Brentuximab vedotin in the treatment of cutaneous T-cell lymphomas: Data from the Spanish Primary Cutaneous Lymphoma Registry.

BACKGROUND: Brentuximab vedotin (BV) has been approved for CD30-expressing cutaneous T-cell lymphoma (CTCL) after at least one previous systemic treatment. However, real clinical practice is still limited. OBJECTIVES: To evaluate the response and tolerance of BV in a cohort of patients with CTCL. METHODS: We analysed CTCL patients treated with BV from the Spanish Primary Cutaneous Lymphoma Registry (RELCP). RESULTS: Sixty-seven patients were included. There were 26 females and the mean age at diagnosis was 59 years. Forty-eight were mycosis fungoides (MF), 7 Sézary syndrome (SS) and 12 CD30+ lymphoproliferative disorders (CD30 LPD). Mean follow-up was 18 months. Thirty patients (45%) showed at least 10% of CD30+ cells among the total lymphocytic infiltrate. The median number of BV infusions received was 7. The overall response rate (ORR) was 67% (63% in MF, 71% in SS and 84% in CD30 LPD). Ten of 14 patients with folliculotropic MF (FMF) achieved complete or partial response (ORR 71%). The median time to response was 2.8 months. During follow-up, 36 cases (54%) experienced cutaneous relapse or progression. The median progression free survival (PFS) was 10.3 months. The most frequent adverse event was peripheral neuropathy (PN) (57%), in most patients (85%), grades 1 or 2. CONCLUSIONS: These results confirm the efficacy and safety of BV in patients with advanced-stage MF, and CD30 LPD. In addition, patients with FMF and SS also showed a favourable response. Our data suggest that BV retreatment is effective in a proportion of cases.

Single muscle fiber contractile properties in diabetic RAT muscle.

INTRODUCTION: Diabetes is associated with accelerated loss of muscle mass and function. We compared the contractile properties of single muscle fibers in young rat soleus muscle of uncontrolled streptozotocin-induced diabetic animals (n = 10) and nondiabetic controls (n = 10). METHODS: Single fiber maximal force, shortening velocity, and power were assessed during maximal activation with calcium using the slack test 4 weeks after induction. Myosin heavy chain expression was determined using sodium dodecyl sulfate polyacrylamide gel electrophoresis. Oxidized myosin levels were detected by analyzing protein carbonyls in muscle homogenates. All fibers expressed the type I myosin heavy chain isoform. RESULTS: Diabetic rats had higher blood glucose (537 vs. 175 mg/dl; P < 0.001) and lower body weight (171 vs. 356 g; P < 0.001) than controls. Muscle fibers from diabetic rats showed smaller cross-sectional area (1128 vs. 1812 µm(2) ), lower maximal force (258 vs. 492 µN), and reduced absolute power (182 vs. 388 µN FL/s) (all P < 0.0001). No differences were seen in shortening velocity, specific force or specific power. Myosin carbonylation was higher (P < 0.01) in diabetic rats. CONCLUSIONS: After 4 weeks of untreated diabetes, there are significant alterations in muscle at the level of isolated single fibers and myosin protein, although some contractile properties seem to be protected. Muscle Nerve, 2015 Muscle Nerve 53: 958-964, 2016.

The Impact of Healthcare-Associated Infections in Patients Undergoing Oncological Microvascular Head and Neck Reconstruction: A Prospective Multicentre Study.

(1) Background: Healthcare-associated infections (HAIs) after head and neck free-flap reconstruction are a common postoperative complication. Risk factors for HAIs in this context and their consequences have not been adequately described. (2) Methods: Ongoing prospective multicentre study between 02/2019 and 12/2020. Demographic characteristics and outcomes were analysed, focusing on infections. (3) Results: Forty out of 65 patients (61.54%) suffered HAIs (surgical site infection: 52.18%, nosocomial pneumonia: 23.20%, bloodstream infection: 13% and urinary tract infection: 5.80%). Methicillin-resistant Staphylococcus aureus (MRSA) and resistant Pseudomonas aeruginosa, Klebsiella pneumoniae and Enterobacter cloacae were the most frequently implicated. The significant risk factors for infection were: previous radiotherapy (Odds ratio (OR): 5.42; 95% confidence interval (CI), 1.39-21.10), anaemia (OR: 8.00; 95% CI, 0.96-66.95), salvage surgery (eight out of eight patients), tracheostomy (OR: 2.86; 95% CI, 1.01-8.14), surgery duration (OR: 1.01; 95% CI, 1.00-1.02), microvascular reoperation <72 h (eight/eight) and flap loss (eight/eight). The major surgical complications were: a need to reoperate (OR: 6.89; 95% CI, 1.42-33.51), prolonged hospital admission (OR: 1.16; 95% CI, 1.06-1.27) and delay in the initiation of postoperative radiotherapy (OR: 9.07; 95% CI, 1.72-47.67). The sixth month mortality rate in patients with HAIs was 7.69% vs. 0% in patients without HAIs (p = 0.50). (4) Conclusions: HAIs were common after this type of surgery, many of them caused by resistant microorganisms. Some modifiable risk factors were identified. Infections played a role in cancer prognosis by delaying adjuvant therapy.

