Sinonasal mucormycosis during 2 years of COVID-19 pandemic in central Iran: incidence rate and clinical features / Mucormicosis sinonasal durante 2 años de pandemia de COVID-19 en el centro de Irán: tasa de incidencia y características clínicas

Introduction As a novel infectious disease, COVID-19 is caused by SARS-COV-2, spreading rapidly worldwide. ENT specialists have faced this challenging disease in various ways since the emergence of the COVID-19 pandemic. We are currently facing an increase in cases referred due to sinonasal mucormycosis which is a rare but invasive, rapidly progressive, and life-threatening infection. We provide an overview of this disease's incidence rate and clinical features. Methods This descriptive cross-sectional study was conducted on 46 sinonasal mucormycosis patients who were histopathologically confirmed after sinonasal endoscopic surgery in our educational therapeutic hospital during 2 years of the COVID-19 pandemic from March 20, 2020, to March 20, 2022. Results There was an increase in the incidence of mucormycosis more than twice as much as before. All patients had a history of COVID-19 and 69.6% were diabetic. The median time to symptom onset from COVID-19 detection was 3.3 weeks. A total of 60.9% received steroids while 85.7% were prescribed during COVID-19 treatment. The most common manifestation was orbital involvement (80.4%). Of the 46 study cases, unfortunately, 17 (37%) died. An exciting point in our study was the incidence of peripheral facial palsy which is associated involvement of multiple other cranial nerves (II, III, IV, V, VI) considered to be the likely occurrence of a rare phenomenon called Garcin's syndrome. Conclusion Based on the results of this study, during 2 years of the COVID -19 pandemic, there was an increase in the incidence of sinonasal mucormycosis more than twice as much as before. (AU)

Introducción La enfermedad infecciosa COVID-19, causada por el SARS.COV-2 se ha extendido rápidamente por el mundo. Como otorrinolaringólogos, nos hemos enfrentado a esta enfermedad de diversas maneras durante el periodo de pandemia. Actualmente evidenciamos a un aumento de casos de mucormicosis nasosinusal, infección rara pero invasiva, rápidamente progresiva y amenazante para la vida. Presentamos una descripción general de la incidencia de esta enfermedad, así como sus características clínicas. Métodos Estudio descriptivo transversal de 46 pacientes con mucormicosis nasosinusal confirmados histopatológicamente en un hospital universitario de Irán desde el 20 marzo de 2020 hasta el 20 de marzo de 2022. Resultados El aumento en la incidencia de mucormicosis ha sido más del doble en comparación con el pasado. Los pacientes todos tenían antecedentes de COVID-19, y el 69.6 % de ellos eran diabéticos. El plazo promedio de aparición de los síntomas ha sido 3.3 semanas desde la detección del COVID-19. Un total de 60.9% de pacientes recibieron esteroides mientras que el 85.7% fueron recetados durante el tratamiento contra el COVID-19. La manifestación más frecuente fue la afectación orbitaria (80.4%). De los 46 casos de estudio, lamentablemente, 17 (37%) fallecieron. Un punto emocionante en nuestro estudio fue la incidencia de parálisis facial periférica que está afectada y asociada con la participación de muchos otros nervios craneales (II, III, IV, V, VI) que se considera que es la probable ocurrencia de un fenómeno raro llamado síndrome de Garcin. Conclusión En base a los resultados de este estudio podemos concluir que la incidencia de mucormicosis nasosinusal se duplicó en Irán durante el periodo de pandemia. (AU)

Behçet disease in Latin American countries: A systematic literature review of demographic and clinical features, and HLA-B*51 allele frequency.

OBJECTIVE: To describe the demographic and clinical features, as well as the frequency of the HLA-B*51 allele in Behçet disease (BD) patients in Latin American countries. METHODS: A systematic literature review of PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) guidelines was conducted without performing a meta-analysis. We included observational studies (cross-sectional or cohort) of BD patients fulfilling the International Study Group for BD classification criteria and reported the demographic, clinical, and laboratory features of the disease in adult patients. RESULTS: Twelve studies were included in the SLR. Information from 532 patients across 5 Latin American countries was included for the analysis. Mean age at disease diagnosis was 33 years, 58.3% were female and 41.7% male; most patients were non-Caucasian. The most common clinical manifestations were recurrent oral ulcers and genital ulcers, followed by skin, eye, joint, neurological, gastrointestinal, vascular, and cardiac involvement. The prevalence of BD was described in 2 studies, 1 conducted in Brazil that reported a prevalence of .3/100,000 inhabitants, and another in Colombia with a prevalence of 1.1/100,000 inhabitants. The frequency of HLA-B*51 allele in BD patients was 38%, 30.1%, and 9% in Argentina, Brazil, and Mexico, respectively. CONCLUSIONS: The prevalence of BD in the Latin American countries seems to be low, as well as the frequency of HLA-B*51 allele. However, the strength of association between HLA-B*51 and BD remains high in our population. The key clinical features of BD are like those reported in countries/regions where BD is endemic.

Behçet disease in Latin American countries: A systematic literature review of demographic and clinical features, and HLA-B*51 allele frequency / Enfermedad de Behçet en países de Latinoamérica: Revisión sistemática de la literatura sobre datos demográficos, hallazgos clínicos y frecuencia del alelo HLA-B*51

Objective: To describe the demographic and clinical features, as well as the frequency of the HLA-B*51 allele in Behçet disease (BD) patients in Latin American countries. Methods: A systematic literature review of PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) guidelines was conducted without performing a meta-analysis. We included observational studies (cross-sectional or cohort) of BD patients fulfilling the International Study Group for BD classification criteria and reported the demographic, clinical, and laboratory features of the disease in adult patients. Results: Twelve studies were included in the SLR. Information from 532 patients across 5 Latin American countries was included for the analysis. Mean age at disease diagnosis was 33 years, 58.3% were female and 41.7% male; most patients were non-Caucasian. The most common clinical manifestations were recurrent oral ulcers and genital ulcers, followed by skin, eye, joint, neurological, gastrointestinal, vascular, and cardiac involvement. The prevalence of BD was described in 2 studies, 1 conducted in Brazil that reported a prevalence of .3/100,000 inhabitants, and another in Colombia with a prevalence of 1.1/100,000 inhabitants. The frequency of HLA-B*51 allele in BD patients was 38%, 30.1%, and 9% in Argentina, Brazil, and Mexico, respectively. Conclusions: The prevalence of BD in the Latin American countries seems to be low, as well as the frequency of HLA-B*51 allele. However, the strength of association between HLA-B*51 and BD remains high in our population. The key clinical features of BD are like those reported in countries/regions where BD is endemic.(AU)

