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The Argentine Osteoporosis Society convened renowned specialists in the care of transgender people to prepare the first local position on the evaluation of bone health in this population. Law 26.743 on "Gender Identity" recognize all identities and guarantees free care throughout the health system. The impact of different gender affirmation treatments on bone mass has been topic of international debate. To date the evidence remains limited and different societies have issued suggestions and recommendations. For this reason, we believe it is relevant to mention our experience, capturing through this document a series of suggestions to be used in medical care.

La Sociedad Argentina de Osteoporosis convocó a especialistas reconocidos en la atención de personas transgénero para la elaboración del primer posicionamiento local sobre la evaluación de la salud ósea en esta población. La ley 26.743 de "Identidad de género" reconoce todas las identidades y garantiza su atención de manera gratuita en el sistema de salud. El impacto de los diferentes tratamientos de afirmación de género sobre la masa ósea ha sido tópico de debate internacional. Hasta la fecha la evidencia sigue siendo limitada y diferentes sociedades han emitido sugerencias y recomendaciones. Por tal motivo, creemos relevante mencionar nuestra experiencia plasmando mediante este documento una serie de sugerencias para ser utilizadas en la atención médica.

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Resumen La Sociedad Argentina de Osteoporosis convocó a especialistas reconocidos en la atención de personas transgénero para la elaboración del primer posiciona miento local sobre la evaluación de la salud ósea en esta población. La ley 26.743 de "Identidad de género" reco noce todas las identidades y garantiza su atención de manera gratuita en el sistema de salud. El impacto de los diferentes tratamientos de afirmación de género sobre la masa ósea ha sido tópico de debate internacional. Hasta la fecha la evidencia sigue siendo limitada y diferentes sociedades han emitido sugerencias y recomendaciones. Por tal motivo, creemos relevante mencionar nuestra experiencia plasmando mediante este documento una serie de sugerencias para ser utilizadas en la atención médica.

Abstract The Argentine Osteoporosis Society convened renowned specialists in the care of transgender people to prepare the first local position on the evaluation of bone health in this population. Law 26.743 on "Gender Identity" recognize all identities and guarantees free care throughout the health system. The impact of different gender affirmation treatments on bone mass has been topic of international debate. To date the evidence remains limited and different societies have issued suggestions and recommendations. For this reason, we believe it is relevant to mention our experience, capturing through this document a series of suggestions to be used in medical care.

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Objectives: This study aimed to evaluate the clinical and serological profile in systemic sclerosis (SSc) by comparing females and males. Patients and methods: This retrospective study was conducted with 215 SSc patients (193 females, 22 males; mean age: 50.1±14.5 years; range, 16 to 88 years) between September 2005 and September 2020. Disease severity was calculated by the Medsger severity score. Males and females were compared for clinical and serological markers. Results: Females more frequently had esophageal involvement (p=0.003), telangiectasias (p=0.03), and antinuclear antibodies (p=0.04). Males more frequently had fingertip scars (p=0.03), digital ulcers (p=0.006), and a worse median Medsger severity score (6 in males vs. 4 in females, p=0.05). Conclusion: In the studied sample, males had more severe disease than females with greater repercussions in periferic circulatory system.

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Cutaneous fibrosis is one of the main features of systemic sclerosis (SSc). Recent findings correlated abnormal collagen V (Col V) deposition in dermis with skin thickening and disease activity in SSc. Considering that Col V is an important regulator of collagen fibrillogenesis, understanding the role of Col V in the first two years of the skin fibrosis in SSc (early SSc) can help to determine new targets for future treatments. In this study, we analyzed the morphological, ultrastructural and molecular features of α1(V) and α2(V) chains and the expression of their coding genes COL5A1 and COL5A2 in collagen fibrillogenesis in early-SSc. Skin biopsies were obtained from seven consecutive treatment-naïve patients with SSc-related fibrosis and four healthy controls. Our data showed increased α1(V) and α2(V) chain expression in the reticular dermis of early-SSc patients; however, immunofluorescence and ultrastructural immunogold staining determined a significant decreased expression of the α1(V) chain along the dermoepidermal junction in the papillary dermis from early-SSc-patients in relation to the control (12.77 ± 1.34 vs. 66.84 ± 3.36; p < 0.0001). The immunoblot confirmed the decreased expression of the α1(V) chain by the cutaneous fibroblasts of early-SSc, despite the increased COL5A1 and COL5A2 gene expression. In contrast, the α2(V) chain was overexpressed in the small vessels (63.18 ± 3.56 vs. 12.16 ± 0.81; p < 0.0001) and capillaries (60.88 ± 5.82 vs. 15.11 ± 3.80; p < 0.0001) in the reticular dermis of early-SSc patients. Furthermore, COLVA2 siRNA in SSc cutaneous fibroblasts resulted in a decreased α1(V) chain expression. These results highlight an intense decrease in the α1(V) chain along the dermoepidermal junction, suggesting an altered molecular histoarchitecture in the SSc papillary dermis, with a possible decrease in the expression of the α1(V)3 homotrimeric isoform, which could interfere with the thickening and cutaneous fibrosis related to SSc.

