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Sample records for afectados con fibrosis

  1. Efectos del entrenamiento con electroestimulación muscular en pacientes afectados de hemofilia A

    OpenAIRE

    Gomis Bataller, Manuel

    2007-01-01

    RESUMEN La propuesta de la presente investigación fue examinar los efectos producidos por un programa de electroestimulación muscular aplicado sobre el biceps brachii en pacientes afectados de hemofilia A. Treinta sujetos tomaron parte en el presente estudio, 15 pacientes con hemofilia A clínicamente graves (nivel de factor

  2. Abordaje integral de pacientes costarricenses afectados con la enfermedad de Huntington y sus familiares

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    Melissa Vasquez-Cerdas

    2011-09-01

    Full Text Available Objetivo: Realizar el diagnóstico molecular a personas afectadas con la enfermedad de Huntington y familiares con el 50% de riesgo, y brindarles asesoramiento genético, seguimiento y evaluación psicológica y clínica, con el fin de mejorar su calidad de vida y prevenir la ocurrencia y recurrencia de la enfermedad de Huntington. Métodos: Diagnóstico molecular a pacientes con diagnóstico clínico, familiares asintomáticos con 50% de riesgo y pacientes con diagnóstico confuso. Se les brindó asesoramiento genético y evaluación psicológica. Los pacientes positivos que no tenían un control regular fueron referidos al neurólogo. Resultados: El diagnóstico molecular se realizó a 64 personas (35 mujeres y 29 hombres. De estas, seis tenían diagnóstico clínico de Huntington, el cual se confirmó, y 6 tenían diagnóstico confuso; de estas últimas, cinco resultaron negativas para la enfermedad de Huntington, y una, positiva. Las restantes 52 personas correspondían a familiares en riesgo, y de estas, 17 resultaron ser portadoras. En total, 20 mujeres y 17 hombres fueron efectivamente evaluados en el nivel psicológico. Los análisis moleculares mostraron un perfil de repeticiones similar al de otras poblaciones. Conclusión: El diagnóstico molecular es de gran ayuda, pues algunas enfermedades pueden confundirse con la de Huntington. El diagnóstico presintomático cubre satisfactoriamente las siguientes expectativas de las personas: aliviar la incertidumbre, planear el cuidado de la salud y conocer si los hijos tienen riesgo. En general, no se ha encontrado grandes diferencias entre las personas evaluadas en el nivel psicológico, ya sea que porten un diagnóstico molecular positivo o negativo.

  3. Abordaje integral de pacientes costarricenses afectados con la enfermedad de Huntington y sus familiares Integral Management of Costa Rican Patients with Huntington’s Disease and their Families

    OpenAIRE

    2011-01-01

    Objetivo: Realizar el diagnóstico molecular a personas afectadas con la enfermedad de Huntington y familiares con el 50% de riesgo, y brindarles asesoramiento genético, seguimiento y evaluación psicológica y clínica, con el fin de mejorar su calidad de vida y prevenir la ocurrencia y recurrencia de la enfermedad de Huntington. Métodos: Diagnóstico molecular a pacientes con diagnóstico clínico, familiares asintomáticos con 50% de riesgo y pacientes con diagnóstico confuso. Se les brindó asesor...

  4. Caracterización molecular de la cadena gama común y Jak3 en un individuo afectado con inmunodeficiencia severa combinada

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    Pablo Javier Patiño Grajales

    2001-04-01

    Full Text Available

    La Inmunodeficiencia Severa Combinada (IDSC es una enfermedad
    de origen genético, que se puede heredar de forma autosómica
    recesiva o ligada al cromosoma X. La IDSC se caracteriza por un
    defecto en el número y la diferenciación de los linfocitos T y NK. Los
    individuos afectados desarrollan diarrea crónica, infecciones persistentes y severas como neumonía, septicemia e infecciones fúngicas.
    Estos pacientes presentan retardo en el crecimiento y pueden morir a
    temprana edad si no se realiza una terapia de corrección genética o un
    trasplante de células hematopoyéticas. Las mutaciones responsables
    de la IDSC comprometen principalmente el gen de la cadena gama
    común (γc y la proteína Jak3 que son proteínas fundamentales en la
    transducción de señales de los receptores para varias citoquinas esenciales en la diferenciación y activación de células del sistema inmune, las cuales incluyen IL-2, IL-4, IL-7, IL-9 e IL-15 (1,2.

     

     

  5. Abordaje integral de pacientes costarricenses afectados con la enfermedad de Huntington y sus familiares Integral Management of Costa Rican Patients with Huntington’s Disease and their Families

    Directory of Open Access Journals (Sweden)

    Melissa Vasquez-Cerdas

    2011-09-01

    Full Text Available Objetivo: Realizar el diagnóstico molecular a personas afectadas con la enfermedad de Huntington y familiares con el 50% de riesgo, y brindarles asesoramiento genético, seguimiento y evaluación psicológica y clínica, con el fin de mejorar su calidad de vida y prevenir la ocurrencia y recurrencia de la enfermedad de Huntington. Métodos: Diagnóstico molecular a pacientes con diagnóstico clínico, familiares asintomáticos con 50% de riesgo y pacientes con diagnóstico confuso. Se les brindó asesoramiento genético y evaluación psicológica. Los pacientes positivos que no tenían un control regular fueron referidos al neurólogo. Resultados: El diagnóstico molecular se realizó a 64 personas (35 mujeres y 29 hombres. De estas, seis tenían diagnóstico clínico de Huntington, el cual se confirmó, y 6 tenían diagnóstico confuso; de estas últimas, cinco resultaron negativas para la enfermedad de Huntington, y una, positiva. Las restantes 52 personas correspondían a familiares en riesgo, y de estas, 17 resultaron ser portadoras. En total, 20 mujeres y 17 hombres fueron efectivamente evaluados en el nivel psicológico. Los análisis moleculares mostraron un perfil de repeticiones similar al de otras poblaciones. Conclusión: El diagnóstico molecular es de gran ayuda, pues algunas enfermedades pueden confundirse con la de Huntington. El diagnóstico presintomático cubre satisfactoriamente las siguientes expectativas de las personas: aliviar la incertidumbre, planear el cuidado de la salud y conocer si los hijos tienen riesgo. En general, no se ha encontrado grandes diferencias entre las personas evaluadas en el nivel psicológico, ya sea que porten un diagnóstico molecular positivo o negativo.Aim: To perform the molecular diagnosis to affected persons and their relatives at 50% risk of Huntington’s Disease and to give genetic counselling, psychological and clinical follow-up. This will improve the clinical management of the patients

  6. Parálisis de Bell: generalidades y manejo terapéutico en los pacientes afectados con esta condición

    OpenAIRE

    Lizano Barrantes, Catalina; Ortiz Ureña, Angie

    2013-01-01

    La parálisis de Bell es una forma de parálisis facial temporal, provocada por el daño  o trauma de uno de los dos nervios faciales. Es la causa más común de parálisis facial; afecta de 11 a 40 personas por cada 100 000 cada año, con igual proporción entre hombres y mujeres. Se desconoce la causa, pero se supone se origina por la inflamación o compresión del nervio, como respuesta a un trastorno inmune o viral. Aparece de forma repentina  y la parálisis completa se desarrolla en un máximo de 4...

  7. Aislamiento de Clostridium perfringens tipo D en un ternero lactante afectado con abomasitis ulcerativa Isolation of Clostridium perfringens type D from a suckling calve with ulcerative abomasitis

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    R. A ASSIS

    2002-01-01

    Full Text Available Se describe un brote de abomasitis ulcerativa asociada con infección por Clostridium perfringens tipo D en terneros lactantes. Seis terneros Holstein, de 2 semanas de edad, murieron después de presentar anorexia y apatía. Otros animales del mismo establecimiento habían muerto de la misma forma seis meses antes. A la necropsia el abomaso estaba muy distendido con contenido fluido y oscuro, la mucosa estaba edematosa y presentaba gran cantidad de úlceras milimétricas y habían depósitos de fibrina en la serosa. En el ciego de un animal se observaron extensas areas de infarto. En frotis de la mucosa abomasal se observaron bacilos cortos Gram positivos, no esporulados, aislándose de ella un cultivo rico de C. perfringens tipo D. Es probable que la baja inmunidad de los terneros debido a falta de calostro y estrés alimenticio, haya sido el predisponente para la infección por C. perfringens tipo DAn outbreak of ulcerative abomasitis in suckling calves associated with Clostridium perfrigens type D infection is described. Six twoweek old Holstein calves died after showing loss of appetite and lethargy. Other animals had died in similar circumstances during the previous six months. At necropsy, the abomasum was severely distended with dark fluid and the mucosa was oedematous and covered with many millimetric ulcers, while the serous surface of this organ was covered with fibrin. Several irregular black areas of infarcts were observed in the cecum of one animal. Histologically, the abomasal mucosa showed ulcers and haemorrhage, while the submucosa showed severe oedema and infiltration of neutrophils, lymphocytes and plasma cells. Short, thick, nonsporulated Gram positive rods were observed on smears of abomasal mucosa. C. perfringens type D was isolated from the abomasal mucosa. Low immunity and stress could have contributed to the pathogenesis of the lesions described

  8. Efecto de los cambios posturales en las crepitaciones de pacientes con fibrosis quística versus fibrosis pulmonar. Comunicación preliminar POSITION DEPENDENCE OF CRACKLES IN CYSTIC FIBROSIS AND PULMONARY FIBROSIS

    OpenAIRE

    2002-01-01

    La influencia de los cambios posturales en la generación de crepitaciones se ha demostrado en pacientes con Fibrosis Pulmonar (FP). En la posición decúbito lateral, las crepitaciones pueden disminuir en el pulmón superior o aumentar en el pulmón que queda en la parte inferior. El objetivo del presente trabajo fue evaluar la dependencia postural de las crepitaciones en pacientes con Fibrosis Quística (FQ) y FP, a través del análisis computacional de los ruidos respiratorios. Se estudiaron 14 p...

  9. Manejo de la rinosinusitis crónica en pacientes con fibrosis quística

    OpenAIRE

    Silva, M

    2015-01-01

    La rinosinusitis crónica es una patología que se presenta en pacientes con fibrosis quística y se asocia a la expresión de numerosos genes que determinan una alteración en la secreción de cloro del canal CFTR e hiperplasia de las glándulas submucosas del epitelio sinusal. En este artículo se efectúa una revisión acerca de las indicaciones y distintas alternativas de cirugía endoscópica nasal para la rinosinusitis crónica en pacientes con fibrosis quística. Se realizó una búsqueda de artículos...

  10. Efecto de los cambios posturales en las crepitaciones de pacientes con fibrosis quística versus fibrosis pulmonar. Comunicación preliminar POSITION DEPENDENCE OF CRACKLES IN CYSTIC FIBROSIS AND PULMONARY FIBROSIS

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    IGNACIO SANCHEZ D.

    2002-01-01

    Full Text Available La influencia de los cambios posturales en la generación de crepitaciones se ha demostrado en pacientes con Fibrosis Pulmonar (FP. En la posición decúbito lateral, las crepitaciones pueden disminuir en el pulmón superior o aumentar en el pulmón que queda en la parte inferior. El objetivo del presente trabajo fue evaluar la dependencia postural de las crepitaciones en pacientes con Fibrosis Quística (FQ y FP, a través del análisis computacional de los ruidos respiratorios. Se estudiaron 14 pacientes (7 con FQ y 7 con FP, a través de la grabación de los ruidos pulmonares, utilizando micrófonos de contacto en los lóbulos inferiores derecho e izquierdo, primero en posición sentada, a derecha e izquierda en decúbito lateral y por último nuevamente en posición sentada. En pacientes con FQ la dependencia postural se observó en 3/7 pacientes, en cambio en FP se presentó en 6/7 pacientes, bilateral en 4 y unilateral en 2. Nuestros resultados sugieren que la dependencia postural de las crepitaciones es frecuente en FP pero no así en FQ. Lo cual se podría explicar porque existen diferentes mecanismos en la generación de crepitaciones, dentro de los que se destaca aumento de la retracción elástica pulmonar en FP versus el aumento de secreciones en la vía aérea en FQPosition dependence of crackles is noticed in patients with interstitial pulmonary fibrosis (IPF. In the lateral decubitus position (LDP, crackles may diminish in the upper lung or increase in the dependent lung. We hypothesized that different crackle mechanisms would lead to position dependence in IPF but not in Cystic Fibrosis (CF. We investigated this clinical finding by computerized sound analysis. Lung sounds over corresponding sites of the left and right lower lobes were recorded in 14 patients, 7 with CF and 7 with IPF, first in the sitting position, then the left and right LDP and again in the sitting position. In CF unilateral position dependence was observed in

  11. Volúmenes pulmonares normales en pacientes con fibrosis pulmonar idiopática y enfisema Normal lung volumes in patients with idiopathic pulmonary fibrosis and emphysema

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    Juan Pablo Casas

    2008-08-01

    Full Text Available La fibrosis pulmonar idiopática (FPI es una enfermedad que se caracteriza por presentar un compromiso pulmonar de tipo restrictivo, resultante de una reducción en la complacencia pulmonar secundaria a fibrosis difusa. En el enfisema, la pérdida de elasticidad pulmonar y el colapso de las vías aéreas periféricas generan obstrucción e hiperinflación. El efecto simultáneo que ambas enfermedades producen sobre la fisiología pulmonar no es del todo claro y se han descripto volúmenes pulmonares normales o casi normales. Presentamos 4 pacientes de sexo masculino de 64, 60, 73 y 70 años, con antecedentes de tabaquismo e historia de disnea progresiva, tres de ellos con grave limitación en su calidad de vida al momento de la consulta. En la tomografía de tórax de alta resolución todos los pacientes presentaban signos de enfermedad intersticial pulmonar avanzada, con cambios de tipo fibrótico con predominio basal y subpleural, que coexistían con enfisema centroacinar con predominio en lóbulos superiores. Uno de ellos tuvo confirmación diagnóstica de ambas condicioes por biopsia pulmonar a cielo abierto. En los cuatro pacientes la espirometría y volúmenes pulmonares fueron normales, pero tenían importante compromiso del intercambio gaseoso evaluado mediante el test de caminata de 6 minutos. Tres de los pacientes tenían hipertensión pulmonar grave diagnosticado por ecocardiograma. La presencia de volúmenes pulmonares normales no excluye un diagnóstico de fibrosis pulmonar idiopática en pacientes fumadores si coexisten evidencias tomográficas de enfisema. En estos pacientes el grado de compromiso funcional, determinado por la reducción de los volúmenes pulmonares, no debería ser considerado en la evaluación de la gravedad.Pulmonary function tests in idiopathic pulmonary fibrosis characteristically show a restrictive pattern, resulting from reduction of pulmonary compliance due to diffuse fibrosis. Conversely, an obstructive

  12. Neumotórax espontáneo asociado a fibrosis pulmonar en un paciente con neurofibromatosis tipo 2

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    Alcalá Cerra, Gabriel; Moscote Salazar, Luis Rafael; Lozano Tagua, Carlos Fernando; Sabogal Barrios, Rubén

    2010-01-01

    El compromiso pulmonar en pacientes con neurofibromatosis ha sido reiteradamente descrito como una complicación muy rara en la variedad tipo 1. Se caracteriza por enfermedad pulmonar intersticial difusa, fibrosis pulmonar, neoplasias torácicas y formación de bulas, estas últimas, con alto riesgo de ruptura. Describimos un caso de neumotórax espontáneo en una paciente con neurofibromatosis tipo 2, como consecuencia de cambios fibróticos pulmonares. A nuestro conocimiento, esta asociación no ha...

  13. Enfermedad cardiovascular en pacientes cubanos afectados por Ataxia de Friedreich.

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    Tania Cruz Mariño

    2010-01-01

    Full Text Available Al describir la ataxia de Friedreich, Nicholaus hizo referencia a la patología cardiaca. Esta enfermedad autosómica recesiva se debe a una mutación dinámica en el gen FRDA, codificándose deficientemente la proteína Frataxina, conduciendo a estrés oxidativo y muerte celular cardiaca. La presente investigación se desarrolló con el objetivo de describir las anomalías cardiovasculares presentes en los pacientes cubanos afectados por ataxia de Friedreich. A los individuos con diagnóstico molecular confirmatorio de la enfermedad se les realizó electrocardiograma y ecocardiograma, así como evaluación clínica mediante escalas validadas internacionalmente: ICARS y SARA. Los trastornos de repolarización ventricular difusos, los trastornos de conducción intraauricular, así como los trastornos de la función diastólica resultaron hallazgos frecuentes. El patrón restrictivo apreciado provee evidencia invivo de que la enfermedad conduce a disfunción diastólica del ventrículo izquierdo. La ocurrencia de un Infarto Agudo del Miocardio silente indica la importancia de identificar formas incipientes de afectación miocárdica.

  14. El suero salino hipertónico no es eficaz en niños menores de seis años con fibrosis quística

    OpenAIRE

    Gimeno Díaz de Atauri, Álvaro; Rivas Juesas, Cristina

    2012-01-01

    La principal conclusión de este artículo es que el suero salino hipertónico nebulizado durante 48 semanas no reduce la tasa de exacerbaciones en comparación con suero salino fisiológico en niños menores de seis años con fibrosis quística.

  15. Cuidadores de crianças com câncer: aspectos da vida afetados pela atividade de cuidador Cuidantes de niños con cáncer: aspectos de la vida afectados por la actividad de cuidar Caregivers of children with cancer: aspects of life affectcet by the caregiver role

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    Ana Raquel Medeiros Beck

    2007-12-01

    Full Text Available Pretendeu-se, neste estudo, descrever como as atividades relacionadas ao cuidar afetam a vida de cuidadores de crianças com câncer, o grau de dependência da criança para desempenhar as atividades de vida diária (AVD, o grau de ajuda recebida de outros e o quanto alguns aspectos da vida do cuidador são afetados pela atividade de cuidar. O estudo foi descritivo, comparativo e transversal. Foram entrevistados 50 cuidadores de crianças entre três e dez anos num hospital infantil, referência no tratamento de doenças onco-hematológicas, em Campinas, SP. As atividades relacionadas ao cuidar ocasionaram sérios prejuízos na vida dos cuidadores. Portanto, avaliar estes aspectos pode auxiliar o enfermeiro a evidenciar, amenizar e planejar a assistência aos cuidadores de crianças com câncer.Se pretendió en este estudio describir de que forma las actividades relacionadas al cuidar afectan la vida de los cuidantes de niños con cáncer, el grado de dependencia del niño para desempeñar las actividades de la vida diaria (AVD, el grado de ayuda recibida de otros, y en que medida algunos aspectos da la vida del cuidante eran afectados por la actividad de cuidar. El estudio fué descriptivo, comparativo y transversal. Fueron entrevistados 50 cuidantes de niños entre 3 y 10 años en un hospital infantil modelo en el tratamiento de dolencias onco-hematológicas, en Campinas, SP. Las actividades relacionadas al cuidar ocasionaron serios daños en la vida de los cuidantes. Por lo tanto, evaluar estos aspectos puede auxiliar al enfermero a identificar, amenizar y planear la asistencia a los cuidantes de niños con cáncer.This study aimed at demonstrating that the caregiving role affected the lives of caregivers of children with cancer, the child's degree of dependence regarding the performance of daily life activities (DLA, the degree of help offered by others and the degree to which some aspects of the caregiver's life were affected by caregiving

  16. Terapia con antivirali ad azione diretta in pazienti con epatite cronica HCV e severa fibrosi o cirrosi

    OpenAIRE

    Vitale, Giovanni

    2016-01-01

    Introduzione: l’epatite cronica C è la più comune infezione virale trasmessa per via ematica e la principale causa di mortalità tra le epatopatie. La terapia antivirale può prevenire la progressione della malattia nei pazienti HCV. Telaprevir e simeprevir sono Direct Acting Antivirals e due inibitori delle proteasi, utili nell’eradicazione del virus. Scopo: stabilire l’efficacia e sicurezza di un regime di terapia antivirale con telaprevir, pegIFN/ribavirin e di uno con simeprevir-sofosbuvir+...

  17. DNA-fingerprinting di stipiti di Chryseobacterium spp isolati da pazienti con Fibrosi Cistica

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    Antonietta Lambiase

    2007-03-01

    Full Text Available Objectives: Pulmonary infections by Gram-negative bacteria, as Pseudomonas aeruginosa, Burkholderia cepacia, Stenotrophomonas maltophilia, are the major cause of morbidity in Cystic Fibrosis patients. In the past decade, several pathogens as Alcaligenes spp and no tuberculosis mycobacteria have been recovered in these patients. Bacteria of genus Chryseobacterium are widespread Gram-negative microrganisms and involved in human infections. Aims of this study were to value the isolation frequency of Chryseobacterium strains in a cohort of Cystic Fibrosis patients, to investigate their antimicrobial sensibility and to establish possible clonal likeness between strains. Methods:A retrospective study was undertaken between January 2003 and December 2005 on 300 patients receiving care at the Regional Cystic Fibrosis Centre of Naples University “Federico II”. Sputum samples were checked: for bacterial identification, selective media and commercial identification systems were used.The activity of antimicrobial agents was determined using diffusion and microdiluthion methods. For DNA-fingerprinting, a genomic DNA macrorestriction followed by pulsed-field electrophoresis was carried out. Results:A total of 26 strains from 17 patients were isolated (7 C. meningosepticum, 14 C. indologenes, 5 C. gleum. Strains were resistant to cephalosporins and carbapenems; some were sensitive to ciprofloxacin, levofloxacin and trimethoprim-sulphamethoxazole. Macrorestriction analysis showed substantial heterogeneity among strains. Conclusions: Actually, the prognostic role of Chryseobacterium in Cystic Fibrosis is unclear and although the small number of isolations, it is need to be on the look out regard such microorganisms. The considerable resistance implies difficulties on therapeutic approach. Results of DNA-fingerprinting indicate no evidence of clonal likeness and then of patient-to-patient spread.

  18. Expandiendo el espectro mutacional en pacientes chilenos con fibrosis quística Expanding the CFTR mutation spectrum in Chilean patients with cystic fibrosis

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    Marcos Vásquez D

    2012-06-01

    Full Text Available Introducción: La fibrosis quística (FQ es una enfermedad con herencia autosómica recesiva, que presenta una incidencia de 1 en 8.000 a 9.000 recién nacidos en Chile. A la fecha se han descrito más de 1.800 mutaciones diferentes en el gen CFTR. El diagnóstico molecular disponible consiste en el análisis de las 36 mutaciones presentes con mayor frecuencia en población caucásica, donde se describe una tasa de detección de un 85%. Sin embargo, en Chile el rendimiento corresponde a un 42%. Por esta razón, hemos iniciado un análisis sistemático en la región codificante del gen CFTR con elfin de identificar los restantes alelos en pacientes chilenos con FQ. Métodos: Análisis por secuenciación de los exones 6,7,14,19y 20, en 48pacientes chilenos del Programa Nacional de FQ. Se incluyeron pacientes con criterios clínicos y de laboratorio de FQ, y con sólo una mutación identificada en el panel de 36 mutaciones. Resultados: Se identificaron 3 mutaciones diferentes que no se analizan en el panel de diagnóstico molecular y que no habían sido reportadas en pacientes chilenos, totalizando 14 casos. Cuatro casos corresponden a una nueva mutación en el exón 14, que produce un corrimiento en el marco de lectura y un codón de término prematuro (c.2462_2463delGT/p.Ser821ArgfsX4. Ocho casos presentan la mutación c.3196C>T en el exón 20, mientras que en 2 casos se encontró la mutación c.3039delC en el exón 19. Ambas mutaciones han sido descritas previamente en otras poblaciones. Discusión: La identificación de estas mutaciones ha incrementado notablemente la tasa de detección obtenida en nuestros pacientes. Esto crea la necesidad de adaptar el análisis molecular inicial en pacientes chilenos con FQ, redundando en un diagnóstico de certeza en gran parte de los casos y permitiendo un adecuado asesoramiento genético para las familias.Introduction: Cystic Fibrosis (CF is an autosomal recessive disease and affects 1 in 8000

  19. Evolución de la fibrosis hepática en reclusos coinfectados por VIH y VHC que inician tratamiento con inhibidores de la proteasa potenciados

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    P. Saiz de la Hoya Zamácola

    2013-10-01

    Full Text Available Objetivos: Analizar la evolución de la fibrosis hepática medida por elastografía y pruebas bioquímicas en reclusos coinfectados por VIH y VHC que han iniciado tratamiento antirretroviral con lopinavir/ritonavir u otros inhibidores de la proteasa potenciados con ritonavir. Métodos: Estudio prospectivo, observacional y multicéntrico. Se comprobó durante 48 semanas la evolución de la fibrosis hepática medida mediante elastografía de transición (FibroScan y pruebas bioquímicas en población penitenciaria española coinfectada por VIH y VHC. Resultados: De los 94 pacientes incluidos, 54 (57,4% fueron seguidos durante 48 semanas. En la semana 48, no hubo cambios significativos en el grado de fibrosis medida mediante FibroScan (8,1 Kpa vs 8,3; p=0.20 o índice de FORNS (5,6 vs 5,1; p=0,50, aunque sí con el índice APRI (0.7 vs 0.6; p=0.05 y el índice FIB-4 (p=0,02. Cuando la medición se realizó en función del grado de fibrosis basal, se observó que el tratamiento redujo el porcentaje de pacientes con fibrosis basal de grado 3/4 (50% vs 15%; p=0,001, pero no hubo cambios en los que ya tenían basalmente grado 4 (20,4% vs 20,4%. Conclusión: Los reclusos coinfectados por VIH y VHC que inician tratamiento antirretroviral con lopinavir/ritonavir muestran una estabilización de la fibrosis hepática medida con FibroScan® tras un año de seguimiento. En conjunto, el tratamiento mejoró la fibrosis cuando la referencia de medición fue el índice APRI y el FIB-4, pero no con el índice FORNS o la elastografía.

  20. Estatus vitamínico actual en pacientes pediátricos y adultos jóvenes con fibrosis quística

    OpenAIRE

    D. González Jiménez; C. Bousoño García; J.J. Díaz Martín; M.F. Rivas Crespo; M.D. Acuña Quirós; S. Heredia González; A. Sojo Aguirre; A. Lázaro Almarza

    2014-01-01

    Introducción y objetivos. Las enfermedades como la fibrosis quística (FQ) que asocian malabsorción de grasas, precisan de suplementación de vitaminas liposolubles para evitar su deficiencia. El objetivo de nuestro objetivo fue conocer el estado de las vitaminas A, D y E en pacientes pediátricos y adultos con FQ. Pacientes y métodos. Estudio multicéntrico descriptivo y transversal, realizado en cinco hospitales nacionales, 126 pacientes con FQ sin agudización respiratoria, con edades entre...

  1. Aspergillus fumigatus in the cystic fibrosis lung: pros and cons of azole therapy.

    Science.gov (United States)

    Burgel, Pierre-Régis; Paugam, André; Hubert, Dominique; Martin, Clémence

    2016-01-01

    Aspergillus fumigatus is the main fungus cultured in the airways of patients with cystic fibrosis (CF). Allergic bronchopulmonary aspergillosis occurs in ~10% of CF patients and is clearly associated with airway damage and lung function decline. The effects of A. fumigatus colonization in the absence of allergic bronchopulmonary aspergillosis are less well established. Retrospective clinical studies found associations of A. fumigatus-positive cultures with computed tomography scan abnormalities, greater risk of CF exacerbations and hospitalizations, and/or lung function decline. These findings were somewhat variable among studies and provided only circumstantial evidence for a role of A. fumigatus colonization in CF lung disease progression. The availability of a growing number of oral antifungal triazole drugs, together with the results of nonrandomized case series suggesting positive effects of azole therapies, makes it tempting to treat CF patients with these antifungal drugs. However, the only randomized controlled trial that has used itraconazole in CF patients showed no significant benefit. Because triazoles may have significant adverse effects and drug interactions, and because their prolonged use has been associated with the emergence of azole-resistant A. fumigatus isolates, it remains unclear whether or not CF patients benefit from azole therapy.

  2. Studio di prevalenza batterica e fungina su espettorati provenienti da pazienti con Fibrosi Cistica

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    Marina Brugia

    2005-06-01

    Full Text Available Over the past 20 years there has been a grater interest in infection control in cystic fibrosis (CF.The epidemiology of pathogens in CF patients has become more complex; many common and complex pathogens species may be pathogens in patients with CF. In addition the number of different bacterial species isolated from respiratory specimens from this patient population are also numerous.The main objective when treating people who have CF is to prevent, eradicate or control all types of respiratory infection, particularly endobronchial and pulmonary infection. We examined 475 sputum of 69 patients with FC during 2003. Pseudomonas aeruginosa was prevalent, occurs in 85% of CF patients studied.The mucoid phenotype was isolated in 44,4% of patients.The prevalence of S. maltophilia, A. xylosoxidans, Burkholderia cepacia and Aspergillus spp. in our patients is 1,8%, 2,1%, 2,9% and 26,1% respectively. CF isolates are tested for antibiotic susceptibility for control of antimicrobial resistance and the emergence of multidrug-resistant organism.The choice of antibiotic depends on in vitro sensitivity pattern.The regular microbiological monitoring can contribute to extend life for many CF patients but also contribute to improving the quality of life for this population.