Objetivo: Describir las características demográficas, clínicas y la frecuencia del alelo HLA-B*51 en pacientes con enfermedad de Behçet (EB) en países de América Latina. Métodos: Se llevó a cabo una revisión sistemática de la literatura (RSL) según la guía PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) sin realizar un metaanálisis final. Se incluyeron estudios observacionales (transversales o de cohortes) de pacientes con EB que cumplieron con los criterios de clasificación del Grupo Internacional de Estudio de la EB e informaron las características demográficas, clínicas y de laboratorio en pacientes adultos con EB. Resultados: Doce estudios fueron incluidos para la RSL. La información de 532 pacientes provenientes de 5 países de América Latina se incluyó para el análisis. La edad media al diagnóstico fue de 33 años, el 58,3% fueron mujeres y el 41,7% hombres; la mayoría de los pacientes fueron no caucásicos. Las manifestaciones clínicas más comunes fueron las úlceras orales y genitales recurrentes, seguidas del compromiso cutáneo, ocular, articular, neurológico, gastrointestinal, vascular y cardíaco. La prevalencia de la EB fue descrita en 2 estudios, uno realizado en Brasil que reportó una prevalencia de 0,3/100.000 habitantes, y otro en Colombia con una prevalencia de 1,1/100.000 habitantes. La frecuencia del HLA-B*51 en pacientes con EB fue del 38%, 30,1% y 9% en Argentina, Brasil y México, respectivamente. Conclusiones: La prevalencia de la EB en los países latinoamericanos parece ser baja, así como la frecuencia del alelo HLA-B*51. Sin embargo, la fuerza de asociación entre el HLA-B*51 y la EB sigue siendo alta en nuestra población. Las características clínicas claves de la EB son similares a las reportadas en países/regiones donde es endémica.(AU)

The role of anti-HMGB1 antibody and anti-moesin antibody in ANCA-associated vasculitis / Papel de los anticuerpos anti-HMGB1 y antimoesina en la vasculitis asociada a ANCA

Objective The study aims to evaluate the role of anti-high mobility group box 1 (HMGB1) antibody and anti-moesin antibody in the diagnosis of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and its possible relationship with the different clinical manifestations. Methods The study involved 60 AAV patients, 58 patients with autoimmune disease other than AAV and 50 healthy subjects. The serum levels of anti-HMGB1 and anti-moesin antibodies were determined by enzyme-linked immunosorbent assay (ELISA), and the second determination was made 3 months after treatment of AAV patients. Results Serum levels of anti-HMGB1 and anti-moesin antibodies in AAV group were significantly higher than those in non-AAV group and HC group. The area under the curve (AUC) of anti-HMGB1 and anti-moesin in diagnosing AAV were 0.977 and 0.670, respectively. Anti-HMGB1 levels were significantly elevated in AAV patients with pulmonary involvement, while the concentrations of anti-moesin were significantly increased in patients with renal damage. Anti-moesin were positively correlated with BVAS (r=0.261, P=0.044), creatinine (r=0.296, P=0.024) and negatively correlated with complement C3 (r=−0.363, P=0.013). Besides, anti-moesin levels of active AAV patients were significantly higher than those in inactive patients. The concentrations of serum anti-HMGB1 could be significantly decreased after induction remission treatment (P<0.05). Conclusion Anti-HMGB1 and anti-moesin antibodies play important roles in the diagnosis and prognosis of AAV, which may act as potential disease markers for AAV (AU)

Objetivo El estudio tiene como objetivo evaluar el papel del anticuerpo antigrupo de alta movilidad box 1 (HMGB1) y el anticuerpo antimoesina en el diagnóstico de la vasculitis asociada (VAA) a anticuerpos citoplasmáticos antineutrófilos (ANCA) y su posible relación con las diferentes manifestaciones clínicas. Métodos En el estudio participaron 60 pacientes con VAA, 58 pacientes con enfermedad autoinmune distinta de la VAA y 50 sujetos sanos. Los niveles séricos de anticuerpos anti-HMGB1 y antimoesina se determinaron mediante ensayo inmunoabsorbente ligado a enzimas (ELISA), y la segunda determinación se realizó tres meses después del tratamiento de pacientes con VAA. Resultados Los niveles séricos de anticuerpos anti-HMGB1 y antimoesina en el grupo AAV fueron significativamente más altos que los del grupo sin VAA y el grupo control sanitario. El área bajo la curva (AUC) de anti-HMGB1 y antimoesina en el diagnóstico de VAA fueron 0,977 y 0,670, respectivamente. Los niveles de anti-HMGB1 se elevaron significativamente en pacientes con VAA con afectación pulmonar, mientras que las concentraciones de antimoesina aumentaron significativamente en pacientes con daño renal. La antimoesina se correlacionó positivamente con puntuación de actividad vascular de Birmingham (r=0,261, p=0,044), creatinina (r=0,296, p=0,024) y se correlacionó negativamente con el complemento C3 (r=−0,363, p=0,013). Además, los niveles de antimoesina de los pacientes activos con VAA fueron significativamente más altos que los de los pacientes inactivos. Las concentraciones séricas de anti-HMGB1 podrían disminuir significativamente después del tratamiento de remisión de inducción (p<0,05). Conclusión Los anticuerpos anti-HMGB1 y antimoesina juegan un papel importante en el diagnóstico y pronóstico de VAA, que pueden actuar como marcadores potenciales de enfermedad para VAA (AU)

Características epidemiológicas y clínicas autorreportadas en pacientes con obesidad y COVID-19: un estudio de corte transversal / Epidemiological and clinical characteristics self-reported in patients with obesity and COVID-19 disease: a cross-sectional study

RESUMEN INTRODUCCIÓN En Argentina, la obesidad en adultos es una problemática con una prevalencia media de 25,4%. En la relación entre COVID-19 y obesidad, se observa mayor prevalencia de sobrepeso/obesidad y enfermedad severa entre los pacientes con COVID-19. Por ello, el objetivo del estudio fue conocer las características clínicas y epidemiológicas en pacientes con diagnóstico de obesidad sobrevivientes a la infección por SARS-CoV-2. MÉTODOS Se realizó un estudio observacional descriptivo y analítico de corte transversal. Se aplicó una encuesta autoadministrada. RESULTADOS La mediana de edad fue 41 años, y hubo un 69,9% de mujeres. Los síntomas de presentación más prevalentes fueron astenia (86,7%), anosmia (64,5%) y tos (64%). Con respecto a los síntomas persistentes luego de las 12 semanas del diagnóstico, los más prevalentes fueron astenia (52,9%), trastornos de sueño (32,4%) y falta de concentración/memoria (31,7%). Hubo mayor riesgo en mujeres (OR: 2,86; IC95%: 2,23-3,67) con obesidad (OR: 1,58; IC95%: 1,16-2,16). La enfermedad grave en obesos fue casi el triple comparado con no obesos (15,7% vs. 5,4%; p<0,001). DISCUSIÓN Los pacientes obesos mostraron síntomas de presentación que orientan a un estado inflamatorio sistémico, con disnea y tos más frecuentes, y tienen mayor prevalencia de enfermedad grave y neumonía, así como mayor riesgo de desarrollar síntomas persistentes, sobre todo si las personas son mujeres y sedentarias.