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La acrodisostosis es una displasia esquelética rara, de herencia autosómica dominante, que se caracteriza por la presencia de disostosis facial y periférica, talla baja y diferentes grados de obesidad. La acrodisostosis de tipo 1, secundaria a la mutación heterocigota en el gen PRKAR1A (17q24.2), se caracteriza por la asociación de resistencia hormonal múltiple con anomalías esqueléticas. Su incidencia está infradiagnosticada debido a que comparte rasgos clínicos y de laboratorio con otras entidades como el seudohipoparatiroidismo. Presentamos el caso de una niña de 8 años, con acrodisostosis tipo 1, confirmada mediante estudio genético. Además del fenotipo característico descrito, la talla baja y la resistencia hormonal, la paciente presentó una afectación progresiva de la función pulmonar: un patrón pulmonar obstructivo no reversible. En la literatura revisada, no se han encontrado otros casos que describan esta asociación entre acrodisostosis y afectación respiratoria.

Acrodysostosis is a rare skeletal displasia, of autosomal dominant inheritance, characterized by the presence of facial and peripheral dysostosis, short stature and obesity. Type 1 acrodysostosis is secondary to a mutation in the PRKAR1A (17q24.2) gene, which results in multi hormonal resistance and skeletal anomalities. This syndrome is under-diagnosed as it shares analytical and clinical characteristics with other entities, such as pseudohypoparathyroidism. We report the case of an eight-year-old girl with genetically confirmed type 1 acrodysostosis. In addition to the characteristic phenotype described, the short stature and the hormonal resistance, the patient suffered a progressive lung function deterioration: an irreversible pulmonary obstructive pattern. We have not found in previous literature cases reporting an association between acrodysostosis and lung function impairement.

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Acrodysostosis is a rare skeletal displasia, of autosomal dominant inheritance, characterized by the presence of facial and peripheral dysostosis, short stature and obesity. Type 1 acrodysostosis is secondary to a mutation in the PRKAR1A (17q24.2) gene, which results in multi hormonal resistance and skeletal anomalities. This syndrome is under-diagnosed as it shares analytical and clinical characteristics with other entities, such as pseudohypoparathyroidism. We report the case of an eight-year-old girl with genetically confirmed type 1 acrodysostosis. In addition to the characteristic phenotype described, the short stature and the hormonal resistance, the Afectación respiratoria en paciente con acrodisostosis: una asociación infrecuente de una enfermedad rara Respiratory impairment in a patient with acrodysostosis: A rare association of an uncommon pathology patient suffered a progressive lung function deterioration: an irreversible pulmonary obstructive pattern. We have not found in previous literature cases reporting an association between acrodysostosis and lung function impairement.

La acrodisostosis es una displasia esquelética rara, de herencia autosómica dominante, que se caracteriza por la presencia de disostosis facial y periférica, talla baja y diferentes grados de obesidad. La acrodisostosis de tipo 1, secundaria a la mutación heterocigota en el gen PRKAR1A (17q24.2), se caracteriza por la asociación de resistencia hormonal múltiple con anomalías esqueléticas. Su incidencia está infradiagnosticada debido a que comparte rasgos clínicos y de laboratorio con otras entidades como el seudohipoparatiroidismo. Presentamos el caso de una niña de 8 años, con acrodisostosis tipo 1, confirmada mediante estudio genético. Además del fenotipo característico descrito, la talla baja y la resistencia hormonal, la paciente presentó una afectación progresiva de la función pulmonar: un patrón pulmonar obstructivo no reversible. En la literatura revisada, no se han encontrado otros casos que describan esta asociación entre acrodisostosis y afectación respiratoria.

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OBJECTIVE: To study a sample of rheumatoid arthritis (RA) patients for their gynecological/obstetric history and compare them to controls to determine their influences on number of pregnancies, menarche, menopause and reproductive years following RA onset. METHODS: This is a cross-sectional study of 122 RA patients and 126 controls. Patients and controls were questioned about age of menarche, age of menopause, number of pregnancies and abortions. Reproductive years were calculated as the difference between age at menopause and age at menarche. For comparison, we used the Mann-Whitney, unpaired t, chi-squared, and Spearman tests. The adopted significance was 5%. RESULTS: In the RA patients with disease beginning in the postmenopausal years, the period of reproductive years (age at menopause - age of menarche) showed a positive correlation with age at disease onset (rho = 0.46; 95% confidence interval [CI] = 0.20-0.55 with p = 0.0008). The number of pregnancies was higher in patients with postmenopausal disease onset when compared with those with premenopausal disease onset (median of 3 with interquartile range [IQR] = 2-4 versus median of 2 with IQR = 1-3; p = 0.009), and RA patients had more pregnancies than controls (p = 0.0002). CONCLUSION: The present study shows that, in our population, the duration of reproductive years and the number of pregnancies are linked to the onset of RA.

