Type I interferon response and vascular alteration in chilblain-like lesions during the COVID-19 outbreak.

BACKGROUND: The outbreak of chilblain-like lesions (CLL) during the COVID-19 pandemic has been reported extensively, potentially related to SARS-CoV-2 infection, yet its underlying pathophysiology is unclear. OBJECTIVES: To study skin and blood endothelial and immune system activation in CLL in comparison with healthy controls and seasonal chilblains (SC), defined as cold-induced sporadic chilblains occurring during 2015 and 2019 with exclusion of chilblain lupus. METHODS: This observational study was conducted during 9-16 April 2020 at Saint-Louis Hospital, Paris, France. All patients referred with CLL seen during this period of the COVID-19 pandemic were included in this study. We excluded patients with a history of chilblains or chilblain lupus. Fifty patients were included. RESULTS: Histological patterns were similar and transcriptomic signatures overlapped in both the CLL and SC groups, with type I interferon polarization and a cytotoxic-natural killer gene signature. CLL were characterized by higher IgA tissue deposition and more significant transcriptomic activation of complement and angiogenesis factors compared with SC. We observed in CLL a systemic immune response associated with IgA antineutrophil cytoplasmic antibodies in 73% of patients, and elevated type I interferon blood signature in comparison with healthy controls. Finally, using blood biomarkers related to endothelial dysfunction and activation, and to angiogenesis or endothelial progenitor cell mobilization, we confirmed endothelial dysfunction in CLL. CONCLUSIONS: Our findings support an activation loop in the skin in CLL associated with endothelial alteration and immune infiltration of cytotoxic and type I IFN-polarized cells leading to clinical manifestations.

Two enemies, one fight: An update of oral cancer in patients with Fanconi anemia.

Fanconi anemia (FA) is a rare inherited genetic condition that may lead to bone marrow failure, leukemia, and/or solid tumors. It is caused by the loss of function of at least 1 gene of the FA/BRCA pathway, which is necessary for DNA repair. Patients with FA have a 200-fold to 1000-fold risk of developing head and neck cancer, mainly oral squamous cell carcinoma (OSCC), and of doing so at a much younger age than individuals within the general population. Also, patients who have FA with OSCC have poor overall survival rates, reinforcing the necessity to detect OSCC early. The scope of the current review is to provide an update on OSCC in patients with FA.

Influência da altura da câmera em fotogrametria computadorizada: amplitude articular / Influence of the photographic camera height by computerized photogrammetry: joint amplitude

O movimento humano, e suas disfunções, constituem o principal objeto de estudos da fisioterapia. Nesse sentido, compreender e analisar de forma objetiva e precisa a complexidade dos gestos motores do ser humano, trata-se de uma questão essencial que demanda o desenvolvimento de ferramentas que possibilitem alcançar um diagnóstico funcional preciso. Objetivou-se com o presente trabalho verificar se a mudança de posição da altura da câmera fotográfica promove variação do resultado da análise de amplitude articular do cotovelo, por meio da fotogrametria computadorizada e se altura influência na análise fotogramétrica. Foram selecionados entre os acadêmicos do 4° e 5° ano do curso de Fisioterapia da UEPA, 6 examinadores. Esses examinadores se dedicaram a obter os valores angulares de ADM da articulação do cotovelo. De vários ângulos, apenas um ângulo foi levado em consideração para a análise estatística, chamado ângulo-chave, posteriormente os dados foram analisados no programa BioEstat 5.0 e SPSS 18. Através dos resultados encontrados, observou-se distribuição normal para os dados obtidos em todas as diferentes alturas da câmera fotográfica, exceto para a altura de 50 cm. Notouse também, o mesmo padrão de variância em todas as alturas testadas, demonstrando variação não significante entre os resultados da amostra. Conclui-se, que as variações na altura da câmera fotográfica, não foram capazes de alterar significativamente o resultado final da análise por fotogrametria computadorizada. Identificou-se que a altura de 50 cm influencia no resultado final, pois a mesma demonstrou nesta investigação um risco de apresentar resultados inconsistentes estatisticamente (p=0.01). Desse modo, não se recomenda a utilização dessa altura de câmera fotográfica, evitando erros na interpretação das análises fotogramétricas....(AU)