ABSTRACT INTRODUCTION In Argentina, obesity in adults is a problem with an average prevalence of 25.4%. The relationship between COVID-19 and obesity shows a higher prevalence of overweight/obesity and severe disease among patients with COVID-19. Therefore, the objective of the study was to know the clinical and epidemiological characteristics of patients diagnosed with obesity who survived SARSCoV- 2 infection. METHODS A descriptive and analytical cross-sectional observational study was carried out. A self-administered survey was applied. RESULTS The median age was 41 years and 69.9% were women. The most prevalent presenting symptoms were asthenia (86.7%), anosmia (64.5%) and cough (64%). Regarding persistent symptoms 12 weeks after diagnosis, the most prevalent ones were asthenia (52.9%), sleep disorders (32.4%) and lack of concentration/memory (31.7%). The risk was higher for women (OR: 2.865; CI95%: 2.23-3.67) with obesity (OR: 1.58; CI95%: 1.16-2.16). Severe disease in obese was almost threefold that in non-obese patients (15.7% vs. 5.4%; p<0.001). DISCUSSION Obese patients showed presenting symptoms that point to a systemic inflammatory state, with dyspnea and cough being more common, and have a higher prevalence of severe disease and pneumonia, as well as a higher risk of developing persistent symptoms, especially if they are women and sedentary.

The role of anti-HMGB1 antibody and anti-moesin antibody in ANCA-associated vasculitis.

OBJECTIVE: The study aims to evaluate the role of anti-high mobility group box 1 (HMGB1) antibody and anti-moesin antibody in the diagnosis of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and its possible relationship with the different clinical manifestations. METHODS: The study involved 60 AAV patients, 58 patients with autoimmune disease other than AAV and 50 healthy subjects. The serum levels of anti-HMGB1 and anti-moesin antibodies were determined by enzyme-linked immunosorbent assay (ELISA), and the second determination was made 3 months after treatment of AAV patients. RESULTS: Serum levels of anti-HMGB1 and anti-moesin antibodies in AAV group were significantly higher than those in non-AAV group and HC group. The area under the curve (AUC) of anti-HMGB1 and anti-moesin in diagnosing AAV were 0.977 and 0.670, respectively. Anti-HMGB1 levels were significantly elevated in AAV patients with pulmonary involvement, while the concentrations of anti-moesin were significantly increased in patients with renal damage. Anti-moesin were positively correlated with BVAS (r=0.261, P=0.044), creatinine (r=0.296, P=0.024) and negatively correlated with complement C3 (r=-0.363, P=0.013). Besides, anti-moesin levels of active AAV patients were significantly higher than those in inactive patients. The concentrations of serum anti-HMGB1 could be significantly decreased after induction remission treatment (P<0.05). CONCLUSION: Anti-HMGB1 and anti-moesin antibodies play important roles in the diagnosis and prognosis of AAV, which may act as potential disease markers for AAV.

Clinical Unmet Needs and Treatment Patterns of Paediatric Psoriasis Patients: A Real-World Evidence Study in Spain / Necesidades clínicas no satisfechas y patrones de tratamiento de los pacientes pediátricos con psoriasis: estudio de evidencia en el mundo real en España

Background and objective Real-world evidence of paediatric psoriasis (PsO) is lacking in Spain. The purpose of this study was to identify physician-reported disease burden and current treatment patterns in a real-world paediatric PsO patient cohort in Spain. This will enhance our understanding of the disease and contribute to the development of regional guidelines. Material and method This retrospective analysis of a cross-sectional market research survey assessed the clinical unmet needs and treatment patterns in patients with paediatric PsO in Spain, as reported by their primary care and specialist physicians, using data collected as part of the Adelphi Real World Paediatric PsO Disease-Specific Program (DSP™) between February and October 2020. Results Survey data from 57 treating physicians were included (71.9% [N=41] dermatologists, 17.6% [N=10] general practitioners/primary care physicians, and 10.5% [N=6] paediatricians); the final analysis included 378 patients. At sampling, 84.1% (318/378) of patients had mild disease, 15.3% (58/378) had moderate disease and 0.5% (2/378) had severe disease. Retrospectively reported physician-judged severity at the time of PsO diagnosis recorded 41.8% (158/378) of patients with mild disease, 51.3% (194/378) with moderate disease and 6.9% (26/378) with severe disease. Overall, 89.3% (335/375) of patients were currently receiving topical PsO therapy, while 8.8% (33/375), 10.4% (39/375) and 14.9% (56/375) of patients were currently receiving phototherapy, conventional systemics and biologics, respectively. Conclusions These real-world data reflect the current burden and treatment landscape of paediatric PsO in Spain. The management of patients with paediatric PsO could be improved by further educating healthcare professionals and developing regional guidelines (AU)

Antecedente y objetivo En España se carece de evidencia sobre psoriasis (PsO) pediátrica en el mundo real. El objetivo de este estudio fue identificar la carga de la enfermedad reportada por el facultativo y los patrones actuales de tratamiento en una cohorte de pacientes psoriásicos pediátricos en el mundo real. Ello ampliará nuestra comprensión de la enfermedad y contribuirá al desarrollo de directrices regionales. Material y método Este análisis retrospectivo transversal de una encuesta de investigación mercado evaluó las necesidades clínicas no satisfechas y los patrones de tratamiento en pacientes con PsO pediátrica en España, según lo reportado por sus médicos de atención primaria y especialistas, utilizando datos recopilados como parte del Disease-specific program (DSP™) de Adelphi para PsO pediátrica en el mundo real, entre los meses de febrero y octubre de 2020. Resultados Se incluyeron los datos de la encuesta realizada a 57 facultativos médicos tratantes (71,9% [N=41] de dermatólogos, 17,6% [N=10] de médicos generales de atención primaria y 10,5% [N=6] de pediatras); el análisis final incluyó 378 pacientes. En la muestra, el 84,1% (318/378) de los pacientes padeció enfermedad leve, el 15,3% (58/378) enfermedad moderada y el 0,5% (2/378) enfermedad severa. De acuerdo con el reporte retrospectivo, la gravedad juzgada por el facultativo en el momento de diagnosticarse la PsO pediátrica registró un 41,8% (158/378) de pacientes con enfermedad leve, un 51,3% (194/378) con enfermedad moderada y un 6,9% (26/378) con enfermedad severa. En general, el 89,3% (335/375) de los pacientes recibía en la actualidad terapia tópica para PsO pediátrica, mientras que el 8,8% (33/375), el 10,4% (39/375) y el 14,9% (56/375) de los pacientes recibía en la actualidad fototerapia, sistémicos y biológicos convencionales, respectivamente. Conclusiones Estos datos del mundo real reflejan la carga actual y el panorama de la PsO pediátrica en España (AU)

[Artículo traducido] Necesidades clínicas no satisfechas y patrones de tratamiento de los pacientes pediátricos con psoriasis: estudio de evidencia en el mundo real en España / Clinical Unmet Needs and Treatment Patterns of Paediatric Psoriasis Patients: A Real-World Evidence Study in Spain

