Citations

AbstractBackground/ObjectivesPyoderma gangrenosum (PG) is a neutrophilic dermatosis rarely seen in children. Its features have not been well characterized in children. We sought to characterize the clinical features, etiologic associations, and treatment of PG in children younger than 18 years.MethodsWe performed a retrospective review of children younger than 18 years with PG at the Mayo Clinic from January 1976 to August 2013.ResultsThirteen children with PG were identified (n = 8; 62% female). All had ulcerations, with 62% having pustular lesions. Sites of involvement included the trunk (77%), lower extremities (77%), upper extremities (38%), and head and neck (38%). Nine (69%) had an underlying comorbidity, including seven with Crohn's disease (54%), one with juvenile idiopathic arthritis (8%), and one with pyogenic arthritis, pyoderma gangrenosum, and acne syndrome (8%). Treatments included topical or local care (92%) and systemic therapies (85%) such as oral corticosteroids (62%) and sulfasalazine or related 5‐aminosalicylate drugs (46%). The clinical course did not correlate with that of the underlying systemic disease and response to treatment varied.ConclusionPediatric PG has a more varied anatomic distribution and a greater predominance of pustular lesions than PG in adults and a strong association with inflammatory bowel disease.

AbstractChildren are at risk of developing allergic contact dermatitis to fragrances. Personal hygiene products, even those labeled hypoallergenic or considered all natural, may be a significant source of fragrance exposure in this population.

Abstract Objective: Several attributes of female reproductive history, including age at natural menopause (ANM), have been related to primary open-angle glaucoma (POAG). We assembled 18 previously reported common genetic variants that predict ANM to determine their association with ANM or POAG. Methods: Using data from the Nurses’ Health Study (7,143 women), we validated the ANM weighted genetic risk score in relation to self-reported ANM. Subsequently, to assess the relation with POAG, we used data from 2,160 female POAG cases and 29,110 controls in the National Eye Institute Glaucoma Human Genetics Collaboration Heritable Overall Operational Database (NEIGHBORHOOD), which consists of 8 datasets with imputed genotypes to 5.6+ million markers. Associations with POAG were assessed in each dataset, and site-specific results were meta-analyzed using the inverse weighted variance method. Results: The genetic risk score was associated with self-reported ANM (P = 2.2 × 10–77) and predicted 4.8% of the variance in ANM. The ANM genetic risk score was not associated with POAG (Odds Ratio (OR) = 1.002; 95% Confidence Interval (CI): 0.998, 1.007; P = 0.28). No single genetic variant in the panel achieved nominal association with POAG (P ≥0.20). Compared to the middle 80 percent, there was also no association with the lowest 10th percentile or highest 90th percentile of genetic risk score with POAG (OR = 0.75; 95% CI: 0.47, 1.21; P = 0.23 and OR = 1.10; 95% CI: 0.72, 1.69; P = 0.65, respectively). Conclusions: A genetic risk score predicting 4.8% of ANM variation was not related to POAG; thus, genetic determinants of ANM are unlikely to explain the previously reported association between the two phenotypes.

Purpose To examine internal medicine (IM) residency program directors’ (PDs’) perspectives on the Core Entrustable Professional Activities for Entering Residency (Core EPAs)—introduced into undergraduate medical education to further competency-based assessment—and on communicating competency-based information during transitions. Method A spring 2015 Association of Program Directors in Internal Medicine survey asked PDs of U.S. IM residency programs for their perspectives on which Core EPAs new interns must or should possess on day 1, which are most essential, and which have the largest gap between expected and observed performance. Their views and preferences were also requested regarding communicating competency-based information at transitions from medical school to residency and residency to fellowship/employment. Results The response rate was 57% (204/361 programs). The majority of PDs felt new interns must/should possess 12 of the 13 Core EPAs. PDs’ rankings of Core EPAs by relative importance were more varied than their rankings by the largest gaps in performance. Although preferred timing varied, most PDs (82%) considered it important for medical schools to communicate Core EPA-based information to PDs; nearly three-quarters (71%) would prefer a checklist format. Many (60%) would be willing to provide competency-based evaluations to fellowship directors/employers. Most (> 80%) agreed that there should be a bidirectional communication mechanism for programs/employers to provide feedback on competency assessments. Conclusions The gaps identified in Core EPA performance may help guide medical schools’ curricular and assessment tool design. Sharing competency-based information at transitions along the medical education continuum could help ensure production of competent, practice-ready physicians.