  3. Aspergillus fumigatus in the cystic fibrosis lung: pros and cons of azole therapy

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    Burgel PR

    2016-09-01

    Full Text Available Pierre-Régis Burgel,1,2 André Paugam,2,3 Dominique Hubert,1,2 Clémence Martin1,2 1Department of Respiratory Medicine, Cochin Hospital, Assistance Publique – Hôpitaux de Paris, 2Université Paris Descartes, Sorbonne Paris Cité, 3Parasitology-Mycology Laboratory, Cochin Hospital, Assistance Publique – Hôpitaux de Paris, Paris, France Abstract: Aspergillus fumigatus is the main fungus cultured in the airways of patients with cystic fibrosis (CF. Allergic bronchopulmonary aspergillosis occurs in ~10% of CF patients and is clearly associated with airway damage and lung function decline. The effects of A. fumigatus colonization in the absence of allergic bronchopulmonary aspergillosis are less well established. Retrospective clinical studies found associations of A. fumigatus-positive cultures with computed tomography scan abnormalities, greater risk of CF exacerbations and hospitalizations, and/or lung function decline. These findings were somewhat variable among studies and provided only circumstantial evidence for a role of A. fumigatus colonization in CF lung disease progression. The availability of a growing number of oral antifungal triazole drugs, together with the results of nonrandomized case series suggesting positive effects of azole therapies, makes it tempting to treat CF patients with these antifungal drugs. However, the only randomized controlled trial that has used itraconazole in CF patients showed no significant benefit. Because triazoles may have significant adverse effects and drug interactions, and because their prolonged use has been associated with the emergence of azole-resistant A. fumigatus isolates, it remains unclear whether or not CF patients benefit from azole therapy. Keywords: itraconazole, voriconazole, posaconazole, azole resistance, allergic bronchopulmonary aspergillosis

  4. Fibrosis retroperitoneal

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    Claudio Orlich-Castelán

    2005-07-01

    Full Text Available Se reporta el caso de una mujer de 61 años de edad, con antecedente de tuberculosis pélvica en la adolescencia, que se presentó con insuficiencia renal aguda y dolor lumbar y a quien se le diagnosticó fibrosis retroperitoneal. Se revisa la bibliografía reciente y los principales aspectos de esta enfermedadRetroperitoneal fibrosis. is an uncommon disease complicated by ureteral entrapment causing hydronephrosis and obstructive renal failure. We herein report a case recently diagnosed at our institution and review the literature on this topic

  5. Cumplimiento de las recomendaciones en rehabilitación respiratoria de la British Thoracic Society en pacientes con fibrosis quística: estudio en fisioterapeutas colombianos

    OpenAIRE

    Diana Duran-Palomino; Olga Chapetón; Jaime Martínez-Santa; Adriana Campos-Rodríguez; Robinson Ramírez-Vélez

    2013-01-01

    Con el objetivo de evaluar el cumplimiento de las recomendaciones en rehabilitación respiratoria (ReR), planteadas por la British Thoracic Society (BTS) en pacientes con fibrosis quística (FQ) se realizó un estudio transversal entre 224 fisioterapeutas colombianos para identificar el tipo de intervenciones y las características de los programas de ReR como componentes del cumplimiento de las guías clínicas basadas en la evidencia de la BTS. Un elevado porcentaje de profesionales respondieron ...

  6. Fibrosis retroperitoneal

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    Claudio Orlich-Castelán

    2005-07-01

    Full Text Available Se reporta el caso de una mujer de 61 años de edad, con antecedente de tuberculosis pélvica en la adolescencia, que se presentó con insuficiencia renal aguda y dolor lumbar y a quien se le diagnosticó fibrosis retroperitoneal. Se revisa la bibliografía reciente y los principales aspectos de esta enfermedad

  7. Dificultades en el estado emocional de jóvenes afectados por degeneración retiniana y sus familiares

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    HELENA CHACÓN-LÓPEZ

    2011-02-01

    Full Text Available Varios estudios han resaltado los cambios emocionales y funcionales derivados de la pérdida visual en personas con enfermedades degenerativas de retina, como la Retinosis Pigmentaria (RP. El objetivo del presente estudio era determinar la posible presencia de depresión y/o ansiedad en jóvenes con Retinosis Pigmentaria y sus familiares frente a un grupo sin alteraciones visuales. La muestra estaba compuesta por 78 personas distribuidas en tres grupos: 22 personas afectadas por RP, 22 familiares y 34 personas que formaban el grupo control. Todos ellos fueron evaluados con el Cuestionario de Ansiedad STAI y el Inventario de Depresión de Beck. Los resultados, aplicando un MANOVA y pruebas post-hoc, mostraban depresión leve en los afectados y mayores niveles de ansiedad, como estado y como rasgo, tanto en ellos como en sus familiares. Se concluye recomendando evaluar estas variables tras el diagnóstico de la enfermedad y transcurrido un tiempo, con el fin de proporcionar el apoyo psicológico adecuado tanto a los afectados como a los familiares; ello permitirá resolver posibles problemas asociados al progreso de la enfermedad, mejorar su estado emocional y la calidad de vida de ambos grupos.

  8. Cumplimiento de las recomendaciones en rehabilitación respiratoria de la British Thoracic Society en pacientes con fibrosis quística: estudio en fisioterapeutas colombianos

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    Diana Duran-Palomino

    2013-06-01

    Full Text Available Con el objetivo de evaluar el cumplimiento de las recomendaciones en rehabilitación respiratoria (ReR, planteadas por la British Thoracic Society (BTS en pacientes con fibrosis quística (FQ se realizó un estudio transversal entre 224 fisioterapeutas colombianos para identificar el tipo de intervenciones y las características de los programas de ReR como componentes del cumplimiento de las guías clínicas basadas en la evidencia de la BTS. Un elevado porcentaje de profesionales respondieron “realizar siempre” intervenciones con alto nivel de evidencia (grado A como: técnicas de higiene bronquial (54,0% y ciclo activo de la respiración (35,3%. Se observaron también intervenciones con menor grado de recomendación (grado D como: uso de solución salina hipertónica y broncodilatador para evitar el broncoespasmo (33,9%, y técnicas de terapia manual o ejercicios de movilidad torácica y resistida (38,4% para corregir problemas posturales y respiratorios. En conclusión, se encontraron importantes discrepancias con las intervenciones y componentes sugeridos por BTS en pacientes con FQ.

  9. Factores que influyen en la adherencia al tratamiento kinésico en los pacientes con fibrosis quística.

    OpenAIRE

    Pucciarelli, Daiana Díaz

    2010-01-01

    La Fibrosis Quística (FQ) es una enfermedad genética caracterizada por afectación pulmonar crónica, malabsorción de los alimentos y sudor salado. Se produce como consecuencia de la mutación del gen que controla el ingreso y escape de cloro y sodio (sal) a través de las paredes de las células y tejidos del cuerpo. Se trata de una exocrinosis, es decir que todas las glándulas exócrinas pueden estar afectadas, con producción de moco anormal, viscoso y adherente, que obstruye ...

  10. Caracterización, por RAPD-PCR, de aislados de Pseudomonas aeruginosa obtenidos de pacientes con fibrosis quística RAPD-PCR characterization of Pseudomonas aeruginosa strains obtained from cystic fibrosis patients

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    Maribel Ortiz-Herrera

    2004-04-01

    Full Text Available OBJETIVO: Caracterizar a las cepas de P aeruginosa aisladas de lavados broncoalveolares de pacientes con fibrosis quística a lo largo de un periodo de tres años. MATERIAL Y MÉTODOS: Estudio prospectivo, de seguimiento de una población de pacientes con fibrosis quística. Se utilizó la técnica de la amplificación del ADN empleando PCR con bajas condiciones de especificidad (Random amplified polymorphic DNA, RAPD-PCR para la amplificación del ADN de cepas de P aeruginosa aisladas de lavados broncoalveolares de cinco pacientes con fibrosis quística, provenientes del Servicio de Neumología y Cirugía del Tórax del Instituto Nacional de Pediatría de la Ciudad de México, en el periodo de junio de 1996 a junio de 2002; se establecieron los patrones de amplificación de cada aislamiento, lo que permitió la identificación precisa de todas las cepas aisladas y el estudio de la epidemiología de P aeruginosa en los pacientes seleccionados con dicha enfermedad. RESULTADOS: Se definieron 18 patrones de amplificación del ADN que permitieron identificar a cada cepa de P aeruginosa aislada en las diferentes muestras de lavado broncoalveolar; no se encontró relación entre el fenotipo de P aeruginosa (mucoide o no mucoide y el genotipo de cada aislamiento, ya que cepas con fenotipos distintos mostraron patrones de amplificación semejantes; en nuestros pacientes se identificaron cepas con patrones de amplificación distintos a partir de una misma muestra, lo que sugiere la presencia de infecciones simultáneas por más de una cepa de P aeruginosa; se demostró que dos hermanos con la enfermedad compartían cepas con genotipos semejantes, lo que sugiere una contaminación cruzada entre ambos, y se demostró el aislamiento de cepas de P aeruginosa con genotipos semejantes a lo largo de los periodos estudiados. CONCLUSIONES: La identificación mediante la caracterización genotípica de las cepas de P aeruginosa aisladas de los pacientes con

  11. Utilidad de los diferentes métodos no invasivos de predicción de fibrosis hepática en pacientes del País Vasco con hemocromatosis fenotípica Utility of various non-invasive methods for fibrosis prediction among Basque Country patients with phenotypic hemochromatosis

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    A. Castiella

    2008-10-01

    Full Text Available Objetivo: determinar si el producto de la edad por la concentración de hierro hepático (índice de fibrosis y los valores de plaquetas, ferritina y transaminasas están relacionados con el riesgo de padecer fibrosis avanzada (F ≥ 3 en hemocromatosis. Métodos: estudio retrospectivo de 32 pacientes con hemocromatosis hereditaria con expresión fenotípica. Todos los pacientes fueron biopsiados obteniéndose la concentración de hierro hepático. Resultados: en 7 pacientes se realizó RM (1,5T con obtención de concentración de hierro hepático (protocolo de Alustiza. Biopsia hepática: en 23 pacientes fibrosis 0-2; en 9 fibrosis 3-4. El índice de fibrosis mostró una especificidad del 68%, sensibilidad del 85,7%, VPP del 42,8% y VPN del 94,4% para fibrosis avanzada. La cifra de plaquetas ( 1.000 VPN 75% y el índice de fibrosis por RMN (punto corte 480.000 VPN 80%. La combinación de los mismos, el índice de fibrosis (por biopsia o por RM con las transaminasas y las plaquetas con las transaminasas, reveló un VPN del 100%. Conclusiones: el índice de fibrosis (> 480.000 y las plaquetas (Objective: to determine whether the product of multiplying age by liver iron concentration (LIC (fibrosis index; cut-off, 480,000, platelets, transaminases, and ferritin values are related to the risk of high grade fibrosis. Methods: a retrospective study of 32 patients with hereditary hemochromatosis (HH with phenotypic expression. All patients had a liver biopsy with LIC. Results: in 7 patients a magnetic resonance imaging (MRI scan (1.5 T was obtained with LIC following Alustiza's protocol. Liver biopsy: fibrosis grade (F 0-2 in 23 patients; F 3-4 in 9. Fibrosis index (FI showed a specificity of 68%, sensitivity of 85.7%, positive predictive value (PPV of 42.8%, and negative predictive value (NPV of 94.4% for high-grade fibrosis. Platelet count ( 1,000 a NPV of 75%, and MRI-derived LIC x age (> 480,000 a NPV of 80%. The combination of FI (either by

  12. Utilidad de la elastografía de transición (Fibroscan® en la evaluación de la fibrosis hepática en pacientes con hepatopatía crónica

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    Daniel Álvarez

    2012-02-01

    Full Text Available El pronóstico de la enfermedad crónica hepática depende de la extensión y la progresión de la fibrosis hepática. Actualmente la biopsia hepática es la técnica de elección para determinar el grado de fibrosis, pero es una prueba invasiva, no exenta de complicaciones. Por ello, el desarrollo de marcadores no invasivos de fibrosis hepática se convirtió en una necesidad indiscutible. Se propuso la elastografìa por transición (Fibroscan® para valorar la fibrosis hepática en pacientes con enfermedad crónica hepática, mediante la medición de la rigidez hepática. Nuestro objetivo fue evaluar la efectividad, la objetividad y la seguridad de esta técnica. Se estudiaron 68 pacientes a los que se les realizó una biopsia hepática en los 18 meses previos al estudio. Todos los procedimientos de elastografia y biopsia hepática fueron analizados por un mismo profesional (DA y MA, respectivamente. Para la valoración de la biopsia hepática se utilizó la escala METAVIR. El valor medio de rigidez en pacientes sin fibrosis o con fibrosis leve (F0-F1 y en los pacientes con fibrosis avanzada o cirrosis (F3-F4 fue 6.8 ± 3.0 kPa y 21.0 ± 15.1 kPa, respectivamente (con diferencia significativa, p < 0.01. Las áreas debajo de la curva ROC definieron los niveles de corte en cada grupo. Con independencia del diagnóstico etiológico de enfermedad hepática, hallamos una correlación positiva, en todos los pacientes, entre rigidez hepática medida por elastografìa y grado de fibrosis hepática en la biopsia. En conclusión, podemos considerar que el Fibroscan® es un método no invasivo, seguro, fácil y rápido, que lo convierte en la alternativa a la biopsia para identificar fibrosis significativa o cirrosis.

  13. Insulin resistance as a non-invasive method for the assessment of fibrosis in patients with hepatitis C: a comparative study of biochemical methods La resistencia a la insulina en la valoración no invasiva de la fibrosis en pacientes con hepatitis C: Estudio comparativo de métodos bioquímicos

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    M. Romera

    2006-03-01

    Full Text Available Introduction: insulin resistance (IR promotes the progression of fibrosis and diminishes response to treatment in patients with hepatitis C. Recently, Sydney's index (includes IR has been proposed as a non-invasive method for the prediction of fibrosis. Objective: to assess the usefulness of Sydney's index for the prediction of advanced fibrosis (F3-F4 or absence of significant fibrosis (F0-F1 in patients with chronic hepatitis C. Patients and methods: we included 131 patients suffering from chronic hepatitis C. Mean age was 40 ± 11, 78 men and 53 women. Fibrosis stage was (F0-F1 69 patients, F2: 40, and advanced (F3-F4 in 22 patients. We measured baseline AST, ALT, GGT, platelet, cholesterol, alcohol, and IR (HOMA - IR levels. Sydney, Forns' and APRI indexes were calculated. Results: the area under the curve for the diagnosis of absence of significant fibrosis in each method was: Sydney: 0.80, Forns: 0.71, APRI: 0.70; p = ns. Moreover, the diagnostic capacity of advanced fibrosis was: Sydney: 0.88, Forns: 0.83, APRI: 0.82; p = ns. The predictive negative value of significant fibrosis was 74, 72, and 67%, respectively. Due to the presence of intermediate values, the indexes were not applicable to 36, 44 and 43% of patients respectively. Conclusions: the incorporation of insulin resistance among biochemical non-invasive methods slightly improves the yield of other indexes. Nevertheless, results are suboptimal, and more than one third of patients might not be correctly classified.Introducción: la resistencia a la insulina (RI promueve la progresión de la fibrosis y disminuye la respuesta al tratamiento en pacientes con hepatitis C. Recientemente, se ha propuesto el índice de Sidney como método no invasivo de predicción de la fibrosis que incluye la RI. Objetivo: valorar la utilidad del índice de Sidney en la predicción de fibrosis avanzada (F3-F4 o ausencia de fibrosis significativa (F0-F1 en pacientes con hepatitis C. Pacientes y m

  14. Características clínico-epidemiológicas de pacientes afectados por condilomatosis

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    Graciela Caridad Cabrera Acea

    2011-08-01

    Full Text Available Fundamento: el condiloma acuminado es una afección frecuente en nuestro país y en el mundo, considerada una infección de transmisión sexual, pero que no es notificada habitualmente. Objetivo: determinar las principales características clínico-epidemiológicas de pacientes afectados por condilomatosis. Métodos: estudio de serie de casos, realizado en las Áreas II, V, VII del municipio Cienfuegos, que incluyó 72 pacientes con diagnóstico de condiloma acuminado. Se analizaron las variables: edad, sexo, nivel de escolaridad, área de procedencia, localización de las lesiones, número de parejas sexuales referidas en los últimos 12 meses, antecedentes patológicos personales de infecciones de transmisión sexual e infecciones asociadas, preferencia sexual y modo de detección de la enfermedad. Resultados: la condilomatosis genital fue la más frecuente, predominó en el sexo femenino, edades entre 20 y 39 años (51,4 % y en el nivel escolar medio superior (66,7 %; el 55,6 % de los pacientes presentaron antecedentes patológicos personales de infecciones de transmisión sexual e infecciones asociadas previas al diagnóstico; el 23, 6 % presentó infección concomitante con el diagnóstico de condilomatosis y de ellos, 70,6 % correspondió a vaginosis bacteriana. La enfermedad predominó en heterosexuales y en los que refirieron entre 2 y 3 parejas sexuales en los últimos 12 meses. La mayoría de los casos fueron diagnosticados de forma espontánea. Conclusiones: las características clínico-epidemiológicas de los pacientes diagnosticados con condilomatosis pertenecientes a las áreas de salud II, V, VII son similares a las reportadas en los casos estudiados en el país y en el mundo.

  15. Utilidad de la elastografía de transición (Fibroscan® en la evaluación de la fibrosis hepática en pacientes con hepatopatía crónica Usefulness of transient elastography (Fibroscan® in the assessment of fibrosis in patients with chronic liver disease

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    Daniel Álvarez

    2012-02-01

    Full Text Available El pronóstico de la enfermedad crónica hepática depende de la extensión y la progresión de la fibrosis hepática. Actualmente la biopsia hepática es la técnica de elección para determinar el grado de fibrosis, pero es una prueba invasiva, no exenta de complicaciones. Por ello, el desarrollo de marcadores no invasivos de fibrosis hepática se convirtió en una necesidad indiscutible. Se propuso la elastografìa por transición (Fibroscan® para valorar la fibrosis hepática en pacientes con enfermedad crónica hepática, mediante la medición de la rigidez hepática. Nuestro objetivo fue evaluar la efectividad, la objetividad y la seguridad de esta técnica. Se estudiaron 68 pacientes a los que se les realizó una biopsia hepática en los 18 meses previos al estudio. Todos los procedimientos de elastografia y biopsia hepática fueron analizados por un mismo profesional (DA y MA, respectivamente. Para la valoración de la biopsia hepática se utilizó la escala METAVIR. El valor medio de rigidez en pacientes sin fibrosis o con fibrosis leve (F0-F1 y en los pacientes con fibrosis avanzada o cirrosis (F3-F4 fue 6.8 ± 3.0 kPa y 21.0 ± 15.1 kPa, respectivamente (con diferencia significativa, p The prognosis and management of chronic liver disease largely depends on the extent and progression of liver fibrosis. Unfortunately, liver biopsy, an invasive and painful technique with several limitations, continues to be the gold standard for the staging and grading of fibrosis. Therefore, accurate noninvasive tests for liver injury are urgently needed. During the last years, transient elastography (Fibroscan® has been proposed for the assessment of hepatic fibrosis in patients with chronic liver disease, by measuring liver stiffness. The aim of this study was to evaluate the effectiveness, objectivity and safety of this technique. We included 68 patients who underwent a liver biopsy in the last 18 months with a wide spectrum of chronic liver

  16. Revisión sistemática con meta-ánálisis: evidencia clínica de los beneficios del ejercicio físico para la salud en niños y adolescentes con fibrosis quística

    OpenAIRE

    Chinchilla Portilla, Arelys

    2013-01-01

    Tesis a texto completo Presenta una revisión sistemática con meta-análisis en el cual se reúne la evidencia clínica disponible sobre los beneficios del ejercicio físico en niños y adolescentes con fibrosis quística, con el objetivo de analizar las principales variables moderadoras de las investigaciones experimentales que han incluido programas de entrenamiento físico en dicha población y así poder cuantificar efectos sobre la salud. Se inició con una búsqueda exhaustiva sobre las interven...

  17. Chronic hepatitis C treatment in a cystic fibrosis patient in the pulmonary pre-transplant stage Tratamiento de hepatitis crónica C en un paciente con fibrosis quística en situación de pretrasplante pulmonar

    Directory of Open Access Journals (Sweden)

    L. Adán Merino

    2010-10-01

    Full Text Available The standard treatment of chronic hepatitis C, pegylated interferon and ribavirin (pegI/R, has many limitations in both effectiveness and secondary effects, which makes it unsuitable or even contraindicated for some patients. In hepatitis C virus-infected cystic fibrosis patients this treatment could increase respiratory infections with subsequent pulmonary function deterioration. On the contrary, hepatitis C virus (HCV infection may make lung transplant (LT unfeasible. We present the case of a cystic fibrosis-young man diagnosed with HCV infection during LT assessment who was treated with pegI/R. In spite of the lung function worsening and respiratory infections, he managed to complete treatment and even sustained virological response (SVR. At present he is on LT waiting list.El tratamiento estándar de la hepatitis crónica C, interferón pegilado (INF-peg y ribavirina (RBV, puede ser inadecuado o incluso estar contraindicado en algunos pacientes debido a sus limitaciones en cuanto a eficacia y efectos adversos. En pacientes con fibrosis quística infectados por el virus de la hepatitis C (VHC el tratamiento antiviral podría aumentar las infecciones respiratorias con el consiguiente empeoramiento de la función pulmonar. Por contra, la infección por VHC podría desestimar a estos pacientes para un necesario trasplante pulmonar. Presentamos el caso de un varón con fibrosis quística diagnosticado de infección VHC durante su evaluación previa al trasplante pulmonar. El paciente fue tratado con INF-peg y RBV. A pesar del empeoramiento en la función pulmonar y numerosas infecciones respiratorias intercurrentes, logró completar el tratamiento y obtener respuesta viral sostenida, encontrándose actualmente en lista de espera.

  18. Usual interstitial pneumonitis UIP presenting with Wells grade 3. Can imaging methods help predict further progression of disease?; Fibrosi polmanare idiopatica con grado 3 di Wells all'esordio: possono le metodiche di diagnostica per immagini aiutare a predire la progressione ulteriore della malattia?

    Energy Technology Data Exchange (ETDEWEB)

    Fasano, L.; Pacilli, A. M.G. [Bologna Policlinico, Bologna (Italy). Ist. di Fisiopatologia Respiratoria; Zompatori, M.; Monetti, N. [Bologna Policlinico, Bologna (Italy). Servizio di Medicina Nucleare; Battista, G. [Bologna Policlinico, Bologna (Italy). Ist. di Radiologia, Radiodiagnostica 1; Di Scioscio, V.; Sciascia, N.

    1999-10-01

    Three different grades of idiopathic pulmonary fibrosis can be identified by HRCT pattern. Patients with predominant ground-glass opacity (grade 1) usually improve after treatment and may have a better prognosis. The subjects with a predominant reticular pattern and honeycombing (grade 3.) have irreversible fibrosis and usually do not improve after immunosuppressive therapy. Nevertheless, these patients may worsen even in the absence of HRCT features of the so-called alveolitis. The aim of this report is to investigate the predictive role of some noninvasive imaging methods (HRCT with visual score of disease extent; Gallium scintigraphy; DTPA scintigraphy) in patients with idiopathic fibrosis and a prevalent macroscopic fibrosis at HRCT study. [Italian] La fibrosi polomare idiopatica viene distinta in 3 gradi con diversa prognosi in base alla predominanza di opacita' a vetro smerigliato da alveolite o di fibrosi irreversibile. La fibrosi irreversibile tuttavia non e' necessariamente una situazione stabile ma puo progredire ed evolvere ulteriormente. In particolare i pazienti che gia all'esordio presentano solo i segni della fibrosi possono peggiorare a distanza di tempo nonostante la terapia. Scopo del lavoro e' stato quello di individuare in un gruppo di pazienti con prevalente fibrosi macroscopica quale possa essere un parametro preditivo della successiva evoluzione della malattia.

  19. Redes y movimientos sociales en contra de la construcción de presas en México. El caso del Movimiento Mexicano de Afectados por las Presas y en Defensa de los Ríos.

    Directory of Open Access Journals (Sweden)

    Anahí Copitzy Gómez Fuentes

    2015-03-01

    Full Text Available La construcción de presas en México ha supuesto la generación de importantes conflictos y de numerosos movimientos sociales, a través de los cuales comunidades afectadas, organizaciones no gubernamentales, activistas y especialistas, manifiestan su oposición y rechazo al modelo energético y de gestión de agua vigentes en el país, los cuales promueven la construcción de grandes presas. El Movimiento Mexicano de Afectados por las Presas y en Defensa de los Ríos (MAPDER, representa un ejemplo de la organización en red de los pueblos afectados por la construcción de presas en México. El Movimiento surgió en el 2004 como parte de los esfuerzos internacionales para la consolidación de un movimiento mundial de afectados. Surge además de la necesidad de hacer un recuento histórico de todas las afectaciones sufridas por la construcción de presas, y en respuesta a una serie de conflictos que comenzaron a surgir y  a visibilizarse en el país a partir del 2000. El movimiento ha denunciado los impactos negativos de las presas, así como las violaciones a los derechos humanos de los pueblos afectados. Al mismo tiempo, se han propuesto alternativas y soluciones, con el objetivo de frenar los impactos y afectaciones a diversas comunidades.

  20. Pulmonary Fibrosis

    Science.gov (United States)

    Pulmonary fibrosis is a condition in which the tissue deep in your lungs becomes scarred over time. This ... blood may not get enough oxygen. Causes of pulmonary fibrosis include environmental pollutants, some medicines, some connective tissue ...

  1. PCR-heterodúplex por agrupamiento: Implementación de un método de identificación de portadores de la mutación más común causal de fibrosis quística en Colombia

    Directory of Open Access Journals (Sweden)

    Lina Manuela Jay

    2006-09-01

    Full Text Available Introducción: La fibrosis quística (FQ es una enfermedad autosómica recesiva frecuente, con una incidencia de 1 en 2,500 recién nacidos. La causan más de 1,300 mutaciones distintas en el gen regulador de la conductancia transmembranal de la fibrosis quística (CFTR. Sin embargo, la mutación F508del es la más común en la mayoría de las poblaciones. Objetivos: Desarrollo de una técnica rápida, de bajo costo y confiable que permita filtrar con rapidez a los portadores o afectados por esta mutación que mediante el asesoramiento genético, contribuya a disminuir la aparición de nuevos casos y a un diagnóstico temprano de los enfermos y así lograr un descenso en la morbilidad y la mortalidad asociadas con la fibrosis quística en Colombia. Metodología: En el presente estudio se aplicó la técnica PCR-heterodúplex por agrupamientos, gracias al análisis de 400 muestras de sangre en papel filtro obtenidas de individuos asintomáticos para la FQ. Resultados: En las pruebas de validación de la técnica PCR-heterodúplex por agrupamiento se obtuvo una eficiencia, reproducibilidad y especificidad de 100% y una sensibilidad de 92%. Conclusiones: Se demostró la sensibilidad y reproducibilidad de la técnica PCR Directa-heterodúplex por agrupamientos de hasta 10 muestras, que se pueden emplear en programas para filtrar heterocigotos y afectados de F508del.

  2. Intervención nutricional en niños y adolescentes con fibrosis quística: Relación con la función pulmonar Nutritional intervention in children and adolescents with cystic fibrosis: Relationship with pulmonary function

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    C. Martínez-Costa

    2005-06-01

    Full Text Available Objetivo: Evaluar los efectos de la intervención nutricional precoz y continuada en pacientes pediátricos con fibrosis quística (FQ y su posible implicación en la función pulmonar. Pacientes y métodos: Se incluyen el seguimiento de 19 pacientes con FQ (11 niñas, 8 niños de 17 meses a 18 años de edad y promedio de 10 años de enfermedad. El genotipo de 16 pacientes es delta F 508 (10 homo, 6 heterocigotos. Cada 2-3 meses se les ha realizado: valoración clínica, dietética y antropométrica clasificando el estado de nutrición (EN según Z pesto/talla, % peso para la talla, índice de masa corporal y Z talla/edad. Coincidentemente se obtuvo cultivo de secreciones respiratorias y espirometría; y anualmente determinaciones bioquímicas, hematológicas y de heces. La intervención nutricional incluyó: recomendaciones dietéticas, nutrición enteral (NE oral o invasiva y tratamiento farmacológico con ezimas pancreáticos, vitaminas liposolubles, minerales y oligoelementos. Resultados y comentarios: La mayoría experimentaron mejoría antropométrica resultando significativa para el peso, relaciónpeso/talla y pliegue tricipital (p Objective: To assess the effects of early and continuous nutritional intervention in pediatric patients with cystic fibrosis (CF and its possible implication in pulmonary function. Patients and methods:Included is the follow-up of 19 patients with CF (11 female and 8 male children, from 17 months to 18 years of age, and a mean disease duration of 10 years. Genotype from 16 patients is delta F 508 (10 homozygotic, 6 heterozygotic. The following items have been performed every 2-3 months: clinical, dietary and anthropometrical assessment, classifying nutritional status (NS by Z scores of weight/height, % of weight to height, body mass index, and Z scores of height/age. Concurrently, respiratory secretions culture and spiro-metry were obtained; and annually, biochemistry, hematologic and feces determinations

  3. Evaluaci??n de la fuerza muscular y de los efectos sobre la misma de un programa de entrenamiento y de desentrenamiento en afectados por esclerosis m??ltiple de Castilla y Le??n

    OpenAIRE

    Medina P??rez, Carlos

    2012-01-01

    210 p. Con este trabajo nos hemos propuesto evaluar la fuerza muscular, observar los efectos de un programa de entrenamiento de fuerza de doce semanas de duraci??n, realizado con una intensidad entre el 35% y el 70% de la fuerza isom??trica m??xima y determinar los efectos del mismo durante un periodo de desentrenamiento de doce semanas, en los afectados por esclerosis m??ltiple en la Comunidad Aut??noma de Castilla y Le??n. En base a ello, las principales conclusiones de este trabajo son:...

  4. Síndrome de obstrucción intestinal distal en pacientes adultos con fibrosis quística: Casos clínicos

    OpenAIRE

    HEINE T,CLAUDIO; PARADA C,MARÍA TERESA; Gil D,Rodrigo; LÓPEZ K,FRANCISCO; LIZANA S,CARLOS; FERNÁNDEZ A,MANUEL; Quera P,Rodrigo

    2010-01-01

    Distal Intestinal Obstruction Syndrome (DIOS) has a 16% incidence among patients with Cystic Fibrosis (CF). It is characterized by an intestinal obstruction secondary to fecal impaction in distal ileum or cecum. We report two adult patients with DIOS. A female with CF and subjected to lung transplantation at the age of 13 years old. Five years later, she consulted for an intestinal obstruction. She was treated conservatively with a good clinical evolution. She had a new episode of DIOS eight ...

  5. Aspergilosis broncopulmonar alérgica, una complicación del paciente con fibrosis quística: reporte de dos casos y revisión de la literatura = Allergic bronchopulmonary aspergillosis, a complication of patients with cystic fibrosis: Report of two cases and review of the literature

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    Ballesteros Calderón, Alicia Lucía

    2012-01-01

    Full Text Available La aspergilosis broncopulmonar alérgica (ABPA es la enfermedad pulmonar resultante de hipersensibilidad a Aspergillus, que lleva a alteración de la depuración mucociliar, impactación mucosa con obstrucción de la vía aérea e infiltración pulmonar. Se caracteriza clínicamente por disnea, sibilancias, fiebre, malestar general y expectoración de color marrón o negro, y por la mala respuesta clínica al tratamiento habitual instaurado para las exacerbaciones en pacientes con enfermedad pulmonar crónica. En los pacientes con fibrosis quística (FQ puede ser difícil establecer el diagnóstico de ABPA debido a la superposición de las características clínicas, radiológicas e inmunológicas de las dos enfermedades y a la presencia frecuente de infecciones bacterianas pulmonares. Por ello, se recomienda que en el seguimiento de dichos pacientes se haga medición anual de la inmunoglobulina E (IgE sérica total y, según su resultado y el contexto clínico, se plantee el diagnóstico de ABPA y se haga el tratamiento oportuno con esteroides con el cual mejoran los síntomas, la función pulmonar, la calidad de vida y el pronóstico. Presentamos dos pacientes pediátricos con diagnóstico de FQ de difícil control, en quienes se hizo el diagnóstico de ABPA y cuya sintomatología pulmonar mejoró con el tratamiento esteroide.