Human movement, and its dysfunctions, are the main object of physical therapy studies. In this sense, to understand and analyze in an objective and precise way the complexity of the human motor gestures, it is an essential question that demands the development of tools that allow to reach a precise functional diagnosis. The objective of this study was to verify if the change of position of the photographic camera height promotes variation of the results of the joint amplitude analysis of the elbow, through computerized photogrammetry and if height influence in the photogrammetric analysis. Six examiners were selected among the academics of the 4th and 5th year of the UEPA Physiotherapy course. These examiners were dedicated to obtaining the angular values of ADM from the elbow joint. From several angles, only one angle was taken into account for the statistical analysis, called the key angle, later the data were analyzed in the program BioEstat 5.0 and SPSS 18. Through the results found, it was observed a normal distribution for the data obtained in all the different heights of the camera, except for the height of 50cm. The same pattern of variance was also observed in all the tested heights, demonstrating nonsignificant variation among the results of the sample. It was concluded that the variations in the height of the photographic camera were not able to significantly alter the final result of the analysis by computerized photogrammetry. It was identified that the height of 50cm influenced the final result, since it showed in this investigation a risk of presenting inconsistent results statistically (p = 0.01). Therefore, the use of this camera height is not recommended, avoiding errors in the interpretation of photogrammetric analyzes....(AU)

Crossed looks on the dermatologist's position and the patient's preoccupations as to psoriasis and pregnancy: preliminary results of the PREGNAN-PSO study.

BACKGROUND: While some information on psoriasis impact on pregnancy is available, very little is known on the preoccupations of women afflicted by the disease or on the dermatologists' (D) positioning as to psoriasis and pregnancy. The 'Objectifs Peau' project demonstrated a 4.7% prevalence of psoriasis in women aged 18-45 years in France. OBJECTIVE: This project sought to further address these issues in view of a targeted action plan. METHODS: A questionnaire was made available to 361 D of different types. RESULTS: Overall, 152 D answered the questionnaire, 50.7% working in private or mixed practice and 49.3% in hospitals, with 63% females (DF) and 37% males (DM). Over the last 3 months, the mean percentage of women of child-bearing age seen by these D was 28.6%. The main issue addressed by D upon psoriasis diagnosis was the patient's wish to become pregnant in the short-term (84%), while the compatibility of drug treatment with pregnancy was the issue prioritized by patients (64%). Among DM and DF, 46% and 29% reported having been confronted with an unplanned pregnancy, with their reaction mainly dependent on the treatment taken in 66%. Regarding follow-up, 26% D declared having shared their decision-making with gynaecologists, while 56% considered the first pregnancy trimester to be the highest-risk period. Only 28% D were familiar with existing recommendations, with only 21% of them considering them appropriate. CONCLUSION: Overall, 26.2% of French psoriasis women are of child-bearing potential, in line with our dataset (28.6%). Only 56% D considered the 1st pregnancy trimester to be the highest-risk period, with only one-third familiar with existing recommendations. The gap between recommendations and actual practices must be addressed through policies that take women's preoccupations better into account.

Long-term Survival, Organ Function, and Malignancy after Hematopoietic Stem Cell Transplantation for Fanconi Anemia.

We report on long-term survival in 157 patients with Fanconi anemia (FA) who survived 2 years or longer after their first transplantation with a median follow-up of 9 years. Marrow failure (80%) was the most common indication for transplantation. There were 20 deaths beyond 2 years after transplantation, with 12 of the deaths occurring beyond 5 years after transplantation. Donor chimerism was available for 149 patients: 112 (76%) reported > 95% chimerism, 27 (18%) reported 90% to 95% chimerism, and 8 (5%) reported 20% to 89% donor chimerism. Two patients have < 20% donor chimerism. The 10- and 15-year probabilities of survival were 90% and 79%, respectively. Results of multivariate analysis showed higher mortality risks for transplantations before 2003 (hazard ratio [HR], 7.87; P = .001), chronic graft-versus-host disease (GVHD) (HR, 3.80; P = .004) and squamous cell carcinoma after transplantation (HR, 38.17; P < .0001). The predominant cause of late mortality was squamous cell carcinoma, with an incidence of 8% and 14% at 10 and 15 years after transplantation, respectively, and was more likely to occur in those with chronic GVHD. Other causes of late mortality included chronic GVHD, infection, graft failure, other cancers, and hemorrhage. Although most patients are disease free and functional long term, our data support aggressive surveillance for long periods to identify those at risk for late mortality.