Caracterización en una cohorte de pacientes cubanos con esclerosis sistémica / Characterization of a Systemic Sclerosis Cohort of Cuban Patients

Introducción: La esclerosis sistémica es una enfermedad autoinmune, de causa desconocida, que produce fibrosis en la piel y en algunos órganos internos junto a alteraciones vasculares. Objetivo: Caracterizar una cohorte de pacientes con esclerosis sistémica. Métodos: Se realizó un estudio observacional descriptivo transversal, en pacientes con esta entidad, atendidos en el Servicio de Reumatología del Hospital Hermanos Ameijeiras en el período comprendido de 2005 hasta el 2016, se incluyeron en el estudio 179 pacientes. Resultados: En los grupos estudiados el 42,45 por ciento tenía entre 45 y 59 años, el 91,62 por ciento eran mujeres y el 68,16 por ciento blancos. El 59,22 por ciento tenían una esclerosis sistémica difusa. El 52,51 por ciento tenían neumopatía intersticial como afectación visceral. El ANA y el antiScL-70, fueron positivos en el 54,19 por ciento y 25,70 por ciento respectivamente. El 25,70 por ciento de los casos falleció por complicaciones propias de la enfermedad. Conclusiones: La caracterización de la cohorte de pacientes con esclerosis sistémica arrojó que la edad de inicio de la enfermedad y el sexo fue igual que el reportado en la literatura, no así para el color de la piel. Predominó la esclerosis sistémica difusa. La afectación visceral más frecuente fue la piel seguida del daño pulmonar(AU)

Introduction: Systemic sclerosis is an autoimmune disease of unknown cause that produces fibrosis in the skin and some internal organs together with vascular alterations. Objective: To characterize a systemic sclerosis cohort of patients. Methods: A cross-sectional descriptive observational study was carried out in patients with this entity, treated at the Rheumatology Service of Hermanos Ameijeiras Hospital from 2005 to 2016. One hundred seventy nine (179) patients formed the sample. Results: In the groups studied, 42.45percent were between 45 and 59 years old, 91.62percent were women and 68.16percent were white. 59.22percent had diffuse systemic sclerosis. 52.51percent had interstitial lung disease as visceral involvement. ANA and antiScL-70 were positive in 54.19percent and 25.70percent respectively. 25.70percent of the cases died due to complications of the disease. Conclusions: The characterization of the cohort of patients with systemic sclerosis showed that the onset age of the disease and the sex were the same as those reported in the literature, but not for skin color. Diffuse systemic sclerosis predominated. The most frequent visceral involvement was the skin, followed by lung damage(AU)

Factores de riesgo cardiovascular en pacientes con artritis psoriásica / Cardiovascular Risk Factors in Patients with Psoriatic Arthritis

Introducción: La presencia de factores de riesgo cardiovascular agrava el pronóstico de los pacientes con artritis psoriásica. Objetivo: Determinar los factores de riesgo cardiovascular en pacientes con diagnóstico de artritis psoriásica. Métodos: Se realizó un estudio descriptivo de corte transversal, en el Servicio de Reumatología del Hospital Hermanos Ameijeiras, en el período de enero de 2021 a abril de 2022. Se recogieron variables demográficas, clínicas y hemoquímicas relacionadas con la enfermedad, así como el score de riesgo de Framingham y la evaluación de aterosclerosis carotídea subclínica. Resultados: Se incluyeron 89 pacientes con edad media de 56,3 ± 11,8 años, 69,7 por ciento del sexo femenino 48,3 por ciento con evolución mayor de 10 años y 77,5 por ciento con afectación periférica. La actividad de la enfermedad por el índice ASDAS fue alta (55,1 por ciento) igual que por BASDAI (68,5 por ciento). Los factores de riesgo cardiovascular más frecuentes fueron: dislipidemia (61,8 por ciento), obesidad (59,6 por ciento) hipertensión arterial (50,6 por ciento). La obesidad, la diabetes y la hipertensión arterial fueron mayores en pacientes con elevada actividad de la enfermedad. La aterosclerosis carotídea fue mayor en pacientes con tabaquismo, diabetes y dislipidemia. El 39,3 por ciento presentó grosor íntima media aumentado, y el 27,0 por ciento con presencia de placa carotídea. En pacientes con aterosclerosis carotídea el 25,7 por ciento fue considerado como riesgo bajo por Framingham. Conclusiones: Los factores de riesgo cardiovascular se presentaron con una frecuencia elevada, asociado a la aterosclerosis subclínica, a la actividad inflamatoria y a una subestimación de riesgo por la escala de Framingham(AU)

Introduction: The presence of cardiovascular risk factors aggravates the prognosis of patients with psoriatic arthritis. Objective: To determine cardiovascular risk factors in patients diagnosed with psoriatic arthritis. Methods: A descriptive cross-sectional study was carried out at the Rheumatology Service of Hermanos Ameijeiras Hospital from January 2021 to April 2022. Demographic, clinical, and hemochemical variables related to the disease were collected, as well as Framingham risk score and he evaluation of subclinical carotid atherosclerosis. Results: Eighty nine patients with mean age of 56.3 ± 11.8 years were included, 69.7percent female, 48.3percent with evolution older than 10 years and 77.5percent with peripheral involvement. Disease activity by ASDAS index was high (55.1percent), the same as by BASDAI (68.5percent). The most frequent cardiovascular risk factors were dyslipidemia (61.8percent), obesity (59.6percent) and arterial hypertension (50.6percent). Obesity, diabetes and arterial hypertension were higher in patients with high disease activity. Carotid atherosclerosis was higher in patients with smoking habits, diabetes, and dyslipidemia. 39.3percent showed increased intima media thickness and 27.0% had carotid plaque. In patients with carotid atherosclerosis, 25.7percent were considered low risk by Framingham. Conclusions: Cardiovascular risk factors occurred with high frequency, associated with subclinical atherosclerosis, inflammatory activity and underestimation of risk by the Framingham scale(AU)

Composición corporal en una serie de pacientes con esclerosis sistémica / Body Composition in a Series of Systemic Sclerosis Patients