  6. Association of nutritional status, plasma, albumin levels and pulmonary function in cystic fibrosis Asociación entre el estado nutricional, niveles de albúmina plasmática y función pulmonar en pacientes con fibrosis quística

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    M. I. Souza dos Santos Simon

    2011-12-01

    Full Text Available Background & aims: Malnutrition is related with pulmonary disease. The aim was to analyze the association of lung function respectively to nutritional status, identified pulmonary pathogens and socioeconomic condition of patients attending a pediatric CF reference center. Methods: Cross-sectional study performed with CF patients aged 6 to 18 years attending a CF-Center in southern Brazil. Nutritional status, plasma albumin level and pulmonary bacterial colonization were assessed. The outcome studied was forced expiratory volume in 1 second (FEV1. Results: Eighty-five patients were included in this study. FEV1 was significantly associated with body mass index (BMI percentiles, plasma albumin level and methicillin resistant Staphylococcus aureus (MRSA pulmonary colonization. Regression analysis showed that BMI below the 10th percentile was associated with a 25.58% drop in FEV1, and plasma albumin levels equal to or lower than 4.1 mg/dL was associated with 18.6% FEV1 reduction. FEV1 was 14.4% lower in the MRSA infected patients. Plasma albumin of 4.25 mg/dL predicted FEV1 of 60% with 76.9% sensitivity and 72.2% specificity, and 85.7% accuracy. The socioeconomic status was not association with pulmonary function. Conclusion: BMI below the 10th percentile and albumin below 4.1 mg/dL were predictors of low FEV1. Chronic MRSA infection was associated with lower FEV1. Longitudinal studies may better complement these results.Objetivos: Analizar la asociación entre la función pulmonar y tres factores: el estado nutricional, el estado de patógenos pulmonares y el estado socio-económico de pacientes en un centro de referencia en atención a pacientes pediátricos con fibrosis quística. Métodos: Se realizó un estudio longitudinal en pacientes con fibrosis quística, de 6 a 18 años atendidos en un centro de fibrosis quística del Sur del Brasil. Fueron evaluados: estado nutricional, niveles de albúmina plasmática y colonización bacteriana pulmonar

  7. Determinación de volúmenes pulmonares mediante pletismografía en pacientes con fíbrosis quística Lung volume determinations by whole plethysmography in cystic fibrosis patients: A preliminary report

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    ILSE CONTRERAS E

    2008-03-01

    Full Text Available Introducción: La fibrosis quística (FQ exhibe un deterioro progresivo de la función pulmonar (FP asociada a infecciones recurrentes. Objetivo: Evaluar la FP en sujetos con FQ mediante espirometría, saturometría y pletismografía (PG determinando alguna relación matemática. Pacientes y Métodos: Se identificaron 10 pacientes capaces de realizar CVF, CRF, VR, CPT, VEF1, y FEF25-75 %; comparándose con los valores de Zapletal y de 33 controles sanos. Resultados: Edad promedio 13,6 años (rango: 9-20. Sa0(2y VEF1fueron normales en 10 y 5 sujetos, respectivamente (4 tenían VEF1entre 65-80%. La PG mostró 4 pacientes con VR/CPT > 30%, 3 con aumento de CRF y VR/CPT, sólo uno tuvo PG normal. Los promedios de CPT y VR fueron superiores a valores de referencia y controles (p Introduction: Cystic fibrosis (CF patients develop a progressive impairment of pulmonary function tests (PFT by recurrent infection. Objective: To evaluate PFT in CF patients using spirometry, oxygen saturometry (Sa0(2 and whole plethysmography (WP for determining possible mathematical relationships. Methods: FVC, FRC, RV, TLC, FEV1, FEF25.75, obtained in 10 CF patients were compared with Zapletal's reference values and with the values obtained in 33 healthy-school children. Results: Mean age 13.6 years-old (range: 9-20. All CF patients had normal Sa0(2, FEV1 was normal in five (four had FEV1 between 65-80%, 4 had RV/TLC > 30%, 3 had increased FRC and RV/TLC, only one CF patient had WP normal. The average of TLC and RV were higher than reference values and control group (p < 0.05. We found an inverse relationship between FEV1, FEF25-75 and RV/TLC [r = -0.642 (p < 0.05 and r = -0.803 (p < 0.01; respectively]. Conclusion: This study suggests an increase of pulmonary volume in CF patients with not necessarily abnormal spirometry and an inverse mathematical relationship between FEV1, FEF25-75 and RV/TLC

  8. La plataforma de afectados por la hipoteca y la lucha contra los desahucios en el Estado español

    OpenAIRE

    Carretero Miramar, José Luis; Amodio, Marcelo

    2014-01-01

    La Plataforma de Afectados por la Hipoteca (PAH), dedicada a la lucha contra los desahucios, se ha convertido en uno de los principales movimientos sociales del Estado Español. Tratamos de explicar el por qué y el cómo de su aparición.

  9. Learning about Cystic Fibrosis

    Science.gov (United States)

    ... Cystic Fibrosis Consensus Development Conference Statement Learning About Cystic Fibrosis What do we know about heredity and cystic ... Information What do we know about heredity and cystic fibrosis? Cystic fibrosis (CF) is the most common, fatal ...

  10. O que é ser mãe de uma criança com fibrose cística Qué significa ser madre de un niño con fibrosis quística What is being a mother of a child with cystic fibrosis

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    Keila Okuda Tavares

    2010-12-01

    Full Text Available Conhecer o que é ser mãe de uma criança que apresenta fibrose cística é fundamental para o cuidado desses indivíduos, pois geralmente ela divide as responsabilidades relacionadas ao tratamento com o filho que apresenta essa doença grave e sem cura. Nesse sentido, este estudo teve como objetivo conhecer a vivência de ser mãe de uma criança com fibrose cística. Trata-se de um estudo descritivo-exploratório, qualitativo, de caráter fenomenológico. Foram entrevistadas 14 mulheres e a coleta de dados foi realizada por meio de uma entrevista semiestruturada. A análise das falas transcritas na íntegra seguiu direcionamentos propostos pela fenomenologia. Essa é uma experiência que a tristeza, angústia, dúvidas, sofrimento e medo estão presentes, levando essas mulheres a refletirem sobre suas vidas e a de seus filhos, promovendo mudanças em seu mundo vida e uma reorganização de toda a família.Conocer o que es ser madre de un niño con fibrosis quística es esencial para el cuidado de estas personas, porque generalmente ella divide las responsabilidades relacionadas con el tratamiento, con el niño que tiene esta enfermedad grave y sin cura. Este estudio tuvo como objetivo conocer la vivencia de ser madre de un niño con fibrosis quística. Se trata de un estudio descriptivo-exploratorio, cualitativo, fenomenológico. Fueron entrevistadas 14 mujeres y la colecta de datos se realizó por medio de una entrevista semiestructurada. El análisis de las elocuciónes transcriptas en la íntegra siguió direcciones propuestas por la fenomenologia. Esta es una experiencia donde la tristeza, angustia, dudas, sufrimiento y el temor están presentes, haciendo que esas mujeres reflexionen a respecto de sus vidas y la de sus hijos, promoviendo cambios en su mundo vida y una reorganización de toda familia.Knowing what is being a mother of a child with cystic fibrosis is essential to the care of these individuals, because she usually divides the

  11. Lola tinha uma coisa: construção de um livro educativo para crianças com fibrose cística Lola tenía una cosa: construcción de un libro educativo para niños con fibrosis cística Lola had one thing: building an educational book for children with cystic fibrosis

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    Tainá Maués Pelúcio Pizzignacco

    2012-01-01

    Full Text Available Este trabalho descreve a experiência da construção de um livro de história com o objetivo de auxiliar profissionais de saúde e familiares a contarem para crianças menores de 5 anos sobre seu diagnóstico de Fibrose Cística. O livro traz a história de Lola, uma criança que descobre que tem Fibrose Cística e cria um monstro imaginário como representação da doença. A experiência emergida da prática profissional em um ambulatório multiprofissional de atendimento a esses pacientes, traduz a importância de conciliar teoria e prática, além da interdisciplinaridade para a construção de estratégias inovadoras e criativas no desenvolvimento do cuidado integral.Este trabajo describe la experiencia de la construcción de un libro de historia con el objetivo de auxiliar a profesionales de salud y familiares a contar a los niños menores de 5 años sobre su diagnóstico de Fibrosis Cística. El libro muestra la historia de Lola, un niño que descubre que tiene Fibrosis Cística y crea un monstruo imaginario como representación de la enfermedad. La experiencia emergida de la práctica profesional en un consultorio externo multiprofesional de atención a esos pacientes, traduce la importancia de conciliar la teoría y la práctica, además de la interdisciplinaridad para la construcción de estrategias innovadoras y creativas en el desarrollo del cuidado integral.This work describes the experience of constructing a story book with the objective of helping health professionals and families to share with children less than 5 years about their diagnosis of cystic fibrosis. The book tells the story of Lola, a child who discovers she has cystic fibrosis and creates an imaginary monster as a representation of the disease. The experience emerged out of professional practice in an outpatient multidisciplinary service for these patients, translating the importance of reconciling theory and practice, and interdisciplinarity for the construction of

  12. Responsabilidad social de las empresas en una sociedad de "afectados" (stakeholder society

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    Anita Weiss Belalcazar

    2003-11-01

    Full Text Available El presente artículo se refiere a los "sectores que afectan o son afectados por el quehacer de las empresas". El concepto se vincula a la problemática de la Gobernabilidad Corporativa (corporate governance y se refiere a la necesidad de las empresas de considerar los intereses de los bancos y de los trabajadores y no solo los intereses de los accionistas. Posteriormente el concepto se amplía a los proveedores y los clientes, integrandolos a la discusión sobre la Responsabilidad Social de las Empresas no solo frente a los sectores mencionados, sino también frente al Estado, la Sociedad y el Medio Ambiente. A modo de ejemplo se señala como podría analizarse mediante un método deductivo-inductivo la situación de los trabajadores en la sociedad contemporánea, a partir de aspectos sugeridos por la teoría sociológica.

  13. Citizens against Austerity: a Comparative Reflection on Plataforma de Afectados por la Hipoteca (PAH and Bündnis Zwangsräumung Verhindern (BZV

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    Vicente Ordóñez

    2015-01-01

    Full Text Available A pesar de las significativas diferencias socioeconómicas entre España y Alemania, ambos países han sido testigos de la creciente presencia de las iniciativas activistas que abordan los problemas de acceso a la vivienda. Plataforma de Afectados por la Hipoteca (PAH en España y Bündnis Zwangsräumung Verhindern (BZV en Berlín luchan para detener los desahucios y defender los derechos de vivienda de la ciudadanía. El objetivo de este trabajo consiste en reflexionar sobre cómo se está desarrollando la política en relación con la austeridad y la falta de bienes de primera necesidad para sectores de la población. En este trabajo se adopta una metodología cualitativa basada en un estudio de caso comparativo entre PAH y BZV con la finalidad de estudiar las similitudes y diferencias entre ambas plataformas. El artículo se centra sobre todo en los orígenes ideológicos y sociológicos, los repertorios y las lógicas políticas de PAH y BZV.

  14. IDENTIFICACIÓN DE DELECIONES EN AFECTADOS DE DISTROFIA MUSCULAR DE DUCHENNE Y BECKER (DMD/DMB Y DIAGNÓSTICO DE PORTADORAS POR METODOLOGÍAS MOLECULARES

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    Patricia Hernández Rodríguez

    2002-06-01

    Full Text Available Se diseñó un ensayo de PCR múltiplex (6-plex que amplifica simultáneamente 6 exones del gen de la distrofina, estos exones son los que presentan mayor frecuencia de mutación. La proporción de deleciones observada en este estudio mediante el sistema 6-plex correspondió al31,25%, además el60% del total de las deleciones involucró los exones 44 al 52. Con el fin de identificar mujeres portadoras de DMD y DMB se utilizó el cálculo de dosis génica, a través de esta metodología fueron identificadas 7 mujeres como portadoras y 15 como no portadoras de deleción para los exones analizados, en este estudio no se encontró ninguna mujer como portadora de duplicación. Con la utilización de polimorfismos dinucleotídicos (CAn localizados en el interior del gen fue posible establecer inforrnación sobre el cromosoma X que posiblemente está afectado en el 63% de las mujeresanalizadas.

  15. Características clínico-epidemiológicas de pacientes en edad pediátrica afectados por dermatofitosis

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    Yoan Bernardez Cruz

    2011-12-01

    Full Text Available Fundamento: a pesar de que pocas especies de hongos son patógenos para los humanos y aun cuando su importancia  es menor que las de las bacterias o los virus, no son insignificantes como causa de enfermedades. Desde el punto de vista práctico se ha comprobado que las micosis que estos causan representan uno de los primeros motivos de consulta dermatológica en nuestro país y el mundo. Objetivo: determinar las principales características clínico-epidemiológicas y de laboratorio de pacientes en edad pediátrica afectados por dermatofitosis. Métodos: estudio descriptivo, de serie de casos que incluyó 102 pacientes con diagnóstico de dermatofitosis cuya información fue  recogida de historias clínicas y en el laboratorio al realizarse raspado y cultivo de las lesiones. Resultados: se obtuvo mayor positividad en el examen por cultivo (70,59 %, frente a 66, 7 % en el examen por raspado; el Trichophyton rubrum fue el agente causal más frecuente en las uñas y en el cuerpo,  predominó en el grupo de 15 años y más, en los pacientes de color de piel blanca y en las féminas. La mayoría de los pacientes se infectaron a través del contacto con canes. Conclusiones: no son bajos los por cientos de pacientes pediátricos con dermatofitosis, la cual continúa siendo causa frecuente de consulta médica

  16. [Retroperitoneal fibrosis].

    Science.gov (United States)

    Babski, Paweł; Wojtuń, Stanisław; Gil, Jerzy

    2007-05-01

    Retroperitoneal fibrosis is a rare clinical entity characterised by the presence of patologic collagen tissue in a retroperitoneal space. The fibrous mass covers abdominal organs causing their disfunctions. RPF was described at the begining of XX century but its etiology is not clear yet. Usually it causes an ureter obstuction and hydronephrosis, that is why most commonly is diagnosed by urologists and nephrologists. However, retroperitoneal fibrosis can be multifacial disease. In some patients localisation of fibrosis is atypical and manifestationns can be varied. Gastrological symptoms like jaundice, bowel obstuction, ascites can occure. Besides, some early signs of RPF are nonspecific and can imitate alarming symptoms of neoplasma, e.g.: weight loss, anemia, malaise, anorexia, fever. This force us to initiate gastrological investigation. The awareness of this disease is important. The early diagnosis and treatment improves prognosis and alows to avoid heavy complications. In typical cases radiology is often enough for diagnosis. However, histological examination is needed in many cases, especialy when patological mass is located atypical. A treatment is made up of farmacology and surgery. The first one is based on steroids, immunossuppressant and tamoxifen. Surgery is needed to eliminate organs obstruction.

  17. Cystic Fibrosis and Pregnancy

    Science.gov (United States)

    ... Complications & Loss > Pregnancy complications > Cystic fibrosis and pregnancy Cystic fibrosis and pregnancy E-mail to a friend Please ... this page It's been added to your dashboard . Cystic fibrosis (CF) is a condition that affects breathing and ...

  18. Cystic Fibrosis Research

    Science.gov (United States)

    ... please turn Javascript on. Feature: Steady Advances Against Cystic Fibrosis Cystic Fibrosis Research Past Issues / Fall 2012 Table of Contents "Remarkable strides in cystic fibrosis research over the past two decades have culminated ...

  19. Efecto de la maniobra inspiratoria y de la hiperinsuflación pulmonar en los flujos espiratorios máximos en pacientes con fibrosis quística EFFECT OF INSPIRATORY MANEUVER AND LUNG HYPERINFLATION ON MAXIMAL EXPIRATORY FLOW-VOLUME CURVES IN CYSTIC FIBROSIS PATIENTS

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    IGNACIO SÁNCHEZ D.

    2003-01-01

    Full Text Available El tiempo inspiratorio previo a la realización de una maniobra espiratoria forzada máxima (FEM es una variable que no ha sido completamente estudiada en Fibrosis Quística (FQ. El objetivo de este estudio fue evaluar la hipótesis que niños con FQ tendrían mayores volúmenes y flujos espiratorios forzados al disminuir la duración del tiempo inspiratorio. Se estudiaron 16 niños con FQ, en etapa estable, sin haber presentado una exacerbación respiratoria en los últimos dos meses, (10 hombres, de edad 11,9 ± 0,7 años (promedio ± ES, con un FEV1 de 62,8 ± 6,4% predicho. Las pruebas de función pulmonar consistieron en la medición de volúmenes pulmonares estáticos y resistencia de la vía aérea, seguido de evaluación dinámica de la función pulmonar. Esta última medición fue precedida de 3 maniobras inspiratorias diferentes en forma aleatoria [inspiración y detención de 0 segundos (M0, 2 segundos (M2 o 5 segundos (M5]. El grupo se dividió en pacientes con hiperinsuflación pulmonar detectada por VR/CPT > 30%, (n = 9 y sin hiperinsuflación pulmonar RV/CPT The time course of the inspiration preceding the maximal forced expiratory maneuver (MFEM has not been studied in cystic fibrosis (CF. We tested the hypothesis that CF children would have significantly higher forced expiratory volumes (FVC and flows with decreasing duration of the inspiratory breathhold. We studied 16 stable CF patients (10 males with no pulmonary exacerbation 2 months prior to the study, aged 11.9 ± 0.7 years (mean ± SEM with a FEV1 of 62.8 ± 6.4% predicted. Pulmonary function tests included static lung volume and airway resistance measurements, followed by dynamic lung function measurements. The latter were preceded by 3 different inspiratory maneuvers in a random order [breathhold of 0 (M0, 2 (M2 or 5 seconds (M5]. Patients were divided by the presence of lung hyperinflation (RV/TLC > 30%, with 9 children with hyperinflation and 7 no

  20. Biomarkers for liver fibrosis

    Science.gov (United States)

    Jacobs, Jon M.; Burnum-Johnson, Kristin E.; Baker, Erin M.; Smith, Richard D.; Gritsenko, Marina A.; Orton, Daniel

    2015-09-15

    Methods and systems for diagnosing or prognosing liver fibrosis in a subject are provided. In some examples, such methods and systems can include detecting liver fibrosis-related molecules in a sample obtained from the subject, comparing expression of the molecules in the sample to controls representing expression values expected in a subject who does not have liver fibrosis or who has non-progressing fibrosis, and diagnosing or prognosing liver fibrosis in the subject when differential expression of the molecules between the sample and the controls is detected. Kits for the diagnosis or prognosis of liver fibrosis in a subject are also provided which include reagents for detecting liver fibrosis related molecules.

  1. Hepatic fibrosis in patients with chronic hepatitis C assessed by transient elastography: implications for determining the efficacy of antiviral therapy Evaluación de la fibrosis hepática en pacientes con hepatopatía crónica C mediante elastografía transitoria: implicaciones para determinar la eficacia del tratamiento antiviral

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    J. Mendoza

    2010-07-01

    Full Text Available Background: the efficacy of combination therapy with peginterferon plus ribavirin to eradicate viral infection in patients with chronic hepatitis C (CHC is well established; moreover, it is able to arrest or even reverse liver fibrosis. Aims: to analyze the measurements of hepatic stiffness as an index of liver fibrosis using transient elastography (TE in patients who underwent a sustained virological response (SVR during long-term follow-up; comparing the changes in the severity of fibrosis with non-responders patients. Material and methods: after hepatic fibrosis was studied in three patients with CHC who underwent a SVR during long-term follow up, a prospective study was initiated in 24 patients with CHC who received combination therapy to compare the evolution of fibrosis in those with SVR and those who were non-responders. The genotype of hepatitis C virus (HCV and the degree of viremia were determined. METAVIR scoring system was used for liver fibrosis. Hepatic stiffness was measured by TE. Results: of the initial three patients pre-treatment liver biopsies revealed active disease and fibrosis (stage 3 in two and mild fibrosis (stage 1 in one. After several years of follow up serum AST/ALT levels were normal and HCV RNA was undetectable in each case; in contrast to the baseline histological assessments of fibrosis, values for hepatic stiffness (3.4-6.9 KPa were compatible with an absence of any appreciable hepatic fibrosis. In the prospective study, 8 patients underwent a SVR and 16 were non-responders. TE indicated that the severity of hepatic fibrosis in the SVR group improved in 7 (88% patients, whereas in the non-responder it improved in only 4 (25% (p < 0.05. The difference between development of severe fibrosis (F ≥ 3 in responders and non-responders was not significant (p = 0.23, possibly due to the small sample size. Conclusions: regression of hepatic fibrosis appears to be common in patients with CHC who undergo a SVR. TE is a

  2. ANÁLISIS COMPARATIVO DE CARGAS DE COMBUSTIBLES EN ECOSISTEMAS FORESTALES AFECTADOS POR INCENDIOS

    OpenAIRE

    Jaqueline Xelhuantzi Carmona; José Germán Flores Garnica; Álvaro Agustín Chávez Durán

    2011-01-01

    En la actualidad existe poca información sobre las cargas de combustibles forestales en diferentes condiciones y ecosistemas. De acuerdo con esto, el objetivo del presente trabajo es compararla en bosques templados y selvas. Para lo cual se utilizó la metodología de intersecciones planares. Una vez recabada la información en campo, se estimaron los combustibles en toneladas por hectárea de las diferentes categorías (tiempo de retardo). Se evaluaron 24 conglomerados en seis estados de la Repúb...

  3. Socialization of children and adolescents with cystic fibrosis: support for nursing care El proceso de socialización de niños y adolescentes con fibrosis quística: apoyo para la atención de enfermería O processo de socialização de crianças e adolescentes com fibrose cística: subsídios para o cuidado de enfermagem

    Directory of Open Access Journals (Sweden)

    Tainá Maués Pelúcio Pizzignacco

    2006-08-01

    Full Text Available The aim of this paper is to identify the routine (school, work, free time activities and relationships with friends and family of children and adolescents with Cystic Fibrosis (CF through their experiences and identify situations that can affect these routines. The objects of analysis of this research are children and adolescents with CF who attend a teaching hospital in a city of the State of São Paulo - Brazil. It is a qualitative research, with data collection based on open interviews and patient charts. The data brought the following themes: misleading knowledge about the disease, concern with self-image, search for self-care and hope of improvement in the future. The results evidence the repercussion of CF in those patients' socialization process, evidencing the importance of health professionals knowing about these demands and incorporating them into the care plan, with a view to effective interventions to promote infant-juvenile growth and development.La finalidad de este estudio es conocer el día a día (escuela, trabajo, actividades de ocio e interacciones con la familia y amigos del niño y del adolescente con Fibrosis Quística (FQ a partir de sus propias vivencias e identificar situaciones que puedan interferir en esa cotidianidad. Los sujetos de la investigación son niños y adolescentes portadores de Fibrosis Quística bajo seguimiento en un hospital-escuela del interior del Estado de São Paulo, Brasil, con edad entre 7 y 18 años. Efectuamos una investigación con aproximación cualitativa. Los datos empíricos fueron recopilados mediante el análisis de registros médicos y entrevistas abiertas, revelando los siguientes temas: conocimiento equivocado sobre la enfermedad; preocupación con el autoimagen; búsqueda por autocuidado y esperanza de mejoras en el futuro. Los resultados evidencian las repercusiones de la Fibrosis Quística en el proceso de socialización de esos pacientes, es importante que los profesionales de

  4. Tamizaje neonatal para fibrosis quística en una muestra de la ciudad de Bogotá

    OpenAIRE

    Amado González, Paula

    2011-01-01

    La Fibrosis Quística es la enfermedad autosómica recesiva mas frecuente en caucásicos. En Colombia no se conoce la incidencia de la enfermedad, pero investigaciones del grupo de la Universidad del Rosario indican que podría ser relativamente alta. Objetivo: Determinar la incidencia de afectados por Fibrosis Quística en una muestra de recién nacidos de la ciudad de Bogotá. Metodología: Se analizan 8.297 muestras de sangre de cordón umbilical y se comparan tres protocolos de tamizaje neonatal: ...

  5. Cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Radlović Nedeljko

    2012-01-01

    Full Text Available Cystic fibrosis (CF is a multisystemic autosomal recessive disease caused by a defect in the expression of CFTR protein, i.e. chloride channel present in the apical membrane of respiratory, digestive, reproductive and sweat glands epithelium. It primarily occurs in the Caucasians, while being considerably or exceptionally rare in persons of other races. Absence, deficit or structural and functional abnormalities of CFTR protein lead to mucosal hyperconcentration in the respiratory, digestive and reproductive systems and malabsorption of chloride and sodium in the sweat glands. Thus, the clinical features of patients’ with CF are predominated by respiratory, digestive and reproductive disorders, as well as the tendency to dehydration in the condition of increased sweating. Beside genotype variations, the degree of disease manifestation is also essentially influenced by various exogenous factors, such as the frequency and severity of respiratory infections, the level of aero-pollution, quality of immunoprophylaxis, patients’ nutritional condition and other. Chloride concentration of over 60 mmol/L in sweat, a high level of immunoreactive chymotrypsinogen in blood and the verification of homozygous mutation of CFTR gene are the basic methods in the diagnostics of the disease. CF belongs to the group of severe and complex chronic diseases, and therefore requires multidisciplinary therapeutic approach. Owing to the improvement of healthcare provision, most patients with CF now survive into adulthood. In addition, their quality of life is also considerably improved.

  6. Cystic fibrosis - nutritional considerations

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/002437.htm Cystic fibrosis - nutrition To use the sharing features on this page, please enable JavaScript. Cystic fibrosis (CF) is a life-threatening disease that causes ...

  7. What Causes Cystic Fibrosis?

    Science.gov (United States)

    ... page from the NHLBI on Twitter. What Causes Cystic Fibrosis? A defect in the CFTR gene causes cystic ... in the severity of the disease. How Is Cystic Fibrosis Inherited? Every person inherits two CFTR genes—one ...

  8. Cystic fibrosis: case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Si Hyun; Lee, Hyun Ju; Kim, Ji Hye; Park, Chol Heui [Gachon Medical School, Inchon (Korea, Republic of)

    2002-12-01

    Cystic fibrosis is an autosomal recessive genetic disease. Among Caucasians, it is the most common cause of pulmonary insufficiency during the first three decades of life. The prevalence of cystic fibrosis varies according to ethnic origin: it is common among Caucasians but rare among Asians. We report a case in which cystic fibrosis with bronchiectasis and hyperaeration was revealed by high-resolution CT, and mutation of the cystic fibrosis conductance transmembrane regulator gene (CFTR) by DNA analysis.

  9. El ejercicio es medicina en la fibrosis quística

    OpenAIRE

    Pérez Ruiz, Margarita

    2015-01-01

    Proyecto PS09/00194. Prescripción de un programa de ejercicio físico intrahospitalario para niños con fibrosis quística; Proyecto de investigación intrahospitalario para niños con fibrosis quística (Hospital Universitario Niño Jesús) No data (2015) UEM

  10. Autophagy in Hepatic Fibrosis

    Directory of Open Access Journals (Sweden)

    Yang Song

    2014-01-01

    Full Text Available Hepatic fibrosis is a leading cause of morbidity and mortality worldwide. Hepatic fibrosis is usually associated with chronic liver diseases caused by infection, drugs, metabolic disorders, or autoimmune imbalances. Effective clinical therapies are still lacking. Autophagy is a cellular process that degrades damaged organelles or protein aggregation, which participates in many pathological processes including liver diseases. Autophagy participates in hepatic fibrosis by activating hepatic stellate cells and may participate as well through influencing other fibrogenic cells. Besides that, autophagy can induce some liver diseases to develop while it may play a protective role in hepatocellular abnormal aggregates related liver diseases and reduces fibrosis. With a better understanding of the potential effects of autophagy on hepatic fibrosis, targeting autophagy might be a novel therapeutic strategy for hepatic fibrosis in the near future.

  11. How Is Cystic Fibrosis Treated?

    Science.gov (United States)

    ... page from the NHLBI on Twitter. How Is Cystic Fibrosis Treated? Cystic fibrosis (CF) has no cure. However, ... help oral pancreatic enzymes work better. Treatments for Cystic Fibrosis Complications A common complication of CF is diabetes . ...

  12. Cystic Fibrosis: Diet and Nutrition

    Science.gov (United States)

    ... Loss Surgery? A Week of Healthy Breakfasts Shyness Cystic Fibrosis: Diet and Nutrition KidsHealth > For Teens > Cystic Fibrosis: ... Food Enzyme Supplements Beating the Frustration What Is Cystic Fibrosis? At lunch, Lindsay often gets bored with having ...

  13. Genetics Home Reference: cystic fibrosis

    Science.gov (United States)

    ... Me Understand Genetics Home Health Conditions cystic fibrosis cystic fibrosis Enable Javascript to view the expand/collapse boxes. Download PDF Open All Close All Description Cystic fibrosis is an inherited disease characterized by the buildup ...

  14. Neonatal cystic fibrosis screening test

    Science.gov (United States)

    Cystic fibrosis screening - neonatal; Immunoreactive trypsinogen; IRT test; CF - screening ... Cystic fibrosis is a disease passed down through families. CF causes thick, sticky mucus to build up in ...

  15. Fibrosis and Cardiac Arrhythmias

    NARCIS (Netherlands)

    de Jong, Sanne; van Veen, Toon A. B.; van Rijen, Harold V. M.; de Bakker, Jacques M. T.

