Citations

Abstract Anti‐glomerular basement membrane (anti‐GBM) disease (formerly known as Goodpasture's syndrome) is a rare autoinflammatory condition that affects the renal and/or pulmonary capillaries. The standard therapeutic regimen for anti‐GBM disease involves therapeutic plasma exchange (TPE), cyclophosphamide, and corticosteroids to rapidly remove and inhibit autoantibody production and reduce organ inflammation. Herein we report an 82‐year‐old female who developed anti‐GBM disease but expired despite therapy, secondary to multi‐organ failure in the setting of disseminated adenovirus disease. We discuss the utility and potential adverse effect of daily TPE for a protracted course (ie, 10‐14 days), the recommended TPE intensity in the 2023 American Society for Apheresis guidelines, updated from every‐other‐day TPE in the 2019 guidelines, despite no new data. We also highlight the potential for unusual infections to occur in these patients due to the profound immunosuppression, and discuss the importance of balancing immunosuppression to treat the disease with close surveillance of any potential opportunistic infections.

SummaryBackgroundGastroparesis is defined by delayed gastric emptying with associated symptoms in the absence of mechanical obstruction.AimTo evaluate pharmacokinetics and pharmacodynamics of felcisetrag, a highly selective 5‐HT4 receptor agonist, on total gut transit in patients with documented delayed gastric emptying of solids.MethodsSingle‐centre, placebo‐controlled study of 36 participants receiving placebo, 0.1mg, 0.3mg or 1.0mg of felcisetrag I.V. infusion, daily, for 3 days. At baseline, each participant completed a 4h, 99mTc‐egg meal (300 kcal, 30% fat) gastric emptying test. Following infusion (Day 2), gastric, small bowel and colonic transit of solids were measured over 48h (same meal plus 111In‐charcoal delivered in methacrylate‐coated capsule). Samples were collected for pharmacokinetics. The primary endpoint was gastric emptying T1/2. Statistical analysis used baseline parameters as covariates (ANCOVA).ResultsPatients (22 idiopathic, 14 diabetic gastroparesis) were randomised to felcisetrag (0.1 mg, n = 10; 0.3 mg, n = 9; 1.0 mg, n = 7) or placebo (n = 10). Compared to placebo, felcisetrag significantly accelerated gastric emptying T1/2, colonic filling at 6h, and 10% small bowel transit time (overall P < 0.01; all three doses individually Bonferroni corrected P < 0.05) for all three measurements. Ascending colon emptying (T1/2) was significantly accelerated (all doses), and colonic transit at 48 hours was accelerated with 0.1 mg and 0.3 mg felcisetrag compared to placebo. Pharmacokinetic results were dose proportional. Felcisetrag was well tolerated with no clinically significant findings from clinical laboratory, vital signs or ECG.ConclusionI.V. felcisetrag significantly accelerated gastric, small bowel and colonic transit in patients with gastroparesis, and should be further evaluated for short‐term treatment of gastric and intestinal motility disorders.ClinicalTrials.gov #NCT03281577

Background Matters of workplace harassment are an important issue. This issue needs to be recognized and studied to prevent occurrences. These important sensitive areas of effective workplace management are increasingly gaining more interest. We aimed to identify the prevalence of workplace sexual, verbal and physical harassment among headache professionals. Methods We adopted a cross‑sectional exploratory survey approach with quantitative design. The survey was distributed electronically among headache healthcare and research professionals globally through the International Headache Society (IHS). Results Data were obtained from 579 respondents (55.3%; 320/579 women). A large percentage of respondents (46.6%; 270/579) had experienced harassment; specifically, 16.1% (93/578) reported sexual harassment, 40.4% (234/579) verbal harassment and 5.5% (32/579) physical harassment. Women were almost seven times more likely to experience sexual harassment compared to men (odds ratio = 6.8; 95% confidence interval = 3.5–13.2). Although women did also more frequently report other types of harassment, this was not statistically significant (odds ratio = 1.4; 95% confidence interval = 1.0–2.0). Conclusions Lifetime exposure to workplace harassment is prevalent among headache professionals, especially in women. The present study uncovers a widespread issue and calls for strategies to be implemented for building a healthy and safe workplace environment.