Introducción: La esclerosis sistémica es una enfermedad de etiología desconocida crónica, caracterizada por fibrosis tisular, disfunción vascular y autoinmunidad. Entre otros síntomas, las alteraciones gastrointestinales son responsables del riesgo de malnutrición. De manera que la evaluación de la composición corporal es fundamental. Objetivo: Caracterizar la composición corporal de los pacientes con esclerosis sistémica. Métodos: Se realizó un estudio descriptivo y transversal de pacientes que se atienden en la consulta protocolizada del Servicio de Reumatología del Hospital Hermanos Ameijeiras. La muestra quedó conformada por 90 pacientes con diagnóstico confirmado de esclerosis sistémica, atendidos entre los años de 2019 y 2021. Resultados: Dentro de las manifestaciones clínicas, predominó en estos pacientes la forma difusa. Se observó que a mayor tiempo de la evolución se presentaban más alteraciones gastrointestinales y úlceras digitales. Más de la mitad de los pacientes tenían sobrepeso u obesidad y se encontraron valores altos y muy altos de grasa corporal. A mayor índice de masa corporal, mayores valores de grasa corporal, grasa visceral y menor masa magra. Los pacientes con bajo índice de masa corporal tenían una tendencia significativa a presentar alteraciones gastrointestinales y no se evidenciaron diferencias en los porcentajes de grasa visceral ni de grasa corporal sin embargo, el porciento de la masa magra fue significativamente inferior. Conclusiones: Se caracterizó la identificación temprana y detallada de esta enfermedad para disminuir la mortalidad y mejorar el pronóstico de estos pacientes, mediante el análisis de la composición corporal(AU)

Introduction: Systemic sclerosis is a chronic disease of unknown etiology, characterized by tissue fibrosis, vascular dysfunction and autoimmunity. Among other symptoms, gastrointestinal disturbances are responsible for the risk of malnutrition. In this sense, the evaluation of body composition is fundamental. Objective: To characterize the body composition of patients with systemic sclerosis. Methods: A descriptive and cross-sectional study was carried out in patients assisted in the protocolized consultation of the Rheumatology Service of Hospital Hermanos Ameijeiras. The sample was made up of 90 patients with confirmed diagnosis of systemic sclerosis who were treated from 2019 to 2021. Results: Diffuse form predominated in these patients as the clinical manifestations. It was observed that the longer the evolution, the more gastrointestinal alterations and digital ulcers were presented. More than half of the patients were overweight or obese, as high and very high values of body fat were found. The higher the body mass index, the higher the values of body fat, visceral fat and lower lean mass. Patients with low body mass index had significant tendency to present gastrointestinal alterations and no differences were found in the percentages of visceral fat or body fat. However, the percentage of lean mass was significantly lower. Conclusions: The early and detailed identification of this disease was characterized to reduce mortality and improve the prognosis of these patients through the analysis of body composition(AU)

Caracterización de la enfermedad pulmonar intersticial en los pacientes con esclerosis sistémica / Description of Interstitial Lung Disease in Systemic Sclerosis Patients

Introducción: La esclerosis sistémica es una enfermedad autoinmune crónica, caracterizada por vasculopatía, activación del sistema inmune y aumento de depósitos de matriz extracelular. En los últimos años, el compromiso pulmonar ha cobrado gran importancia, ha pasado a ser la primera causa de muerte en estos pacientes. La afección pulmonar puede ocurrir como hipertensión o enfermedad pulmonar intersticial. La meta del tratamiento es detener el deterioro de la función pulmonar. Objetivo: Caracterizar las manifestaciones clínicas, imagenológicas y la función respiratoria en pacientes con esclerosis sistémica y enfermedad pulmonar intersticial. Métodos: Se realizó un estudio observacional, descriptivo de corte transversal en el período comprendido entre diciembre de 2018 y diciembre de 2019. En el Servicio de Reumatología para caracterizar la enfermedad pulmonar intersticial en pacientes con esclerosis sistémica. El universo estuvo constituido por 168 pacientes, diagnosticados con esa enfermedad y la muestra se conformó por 55 pacientes que cumplieron los criterios de inclusión establecidos. Resultados: La enfermedad pulmonar intersticial fue más frecuente en pacientes mayores de 40 años, del sexo femenino, piel mestiza, predominó la forma clínica difusa, el síntoma más frecuente fue la disnea de esfuerzo, la mayoría tuvo ANA positivo y el patrón tomográfico en panal de abejas. La capacidad vital forzada estaba disminuida con mayor frecuencia, se asoció a un comportamiento autoinmune positivo para anti-ScL-70. Conclusiones: Se caracterizó las manifestaciones clínicas y radiográficas de la enfermedad pulmonar intersticial fueron comprobadas por la utilidad de la tomografía computarizada y la espirometría para identificar la presencia de fibrosis pulmonar(AU)

Introduction: Systemic sclerosis is a chronic autoimmune disease, characterized by vasculopathy, activation of the immune system and increased extracellular matrix deposits. In recent years, lung involvement has gained great importance, it has become the first cause of death in these patients. Lung involvement can occur as hypertension or interstitial lung disease. The goal of treatment is to stop the decline in lung function. Objective: To characterize the clinical and imaging manifestations and respiratory function in patients with systemic sclerosis and interstitial lung disease. Methods: An observational, descriptive, cross-sectional study was carried out from December 2018 to December 2019 in the rheumatology service to characterize interstitial lung disease in patients with systemic sclerosis. The universe consisted of 168 patients diagnosed with this disease and the sample was made up of 55 patients who met the established inclusion criteria. Results: Interstitial lung disease was more frequent in patients older than 40 years, female, mixed-race skin color, the diffuse clinical form predominated, the most frequent symptom was exertional dyspnea, the majority had positive ANA and the pattern honeycomb tomography. Forced vital capacity was more frequently decreased, associated with positive autoimmune behavior for Anti-ScL-70. Conclusions: The radiographic and clinical manifestations of PID were verified by the usefulness of computed tomography and spirometry to identify the presence of pulmonary fibrosis(AU)