    2011-01-01

    In this review article about fibrosis and arrhythmias, we show that the amount of collagen, a normal element of the heart muscle, increases with age and in heart disease. The relation between fibrosis and electrophysiological parameters such as conduction, fractionation of electrograms, abnormal imp

  16. O cotidiano da família com filhos portadores de fibrose cística: subsídios para a enfermagem pediátrica El cotidiano de la familia con hijos portadores de fibrosis quística: aportes para la enfermería pediátrica The routine of families with children bearing cystic fibrosis: subsidies for pediatric nursing

    Directory of Open Access Journals (Sweden)

    Maria Cândida de Carvalho Furtado

    2003-02-01

    Full Text Available A fibrose cística é uma doença genética caracterizada pelo aumento na produção de muco que, depositado em alguns órgãos, causa: doença pulmonar obstrutiva crônica, insuficiência pancreática e nível elevado de eletrólitos no suor. Pretende-se, neste estudo, descrever o cotidiano de famílias com filhos portadores de fibrose cística nos aspectos relacionados à repercussão da doença crônica na dinâmica familiar. Para tanto, realizou-se um estudo qualitativo, com coleta de dados empíricos a partir de entrevistas com 14 famílias de pacientes menores de 18 anos em tratamento no Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto, da Universidade de São Paulo. Dos dados emergiram os seguintes temas: envolvimento da família, aceitação/entendimento da doença e enfrentando desafios. Identificamos uma vida de dependência relacionada à doença, com desgaste físico e emocional, tanto da criança quanto da família. Implicações para a enfermagem: constituição de modelos assistenciais que tenham a família como foco de atenção, considerando meio ambiente, estilo de vida e promoção à saúde como seus fundamentos básicos.La fibrosis quística es una enfermedad genética caracterizada por el aumento en la producción de moco que es depositado en algunos órganos causando: enfermedad pulmonar obstructiva crónica, insuficiencia pancreática y nivel elevado de electrolitos en el sudor. El objetivo de este estudio es describir el cotidiano de las familias con hijos portadores de fibrosis quística en los aspectos relacionados a la repercusión de la enfermedad crónica en la dinámica familiar. Por lo tanto, se realizó un estudio cualitativo, a través de la recolección de datos empíricos a partir de entrevistas con 14 familias de pacientes menores de 18 años en tratamiento en el Hospital Clínicas de la Facultad de Medicina de Ribeirão Preto de la Universidad de São Paulo. De los datos emergieron los

  17. Crianças hospitalizadas com Fibrose Cística: percepções sobre as múltiplas hospitalizações Niños con Fribrosis Quistica: percepciones sobre las multiples hospitalizaciones Children with Cystic Fibrosis: perceptions about multiple hospital admissions

    Directory of Open Access Journals (Sweden)

    Ruth Irmgard Bärtschi Gabatz

    2007-02-01

    Full Text Available As crianças com fibrose cística (FC geralmente são hospitalizadas inúmeras vezes ao longo de suas vidas. Este estudo objetiva conhecer as percepções destas crianças em relação às múltiplas hospitalizações, bem como identificar o que percebem sobre o tratamento e os cuidados recebidos. Trata-se de um estudo descritivo com abordagem qualitativa. Os sujeitos são sete crianças portadoras de FC, entre 6 e 11 anos. Os dados são coletados em uma unidade pediátrica de um hospital geral, através de uma entrevista focalizada, pouco estruturada, e analisados pelo método da análise compreensiva. Os resultados deste estudo demonstram que as crianças prezam muito sua liberdade, que durante a hospitalização fica restringida, o que pode ser amenizado através da nossa escuta, compreensão e atenção.Los niños con fibrosis quística generalmente son hospitalizados innumerables veces a lo largo de sus vidas. Este estudio objetiva conocer las percepciones de estos niños con relación a las múltiplas hospitalizaciones, así como reconocer lo que se dan cuenta sobre el tratamiento y los cuidados recibidos. Se trata de un estudio descriptivo con abordaje cualitativa. Los sometidos son siete niños portadores de fibrosis quística, entre 6 y 11 anos. Los datos son colectados en una unidad pediátrica de un hospital general, por medio de una entrevista enfocada, poco estructurada y examinados por el método de la análisis comprensiva. Los resultados de este estudio enseñan que los niños estiman mucho su libertad, donde durante la internación se pone restriñida, lo que puede ser amenizado por medio de nuestra escucha, comprensión y atención.Children with cystic fibrosis are usually admitted to the hospital many times through their lives. This study aims to know the perceptions of these children towards the multiple hospital admissions, as well to identify what they realize about the treatment and the care they get. This is a descriptive

  18. El trabajo de las personas con discapacidad ante la crisis

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    Vanesa Rodríguez

    2013-12-01

    Full Text Available En un contexto de crisis económica como el actual, determinados colectivos, que ya partían de una peor posición dentro del mercado de trabajo, se han visto más afectados por la destrucción de empleo. El objetivo con el que se plantea este trabajo es identificar cómo ha afectado el cambio de ciclo económico al empleo de las personas con discapacidad, distinguiendo entre aquellos que prestan servicios en un centro especial de empleo (CEE y los que lo hacen en la empresa ordinaria. Para ello se utilizan datos de la Muestra continua de vidas laborales (MCVL en sus oleadas de 2005 a 2011, lo que nos permite realizar un seguimiento longitudinal de los individuos en dos momentos de muy distinta coyuntura económica: bonanza y crisis. A través de la MCVL podemos identificar a trabajadores con discapacidad tanto en empresas ordinarias como en CEE, lo que nos permite estudiar qué tipo de centro ofrece una mejor «protección» al empleo de sus trabajadores con discapacidad frente a la crisis. Los resultados preliminares muestran que son los trabajadores con una mayor relación con los CEE los que ven reducida en menor proporción su participación en el empleo.

  19. Angiogenesis and liver fibrosis

    Institute of Scientific and Technical Information of China (English)

    Gülsüm ?zlem Elpek

    2015-01-01

    Recent data indicate that hepatic angiogenesis,regardless of the etiology, takes place in chronic liverdiseases (CLDs) that are characterized by inflammationand progressive fibrosis. Because antiangiogenictherapy has been found to be efficient inthe prevention of fibrosis in experimental models ofCLDs, it is suggested that blocking angiogenesis couldbe a promising therapeutic option in patients withadvanced fibrosis. Consequently, efforts are beingdirected to revealing the mechanisms involved inangiogenesis during the progression of liver fibrosis.Literature evidences indicate that hepatic angiogenesisand fibrosis are closely related in both clinical andexperimental conditions. Hypoxia is a major inducer ofangiogenesis together with inflammation and hepaticstellate cells. These profibrogenic cells stand at theintersection between inflammation, angiogenesis andfibrosis and play also a pivotal role in angiogenesis.This review mainly focuses to give a clear view on therelevant features that communicate angiogenesis withprogression of fibrosis in CLDs towards the-end point ofcirrhosis that may be translated into future therapies.The pathogenesis of hepatic angiogenesis associatedwith portal hypertension, viral hepatitis, non-alcoholicfatty liver disease and alcoholic liver disease are alsodiscussed to emphasize the various mechanisms involvedin angiogenesis during liver fibrogenesis.

  20. Restauración ambiental y revegetación de los terrenos afectados por una obra civil lineal. Breve análisis del control de calidad realizado

    Directory of Open Access Journals (Sweden)

    Canga Cabañes, José Luis

    1996-04-01

    Full Text Available The report presents the results of an experience involving quality control for the environmental restoration and revegetation of the area affected by construction of a gas pipeline over 140 km long with an execution cost of 8,000 million pesetas. A brief account is given of the most relevant details of the work and the Revegetation Project. Most of the report centers on a description of the functions of the Environmental Control and Monitoring Team, discussing the main aspects of these functions, the advantages derived from them as regards the satisfactory result of the project and the economic profitability provided by the team's presence. The most important matters to be supervised in quality control are highlighted and illustrated by the experience gained on this project.

    Se exponen los resultados de una experiencia de control de calidad de los trabajos de restauración ambiental y revegetación de los terrenos afectados por la construcción de un gasoducto de más de 140 km de longitud y 8.000 millones de pesetas de coste de ejecución. Se indican brevemente los datos más relevantes de la obra y las principales magnitudes del Proyecto de Revegetación. La mayor parte del articulo se centra en explicar las funciones desempeñadas por el Equipo de Control y Vigilancia Ambiental, comentando sus aspectos esenciales, las ventajas que de ellas se han derivado para un buen resultado de las obras y la rentabilidad económica obtenida con su presencia. Se resaltan también las cuestiones más importantes a supervisar en un control de calidad, ilustrándolas con la experiencia obtenida.

  1. Intervención psicopedagógica en niños afectados por el síndrome TDAH. Estudio experimental

    OpenAIRE

    Campeño Martínez, Yoana

    2013-01-01

    El objetivo principal de esta investigación ha sido analizar la efectividad de la aplicación de un programa de intervención basado en el desarrollo de estrategias metacognitivas para mejorar la atención y la reflexividad denominado PIIAR-RI, (Gargallo, 1997) a un grupo de alumnos afectados por el síndrome TDAH, fundamentando los resultados en las percepciones de profesores y padres a través de la aplicación del EMA-DDA y comprobando, asimismo, la congruencia entre las percepciones de ambos, r...

  2. Reclutando profesionales con deseos de empleo.

    Directory of Open Access Journals (Sweden)

    Teresa Farfán Cabrera

    2014-01-01

    Full Text Available En los últimos años la vinculación retórica de la globalización con el empleo ha creado una serie de retos y prejuicios respecto a la forma en que la primera ha afectado la organización y producción del conocimiento dentro de las instituciones de educación superior públicas, lo que llevó a tomar caminos separados, pero ya es tiempo de generar nuevas formas de acercamiento que vinculen a los profesionistas con el actual mercado laboral y sus formas de contratación como un ejerció donde ambas partes puedan generar soluciones a problemas comunes.

  3. con dietas suplementadas con Cromo-L-metionina

    Directory of Open Access Journals (Sweden)

    Ram\\u00F3n Garc\\u00EDa-Castillo

    2006-01-01

    Full Text Available Un total de 48 cerdos (Sus scrofa domesticus; 24 machos castrados y 24 hembras cruzados (Yorkshire, Hampshire, Duroc y Landrace de 3,5 a 4,0 meses de edad y 60,0 ± 5,0 kg PV en finalización. Se alimentaron con dietas isoproteícas (14,5 % PC e isoenergéticas (3.400 kcal EM/kg de MS, adicionadas con Cr-L-metionina (MiCroPlex® (0, 200, 400 y 600 ppb. El experimento tuvo una duración de 45 días y se realizó de agosto a noviembre del 2002 en las instalaciones de la Universidad Autónoma Agraria Antonio Narro, localizada en Saltillo, Coahuila, México. Al tener los animales aproximadamente 95 kg PV, se tomó muestra de 15 ml de sangre por cada animal para determinar la concentración de glucosa, ácido úrico, creatinina, urea, proteinas totales y colesterol. Se aplicó un diseño completamente al azar con arreglo factorial 2 x 4; dos para el factor sexo y cuatro para nivel de cromo. Los metabolitos en suero no fueron afectados (P>0,05 por el factor sexo. La glucosa en suero disminuyó (P<0,05 y el colesterol incrementó (P<0,05 con cromo en la dieta. Se concluye que el Cr incrementa el metabolismo de glucosa y disminuye el de colesterol, con lo cual puede haber energía disponible para síntesis de proteína la cual es necesaria para el crecimiento de los animales

  4. Endomyocardial fibrosis in infancy

    Directory of Open Access Journals (Sweden)

    Jatene Marcelo Biscegli

    2003-01-01

    Full Text Available The patient was a 4-month-old infant, who underwent persistent ductus arteriosus interruption with titanium clips at the age of 13 days and, since the age of 2 months, had crises of hypoxia and hypertonicity. After clinical investigation, the presence of pulmonary hypertension was confirmed and left ventricular inflow tract obstruction was suspected. The patient underwent surgical treatment at the age of 4 months, during which right and left ventricular endocardial fibrosis was identified. The fibrosis was resected, but the infant had an unfavorable clinical evolution with significant diastolic restriction and died on the sixth postoperative day. Anatomicopathological and surgical findings suggested endomyocardial fibrosis, although that pathology is very rare at the patient's age.

  5. Serum markers of liver fibrosis

    DEFF Research Database (Denmark)

    Veidal, Sanne Skovgård; Bay-Jensen, Anne-Christine; Tougas, Gervais

    2010-01-01

    BACKGROUND: Fibrosis is a central histological feature of chronic liver diseases and is characterized by the accumulation and reorganization of the extracellular matrix. The gold standard for assessment of fibrosis is histological evaluation of a percutaneous liver biopsy. Albeit a considerable......-epitopes, may be targeted for novel biochemical marker development in fibrosis. We used the recently proposed BIPED system (Burden of disease, Investigative, Prognostic, Efficacy and Diagnostic) to characterise present serological markers. METHODS: Pubmed was search for keywords; Liver fibrosis, neo...

  6. Pergolide-induced pleuropulmonary fibrosis

    NARCIS (Netherlands)

    Bleumink, G S; van der Molen-Eijgenraam, M; Strijbos, J H; Sanwikarja, S; van Puijenbroek, E P; Stricker, B H Ch

    2002-01-01

    Pleuropulmonary fibrosis is a rare, but well-recognized adverse effect of ergot alkaloids. We report on four patients who developed pleural and/or pulmonary fibrosis during treatment with pergolide and give characteristics of 87 cases with one or more symptoms of serosal fibrosis. Retroperitoneal an

  7. Análisis de Riesgo Ambiental aplicado a un establecimiento afectado por el R.D. 1254/1999.

    OpenAIRE

    Figueroa Moreno, Ana

    2010-01-01

    Se pretende desarrollar el Análisis de Riesgo Ambiental de una empresa dedicada al metalizado por deposición electrolítica de piezas de plástico, situada en el municipio de Igualada, con objeto de dar respuesta a la Ley 26/2007, de 23 de octubre, de Responsabilidad Medioambiental, por tratarse de un establecimiento sujeto al ámbito de aplicación del Real Decreto 1254/1999, de 16 de julio, por el que se aprueban medidas de control de los riesgos inherentes a los accidentes graves en los que in...

  8. Esclerosis múltiple: análisis de necesidades y calidad de vida de los afectados y su entorno

    Directory of Open Access Journals (Sweden)

    José Carrón Sánchez

    2013-12-01

    Full Text Available La evolución en el paradigma desde el que se contempla la discapacidad, y en concreto la esclerosis múltiple, ha estado marcada por los cambios en el concepto de salud, en el avance en los derechos sociales subjetivos y en la implantación progresiva de un modelo inclusivo para su abordaje. Con esta investigación se pretende responder a las preguntas de ese nuevo paradigma, revisando la situación epidemiológica, los estudios anteriores, y el desarrollo de una metodología complementaria desde lo cuantitativo y lo cualitativo. El trabajo de campo –desarrollado en el ámbito de la Comunidad Foral de Navarra– y su posterior análisis nos ofrece una prevalencia considerablemente mayor que la habitualmente manejada hasta ahora, un perfil con características bastante estables en el que destaca el importante grado incapacitante y su correlato socioeconómico, y el aumento en los tratamientos farmacológicos respecto a datos anteriores. La ausencia de una respuesta curativa y el avance en las terapias paliativas exigen, como paso ineludible, la necesidad de poner el foco en aquellos aspectos que mejoren la calidad de vida de las personas afectadas y su entorno.

  9. Nephrogenic systemic fibrosis

    DEFF Research Database (Denmark)

    Khurram, Misbah; Skov, Lone; Rossen, Kristian;

    2007-01-01

    Nephrogenic systemic fibrosis (NSF) is a fibrotic disease seen in renal failure patients that may lead to severe physical disability. It has been demonstrated in recent studies that NSF can be caused by some gadolinium-containing MRI contrast agents. In this report we present one of a total of 26...

  10. Understanding Nephrogenic Systemic Fibrosis

    Directory of Open Access Journals (Sweden)

    Tushar Chopra

    2012-01-01

    Full Text Available Nephrogenic systemic fibrosis (NSF is a rare and a debilitating disease noted uncommonly in patients with impaired renal function when exposed to low-stability gadolinium-based contrast agents (Gd-CAs. According to experimental studies, cytokines released by the stimulation of effector cells such as skin macrophages and peripheral blood monocytes activate circulating fibroblasts which play a major role in the development of NSF lesions. The presence of permissive factors, presumably, provides an environment conducive to facilitate the process of fibrosis. Multiple treatment modalities have been tried with variable success rates. More research is necessary to elucidate the underlying pathophysiological mechanisms which could potentially target the initial steps of fibrosis in these patients. This paper attempts to collate the inferences from the in vivo and in vitro experiments to the clinical observations to understand the pathogenesis of NSF. Schematic representations of receptor-mediated molecular pathways of activation of macrophages and fibroblasts by gadolinium and the final pathway to fibrosis are incorporated in the discussion.

  11. REHABILITACIÓN DE EDIFICIO AFECTADO POR EL TERREMOTO DEL 16 DE ABRIL DE 2016 DE ECUADOR EN LA ULEAM

    Directory of Open Access Journals (Sweden)

    R. Aguiar

    2016-06-01

    Full Text Available Posterior al terremoto del 16 de abril del 2016 en Ecuador, el primer edificio en ser rehabilitado fue la Facultad de Informática del campus de la Universidad Laica Eloy Alfaro de Manabí (ULEAM, el cual tuvo daño moderado en las paredes debido a que no eran completamente rectas, y daño severo en las paredes del descanso de la grada. Para la reconstrucción se reemplazaron dichas paredes que eran de bloque o ladrillo, por paredes con Gypsum livianas y flexibles. En este artículo se presenta el buen comportamiento que tuvieron las paredes de Gypsum, en otras estructuras en contraste con las paredes de bloque o ladrillo, detallándose así mismo, algunos aspectos constructivos sobre la forma correcta de instalarlas. Finalmente, se realizó un análisis espectral de la estructura empleando el espectro del terremoto del 16 de abril de 2016, de magnitud 7.8, obtenido en Manta. En la estructura estudiada, se encontró los desplazamientos laterales y derivas de piso, para asociar éste último valor con la no existencia de daño estructural, y solo daño moderado en la mampostería de las aulas y oficinas y severo en la grada. Posteriormente se realizó un nuevo análisis sísmico considerando la reducción del peso por el cambio del material de las paredes, determinando un mejor comportamiento sísmico. After the earthquake of April 16, 2016 in Ecuador, the first building to be rehabilitated was the Faculty of Computer Science Campus of the University Laica Eloy Alfaro of Manabi (ULEAM, which had moderate damage in the walls due They were not straight and severe damage to the walls of the tier. For Reconstruction these walls were replaced that were made of block or brick with plaster walls flexible and lightweight. In this article it is presented the Good behavior plaster walls had in contrast to the brick or block walls, detailing also, some constructive aspects on the correct way of installing. Finally, UN Spectral analysis of the structure was

  12. Percepciones comunitarias relativas a la prevención del dengue en asentamientos humanos afectados. Lima-Perú, 2015

    Directory of Open Access Journals (Sweden)

    Gloria Carmona

    2016-01-01

    Full Text Available Con el objetivo de describir las percepciones comunitarias para la prevención del dengue en las localidades afectadas de Lima, realizamos un estudio cualitativo a través de entrevistas desde el modelo de creencias en salud. Los entrevistados mencionaron diversos factores de saneamiento, conductas e información que incrementan su exposición al dengue, expresando que los afectaría en aspectos laborales, familiares y económicos, asumiendo parte de la responsabilidad en la prevención y recalcando la labor de los promotores de salud, medios de comunicación y líderes comunitarios. Entre las medidas preventivas mencionadas, se incluyeron medidas sin evidencia de efectividad. Las principales barreras expresadas fueron las siguientes, caraterizadas como insuficientes: cantidad de profesionales de salud, participación comunitaria, organización en las actividades preventivas y discontinuidad luego de los brotes. La discusión y reflexión sobre estos contextos promueve una oportunidad en el abordaje de la labor preventiva hacia el dengue.

  13. RELOCALIZADOS, AFECTADOS Y CONSTRUCCIÓN DE LA DEMANDA REIVINDICATIVA. LA EXPERIENCIA ORGANIZATIVA DE UNA POBLACIÓN DESPLAZADA.

    Directory of Open Access Journals (Sweden)

    Walter Fernando Brites

    2011-12-01

    Full Text Available Este trabajo aborda el caso de un conjunto habitacional de población relocalizada en la periferia urbana segregada de la ciudad de Posadas, Argentina. El análisis gira en torno a cómo el desplazamiento más allá de segregar generó un conjunto de adversidades vinculado a la carencia de servicios y equipamientos básicos, provocando la emergencia de diversas iniciativas colectivas, así como demandas, conflictos y movimientos de pobladores relocalizados. En grandes rasgos, junto con estas acciones emergieron demandas reivindicativas que tomaron formas de protestas y resistencia, pero también hubo instancias más conciliadoras como las mesas de gestión asociada gobierno-vecinos. Las dos formas de acción colectiva ofrecen márgenes para la capacidad de agencia, la participación y alternativas de organización vecinal. En este sentido, adquiere importancia el análisis de hechos y procesos en los que se desenvuelven la participación, los conflictos, las formas de deliberación y las estrategias reivindicativas. 

  14. Intubación submental: experiencia con 30 casos

    OpenAIRE

    Luiz Fernando Lobo Leandro; Henry Arturo García Guevara; Kelly Marinho; Carlos Sanchez Rivero; Mario Alberto Lagunes Lopez

    2015-01-01

    Introducción: Cuando un paciente se encuentra politraumatizado, involucrando fracturas serias en la región maxilofacial, así como en la base del cráneo, se opta por realizar una traqueostomía con el fin de permitir un abordaje simultáneo para los diferentes tercios faciales afectados. La intubación submental ofrece una alternativa a dicho manejo de la vía aérea, significando menor riesgo para el paciente por su seguridad y versatilidad en el manejo de las fracturas nasales y el establecimient...

  15. Cystic fibrosis. Diagnosis.

    Directory of Open Access Journals (Sweden)

    Luis Ortigosa

    2009-11-01

    Full Text Available Cystic fibrosis (CF is one of the most frequent inherited mortal diseases in Caucasian population. Dysfunction in exocrine glands is described in CF patients, with severe pancreatic insufficiency and chronic lung disease. CF is inherited as an autosomal recessive disorder. More than 1000 disease-associated mutations in the cystic fibrosis transmembrane conductance regulator (CFTR gene have been described. DF508 mutation is the most common mutation in the CF gen. Diagnosis in CF is based on clinical and laboratory tests findings. Meconial ileus, CF in other relatives, chronic lung disease, congenital absence of the vas deferens with azoospermia are among other clinical findings, main criteria in CF patients. Two positive results in sweat chloride test , or demonstration in nasal epithelial ionic transport alteration (nasal potential difference and identification of two CF mutations in the patient are laboratory findings in CF.

  16. Adipose tissue fibrosis

    Institute of Scientific and Technical Information of China (English)

    2015-01-01

    The increasing prevalence of obesity causes a majorinterest in white adipose tissue biology. Adipose tissuecells are surrounded by extracellular matrix proteinswhose composition and remodeling is of crucial importancefor cell function. The expansion of adipose tissue inobesity is linked to an inappropriate supply with oxygenand hypoxia development. Subsequent activation ofhypoxia inducible factor 1 (HIF-1) inhibits preadipocytedifferentiation and initiates adipose tissue fibrosis. Therebyadipose tissue growth is limited and excess triglyceridesare stored in ectopic tissues. Stressed adipocytes andhypoxia contribute to immune cell immigration andactivation which further aggravates adipose tissuefibrosis. There is substantial evidence that adipose tissuefibrosis is linked to metabolic dysfunction,both in rodentmodels and in the clinical setting. Peroxisome proliferatoractivated receptor gamma agonists and adiponectin bothreduce adipose tissue fibrosis, inflammation and insulinresistance. Current knowledge suggests that antifibroticdrugs, increasing adipose tissue oxygen supply or HIF-1antagonists will improve adipose tissue function andthereby ameliorate metabolic diseases.

  17. Gadolinium-Induced Fibrosis.

    Science.gov (United States)

    Todd, Derrick J; Kay, Jonathan

    2016-01-01

    Gadolinium-based contrast agents (GBCAs), once believed to be safe for patients with renal disease, have been strongly associated with nephrogenic systemic fibrosis (NSF), a severe systemic fibrosing disorder that predominantly afflicts individuals with advanced renal dysfunction. We provide a historical perspective on the appearance and disappearance of NSF, including its initial recognition as a discrete clinical entity, its association with GBCA exposure, and the data supporting a causative relationship between GBCA exposure and NSF. On the basis of this body of evidence, we propose that the name gadolinium-induced fibrosis (GIF) more accurately reflects the totality of knowledge regarding this disease. Use of high-risk GBCAs, such as formulated gadodiamide, should be avoided in patients with renal disease. Restriction of GBCA use in this population has almost completely eradicated new cases of this debilitating condition. Emerging antifibrotic therapies may be useful for patients who suffer from GIF.

  18. Congenital hepatic fibrosis associated with von Recklinghausen's disease Fibrosis hepática congénita asociada a enfermedad de von Recklinghausen

    Directory of Open Access Journals (Sweden)

    O. A. Jorge

    2006-09-01

    Full Text Available Congenital hepatic fibrosis is characterized by a ductal plate malformation with duct-like structures and fibrosis. It manifests clinically with portal hypertension and may be associated with multiple congenital defects. We present the case of a 16-year-old male with splenomegaly, leukopenia and thrombocytopenia, esophageal varices, and a histopathological diagnosis of congenital hepatic fibrosis. He exhibits "café au lait' spots and "Lisch' nodules, with a diagnosis of von Recklinghausen's disease. Congenital hepatic fibrosis belongs to the so-called fibropolycystic diseases, in which there is a disordered interaction between cells and the extracellular matrix. Von Recklinghausen's disease affects tissues derived from the neural crest and its diagnosis is based on clinical criteria. It is associated with multiple diseases. We describe its association with congenital hepatic fibrosis for the first time.La fibrosis hepática congénita se origina como consecuencia de una malformación de la placa ductal con estructuras tipo ductales acompañadas de fibrosis. Se manifiesta con hipertensión portal y puede asociarse a múltiples defectos congénitos. Presentamos un varón de 16 años con esplenomegalia, leuco- y plaquetopenia, varices esofágicas y diagnóstico histopatológico de fibrosis hepática congénita. La exploración física mostraba la existencia de manchas de "café con leche' y nódulos de "Lisch' con diagnóstico de enfermedad de von Recklinghausen. La fibrosis hepática congénita forma parte de las enfermedades fibropoliquísticas donde existiría una alteración en la interacción entre las células y la matriz extracelular. La enfermedad de von Recklinghausen afecta a los tejidos derivados de la cresta neural y su diagnóstico se basa en criterios clínicos. Se asocia a múltiples patologías. Presentamos por primera vez su asociación con fibrosis hepática congénita.

  19. Molecular Diagnosis of Cystic Fibrosis.

    Science.gov (United States)

    Deignan, Joshua L; Grody, Wayne W

    2016-01-01

    This unit describes a recommended approach to identifying causal genetic variants in an individual suspected of having cystic fibrosis. An introduction to the genetics and clinical presentation of cystic fibrosis is initially presented, followed by a description of the two main strategies used in the molecular diagnosis of cystic fibrosis: (1) an initial targeted variant panel used to detect only the most common cystic fibrosis-causing variants in the CFTR gene, and (2) sequencing of the entire coding region of the CFTR gene to detect additional rare causal CFTR variants. Finally, the unit concludes with a discussion regarding the analytic and clinical validity of these approaches.

  20. Nephrogenic systemic fibrosis

    DEFF Research Database (Denmark)

    Marckmann, Peter

    2008-01-01

    that gadolinium-containing contrast agents used for magnetic resonance imaging have an essential causative role in most, if not all, cases of nephrogenic systemic fibrosis. One particular agent, gadodiamide, caused the majority of cases, but gadopentetate dimeglumine has also been implicated in several cases....... Increasingly poor renal function, aberrations in calcium-phosphate metabolism and erythropoietin treatment seem to increase the risk of the disease and its severity. Up to 25-30% of patients with renal failure exposed to gadolinium-based contrast agents may develop nephrogenic systemic disease. The figure...

  1. [Nephrogenic systemic fibrosis].

    Science.gov (United States)

    Cavallini, L; Abaterusso, C; Bedogna, V; Pertica, N; Loschiavo, C; Lupo, A

    2008-01-01

    Nephrogenic systemic fibrosis (NSF) is a new, rare, and severe disease occurring in patients with renal failure who have been exposed to gadolinium. The pathogenesis of NSF is not completely known. In fact, the first warning about a significant relationship between NSF and gadolinium (a contrast medium used in magnetic resonance imaging) was only issued in 2006. No cases of NSF have been reported in Italy to date. A nationwide investigation should therefore be carried out to assess the real prevalence of NSF within the Italian uremic population. Furthermore, we need guidelines to reduce the risk of NSF in renal patients undergoing MRI with contrast medium.

  2. Management strategies for liver fibrosis.

    Science.gov (United States)

    Altamirano-Barrera, Alejandra; Barranco-Fragoso, Beatriz; Méndez-Sánchez, Nahum

    2017-01-01

    Liver fibrosis resulting from chronic liver injury are major causes of morbidity and mortality worldwide. Among causes of hepatic fibrosis, viral infection is most common (hepatitis B and C). In addition, obesity rates worldwide have accelerated the risk of liver injury due to nonalcoholic fatty liver disease (NAFLD) and nonalcoholic steatohepatitis (NASH). Also liver fibrosis is associated with the consumption of alcohol, or autoimmune hepatitis and chronic cholangiophaties. The response of hepatocytes to inflammation plays a decisive role in the physiopathology of hepatic fibrosis, which involves the recruitment of both pro- and anti-inflammatory cells such as monocytes and macrophages. As well as the production of other cytokines and chemokines, which increase the stimulus of hepatic stellate cells by activating proinflammatory cells. The aim of this review is to identify the therapeutic options available for the treatment of the liver fibrosis, enabling the prevention of progression when is detected in time.

  3. Delimitación de zonas federales y áreas de amortiguamiento en ríos afectados por el crecimiento urbano como estrategia para prevenir inundaciones

    Directory of Open Access Journals (Sweden)

    Adrián Leonardo Ferriño Fierro

    2015-12-01

    Full Text Available En la actualidad, debido al crecimiento de las zonas urbanas y la ocupación de las márgenes de los ríos en las ciudades, vivimos en constante riesgo, invirtiendo enormes cantidades de recursos económicos en reconstrucciones y apoyo a damnificados por las inundaciones. Si bien las inundaciones son eventos naturales que se presentan en todo el mundo, sus consecuencias pueden variar según características de desarrollo histórico, socioeconómicas y culturales de las ciudades, y del respeto de las áreas hidráulicas y zonas de amortiguamiento de sus corrientes de agua. En la presente investigación, se desarrolló una propuesta para determinar áreas de amortiguamiento en ríos afectados por el crecimiento urbano para prevenir inundaciones, basada en aspectos técnicos, legales y ambientales. El caso de estudio fue el río Pesquería dentro de la zona metropolitana de Monterrey al cruzar por los municipios de García, Monterrey, General Escobedo, Apodaca, y Pesquería, Nuevo León.