Antecedente y objetivo En España se carece de evidencia sobre psoriasis (PsO) pediátrica en el mundo real. El objetivo de este estudio fue identificar la carga de la enfermedad reportada por el facultativo y los patrones actuales de tratamiento en una cohorte de pacientes psoriásicos pediátricos en el mundo real. Ello ampliará nuestra comprensión de la enfermedad y contribuirá al desarrollo de directrices regionales. Material y método Este análisis retrospectivo transversal de una encuesta de investigación mercado evaluó las necesidades clínicas no satisfechas y los patrones de tratamiento en pacientes con PsO pediátrica en España, según lo reportado por sus médicos de atención primaria y especialistas, utilizando datos recopilados como parte del Disease-specific program (DSP™) de Adelphi para PsO pediátrica en el mundo real, entre los meses de febrero y octubre de 2020. Resultados Se incluyeron los datos de la encuesta realizada a 57 facultativos médicos tratantes (71,9% [N=41] de dermatólogos, 17,6% [N=10] de médicos generales de atención primaria y 10,5% [N=6] de pediatras); el análisis final incluyó 378 pacientes. En la muestra, el 84,1% (318/378) de los pacientes padeció enfermedad leve, el 15,3% (58/378) enfermedad moderada y el 0,5% (2/378) enfermedad severa. De acuerdo con el reporte retrospectivo, la gravedad juzgada por el facultativo en el momento de diagnosticarse la PsO pediátrica registró un 41,8% (158/378) de pacientes con enfermedad leve, un 51,3% (194/378) con enfermedad moderada y un 6,9% (26/378) con enfermedad severa. En general, el 89,3% (335/375) de los pacientes recibía en la actualidad terapia tópica para PsO pediátrica, mientras que el 8,8% (33/375), el 10,4% (39/375) y el 14,9% (56/375) de los pacientes recibía en la actualidad fototerapia, sistémicos y biológicos convencionales, respectivamente. Conclusiones Estos datos del mundo real reflejan la carga actual y el panorama de la PsO pediátrica en España (AU)

Background and objective Real-world evidence of paediatric psoriasis (PsO) is lacking in Spain. The purpose of this study was to identify physician-reported disease burden and current treatment patterns in a real-world paediatric PsO patient cohort in Spain. This will enhance our understanding of the disease and contribute to the development of regional guidelines. Material and method This retrospective analysis of a cross-sectional market research survey assessed the clinical unmet needs and treatment patterns in patients with paediatric PsO in Spain, as reported by their primary care and specialist physicians, using data collected as part of the Adelphi Real World Paediatric PsO Disease-Specific Program (DSP™) between February and October 2020. Results Survey data from 57 treating physicians were included (71.9% [N=41] dermatologists, 17.6% [N=10] general practitioners/primary care physicians, and 10.5% [N=6] paediatricians); the final analysis included 378 patients. At sampling, 84.1% (318/378) of patients had mild disease, 15.3% (58/378) had moderate disease and 0.5% (2/378) had severe disease. Retrospectively reported physician-judged severity at the time of PsO diagnosis recorded 41.8% (158/378) of patients with mild disease, 51.3% (194/378) with moderate disease and 6.9% (26/378) with severe disease. Overall, 89.3% (335/375) of patients were currently receiving topical PsO therapy, while 8.8% (33/375), 10.4% (39/375) and 14.9% (56/375) of patients were currently receiving phototherapy, conventional systemics and biologics, respectively. Conclusions These real-world data reflect the current burden and treatment landscape of paediatric PsO in Spain. The management of patients with paediatric PsO could be improved by further educating healthcare professionals and developing regional guidelines (AU)

Clinical Unmet Needs and Treatment Patterns of Paediatric Psoriasis Patients: A Real-World Evidence Study in Spain. / [Artículo traducido] Necesidades clínicas no satisfechas y patrones de tratamiento de los pacientes pediátricos con psoriasis: estudio de evidencia en el mundo real en España.

BACKGROUND AND OBJECTIVE: Real-world evidence of paediatric psoriasis (PsO) is lacking in Spain. The purpose of this study was to identify physician-reported disease burden and current treatment patterns in a real-world paediatric PsO patient cohort in Spain. This will enhance our understanding of the disease and contribute to the development of regional guidelines. MATERIAL AND METHOD: This retrospective analysis of a cross-sectional market research survey assessed the clinical unmet needs and treatment patterns in patients with paediatric PsO in Spain, as reported by their primary care and specialist physicians, using data collected as part of the Adelphi Real World Paediatric PsO Disease-Specific Program (DSP™) between February and October 2020. RESULTS: Survey data from 57 treating physicians were included (71.9% [N = 41] dermatologists, 17.6% [N = 10] general practitioners/primary care physicians, and 10.5% [N = 6] paediatricians); the final analysis included 378 patients. At sampling, 84.1% (318/378) of patients had mild disease, 15.3% (58/378) had moderate disease and 0.5% (2/378) had severe disease. Retrospectively reported physician-judged severity at the time of PsO diagnosis recorded 41.8% (158/378) of patients with mild disease, 51.3% (194/378) with moderate disease and 6.9% (26/378) with severe disease. Overall, 89.3% (335/375) of patients were currently receiving topical PsO therapy, while 8.8% (33/375), 10.4% (39/375) and 14.9% (56/375) of patients were currently receiving phototherapy, conventional systemics and biologics, respectively. CONCLUSIONS: These real-world data reflect the current burden and treatment landscape of paediatric PsO in Spain. The management of patients with paediatric PsO could be improved by further educating healthcare professionals and developing regional guidelines.

Reporte preliminar de las características sociodemográficas y clínicas de los pacientes con hemopatías malignas: experiencia de un hospital universitario

Introducción: debido a su amplia diversidad clínica y biológica, es de gran importancia para el médico conocer cómo se presentan los pacientes diagnosticados con una hemopatía maligna. Objetivo: describir las características sociodemográficas y clínicas de los pacientes con diagnóstico de una hemopatía maligna que consultaron en un Hospital Universitario de Paraguay. Metodología: estudio observacional, retrospectivo, descriptivo, de corte transversal, con muestreo no probabilístico, de casos consecutivos. Se incluyó a pacientes adultos, de ambos sexos, registrados en el archivo de Hematología del Hospital de Clínicas de la Facultad de Ciencias Médicas de la Universidad Nacional de Asunción (Paraguay), con diagnóstico confirmado por anatomía patológica de una neoplasia hematológica, según la clasificación de la Organización Mundial de la Salud, durante el periodo comprendido entre enero 2019 y agosto de 2021. Se estudiaron variables sociodemográficas y clínicas. Resultados: se estudió a 129 pacientes. El 58 % correspondía al sexo masculino. La edad promedio de los pacientes fue de 50,3±17,38. La mayoría de los pacientes procedía del Departamento Central de Paraguay (56,58 %). La mayoría de los pacientes (n=37) fue diagnosticado con un linfoma de tipo no Hodgkin o con una neoplasia con origen en células plasmáticas (n=32). El tiempo promedio de aparición de los síntomas hasta la consulta fue de 3,5 meses. El síntoma más reportado fue la astenia (35 %). La palidez fue el hallazgo positivo más frecuente al examen físico. Las comorbilidades más frecuentes fueron la hipertensión arterial (39,44 %) y la diabetes mellitus (15,59 %). Discusión: el paciente típico con una hemopatía que consulta en el Hospital de Clínicas es hombre, mayor de 50 años, proveniente del Departamento Central, consulta por astenia, presenta palidez al examen físico y ha tardado más de 3 meses en consultar. Se le ha diagnosticado un linfoma no Hodgkin y tiene comorbilidades cardiovasculares o metabólicas.

