What to measure in biliary atresia research: study protocol for developing a core outcome set.

AIM: Extrahepatic biliary atresia is a rare disorder. This creates challenges in the quality and quantity of research conducted. This issue is exacerbated by the potential heterogeneity in the reported outcomes in research examining the management of biliary atresia. A core outcome set is required to standardise reporting on the management of biliary atresia in research, facilitate systematic reviews that include outcomes of greatest importance to patients and clinicians, and to evaluate the quality of the existing evidence base on the management of biliary atresia. METHODS: A list of all potential outcomes will be developed through a systematic review of the literature. This list will be refined through a three-stage Delphi approach, involving key stakeholders in the management of biliary atresia. This will include patients and their parents, clinicians, nurses and allied health professionals. In this way, outcomes will be prioritised into a set of consensus core outcomes. CONCLUSION: The development of a core outcome set in biliary atresia management is needed to guide future research and assist in evaluating the quality of existing research. ETHICS AND DISSEMINATION: Ethical approval has been granted by the Children's Health Queensland Hospital and Health Service Human Research Ethics Committee (HREC), Ref: HREC/20/QCHQ/62448. Results of the study will be published in an open access format.

Incidental finding of heterotopic supradiaphragmatic liver.

Here we describe a case of heterotopic, supradiaphragmatic liver in a 65-year-old woman who was referred for investigation of a soft tissue gallbladder mass. Contrast-enhanced magnetic resonance imaging revealed adenomyomatosis of the gallbladder and supradiaphragmatic accessory liver tissue. This is a remarkably rare normal variant.

Hepatic tuberculosis masquerading as cholangiocarcinoma: an unusual differential for a liver mass.

A 54-year-old woman presented with an incidentally identified asymptomatic liver lesion with imaging characteristics suspicious for malignancy. She underwent a left hemihepatectomy for presumed cholangiocarcinoma. Histopathology revealed granulomas with microbiological investigations later revealing a diagnosis of isolated hepatic tuberculosis. There were no pulmonary or other disease sites identified. The patient has been medically managed for primary hepatic tuberculosis and remains well postoperatively. This case identifies a rare differential for a liver mass that needs to be considered in the clinicians' workup.

A first case of hepatocellular carcinoma in the baboon (Papio spp.) placenta.

We present a case of hepatocellular carcinoma (HCC) in the placenta of healthy baboon (Papio spp.). Grossly, the fetal, maternal, and placental tissues were unremarkable. Histologically, the placenta contained an unencapsulated, poorly demarcated, infiltrative, solidly cellular neoplasm composed of cells that resembled hepatocytes. The neoplastic cells were diffusely positive for vimentin and focally positive for Ae1/Ae3, Arginase -1, glutamine synthetase, and CD10, and negative for ER, vascular markers (CD31 and D240), S100, glypican, C-reactive protein, FABP, desmin, and beta-catenin; INI1 positivity was similar to non-neoplastic tissues. The case likely represents a unique subtype of HCC.

Intentional self-inflicted and peer-inflicted aerosol skin injuries called 'frosties': Cohort series and systematic literature review.

BACKGROUND AND OBJECTIVES: 'Frosties' are deliberate cold skin burns caused by an aerosol device. The aim of this article was to examine our own cohort, and those previously published, to identify the key features of patients presenting with frosties and inform appropriate early clinical interventions. METHOD: We compared cases in our dataset that occurred between 1 January 2013 and 30 June 2017 with those reported in the literature, focusing on seven domains: sex, age at injury, days to presentation, first aid, depth of injury and outcome. RESULTS: The median patient age was 13 years; 70.5% were female. Adequate first aid was not reported in any patient. Where recorded, the median time to presentation to a health service was six days. Where severity of injury was recorded, 13 of 37 cases (35.1%) were full thickness, and 10 patients received a split thickness skin graft. Two subgroups were identified: cluster injuries and psychological distress. DISCUSSION: Cluster injuries occur as the result of a mutual 'test of courage'. Solo injuries may point to underlying psychological distress. Frosties frequently result in significant burn injuries and often require skin grafting. The severity of frosties is underappreciated and, as a consequence, treatment, through first aid or presentation to a health service, is delayed or absent. General practitioners should be familiar with the appearance of frosties in order to identify them in unrelated consultations.

Giant congenital melanocytic nevi and malignant transformation: a case for early radical intervention.