  4. Virus del papiloma humano asociado con cáncer de cabeza y cuello

    OpenAIRE

    José Francisco Gallegos-Hernández; Eduardo Paredes-Hernández; Rutilio Flores-Díaz; Gabriel Minauro-Muñoz; Teresa Apresa-García; Dulce María Hernández-Hernández

    2007-01-01

    Introducción: El carcinoma epidermoide de cabeza y cuello (CECC) ocupa el sexto lugar de las neoplasias; 400 mil casos fueron diagnosticados en 2005 en todo el mundo; los sitios más afectados son cavidad oral y orofaringe. El alcohol y el tabaco son los factores de riesgo más importantes. Desde 1983 se ha asociado el virus del papiloma humano (VPH) con CECC, principalmente con el originado en orofaringe. El objetivo de la presente investigación fue conocer la prevalencia y tipos de VPH en pac...

  5. LA MATERIA DE MÚSICA EN EL TRATAMIENTO DE ALUMNOS DE SECUNDARIA CON TDAH

    OpenAIRE

    Violero-López, Ana Pilar

    2013-01-01

    El presente estudio, propone la materia de música en Educación Secundaria como alternativa terapéutica para ayudar a los alumnos con TDAH a afrontar este trastorno. El trastorno por déficit de atención e hiperactividad (TDAH), afecta entorno al 3-7% de la población infantil, en su mayoría varones. A pesar de que este porcentaje se encuentra por debajo de otros trastornos infantiles, los expertos calculan que por cada aula hay un alumno afectado con TDAH. Aunque durante la adolesce...

  6. Clinical and Epidemiological Characteristics of Pediatric Patients Affected by Dermatophytosis Características clínico-epidemiológicas de pacientes en edad pediátrica afectados por dermatofitosis

    Directory of Open Access Journals (Sweden)

    Yoan Bernardez Cruz

    2011-12-01

    Full Text Available Background: Although few fungal species are considered to be pathogenic to humans and even when they are commonly less relevant than bacteria or viruses, they are not insignificant as a cause of disease. From a practical point of view, it was found that the mycosis fungoides they cause is one of the first reasons for consultation in our country and the world. Objective: To determine the main clinical and epidemiological features and laboratory findings of pediatric patients affected by ringworm. Methods: A descriptive, case series study was conducted. It included 102 patients with dermatophytosis. The information about these patients was collected from medical records and laboratory results of scraping and cultures of the lesions. Results: We obtained more positives in the test by culture (70.59%, compared to a 66, 7% in the examination by scraping, being Trichophyton rubrum the most common causative agent in the nails and body. The age group from 15 years old on predominated, as well as white skinned and female patients. Most patients were infected through contact with dogs. Conclusions: The percentage of pediatric patients with dermatophytosis is not low. This remains a frequent cause of medical consultation

    Fundamento: a pesar de que pocas especies de hongos son patógenos para los humanos y aun cuando su importancia  es menor que las de las bacterias o los virus, no son insignificantes como causa de enfermedades. Desde el punto de vista práctico se ha comprobado que las micosis que estos causan representan uno de los primeros motivos de consulta dermatológica en nuestro país y el mundo. Objetivo: determinar las principales características clínico-epidemiológicas y de laboratorio de pacientes en edad pediátrica afectados por dermatofitosis. Métodos: estudio descriptivo, de serie de casos

  7. Role of vitamin A in liver fibrosis

    NARCIS (Netherlands)

    Knook, D.L.; Bosma, A.; Seifert, W.F.

    1995-01-01

    The relationship between vitamin A and liver fibrosis was studied with a CCl4-induced fibrosis model in rats. Depending on the time of administration, vitamin A can potentiate or reduce fibrosis: when present during CCl4-treatment parenchymal cell damage and fibrosis were enhanced, whereas vitamin A

  8. Progressive fibrosis of the quadriceps muscle FIBROSIS PROGRESIVA DEL CUÁDRICEPS

    Directory of Open Access Journals (Sweden)

    Enrique Vergara-Amador

    2011-06-01

    Full Text Available Background. Fibrosis of the quadriceps in children is a frequently reported pathology which is associated with antibiotics having been injected into the thigh. This study presents a series of patients having a common background of having had a single pentavalent vaccine dose or injectable vitamin K and presenting progressive fibrosis of the quadriceps muscle. Materials and methods. Seven children were found who had progressive unilateral retraction of the knee. Six of them had a background of a single dose of vaccine having been given when they were four months old, suffering muscle contractions/spasms 4 to 8 weeks after the application; one child had been given vitamin K when born. Results. Magnetic resonance revealed alterations in the intermediate vastus medialis obliquus (VMO without intra-articular pathology. Average age when surgery was performed was 18 months (16-20 month range and preoperational flexion was 26° (20°-30°. The quadriceps central tendon was stretched, without quadriceplasy, flexion being improved to 90° in 1 case and 100° to 120° in the remaining ones. Minimum follow-up lasted six months, average post-operational flexion being 109° (90°-120°. Discussion. The intermediate VMO has been used for applying drugs. There is evidence that this muscular portion has poor perfusion in children. The fibrosis's physiopathology is still not exactly known. Many children are given a poor diagnosis, this being confused with articular pathologies so that they wrongly receive other types of surgery.Antecedentes. La fibrosis del cuádriceps en niños es una patología reportada con frecuencia y asociada a la aplicación de antibióticos en el muslo. Este estudio presenta una serie de pacientes que tenían como antecedente común la aplicación de una dosis única de la vacuna pentavalente o de vitamina K y presentaron fibrosis del cuádriceps progresivo. Material y métodos. Se encontraron siete niños con retracción progresiva

  9. Nephrogenic systemic fibrosis.

    LENUS (Irish Health Repository)

    Kennedy, C

    2010-11-05

    Nephroaenic systemic fibrosis (NSF) is a potentiallv fatal dermatiological condition found exclusively in patients with advanced renal I failure. There is minimal literature regarding the epidemiology and outcomes of patients with NSF in Ireland. A retrospective chart review was performed for all patients with NSF in Ireland. Ireland\\'s experience with the disease was examined in light of international reports. There have been three cases of NSF in Ireland; an area which serves 1915 dialysis patients--giving a point prevalence among Irish end-stage kidney disease patients of 0.002. There was a large variation in disease severity between the three patients. All three patients had significant exposure to gadolinium chelate. Caution with gadolinium administration must be exercised in patients with advanced renal failure.

  10. Epigenetic regulation in cardiac fibrosis

    Institute of Scientific and Technical Information of China (English)

    Li-Ming; Yu; Yong; Xu

    2015-01-01

    Cardiac fibrosis represents an adoptive response in the heart exposed to various stress cues. While resolution of the fibrogenic response heralds normalization of heart function, persistent fibrogenesis is usually associated with progressive loss of heart function and eventually heart failure. Cardiac fibrosis is regulated by a myriad of factors that converge on the transcription of genes encoding extracellular matrix proteins, a process the epigenetic machinery plays a pivotal role. In this minireview, we summarize recent advances regarding the epigenetic regulation of cardiac fibrosis focusing on the role of histone and DNA modifications and non-coding RNAs.

  11. Nutritional Issues in Cystic Fibrosis.

    Science.gov (United States)

    Solomon, Missale; Bozic, Molly; Mascarenhas, Maria R

    2016-03-01

    The importance of maintaining adequate nutrition in patients with cystic fibrosis has been well known for the past 3 decades. Achieving normal growth and maintaining optimal nutrition is associated with improved lung function. Comprehensive and consistent nutritional assessments at regular intervals can identify those at risk of nutritional failure and uncover micronutrient deficiencies contributing to malnutrition. Management of malnutrition in cystic fibrosis should follow a stepwise approach to determine the causes and comorbidities and to develop a nutritional plan. Nutritional management is crucial at every stage in a person's life with cystic fibrosis and remains a cornerstone of management.

  12. Profile of cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Mona M. El-Falaki

    2014-09-01

    Full Text Available It was generally believed that Cystic fibrosis (CF is rare among Arabs; however, the few studies available from Egypt and other Arabic countries suggested the presence of many undiagnosed patients. The aim of the present study was to determine the frequency of CF patients out of the referred cases in a single referral hospital in Egypt. A total of 100 patients clinically suspected of having CF were recruited from the CF clinic of the Allergy and Pulmonology Unit, Children’s Hospital, Cairo University, Egypt, throughout a 2 year period. Sweat chloride testing was done for all patients using the Wescor macroduct system for collection of sweat. Quantitative analysis for chloride was then done by the thiocyanate colorimetric method. Patients positive for sweat chloride (⩾60 mmol/L were tested for the ΔF508 mutation using primer specific PCR for cystic fibrosis transmembrane conductance regulator (CFTR gene. Thirty-six patients (36% had a positive sweat chloride test. The main clinical presentations in patients were chronic cough in 32 (88.9%, failure to thrive in 27 (75%, steatorrhea in 24 (66.7%, and hepatobiliary involvement in 5 (13.9%. Positive consanguinity was reported in 50% of CF patients. Thirty-two patients were screened for ΔF508 mutation. Positive ΔF508 mutation was detected in 22 (68.8% patients, 8 (25% were homozygous, 14 (43.8% were heterozygous, and 10 (31.3% tested were negative. CF was diagnosed in more than third of patients suspected of having the disease on clinical grounds. This high frequency of CF among referred patients indicates that a high index of suspicion and an increasing availability of diagnostic tests lead to the identification of a higher number of affected individuals.

  13. Gadolinio y fibrosis sistémica nefrogénica Gadolinium and nephrogenic systemic fibrosis

    Directory of Open Access Journals (Sweden)

    Claudia Cejas

    2012-06-01

    Full Text Available Inicialmente llamada dermopatía fibrosante nefrogénica, la fibrosis sistémica nefrogénica (FSN está fuertemente ligada a la inyección endovenosa de medios de contraste -basados en gadolinio- en pacientes con insuficiencia renal. En esta revisión corta se analiza la fisiopatología y la clínica, la responsabilidad del radiólogo en la prevención a través de la puesta en práctica de las reglamentaciones vigentes y, por último, se mencionan algunos conceptos sobre el tratamiento de los pacientes con insuficiencia renal crónica que deben exponerse a la inyección de gadolinio.Nephrogenic systemic fibrosis (NSF, initially called nephrogenic fibrosing dermopathy, is strongly linked to the intravenous injection of gadolinium-based contrast agents in patients with renal insufficiency. In this short review, we analyze the pathophysiology and clinical signs and symptoms, the medical responsibility of the radiologist in prevention through the implementation of the existing regulations and, finally, some concepts on the treatment of patients with chronic renal insufficiency who must receive a gadolinium injection.

  14. Osteosarcoma de huesos del pie con alto grado de malignidad

    OpenAIRE

    Alain Martínez Milián; Rafael Ramírez Bichilik

    2015-01-01

    El osteosarcoma es la neoplasia primaria del hueso más frecuente, los hombres son los más afectados en una relación 1,2 - 1,5 por cada mujer y con una tasa de mortalidad cercana a 0,15/100.000 habitantes/año. Se reporta el caso de una paciente femenina de 57 años de edad, con dolor, aumento de volumen e impotencia funcional de aproximadamente seis meses de evolución, los exámenes diagnósticos utilizados fueron radiografía de pie, tomografía axial computarizada de miembro inferior y biopsia de...

  15. Déficit en Habilidades Sociales en Niños con Trastorno por Déficit de Atención- Hiperactividad, Evaluados con la Escala BASC

    Directory of Open Access Journals (Sweden)

    PEDRO PUENTES ROZO

    2014-01-01

    Full Text Available Se analizaron las Habilidades Sociales ( HS de 159 niños de ambos sexos, escolarizados, con Trastorno por Déficit de Atención-Hiperactividad ( TDAH , entre los 6 y los 11 años de edad. La valo - ración de las HS se realizó con la escala Behavioral Assessment System for Children para padres y maestros. Los resultados mostraron que los niños con TDAH presentan puntajes más bajos en las dimensiones de habilidades sociales como escu - char, esperar turnos, reconocer señales sociales y tener capacidad de adaptabilidad. Sin embargo, en compañerismo, los participantes con TDAH combinado poseen habilidades semejantes a los no afectados. Esto replantea lo encontrado en la mayoría de los estudios en donde únicamente se evidencian deficiencias.

  16. Pulmonary fibrosis associated with nabumetone.

    OpenAIRE

    Morice, A.; Atherton, A.; GLEESON, F; Stewart, S.

    1991-01-01

    A patient is described who developed a rapid onset of pulmonary fibrosis following treatment with a new non-steroidal anti-inflammatory drug, nabumetone. Resolution of symptoms, physical signs and radiographic changes followed drug withdrawal and steroid therapy.

  17. Fibrosis quística

    OpenAIRE

    Arturo Solís-Moya; José Pablo Gutiérrez-S

    2003-01-01

    Enfermedades Raras en Asturias. Dirección General de Salud Pública y Participación. Informes breves 09 Cystic fibrosis is a multisystem disease generates the formation and accumulation of viscous mucus that affects everything lungs, digestive system including liver and pancreas. Formerly known as mucoviscidosis or cystic fibrosis pancreas. Este proyecto ha sido financiado a cargo de los fondos para la cohesión territorial 2010 del Ministerio de Sanidad y Política Socia...

  18. Alveolar inflammation in cystic fibrosis

    DEFF Research Database (Denmark)

    Ulrich, Martina; Worlitzsch, Dieter; Viglio, Simona

    2010-01-01

    BACKGROUND: In infected lungs of the cystic fibrosis (CF) patients, opportunistic pathogens and mutated cystic fibrosis transmembrane conductance regulator protein (CFTR) contribute to chronic airway inflammation that is characterized by neutrophil/macrophage infiltration, cytokine release...... accumulated in type II alveolar epithelial cells, lacking CFTR. P. aeruginosa organisms were rarely present in inflamed alveoli. CONCLUSIONS: Chronic inflammation and remodeling is present in alveolar tissues of the CF lung and needs to be addressed by anti-inflammatory therapies....

  19. Cytokines in human lung fibrosis.

    Science.gov (United States)

    Martinet, Y; Menard, O; Vaillant, P; Vignaud, J M; Martinet, N

    1996-01-01

    Fibrosis is a pathological process characterized by the replacement of normal tissue by mesenchymal cells and the extracellular matrix produced by these cells. The sequence of events leading to fibrosis of an organ involves the subsequent processes of injury with inflammation and disruption of the normal tissue architecture, followed by tissue repair with accumulation of mesenchymal cells in the area of derangement. The same sequence of events occurs in wound healing with normal granulation tissue and scar formation, but, while normal scar formation is very localized and transient, in contrast, in fibrosis, the repair process is exaggerated and usually widespread and can be chronic. Inflammatory cells (mainly mononuclear phagocytes), platelets, endothelial cells, and type II pneumocytes play a direct and indirect role in tissue injury and repair. The evaluation of three human fibrotic lung diseases, two diffuse [idiopathic pulmonary fibrosis (IPF), and the adult respiratory distress syndrome (ARDS)], and one focal (tumor stroma in lung cancer), has shown that several cytokines participate to the local injury and inflammatory reaction [interleukin-1 (IL-1), interleukin-8 (IL-8), monocyte chemotactic protein-1 (MCP-1), tumor necrosis factor-alpha (TNF-alpha)], while other cytokines are involved in tissue repair and fibrosis [platelet-derived growth factor (PDGF), insulin-like growth factor-1 (IGF-1), transforming growth factor-beta (TGF-beta), and basic-fibroblast growth factor (b-FGF)]. A better understanding of the cytokines and cytokine networks involved in lung fibrosis leads to the possibility of new therapeutic approaches.

  20. Idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Meltzer, Eric B; Noble, Paul W

    2008-01-01

    Idiopathic pulmonary fibrosis (IPF) is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000) than in women (13.2/100,000). The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock). IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP). The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis), forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational) exposures. IPF is typically progressive and leads to significant disability. The median

  1. Idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Noble Paul W

    2008-03-01

    Full Text Available Abstract Idiopathic pulmonary fibrosis (IPF is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000 than in women (13.2/100,000. The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock. IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP. The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis, forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational exposures. IPF is typically progressive and leads to significant

  2. [Nephrogenic systemic fibrosis].

    Science.gov (United States)

    Artunc, F; Schanz, S; Metze, D; Heyne, N

    2008-01-01

    Nephrogenic systemic fibrosis (NSF) is a novel disease entity, increasingly diagnosed over the last years in patients with renal functional impairment and chronic kidney disease. Recently, gadolinium-containing MR contrast agents have been causally associated with the development NSF. Herein, we present the case of a dialysis-dependent young patient with systemic lupus erythematodes, who developed disabling cutaneous sclerosis of extremities, abdomen and mammae. Clinical and laboratory investigations revealed no signs of activity of the underlying disease. Histopathological examination of a skin biopsy was consistent with NSF showing profound thickening of tissue septae with mucine deposition and slight fibroblast proliferation without inflammatory reaction. Analysis of the patient's medical history revealed that she had undergone repeated contrast enhanced MR scans, including MR angiographies with high doses of gadopentetate. UV phototherapy was little effective, and not until kidney transplantation two years later with good allograft function, improvement of clinical symptoms was observed. Discussion of this case summarizes the current knowledge of clinical features and pathogeneses of NSF, including the role of gadolinium-containing contrast agents. Evolving clinical implications are summarized in the current Tübingen University Hospital guideline for the use of contrast-enhanced MR scans in patients with impaired renal function.

  3. What Are the Signs and Symptoms of Cystic Fibrosis?

    Science.gov (United States)

    ... Twitter. What Are the Signs and Symptoms of Cystic Fibrosis? The signs and symptoms of cystic fibrosis (CF) ... respiratory, digestive, or reproductive systems of the body. Cystic Fibrosis Figure A shows the organs that cystic fibrosis ...

  4. Ordenación de unidades prodcutivas mediante optimización con incertidumbre

    Directory of Open Access Journals (Sweden)

    Sala Garrido, Ramon

    2006-01-01

    Full Text Available En este trabajo mostramos dos maneras de ordenar un conjunto de unidades productivasde acuerdo con su índice de eficiencia cuando los datos que se manejan están afectados de incertidumbre. La eficiencia se analiza con técnicas DEA y, dependiendode la naturaleza de la incertidumbre con la que se trabaja, éstas están basadas en la teoría de conjuntos fuzzy o en el análisis de 81 casos que contemplanlas situaciones representativas. Con estas dos posibilidades mostramos un método que permite la ordenación de números fuzzy y otro basado en el análisis estadístico exhaustivo de casos. Para ilustrar ambas alternativas aplicamos ambos métodos deordenación a una aplicación referenciada en un artículo científico y comparamos los resultados.

  5. ENDOMYOCARDIAL FIBROSIS IN CHINA

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    Purpose.To introduce the epidemical, pathological, and clinical characteristics as well as the diagnostic and therapeutical experiences of endomyocardial fibrosis(EMF) in China. Data sources.A CMBdisc search was done of the Chinese-language literature published from January 1983 through June 1997 about EMF and/or restrictive cardiomyopathy. A manual search was then done for other contributions, including abstracts, between January 1965 and June 1997.Results. Eighty-seven Chinese cases of EMF were collected in this paper. There were 49 men and 38 women, with a mean age of 28±13 years(range, 8 to 68 years). The distribution of the cases is mainly in the south of China. Combined right and left ventricular disease occurs in 48 percent of cases, with pure right ventricular involvement occurring in 42 percent and pure left ventricular involvement in the remaining 10 percent of patients who are examined postmortem. The diagnosis of EMF was confirmed in 21 cases at autopsy, and in 66 cases by echocardiography, angiocardiography, and/or endomyocardial biopsy which showed the characteristic changes. Clinically, right-sided disease is the commonest variety. Endocardiectomy and tricuspid(n=7) or mitral(n=1) valves replacement have been performed in 8 patients. There were 2 operative deaths. Six patients had a satisfactory recovery postoperatively and living well in the follow-up duration. Conclusion.EMF has been diagnosed clinically and confirmed at necropsy in a number of cases in the south of China. The etiology, incidence and epidemiology are still unknown. The pathological and clinical features are similar to those in tropical areas, but right ventricular involvement is the commonest type in our country.

  6. Nephrogenic systemic fibrosis

    Directory of Open Access Journals (Sweden)

    Bhushan Madke

    2011-01-01

    Full Text Available Nephrogenic systemic fibrosis (NSF is a relatively new fibrosing disorder which has caught the attention of various specialities in the past decade. NSF is an extremely disabling and often painful condition, affecting up to 13% of the individuals with chronic kidney disease. The administration of a gadolinium chelate contrast agent has been reported to induce the development of NSF, particularly in patients who have acute or chronic renal disease with a glomerular filtration rate (GFR lower than 30-mL/min/1.73 m 2 and in those with acute renal insufficiency. Mass spectroscopy studies have demonstrated particles of gadolinium in the lesional tissue. The exact pathogenesis of this curious sclerosing condition is unknown. The role of the aberrant targeting of ′circulating fibrocytes′ to the peripheral tissues and viscera has been hypothesized. NSF has distinct clinicopathological features in the setting of renal failure and needs to be looked upon as a new entity on the block. The condition is characterized by irregular indurated plaques, with amoeba-like projections and islands of sparing, chiefly on the trunk and extremities. Flexion contractures of fingers, knees, and elbow joints are known to occur in advanced cases of NSF. The course is frequently associated with painful episodes and loss of ambulation. Histopathology shows haphazard arrangement of thickened bundles of collagen, varying amount of mucin, and increased population of fibroblast-like cells in the dermis. Immunohistochemistry shows increased deposition of type-I procollagen and CD 34+ cells having fibroblastic activity. The condition is refractory to treatment with corticosteroids and immunosuppressive agents. Various modalities of therapy such as UVA1 phototherapy, imatinib mesylate, photodynamic therapy, plasmapheresis, extracorporeal photochemotherapy, and high-dose intravenous immunoglobulin have shown a moderate degree of improvement in skin thickness scores. A prudent

  7. Nuestra Experiencia con la técnica de Barcat y Godart en el Torticolis Muscular Congénito

    OpenAIRE

    1990-01-01

    Los autores exponen los resultados obtenidos en diez casos de T.M.C. tratados quirúrgicamente con la técnica de Barcat y Godard, tanto estéticos como funcionales. Se analizan los parámetros de sexo, edad de intervención, lado afectado, tipo de parto, anomalías asociadas, y presencia de asimetría facial, movilidad craneal, pérdida de relieve muscular y aparición de bandas laterales postoperatorias. Estos resultados son comparados con los otros autores, mostrándose superiores....

  8. Microdureza de caries incipientes artificiales infiltradas con resinas de baja viscosidad antes y después del termociclado

    OpenAIRE

    X. Zamorano Pino; V. Valenzuela Aránguiz; C. Vial Prado; M. Vidal Tardón

    2015-01-01

    Introducción: En Odontología Mínimamente Invasiva, el procedimiento denominado Infiltración de caries, pretende detener la progresión de las caries incipientes mediante el sellado de las microporosidades del esmalte afectado. Este tratamiento se realiza con la aplicación de resinas de baja viscosidad. El objetivo de este trabajo fue evaluar la microdureza superficial de caries incipientes artificiales infiltradas con resinas de baja viscosidad antes y después del proceso de termociclado. Meto...

  9. Using Cystic Fibrosis Therapies for Non-Cystic Fibrosis Bronchiectasis.

    Science.gov (United States)

    ElMaraachli, Wael; Conrad, Douglas J; Wang, Angela C C

    2016-03-01

    Non-cystic fibrosis bronchiectasis (NCFB) is an increasingly prevalent disease that places a significant burden on patients and health systems globally. Although many of the therapies used to treat NCFB were originally developed as cystic fibrosis (CF) therapies, not all of them have been demonstrated to be efficacious in NCFB and some may even be harmful. This article explores the evidence for which therapeutic strategies used to treat CF have been translated into the care of NCFB. The conclusion is that therapies for adult NCFB cannot be simply extrapolated from CF clinical trials, and in some instances, doing so may actually result in harm.

  10. [Laparoscopic treatment of retroperitoneal fibrosis].

    Science.gov (United States)

    Joual, Abdenbi; Rabii, Redouane; El Mejjad, Amine; Fekak, Hamid; Debbagh, Adil; El Mrini, Mohamed

    2004-04-01

    The authors report a case of idiopathic retroperitoneal fibrosis (RPF) in a 38-year-old man presenting with obstructive acute renal failure. The initial management consisted of urinary diversion by bilateral double-J ureteric stenting. After restoration of normal renal function, CT urography demonstrated retroperitoneal fibrosis surrounding the two ureters. Surgical treatment was performed by laparoscopy using four trocars. The operation consisted of detachment of the ascending and descending colon followed by release of the ureters from the lumbar segment to the pelvic segment and finally intraperitonealization of the ureters. The operating time was six hours, the postoperative course was uneventful and the double-J stents were removed at the third week. Laparoscopic treatment of RPF is a treatment option providing all of the benefits of minimally invasive surgery. In the light of this case and a review of the literature, the authors describe the laparoscopic treatment of idiopathic retroperitoneal fibrosis.

  11. Preventive effect of halofuginone on concanavalin A-induced liver fibrosis.

    Directory of Open Access Journals (Sweden)

    Jie Liang

    Full Text Available Halofuginone (HF is an active component of extracts derived from the plant alkaloid febrifugine and has shown therapeutic promise in animal models of fibrotic disease. Our main objectives were to clarify the suppressive effect of HF on concanavalin A (ConA-induced liver fibrosis. ConA injection into the tail vein caused a great increase in the serum aspartate aminotransferase (AST and alanine aminotransferase (ALT levels, while orally administration of HF significantly decreased the levels of the transaminases. In addition, the levels of hyaluronic acid (HA, procollagen III (PCIII and TGF-β1 in the serum and collagen I, α-SMA, tissue inhibitors of metalloproteinase 2 (TIMP2 and Smad3 in the liver tissue were significantly lowered with the treatment of HF. Histological examination also demonstrated that HF significantly reduced the severity of liver fibrosis. Since ConA-induced liver fibrosis is caused by the repeated activation of T cells, immunomodulatory substances might be responsible for the suppressive effect of HF. We found that the production of nuclear factor (NF-kB in the serum was increased in ConA-treated group, while decreased significantly with the treatment of HF. The changes of inflammatory cytokines tumor necrosis factor (TNF-α, IL-6 and IL-1β in the serum followed the same rhythm. All together, our findings indicate that orally administration HF (10ppm would attenuate the liver fibrosis by suppressing the synthesis of collagen I and inflammation-mediated liver injury.

  12. Nephrogenic systemic fibrosis: epidemiology update

    DEFF Research Database (Denmark)

    Marckmann, P.

    2008-01-01

    Purpose of review The aim of this article is to outline the history of nephrogenic systemic fibrosis, a new and serious disease of patients with renal failure, and to give an update on its aetiology and prevalence. Recent findings Epidemiological and histochemical studies demonstrated that gadoli......Purpose of review The aim of this article is to outline the history of nephrogenic systemic fibrosis, a new and serious disease of patients with renal failure, and to give an update on its aetiology and prevalence. Recent findings Epidemiological and histochemical studies demonstrated...

  13. Transient elastography for liver fibrosis diagnosis

    DEFF Research Database (Denmark)

    Andersen, Ellen Sloth; Christensen, Peer Brehm; Weis, Nina

    2009-01-01

    fibrosis. Most of the studies have been conducted on patients with chronic hepatitis but a few studies have also covered fibrosis and cirrhosis due to other etiologies, and they also demonstrate the high sensitivity and specificity. Transient elastography for assessment of fibrosis may turn out...

  14. Cystic Fibrosis (CF): Chloride Sweat Test

    Science.gov (United States)

    ... Old Feeding Your 1- to 2-Year-Old Cystic Fibrosis (CF) Chloride Sweat Test KidsHealth > For Parents > Cystic Fibrosis (CF) Chloride Sweat Test Print A A A ... It Is A chloride sweat test helps diagnose cystic fibrosis (CF) , an inherited disorder that makes kids sick ...

  15. What's it Like to Have Cystic Fibrosis?

    Science.gov (United States)

    ... Emergency Room? What Happens in the Operating Room? Cystic Fibrosis KidsHealth > For Kids > Cystic Fibrosis Print A A A What's in this article? ... with a condition she's known all her life — cystic fibrosis (say: SIS-tik fi-BRO-sus). Her parents ...

  16. OCURRENCIA DE UN TOBAMOVIRUS ASOCIADO CON MANCHAS ANULARES AMARILLAS EN NOPAL TUNERO EN MÉXICO

    OpenAIRE

    Rodolfo De La Torre-Almaraz; Mario Salazar-Segura; Roberto Ruiz-Medrano

    2007-01-01

    Se detectó un virus en cladodios de nopal tunero cultivado en la región de San Martín de las Pirámides, Estado de México, con manchas anulares de color amarillo. Los cladodios afectados desarrollan, en ataques severos, clorosis y marchitez. El virus se transmitió mecánicamente a algunas especies de plantas hospedantes indicadoras, causando lesiones locales cloróticas y después necróticas en las hojas inoculadas; sólo Nicotiana tabacum L. var. White Burley y Capsicum annuum L. desarrollaron ad...

  17. Imagen corporal, funcionamiento sexual, autoestima y optimismo en mujeres con cáncer de mama

    OpenAIRE

    Dehisy Marisol Juárez García; René Landero Hernández

    2011-01-01

    Introducción: La image corporal y el funcionamiento sexual son aspectos afectados por el cáncer de mama, la autoestima y el optimismo son considerados recursos psicologicos que permiten afrontar las situaciones estresantes, por lo que el objetivo de este estudio fue evaluar los niveles y la relación de la imagen corporal, el funcionamiento sexual, la autoestima y el optimismo en un grupo de mujeres con cáncer de mama, así como evaluar las propiedades psicométricas del cuestionario de imagen c...

  18. Mucormicosis yeyunal en paciente con linfoma de Hodgkin Jejunal mucormycosis in a patient with Hodgkin's lymphoma

    OpenAIRE

    B. Madrigal; J. J. Arenal; Torres, A.; M. J. Peñarrubia; A. Vara; Ruiz, M.; Hernández, A.; P. Enríquez

    2008-01-01

    Comunicamos un caso de mucormicosis intestinal en un hombre de 46 años de edad, diagnosticado de enfermedad de Hodgkin clásica, estadio IV-B. Durante la primera fase de la quimioterapia, sufrió una hemorragia digestiva masiva secundaria a una úlcera yeyunal por zigomicosis tipo mucor, diagnosticada por biopsia endoscópica. El paciente fue tratado con antifúngicos y resección quirúrgica del intestino afectado. En la cirugía, se apreció una doble perforación yeyunal cubierta. El estudio anatomo...