Introduction: due to their wide clinical and biological diversity, it is of great importance for the physician to know how patients diagnosed with a hematological malignancy present. Objective: to describe the sociodemographic and clinical characteristics of patients diagnosed with a hematological malignancy who consulted at a University Hospital in Paraguay. Methods: observational, retrospective, descriptive, cross-sectional, retrospective, descriptive, cross-sectional study, with non-probabilistic sampling of consecutive cases. We included adult patients, of both sexes, registered in the Hematology file of the Hospital de Clínicas of the Faculty of Medical Sciences of the National University of Asunción (Paraguay), with diagnosis confirmed by pathological anatomy of a hematological neoplasm, according to the classification of the World Health Organization, during the period from January 2019 to August 2021. Sociodemographic and clinical variables were studied. Results: 129 patients were studied. Fifty-eight percent were male. The mean age of the patients was 50.3±17.38. Most of the patients were from the Central Department of Paraguay (56.58 %). Most of the patients (n=37) were diagnosed with a non-Hodgkin's lymphoma or a neoplasm with plasma cell origin (n=32). The average time from symptom onset to consultation was 3.5 months. The most reported symptom was asthenia (35%). Pallor was the most frequent positive finding on physical examination. The most frequent comorbidities were arterial hypertension (39.44%) and diabetes mellitus (15.59%). Discussion: the typical patient with a hemopathy who consults at the Hospital de Clínicas is male, over 50 years of age, from the Central Department, consults for asthenia, presents pallor on physical examination and has taken more than 3 months to consult. He has been diagnosed with non-Hodgkin's lymphoma and has cardiovascular or metabolic comorbidities.

Clinical Unmet Needs and Treatment Patterns of Paediatric Psoriasis Patients: A Real-World Evidence Study in Spain.

BACKGROUND AND OBJECTIVE: Real-world evidence of paediatric psoriasis (PsO) is lacking in Spain. The purpose of this study was to identify physician-reported disease burden and current treatment patterns in a real-world paediatric PsO patient cohort in Spain. This will enhance our understanding of the disease and contribute to the development of regional guidelines. MATERIAL AND METHOD: This retrospective analysis of a cross-sectional market research survey assessed the clinical unmet needs and treatment patterns in patients with paediatric PsO in Spain, as reported by their primary care and specialist physicians, using data collected as part of the Adelphi Real World Paediatric PsO Disease-Specific Program (DSP™) between February and October 2020. RESULTS: Survey data from 57 treating physicians were included (71.9% [N=41] dermatologists, 17.6% [N=10] general practitioners/primary care physicians, and 10.5% [N=6] paediatricians); the final analysis included 378 patients. At sampling, 84.1% (318/378) of patients had mild disease, 15.3% (58/378) had moderate disease and 0.5% (2/378) had severe disease. Retrospectively reported physician-judged severity at the time of PsO diagnosis recorded 41.8% (158/378) of patients with mild disease, 51.3% (194/378) with moderate disease and 6.9% (26/378) with severe disease. Overall, 89.3% (335/375) of patients were currently receiving topical PsO therapy, while 8.8% (33/375), 10.4% (39/375) and 14.9% (56/375) of patients were currently receiving phototherapy, conventional systemics and biologics, respectively. CONCLUSIONS: These real-world data reflect the current burden and treatment landscape of paediatric PsO in Spain. The management of patients with paediatric PsO could be improved by further educating healthcare professionals and developing regional guidelines.

Sinonasal mucormycosis during 2 years of COVID-19 pandemic in central Iran: incidence rate and clinical features.

INTRODUCTION: As a novel infectious disease, COVID-19 is caused by SARS-COV-2, spreading rapidly worldwide. ENT specialists have faced this challenging disease in various ways since the emergence of the COVID-19 pandemic. We are currently facing an increase in cases referred due to sinonasal mucormycosis which is a rare but invasive, rapidly progressive, and life-threatening infection. We provide an overview of this disease's incidence rate and clinical features. METHODS: This descriptive cross-sectional study was conducted on 46 sinonasal mucormycosis patients who were histopathologically confirmed after sinonasal endoscopic surgery in our educational therapeutic hospital during 2 years of the COVID-19 pandemic from March 20, 2020, to March 20, 2022. RESULTS: There was an increase in the incidence of mucormycosis more than twice as much as before. All patients had a history of COVID-19 and 69.6% were diabetic. The median time to symptom onset from COVID-19 detection was 3.3 weeks. A total of 60.9% received steroids while 85.7% were prescribed during COVID-19 treatment. The most common manifestation was orbital involvement (80.4%). Of the 46 study cases, unfortunately, 17 (37%) died. An exciting point in our study was the incidence of peripheral facial palsy which is associated involvement of multiple other cranial nerves (II, III, IV, V, VI) considered to be the likely occurrence of a rare phenomenon called Garcin's syndrome. CONCLUSION: Based on the results of this study, during 2 years of the COVID -19 pandemic, there was an increase in the incidence of sinonasal mucormycosis more than twice as much as before.

Clínica, epidemiología e imagenología del quiste óseo aneurismático en el territorio craneofacial, análisis de sus patrones histopatólogicos: revisión narrativa / Clinical, epidemiology and imaging of the aneurysmal bone cyst in the craniofacial territory, analysis of its histopathological patterns: a narrative review

Introducción: El quiste óseo aneurismático (QOA) tiene una diversidad de características clínicase imagenológicas. Existe poca información respecto a la posible relación entre algunas de estas características y los patrones histopatológicos del QOA. El propósito de la presente revisión fue analizar la clínica, epidemiología e imagenología de casos de QOA, y determinar qué características podrían tener relación con algún tipo de patrón histopatológico del QOA en el territorio craneofacial. Materiales y métodos:Se realizó una búsqueda de casos de QOA en el territorio craneofacial publicados desde el año 2000, en PubMed, Scopus y Web of Science, mediante la búsqueda ("Aneurysmal bone cyst") AND ("Skull" OR "Jaw" OR "Mandible" OR "Maxilla"). Los casos debían tener información histopatológica, clínica, epidemiológica e imagenológica. Revisión: Se incluyó 40 artículos con 41 lesiones, siendo 34 de patrón clásico y 7 de patrón mixto-sólido (M-S). El patrón M-S se presentó mayoritariamente sin dolor y en el sexo masculino, mientras que el patrón clásico con dolor asociado y en el sexo femenino. Respecto a la imagenología, el patrón M-S se presentó sin límites definidos-corticalizados y generó perforación ósea con mayor frecuencia que el patrón clásico. Conclusiones: Los patrones histopatológicos del QOA tienen características clínicas, epidemiológicas e imagenológicas similares. Sin embargo, difieren en sexo, dolor asociado, límites definidos corticalizados y perforación ósea, lo que permitiría orientar al diagnóstico hacia un patrón histopatológico. (AU)