OBJETIVO: Estudar uma amostra de pacientes com artrite reumatoide (AR), com investigação da história ginecológica e obstétrica, comparando-a com controles, visando conhecer suas influências no número de gestações, menarcas, menopausa e anos reprodutivos no início da AR. MéTODOS: Trata-se de um estudo transversal de 122 pacientes com AR e 126 controles. Pacientes e controles foram questionados sobre idade da menarca, idade da menopausa, número de gestações e abortos. Os anos reprodutivos foram calculados com a diferença entre a idade da menopausa e a idade da menarca. Para comparação, foram utilizados Mann Whitney, Teste t não pareados, Teste qui-quadrado e teste de Spearman. A significância adotada foi de 5%. RESULTADOS: Nas pacientes com AR e início da doença na pós-menopausa, o período de anos reprodutivos (idade da menopausa - idade da menarca) apresentou correlação positiva com a idade de início da doença (rho = 0,46; intervalo de confiança de 95% [IC95%] = 0,20­0,55 com p = 0,0008). O número de gestações foi maior nas pacientes com início da doença no período pós-menopausa quando comparadas às pacientes em pré-menopausa (mediana de 3 com intervalo interquartil [IIQ] = 2­4 versus mediana de 2 com IIQ = 1­3; p = 0,009). Nas pacientes com AR, foi observado um maior número de gestações do que no grupo controle (p = 0,0002). CONCLUSãO: O presente estudo mostra que, em nossa população, a diminuição dos anos reprodutivos e o alto número de gestações estão relacionados ao surgimento da AR.

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Resumen El artículo presenta un estudio de caso cuya objetivo es mejorar las condiciones de accesibilidad web de un dispositivo de comunicación pública de ciencia y tecnología titulado Dispositivo Hipermedial Dinámico Memoria y experiencia Cossettini. El mismo, publica en Acceso Abierto documentos digitalizados del Archivo pedagógico Cossettini conteniendo una gran cantidad de elementos estéticos visuales. Desde el marco teórico se sostiene que la elaboración colaborativa de objetos digitales multimodales contextualizados podría posibilitar a quienes participan del DHD MyECossettini una experiencia sensible/accesible de exploración, interpretación y recreación no excluyente de los archivos patrimoniales digitalizados, sin desmedro de lo singular y significativo de la fuente original. En este sentido, se ha propuesto la Accesibilidad-DHD basada en los principios de Percepción, Comprensión, Acción y Flexibilidad. En lo metodológico, se abordó el análisis y desarrollo de objetos digitales accesibles con un enfoque socio-técnico e interdisciplinar en atención al principio de percepción. Los resultados alcanzados constatan que las alternativas accesibles elaboradas habilitan nuevos canales perceptivos y proporcionan información contextual que constituye un valor agregado para todas las personas. Finalmente, se concluye que participar de la Accesibilidad-DHD no requiere necesariamente de experticia en informática, habilitando a que cada persona, desde su lugar, colabore en garantizar el acceso equitativo a la información. No obstante se advierte que, el desafío más complejo se presenta en la construcción de una toma de conciencia colectiva activa que asuma con responsabilidad de garantizar el Derecho Humano a habitar sin exclusiones el ciberespacio.

Abstract This article presents a case study with the aim to improve the conditions of web accessibility of a science and technology public communication device, "Dynamic Hypermedia Device, Memoria y Experiencia Cossettini". It publishes in Open Access digitized documents from the "Cossettini pedagogical archive" containing a large number of visual aesthetic elements. From the theoretical framework, the collaborative elaboration of contextualized multimodal digital objects could allow those who participate in the DHD MyECossettini a sensitive/accessible experience of exploration, interpretation and non-exclusive recreation of digitized heritage archives, without detriment to the singular and significant of the original source. Therefore, it is proposed DHD-Accessibility based on the principles of Perception, Comprehension, Action and Flexibility. Methodologically, the analysis and development of accessible digital objects are developed with a socio-technical and interdisciplinary approaches, focused on the principle of perception. The results achieved confirm that the accessible alternatives developed enable new perceptual channels and provide contextual information that constitutes added value for all people. Finally, it is highlighted that participating in "DHD-Accessibility" does not necessarily require IT expertise, enabling each person, from their place, to collaborate in guaranteeing equitable access to information. However, it is noted that the most complex challenge is presented in the construction of an active collective awareness that assumes with responsibility to guarantee the Human Right to inhabit cyberspace without exclusions.