Obtener un mejor tratamiento para el paciente con hipotiroidismo / Establishing Better Treatment for Hypothyroidism Patients

Introducción: El hipotiroidismo es una entidad de visita frecuente al endocrinólogo, establecer el mejor tratamiento es un desafío, a pesar del manejo adecuado y de un control hormonal óptimo, en ocasiones los pacientes continúan con los síntomas que afectan su calidad de vida, por lo que el tratamiento debe ser individualizado, basado en la mejor evidencia. Objetivo: Establecer las mejores opciones terapéuticas en las diferentes formas de presentación del hipotiroidismo. Métodos: Se realizó una búsqueda bibliográfica no sistemática en las bases de datos de PubMed, Medline, LILACS, EMBASE, Redalyc y guías internacionales. Los criterios de inclusión fueron publicaciones en inglés y español, en las que el título, palabras clave o resumen incluyen información pertinente al objetivo de estudio, periodicidad no mayor a los 5 años a excepción de las guías que son las últimas revisiones. En la búsqueda se obtuvieron 30 artículos de los cuales fueron 14 seleccionados. Conclusiones: Se establecieron las opciones terapéuticas con el fin de obtener un mejor tratamiento para el paciente hipotiroideo que debe ser individualizado y basado en la mejor evidencia, para alcanzar un control adecuado de su enfermedad, mejorar la calidad de vida y evitar complicaciones relacionadas con esta patología(AU)

Introduction: Hypothyroidism is an entity that is frequently seen at the endocrinologist´s. establishing the best treatment is a challenge, despite proper management and optimal hormonal control, sometimes patients continue with symptoms which affect their quality of life, therefore that treatment should be individualized, based on the best evidence. Objective: To establish the best therapeutic options in hypothyroidism different forms of presentation. Methods: A non-systematic bibliographic search was carried out in PubMed, Medline, LILACS, EMBASE, Redalyc databases and in the international guidelines. The inclusion criteria were publications in English and Spanish, in which the title, keywords or abstract include relevant information to the objective of the study, with a periodicity of no more than 5 years, except for the guidelines that the latest revisions were used. In the search, 30 articles were retrieved, 14 which were selected. Conclusions: The therapeutic options were established in order to find better treatment for hypothyroid patients, which must be individualized and based on the best evidence, to achieve adequate control of the disease, to improve the quality of life and to avoid related complications(AU)

Comorbilidades en pacientes con artropatía psoriásica / omorbidities in patients with psoriatic arthropathy

Introducción: En los últimos años, diversas investigaciones han demostrado que la presencia de comorbilidad agrava el pronóstico de los pacientes con artritis psoriásica. Objetivo: Determinar las comorbilidades en pacientes con diagnóstico de artritis psoriásica. Métodos: Se realizó un estudio descriptivo de corte transversal, en el servicio de Reumatología del Hospital Clínico Quirúrgico Hermanos Ameijeiras, que incluyó 111 pacientes con artritis psoriásica durante el periodo comprendido entre enero de 2016 a febrero de 2018. Resultados: El grupo de edad más representado fue el de 50 a 60 años, predominó el sexo femenino (63,1 por ciento) y el color de piel blanca (77,5 por ciento). El 50,5 por ciento presentó un tiempo de evolución de la artritis psoriásica mayor de 10 años. El 94,5 por ciento expresó el patrón articular periférico y la forma clínica articular más frecuente fue la oligoarticular (56,7 por ciento). Las comorbilidades identificadas con mayor frecuencia fueron la obesidad (80,18 por ciento), dislipidemia (68,46 por ciento), hipertensión arterial (HTA) (54,95 por ciento) y el hígado graso no alcohólico (53,15 por ciento). El 73,0 por ciento presentó tres o más comorbilidades. El sexo y el tiempo de evolución de la enfermedad y la actividad inflamatoria no mostraron una relación significativa. La actividad inflamatoria de la enfermedad se demostró en el 83,8 por ciento. Conclusiones: Se evidenció la presencia de comorbilidades asociadas en pacientes con APs, presentándose con mayor frecuencia, la obesidad, dislipidemia, HTA e hígado graso no alcohólico. Un porcentaje elevado de pacientes presentaron más de tres comorbilidades y no se demostró asociación significativa entre el número de comorbilidades y variables de la enfermedad(AU)

Introduction: In recent years, various investigations have shown that the presence of comorbidity worsens the prognosis of patients with psoriatic arthritis. Objective: To determine the comorbidities in patients diagnosed with psoriatic arthritis. Methods: A descriptive cross-sectional study was conducted in the Rheumatology service of the Hermanos Ameijeiras Clinical-Surgical Hospital, which included 111 patients with psoriatic arthritis from January 2016 to February 2018. Results: The age group most represented was that of 50 to 60 years, female sex (63.1`percent) and white skin color (77.5percent) patients predominated. 50.5percent had a psoriatic arthritis evolution time greater than 10 years. 94.5percent expressed the peripheral joint pattern and the most frequent joint clinical form was oligoarticular (56.7percent). The most frequently identified comorbidities were obesity (80.18percent), dyslipidemia (68.46percent), arterial hypertension (HBP) (54.95percent) and non-alcoholic fatty liver (53.15percent). 73.0percent had three or more comorbidities. The sex and the time of evolution of the disease and the inflammatory activity did not show significant relationship. The inflammatory activity of the disease was demonstrated in 83.8percent. Conclusions: The presence of associated comorbidities was evidenced in patients with PsA, with obesity, dyslipidemia, hypertension, and non-alcoholic fatty liver occurring more frequently. A high percentage of patients had more than three comorbidities and no significant association was demonstrated between the number of comorbidities and disease variables(AU)