Background and Purpose— Hepatocyte growth factor (HGF) is positively associated with ischemic and hemorrhagic stroke risk factors. However, understanding the relation between HGF and stroke is in its infancy. Therefore, we sought to examine the association of circulating HGF with incident stroke using data from the MESA (Multi-Ethnic Study of Atherosclerosis). We hypothesized that circulating HGF would be positively associated with an increased risk of stroke. Methods— Participants aged 45 to 84 years (n=6711) had HGF measured between 2000 and 2002 and were followed for incident stroke through 2013 (n=233). Cox proportional hazards regression was used to calculate hazard ratios and 95% confidence intervals for incident stroke. A secondary analysis stratified results by adjudicated stroke type (n=183 ischemic; n=39 hemorrhagic; n=11 other). Results— After adjustment for potential confounding variables, risk of stroke was 17% higher with each standard deviation increase in HGF (hazard ratio, 1.17; 95% confidence interval, 1.03–1.34). This association was mainly driven by ischemic stroke and did not change on exclusion of cardioembolic strokes, although the number of excluded cases was small. The few hemorrhagic and other types of stroke were not associated with HGF. Conclusions— Circulating HGF was positively associated with the incidence of stroke in a diverse, population-based cohort of men and women from the United States. Our findings support the hypothesis that circulating HGF is a marker of endothelial damage and suggest that HGF may have utility as a prognostic marker of stroke risk.

Key PointsThe monotypic and polytypic PC compartments assessed by MFC are prognostic in AL amyloidosis. MFC may play a role in the definition of hematologic response to treatment.

ObjectivesThe bony shoulder stability ratio (BSSR) allows for quantification of the bony stabilisers in vivo. We aimed to biomechanically validate the BSSR, determine whether joint incongruence affects the stability ratio (SR) of a shoulder model, and determine the correct parameters (glenoid concavity versus humeral head radius) for calculation of the BSSR in vivo.MethodsFour polyethylene balls (radii: 19.1 mm to 38.1 mm) were used to mould four fitting sockets in four different depths (3.2 mm to 19.1mm). The SR was measured in biomechanical congruent and incongruent experimental series. The experimental SR of a congruent system was compared with the calculated SR based on the BSSR approach. Differences in SR between congruent and incongruent experimental conditions were quantified. Finally, the experimental SR was compared with either calculated SR based on the socket concavity or plastic ball radius.ResultsThe experimental SR is comparable with the calculated SR (mean difference 10%, sd 8%; relative values). The experimental incongruence study observed almost no differences (2%, sd 2%). The calculated SR on the basis of the socket concavity radius is superior in predicting the experimental SR (mean difference 10%, sd 9%) compared with the calculated SR based on the plastic ball radius (mean difference 42%, sd 55%).ConclusionThe present biomechanical investigation confirmed the validity of the BSSR. Incongruence has no significant effect on the SR of a shoulder model. In the event of an incongruent system, the calculation of the BSSR on the basis of the glenoid concavity radius is recommended. Cite this article: L. Ernstbrunner, J-D. Werthel, T. Hatta, A. R. Thoreson, H. Resch, K-N. An, P. Moroder. Biomechanical analysis of the effect of congruence, depth and radius on the stability ratio of a simplistic ‘ball-and-socket’ joint model. Bone Joint Res 2016;5:453–460. DOI: 10.1302/2046-3758.510.BJR-2016-0078.R1.