Diagnosis and Management of a Chronic Lower-Limb Wound in a Patient with Felty Syndrome.

ABSTRACT: The authors report the case of a 55-year-old patient with a chronic lower-limb wound thought to be secondary to vasculitis. This case illustrates the importance of maintaining a high index of suspicion for vasculitic ulcers in patients with autoimmune disease. Management considerations in this context are also discussed.

Morphologic and Molecular Findings in Myxoid Hepatic Adenomas.

Myxoid hepatic adenomas are a rare subtype of hepatic adenomas with distinctive deposition of extracellular myxoid material between the hepatic plates. A total of 9 cases were identified in 6 women and 3 men with an average of 59±12 years. The myxoid adenomas were single tumors in 5 cases and multiple in 4 cases. In 1 case with multiple adenomas, the myxoid adenoma arose in the background of GNAS-mutated hepatic adenomatosis. Myxoid hepatic adenomas had a high frequency of malignant transformation (N=5 cases). They were characterized at the molecular level by HNF1A inactivating mutations, leading to loss of LFABP protein expression. In addition, myxoid adenomas had recurrent mutations in genes within the protein kinase A (PKA) pathway or in genes that regulate the PKA pathway: GNAS, CDKN1B (encodes p27), and RNF123. In sum, myxoid adenomas are rare, occur in older-aged persons, have a high risk of malignant transformation, and are characterized by the combined inactivation of HNF1A and additional mutations that appear to cluster in the PKA pathway.

Factors associated with COVID-19 related hospitalisation, critical care admission and mortality using linked primary and secondary care data.

BACKGROUND: It is important that population cohorts at increased risk of hospitalisation and death following a COVID-19 infection are identified and protected. OBJECTIVES: We identified risk factors associated with increased risk of hospitalisation, intensive care unit (ICU) admission and mortality in inner North East London (NEL) during the first UK COVID-19 wave. METHODS: Multivariate logistic regression analysis on linked primary and secondary care data from people aged 16 or older with confirmed COVID-19 infection between 01/02/2020 and 30/06/2020 determined odds ratios (OR), 95% confidence intervals (CI) and P-values for the association between demographic, deprivation and clinical factors with COVID-19 hospitalisation, ICU admission and mortality. RESULTS: Over the study period, 1781 people were diagnosed with COVID-19, of whom 1195 (67%) were hospitalised, 152 (9%) admitted to ICU and 400 (23%) died. Results confirm previously identified risk factors: being male, or of Black or Asian ethnicity, or aged over 50. Obesity, type 2 diabetes and chronic kidney disease (CKD) increased the risk of hospitalisation. Obesity increased the risk of being admitted to ICU. Underlying CKD, stroke and dementia increased the risk of death. Having learning disabilities was strongly associated with increased risk of death (OR = 4.75, 95% CI = [1.91, 11.84], P = .001). Having three or four co-morbidities increased the risk of hospitalisation (OR = 2.34, 95% CI = [1.55, 3.54], P < .001; OR = 2.40, 95% CI = [1.55, 3.73], P < .001 respectively) and death (OR = 2.61, 95% CI = [1.59, 4.28], P < .001; OR = 4.07, 95% CI = [2.48, 6.69], P < .001 respectively). CONCLUSIONS: We confirm that age, sex, ethnicity, obesity, CKD and diabetes are important determinants of risk of COVID-19 hospitalisation or death. For the first time, we also identify people with learning disabilities and multi-morbidity as additional patient cohorts that need to be actively protected during COVID-19 waves.

Diagnostic laboratory standardization and validation of platelet transmission electron microscopy.

Platelet transmission electron microscopy (PTEM) is considered the gold standard test for assessing distinct ultrastructural abnormalities in inherited platelet disorders (IPDs). Nevertheless, PTEM remains mainly a research tool due to the lack of standardized procedures, a validated dense granule (DG) count reference range, and standardized image interpretation criteria. The aim of this study was to standardize and validate PTEM as a clinical laboratory test. Based on previously established methods, we optimized and standardized preanalytical, analytical, and postanalytical procedures for both whole mount (WM) and thin section (TS) PTEM. Mean number of DG/platelet (plt), percentage of plts without DG, platelet count (PC), mean platelet volume (MPV), immature platelet fraction (IPF), and plt light transmission aggregometry analyses were measured on blood samples from 113 healthy donors. Quantile regression was used to estimate the reference range for DG/plt, and linear regression was used to assess the association of DG/plt with other plt measurements. All PTEM procedures were standardized using commercially available materials and reagents. DG interpretation criteria were established based on previous publications and expert consensus, and resulted in improved operator agreement. Mean DG/plt was stable for 2 days after blood sample collection. The median within patient coefficient of variation for mean DG/plt was 22.2%; the mean DG/plt reference range (mid-95th %) was 1.2-4.0. Mean DG/plt was associated with IPF (p = .01, R2 = 0.06) but not age, sex, PC, MPV, or plt maximum aggregation or primary slope of aggregation (p > .17, R2 < 0.02). Baseline ultrastructural features were established for TS-PTEM. PTEM was validated using samples from patients with previously established diagnoses of IPDs. Standardization and validation of PTEM procedures and interpretation, and establishment of the normal mean DG/plt reference range and PTEM baseline ultrastructural features, will facilitate implementation of PTEM as a valid clinical laboratory test for evaluating ultrastructural abnormalities in IPDs.