Introduction: The aneurysmal bone cyst (ABC) has a variety of clinical and imaging characteristics. There is little information regarding the possible relationship between some of these characteristics and its histopathological patterns. The aim of this review was to analyze the clinical, epidemiology and imaging characteristics of ABC cases, and determine what characteristics might be related to some type of histopathological pattern of ABC in the craniofacial territory. Materials and methods: A search was conducted for ABC cases in the craniofacial territory published since 2000, on PubMed, Scopus and Web of Science, by searching ("Aneurysmal bone cyst") AND ("Skull" OR "Jaw" OR "Mandible" OR "Maxilla"). Cases had to have histopathological, clinical, epidemiological, and imaging information. Review: 40 articles with 41 ABC lesions were included, with 34 being classic pattern and 7 mixed-solid (M-S). The M-S pattern was mostly painless and in males, while the classic pattern, with associated pain was mostly in females. Regarding imaging, the M-S pattern was without defined-corticated limits and with bone perforation more frequently than the classic pattern. Conclusions: Histopathological patterns of ABC have similar clinical, epidemiological and imaging characteristics. However, they differ in sex, associated pain, defined-corticated limits and bone perforation, which would allow the diagnosis to be directed towards a specific histopathological pattern. (AU)

Clinical characteristics, management, and outcome of eosinophilic pneumonia associated with daptomycin

Objective: The association between daptomycin exposure and eosinophilic pneumonia (EP) is mainly based on case reports. The purpose of this study was to evaluate the clinical characteristics and provide more evidence for better identify and management of daptomycin-induced eosinophilic pneumonia in clinical practice.MethodsLiterature from 1991 to October 31, 2021 on EP induced by daptomycin were collected for retrospective analysis.ResultsA total of 47 patients (40 male and 7 female) from 35 studies were included. The median age was 67 years (range 28–89), and 78.7% of patients were ≥60 years. Daptomycin was mainly used in patients undergoing osteoarticular infections (63.8%). Typical initial symptoms were fever (91.5%), cough (55.3%) and dyspnea (59.6%). The median onset time of symptom was 3 weeks. EP recurred in 14.9% of patients after the re-administration of daptomycin, and 57.1% of EP recurred within 24h. Most cases were accompanied by marked accumulation of eosinophils in peripheral (41 cases) and/or bronchoalveolar lavage fluid (27 cases). The main radiological features were pulmonary infiltration, ground glass opacity or consolidation in CT/CXR. All patients had symptom resolution after discontinuation of daptomycin except for one patient died due to the progression of the primary disease, the median time to symptoms relief was 3 days. Corticosteroids have been shown to help symptoms relief in some cases (59.6%).ConclusionDaptomycin-induced eosinophilic pneumonia is a rare and serious complication. Physicians should consider eosinophilic pneumonia as a differential diagnosis when receiving daptomycin therapy, particularly in elderly male patients. (AU)

Objetivo: La asociación entre la exposición a daptomicina y la neumonía eosinofílica (NE) se basa principalmente en informes de casos. El propósito de este estudio fue evaluar las características clínicas y proporcionar más evidencia para una mejor identificación y tratamiento de la NE inducida por daptomicina en la práctica clínica.MétodosSe recopiló literatura médica desde 1991 hasta el 31 de octubre de 2021 sobre NE inducida por daptomicina para un análisis retrospectivo.ResultadosSe incluyeron un total de 47 pacientes (40 hombres y 7 mujeres) de 35 estudios. La mediana de edad fue de 67 años (rango 28-89), y el 78,7% de los pacientes tenían≥60 años. La daptomicina se utilizó principalmente en pacientes con infecciones osteoarticulares (63,8%). Los síntomas iniciales típicos fueron fiebre (91,5%), tos (55,3%) y disnea (59,6%). La mediana del tiempo de aparición de los síntomas fue de 3 semanas. La NE reapareció en el 14,9% de los pacientes después de la readministración de daptomicina, y el 57,1% lo hizo dentro de las primeras 24h. La mayoría de los casos se acompañó de una marcada acumulación de eosinófilos en tejidos periféricos (91,1%)/pulmonares (7 casos) y/o líquido de lavado broncoalveolar (27 casos). Las principales características radiológicas fueron infiltración pulmonar, opacidad «en vidrio deslustrado» o consolidación en TC/CXR. Todos los pacientes tuvieron una resolución de los síntomas después de la interrupción de la daptomicina, excepto uno que falleció debido a la progresión de la enfermedad primaria; la mediana de tiempo hasta el alivio de los síntomas fue de 3 días. Se ha demostrado que los corticoides ayudan al alivio de los síntomas en algunos casos (59,6%).ConclusiónLa NE inducida por daptomicina es una complicación rara y grave. Los médicos deben considerar la NE como un diagnóstico diferencial cuando un paciente recibe tratamiento con daptomicina, particularmente en varones de edad avanzada. (AU)

Clinical characteristics, management, and outcome of eosinophilic pneumonia associated with daptomycin.

OBJECTIVE: The association between daptomycin exposure and eosinophilic pneumonia (EP) is mainly based on case reports. The purpose of this study was to evaluate the clinical characteristics and provide more evidence for better identify and management of daptomycin-induced eosinophilic pneumonia in clinical practice. METHODS: Literature from 1991 to October 31, 2021 on EP induced by daptomycin were collected for retrospective analysis. RESULTS: A total of 47 patients (40 male and 7 female) from 35 studies were included. The median age was 67 years (range 28-89), and 78.7% of patients were ≥60 years. Daptomycin was mainly used in patients undergoing osteoarticular infections (63.8%). Typical initial symptoms were fever (91.5%), cough (55.3%) and dyspnea (59.6%). The median onset time of symptom was 3 weeks. EP recurred in 14.9% of patients after the re-administration of daptomycin, and 57.1% of EP recurred within 24h. Most cases were accompanied by marked accumulation of eosinophils in peripheral (41 cases) and/or bronchoalveolar lavage fluid (27 cases). The main radiological features were pulmonary infiltration, ground glass opacity or consolidation in CT/CXR. All patients had symptom resolution after discontinuation of daptomycin except for one patient died due to the progression of the primary disease, the median time to symptoms relief was 3 days. Corticosteroids have been shown to help symptoms relief in some cases (59.6%). CONCLUSION: Daptomycin-induced eosinophilic pneumonia is a rare and serious complication. Physicians should consider eosinophilic pneumonia as a differential diagnosis when receiving daptomycin therapy, particularly in elderly male patients.