Características clínicas, inmunológicas y daño de órganos en pacientes con miopatías inflamatorias idiopáticas / Organ damage clinical and immunological characteristics in patients with idiopathic inflammatory myopathies

Introducción: Las miopatías inflamatorias idiopáticas constituyen un grupo de enfermedades musculares caracterizadas por debilidad muscular crónica e inflamación muscular de etiología desconocida. Objetivo: Identificar las características clínicas e inmunológicas y su relación con el daño de órganos en los pacientes con miopatías inflamatorias idiopáticas. Métodos: Se realizó estudio observacional, descriptivo, transversal, en 52 pacientes con diagnóstico de miopatía inflamatoria idiopática, seguidos en la consulta protocolizada de Reumatología del Hospital Clínico Quirúrgico Hermanos Ameijeiras entre enero 2016 y enero 2017. Para las variables cualitativas se calcularon los porcentajes de cada grupo. Se utilizó Chi-cuadrado de Pearson (estadístico exacto de Fisher). Nivel de significación del 95 por ciento (α = 0,05) para relacionar la presencia de anticuerpos y el tipo de miopatía así como la presencia de manifestaciones clínicas de MII. Resultados: El 80,8 por ciento fueron mujeres y 86,5 por ciento de procedencia urbana. La edad media al comienzo fue 42,8 ± 13,2 años, tiempo de demora al diagnóstico de 8,8 ± 7,0 meses, tiempo medio de evolución de la enfermedad de 7,5 ± 7,1 años. El 80,8 por ciento estaba en remisión, 50 por ciento tenía anticuerpos específicos. La hipertensión arterial se encontró en 28,8 por ciento de los pacientes y 23,1 por ciento presentó neumonía intersticial. La artritis estuvo presente en 96,2 por ciento. El 26,9 por ciento presentaron anticuerpos específicos Jo-1 y 21,2 por ciento Ro 52. Conclusiones: Predominaron los pacientes del sexo femenino en la cuarta década de la vida de procedencia urbana, los anticuerpos específicos encontrados más frecuentes fue el anti Jo-1, asociado a la presencia de neumopatía intersticial(AU)

Introduction: Idiopathic inflammatory myopathies constitute a group of muscle diseases characterized by chronic muscle weakness and muscle inflammation of unknown etiology. Objective: To identify the clinical and immunological characteristics and their relationship with organ damage in patients with idiopathic inflammatory myopathies. Methods: An observational, descriptive, cross-sectional study was carried out in 52 patients with diagnosis of idiopathic inflammatory myopathy, followed in the protocolized consultation of Rheumatology at Hermanos Ameijeiras Clinical and Surgical Hospital from January 2016 to January 2017. For the qualitative variables, the percentages of each group were calculated. Pearson's Chi-square (Fisher's exact statistic) was used. 95percent significance level (α = 0.05) was used to relate the presence of antibodies and the type of myopathy as well as the presence of clinical manifestations of MII. Results: 80.8percent were women and 86.5percent of urban origin. The mean age at the beginning was 42.8 ± 13.2 years, time delay to diagnosis was 8.8 ± 7.0 months, mean time of evolution of the disease of 7.5 ± 7.1 years. 80.8percent were in remission, 50percent had specific antibodies. Hypertension was found in 28.8percent of the patients and 23.1percent had interstitial pneumonia. Arthritis was present in 96.2percent. 26.9percent had specific Jo1 antibodies and 21.2percent had Ro 52. Conclusions: Urban female patients in the fourth decade of life predominated, the most frequent specific antibodies found was anti-Jo-1, associated with the presence of interstitial lung disease(AU)

Características clínicas, inmunológicas y daño de órganos en pacientes con miopatías inflamatorias idiopáticas / Clinical and immunological characteristics and organ damage in patients with idiopathic inflammatory myopathies

RESUMEN Introducción: Las miopatías inflamatorias idiopáticas constituyen un grupo de enfermedades musculares caracterizadas por debilidad muscular crónica e inflamación muscular de etiología desconocida. Objetivo: Identificar las características clínicas e inmunológicas y daño de órganos en pacientes con miopatías inflamatorias idiopáticas. Método: Se realizó estudio observacional, descriptivo, transversal en 52 pacientes con diagnóstico de miopatía inflamatoria idiopática, seguidos en la consulta protocolizada de Reumatología del Hospital Clínico Quirúrgico "Hermanos Ameijeiras" entre enero 2016 y enero 2017. Para las variables cualitativas se calcularon los porcentajes de cada grupo. Se utilizó Chi-cuadrado de Pearson (Estadístico exacto de Fisher), nivel de significación del 95 % (α=0,05) para relacionar la presencia de anticuerpos y el tipo de miopatía, así como la presencia de manifestaciones clínicas de miopatías inflamatorias idiopáticas. Resultados: Del total de pacientes estudiadas, 80,8 % fueron mujeres, 61,5 % de color de piel negra, 86,5 % de procedencia urbana. La edad media al comienzo fue 42,8 ± 13,2 años, tiempo de demora al diagnóstico de 8,8 ± 7,0 meses, tiempo medio de evolución de la enfermedad de 7,5 ± 7,1 años, 80,8 % estaban en remisión, 50 % tenía anticuerpos específicos. La hipertensión arterial se encontró en 28,8 % de los pacientes y 23,1 % presentó neumonía intersticial. La artritis estuvo presente en 96,2 %, 26,9 % presentaron anticuerpos específicos Jo1 y 21,2 % Ro 52. Conclusiones: Predominaron los pacientes del sexo femenino, en la cuarta década de la vida, de procedencia urbana. Los anticuerpos específicos encontrado con más frecuencia fue el anti Jo-1, que se asoció a la presencia de neumopatía intersticial.