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Abstract Objective To study a sample of rheumatoid arthritis (RA) patients for their gynecological/obstetric history and compare them to controls to determine their influences on number of pregnancies, menarche, menopause and reproductive years following RA onset. Methods This is a cross-sectional study of 122 RA patients and 126 controls. Patients and controls were questioned about age of menarche, age of menopause, number of pregnancies and abortions. Reproductive years were calculated as the difference between age at menopause and age at menarche. For comparison, we used the Mann-Whitney, unpaired t, chi-squared, and Spearman tests. The adopted significance was 5%. Results In the RA patients with disease beginning in the postmenopausal years, the period of reproductive years (age at menopause - age of menarche) showed a positive correlation with age at disease onset (rho=0.46; 95% confidence interval [CI]=0.20- 0.55 with p=0.0008). The number of pregnancies was higher in patients with postmenopausal disease onset when compared with those with premenopausal disease onset (median of 3 with interquartile range [IQR]=2-4 versus median of 2 with IQR=1-3; p=0.009), and RA patients had more pregnancies than controls (p=0.0002). Conclusion The present study shows that, in our population, the duration of reproductive years and the number of pregnancies are linked to the onset of RA.

Resumo Objetivo Estudar uma amostra de pacientes com artrite reumatoide (AR), com investigação da história ginecológica e obstétrica, comparando-a com controles, visando conhecer suas influências no número de gestações, menarcas, menopausa e anos reprodutivos no início da AR. Métodos Trata-se de um estudo transversal de 122 pacientes com AR e 126 controles. Pacientes e controles foram questionados sobre idade da menarca, idade da menopausa, número de gestações e abortos. Os anos reprodutivos foram calculados com a diferença entre a idade da menopausa e a idade da menarca. Para comparação, foram utilizados Mann Whitney, Teste t não pareados, Teste qui-quadrado e teste de Spearman. A significância adotada foi de 5%. Resultados Nas pacientes comAR e início da doença na pós-menopausa, o período de anos reprodutivos (idade da menopausa - idade da menarca) apresentou correlação positiva com a idade de início da doença (rho=0,46; intervalo de confiança de 95% [IC95%]=0,20-0,55 com p=0,0008). O número de gestações foi maior nas pacientes cominício da doença no período pós-menopausa quando comparadas às pacientes em pré-menopausa (mediana de 3 comintervalo interquartil [IIQ]=2-4 versusmediana de 2 com IIQ=1-3; p=0,009). Nas pacientes com AR, foi observado ummaior número de gestações do que no grupo controle (p=0,0002). Conclusão O presente estudo mostra que, em nossa população, a diminuição dos anos reprodutivos e o alto número de gestações estão relacionados ao surgimento da AR.

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Racional: Esclerose sistêmica (ES) e lúpus eritematoso sistêmico (LES) são doenças difusas do tecido conjuntivo que, quando agregadas, geram a síndrome de sobreposição (SP ES-LES), condição ainda não claramente caracterizada do ponto de vista clínico e epidemiológico. Objetivo: Caracterizar clínica e epidemiologicamente pacientes com SP ES-LES. Método: Trata-se de um estudo retrospectivo, descritivo de análise de prontuários. Coletaram-se dados demográficos, clínicos, laboratoriais e exames de imagens de pacientes com SP ES-LES.Resultados: O perfil encontrado foi o de mulher, jovem, caucasiana, não tabagista e com forma de limitada de ES. Foram comuns: fenômeno de Raynaud; acometimento gastroesofágico, pulmonar e cutâneo; anemia; linfopenia; FAN pontilhado grosso; anti-RNP; anti-Ro e anti-Sm. Nunca ter fumado foi achado protetor para serosite e lesões discoides, como também foram significativas associações anti-Scl-70 com lúpus discoide, anti-La com síndrome de Sjögren e anti-DNA com úlceras digitais. Conclusão: A SP ES-LES é multissistêmica, com aspectos clínicos predominantes de ES. Devido à grande prevalência de envolvimento pulmonar nesses pacientes, justifica-se o rastreamento de possíveis complicações.

