reconstructive microsurgery: an evidence-based recommendation for anastomosis thrombosis prophylaxis.

BACKGROUND: Despite significant improvements in survival rates, free flap failures still occur even in experienced hands and are most commonly due to arterial or venous thrombosis. In the absence of an evidence-based guideline on the prevention of thrombosis, we reviewed the literature to assess the evidence base for commonly used interventions aimed at its prevention. METHODS: A comprehensive literature search was performed using the following keywords "free flap" and microsurgery with "pre-operative screening," "prevention of thrombosis," "ketorolac," "heparin," "low molecular weight heparin," "aspirin," "dextran," and "statins." RESULTS: Thirteen clinical studies were included in this review. No high-level evidence is available to support any perioperative or postoperative interventions aimed at reducing the risk of flap thrombosis. CONCLUSIONS: Higher level studies are needed to investigate the clinical use of antithrombotic medications in microsurgery; however, given the small failure rates in modern practice, these will need to be large multicenter trials in order to reach sufficient power.Level of Evidence: Level III, risk/prognostic study.

Lightning injuries in Northern Ireland.

INTRODUCTION: Lightning injuries are uncommon in Northern Ireland (NI) with scarce reports detailing incidence and local experience. We present a case study of 3 patients involved in a single lightning strike with a review of the incidence of similar injuries in the province. METHODS: Data from TORRO's National Lightning Incidents Database between 1987 and 2016 (30 years) were searched to identify victims of lightning injuries in NI. Information on 3 patients with lightning injuries that were managed in our regional burns and plastic surgery service was collected and examined. A supplementary search in hospital records was conducted over the last 20 years to identify additional data. RESULTS: Prior to our study, 6 victims of lightning injuries were identified of whom 5 survived and 1 died. Our 3 patients comprised of 2 children and 1 accompanying adult. All survived but the adult suffered cardiac arrest and required a prolonged period of cardiopulmonary resuscitation. CONCLUSION: While lightning injuries are rare in NI, this is the first report of more than one person affected by a single lightning incident in the province. In our limited experience, immediate public response and prolonged cardiopulmonary resuscitation efforts facilitated by automated defibrillators result in a favourable outcome.

Laparoscopic appendicectomy in obese is associated with improvements in clinical outcome: systematic review.

BACKGROUND: Obese patients with general surgical emergencies provide unique challenges to the emergency surgical teams. Acute appendicitis is the most common adult acute surgical emergency encountered in practice. This systematic review evaluates the role of laparoscopic appendicectomy in obese by comparing the outcomes of laparoscopic appendicectomy in obese versus non-obese and the laparoscopic versus open appendicectomy in obese patients. METHODS: Relevant comparative studies were identified from the Cochrane Central Register of Controlled Trials, MEDLINE, Embase and PubMed (1990-2013). Primary outcomes evaluated were mortality, overall morbidity and duration of surgery. Secondary outcomes evaluated were superficial (superficial wound infection) and deep surgical site infection (intra-abdominal abscesses), conversion to open surgery, and cost of the procedure. RESULTS: Seven retrospective cohort studies and one prospective randomized controlled trial met the inclusion criteria. There was no statistically significant difference in the primary and secondary outcomes between the obese and non-obese patients undergoing laparoscopic appendicectomy. Laparoscopic appendicectomy in obese patients is associated with reduced mortality (risk ratio [RR]: 0.19 (95% CI 0.12-0.30), reduced overall morbidity (RR: 0.49 (95% CI: 0.47-0.51)), reduced superficial wound infections (RR: 0.27 (95% CI 0.21-0.35)), shorter operating times and post-operative length of hospital stay, compared to open appendicectomy. Methodological quality of the included studies is low. CONCLUSION: Laparoscopic appendicectomy appears to be a safer alternative approach to open surgery in obese adult patents. There is no significant difference in the outcomes between the obese and non-obese patients undergoing laparoscopic appendicectomy.

Chemical investigation of predator-deterred macroalgae from the Antarctic peninsula.

Chemical investigation of five Antarctic macroalgae whose tissues and crude extracts displayed ecologically relevant feeding deterrence in field bioassays was performed. Eleven compounds were characterized from the three red algae studied, of which four (1-3 and 9) were previously unreported, and four compounds were found from two brown algae, two (12 and 14) of which are new natural products. Several of these pure compounds have been individually investigated in ecological and/or pharmacological bioassays.