ABSTRACT Introduction: Idiopathic inflammatory myopathies constitute a group of muscle diseases characterized by chronic muscle weakness and muscle inflammation of unknown etiology. Objective: To identify the clinical and immunological characteristics and organ damage in patients with idiopathic inflammatory myopathies. Method: An observational, descriptive, cross-sectional study was carried out in 52 patients with diagnosis of idiopathic inflammatory myopathy, followed up in the protocolized service of Rheumatology at Hermanos Ameijeiras Clinical Surgical Hospital from January 2016 to January 2017. The qualitative variables were calculated with the percentages in each group. Pearson's Chi-square (Fisher's exact statistic) (95% significance level (α = 0.05) was used to relate the presence of antibodies and the type of myopathy as well as the presence of clinical manifestations of idiopathic inflammatory myopathies. Results: 80.8% were women of the total patients studied, 61.5% non-white skin color, 86.5% of urban origin. The mean age at the beginning was 42.8 ± 13.2 years, time delay to diagnosis was 8.8 ± 7.0 months, mean time of evolution of the disease of 7.5 ± 7.1 years. 80.8% were in remission, 50% had specific antibodies. Hypertension was found in 28.8% of the patients and 23.1% had interstitial pneumonia. Arthritis was present in 96.2%. We found 26.9% had specific Jo1 antibodies and 21.2% Ro 52. Conclusions: Urban origin female patients predominated, in their fourth decade of life, the more frequent specific antibodies found was anti Jo-1, which was associated with the presence of interstitial lung disease.

A new paradigm in respiratory hygiene: increasing the cohesivity of airway secretions to improve cough interaction and reduce aerosol dispersion.

BACKGROUND: Infectious respiratory diseases are transmitted to non-infected subjects when an infected person expels pathogenic microorganisms to the surrounding environment when coughing or sneezing. When the airway mucus layer interacts with high-speed airflow, droplets are expelled as aerosol; their concentration and size distribution may each play an important role in disease transmission. Our goal is to reduce the aerosolizability of respiratory secretions while interfering only minimally with normal mucus clearance using agents capable of increasing crosslinking in the mucin glycoprotein network. METHODS: We exposed mucus simulants (MS) to airflow in a simulated cough machine (SCM). The MS ranged from non-viscous, non-elastic substances (water) to MS of varying degrees of viscosity and elasticity. Mucociliary clearance of the MS was assessed on the frog palate, elasticity in the Filancemeter and the aerosol pattern in a "bulls-eye" target. The sample loaded was weighed before and after each cough maneuver. We tested two mucomodulators: sodium tetraborate (XL"B") and calcium chloride (XL "C"). RESULTS: Mucociliary transport was close to normal speed in viscoelastic samples compared to non-elastic, non-viscous or viscous-only samples. Spinnability ranged from 2.5 +/- 0.6 to 50.9 +/- 6.9 cm, and the amount of MS expelled from the SCM increased from 47 % to 96 % adding 1.5 microL to 150 microL of XL "B". Concurrently, particles were inversely reduced to almost disappear from the aerosolization pattern. CONCLUSION: The aerosolizability of MS was modified by increasing its cohesivity, thereby reducing the number of particles expelled from the SCM while interfering minimally with its clearance on the frog palate. An unexpected finding is that MS crosslinking increased "expectoration".

Hiperparatiroidismo primario por adenoma con gammagrafía negativa / Primary hyperparathyroidism due to negative scintigraphy adenoma

Introducción: El hiperparatiroidismo es una de las causas más frecuente de hipercalcemia. El 85 por ciento de los pacientes son mayores de treinta años, su frecuencia más alta se encuentra por encima de 60 años, a largo plazo puede producir una serie de complicaciones. Objetivo: Describir el caso clínico de hiperparatiroidismo primario que resultó negativa al estudio de gammagrafía con localización ectópica del adenoma. Caso Clínico: Paciente femenina de 20 años con antecedentes de litiasis renal y uretral de 7 años de evolución, la cual se sometió a 13 sesiones de litotricia y dos cirugías renales con recidivas, hace 6 meses se detectó cifras elevadas de calcio. Se realiza protocolo de estudio de hipercalcemia determinándose hiperparatiroidismo primario, ultrasonido y tomografía de cuello reportan lesión que podría corresponder a un adenoma paratiroideo paratraqueal; sin embargo, la gammagrafía con metoxi-isobutilisonitrilo y tecnecio 99 fue negativa. Se realizó cirugía de cuello convencional ya que la cirugía radioguiada no fue útil para la localización del adenoma paratiroideo intraoperatorio. Estudio histopatológico reportó adenoma paratiroideo. Conclusiones: En adenomas paratiroideo con gammagrafía negativa se puede hacer el diagnostico con estudios de imagen convencional. La localización ectópica del adenoma paratiroideo constituye una causa de gammagrafía negativa. La cirugía radioguiada no es útil en los adenomas paratiroides ectópicos con gammagrafía negativa(AU)