Oral and Maxillofacial Complex Lesions in Adolescents: A Retrospective Study of 20 Years / Lesiones Orales y del Complejo Maxilofacial en Adolescentes: Un Estudio Retrospectivo de 20 Años

Studies assessing the prevalence of oral lesions show that 5.2% to 12.8% of biopsy specimens are from children and adolescents. In Brazil, there are few studies analyzing the distribution of oral lesions in that population. Therefore, the aim of this study was to evaluate the distribution of oral and maxillomandibular lesions of adolescents, admitted to the Department of Oral Diagnosis of the Federal University of Paraná (UFPR) from 1994 to 2013. A study based on the review of the charts was done and the lesions were classified in 11 categories: salivary glands disease, dental pathology, gingival and periodontal pathology, odontogenic cysts, odontogenic tumors, non odontogenic cysts, bone pathology, mucosal pathology, connective tissue pathology, malignant tumors and other pathology. Variables including age, sex, ethnicity, diagnosis and lesion location were also evaluated. Upon analysis, 376 lesions were identified, most in girls, 51.9% and 77.1 % of patients were white. The most frequent site was the lower lip followed by the gingiva. Mucocele was the most common lesion (27.6%), followed by fibroepithelial hyperplasia (8.2%) and pyogenic granuloma (5.3%). Although there is a wide range of pathologies that can occur in the oral cavity, traumatic injuries are the most common in this age group.

Los estudios que evalúan la prevalencia de lesiones orales muestran que entre el 5,2% hasta el 12,8% de las muestras de biopsia corresponden a niños y adolescentes. En Brasil, hay pocos estudios que hayan analizado la distribución de las lesiones orales enesa población. Por lo tanto, el objetivo de este estudio fue evaluar la distribución de las lesiones orales y maxilofaciales de adolescentes, ingresados en el Servicio de Diagnóstico Oral de la Universidad Federal de Paraná (UFPR) entre 1994 a 2013. Se llevó a cabo la revisión de historias clínicas y las lesiones halladas fueron clasificadas en 11 categorías: enfermedad de glándulas salivales, patología dental, patología gingival y periodontal, quistes odontogénicos, tumores odontogénicos, quistes no odontogénicos, patología ósea, patología de la mucosa, patología del tejido conectivo, tumores malignos y otras patologías. También se evaluaron otras variables como edad, género, etnia, diagnóstico y localización de la lesión. Luego del análisis, se identificaron 376 lesiones, la mayoría en niñas (51,9%), siendo el 77,1% de los pacientes de raza blanca. La localización más frecuente fue el labio inferior seguida de la encía. El mucocele fue la lesión más común (27,6%), seguido de hiperplasia fibroepitelial (8,2%) y granuloma piógeno (5,3%). Aunque existe una amplia serie de patologías que pueden ocurrir en la cavidad oral, las lesiones traumáticas son las más comunes en este grupo de edad.

Oral manifestations compatible with chronic graft-versus-host disease in patients with Fanconi anemia.

Fanconi anemia (FA) is a genetic disease that is characterized by several congenital abnormalities and progressive bone marrow failure and is associated with an increased susceptibility to malignant disorders. Currently, the only potential cure for hematological disorders is hematopoietic stem cell transplantation (HSCT). However, 1 of the most common complications after HSCT is the development of oral chronic graft-versus-host disease (cGVHD), which is also a risk factor for the development of cancer, particularly oral squamous cell carcinoma. Therefore, the purpose of this study was to describe the prevalence and characteristics of oral manifestations compatible with cGVHD in patients diagnosed with FA according to the National Institutes of Health (NIH) consensus criteria. A total of 96 patients (51 females, 45 males; median age, 16 years) with FA, who were in medical follow-up after HSCT at the outpatient clinic of the bone marrow transplantation unit (Hospital de Clínicas from the Universidade Federal do Paraná) underwent an oral evaluation between January 2013 and December 2013. Post-HSCT periods varied from 1 to 261 months and were divided into 3 periods: immediate post-HSCT period; intermediate post-HSC period, and late post-HSCT period. Among the evaluated patients, 40 of 96 (42%) presented with oral manifestations of cGVHD, with 29 of 40 (73%) of these patients in the late post-HSCT period. NIH scale scores varied from 0 to 10, and lichenoid and hyperkeratotic lesions were the abnormalities most frequently observed (100%). Overall, a high prevalence of oral manifestations was observed for cGVHD patients with FA. These data highlight the importance of monitoring oral manifestations compatible with cGVHD to identify and treat individuals with a higher risk of developing oral cancer.