  19. Identificación y caracterización de las mutaciones en el gen GAA en pacientes colombianos afectados por la enfermedad de Pompe

    OpenAIRE

    2013-01-01

    La Enfermedad de Pompe (EP) es un desorden metabólico caracterizado por la deficiencia de alfa-glucosidasa acida (GAA), una enzima que cataliza la hidrolisis de las uniones glucosidicas α-1.4 y α-1,6 de glucógeno. Esta deficiencia resulta en acumulación de glucógeno en todos los tejidos, especialmente en musculo esquelético. Los pacientes con EP muestran un gran espectro de fenotipos con respecto a la edad de inicio, progresión de la enfermedad, severidad y tasa de progresión a muerte. El cur...

  20. Sistema de Vigilancia Adaptativo basado en la Coordinación de UAVs en Entornos Afectados por Catástrofes.

    OpenAIRE

    Pérez Ramos, Juan Manuel

    2016-01-01

    En este proyecto se analizan los vehículos aéreos no tripulados (más comúnmente conocidos como drones) como parte fundamental para la ayuda, a los equipos de emergencia, en labores relacionadas con situaciones de catástrofe, bien sea natural o artificial. Las situaciones de desastre pueden ser el escenario ideal para trabajar con drones, debido a las ventajas que proporcionan en este tipo de entornos. Por esta razón, se diseña y desarrolla un sistema adaptativo que permita la c...

  1. Lactate in cystic fibrosis sputum

    DEFF Research Database (Denmark)

    Bensel, Tobias; Stotz, Martin; Borneff-Lipp, Marianne

    2011-01-01

    Antibiotic therapy is thought to improve lung function in patients with cystic fibrosis (CF) by decreasing neutrophil-derived inflammation. We investigated the origin and clinical significance of lactate in the chronically inflamed CF lung. Methods Lactate was measured in sputa of 18 exacerbated ...

  2. Pseudomembranous colitis in cystic fibrosis.

    Science.gov (United States)

    Nagakumar, Prasad

    2013-05-01

    Cystic fibrosis (CF) patients may require frequent courses of antibiotics and repeated hospital admissions. Although children with CF have high carriage rate for C.difficile, they rarely develop colitis. Pseudomembranous colitis is more common in adult post lung transplant CF patients. Although rare, paseudomembranous colitis should be considered in CF patients presenting with abdominal symptoms even in the absence of diarrhoea.

  3. Hepatic fibrosis: Concept to treatment.

    Science.gov (United States)

    Trautwein, Christian; Friedman, Scott L; Schuppan, Detlef; Pinzani, Massimo

    2015-04-01

    Understanding the molecular mechanisms underlying liver fibrogenesis is fundamentally relevant to developing new treatments that are independent of the underlying etiology. The increasing success of antiviral treatments in blocking or reversing the fibrogenic progression of chronic liver disease has unearthed vital information about the natural history of fibrosis regression, and has established important principles and targets for antifibrotic drugs. Although antifibrotic activity has been demonstrated for many compounds in vitro and in animal models, none has been thoroughly validated in the clinic or commercialized as a therapy for fibrosis. In addition, it is likely that combination therapies that affect two or more key pathogenic targets and/or pathways will be needed. To accelerate the preclinical development of these combination therapies, reliable single target validation is necessary, followed by the rational selection and systematic testing of combination approaches. Improved noninvasive tools for the assessment of fibrosis content, fibrogenesis and fibrolysis must accompany in vivo validation in experimental fibrosis models, and especially in clinical trials. The rapidly changing landscape of clinical trial design for liver disease is recognized by regulatory agencies in the United States (FDA) and Western Europe (EMA), who are working together with the broad range of stakeholders to standardize approaches to testing antifibrotic drugs in cohorts of patients with chronic liver diseases.

  4. Cough in idiopathic pulmonary fibrosis

    NARCIS (Netherlands)

    M.J.G. Van Manen (Mirjam J.G.); S.S. Birring (Surinder S.); C. Vancheri (Carlo); V. Cottin (Vincent); Renzoni, E.A. (Elisabetta A.); Russell, A.-M. (Anne-Marie); M.S. Wijsenbeek (Marlies)

    2016-01-01

    textabstractMany patients with idiopathic pulmonary fibrosis (IPF) complain of chronic refractory cough. Chronic cough is a distressing and disabling symptom with a major impact on quality of life. During recent years, progress has been made in gaining insight into the pathogenesis of cough in IPF,

  5. Lung transplantation for cystic fibrosis

    NARCIS (Netherlands)

    Adler, Frederick R; Aurora, Paul; Barker, David H; Barr, Mark L; Blackwell, Laura S; Bosma, Otto H; Brown, Samuel; Cox, D R; Jensen, Judy L; Kurland, Geoffrey; Nossent, George D; Quittner, Alexandra L; Robinson, Walter M; Romero, Sandy L; Spencer, Helen; Sweet, Stuart C; van der Bij, Wim; Vermeulen, J; Verschuuren, Erik A M; Vrijlandt, Elianne J L E; Walsh, William; Woo, Marlyn S; Liou, Theodore G

    2009-01-01

    Lung transplantation is a complex, high-risk, potentially life-saving therapy for the end-stage lung disease of cystic fibrosis (CF). The decision to pursue transplantation involves comparing the likelihood of survival with and without transplantation as well as assessing the effect of wait-listing

  6. Update on nephrogenic systemic fibrosis

    DEFF Research Database (Denmark)

    Marckmann, P.; Logager, V.B.; Thomsen, Henrik S.

    2008-01-01

    Gadolinium-based contrast agents were for many years considered safe, but this is no longer the case. The least stable agents may trigger the development of nephrogenic systemic fibrosis (NSF), a generalized fibrotic disorder, in renal failure patients. The use of gadodiamide and gadopentetate di...

  7. Estado nutricional en niños con parálisis cerebral infantil

    OpenAIRE

    Drianet Castillo Peña; Orelvis Pérez Duerto; Alicia Velázquez Pérez

    2014-01-01

    Se realizó un estudio descriptivo, con el objetivo de caracterizar el estado nutricional de los niños que ingresaron en el Hospital Pediátrico Provincial “Mártires de las Tunas”con parálisis cerebral, en el periodo comprendido de enero a diciembre de 2012. La muestra estuvo integrada por 25 niños. La evaluación nutricional se hizo según las tablas cubanas. Se observó que predominó el sexo masculino, siendo más afectado el grupo de mayores de siete años, prevaleció la desnutrición en niños con...

  8. Estado nutricional en niños con parálisis cerebral infantil

    Directory of Open Access Journals (Sweden)

    Drianet Castillo Peña

    2014-08-01

    Full Text Available Se realizó un estudio descriptivo, con el objetivo de caracterizar el estado nutricional de los niños que ingresaron en el Hospital Pediátrico Provincial “Mártires de las Tunas”con parálisis cerebral, en el periodo comprendido de enero a diciembre de 2012. La muestra estuvo integrada por 25 niños. La evaluación nutricional se hizo según las tablas cubanas. Se observó que predominó el sexo masculino, siendo más afectado el grupo de mayores de siete años, prevaleció la desnutrición en niños con parálisis cerebral severa. Los déficits nutricionales más frecuentes fueron la anemia, seguido de la hipoalbuminemia

  9. Simulación de los shocks estructurales y coyunturales del sector turístico ern un destino canario afectado por estancamiento y declive

    Directory of Open Access Journals (Sweden)

    González Rodríguez, José Manuel

    2011-01-01

    Full Text Available En este artículo modelizamos la tendencia de la oferta turística en un área de la isla de Tenerife, en las Islas Canarias, cuyo crecimiento en los últimos años ha sido afectada por la conocida como fase de declive según la terminología de Butler. Utilizamos una versión modificada del “Point Matching Method” para resolver el problema deidentificación de parámetros en las ecuaciones logísticas y hemos obtenido una nueva versión de la ecuación de Bass que nos simula la tendencia real de la citada variable. Tal metodología se corresponde con la particular evolución de los diferentes destinos de atracción turística en el Archipiélago, que han completado sus ciclos de vida a despecho de intereses exógenos, asociados con la “particular” vinculación histórica de Canarias con la conocida Expansión Atlántica de ciertos países europeos. En concreto, basándonos en estudios previos podemos afrontar nuestro estudio al modo de la Teoría de la Lucha por Recursos Escasos, tal como fuera formulada por Vito Volterra. Por lo demás, los shocks de etiología coyuntural han sido enfrentados con un tratamiento de carácter estocástico, que nos ha permitido diferenciar la estructura epistemológica inicial de aquellos efectos puntuales, que habremos de asignar a la coyuntura económica de los países emisores de visitantes a nuestras Islas

  10. Pirfenidona, un nuevo fármaco para el tratamiento de la fibrosis pulmonar idiopática

    OpenAIRE

    Velando Crespo, Alejandro; Sánchez Morales, Santiago

    2013-01-01

    Pirfenidona es un fármaco antifibrótico, con propiedades antiinflamatorias. Recientemente se ha demostrado que reduce el declive de la función pulmonar en los pacientes con Fibrosis Pulmonar Idiopática (FPI) y retarda la progresión de la enfermedad. Aunque no se conoce bien el mecanismo de acción en humanos, más de 40 publicaciones preclínicas describen el mecanismo de acción antifibrótico y antiinflamatorio de Pirfenidona, y en modelos animales se ha demostrado que reduce la fibrosis enpulmó...

  11. [Mechanism of and Therapy for Kidney Fibrosis].

    Science.gov (United States)

    Kuma, Akihiro; Tamura, Masahito; Otsuji, Yutaka

    2016-03-01

    Fibrosis occurs in systemic tissues other than the brain and finally induces dysfunction of the fibrotic organ. Kidney fibrosis is related to scarring after acute kidney injury and the progression of chronic kidney disease. Kidney function decreases with the progression of kidney fibrosis. As fibrotic tissue cannot return to its original status, advanced kidney fibrosis requires the administration of dialysis or kidney transplantation. Thus, elucidation the mechanism of kidney fibrosis is an important research theme. The proliferation and activation of (myo) fibroblasts and the excessive production of an extracellular matrix are common mechanisms in fibrosis in many organs, but it seems that kidney fibrosis has specific pathways. Tubular epithelial, mesangial cells, and erythropoietin producing cells, which exist only in the kidney, participate in forming kidney fibrosis. This review highlights an understanding of the cells and their underlying mechanisms, which are specific to kidney fibrosis process: transforming growth factor-β (TGF-β), epithelial-mesenchymal transition, wingless/int-1 (WNT) signaling, renal anemia, and uremia. Finally, we describe potential therapies that focus on the mechanisms of kidney fibrosis: anti-TGF-β antibody and mammalian target of rapamycin (mTOR).

  12. Eventos adversos en 1395 infusiones con diferentes preparados de gammaglobulina intravenosa

    Directory of Open Access Journals (Sweden)

    Alejandro Malbrán

    2013-10-01

    Full Text Available Los procesos de aislamiento y esterilización de la gammaglobulina endovenosa (IVIG afectan las características del producto terminado y, por lo tanto, su tolerabilidad. Distintos productos tienen diferentes incidencias de reacciones adversas. Este trabajo cuantifica los eventos adversos (EA inmediatos provocados por distintas preparaciones de IVIG. Analizamos 1395 infusiones en 28 pacientes, con una mediana de 32.5 por sujeto (rango 2-214, utilizando seis preparados distintos de IVIG, con una dosis total promedio de 40.3 ± 8.3 g. Analizamos retrospectivamente 1 031 infusiones y 364 prospectivamente. Los pacientes utilizaron una media de 2.68 ± 1.8 IVIG diferentes, con una mediana de 2 (rango 1-6 por persona. El número de marcas comerciales utilizadas se relacionó con el número de infusiones recibidas, r = 0.73. En 24 (2.3% de 1031 infusiones analizadas en forma retrospectiva se registraron EA que afectaron a 11 de los 23 casos incluidos, con una media de 2.18 ± 1.08 EA por afectado. De 24 pacientes y de 364 infusiones prospectivas, en 14 pacientes y en 32 (7.2% procedimientos se observaron EA. Veinticuatro (42.9% de 56 EA fueron leves, 31 (55.5% moderados y uno (1.8% fue grave. La velocidad de infusión fue de 9.04 ± 4.6 g/h para las que presentaron EA vs. 10.6 ± 4.6 g/h para las que no (p = 0.31. La incidencia, la gravedad y la proporción de pacientes afectados con EA para cada marca comercial de IVIG fueron muy diferentes entre sí. Esta información debe ser tomada en cuenta en el momento de selección de la IVIG a utilizar.

  13. Nutrición, fibrosis quística y aparato digestivo Nutrition, cystic fibrosis and the digestive tract

    Directory of Open Access Journals (Sweden)

    Gabriel Olveira

    2008-05-01

    Full Text Available La prevalencia de desnutrición en fibrosis quística es elevada aunque variable según los estudios. La detección de la misma debe realizarse mediante la combinación de diferentes métodos, en función de la disponibilidad de los mismos. No obstante, el criterio más sencillo y validado es medir en cada visita el peso (y la talla en niños para calcular el índice de masa corporal y clasificar la desnutrición en criterios absolutos; en adultos, con valores menores a 18,5 kg/m² y en niños, con percentiles del índice de masa corporal menores a 10. El empeoramiento del estado de nutrición guarda una relación directa con el descenso de los parámetros de función pulmonar y se ha propuesto como un factor predictor de morbilidad e incluso de mortalidad en personas con fibrosis quística, independientemente del grado de disfunción pulmonar. La insuficiencia pancreática exocrina está presente en aproximadamente el 70 al 90% de los pacientes con fibrosis quística y la correlación entre genotipo y fenotipo es alta. La mayoría de los pacientes con insuficiencia pancreática exocrina toleran una dieta alta en grasa si son tratados con enzimas pancreáticas a las dosis adecuadas. La prevalencia de diabetes aumenta con la edad alcanzando hasta el 40% de los casos en pacientes mayores de 30 años. La afectación hepática clínica es menos prevalente (aproximadamente afecta a 1/3 de los pacientes. Otras complicaciones intestinales como el íleo meconial, el reflujo gastroesofágico, la obstrucción del intestino distal o la colopatía fibrosante pueden condicionar también malnutrición. En los pacientes con fibrosis quística se recomienda que la ingesta habitual aporte entre el 120 y 150% de las calorías recomendadas y que contenga alto contenido en grasas. Si no consiguen alcanzar o mantener los objetivos nutricionales previstos con las modificaciones de la dieta, se pueden adicionar suplementos artificiales, si bien la recomendación de su

  14. Epidemiology of idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Ley B

    2013-11-01

    Full Text Available Brett Ley, Harold R Collard Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California San Francisco, San Francisco, California, USA Abstract: Idiopathic pulmonary fibrosis is a chronic fibrotic lung disease of unknown cause that occurs in adults and has a poor prognosis. Its epidemiology has been difficult to study because of its rarity and evolution in diagnostic and coding practices. Though uncommon, it is likely underappreciated both in terms of its occurrence (ie, incidence, prevalence and public health impact (ie, health care costs and resource utilization. Incidence and mortality appear to be on the rise, and prevalence is expected to increase with the aging population. Potential risk factors include occupational and environmental exposures, tobacco smoking, gastroesophageal reflux, and genetic factors. An accurate understanding of its epidemiology is important, especially as novel therapies are emerging. Keywords: idiopathic pulmonary fibrosis, epidemiology, incidence, prevalence, mortality, risk factors

  15. Idiopathic pulmonary fibrosis: treatment update.

    LENUS (Irish Health Repository)

    O'Connell, Oisin J

    2011-11-01

    Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. Despite multiple recent clinical trials, there is no strong evidence supporting a survival advantage for any agent in the management of patients with IPF. The limited effectiveness of current treatment regimes has led to a search for novel therapies including antifibrotic strategies. This article reviews the evidence supporting the treatments currently used in the management of IPF.

  16. Oral submucous fibrosis: an update

    Directory of Open Access Journals (Sweden)

    Wollina U

    2015-04-01

    Full Text Available Uwe Wollina,1 Shyam B Verma,2 Fareedi Mukram Ali,3 Kishor Patil4 1Department of Dermatology and Allergology, Academic Teaching Hospital Dresden-Friedrichstadt, Dresden, Germany; 2Nirvana Skin Clinic, Vadodara, Gujarat, India; 3Departments of Oral and Maxillofacial Surgery, SMBT Dental College, Sangamner, Maharashtra, India; 4Departments of Oral Pathology and Microbiology, SMBT Dental College, Sangamner, Maharashtra, India Abstract: Oral submucous fibrosis (OSF is a premalignant condition caused by betel chewing. It is very common in Southeast Asia but has started to spread to Europe and North America. OSF can lead to squamous cell carcinoma, a risk that is further increased by concomitant tobacco consumption. OSF is a diagnosis based on clinical symptoms and confirmation by histopathology. Hypovascularity leading to blanching of the oral mucosa, staining of teeth and gingiva, and trismus are major symptoms. Major constituents of betel quid are arecoline from betel nuts and copper, which are responsible for fibroblast dysfunction and fibrosis. A variety of extracellular and intracellular signaling pathways might be involved. Treatment of OSF is difficult, as not many large, randomized controlled trials have been conducted. The principal actions of drug therapy include antifibrotic, anti-inflammatory, and antioxygen radical mechanisms. Potential new drugs are on the horizon. Surgery may be necessary in advanced cases of trismus. Prevention is most important, as no healing can be achieved with available treatments. Keywords: betel nut, betel quid, oral disease, squamous cell carcinoma, tobacco, fibrosis

  17. Puesta en evidencia del virus diarrea viral bovina en bovinos clínicamente afectados Isolation of the bovine viral diarrhoea virus from tissue of clinically affected cattle

    Directory of Open Access Journals (Sweden)

    M.O CELEDON

    1997-01-01

    Full Text Available Para conocer la presencia del virus diarrea viral bovina (VDVB en animales sospechosos de estar cursando un cuadro clínico provocado por este virus, se trabajó con un total de 33 animales, correspondiendo a 23 fetos abortados, 2 mortinatos, un nonato, 3 vacas: una madre de mortinato, una madre de aborto y una muerta, 2 novillos muertos y 2 terneros muertos. Muestras de órganos se inocularon en cultivos primarios de pulmón fetal bovino (PFB y en la línea MDBK. Después del primer pasaje en células de PFB, se detectó la presencia de antígenos del VDVB por la prueba de inmunoperoxidasa indirecta (IPI. Todas las muestras con reacción positiva a IPI se inocularon por segunda y tercera vez en células de PFB, aplicándose la prueba de IPI en el tercer pasaje. Sobre un cuarto pasaje se aplicó la prueba de inmunofluorescencia direccta (IFD. Todas las muestras, positivas y negativas a IPI, se inocularon en 3 pasajes seriados en las células MDBK. En 23 de los 33 animales se aisló VDVB cepas no citopatogénicas (NCP, correspondiendo a 14 fetos abortados, un nonato, un mortinato, 3 vacas, 2 novillos y 2 terneros. En 6 fetos abortados, independiente de los infectados con el VDVB, se aisló el virus de la rinotraqueítis infecciosa bovina (RIB. Se concluye que la presencia del VDVB es de alta frecuencia en muestras clínicas de ganado bovino con patologías asociables al VDVB, desconociéndose el rol patógeno del virus en estos aislados.Cattle infected with the bovine viral diarrhoea (BVD virus can present a variety of clinical signs. This research studied the presence of BVD virus in cattle by virus isolation in primary cell cultures of bovine embryo lungs. Virus identification was done using the immunoperoxidase staining assay and the direct fluorescent antibody staining. As a result, 23 out of 33 animals were identified as positive to BVD virus: 14 foetal abortions, 2 stillbirths, 3 dams, 2 steers and 2 calves. No cytopathogenic isolates were

  18. Cystic fibrosis from the gastroenterologist's perspective.

    Science.gov (United States)

    Ooi, Chee Y; Durie, Peter R

    2016-03-01

    Cystic fibrosis is a life-limiting, recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Increased survival outcomes and the multisystem nature of the disease, including the involvement of hepatobiliary and gastrointestinal tracts, now require the need for more extensive knowledge and expertise in cystic fibrosis among gastroenterologists. Manifestations are either a direct consequence of the primary defect in cystic fibrosis or a secondary complication of the disease or therapy. Adult patients with cystic fibrosis also have an increased risk of malignancy in the gastrointestinal and pancreatico-biliary tracts compared with the general population. Novel treatments that target the basic defects in the CFTR protein have emerged, but to date not much is known about their effects on the gastrointestinal and hepatobiliary systems. The introduction of such therapies has provided new opportunities for the application of intestinal endpoints in clinical trials and the understanding of underlying disease mechanisms that affect the gut in cystic fibrosis.

  19. Fibrosis quística: aumento de la sobrevida en un centro especializado a 10 años de seguimiento

    OpenAIRE

    Lentini, Eduardo; López, Adriana; Lores, Ana María; Pesciullesi, María Rosa; Ituarte, Leonor María; Viera, Teresa Beatriz

    2014-01-01

    La Fibrosis Quística (FQ), la enfermedad genética más frecuente de la raza blanca, es una patología multisistémica y como tal requiere un abordaje multidisciplinario coordinado para obtener mejores resultados de sobrevida y calidad de vida. Con este propósito se organizó un Centro de Fibrosis Quística (CDFQ) en Mendoza, Argentina, y se diseñó este estudio con el objetivo de evaluar la hipótesis de que la organización y tratamiento de la FQ de acuerdo con normas internacionales, mejora la sobr...

  20. Orientación estratégica y desempeño de negocios artesanales afectados por crisis en Oaxaca, México

    Directory of Open Access Journals (Sweden)

    Luis Mendoza Ramírez

    2014-01-01

    orientación estratégica (OE sobre el desempeño de las empresas en ambientes hostiles; no obstante, los resultados son de empresas grandes de países desarrollados; en circunstancias de estabilidad económica y social, donde el ambiente hostil se debe a factores tecnológicos, industriales o de mercado. El objetivo de este estudio es analizar el desempeño de negocios artesanales como resultado de la OE de los dueños, en un marco de crisis de deterioro económico, inestabilidad social y cambio climático en Oaxaca, México. Con base en los datos de 101 negocios artesanales, analiza dos con correlación bivariada de Pearson y regresión lineal múltiple, los resultados del estudio indican un efecto positivo de innovación y proactividad sobre el desempeño. En contra de lo esperado, se encuentra un efecto negativo de agresividad competitiva sobre el desempeño. Se concluye que, aunque los dueños de negocios artesanales se comprometen en tres dimensiones de OE para mejorar el desempeño, no todas ellas tienen efecto, ni logran modificarlo de la misma manera, debido a las condiciones contextuales.

  1. HIV Infection Accelerates Hepatitis C-Related Liver Fibrosis

    Science.gov (United States)

    ... HIV Infection Accelerates Hepatitis C–Related Liver Fibrosis HIV Infection Accelerates Hepatitis C–Related Liver Fibrosis Email ... the progression of other chronic diseases as well. HIV and Fibrosis Dr. Kirk and his team tapped ...

  2. The Sociology and Entrenchment. A Cystic Fibrosis Test for Everyone?

    DEFF Research Database (Denmark)

    Koch, Lene; Stemerding, Dirk

    1994-01-01

    Socialmedicine, genetic screening, cystic fibrosis, ethics, political regulation, sociology of technology......Socialmedicine, genetic screening, cystic fibrosis, ethics, political regulation, sociology of technology...

  3. Experiencia del Instituto de Ortopedia Infantil Roosevelt en el manejo de pacientes con pseudoartrosis congenita de tibia

    OpenAIRE

    Cortes Buitrago, Viviana Ines

    2010-01-01

    La pseudoartrosis congénita de la tibia es una enfermedad de muy baja incidencia, pero con gran morbilidad en el paciente afectado por la misma. Es un reto para el ortopedista infantil porque no existe un consenso sobre su etiología y tratamiento. La dificultad en el manejo, esta en obtener y mantener unión o consolidación y simultáneamente proveer una extremidad funcional. El Propósito de este estudio es describir los resultados en el manejo de esta patología en el Instituto de ortopedia Inf...

  4. Salud autopercibida por los cuidadores familiares de pacientes con Alzheimer. Una revisión de la literatura

    OpenAIRE

    Fructuoso Llinares, Guillermo

    2016-01-01

    En la actualidad, el creciente aumento de la esperanza de vida ha traído con sigo el envejecimiento progresivo de la población, lo que se traduce en una aumento de las enfermedades neurodegenerativas asociadas a la vejez, principalmente la enfermedad de Alzheimer. Dicha enfermedad es la forma más prevalente de demencia, de hecho, las estadísticas apuntan a que en 2050 el número de afectados mundiales podría rondar los 100 millones. El Alzheimer es una enfermedad degenerativa de afectación neu...

  5. Recent concepts on cystic fibrosis Fibrosis quística

    Directory of Open Access Journals (Sweden)

    William Parra

    1991-01-01

    Full Text Available

    Up to date concepts on Cystic Fibrosis are summarized in this article; the following are included: Incidence, pathogenesis, prenatal and postnatal diagnosis, treatment, genetics and future perspectives. Criteria are given for suspecting the disease and emphasis is made on the fact that its frequency in Colombia may be higher than usually thought.

    En este artículo se actualizan algunos conceptos sobre la Fibrosis Quística y su manejo; se incluyen los siguientes: incidencia, patogénesis, bases para el diagnóstico incluyendo el prenatal, tratamiento, aspectos gen éticos y perspectivas futuras. Se hace énfasis en la necesidad de pensar más a menudo en la enfermedad pues su frecuencia en Colombia puede ser mayor de lo que usualmente se acepta.

  6. Endocrine Disorders in Cystic Fibrosis.

    Science.gov (United States)

    Blackman, Scott M; Tangpricha, Vin

    2016-08-01

    Cystic fibrosis is frequently complicated by endocrine disorders. Diabetes can be expected to affect most with CF and pancreatic insufficiency and varies widely in age of onset, but early identification and treatment improve morbidity and mortality. Short stature can be exacerbated by relative delay of puberty and by use of inhaled corticosteroids. Bone disease in CF causes fragility fractures and should be assessed by monitoring bone mineral density and optimizing vitamin D status. Detecting and managing endocrine complications in CF can reduce morbidity and mortality in CF. These complications can be expected to become more common as the CF population ages.

  7. Outcome in cystic fibrosis liver disease.

    LENUS (Irish Health Repository)

    Rowland, Marion

    2011-01-01

    Evidence suggests that cystic fibrosis liver disease (CFLD) does not affect mortality or morbidity in patients with cystic fibrosis (CF). The importance of gender and age in outcome in CF makes selection of an appropriate comparison group central to the interpretation of any differences in mortality and morbidity in patients with CFLD.

  8. Self-management education for cystic fibrosis.

    LENUS (Irish Health Repository)

    Savage, Eileen

    2011-01-01

    Self-management education may help patients with cystic fibrosis and their families to choose, monitor and adjust treatment requirements for their illness, and also to manage the effects of illness on their lives. Although self-management education interventions have been developed for cystic fibrosis, no previous systematic review of the evidence of effectiveness of these interventions has been conducted.

  9. HOME CARE IN CYSTIC-FIBROSIS PATIENTS

    NARCIS (Netherlands)

    VANAALDEREN, WMC; MANNES, GPM; BOSMA, ES; ROORDA, RJ; HEYMANS, HSA

    1995-01-01

    Intravenous antibiotics and enteral tube feeding at home for the treatment of pulmonary exacerbations and underweight condition in cystic fibrosis (CF) patients have become tools that are used in many cystic fibrosis centres, The experience with home care programmes from different countries is quite

  10. Laparoscopic cholecystectomy in adult cystic fibrosis.

    LENUS (Irish Health Repository)

    McGrath, D S

    2012-02-03

    Two female patients with Cystic Fibrosis, attending the Adult Regional Cystic Fibrosis centre at the Cork University Hospital, were investigated for upper abdominal pain and found to have gallstones at ultrasonography. Laparoscopic cholecystectomy was performed successfully and, without complication, in both patients.

  11. European Cystic Fibrosis Society Standards of Care

    DEFF Research Database (Denmark)

    Stern, Martin; Bertrand, Dominique Pougheon; Bignamini, Elisabetta;

    2014-01-01

    Since the earliest days of cystic fibrosis (CF) treatment, patient data have been recorded and reviewed in order to identify the factors that lead to more favourable outcomes. Large data repositories, such as the US Cystic Fibrosis Registry, which was established in the 1960s, enabled successful...

  12. Diferencias según sexo en el tratamiento y la evolución de los pacientes afectados de síndrome coronario agudo

    Directory of Open Access Journals (Sweden)

    M. Ferraz-Torres

    2014-08-01

    Full Text Available Fundamento: La perspectiva de género en salud nos alerta de la diferente prevalencia, incidencia, evolución y letalidad de las patologías coronarias agudas según sexo. Este estudio pretende conocer la diferencia en el tratamiento y la evolución de los pacientes afectos de Síndrome Coronario Agudo (SCA según sexos en Navarra. Métodos: Se analizaron 35 variables de 130 usuarios que acudieron consecutivamente al servicio de Urgencias (SU del Complejo Hospitalario de Navarra (CHN con patología coronaria aguda desde enero hasta abril de 2012. La variable dependiente fue el sexo y las independientes los tiempos, tratamientos y evolución final del proceso. Resultados: Un 74,6% de la muestra fueron varones con una edad media de 67 años, inferior a los 72 años de la muestra femenina (p=0,043. Se obtuvo una mediana de 3 factores de riesgo cardiovascular (FRC en los hombres y de dos en las mujeres (p=0,026. El tiempo de demora generado por los pacientes fue de 161 minutos en varones vs 266 minutos en féminas (p=0,006. El tratamiento llevado a cabo mediante revascularización por angioplastia primaria (AP o fibrinolisis se realizó en un 71,6% de los hombres y un 41,2% de las mujeres (p=0,002. Se registró un 5,9% de muertes en mujeres, sin hallarse casos de fallecimiento en varones (p=0,017. Conclusiones: En Navarra, los procesos coronarios siguen siendo una patología de predominio masculino pero de mayor gravedad en mujeres. El tratamiento se realizó de forma distinta según sexo. Se observó un mayor retraso en la solicitud de atención sanitaria en las mujeres así como la presencia de alta voluntaria en ellas, lo que puede influir en la peor evolución de las mismas.

  13. [Non-invasive assessment of liver fibrosis].