Background: Systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) are diffuse autoimmune diseases of the connective tissue that when combined generate the SSc-SLE overlap syndrome (OS), condition without consistent epidemiological data. Objective: Characterize clinical and epidemiological data in patients with OS SSc-SLE. Method: This is a retrospective descriptive study of medical records. Demographic, clinical, laboratory data and image exams of older patients with SSc-SLE were collected. Results: Epidemiological profile was of female patients, young, caucasian, non-smoker with limited ES. Raynaud's phenomenon, gastroesophageal, pulmonary and skin disorders, anemia and lymphopenia were common as well as thick dotted ANA; anti-RNP; anti-Ro and anti-Sm. No smoking was protective for serositis and for discoid lesions, as well as significant associations of anti-Scl-70 with discoid lupus, anti-La with Sjogren's syndrome and anti-DNA with digital ulcers were found. Conclusion: OS SSc-SLE is multisystemic with clinical predominance of SSc. Due to high prevalence of lung involvement, screening for such possible complication is justified.

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INTRODUÇÃO: A calcinose é uma complicação frequente da esclerose sistêmica e da dermatomiosite, resultando em dor local, contraturas, ulcerações, infecções secundárias e limitação funcional. Não há tratamento eficaz para calcinose mas o tiossulfato de sódio tem se mostrado promissor. OBJETIVOS: Analisar a resposta terapêutica ao tiossulfato de sódio 10% intralesional nas calcinoses de pacientes com dermatomiosite e esclerose sistêmica. MÉTODOS: Estudo prospectivo, não controlado, incluindo 7 pacientes, totalizando 10 calcinoses, sendo um paciente com dermatomiosite e 6 com esclerose sistêmica. Desfechos primários: melhora da dor pela escala visual analógica e diminuição do maior e menor diâmetros das calcinoses ao raio-x. Desfechos secundários: melhora da qualidade de vida pelo SF12 (Medical Outcomes Study Short Form 12) e da função pelo HAQ (Health Assesment Questionnaire). Resultados foram expressos em médias e as variáveis contínuas comparadas pelo teste t-Student ou pelo teste de Wilcoxon. RESULTADOS: Foram realizadas 2 a 8 aplicações intralesionais (média de 3,7), com média de 8,9mg de tiossulfato por injeção a intervalos de 13 a 56 dias, com intervalo médio de 19 dias. Todos os pacientes relataram melhora da dor, porém esse resultado não foi significativamente estatístico. Não houve redução do diâmetro das calcinoses, nem melhora da qualidade de vida ou funcional. CONCLUSÃO: Baixas doses de tiossulfato de sódio administradas por injeções intralesionais, em intervalos médios de 19 dias, em número limitado de aplicações, não foram efetivas para o tratamento da calcinose

BACKGROUND: Calcinosis is a frequent complication of systemic sclerosis and dermatomyositis, resulting in local pain, contractures, ulcerations, secondary infections and functional limitations. There is no effective treatment for calcinosis, but sodium thiosulphate has shown to be promising. OBJECTIVE: To analyze the therapeutic response to 10% intralesional sodium thiosulphate on calcinosis of patients with dermatomyositis and systemic sclerosis. METHODS: Non-controlled prospective study, with 7 patients and 10 calcinoses, of which one had dermatomyositis and six had scleroderma. Primary outcomes: improvement in the pain by the visual analogue scale, and a decrease in both diameters (biggest and smallest) of calcinoses on X-ray. Secondary outcomes: improvement in quality of life by SF12 (Medical Outcomes Study Short Form 12), and of function by HAQ (Health Assessment Questionnaire). Results were expressed as means; continuous variables were compared by the t-Sudent test or by the Wilcoxon test. RESULTS: Two to 8 intralesional applications were performed (mean of 3.7), with mean of 8.9mg of thiosulphate per injection, at 13-56 days intervals (mean of 19 days). All patients reported improvement in pain, albeit such result was not statistically significant. There was no reduction in the diameters of calcinoses, nor there were quality of life or functional improvements. CONCLUSION: Low doses of sodium thiosulphate, administered through intralesional injections, in mean intervals of 19 days, in a limited number of applications, were not effective for calcinosis treatment