Comparación de las características epidemiológicas, clínicas y quirúrgicas de pacientes con y sin recidiva de hernias inguinales operados en el Centro Médico Naval.

BACKGROUND: Recurrent inguinal hernia is currently considered a complex hernia of the abdominal wall after it has been managed with a primary procedure, which represents a more technically challenging hernia. OBJECTIVE: To determine the difference in epidemiological, clinical and surgical characteristics of patients with recurrent inguinal hernia and patients without recurrence. METHOD: Analytical cross-sectional study, in people over 18 years of age after surgery for inguinal hernia at the Naval Medical Center during the period from July 1, 2016 to June 30, 2019, integrating two study groups. RESULTS: 106 patients were included, 29 surgeries for recurrence, predominance of men and older than 60 years. Differences was found between the group of surgery for recurrence and the group of primary surgery in surgical time in minutes, with medians of 115 (p25 75 and p75 154) and 72 (p25 58 and p75 110), respectively (p = 0.001); type of open approach, 62.1% and 88.3% (p = 0.005), and type of regional anesthesia, 48.3% and 75.3% (p = 0.008). Correlation between body mass index and size of the hernial defect was explored, obtaining r of -0.099 and p = 0.652, with a null correlation between them. CONCLUSIONS: With the findings obtained in this study, it is possible to hypothesize the behavior of the naval population which may differ from the general population and thus establish follow-up protocols to give magnitude to already known factors.

ANTECEDENTES: La hernia inguinal recidivante es considerada actualmente como una hernia compleja de la pared abdominal después de que se haya manejado con un procedimiento primario, lo que representa una hernia con un mayor desafío técnico. OBJETIVO: Determinar las diferencias en las características epidemiológicas, clínicas y quirúrgicas de pacientes con hernia inguinal recidivante y sin recidiva. MÉTODO: Estudio transversal analítico en mayores de 18 años operados por hernia inguinal en el Centro Médico Naval durante el periodo del 1 de julio de 2016 al 30 de junio de 2019, integrando dos grupos de estudio. RESULTADOS: Se incluyeron 106 pacientes, 29 cirugías por recidiva, predominio de hombres y mayores de 60 años. Se encontraron diferencias entre el grupo de cirugía por recidiva y el grupo de cirugia primaria en tiempo quirúrgico, con medianas de 115 (p25 75 y p75 154) y 72 (p25 58 y p75 110) minutos, respectivamente (p = 0.001); tipo de abordaje, abierto 62.1% y 88.3% (p = 0.005); y tipo de anestesia, regional 48.3% y 75.3% (p = 0.008). Se exploró la correlación entre el índice de masa corporal y el tamaño del defecto herniario, obteniendo r de −0.099 y p = 0.652, con una correlación nula entre ambas. CONCLUSIONES: Con los hallazgos obtenidos en este estudio es posible plantear hipótesis del comportamiento de la población naval que pueda diferir de la población general, y con esto establecer protocolos de seguimiento para dar magnitud a factores ya conocidos.

Viruela símica: descripción de dos casos en seguimiento en Buenos Aires / Monkeypox: description of two cases under follow-up in Buenos Aires

Resumen La viruela símica es una enfermedad endémica en varios países de África. En mayo de 2022 varios países donde la viruela símica no es endémica notificaron casos, incluyendo algunos países de las Américas. El 23 de julio de 2022, el Director General de la OMS declaró que este brote multinacional constituye una emergencia de salud pública de importancia internacional. Comunicamos dos casos de pacientes en segui miento en la Ciudad de Buenos Aires, Argentina, entre junio y julio de 2022. Ambos eran hombres que tienen sexo con hombres, con aparición de lesiones en zona genital sin período prodrómico. En los dos casos se realizó tratamiento en primera instancia con sospecha de infecciones de transmisión sexual. Señalamos la importancia de considerar esta enfermedad como diagnóstico diferencial teniendo en cuenta el contexto epidemiológico actual.

Abstract Monkeypox is an endemic disease in several African countries. In May 2022, an outbreak was repor ted in dozens of non-endemic countries. On July 23, 2022, the WHO Director-General declared this multinational outbreak a public health emergency of international concern. We report two cases of patients under follow-up in Buenos Aires, Argentina, between June and July 2022. Both were men who have sex with men, with the appea rance of lesions in the genital area without a prodromal period. In both cases, treatment was carried out in the first instance with suspicion of sexually transmitted infections. We highlight the importance of considering this pathology as a differential diagnosis, taking into account the current epidemiological context.

Clinical and serological characteristics of Cuban patients with Systemic Lupus Erythematosus

Introduction: Systemic lupus erythematosus (SLE) is the prototype of systemic autoimmune disease with clinical characteristics that show geographic variations. However, these differences between regions have not been fully described; therefore, the objective of this study is to describe the clinical and sociodemographic characteristics of Cuban patients with SLE. Patients and methods: 149 patients with SLE and 151 with other systemic autoimmune diseases were studied. Sociodemographic and clinical characteristics according on the criteria of the American College of Rheumatology of 1997 were identified. To evaluate the associations between clinical manifestations and SLE, a logistic regression analysis was performed; the odds ratio (OR) was calculated with its corresponding 95% confidence interval and the method of multiple correspondence analysis was also used. By an analysis of the configurations of frequency the typical combinations of criteria related to the patients with SLE were identified. Results: The most frequent criteria in SLE were immunological disorders (85.2%). ANA positive (85.2%) arthritis (78.5%), photosensitivity (77.2%), and malar rash (61%). The renal involvement and immunological disorders criteria were the best (highest OR) at discriminating SLE patients. The combination of only three criteria (malar rash, positive ANA, and immunological disorder) could be enough to classify a homogeneous population. Conclusions: This study enabled us to determine the main clinical characteristics of patients with SLE in Cuba. This information could be useful to improve the efficiency of SLE diagnosis and facilitate more specific treatments.

Introducción: El lupus eritematoso sistémico (LES) es el prototipo de enfermedad autoinmune sistémica, con características clínicas que muestran variaciones geográficas. Sin embargo, estas diferencias entre regiones no están completamente descritas, por lo cual, el objetivo de este trabajo es describir las características clínicas y sociodemográficas de pacientes cubanos con LES. Pacientes y métodos: Se hizo un estudio con 149 pacientes con LES y 151 con otras enfermedades autoinmunes sistêmicas. Se identificaron sus características sociodemográficas y clínicas, basadas principalmente en los criterios del Colegio Americano de Reumatología de 1997. Para evaluar las asociaciones entre las manifestaciones clínicas y el LES se llevó a cabo un análisis de regresión logística, se calculó la odds ratio, con su correspondiente intervalo de confianza al 95%, y se empleó la técnica de análisis de correspondencia múltiple. Mediante un análisis frecuencial de las configuraciones, se identificaron las combinaciones típicas de criterios relacionadas con los pacientes con LES. Resultados: Los criterios más frecuentes en el LES fueron: alteraciones inmunológicas (85,2%), ANA positivo (85,2%), artritis (78,5%), fotosensibilidad (77,2%) y rash malar (61%). Los criterios afección renal y alteraciones inmunológicas son los que mejor (mayor valor de la odds ratio) discriminan a los pacientes con LES. La combinación de solamente tres criterios (rash malar, ANA positivo y alteraciones inmunológicas) podría ser suficiente para clasificar a una población homogénea. Conclusiones: El estudio permitió conocer las principales características clínicas de pacientes con LES en Cuba. Esta información puede ser útil para mejorar la eficacia del diagnóstico del LES y favorecer la aplicación de tratamientos más específicos.