    Science.gov (United States)

    Cohen-Ezra, Oranit; Ben-Ari, Ziv

    2015-03-01

    Chronic liver diseases represent a major public health problem, accounting for significant morbidity and mortality worldwide. Prognosis and management of chronic liver diseases depend on the amount of liver fibrosis. Liver biopsy has long remained the gold standard for assessment of liver fibrosis. Liver biopsy is an invasive procedure with associated morbidity, it is rarely the cause for mortality, and has a few limitations. During the past two decades, in an attempt to overcome the limitations of liver biopsy, non-invasive methods for the evaluation of liver fibrosis have been developed, mainly in the field of viral hepatitis. This review will focus on different methods available for non-invasive evaluation of liver fibrosis including a biological approach which quantifies serum levels of biomarkers of fibrosis and physical techniques which measure liver stiffness by transient elastography, ultrasound or magnetic resonance based elastography, their accuracy, advantages and disadvantages.

  14. Postinjection Muscle Fibrosis from Lupron

    Directory of Open Access Journals (Sweden)

    Erica Everest

    2015-01-01

    Full Text Available We describe the case of a 6.5-year-old girl with central precocious puberty (CPP, which signifies the onset of secondary sexual characteristics before the age of eight in females and the age of nine in males as a result of stimulation of the hypothalamic-pituitary-gonadal axis. Her case is likely related to her adoption, as children who are adopted internationally have much higher rates of CPP. She had left breast development at Tanner Stage 2, adult body odor, and mildly advanced bone age. In order to halt puberty and maximize adult height, she was prescribed a gonadotropin releasing hormone analog, the first line treatment for CPP. She was administered Lupron (leuprolide acetate Depot-Ped (3 months intramuscularly. After her second injection, she developed swelling and muscle pain at the injection site on her right thigh. She also reported an impaired ability to walk. She was diagnosed with muscle fibrosis. This is the first reported case of muscle fibrosis resulting from Lupron injection.

  15. Cystic Fibrosis: Brazilian ENT Experience

    Directory of Open Access Journals (Sweden)

    Tania Sih

    2012-01-01

    Full Text Available Most published studies about Cystic Fibrosis (CF are European or North American. There are still few publications about the characteristics of fibrocystic populations in developing countries. The incidence of cystic fibrosis (CF in Brazil varies among different regions (1 : 10,000 in Minas Gerais, 1 : 9,500 in Paraná, 1 : 8,700 in Santa Catarina, and 1 : 1600 in Rio Grande do Sul. The prevalence of the DF508 mutation also varies according to population: 33% in Sao Paulo, 49% in Rio Grande do Sul, 27% in Santa Catarina, and 52% in Minas Gerais. Cough and nasal obstruction are the most common symptoms. The variation in nasal polyposis prevalence may be explained by population genotypic characteristics in a country that spans a continent. Findings on nasal endoscopy and computed tomography (CT have better correlation than do this information compared with surgical and clinical history. Microbiologic studies suggest a high level of early contamination of the airways. Sensorineural hearing loss (SNHL occurs in these patients as a result of ototoxic antibiotics. The data compiled in this paper is useful, but also lead to the general agreement that more research would be welcome due to the unique characteristics of this country.

  16. Heart involvement in cystic fibrosis: A specific cystic fibrosis-related myocardial changes?

    Science.gov (United States)

    Labombarda, Fabien; Saloux, Eric; Brouard, Jacques; Bergot, Emmanuel; Milliez, Paul

    2016-09-01

    Cystic fibrosis is a complex multi-systemic chronic disease characterized by progressive organ dysfunction with development of fibrosis, possibly affecting the heart. Over the last four decades pathological, experimental, and clinical evidence points towards the existence of a specific myocardial involvement in cystic fibrosis. Multi-modality cardiac imaging, especially recent echocardiographic techniques, evidenced diastolic and/or systolic ventricular dysfunction in cystic fibrosis leading to the concept of a cystic fibrosis-related cardiomyopathy. Hypoxemia and inflammation are among the most important factors for heart involvement in cystic fibrosis. Cystic Fibrosis Transmembrane Regulator was found to be involved in the regulation of cardiomyocyte contraction and may also account for cystic fibrosis-related myocardial dysfunction. This review, mainly focused on echocardiographic studies, seeks to synthesize the existing literature for and against the existence of heart involvement in cystic fibrosis, its mechanisms and prognostic implications. Careful investigation of the heart function may be helpful for risk stratification and therapeutic decisions in patients with cystic fibrosis.

  17. Beyond TGFβ : Novel ways to target airway and parenchymal fibrosis

    NARCIS (Netherlands)

    Boorsma, C. E.; Dekkers, B. G. J.; van Dijk, E. M.; Kumawat, K.; Richardson, J.; Burgess, J.K.; John, A. E.

    2014-01-01

    Within the lungs, fibrosis can affect both the parenchyma and the airways. Fibrosis is a hallmark pathological change in the parenchyma in patients with idiopathic pulmonary fibrosis (IPF), whilst in asthma or chronic obstructive pulmonary disease (COPD) fibrosis is a component of the remodelling of

  18. Matrix Metalloproteinases-7 and Kidney Fibrosis

    Science.gov (United States)

    Ke, Ben; Fan, Chuqiao; Yang, Liping; Fang, Xiangdong

    2017-01-01

    Matrix metalloproteinase-7 (MMP-7) is a secreted zinc- and calcium-dependent endopeptidase that degrades a broad range of extracellular matrix substrates and additional substrates. MMP-7 playsa crucial role in a diverse array of cellular processes and appears to be a key regulator of fibrosis in several diseases, including pulmonary fibrosis, liver fibrosis, and cystic fibrosis. In particular, the relationship between MMP-7 and kidney fibrosis has attracted significant attention in recent years. Growing evidence indicates that MMP-7 plays an important role in the pathogenesis of kidney fibrosis. Here, we summarize the recent progress in the understanding of the role of MMP-7 in kidney fibrosis. In particular, we discuss how MMP-7 contributes to kidney fibrotic lesions via the following three pathways: epithelial-mesenchymal transition (EMT), transforming growth factor-beta (TGF-β) signaling, and extracellular matrix (ECM) deposition. Further dissection of the crosstalk among and regulation of these pathways will help clinicians and researchers develop effective therapeutic approaches for treating chronic kidney disease.

  19. Role of Circulating Fibrocytes in Cardiac Fibrosis

    Institute of Scientific and Technical Information of China (English)

    Rong-Jie Lin; Zi-Zhuo Su; Shu-Min Liang; Yu-Yang Chen; Xiao-Rong Shu; Ru-Qiong Nie; Jing-Feng Wang

    2016-01-01

    Objective: It is revealed that circulating fibrocytes are elevated in patients/animals with cardiac fibrosis, and this review aims to provide an introduction to circulating fibrocytes and their role in cardiac fibrosis.Data Sources: This review is based on the data from 1994 to present obtained from PubMed.The search terms were "circulating fibrocytes" and "cardiac fibrosis".Study Selection: Articles and critical reviews, which are related to circulating fibrocytes and cardiac fibrosis, were selected.Results: Circulating fibrocytes, which are derived from hematopoietic stem cells, represent a subset of peripheral blood mononuclear cells exhibiting mixed morphological and molecular characteristics ofhematopoietic and mesenchymal cells (CD34+/CD45+/collagen I+).They can produce extracellular matrix and many cytokines.It is shown that circulating fibrocytes participate in many fibrotic diseases, including cardiac fibrosis.Evidence accumulated in recent years shows that aging individuals and patients with hypertension, heart failure, coronary heart disease, and atrial fibrillation have more circulating fibrocytes in peripheral blood and/or heart tissue, and this elevation of circulating fibrocytes is correlated with the degree of fibrosis in the hearts.Conclusions: Circulating fibrocytes are effector cells in cardiac fibrosis.

  20. Viral infection drives tissue fibrosis in vitro

    Directory of Open Access Journals (Sweden)

    Andrea P. Malizia

    2008-04-01

    Full Text Available Idiopathic Pulmonary Fibrosis (IPF is a refractory and lethal interstitial lung disease characterized by loss of alveolar epithelial cells, fibroblast proliferation and extra-cellular matrix protein deposition. EBV, localised to alveolar epithelial cells of pulmonary fibrosis patients is associated with a poor prognosis. In this study we utilised a microarray-based differential gene expression analysis strategy to identify molecular drivers of EBV associated with lung fibrosis. A549 cells and an alveolar epithelial cell line infected with EBV (VAAK were used to identify genes whose expression was altered by EBV reactivation. EBV reactivation by TGFbeta1 drives alterations in expression of non-canonical Wnt pathway mediators, implicating it in epithelial mesenchymal transition (EMT, the molecular event underpinning scar production in tissue fibrosis. Cell invasion, EMT correlated transcripts expression, GSK-3b and c-Jun activation were altered in response to non-canonical Wnt pathway regulation. The role of EBV in promoting fibrosis can be attenuated by antiviral strategies and inhibition of Wnt signalling. Activation of non-canonical Wnt signalling pathway by EBV in epithelial cells suggests a novel mechanism of tissue fibrosis. These data present a framework for further description of the link between infectious agents and fibrosis, a significant disease burden.

  1. Cough in idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Mirjam J.G. van Manen

    2016-09-01

    Full Text Available Many patients with idiopathic pulmonary fibrosis (IPF complain of chronic refractory cough. Chronic cough is a distressing and disabling symptom with a major impact on quality of life. During recent years, progress has been made in gaining insight into the pathogenesis of cough in IPF, which is most probably “multifactorial” and influenced by mechanical, biochemical and neurosensory changes, with an important role for comorbidities as well. Clinical trials of cough treatment in IPF are emerging, and cough is increasingly included as a secondary end-point in trials assessing new compounds for IPF. It is important that such studies include adequate end-points to assess cough both objectively and subjectively. This article summarises the latest insights into chronic cough in IPF. It describes the different theories regarding the pathophysiology of cough, reviews the different methods to assess cough and deals with recent and future developments in the treatment of cough in IPF.

  2. Cystic fibrosis-related diabetes

    DEFF Research Database (Denmark)

    Andersen, Henrik U; Lanng, Susanne; Pressler, Tania

    2006-01-01

    of diabetic retinopathy was found in patients with insulin-treated CF-related diabetes, stressing the need for a regular screening program as in type 1 diabetes. Severely impaired kidney function was common in lung transplant patients, probably secondary to cyclosporine treatment.......OBJECTIVE: Cystic fibrosis (CF)-related diabetes has been regarded as a mild form of diabetes with a low risk of severe diabetes complications. The prevalence of CF-related diabetes increases with age, resulting in a 50% prevalence of diabetes at age 30 years. We sought to investigate whether...... microvascular complications in CF-related diabetes appear with a relevant frequency. RESEARCH DESIGN AND METHODS: Thirty-eight patients aged 30 (range 18-55) years with CF-related diabetes for 20 (0-31) years were screened for diabetes complications. Because of chronic pulmonary infections, the majority...

  3. Transient elastography for liver fibrosis diagnosis

    DEFF Research Database (Denmark)

    Andersen, Ellen Sloth; Christensen, Peer Brehm; Weis, Nina

    2008-01-01

    Liver biopsy is considered the "golden standard" for assessment of hepatic fibrosis. However, the procedure has limitations because of inconvenience and rare but serious complications as bleeding. Furthermore, sampling errors are frequent, and interobserver variability often poses problems....... Recently, a modified ultrasound scanner (transient elastography) has been developed to assess fibrosis. The device measures liver elasticity, which correlates well with the degree of fibrosis. Studies have shown that transient elastography is more accurate in diagnosing cirrhosis than minor to moderate...... to be a valuable diagnostic procedure and follow-up of patients with chronic liver diseases....

  4. Noninvasive Biomarkers of Liver Fibrosis: An Overview

    Directory of Open Access Journals (Sweden)

    Hind I. Fallatah

    2014-01-01

    Full Text Available Chronic liver diseases of differing etiologies are among the leading causes of mortality and morbidity worldwide. Establishing accurate staging of liver disease is very important for enabling both therapeutic decisions and prognostic evaluations. A liver biopsy is considered the gold standard for assessing the stage of hepatic fibrosis, but it has many limitations. During the last decade, several noninvasive markers for assessing the stage of hepatic fibrosis have been developed. Some have been well validated and are comparable to liver biopsy. This paper will focus on the various noninvasive biochemical markers used to stage liver fibrosis.

  5. Gastrointestinal manifestations in cystic fibrosis.

    Science.gov (United States)

    Eggermont, E

    1996-08-01

    CFTR, or cystic fibrosis transmembrane conductance regulator, the gene product that is defective in cystic fibrosis, is present in the apical membrane of the epithelial cells from the stomach to the colon. In the foregut, the clinical manifestations are not directly related to the primary defect of the CFTR chloride channel. The most troublesome complaints and symptoms originate from the oesophagus as peptic oesophagitis or oesophageal varices. In the small intestinal wall, the clinical expression of CF depends largely on the decreased secretion of fluid and chloride ions, the increased permeability of the paracellular space between adjacent enterocytes and the sticky mucous cover over the enterocytes. As a rule, the brush border enzyme activities are normal and there is some enhanced active transport as shown for glucose and alanine. The results of continuous enteral feeding of CF patients clearly show that the small intestinal mucosa, in the daily situation, is not functioning at maximal capacity. Although CFTR expression in the colon is lower, the large intestine may be the site of several serious complications such as rectal prolapse, meconium ileus equivalent, intussusception, volvulus and silent appendicitis. In recent years colonic strictures, after the use of high-dose pancreatic enzymes, are being increasingly reported; the condition has recently been called CF fibrosing colonopathy. The CF gastrointestinal content itself differs mainly from the normal condition by the lower acidity in the foregut and the accretion of mucins and proteins, eventually resulting in intestinal obstruction, in the ileum and colon. Better understanding of the CF gastrointestinal phenotype may contribute to improvement of the overall wellbeing of these patients.

  6. Vitamin D deficiency as a risk factor for cystic fibrosis-related diabetes in the Scandinavian Cystic Fibrosis Nutritional Study

    DEFF Research Database (Denmark)

    Pincikova, T; Nilsson, Kristine Kahr; Moen, I E;

    2011-01-01

    Many cystic fibrosis patients are vitamin D-insufficient. Cystic fibrosis-related diabetes is a major complication of cystic fibrosis. The literature suggests that vitamin D might possess certain glucose-lowering properties. We aimed to assess the relationship between vitamin D and cystic fibrosis...

  7. Utilidad de la elastografía hepática (Fibroscan) en la caracterización y seguimiento de pacientes con hepatitis crónica B

    OpenAIRE

    Mena de Cea, Álvaro

    2016-01-01

    [Resumen] Objetivos: el objetivo principal del presente estudio es determinar la prevalencia de fibrosis avanzada y definir los factores asociados a la misma, medida mediante EH, en pacientes con HCB clasificados como portadores inactivos, no portadores inactivos (en tratamiento o no) y coinfectados por VIH. Como objetivos secundarios se encuentran: evaluar el efecto del tratamiento antiviral en la fibrosis hepática, valorar la progresión de la fibrosis hepática en las disti...

  8. Osteosarcoma de huesos del pie con alto grado de malignidad

    Directory of Open Access Journals (Sweden)

    Alain Martínez Milián

    2015-05-01

    Full Text Available El osteosarcoma es la neoplasia primaria del hueso más frecuente, los hombres son los más afectados en una relación 1,2 - 1,5 por cada mujer y con una tasa de mortalidad cercana a 0,15/100.000 habitantes/año. Se reporta el caso de una paciente femenina de 57 años de edad, con dolor, aumento de volumen e impotencia funcional de aproximadamente seis meses de evolución, los exámenes diagnósticos utilizados fueron radiografía de pie, tomografía axial computarizada de miembro inferior y biopsia de hueso. Se tuvo la confirmación diagnóstica de osteosarcoma de alto grado de malignidad por anatomía patológica. El tratamiento realizado fue quirúrgico, practicándose la amputación del miembro inferior, pudiendo así mejorar la supervivencia y extendiéndole la vida a la paciente. El motivo de la presentación del caso está dado por la poca frecuencia de este tipo de tumor óseo en los huesos del pie en pacientes femeninas

  9. Unilateral subretinal fibrosis and uveitis syndrome.

    Science.gov (United States)

    Rehan, S; Javaid, Z; Al-Bermani, A

    2015-05-01

    Subretinal fibrosis and uveitis syndrome is a rare, potentially devastating, posterior uveitis of unknown aetiology, characterised bilaterally by initial multifocal choroiditis with later progressive subretinal fibrosis. We report a rare case of unilateral subretinal fibrosis and uveitis syndrome. To date, there are only two case reports of unilateral disease. Our patient presented with unilateral blur and was found to have reduced visual acuity. A Bartonella profile was positive and a diagnosis of Bartonella posterior uveitis was made. Several positive ocular findings in the anterior chamber and on fundoscopy consistent with the syndrome were found. When steroid therapy alone could no longer control active inflammation, the immunosuppressive agent mycophenolate was added. Over time subretinal fibrosis became established sparing the macula and associated complications occurred, but with mycophenolate, at four years, our patient's visual acuity had improved and remains stable. Moreover, four years after her initial presentation, her condition remains strictly unilateral.

  10. Pathohistological changes in fetuses with cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Đolai Matilda

    2012-01-01

    Full Text Available Introduction. Cystic fibrosis or mucoviscidosis is a genetically caused disease. The intensity of disease and histopathological changes grow throughout the life. According to the literature, pathological changes characteristic of cystic fibrosis become noticeable around the sixth month of life. Case Report. After amniocentesis of a 5-lunar month-old fetus had been done, which confirmed cystic fibrosis, the Ethics Commission approved the preterm labor. The autopsy and histopathological analysis demonstrated the existence of typical histopathological changes in the pancreas and intestines. Discussion. In the late fetal period or during the period around the delivery, cystic fibrosis is usually manifested as meconial cap with or without obstruction of the intestinal lumen. Morphological changes in the exocrine glands usually develop only after birth. In this case, the existence of meconial obstruction, as well as the typical acidofil content in the secretory ducts and acini of the pancreas was confirmed, which is unusual for the fetal age of five months.

  11. Respiratory bacterial infections in cystic fibrosis

    DEFF Research Database (Denmark)

    Ciofu, Oana; Hansen, Christine R; Høiby, Niels

    2013-01-01

    Bacterial respiratory infections are the main cause of morbidity and mortality in patients with cystic fibrosis (CF). Pseudomonas aeruginosa remains the main pathogen in adults, but other Gram-negative bacteria such as Achromobacter xylosoxidans and Stenotrophomonas maltophilia as well...

  12. "End-stage" pulmonary fibrosis in sarcoidosis.

    Science.gov (United States)

    Teirstein, Alvin T; Morgenthau, Adam S

    2009-02-01

    Pulmonary fibrosis is an unusual "end stage" in patients with sarcoidosis. Fibrosis occurs in a minority of patients, and presents with a unique physiologic combination of airways dysfunction (obstruction) superimposed on the more common restrictive dysfunction. Imagin techniques are essential to the diagnosis, assessment and treatment of pulmonary fibrosis. Standard chest radiographs and CT scans may reveal streaks, bullae, cephalad retraction of the hilar areas, deviation of the trachea and tented diaphragm. Positive gallium and PET scans indicate residual reversible granulomatous disease and are important guides to therapy decisions. Treatment, usually with corticosteroids, is effective in those patients with positive scans, but fibrosis does not improve with any treatment. With severe functional impariment and patient disability, pulmonary hypertension and right heart failure may supervene for which the patient will require treatment. Oxygen, careful diuresis, sildenafil and bosentan may be salutary. These patients are candidates for lung transplantation.

  13. Nutrition in Cystic Fibrosis: Macro- and Micronutrients

    NARCIS (Netherlands)

    Oudshoorn, Johanna Hermiena

    2006-01-01

    Cystic fibrosis (CF) is the most common life-threatening autosomal recessive inherited disease in Caucasians, and is characterized by progressive lung disease, pancreatic insufficiency, malnutrition, hepatobiliary disease and elevated sweat electrolyte levels. The increased survival of CF patients d

  14. Molecular and cellular mechanisms of pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Todd Nevins W

    2012-07-01

    Full Text Available Abstract Pulmonary fibrosis is a chronic lung disease characterized by excessive accumulation of extracellular matrix (ECM and remodeling of the lung architecture. Idiopathic pulmonary fibrosis is considered the most common and severe form of the disease, with a median survival of approximately three years and no proven effective therapy. Despite the fact that effective treatments are absent and the precise mechanisms that drive fibrosis in most patients remain incompletely understood, an extensive body of scientific literature regarding pulmonary fibrosis has accumulated over the past 35 years. In this review, we discuss three broad areas which have been explored that may be responsible for the combination of altered lung fibroblasts, loss of alveolar epithelial cells, and excessive accumulation of ECM: inflammation and immune mechanisms, oxidative stress and oxidative signaling, and procoagulant mechanisms. We discuss each of these processes separately to facilitate clarity, but certainly significant interplay will occur amongst these pathways in patients with this disease.

  15. Imaging findings in idiopathic pelvic fibrosis

    Energy Technology Data Exchange (ETDEWEB)

    Wiesner, W.; Bongartz, G. [Inst. of Diagnostic Radiology University Hospital Basel (Switzerland); Stoffel, F. [Inst. of Urology, University Hospital Basel (Switzerland)

    2001-04-01

    Two patients presented with ureteric obstruction, and voiding symptoms and constipation, respectively, and were examined by means of intravenous urography and computed tomography. One patient was additionally examined by means of MR tomography. After CT (performed in both patients) and MRT (performed in one patient) had shown a diffuse, contrast-enhancing, infiltrating process in the small pelvis with infiltration of adjacent organs and vessels, surgical biopsy proved the diagnosis of idopathic pelvic fibrosis. Extension of retroperitoneal fibrosis below the pelvic rim is very rare. Clinical symptoms of pelvic fibrosis are variable and imaging findings may lead to a broad list of differential diagnoses. We present two patients with idiopathic pelvic fibrosis and discuss radiological findings and differential diagnoses of this rare disease. (orig.)

  16. Fibrosis quística. Aspectos diagnósticos

    Directory of Open Access Journals (Sweden)

    Luis Ortigosa

    2007-03-01

    Full Text Available La fibrosis quística (FQ es una de las enfermedades genéticas mortales más frecuentes en la raza caucásica. Se caracteriza por una disfunción de las glándulas exocrinas, con insuficiencia pancreática y bronconeumopatía crónica. Es una enfermedad de transmisión autonómica recesiva, se sabe que el gen defectuoso está localizado en el cromosoma 7 humano, conocido como gen regulador de la conductancia transmembrana de la fibrosis quística (CFTR,y que de las más de mil mutaciones de este gen, la mutación DF508 es la más común, pues se halla en aproximadamente 70% de los alelos CFTR defectuosos. El diagnóstico de la FQ se ha basado clásicamente en la determinación de por lo menos 2-3 determinaciones positivas de electrólitos en sudor, junto con uno de los siguientes criterios clínicos: íleo meconial, historia familiar de FQ, insuficiencia pancreática exocrina, enfermedad pulmonar crónica, azoospermia obstructiva y síndrome de pérdida de sal. Los criterios diagnósticos actuales incluyen, junto a la presencia de las características clínicas, dos determinaciones de concentraciones de cloro en sudor superior a 60 mmol/l, o demostración de alteraciones en el transporte iónico a través del epitelio nasal (diferencia de potencial nasal o la detección de dos mutaciones reconocidas de FQ.

  17. EVALUACIÓN DE LA TOLERANCIA A SUELOS CONTAMINADOS CON ACEITE DIESEL EN ESPECIES VEGETALES CON POTENCIAL BIORREMEDIADOR

    Directory of Open Access Journals (Sweden)

    MARÍA CRISTINA PETENELLO

    2012-01-01

    Full Text Available Los suelos contaminados con petróleo o sus derivados pueden ser remediados a través de diversos métodos, dentro de los cuales se encuentran aquellos que emplean organismos vivos tales como plantas, que poseen la capacidad de mineralizar estos compuestos transformándolos en otros más simples, asimilables a compuestos naturales. Al encarar proyectos de fitorremediación es importante emplear plantas nativas porque están adaptadas a las condiciones ecológicas particulares de la región. En el presente trabajo como primera etapa se evaluó la respuesta de Spartina argentinensis, Paspalum atratum, Paspalum guenoarum y Melilotus albus a la presencia de aceite diesel, considerando la germinación de sus semillas, la emergencia de plántulas y la biomasa alcanzada en suelos que contenían 1 y 2 % de aceite diesel, en condiciones experimentales. Todos estos parámetros se vieron afectados con las concentraciones de contaminante empleadas, sin embargo, las plantas pudieron prosperar demostrando en consecuencia que podrían ser empleadas en proyectos de fitorremediación.

  18. The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)

    OpenAIRE

    Rosenberg, Mark F.; O'Ryan, Liam P.; Hughes, Guy; Zhao, Zhefeng; Aleksandrov, Luba A.; Riordan, John R.; Ford, Robert C.

    2011-01-01

    Cystic fibrosis affects about 1 in 2500 live births and involves loss of transmembrane chloride flux due to a lack of a membrane protein channel termed the cystic fibrosis transmembrane conductance regulator (CFTR). We have studied CFTR structure by electron crystallography. The data were compared with existing structures of other ATP-binding cassette transporters. The protein was crystallized in the outward facing state and resembled the well characterized Sav1866 transporter. We identified ...

  19. Ovarian Fibrosis: A Phenomenon of Concern

    Science.gov (United States)

    Zhou, Feng; Shi, Li-Bing; Zhang, Song-Ying

    2017-01-01

    Objective: Ovarian fibrosis is characterized by excessive proliferation of ovarian fibroblasts and deposition of extracellular matrix (ECM) and it is one of the principal reasons for ovarian dysfunction. This review aimed to investigate the pathogenetic mechanism of ovarian fibrosis and to clarify the relationship between ovarian diseases and fibrosis. Data Sources: We searched PubMed for English language articles published up to November 2016. The search terms included ovarian fibrosis OR fibrosis, ovarian chocolate cyst OR ovarian endometrioma, polycystic ovarian syndrome (PCOS), premature ovarian failure, ECM, matrix metalloproteinases (MMPs), tissue inhibitors of matrix metalloproteinases (TIMPs), transforming growth factor-beta 1 (TGF-β1), connective tissue growth factor (CTGF), peroxisome proliferator-activated receptor gamma (PPAR-γ), vascular endothelial growth factor (VEGF), endothelin-1 (ET-1), and combinations of these terms. Study Selection: Articles were obtained and reviewed to analyze the pathogenic mechanism of ovarian fibrosis and related ovarian diseases. Results: Many cytokines, such as MMPs, TIMPs, TGF-β1, CTGF, PPAR-γ, VEGF, and ET-1, are involved in ovarian fibrogenesis. Ovarian fibrogenesis is associated with various ovarian diseases, including ovarian chocolate cyst, PCOS, and premature ovarian failure. One finding of particular interest is that fibrogenesis in peripheral tissues around an ovarian chocolate cyst commonly causes ovarian function diminution, and therefore, this medical problem should arouse widespread concern in clinicians worldwide. Conclusions: Patients with ovarian fibrosis are susceptible to infertility and tend to have decreased responses to assisted fertility treatment. Thus, protection of ovarian function should be a priority for women who wish to reproduce when making therapeutic decisions about ovarian fibrosis-related diseases. PMID:28139522

  20. Inhibitory effect of liposomal quercetin on acute hepatitis and hepatic fibrosis induced by concanavalin A

    Directory of Open Access Journals (Sweden)

    Y. Wan

    2014-08-01

    Full Text Available Immune response plays an important role in the development of hepatic fibrosis. In the present study, we investigated the effects of quercetin on hepatitis and hepatic fibrosis induced by immunological mechanism. In the acute hepatitis model, quercetin (2.5 mg/kg was injected iv into mice 30 min after concanavalin A (Con A challenge. Mice were sacrificed 4 or 24 h after Con A injection, and aminotransferase tests and histopathological sections were performed. Treatment with quercetin significantly decreased the levels of alanine aminotransferase (ALT and aspartate aminotransferase (AST. Consistent with this observation, treatment with quercetin markedly attenuated the pathologic changes in the liver. A hepatic fibrosis model was also generated in mice by Con A challenge once a week for 6 consecutive weeks. Mice in the experimental group were treated with daily iv injections of quercetin (0.5 mg/kg. Histopathological analyses revealed that treatment with quercetin markedly decreased collagen deposition, pseudolobuli development, and hepatic stellate cells activation. We also examined the effects of quercetin on the nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB and transforming growth factor beta (TGF-β pathways by immunohistochemistry and real-time reverse transcriptase-polymerase chain reaction (RT-PCR. NF-κB and TGF-β production was decreased after treatment with quercetin, indicating that the antifibrotic effect of quercetin is associated with its ability to modulate NF-κB and TGF-β production. These results suggest that quercetin may be an effective therapeutic strategy in the treatment of patients with liver damage and fibrosis.

  1. Role of Circulating Fibrocytes in Cardiac Fibrosis

    Science.gov (United States)

    Lin, Rong-Jie; Su, Zi-Zhuo; Liang, Shu-Min; Chen, Yu-Yang; Shu, Xiao-Rong; Nie, Ru-Qiong; Wang, Jing-Feng; Xie, Shuang-Lun

    2016-01-01

    Objective: It is revealed that circulating fibrocytes are elevated in patients/animals with cardiac fibrosis, and this review aims to provide an introduction to circulating fibrocytes and their role in cardiac fibrosis. Data Sources: This review is based on the data from 1994 to present obtained from PubMed. The search terms were “circulating fibrocytes” and “cardiac fibrosis”. Study Selection: Articles and critical reviews, which are related to circulating fibrocytes and cardiac fibrosis, were selected. Results: Circulating fibrocytes, which are derived from hematopoietic stem cells, represent a subset of peripheral blood mononuclear cells exhibiting mixed morphological and molecular characteristics of hematopoietic and mesenchymal cells (CD34+/CD45+/collagen I+). They can produce extracellular matrix and many cytokines. It is shown that circulating fibrocytes participate in many fibrotic diseases, including cardiac fibrosis. Evidence accumulated in recent years shows that aging individuals and patients with hypertension, heart failure, coronary heart disease, and atrial fibrillation have more circulating fibrocytes in peripheral blood and/or heart tissue, and this elevation of circulating fibrocytes is correlated with the degree of fibrosis in the hearts. Conclusions: Circulating fibrocytes are effector cells in cardiac fibrosis. PMID:26831236

  2. Inhibition of SIRT2 suppresses hepatic fibrosis.

    Science.gov (United States)

    Arteaga, Maribel; Shang, Na; Ding, Xianzhong; Yong, Sherri; Cotler, Scott J; Denning, Mitchell F; Shimamura, Takashi; Breslin, Peter; Lüscher, Bernhard; Qiu, Wei

    2016-06-01

    Liver fibrosis can progress to cirrhosis and result in serious complications of liver disease. The pathogenesis of liver fibrosis involves the activation of hepatic stellate cells (HSCs), the underlying mechanisms of which are not fully known. Emerging evidence suggests that the classic histone deacetylases play a role in liver fibrosis, but the role of another subfamily of histone deacetylases, the sirtuins, in the development of hepatic fibrosis remains unknown. In this study, we found that blocking the activity of sirtuin 2 (SIRT2) by using inhibitors or shRNAs significantly suppressed fibrogenic gene expression in HSCs. We further demonstrated that inhibition of SIRT2 results in the degradation of c-MYC, which is important for HSC activation. In addition, we discovered that inhibition of SIRT2 suppresses the phosphorylation of ERK, which is critical for the stabilization of c-MYC. Moreover, we found that Sirt2 deficiency attenuates the hepatic fibrosis induced by carbon tetrachloride (CCl4) and thioacetamide (TAA). Furthermore, we showed that SIRT2, p-ERK, and c-MYC proteins are all overexpressed in human hepatic fibrotic tissues. These data suggest a critical role for the SIRT2/ERK/c-MYC axis in promoting hepatic fibrogenesis. Inhibition of the SIRT2/ERK/c-MYC axis represents a novel strategy to prevent and to potentially treat liver fibrosis and cirrhosis.