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BACKGROUND: The Hand Mobility in Scleroderma (HAMIS) test was created to measure the degree of dysfunction of hand movements imposed by systemic sclerosis (SSc). The modified version (mHAMIS), with 4 of the 9 original items, was developed later. The goal of the present study was to translate and validate HAMIS and mHAMIS into Brazilian Portuguese and culture. METHODS: After direct and reverse translation and comprehension test in 10 SSc patients, HAMIS-Br was applied to another 32 patients with SSc. To evaluate internal consistency, intraobserver and interobserver agreement, and intraobserver and interobserver reliability, we used respectively the Cronbach's α coefficient, kappa concordance and intraclass correlation (ICC). The correlation between HAMIS-Br and mHAMIS-Br was evaluated and a factorial analysis was performed. RESULTS: HAMIS-Br showed excellent internal consistency (Cronbach's α = 0.997), good intraobserver agreement (kappa between 0.78 [95%CI =0.57-0.99] and 1) and intraobserver and interobserver reliability (ICC = 0.993, 95% CI = 0.973-0.993 and ICC = 0.994, 95% CI = 0.987-0.997, respectively). The mHAMIS-Br presented similar results and excellent correlation with HAMIS-Br (r = 0.923). The factorial analysis extracted three groups of questions that explain 84.4% of the total variance, and that can be understood through the influence of certain movements in the interpretation of others: [1] questions whose interpretation is influenced by the extension of the fingers, [2] questions whose interpretation is influenced by flexion of the fingers, [3] volar flexion of the fingers, with similar correlation with both other factors. CONCLUSIONS: HAMIS-Br and mHAMIS-Br showed good agreement, intraobserver and interobserver reliability, and internal validity. It is necessary to be attentive to the influence of certain limitations of movements in the interpretation of others.

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Abstract Background: The Hand Mobility in Scleroderma (HAMIS) test was created to measure the degree of dysfunction of hand movements imposed by systemic sclerosis (SSc). The modified version (mHAMIS), with 4 of the 9 original items, was developed later. The goal of the present study was to translate and validate HAMIS and mHAMIS into Brazilian Portuguese and culture. Methods: After direct and reverse translation and comprehension test in 10 SSc patients, HAMIS-Br was applied to another 32 patients with SSc. To evaluate internal consistency, intraobserver and interobserver agreement, and intraobserver and interobserver reliability, we used respectively the Cronbach's α coefficient, kappa concordance and intraclass correlation (ICC). The correlation between HAMIS-Br and mHAMIS-Br was evaluated and a factorial analysis was performed. Results: HAMIS-Br showed excellent internal consistency (Cronbach's α = 0.997), good intraobserver agreement (kappa between 0.78 [95%CI =0.57-0.99] and 1) and intraobserver and interobserver reliability (ICC = 0.993, 95% CI = 0.973-0.993 and ICC = 0.994, 95% CI = 0.987-0.997, respectively). The mHAMIS-Br presented similar results and excellent correlation with HAMIS-Br (r = 0.923). The factorial analysis extracted three groups of questions that explain 84.4% of the total variance, and that can be understood through the influence of certain movements in the interpretation of others: [1] questions whose interpretation is influenced by the extension of the fingers, [2] questions whose interpretation is influenced by flexion of the fingers, [3] volar flexion of the fingers, with similar correlation with both other factors. Conclusions: HAMIS-Br and mHAMIS-Br showed good agreement, intraobserver and interobserver reliability, and internal validity. It is necessary to be attentive to the influence of certain limitations of movements in the interpretation of others.

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La infección por el virus de inmunodeficiencia humana produce una enfermedad sistémica crónica. El tratamiento con fármacos antirretrovirales altamente activos ha modificado la evolución de estos pacientes incrementando la sobrevida. En contraposición, asociadas a su enfermedad y/o a la medicación antirretroviral, se describen múltiples enfermedades crónicas que comprometen aún más la calidad de vida de estos pacientes. Nuestro objetivo es describir las alteraciones endocrinometabólicas en nuestros pacientes ambulatorios con infección por el virus de inmunodeficiencia humana y realizar una actualización del tema.

The human immunodeficiency virus causes a chronic systemic disease. Treatment with highly active antiretroviral drugs has changed the evolution of the disease, and increasing the rate of survival. However, due to the disease and/or the antiretroviral drugs, there are other multiple chronic diseases that compromise the quality of life of the patients even more. The aim of this study is to present endocrine and metabolic changes in outpatients with human immunodeficiency virus, and present an update of the related literature.

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Over the past 50 years, survival for children in high-income countries has increased from 30% to over 80%, compared to 10-30% in low and middle income countries (LMIC). Given this gap in survival, established paediatric cancer treatment centres, such as The Hospital for Sick Children (SickKids) are well positioned to share clinical expertise. Through the SickKids Centre for Global Child Health, the SickKids-Caribbean Initiative (SCI) was launched in March 2013 to improve the outcomes and quality of life for children with cancer and blood disorders in the Caribbean. The six participating Caribbean countries are among those defined by the United Nations as Small Island Developing States, due to their small size, remote location and limited accessibility. Telemedicine presents an opportunity to increase their accessibility to health care services and has been used by SCI to facilitate two series of interprofessional rounds. Case Consultation Review Rounds are a forum for learning about diagnostic work-up, management challenges and treatment recommendations for these diseases. To date, 54 cases have been reviewed by SickKids staff, of which 35 have been presented in monthly rounds. Patient Care Education Rounds provide nurses and other staff with the knowledge base needed to safely care for children and adolescents receiving treatment. Five of these rounds have taken place to date, with over 200 attendees. Utilized by SCI for both clinical and non-clinical meetings, telemedicine has enhanced opportunities for collaboration within the Caribbean region. By building capacity and nurturing expert knowledge through education, SCI hopes to contribute to closing the gap in childhood survival between high and low-resource settings.