OBJETIVO: Estimar a prevalência de gestação não planejada (GNP) em oito hospitais públicos universitários, distribuídos nas cinco regiões que compõem o Brasil. MÉTODOS: Análise secundária de um estudo transversal multicêntrico nacional, realizado em oito hospitais universitários públicos, entre 1º de junho e 31 de agosto de 2020, no Brasil. Amostra por conveniência incluindo mulheres que deram à luz em período de sessenta dias consecutivos e atenderam aos seguintes critérios: maiores de 18 anos; idade gestacional acima de 36 semanas no parto; com recém-nascido único e vivo, sem malformações. RESULTADOS: Amostra composta por 1.120 puérperas, das quais 756 (67,5%) declararam que a gravidez não tinha sido programada. A mediana da prevalência de GNP foi de 59,7%. Observou-se diferença significativa na prevalência de GNP entre os hospitais: Campinas (54,8%), Porto Alegre (58,2%), Florianópolis (59%), Teresina (61,2%), Brasília (64,3%), São Paulo (64,6%), Campo Grande (73,9%) e Manaus (95,3%) (p < 0,001). Foram fatores significativamente associados a GNP a idade materna, cor negra, menor renda familiar, maior número de filhos, maior número de pessoas convivendo em casa e não ter parceiro. CONCLUSÃO: na amostra estudada, cerca de dois terços das gestações foram declaradas como não programadas. A prevalência de gestação não planejada teve relação com fatores sociais e demográficos e variou significativamente entre os hospitais universitários avaliados.

Adamantinoma-like Ewing sarcoma (ALES) is a rare malignancy currently considered a variant of Ewing sarcoma with most known cases harboring EWSR1 rearrangements. Herein we present a series of 6 cases of EWSR1-negative ALES. The tumors arose in the sinonasal tract (n=3), major salivary glands (submandibular gland=1; parotid=1), and anterior mediastinum (n=1) in patients ranging from 25 to 79 years of age. Most tumors were basaloid in appearance, growing in large nests separated by interlobular fibrosis without overt squamous pearls. However, 1 case closely resembled a well-differentiated neuroendocrine tumor with uniformly round nuclei, eosinophilic cytoplasm, and trabecular architecture. All cases were diffusely positive for pan-cytokeratin, p40 or p63, and CD99. A subset of cases showed diffuse reactivity for synaptophysin, including 1 sinonasal tumor which also demonstrated sustentacular S100 protein expression. Molecular testing showed FUS rearrangements in all cases. Gene partners included known ETS family members FEV (n=2) and FLI1 (n=1). Our results expand the molecular diagnostic considerations for ALES to include FUS rearrangements. We also show that ALES may harbor FUS::FLI1 fusion, which has not been previously reported in the Ewing family of tumors. Furthermore, ALES may show unusual histologic and immunophenotypic features that can overlap with olfactory carcinoma including S100-positive sustentacular cells. ALES should be considered in the diagnostic differential of small round cell tumors and tumors with neuroendocrine differentiation with immunohistochemical workup to include p40 and CD99/NKX2.2.

Background: Upper facial third morphometrics are an important consideration in aesthetic facial surgery and facial gender-affirming surgery. Although there are generally accepted sexual dimorphic differences, an in-depth analysis of forehead morphometrics in attractive individuals is lacking. Methods: Thirty white female and 30 white male celebrities were included. Three full-face front-view photographs of each celebrity were evaluated by a facial analysis program, using Vision framework and MATLAB. After converting pixel distances to absolute distances, midline and lateral forehead heights were calculated and compared between men and women. Results: Forehead height was similar between attractive men and women, but forehead width was shorter in women. Analysis of forehead height at various points along the hairline demonstrated that forehead measurements above the lateral brow and brow peak were significantly greater in men. Mean forehead height above the lateral eyebrow was 3.51 cm in women and 4.16 cm in men (P = 0.017). Forehead height above the eyebrow peak was 4.34 cm in women and 5.55 cm in men (P < 0.001). Medial forehead height was similar between men and women, indicating that the greatest difference in attractive male and female foreheads is in the lateral forehead and forehead width. Conclusions: Analysis of attractive white celebrities demonstrated no significant differences in central forehead heights between men and women. Forehead width and lateral forehead height were significantly smaller in women, with an overall downward slanting contour. Male hairlines were more horizontal and slanting upward laterally. These results have implications in facial rejuvenation and facial gender-affirming surgery.

The diversity of patients pursuing facial aesthetic and facial gender-affirming surgery (FGAS) is increasing, yet there is a paucity of objective guidelines to facilitate surgical decision-making in patients of color. We conducted a quantitative analysis of black celebrities using standardized frontal photos of 21 female and 21 male celebrities. Celebrities were chosen from popular entertainment magazines and websites, including People Magazine, the Internet Movie Database (IMDb), Cosmopolitan, and Essence. For each celebrity, 100 facial landmarks were detected through a facial analysis artificial intelligence (AI) program. Black males had greater facial height, bizygomatic width, lower facial height, and bigonial width than females. However, the facial height to bigonial width ratio was similar between genders and approximated the golden ratio (1.618). Female faces demonstrated a greater mid-face height to total facial height proportion, and males had a greater lower facial height proportion. Females exhibited an upward-slanted medial brow and shorter total eyebrow length, nose height, and alar width. Forehead height above the lateral brow was greater in males, while central forehead height was similar to females. This is the first study that has utilized AI to provide ethnicity-specific facial morphometrics relevant to facial rejuvenation and FGAS in the black population.