Introduction: Hyperparathyroidism is one of the most frequent causes of hypercalcemia. 85 percent of patients are older than thirty years, its highest frequency is over 60 years, in the long term it can produce a series of complications. Objective: To describe a clinical case of primary hyperparathyroidism that was negative in the scintigraphy study with ectopic location of the adenoma. Clinical case report: A 20-year-old female patient with a 7-year history of renal and urethral lithiasis, who had undergone 13 lithotripsy sessions and two recurrent kidney surgeries. Six months ago, elevated calcium levels were detected. A study protocol for hypercalcemia was carried out, determining primary hyperparathyroidism. Ultrasound and neck tomography report a lesion that could correspond to a paratracheal parathyroid adenoma; however, methoxy-isobutylisonitrile and technetium-99 scintigraphy was negative. Conventional neck surgery was performed since radioguided surgery was not useful for locating the intraoperative parathyroid adenoma. Histopathological study reported parathyroid adenoma. Conclusions: In parathyroid adenomas with negative scintigraphy, the diagnosis can be reached with conventional imaging studies. The ectopic location of the parathyroid adenoma is a cause of negative scintigraphy. Radioguided surgery is not helpful in scan-negative ectopic parathyroid adenomas(AU)

Mitochondria-targeted antioxidant preserves contractile properties and mitochondrial function of skeletal muscle in aged rats.

Mitochondrial dysfunction plays a central role in the pathogenesis of sarcopenia associated with a loss of mass and activity of skeletal muscle. In addition to energy deprivation, increased mitochondrial ROS damage proteins and lipids in aged skeletal muscle. Therefore, prevention of mitochondrial ROS is important for potential therapeutic strategies to delay sarcopenia. This study elucidates the pharmacological efficiency of the new developed mitochondria-targeted ROS and electron scavenger, XJB-5-131 (XJB) to restore muscle contractility and mitochondrial function in aged skeletal muscle. Male adult (5-month old) and aged (29-month old) Fischer Brown Norway (F344/BN) rats were treated with XJB for four weeks and contractile properties of single skeletal muscle fibres and activity of mitochondrial ETC complexes were determined at the end of the treatment period. XJB-treated old rats showed higher muscle contractility associated with prevention of protein oxidation in both muscle homogenate and mitochondria compared with untreated counterparts. XJB-treated animals demonstrated a high activity of the respiratory complexes I, III, and IV with no changes in citrate synthase activity. These data demonstrate that mitochondrial ROS play a causal role in muscle weakness, and that a ROS scavenger specifically targeted to mitochondria can reverse age-related alterations of mitochondrial function and improve contractile properties in skeletal muscle.

Síndrome de Cowden / Cowden syndrome

El síndrome de Cowden es una enfermedad hereditaria, de transmisión autosómica dominante, caracterizada por la presencia de múltiples hamartomas y nódulos en la piel y la mucosa oral, junto con anomalías en mamas, tiroides y pólipos en el tracto gastrointestinal, con un riesgo incrementado de tumores malignos. Se reporta un caso de una paciente con diagnóstico de bocio tóxico nodular, y que presentaba -por los antecedentes y estudios realizados- manifestaciones compatibles con el síndrome de Cowden. El síndrome de Cowden es el síndrome hamartomatoso tumoral del phosphatase and tensinhomolog mejor descrito hasta el momento. Los pacientes con él tienen lesiones mucocutáneas características y un elevado riesgo de cáncer de mama, tiroides, endometrio, colorrectal y renal, así como varias manifestaciones benignas como macrocefalia y gangliocitomadisplásico del cerebelo. Es importante el diagnóstico precoz de este síndrome y el seguimiento a largo plazo, dado el alto riesgo de desarrollar tumores malignos(AU)

Cowden syndrome is a hereditary disease, of autosomal dominant transmission, and characterized by the presence of multiple hamartomas and nodules in the skin and oral mucosa, and also with abnormalities in the breast, thyroid, and polyps in the gastrointestinal tract with an increased risk of malignant tumors. It is reported a case of a patient with a diagnosis of toxic nodular goiter, and who presented -due to the antecedents and studies carried out- manifestations compatible with the Cowden syndrome. Cowden syndrome is the hamartomatous tumor syndrome of phosphatase and tensin homolog which is better described so far. Patients having it present characteristic mucocutaneous lesions and a high risk of breast, thyroid, endometrial, colorectal and renal cancers, as well as several benign manifestations such as macrocephaly and gangliocytoma of the cerebellum. Early diagnosis of this syndrome and long-term follow-up are important given the high risk of developing malignant tumors(AU)

Aging of human muscle: understanding sarcopenia at the single muscle cell level.

The loss of muscle mass with age, also known as sarcopenia, is a major scientific and public health problem. Muscle atrophy is associated with the loss of functional capacity and poor health outcomes in elderly men and women. A detailed understanding of this problem in humans can be enhanced by the use of experiments with single muscle fibers. It is likely that both muscle atrophy and a decrease in muscle-fiber quality contribute to muscle dysfunction among the elderly. A better understanding of sarcopenia at the single-fiber level may lead to the design of more effective rehabilitative interventions.

Blockade of P2 nucleotide receptors after spinal cord injury reduced the gliotic response and spared tissue.

Spinal cord injury (SCI) triggers a sequel of events commonly associated with cell death and dysfunction of glias and neurons surrounding the lesion. Although astrogliosis and glial scar formation have been involved in both damage and repair processes after SCI, their role remains controversial. Our goal was to investigate the effects of the P2 receptors antagonists, PPADS and suramin, in the establishment of the reactive gliosis and the formation of the glial scar. Molecular biology, immunohistochemistry, spared tissue, and locomotor behavioral studies were used to evaluate astrogliosis, in adult female Sprague-Dawley rats treated with P2 antagonists after moderate injury with the NYU impactor device. Semi-quantitative RT-PCR confirmed the presence of P2Y(1,) P2Y(2,) P2Y(4,) P2Y(6,) P2Y(12), and P2X(2) receptors in the adult spinal cord. Immunohistochemistry studies confirmed a significant decrease in GFAP-labeled cells at the injury epicenter as well as a decrease in spared tissue after treatment with the antagonists. Functional open field testing revealed no significant locomotor score differences between treated and control animals. Our work is consistent with studies suggesting that astrogliosis is an important event after SCI that limits tissue damage and lesion spreading.