Respiratory Therapy Faculty Knowledge of and Attitudes Toward Interprofessional Education.

BACKGROUND: Interprofessional education (IPE) improves collaboration and patient care through joint education between health professions. Respiratory therapy (RT) faculty were surveyed to evaluate their knowledge and attitudes toward IPE. We report current opportunities for IPE from faculty and compare responses from associate's, bachelor's, and master's degree programs and profit versus nonprofit institutions. METHODS: We developed an online survey based on IPE literature and questions modified for the RT discipline. The survey was distributed by email to 874 faculty from the Commission on Accreditation for Respiratory Care accredited programs. RESULTS: The response rate was 33%. Faculty identified IPE as an important component of RT education (n = 207, 80%) but reported challenges in integrating IPE into current curriculum. Overall, communication was ranked as the most important IPE competency (n = 104, 39%) and ethics least important (n = 131, 49%). When asked how many credit hours are required to teach IPE, 48% of respondents reported that they were unsure of an appropriate time requirement. Significant differences between associate's and bachelor's/master's degree program faculty were found on the following topics: institutional resources needed for IPE (P < .001), faculty availability (P < .001), curriculum availability for IPE (P = .02), and importance of including IPE at academic health center campuses (P < .001). CONCLUSIONS: IPE is recognized as an important component of RT education by all faculty respondents. However, significant differences in knowledge and attitudes toward IPE exist between faculty in associate's versus bachelor's/master's degree programs. Revisiting the current accreditation standards program may allow IPE to take a more prominent role in RT curricula.

Live birth resulting from a conjoined oocyte confirmed as euploid using array CGH: a case report.

The significance of conjoined oocytes in the clinical IVF laboratory setting has been of question due to the extremely limited data available. The most reliable criterion for true binovularity is the inclusion of two oocytes within a common zona pellucida or their fusion in the zonal region. This is a relatively rare event and owing to the limited number of embryo transfers performed and information on their outcomes, it is highly probable that these oocytes would be discarded without attempts at fertilization and subsequent embryo culture. To our knowledge, this is the first reported pregnancy resulting from a conjoined oocyte. Our experience involved a blastocyst transfer of a genetically screened embryo, performed after removal of the germinal vesicle from the conjoined oocyte/embryo on day 3. A clinical pregnancy with a gestational sac and fetal heartbeat was achieved and a healthy baby girl was delivered via Caesarean section at 37 weeks' gestation.

Rheumatoid arthritis referrals and rheumatologist scarcity: a prioritization tool.

OBJECTIVE: To assess whether applying the 2010 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) criteria for rheumatoid arthritis (RA) to primary care referrals improved triage decisions and reduced waiting times, and to determine the sensitivity and specificity of this strategy. METHODS: The 2010 ACR/EULAR criteria for RA were prospectively applied over 8 months to all new adult rheumatology referrals with possible inflammatory arthritis. If the referral contained insufficient information, a request was sent for more information. Joint count was based on physician report, and definite swelling was not required. Referrals meeting triage criteria were offered an appointment within 6 weeks. Data were collected on rheumatologist diagnosis, disease-modifying antirheumatic drug (DMARD) use, and waiting times. RESULTS: Of 457 referrals screened, 180 met inclusion and exclusion criteria, and 143 had sufficient data after requests for information. Seventy-one referrals met triage criteria, and of the 63 attending the appointment, 25 (40%) received a rheumatologist diagnosis of RA. Seventy-two referrals did not meet criteria, and 1 of 49 attending (2%) had RA. The characteristics of the tool for a diagnosis of RA were sensitivity 96%, specificity 56%, positive predictive value 40%, and negative predictive value 98%. Median wait times for referrals fulfilling and not fulfilling triage tool criteria were 7.9 weeks and 45.4 weeks, respectively. CONCLUSION: Implementing the 2010 ACR/EULAR criteria for RA as a prioritization tool for primary care referrals improved the number of patients subsequently diagnosed with RA. Waiting time was reduced for RA patients. Applying this strategy in areas of rheumatologist scarcity may permit earlier DMARD treatment.

Prediction of embryo developmental potential and pregnancy based on early stage morphological characteristics.

OBJECTIVE: To analyze the association between morphological details at different stages of culture with blastocyst development, with an aim to improve selection for transfer. DESIGN: Retrospective audit of data. SETTING: Tertiary referral center and university hospital. PATIENT(S): Two hundred sixty-eight couples underwent 357 treatment cycles. INTERVENTION(S): Oocyte pickups for IVF or intracytoplasmic sperm injection (ICSI) after ovarian stimulation. Embryos were individually cultured and examined on days 0-2 for morphological details and developmental characteristics, and selected for transfer, freezing, or further culture. MAIN OUTCOME MEASURES: The association of blastocyst development and pregnancy with morphological characteristics. RESULT(S): Five morphological characteristics (appearance of the cytoplasm, pronuclei and nucleoli, cytoplasmic deficit, and developmental rate) showed the strongest association with blastocyst development. By combining information from all days of culture into a cumulative score, prediction was greatly improved, compared to only using day 2 morphology. Cytoplasmic dysmorphisms of the oocyte, including accumulation of smooth endoplasmic reticulum, were associated with poor developmental performance. Differential weighting of these characteristics was included in a new embryo scoring system, which showed a strong correlation with implantation. CONCLUSION(S): Weighting individual morphological characteristics of zygotes and embryos and combining them into a cumulative embryo score can improve selection of embryos for transfer.