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Introdução: Estudos recentes mostram associação entre tireoidites autoimunes e esclerose sistêmica (ES), e sugerem que essa condição pode interferir no fenótipo da ES. Entretanto, esses estudos avaliam as tireoidites autoimunes como um todo e nenhum deles aborda especificamente a tireoidite de Hashimoto (TH) na ES. Objetivo: Investigar a presença de TH em pacientes com ES e sua possível associação com as manifestações da doença. Casuística e métodos: Manifestações clínicas de hipotireoidismo, TSH, T4 livre e anticorpos antitireoidanos (anti-TPO, anti TBG e TRAb) foram pesquisados em 56 pacientes com ES. Pacientes com ES e TH foram comparados com pacientes com ES sem tireoidite. Resultados: TH foi observada em 19,64% dos pacientes com ES. Não foi encontrada associação entre a TH e as diferentes formas de doença ou com o perfil de autoanticorpos. Da mesma forma, não houve diferença entre a média do escore de Rodnan modificado e entre a presença de fenômeno de Raynaud, cicatrizes estelares, necrose digital, miosite, artrite, sintomas sicca, dismotilidade esofágica ou crise renal esclerodérmica quando os grupos foram comparados. Por outro lado, pacientes com TH apresentaram maior frequência de hipertensão pulmonar quando comparados a pacientes sem TH (66,6% vs 22,5%; p = 0,016). Conclusões: Na amostra de ES estudada, a TH está associada a uma maior prevalência de hipertensão pulmonar. Estudos de seguimento a longo prazo, englobando um número maior de pacientes com ES e TH, são necessários para confirmar esses dados. .

Introduction: Recent studies show an association between autoimmune thyroiditis and systemic sclerosis (SSc) and suggest that this condition may interfere with the ES phenotype. However these studies evaluate the autoimmune thyroiditis as a whole and none of them specifically addresses Hashimoto's thyroiditis (HT) in SSc. Objective: To investigate the presence of HT in SSc patients and its possible association with disease manifestations. Methods: Clinical manifestations of hypothyroidism, TSH and anti-thyroid auto antibodies (anti-TPO. anti TBG and TRAb) were studied in 56 patients with SSc. SSc patients with HT were compared with SSc patients without thyroiditis. Results: HT was observed in 19.64% of patients with SSc. No association was observed between HT and the different forms of disease or profile of autoantibodies. Likewise, there was no difference between the mean modified Rodnan score and presence of Raynaud's phenomenon, scars, digital necrosis, myositis, arthritis, sicca symptoms, esophageal dysmotility and scleroderma renal crisis when the groups were compared. On the other hand, patients with HT had higher frequency of pulmonary hypertension in relation to patients without HT (66.6% vs 22.5%, p = 0.016). Conclusions: In the studied sample patients with ES and HT had higher prevalence of pulmonary hypertension. Long-term follow-up studies with a larger number of TH and SSc patients are needed to confirm these data. .

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INTRODUCTION: Recent studies show an association between autoimmune thyroiditis and systemic sclerosis (SSc) and suggest that this condition may interfere with the ES phenotype. However these studies evaluate the autoimmune thyroiditis as a whole and none of them specifically addresses Hashimoto's thyroiditis (HT) in SSc. OBJECTIVE: To investigate the presence of HT in SSc patients and its possible association with disease manifestations. METHODS: Clinical manifestations of hypothyroidism, TSH and anti-thyroid auto antibodies (anti-TPO. anti TBG and TRAb) were studied in 56 patients with SSc. SSc patients with HT were compared with SSc patients without thyroiditis. RESULTS: HT was observed in 19.64% of patients with SSc. No association was observed between HT and the different forms of disease or profile of autoantibodies. Likewise, there was no difference between the mean modified Rodnan score and presence of Raynaud's phenomenon, scars, digital necrosis, myositis, arthritis, sicca symptoms, esophageal dysmotility and scleroderma renal crisis when the groups were compared. On the other hand, patients with HT had higher frequency of pulmonary hypertension in relation to patients without HT (66.6% vs 22.5%, p=0.016). CONCLUSIONS: In the studied sample patients with ES and HT had higher prevalence of pulmonary hypertension. Long-term follow-up studies with a larger number of TH and SSc patients are needed to confirm these data.

