The pruritus severity scale-a novel tool to assess itch in burns patients.

BACKGROUND: Pruritus assessment is difficult due to the varying subjective nature of the experience. There have been several validated tools described to quantify the severity of itch, however these tools fail to provide a comprehensive assessment or are too cumbersome and therefore lack usability. Our novel burn assessment tool, "The Pruritus Severity Scale" (PSS) allows for accurate quantification of itch components. The aim of this study was to assess its use in the burns population. METHODS: A prospective observational study was conducted on all patients over five years of age with a burn injury over a six month period. Patients underwent subjective evaluation of their itch as determined by two validated scores, the Visual Analogue Scale (VAS) and the Itch Man scale (IMS) and in addition to the PSS. The pruritus severity scale was correlated with the previously validated scoring methods using bi-variate correlations. RESULTS: Twenty-two patients were included in the study. The most common cause of injury was due to flame burn. The mean total body surface area was 6.5% (range: 1-26%). Both the IMS and the VAS positively correlated well with the PSS. The Spearman Coefficient for the PSS vs IMS was 0.81, R2 = 0.65 (P<0.05). The Spearman Coefficient for the PSS vs VAS was 0.87 (R2 = 0.76 (P<0.01)). There is a positive linear relationship between our novel scoring methods and the currently validated methods, indicating its validity as a burn assessment too. CONCLUSION: The Pruritus Severity Scale was shown to be an accurate, objective tool that was able to effectively record the patient's experience of itch. We believe that this novel score is quick, easy to use and allows for more comprehensive assessment than other short assessment tools.

Prefibrotic Myelofibrosis Presenting with Multiple Cerebral Embolic Infarcts and the Rare MPL W515S Mutation.

Acquired, activating mutations of MPL W515 are recognised driver mutations of the myeloproliferative neoplasms (MPN), namely, essential thrombocythemia and primary myelofibrosis. The most common mutation at this codon is W515L with several other mutations also described at a lower frequency. Of these less common mutations, MPL W515S has only been reported sporadically with limited information on clinicopathological associations. We describe the case of an elderly man with persistent thrombocytosis presenting with an ischemic cerebral event. Bone marrow biopsy showed evidence of prefibrotic myelofibrosis with targeted sequencing demonstrating the presence of the rare MPL W515S mutation. Thrombolytic and cytoreductive therapies resulted in a favorable outcome and follow-up. This case provides additional, necessary, and phenotypic data for the rare MPN-associated MPL W515S mutation.

Molecular Monitoring in Adult Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia with the Variant e13a3 BCR-ABL1 Fusion.

Monitoring BCR-ABL1 transcript levels in patients with Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph+ ALL) is a widely adopted method to assess response to therapy. However, a small minority of Ph+ ALL patients express variant BCR-ABL1 transcript types, usually due to splicing of alternative BCR or ABL1 exons. Whether patients expressing these rare, variant BCR-ABL1 transcripts have a distinct phenotype or response to therapy is not known due to the limited number of reported cases. Here, we report the presenting features of Ph+ ALL in a young adult with a variant e13a3 BCR-ABL1 fusion. Molecular monitoring reflected the disease response from diagnosis through allogeneic stem cell transplantation which resulted in undetectable e13a3 BCR-ABL1 transcripts. This case highlights the value of molecular monitoring in Ph+ ALL patients with variant BCR-ABL1 transcripts and the requirement for standardization of such assays.

The effects of stem cells on burn wounds: a review.

INTRODUCTION: Stem cell therapy application is at the vanguard of regenerative medicine across all medical disciplines. Stem cells are of special interest in burn wounds, as they have multiple potential indications for application; including - accelerating wound healing, improving skin regeneration to incorporate skin appendages, reducing fibrosis and improving scarring. METHODS: A literature review was performed using both MeSH and keyword searches of PubMed to identify all potentially suitable publications. Search criteria were restricted to the English language, but acceptable English translations were sought for inclusion. Inclusion dates were from 2003 up until and including 2017. Studies included looked at stem cells in burn wounds only. RESULTS: There were 692 potentially suitable publications of which 72 were included for review. These included a systematic reviews and original research articles. CONCLUSIONS: Stem cells accelerate burn wound healing by inducing neo-angiogenesis, collagen deposition and granulation tissue formation. They modulate the inflammatory response and reduce the risk of infection. They can regenerate skin appendages and halt he zone of stasis in acute burn injury. However with these pre-clinical animal model studies we must be cautious with our interpretation of this novel therapy.