The purpose of this paper is to highlight the risk of early malignant transformation in infants with giant congenital melanocytic nevi (GN) and demonstrate the potential for earlier intervention with aggressive surgery. We describe the case of a child born with a GN who developed a metastatic melanoma early in life, despite early commencement of resection of the nevus. This is contrasted against a second case of a child in which a more radical management was conducted. Despite early commencement of serial resection of the GN, the first child in this series died of metastatic melanoma prior to complete excision of the nevus. With the second child, radical excision combined with the use of Integra™ and negative pressure wound therapy allowed total removal of the GN within the first 6 months of life.

Don't take the NHS for granted.

In my final year of training I undertook an elective placement in Nepal, where I spent three weeks in an emergency department in Kathmandu.

A new disaster victim identification management strategy targeting "near identification-threshold" cases: Experiences from the Boxing Day tsunami.

The international disaster victim identification (DVI) response to the Boxing Day tsunami, led by the Royal Thai Police in Phuket, Thailand, was one of the largest and most complex in DVI history. Referred to as the Thai Tsunami Victim Identification operation, the group comprised a multi-national, multi-agency, and multi-disciplinary team. The traditional DVI approach proved successful in identifying a large number of victims quickly. However, the team struggled to identify certain victims due to incomplete or poor quality ante-mortem and post-mortem data. In response to these challenges, a new 'near-threshold' DVI management strategy was implemented to target presumptive identifications and improve operational efficiency. The strategy was implemented by the DNA Team, therefore DNA kinship matches that just failed to reach the reporting threshold of 99.9% were prioritized, however the same approach could be taken by targeting, for example, cases with partial fingerprint matches. The presumptive DNA identifications were progressively filtered through the Investigation, Dental and Fingerprint Teams to add additional information necessary to either strengthen or conclusively exclude the identification. Over a five-month period 111 victims from ten countries were identified using this targeted approach. The new identifications comprised 87 adults, 24 children and included 97 Thai locals. New data from the Fingerprint Team established nearly 60% of the total near-threshold identifications and the combined DNA/Physical method was responsible for over 30%. Implementing the new strategy, targeting near-threshold cases, had positive management implications. The process initiated additional ante-mortem information collections, and established a much-needed, distinct "end-point" for unresolved cases.

Simulation in a disaster drill: comparison of high-fidelity simulators versus trained actors.

OBJECTIVES: High-fidelity patient simulation provides lifelike medical scenarios with real-time stressors. Mass casualty drills must construct a realistic incident in which providers care for multiple injured patients while simultaneously coping with numerous stressors designed to tax an institution's resources. This study compared the value of high-fidelity simulated patients with live actor-patients. METHODS: A prospective cohort study was conducted during two mass casualty drills in December 2006 and March 2007. The providers' completion of critical actions was tested in live actor-patients and simulators. A posttest survey compared the participants' perception of "reality" between the simulators and live actor victims. RESULTS: The victims (n = 130) of the mass casualty drill all had burn-, blast-, or inhalation-related injuries. The participants consisted of physicians, residents, medical students, clerks, and paramedics. The authors compared the team's execution of the 136 critical actions (17 critical actions x 8 scenarios) between the simulators and the live actor-patients. Only one critical action was missed in the simulator group and one in the live actor group, resulting in a miss rate of 0.74% (95% confidence interval [CI] = 0.01% to 4.5%). All questionnaires were returned and analyzed. The vast majority of participants disagreed or strongly disagreed that the simulators were a distraction from the disaster drill. More than 96% agreed or strongly agreed that they would recommend the simulator as a training tool. The mean survey scores for all participants demonstrated agreement that the simulators closely mimicked real-life scenarios, accurately represented disease states, and heightened the realism of patient assessment and treatment options during the drill with the exception of nurse participants, who agreed slightly less strongly. CONCLUSIONS: This study demonstrated that simulators compared to live actor-patients have equivalent results in prompting critical actions in mass casualty drills and increase the perceived reality of such exercises.

Use of gadolinium for hysterosalpingography in iodine allergic women: a case-control study.

OBJECTIVE: To examine the performance of gadolinium in iodine-sensitive women undergoing hysterosalpingography. DESIGN: Case-control study. SETTING: Odessa Regional Hospital, Texas. PATIENT(S): Seven women, three with no allergies to iodinated contrast medium (controls) and four with known allergies. INTERVENTION(S): Use of gadolinium in women with known allergies to iodinated contrast medium. MAIN OUTCOME MEASURE(S): How does gadolinium compare as a diagnostic tool in hysterosalpingogram versus iodinated contrast medium? RESULT(S): The images of the uterus and unobstructed fallopian tubes produced by gadolinium appear to yield equally diagnostic results compared with images produced by iodinated contrast medium. CONCLUSION(S): It appears that hysterosalpingogram with gadolinium is a viable option for patients that are allergic to iodinated contrast medium.