Identificación de predictores serológicos de recaída en pacientes con lupus eritematoso sistémico: estudio prospectivo de 12 meses / Identification of relapse aerological predictors in patients with systemic lupus erythematous: a prospective study of 12 months

Introducción: las investigativos efectuadas no han tenido éxito en la búsqueda de biomarcadores serológicos o clínicos suficientemente confiables para predecir las recaídas en el lupus eritematoso sistémico (LES). Objetivo: definir el valor predictivo de las especificidades de anticuerpos antinucleares para la recaída del LES y de la nefritis lúpica. Métodos: estudio analítico, observacional, longitudinal y prospectivo en 120 pacientes adultos con LES inactivo (SLEDAI-2K ≤ 5 puntos). La presencia basal de siete especificidades antinucleares, C3 y C4 bajos se correlacionaron con la ocurrencia de recaída del LES (incrementos en la puntuación de SLEDAI-2K ≥ 4) y de la nefritis lúpica mediante análisis univariado. Las variables más valiosas fueron evaluadas adicionalmente como predictoras con un modelo de regresión logística multivariada para calcular los odds ratio (OR). Resultados: las recaídas del LES y de la nefritis lúpica se observaron en 51 (42,5 por ciento) y 29 (24,2 por ciento) de los pacientes, respectivamente. En el análisis multivariado emergieron como factores de riesgo para la recaída del LES la presencia de los anticuerpos anti-Nu (OR= 1523,0; p < 0,001) y los anti-DNAdc (OR= 12,1; p= 0,044) y de la nefritis lúpica, los anti-Nu (OR= 92,9; p< 0,001) y el C3 bajo (OR= 7,1; p= 0,007), La sub-representación de los anti-RNP resultó un factor de riesgo para la recaída del LES y de la nefritis lúpica (OR= 0,023; p= 0,009 y OR= 0,1; p= 0,025). Conclusiones: los pacientes con LES positivos de anticuerpos anti-Nu, anti-DNAdc o niveles bajos del C3 presentaron un riesgo mayor de recaída del LES y de la nefritis lúpica en los próximos 12 meses, lo que señala la necesidad de estrechar su monitoreo clínico(AU)

Introduction: the research carried out have not been successful in finding serological biomarkers or sufficiently reliable biomarkers for predicting relapse in systemic lupus erythematosus (SLE). Objective: setermine the predictive value of specific antinuclear antibodies for relapse of SLE and lupus nephritis. Methods: an analytical, observational, longitudinal and prospective study was carried out in 120 adult patients with inactive SLE (SLEDAI-2K ≤ 5 points). The baseline presence of seven antinuclear specificities, low C3 and C4 were correlated with the occurrence of SLE relapse (increases in score SLEDAI-2K ≥ 4) and lupus nephritis by univariate analysis. The most valuable variables were further evaluated as predictors a multivariate logistic regression model to calculate the odds ratio (OR). Results: SLE and lupus nephritis relapses were observed in 51 (42.5 percent) and 29 (24.2 percent) patients, respectively. The presence of anti-Nu (OR= 1523.0, P < 0.001) antibodies and anti-dsDNA (OR= 12.1; p = 0.044) and lupus nephritis, anti-Nu (OR= 92.9; p < 0.001) and low C3 (OR= 7.1; p= 0.007) emerged as risk factors for relapse of SLE in multivariate analysis. The underrepresentation of anti-RNP was a risk factor for relapse of SLE and lupus nephritis (OR = 0.023; p= 0.009; OR = 0.1; p= 0.025). Conclusions: SLE patients with positive anti-Nu, anti-dsDNA and low levels of C3 had a higher risk of relapse of SLE and lupus nephritis in the succeeding 12 months, signaling the need for close clinical monitoring(AU)

Trombosis de senos venosos cerebrales en la enfermedad de Behçet / Cerebral venous sinus thrombosis in Behcet's disease

La enfermedad de Behçet es una vasculitis sistémica inmunomediada que afecta los pequeños vasos sanguíneos y se presenta frecuentemente con ulceraciones de las membranas mucosas y síntomas oculares. Se presentó un paciente masculino de 25 años de edad, con antecedentes de úlceras bucales y genitales recurrentes, foliculitis, eritema nudoso, lesiones papulo-pustulosas, el cual tenía, desde 3 meses antes de su presentación en nuestro servicio, manifestaciones clínicas compatibles con un síndrome de hipertensión endocraneana benigna que no logra una evolución favorable con el tratamiento. Mediante la angio-TAC de cráneo se identificó una trombosis de senos venosos cerebrales. Los hallazgos clínicos y radiológicos son compatibles con una enfermedad de Behçet(AU)

Behcet's disease is an immune-mediated systemic vasculitis that affects small blood vessels and it frequently presents with ulceration of the mucous membranes and eye symptoms. A 25 year-old male patient is presented with history of recurrent cold sores and genital folliculitis, erythema nodosum, papular-pustular lesions. For three months before submission to our service, this patient had clinical manifestations compatible with benign intracranial hypertension syndrome that fails favorable evolution with treatment. By skull venuos CTA, venous sinus cerebral thrombosis was identified. Clinical and radiological findings are compatible with Behcet's disease(AU)