New technologies in global burn care - a review of recent advances.

There have been truly incredible strides in the standard of burn care. The mortality from burn injuries has more than halved since the 1950s, making it hugely unique among major diseases of the developed world. There can be no doubt technology and technological advances have driven this process, dramatically improved every aspect of burn care, from the intensive care management, the surgical management, management of the healing wound to the post burn sequelae, specifically scar management. This review aims to identify key technological advances in burns, in both the developed and developing world, and evaluate their influence in the continued strategy to improve the standards of global burn care.

Postpartum Cardiomyopathy and Considerations for Breastfeeding.

Postpartum cardiomyopathy (PPCM) is a rare condition that develops near the end of pregnancy or in the months after giving birth, manifesting as heart failure secondary to left ventricular systolic dysfunction. Clinical progression varies considerably, with both end-stage heart failure occurring within days and spontaneous recovery seen. Treatment pathways for heart failure are well established, but the evidence about the safety of medicines passed to infants during breastfeeding is scarce and mainly poor; this often leads to an incorrect decision that a mother should not breastfeed. Given its benefits to both mother and infant, breastfeeding should not routinely be ruled out if the mother is taking heart failure medication but the consequences for the infant need to be considered. An informed risk assessment to minimise potential harm to the infant can be carried out using the evidence that is available along with a consideration of drug properties, adverse effects, paediatric use and pharmacokinetics. In most cases, risks can be managed and infants can be monitored for potential problems. Breastfeeding can be encouraged in women with cardiac dysfunction with PPCM although treatment for the mother takes priority with breastfeeding compatibility being the secondary consideration. International research is continuing to establish efficacy and safety of pharmacotherapy in PPCM.

The Use of Thromboelastography to Measure the Influence Inclusion of a Local Anesthetic Agent has on the Mechanical and Kinetic Properties of Fibrin.

CONTEXT: Delivery of slow-release local anesthesia has considerable potential for postoperative analgesia. Fibrin gel has shown huge potential for drug delivery, but has not been fully investigated for the delivery of local anesthetics nor has whether incorporation of anesthetic drugs into fibrin alters its mechanical properties. AIMS: This study aimed to evaluate the effects of bupivacaine inclusion on the mechanical and kinetic properties of fibrin as measured by thromboelastography (TEG). MATERIALS AND METHODS: Serial dilutions of fibrinogen with thrombin were tested with TEG to identify the optimal concentrations to give reproducible results. Following this, fibrinogen samples diluted with bupivacaine 0.5% in place of normal saline (also 1:20 dilution) were added to thrombin to assess what influence this had on clot strength and kinetics as measured by TEG values (with R, K, and α angle relating to clot kinetics and MA and G (or shear elastic modulus strength) relating to clot strength). RESULTS: The mean values yielded for R were higher and lower for α angle, suggesting that the inclusion of bupivacaine produced a fibrin clot at a slower rate. The values for MA and G were both lower when bupivacaine was included, suggesting inclusion of the local anaesthetic also resulted in a fibrin clot of inferior strength. These results were not statistically significant. CONCLUSION: Although TEG failed to consistently measure these properties, the results suggest that inclusion of local anesthetic affects the clotting process of fibrin, potentially interfering with its ability to function as a sealant, adhesive, or hemostat.

The smartphone: an effective tool in transitioning patients from mole mapping to mole excision.

We describe a useful strategy to accurately identify the correct 'atypical' nevus for excision in patients with multiple nevi. We believe this is an effective strategy and eliminates risk of any incorrect procedures being performed, and have used this technique on over 50 patients to date. As the patient stores the images on their own phone, there is no breach of data protection. We find patients prefer this method as opposed to giving others permission to store their sensitive information.

Novel methods of local anesthetic delivery in the perioperative and postoperative setting-potential for fibrin hydrogel delivery.