Clinical features, diagnostic evaluation and responses to treatment in Paget's disease of bone in a center specialized in bone metabolism / Características clínicas, evaluación diagnóstica y respuestas al tratamiento de la enfermedad de Paget ósea en un centro especializado en metabolismo óseo

Abstract The epidemiology of Paget's disease of bone (PDB) has changed in the last years but there is no update data on its clinical presentation, diagnosis and management in Latin America. Our aim was to describe its clinical features, diagnostic evaluation and responses to treatment in a group of PDB patients treated between June 2012 and December 2019 in an institution specialized in bone diseases, in Buenos Aires, Argentina. The frequency of PDB (180/10 714) was 1.68%. Median age was 67 (range 39-97) years and 59.5% were women. Most patients were asymptomatic (58.6%) and had monostotic disease (54.3%). Favorable responses were obtained in all patients who were treated with zoledronate (n = 36), in 10 out of 14 treated with pamidronate, in 9 out of 10 who received intravenous ibandronate and in 12 out of 13 who received oral bisphosphonates. The response rates were not significantly different when we compared monostotic vs. polyostotic disease. Among the biochemical parameters, mean values of bone specific and total alkaline phosphatase, and C-terminal cross-linked telopeptide of type I collagen decreased significantly after treatment with bisphosphonates. It seems that our results reflect the change in PDB epidemiology towards a more indolent disease. In the future, this would probably allow physicians to use lower doses of bisphosphonates than the ones historically recommended for these patients.

Resumen La epidemiología de la en fermedad de Paget ósea (EPO) ha cambiado en los últimos años. Son necesarios datos actualizados sobre su forma de presentación clínica, diagnóstico y tratamiento en nuestra región. Nuestro objetivo fue describir las características clínicas, evaluación diagnóstica y respuestas al tratamiento de un grupo de pacientes con EPO en un centro especializado en salud ósea de Buenos Aires, Argentina. Se evaluaron todos los pacientes que fueron atendidos en nuestra institución por enfermedades óseas entre junio de 2012 y diciembre de 2019. La frecuencia de EPO (180/10 714) fue de 1.68%. La mediana de edad fue de 67 (rango 39-97) años. El 59.5% eran mujeres. La mayoría se encontraba asintomático (58.6%) y tenían enfermedad monostótica (54.3%). Se objetivaron respuestas favorables en todos los que recibieron zoledronato (n = 36), en 10 de 14 pacientes que recibieron pamidronato, en 9 de 10 que utilizaron ibandronato endovenoso y en 12 de 13 con bifosfonatos orales. Los porcentajes de respuesta no variaron significativamente entre pacientes con formas monostóticas y poliostóticas. Entre los parámetros bioquímicos, los valores de fosfatasa alcalina total y ósea y de β cross-laps disminuyeron significativamente luego del tratamiento con bifosfonatos. Nuestros resultados reflejarían un cambio en la epidemiología de la EPO hacia una forma de presentación más indolente. Esto permitiría probablemente el uso de dosis más bajas de bifosfonatos que las históricamente recomendadas para estos pacientes.

Clínica, epidemiología e imagenología del fibroma osificante y fibroma cemento osificante: revisión de casos reportados en la literatura / Clinic, epidemiology and imagenology of the ossifying fibroma and cemento ossifying fibroma: a review of cases reported in the literature

Introducción: El fibroma osificante (FO) y el fibroma cemento osificante (FCO) son patologías distintas, que hasta el 2017 se consideraban como una. A la fecha no se han comparado las características del FO y el FCO. La presente revisión tuvo como objetivo analizar características clínicas, epidemiológicas e imagenológicas del FO y FCO en los casos publicados. Materiales y métodos: Se realizó una búsqueda de casos clínicos de FO y FCOpublicados desde el año 2015 en PubMed, Scopus y Web of Science, mediante la estrategia de búsqueda ("Ossifying Fibroma" OR "Cemento Ossifying Fibroma") AND (Craniofacial OR Jaws). Los casos debían presentar información clínica, imagenológica e histológica suficientes para confirmar su diagnóstico, patrón histológico y comparar sus características. Revisión: Se incluyeron 32 artículos, con 32 casos y 34 lesiones, siendo 6 FCO y 28 FO. Los FO y FCO se diferencian por su edad y ubicación: mientras los FCO se presentan en edades adultas, exclusivamente en los maxilares y en relación con tejidos dentarios, los FO lo hacen principalmente en niños y jóvenes, y en cualquier hueso. Los FO y FCO tienen las mismas características imagenológicas: son lesiones uniloculares o multiloculares, con distintos grados de radiodensidad, límites definidos y una radiolucidez periférica. Sin embargo, los casos de FO trabecular pueden no presentar esta radiolucidez periférica. Conclusión: Los FO y FCO son patologías, clínica y epidemiológicamente similares, y con las mismas características imagenológicas. Por lo que establecer diferencias histológicas es esencial para un correcto diagnóstico. (AU)

Introduction: Ossifying fibroma (OF) and cemento ossifying fibroma (COF) are different pathologies, which until 2017 where considered as one. To date, the features of OF y COF have not been compared. This aim of this review was to analyze clinical, epidemiological and imaging features of OF and COF in published case reports. Materials and methods: A search of clinical cases of OF and COF published since 2015 was performed on PubMed, Scopus and Web of Science, using the search strategy ("Ossifying Fibroma" OR "Cemento Ossifying Fibroma") AND (Craniofacial OR Jaws). The cases had to haveenough clinical, imaging and histological information to confirm their clinical diagnosis, histological pattern and compare their features. Review: 32 articles were included, with 32 cases and 34 lesions, being 28 OF and 6 COF. OF and COF differ by age and location: while COF occur in adult ages, exclusively in the jaws and in relation to dental tissues, OF occur mainly in children and young people, and in any bone. OF and COF have the same imaging characteristics: they are unilocular or multilocular lesions, with different degrees of radiodensity, defined limits and a peripheral radiolucent area. However, cases of trabecular OF may not present this peripheral radiolucent area. Conclusions: OF and COF are similar pathologies clinically and epidemiologically, with the same imaging characteristics. So, establishing histological differences is essential for an accurate diagnosis. (AU)