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OBJECTIVE: The physiological and mechanical properties of the skin, the primary tissue affected by systemic sclerosis, depend on the assembly of collagen types I, III and V, which form heterotypic fibers. Collagen V (COLV) regulates heterotypic fiber diameter, and the maintenance of its properties is important for maintaining normal tissue architecture and function. Based on a COLV-induced experimental SSc model, in which overexpression of abnormal COLV was a prominent feature, we assumed that this abnormality could be present in SSc patients and could be correlated to disease duration, skin thickening and disease activity. METHODS: Skin biopsies from 18 patients (6 early-stage and 12 late-stage) and 10 healthy controls were studied. Skin thickening assessment was performed with the Modified Rodnan Skin Score (MRSS), and activity was calculated using the Valentini Disease Activity Index. Morphology, morphometry of COLV deposition in dermis, as well as, quantitative RT-PCR and 3D-reconstruction of the dermal fibroblast culture were performed. RESULTS: Structurally abnormal COLV was overexpressed in SSc skin, mainly in the early stages of the disease, when compared to normal controls and late-stage. A positive correlation between COLV expression and MRSS and disease activity was observed. Collagen V alpha-1 and alpha-2 mRNA expression levels were higher in SSc. Tridimensional reconstruction of SSc dermal heterotypic fibers confirmed the presence of atypical COLV. CONCLUSION: Increased synthesis of abnormal COLV and its correlation with disease stage, activity and MRSS suggest that this collagen can be a possible trigger involved in the pathogenesis of SSc.

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El objetivo de este estudio fue traducir y analizar las propiedades psico-métricas de la versión española de la Échelle des Perceptions du Soutien á l'Autonomie en Sport, en un contexto deportivo. Los resultados apoyaron la estructura unifactorial de la escala. Asimismo, el modelo de ecuaciones estructurales mostró evidencias de validez concurrente, en el que la percepción del apoyo a la autonomía predice de manera positiva la percepción de autonomía y la percepción de competencia y estas, a su vez, predicen la motivación intrínseca, que muestra un efecto positivo sobre las emociones positivas durante la práctica deportiva. Finalmente, se muestran evidencias de fiabilidad. Los resultados apoyan preliminarmente el uso de la versión española en el contexto deportivo.

The aim of the present research was to translate and to analyze the psychometric properties of the Spanish version of the Échelle des Perceptions du Soutien á l'Autonomie en Sport in sport context. Results supported the unidimentional structure of the scale. The predictive validity was tested using a structural equation model which perceived autonomy support predicted the perceived autonomy and perceived competence and these two the intrinsic motivation. Finally, intrinsic motivation predicted positive emotions. Likewise, results showed evidences of reliability. Results support preliminary the use of the Spanish version of the scale in sport context.

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BACKGROUND: The incidence and outcome of Herpes zoster (HZ) in systemic lupus erythematosus (SLE) are not completely defined as well as the relevance to HZ of disease and therapy factors. OBJECTIVE: To determine HZ features in SLE. PATIENTS AND METHODS: SLE patients (1997 update of the American College of Rheumatology classification criteria) with definitive HZ infection were identified from our Lupus Clinic computerized database of 1145 patients. RESULTS: HZ was diagnosed in 51 SLE patients (4.45%) with an annual incidence rate of 6.4 events/1000 patient-years. At HZ diagnosis, mean disease duration was 9.78 +/- 8.37 years, median Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was 1, and only 17.6% had SLEDAI >or=8. Frequency of manifestations and immunosuppressor use were similar between patients with and without HZ. Forty-two patients (82.5%) with HZ were under prednisone with concomitant immunosuppressive therapy in 66.7%. Thirty-five patients (68.6%) were using immunosuppressors: azathioprine (39.2%), cyclophosphamide (9.8%), and mycophenolate mofetil (9.8%). The mean lymphocyte count was 1219 +/- 803/mm3 (43.1% <1000/mm3 and 17.6% <500/mm3). Only patients using azathioprine and cyclophosphamide had lymphocyte counts <500/mm3 (15% and 40%).All patients received acyclovir, 19.6% had postherpetic neuralgia, and recurrence occurred in only 7.8%. Thoracic nerves were the most involved site (56.8%) followed by lumbar (23.5%). Bacterial suprainfection occurred in 11.7% but was not associated with therapy, lymphocyte count, or SLEDAI scores (P > 0.05). CONCLUSION: This is the largest cohort to determine that HZ is a late SLE complication with some peculiar features, such as good prognosis and typical dermatomal distribution. In addition, we have identified that the major trigger factor for this viral infection in SLE is therapy, particularly the concomitant use of corticosteroid and immunosuppressors, and not active disease.

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