The benefits of high-quality postoperative analgesia are well documented and include earlier mobilization, fewer respiratory and cardiovascular complications, and shorter hospital stay. Local anesthesia-based acute pain regimens are at worst equal to and at best superior to opiate-based regimens from the perspective of analgesia. A multimodal approach limiting opioids by combining with local anesthetics has additional beneficial effect on outcomes such as nausea and vomiting, pruritus, gastrointestinal function, respiratory complications, and neutrophil function. Wound catheters providing continuous infiltration of local anesthetics offer a rational approach to effective perioperative analgesia, but their use is limited by a short duration of action. There is an identified need for further methods to optimize longer-acting delivery of these agents. This article reviews current and evolving longer-acting techniques and their limitations with particular focus on the potential advantages of a fibrin hydrogel-based system.

Utility of Orchidometric Parameters for Assessing Sexual Maturation in Male Cynomolgus Macaques (Macaca fascicularis).

Testicular volume is one of several parameters that have been used in preclinical toxicology to facilitate the identification of sexually mature male cynomolgus macaques when semen evaluation is unavailable. Furthermore, testicular volume provides additional information to pathologists to aid in the interpretation of microscopic findings. Orchidometry has been proposed as a useful tool for assessing testicular volume. To assess its utility for this purpose, we used orchidometry to measure testicular volume in untreated control male cynomolgus macaques during preclinical toxicology studies. Additional parameters including age, body weight, testicular weight, serum testosterone, and testicular histology were also evaluated. Serum inhibin B and the diameter of histologic testicular sections were assessed to determine whether they might provide any additional corroborative evidence for differentiating stages of sexual maturity in males. Orchidometry was easy to use in sedated or awake macaques and, in combination with testicular histology, enabled the establishment of cut-off values by which sexually mature male cynomolgus macaques can be identified with a high degree of confidence. The relative utility of the parameters examined for discriminating sexually mature and immature males was testicular volume ≥ serum testosterone > body weight > age; for differentiation of sexually mature and peripubertal males the order was testicular volume ≥ body weight > serum testosterone > age. Testicular weight and the diameter of histologic testicular sections provided corroborative information for discriminating stages of sexual maturity. Serum inhibin B was of little value in helping to differentiate the different stages of sexual maturation evaluated in this study.

Spectrum of mutations in a cohort of UK patients with ADA deficient SCID: Segregation of genotypes with specific ethnicities.

Severe combined immunodeficiency (SCID) arises from a number of different genetic defects, one of the most common being mutations in the gene encoding adenosine deaminase (ADA). In the UK, ADA deficient SCID compromises approximately 20% of all known cases of SCID. We carried out a retrospective analysis of the ADA gene in 46 known ADA deficient SCID patients on whom DNA had been stored. Here, we report a high frequency of two previously reported mutations and provide a link between the mutations and patient ethnicity within our patient cohort. We also report on 9 novel mutations that have been previously unreported.

Correlation of B-type natriuretic peptide level to 6-min walk test performance in patients with left ventricular systolic dysfunction.

BACKGROUND: B-type natriuretic peptide (BNP) is a neurohormone that can be measured in blood and is useful in patients with congestive heart failure (CHF). We compared whole blood BNP concentrations to distance walked during a 6-min walk test in patients with CHF. METHODS: Forty-four patients with CHF underwent a 6-min walk test. The distance walked was compared to the BNP concentration on blood collected prior to the walk test. Patients were followed for 16 +/- 2.8 months after testing. RESULTS: A significant correlation was observed between the BNP concentration and the distance walked (r = -0.47, p < 0.001). One patient without congestion died suddenly. Two patients died of progressive heart failure, and two other patients underwent cardiac transplantation. Each of the latter four patients had high BNP concentrations (median 1080 ng/l) and walked short distances (median 183 m). This study indicates that the BNP concentration in blood correlates inversely with the degree of physical capability of patients with heart failure. CONCLUSIONS: The BNP concentration could be used as an alternative to the 6-min walk test to assess the severity of heart failure. The assay for BNP is non-invasive, inexpensive, and results are available at the bedside or in a heart failure clinic.