  3. Arteterapia con personas con discapacidad intelectual

    OpenAIRE

    Lorenzo Pipkau, Milena

    2015-01-01

    Este proyecto pretende hacer una aproximaci??n al mundo del Arte Terapia y los beneficios que esta disciplina puede aportar a las personas con discapacidad intelectual. La idea surge de la experiencia previa de la autora en este ??mbito y con este colectivo. A trav??s de la documentaci??n bibliogr??fica se busca ampliar el conocimiento en cuanto al concepto de arteterapia y sus antecedentes, con la finalidad de elaborar una propuesta pr??ctica que se basar?? en el dise??o de un taller de arte...

  4. Superantigen staphylococcal enterotoxin C1 mutant can reduce paraquat pulmonary fibrosis.

    Science.gov (United States)

    Li, Tiegang; Xu, Mingkai; Wang, Nana; Zhao, Min

    2015-01-01

    A network of inflammation factors is related to pulmonary fibrosis induced by paraquat (PQ) poisoning. At high doses, the superantigen staphylococcal enterotoxin C1 (SEC1) can induce immunological unresponsiveness and inhibit release of inflammation factors. In this study, site-directed mutagenesis was performed at the H118 and H122 amino acid residues of SEC1 to reduce SEC1 toxicity. The SEC1 mutant showed significantly decreased pyrogenic toxicity, but retained clonal anergy at high dosages in vitro. Pretreatment with the SEC1 mutant prior to PQ poisoning in mice reduced symptom duration and severity, prolonged survival time, and decreased the splenocyte response to ConA induction. The SEC1 mutant also down-regulated several important cytokines related to fibrosis in the plasma after PQ poisoning. SEC1 decreased the expression of genes related to pulmonary fibrosis, including α-SMA, COL1a1, COL3 and TGF-β1, in PQ poisoned mice. Histomorphological observation indicated alleviation of pathological changes in the lungs after SEC1 pretreatment compared to mice in the PQ group. In conclusion, the SEC1 mutant reduced pulmonary interstitial fibrosis induced by PQ poisoning.

  5. DESARROLLO DE APIO MINIMAMENTE PROCESADO FORTIFICADO CON VITAMINA E, UTILIZANDO LA INGENIERIA DE MATRICES

    Directory of Open Access Journals (Sweden)

    YISELL JOHAN MARTELO CASTAÑO

    2011-01-01

    Full Text Available El objetivo de este trabajo fue desarrollar un producto minimamente procesado fortificado con vitamina E, a partir de apio (Apium graveolens L. var.dulce, utilizando la ingenieria de matrices como metodologia de obtencion de alimentos funcionales. Trozos de apio impregnados al vacio con dl¿¿tocoferol acetato emulsificado en una solucion isotonica al vegetal de NaCl, 1,2%, fueron evaluados en funcion del tiempo de almacenamiento y el envasado (con y sin vacio, en terminos de la cuantificacion de sus propiedades fisicoquimicas, color, textura, estabilidad de la vitamina E y caracteristicas sensoriales. Se formulo la emulsion con el objetivo de adicionar teoricamente un 73% del Valor Diario Recomendado (VDR vitamina E/100 g de apio fresco y su cuantificacion se realizo por HPLC. La respuesta a la impregnacion en la matriz fue de 12,10+-1,15%, lo cual permitio alcanzar un 112% VDR vitamina E/100g apio fresco, manteniendose este contenido durante el almacenamiento de 9 dias en ambos tipos de envasado. Los parametros fisicoquimicos del producto fueron afectados por la IV, el tiempo y el envasado, manteniendo su coloracion verdosa y presentando disminucion en la firmeza. La ingenieria de matrices constituye una metodologia efectiva para el desarrollo de apio minimamente procesado adicionado con vitamina E.

  6. Taurine attenuates radiation-induced lung fibrosis in C57/Bl6 fibrosis prone mice.

    LENUS (Irish Health Repository)

    Robb, W B

    2010-03-01

    The amino acid taurine has an established role in attenuating lung fibrosis secondary to bleomycin-induced injury. This study evaluates taurine\\'s effect on TGF-beta1 expression and the development of lung fibrosis after single-dose thoracic radiotherapy.

  7. Recommendations for quality improvement in genetic testing for cystic fibrosis European Concerted Action on Cystic Fibrosis

    NARCIS (Netherlands)

    Dequeker, E; Cuppens, H; Dodge, J; Estivill, [No Value; Goossens, M; Pignatti, PF; Scheffer, H; Schwartz, M; Schwarz, M; Tummler, B; Cassiman, JJ

    2000-01-01

    These recommendations for quality improvement of cystic fibrosis genetic diagnostic testing provide general guidelines for the molecular genetic testing of cystic fibrosis in patients/individuals. General strategies for testing as well as guidelines for laboratory procedures, internal and external q

  8. Efectividad de la hipoterapia en niños con patología del desarrollo: revisión sistemática

    OpenAIRE

    2014-01-01

    RESUMEN: Introducción: el desarrollo psicomotor es un proceso continuo de cambios que puede verse afectado por enfermedades o por factores externos. Una de las enfermedades más comunes es la parálisis cerebral. Se trata de una lesión cerebral permanente y no progresiva que afecta al movimiento, a la postura y a la función muscular. Otra enfermedad es la discapacidad intelectual, la cual puede estar causada por varias patologías. Una de las terapias alternativas con las que se tratan es la hip...

  9. Miocardiopatía Chagásica con taquicardia ventricular: Hospital Obrero Nº 2 “Caja Nacional de Salud”

    OpenAIRE

    Dr. Raúl Arnez Terrazas; Condori Paxi Jiovana Mariela; Condori Pardo Nancy

    2012-01-01

    La miocardiopatía chagásica es la inflamación del músculo cardiaco producida por el Trypanosoma cruzi. Los parásitos que se encuentran en los excrementos de la vinchuca (Tripanosoma infestans) son transmitidos al organismo luego de la alimentación del vector. Afecta varias áreas del organismo humano como son los nervios periféricos y las paredes del intestino grueso entre otros, sin embargo el corazón es uno de los órganos que con mayor frecuencia es afectado una vez producida la infección. O...

  10. Descripción de la estructura familiar de una muestra de pacientes con hemofilia. Comparación Argentina-México

    OpenAIRE

    Maricela Osorio Guzmán; Silvina Graña

    2016-01-01

    Las enfermedades crónicas como la hemofilia rebasan los marcos institucionales sanitarios y abarcan tanto la vida cotidiana de la persona como su entorno social. Esta situación se agudiza cuando el afectado es un hijo de edad pediátrica. El objetivo de este trabajo fue describir y comparar la estructura de 50 familias que tienen algún miembro que padece hemofilia. Método. Participaron 50 madres y padres de pacientes pediátricos con hemofilia de diferente tipo y grado clínico (27 familias mexi...

  11. Nephrogenic systemic fibrosis: Current concepts

    Directory of Open Access Journals (Sweden)

    Prasanta Basak

    2011-01-01

    Full Text Available Nephrogenic systemic fibrosis (NSF was first described in 2000 as a scleromyxedema-like illness in patients on chronic hemodialysis. The relationship between NSF and gadolinium contrast during magnetic resonance imaging was postulated in 2006, and subsequently, virtually all published cases of NSF have had documented prior exposure to gadolinium-containing contrast agents. NSF has been reported in patients from a variety of ethnic backgrounds from America, Europe, Asia and Australia. Skin lesions may evolve into poorly demarcated thickened plaques that range from erythematous to hyperpigmented. With time, the skin becomes markedly indurated and tethered to the underlying fascia. Extracutaneous manifestations also occur. The diagnosis of NSF is based on the presence of characteristic clinical features in the setting of chronic kidney disease, and substantiated by skin histology. Differential diagnosis is with scleroderma, scleredema, scleromyxedema, graft-versus-host disease, etc. NSF has a relentlessly progressive course. While there is no consistently successful treatment for NSF, improving renal function seems to slow or arrest the progression of this condition. Because essentially all cases of NSF have developed following exposure to a gadolinium-containing contrast agent, prevention of this devastating condition involves the careful avoidance of administering these agents to individuals at risk.

  12. Male fertility in cystic fibrosis.

    LENUS (Irish Health Repository)

    Chotirmall, S H

    2011-04-05

    Infertility rates among males with cystic fibrosis (CF) approximate 97%. No information is currently available within Ireland determining an understanding of fertility issues and the best methods of information provision to this specialized group. This study aimed to determine understanding and preferred approaches to information provision on fertility issues to Irish CF males. A Descriptive Study utilizing prospective coded questionnaires was mailed to a male CF cohort (n=50). Sections included demographics, fertility knowledge & investigation. Response rate was 16\\/50 (32%). All were aware that CF affected their fertility. More than two-thirds (n=11) were able to provide explanations whilst only one-third (n=5) provided the correct explanation. Significant numbers stated thoughts of marriage and a future family. Half have discussed fertility with a healthcare professional (HCP). Mean age of discussion was 21.9 years. One third preferred an earlier discussion. The commonest first source for information was written material which was also the preferred source. Three-quarters requested further information preferring again, written material. Significant gaps in sex education of Irish CF males exist. Discussion should be initiated by HCPs and centre-directed written material devised to address deficiencies.

  13. Rehabilitación deglutoria en un niño con Síndrome Prader Willi

    OpenAIRE

    2015-01-01

    El síndrome de Prader-Willi (SPW) es un trastorno congénito, que afecta tanto a hombres como mujeres de todas las etnias y lugares del mundo por igual, con una prevalencia estimada de 1 de cada 10.000 a 30.000 personas. Dada la poca incidencia y el poco conocimiento de este síndrome, sobre todo en el área logopédica, quiero dar a conocer en este TFG como se debería de realizar una rehabilitación deglutoria en un bebé afectado con este síndrome. El objetivo de este trabajo es obtener un bue...

  14. Evidence of epigenetic tags in cardiac fibrosis.

    Science.gov (United States)

    Grimaldi, Vincenzo; De Pascale, Maria Rosaria; Zullo, Alberto; Soricelli, Andrea; Infante, Teresa; Mancini, Francesco Paolo; Napoli, Claudio

    2017-02-01

    In cardiac fibrosis, following an injury or a stress, non-functional fibrotic tissue substitutes normal myocardium, thus leading to progressive heart failure. Activated fibroblasts are principal determinants of cardiac fibrosis by producing excessive fibrotic extracellular matrix and causing hypertrophy of cardiomyocytes. Epigenetic changes, such as DNA methylation, histone modifications, and miRNAs have been involved in these mechanisms. Therefore, there is a strong interest in reverting such epigenetic transformations in order to arrest myocardial fibrotic degeneration. Demethylating agents, such as 5-aza-2'-deoxycytidine, 5-azacytidine, some selective histone deacetylase inhibitors, including mocetinostat, trichostatin A, and MPT0E014, have a direct action on important inducers of cardiac fibrosis. Also dietary compounds, such as resveratrol, can suppress the differentiation of fibroblasts to myofibroblasts. Although in vivo and in vitro studies suggest specific epigenetic therapies to treat cardiac fibrosis, the related clinical trials are still lacking. A better understanding of the epigenetic effects of dietary compounds (e.g. curcumin and green tea catechins) on the onset and progression of cardiac fibrosis, will allow the identification of protective dietary patterns and/or the generation of novel potential epidrugs.

  15. Toward surface quantification of liver fibrosis progression

    Science.gov (United States)

    He, Yuting; Kang, Chiang Huen; Xu, Shuoyu; Tuo, Xiaoye; Trasti, Scott; Tai, Dean C. S.; Raja, Anju Mythreyi; Peng, Qiwen; So, Peter T. C.; Rajapakse, Jagath C.; Welsch, Roy; Yu, Hanry

    2010-09-01

    Monitoring liver fibrosis progression by liver biopsy is important for certain treatment decisions, but repeated biopsy is invasive. We envision redefinition or elimination of liver biopsy with surface scanning of the liver with minimally invasive optical methods. This would be possible only if the information contained on or near liver surfaces accurately reflects the liver fibrosis progression in the liver interior. In our study, we acquired the second-harmonic generation and two-photon excitation fluorescence microscopy images of liver tissues from bile duct-ligated rat model of liver fibrosis. We extracted morphology-based features, such as total collagen, collagen in bile duct areas, bile duct proliferation, and areas occupied by remnant hepatocytes, and defined the capsule and subcapsular regions on the liver surface based on image analysis of features. We discovered a strong correlation between the liver fibrosis progression on the anterior surface and interior in both liver lobes, where biopsy is typically obtained. The posterior surface exhibits less correlation with the rest of the liver. Therefore, scanning the anterior liver surface would obtain similar information to that obtained from biopsy for monitoring liver fibrosis progression.

  16. Acute exacerbation of airspace enlargement with fibrosis

    Directory of Open Access Journals (Sweden)

    Tomoyuki Kakugawa

    2014-01-01

    Full Text Available In 2008, Kawabata et al. described a lesion which they termed “airspace enlargement with fibrosis” that could be included on the spectrum of smoking-related interstitial lung diseases. This group also reported that patients with airspace enlargement with fibrosis but without coexisting interstitial pneumonia of another type had no acute exacerbations and favorable prognoses on clinical follow-up. Here we describe the first case, to our knowledge, of acute exacerbation of airspace enlargement with fibrosis without coexisting interstitial pneumonia of another type. An 82-year-old man was referred to our department for worsening dyspnea and new alveolar opacities on chest radiograph following left pulmonary segmentectomy (S6 for cancer. A diagnosis of acute exacerbation of airspace enlargement with fibrosis without coexisting interstitial pneumonia of other types was made, based on pathological evidence of airspace enlargement with fibrosis and organizing diffuse alveolar damage. Treatment with high-dose methylprednisolone followed by tapered oral prednisolone resulted in gradual improvement of the clinical condition and chest radiographic findings. Clinicians should be aware that patients with airspace enlargement with fibrosis may experience acute exacerbation.

  17. The Role of Catalase in Pulmonary Fibrosis

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    Takigawa Tomoko

    2010-12-01

    Full Text Available Abstract Background Catalase is preferentially expressed in bronchiolar and alveolar epithelial cells, and acts as an endogenous antioxidant enzyme in normal lungs. We thus postulated epithelial damage would be associated with a functional deficiency of catalase during the development of lung fibrosis. Methods The present study evaluates the expression of catalase mRNA and protein in human interstitial pneumonias and in mouse bleomycin-induced lung injury. We examined the degree of bleomycin-induced inflammation and fibrosis in the mice with lowered catalase activity. Results In humans, catalase was decreased at the levels of activity, protein content and mRNA expression in fibrotic lungs (n = 12 compared to control lungs (n = 10. Immunohistochemistry revealed a decrease in catalase in bronchiolar epithelium and abnormal re-epithelialization in fibrotic areas. In C57BL/6J mice, catalase activity was suppressed along with downregulation of catalase mRNA in whole lung homogenates after bleomycin administration. In acatalasemic mice, neutrophilic inflammation was prolonged until 14 days, and there was a higher degree of lung fibrosis in association with a higher level of transforming growth factor-β expression and total collagen content following bleomycin treatment compared to wild-type mice. Conclusions Taken together, these findings demonstrate diminished catalase expression and activity in human pulmonary fibrosis and suggest the protective role of catalase against bleomycin-induced inflammation and subsequent fibrosis.

  18. A brief review on molecular, genetic and imaging techniques for HCV fibrosis evaluation

    Directory of Open Access Journals (Sweden)

    Sumrin Aleena

    2011-02-01

    Full Text Available Abstract Background Chronic HCV is one of the major causes of morbidity and mortality in the present day world. The assessment of disease progression not only provides useful information for diagnosis and therapeutic supervision judgment but also for monitoring disease. Different invasive and non invasive methods are applied to diagnose the disease from initial to end stage (mild fibrosis to cirrhosis. Although, liver biopsy is still considered as gold standard to identify liver histological stages, an assessment of the disease development based on non-invasive clinical findings is also emerging and this may replace the need of biopsy in near future. This review gives brief insight on non-invasive methods currently available for predicting liver fibrosis in HCV with their current pros and cons to make easier for a clinician to choose better marker to assess liver fibrosis in HCV infected patients. Methods More than 200 studies regarding invasive and noninvasive markers available for HCV liver disease diagnosis were thoroughly reviewed. We examined year wise results of these markers based on their sensitivity, specificity, PPV, NPV and AUROCs. Results We found that in all non-invasive serum markers for HCV, FibroTest, Forn's Index, Fibrometer and HepaScore have high five-year predictive value but with low AUROCs (0.60~0.85 and are not comparable to liver biopsy (AUROC = 0.97. Even though from its beginning, Fibroscan is proved to be best with high AUROCs (> 0.90 in all studies, no single noninvasive marker is able to differentiate all fibrosis stages from end stage cirrhosis. Meanwhile, specific genetic markers may not only discriminate fibrotic and cirrhotic liver but also differentiate individual fibrosis stages. Conclusions There is a need of marker which accurately determines the stage based on simplest routine laboratory test. Genetic marker in combination of imaging technique may be the better non invasive diagnostic method in future.

  19. Fibrocytes in pulmonary fibrosis: a brief synopsis

    Directory of Open Access Journals (Sweden)

    Shyam Maharaj

    2013-12-01

    Full Text Available Fibrocytes are bone marrow-derived, circulating mesenchymal progenitor cells that play a role in several fibrotic disorders, including lung fibrosis. They are attracted to injured tissue by various chemokines. It is likely that fibrocytes play a detrimental role in tissue homeostasis and promote fibrosis, although this paradigm needs further confirmation. This would make fibrocytes a possible novel treatment target for fibrotic disorders. Fibrocytes also have some potential as a biomarker for idiopathic pulmonary fibrosis (IPF and other diseases, but the promising preliminary data from single centre studies still require independent validation. Despite several, as yet, unresolved issues, it has become clear that fibrocytes are more than an incidental finding in lung injury and repair, and may hold great promise for the future of IPF management.

  20. [Regeneration and fibrosis of corneal tissues].

    Science.gov (United States)

    Simirskiĭ, V N

    2014-01-01

    In this review, the features of the regeneration of corneal tissue and its disorders leading to the development of fibrosis are considered. The data on the presence of stem (clonogenic) cell pool in the corneal tissues (epithelium, endothelium, stroma) are given; these cells can serve as a source for regeneration of the tissues at injury or various diseases. The main steps of regeneration of corneal tissues and their disorders that lead to outstripping proliferation of myofibroblasts and secretion of extracellular matrix in the wound area and eventually cause the formation of connective tissue scar and corneal opacity are considered. Particular attention is given to the successes of translational medicine in the treatment of corneal tissue fibrosis. The methods of cell therapy aimed at the restoration of stem cell pool of corneal tissues are the most promising. Gene therapy provides more opportunities; one of its main objectives is the suppression of the myofibroblast proliferation responsible for the development of fibrosis.

  1. Caracterización clínica de familias costarricenses con trastorno afectivo bipolar

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    Javier Contreras-Rojas

    2014-12-01

    Full Text Available Justificación y objetivos: la heterogeneidad clínica del trastorno afectivo bipolar tipo I constituye uno de los principales desafíos en el diagnóstico de dicha enfermedad. Se necesitan múltiples fuentes de información que permitan definir el perfil clínico, demográfico y comorbilidad con otras enfermedades psiquiátricas. El estudio tiene como objetivo analizar las características clínicas, sociodemográficas y curso del trastorno, en familias costarricenses con uno o más miembros afectados. Métodos: se estudió a 167 pacientes con trastorno afectivo bipolar tipo I, los cuales provenían de diferentes familias de Costa Rica. El diagnóstico clínico se llevó a cabo mediante el proceso de mejor estimado diagnóstico y caracterización clínica dimensional a través de la vida. El análisis estadístico incluyó regresión logística, así como curvas de sobrevivencia de Kaplan-Meier. Resultados: 93 sujetos (55,7% fueron mujeres. La edad promedio al momento de la entrevista fue de 43,25 (DE=13,90. De los probandos con edad de inicio de enfermedad posterior a 20 años, 24 (14,0% familiares de primer grado tenían trastorno bipolar tipo I (x²=3,56, p=0,05; OR=1,7; 95% CI=1,2-2,7. La edad promedio de inicio para el trastorno por uso de sustancias en varones, fue 17 años (DE=0,4, versus 23 años (DE=3,2 para mujeres (x²=3.90, p=0.04. Varones con trastorno bipolar tipo y uso comórbido de sustancias presentaron menor edad de inicio de cualquier síntoma psiquiátrico, que aquellos sin TUS (x²=8,99, p=0,003. Conclusiones: el trastorno por uso de sustancias seguido por los trastornos de ansiedad, constituyen las condiciones comórbidas más frecuentes en el trastorno afectivo bipolar tipo I. La edad de inicio más temprana de trastorno afectivo bipolar tipo I en probandos, se asocia con mayor número de afectados por dicho trastorno en familiares de primer grado.

  2. Non-Invasive markers for hepatic fibrosis

    Directory of Open Access Journals (Sweden)

    Lal Priyanka

    2011-08-01

    Full Text Available Abstract With great advancements in the therapeutic modalities used for the treatment of chronic liver diseases, the accurate assessment of liver fibrosis is a vital need for successful individualized management of disease activity in patients. The lack of accurate, reproducible and easily applied methods for fibrosis assessment has been the major limitation in both the clinical management and for research in liver diseases. However, the problem of the development of biomarkers capable of non-invasive staging of fibrosis in the liver is difficult due to the fact that the process of fibrogenesis is a component of the normal healing response to injury, invasion by pathogens, and many other etiologic factors. Current non-invasive methods range from serum biomarker assays to advanced imaging techniques such as transient elastography and magnetic resonance imaging (MRI. Among non-invasive methods that gain strongest clinical foothold are FibroScan elastometry and serum-based APRI and FibroTest. There are many other tests that are not yet widely validated, but are none the less, promising. The rate of adoption of non-invasive diagnostic tests for liver fibrosis differs from country to country, but remains limited. At the present time, use of non-invasive procedures could be recommended as pre-screening that may allow physicians to narrow down the patients' population before definitive testing of liver fibrosis by biopsy of the liver. This review provides a systematic overview of these techniques, as well as both direct and indirect biomarkers based approaches used to stage fibrosis and covers recent developments in this rapidly advancing area.

  3. Telomerase and telomere length in pulmonary fibrosis.

    Science.gov (United States)

    Liu, Tianju; Ullenbruch, Matthew; Young Choi, Yoon; Yu, Hongfeng; Ding, Lin; Xaubet, Antoni; Pereda, Javier; Feghali-Bostwick, Carol A; Bitterman, Peter B; Henke, Craig A; Pardo, Annie; Selman, Moises; Phan, Sem H

    2013-08-01

    In addition to its expression in stem cells and many cancers, telomerase activity is transiently induced in murine bleomycin (BLM)-induced pulmonary fibrosis with increased levels of telomerase transcriptase (TERT) expression, which is essential for fibrosis. To extend these observations to human chronic fibrotic lung disease, we investigated the expression of telomerase activity in lung fibroblasts from patients with interstitial lung diseases (ILDs), including idiopathic pulmonary fibrosis (IPF). The results showed that telomerase activity was induced in more than 66% of IPF lung fibroblast samples, in comparison with less than 29% from control samples, some of which were obtained from lung cancer resections. Less than 4% of the human IPF lung fibroblast samples exhibited shortened telomeres, whereas less than 6% of peripheral blood leukocyte samples from patients with IPF or hypersensitivity pneumonitis demonstrated shortened telomeres. Moreover, shortened telomeres in late-generation telomerase RNA component knockout mice did not exert a significant effect on BLM-induced pulmonary fibrosis. In contrast, TERT knockout mice exhibited deficient fibrosis that was independent of telomere length. Finally, TERT expression was up-regulated by a histone deacetylase inhibitor, while the induction of TERT in lung fibroblasts was associated with the binding of acetylated histone H3K9 to the TERT promoter region. These findings indicate that significant telomerase induction was evident in fibroblasts from fibrotic murine lungs and a majority of IPF lung samples, whereas telomere shortening was not a common finding in the human blood and lung fibroblast samples. Notably, the animal studies indicated that the pathogenesis of pulmonary fibrosis was independent of telomere length.

  4. Stigma and cystic fibrosis Estigma y fibrosis cística Estigma e fibrose c��stica

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    Tainá Maues Peluci Pizzignacco

    2010-02-01

    Full Text Available Cystic Fibrosis (CF, also known as Mucoviscidosis, is a chronic disease of autosomal recessive origin and so far incurable. This analysis considers some characteristics of patients and family members that indicate it is a stigmatizing disease. The CF stigma’s impact on the lives of children and adolescents can affect treatment adherence, socialization, family relationships and the formation of their life histories, with direct consequences on their quality of life.La fibrosis cística (FC, también conocida como mucoviscidosis, es una enfermedad crónica de origen autosómica recesiva y, hasta el momento, incurable. La presente reflexión presenta consideraciones a respecto de algunas características que acompañan a pacientes y familiares, permitiendo comprenderla como enfermedad que estigmatiza. Las repercusiones del estigma en la vida de niños y adolescentes con FC pueden interferir en la adhesión al tratamiento, en el proceso de socialización, en la relación con los familiares y en la formación de su biografía, con reflejo directo en la calidad de vida.A fibrose cística (FC, também conhecida como mucoviscidose, é doença crônica de origem autossômica recessiva e, até o momento, incurável. A presente reflexão traz considerações a respeito de algumas características que acompanham pacientes e familiares, permitindo compreendê-la como doença estigmatizante. As repercussões do estigma na vida de crianças e adolescentes com FC podem implicar na adesão ao tratamento, no processo de socialização, na relação com os familiares e na formação de sua biografia, com reflexo direto em sua qualidade de vida.

  5. [VITAMIN D AND CHRONIC LUNG COLONIZATION IN PEDIATRIC AND YOUNG ADULTS CYSTIC FIBROSIS PATIENTS].

    Science.gov (United States)

    González Jiménez, David; Muñoz Codoceo, Rosana; Garriga García, María; Molina Arias, Manuel; Alvarez Beltran, Marina; García Romero, Ruth; Martínez Costa, Cecilia; Meavilla Olivas, Silvia María; Peña Quintana, Luis; Gallego Gutierrez, Silvia; Marugan de Miguelsanz, Jose Manuel; Suarez Cortina, Lucrecia; Castejón Ponce, Esperanza Natividad; Leis Trabazo, Rosaura; Martín Cruz, Fayna; Díaz Martín, Juan Jose; Bousoño García, Carlos

    2015-10-01

    Introducción y objetivos: conocer la situación en la que se encuentran los pacientes con fibrosis quística en relación con sus niveles de vitamina D y su asociación con las colonizaciones pulmonares crónicas. Material y métodos: estudio multicéntrico transversal. Participaron 12 hospitales nacionales. De noviembre a abril del 2012 al 2014 se incluyeron 377 pacientes con fibrosis quística. Se consideraron insuficientes niveles de vitamina D vitamina D. Se observó una correlación inversa entre edad y niveles de vitamina D (r = -0,20 p vitamina D más altos. Los niveles de vitamina D presentaron una correlación inversa con el número de colonizaciones pulmonares (r = -0,16 p = 0,0015). Ajustando por edad, función pancreática y diagnóstico mediante cribado, la colonización por S. Aureus en menores de seis años y por Pseudomonas sp. en los mayores de esa edad, incrementaban el riesgo de presentar niveles insuficientes de vitamina D: OR 3,17 (IC95% 1,32-7,61) (p=0,010) y OR 3,77 (IC95% 1,37- 10,37)(p = 0,010), respectivamente. Conclusiones: a pesar de una suplementación adecuada, más de la mitad de nuestros pacientes no alcanzan niveles óptimos de vitamina D. La colonización crónica por Pseudomonas sp. en escolares y adolescentes y por S. Aureus en lactantes y preescolares se asocia de forma independiente con la deficiencia de vitamina D.

  6. Genetics of Cystic Fibrosis: Clinical Implications.

    Science.gov (United States)

    Egan, Marie E

    2016-03-01

    Cystic fibrosis (CF) is a common life-shortening autosomal recessive genetic disorder caused by mutations in the gene that encodes for the cystic fibrosis transmembrane conductance regulator protein (CFTR). Almost 2000 variants in the CFTR gene have been identified. The mutational classes are based on the functional consequences on CFTR. New therapies are being developed to target mutant CFTR and restore CFTR function. Understanding specific CF genotypes is essential for providing state-of-the art care to patients. In addition to the variation in CFTR genotype, there are several modifier genes that contribute to the respiratory phenotype.

  7. Diagnosis of Adult Patients with Cystic Fibrosis.

    Science.gov (United States)

    Nick, Jerry A; Nichols, David P

    2016-03-01

    The diagnosis of cystic fibrosis (CF) is being made with increasing frequency in adults. Patients with CF diagnosed in adulthood typically present with respiratory complaints, and often have recurrent or chronic airway infection. At the time of initial presentation individuals may appear to have clinical manifestation limited to a single organ, but with subclinical involvement of the respiratory tract. Adult-diagnosed patients have a good response to CF center care, and newly available cystic fibrosis transmembrane receptor-modulating therapies are promising for the treatment of residual function mutation, thus increasing the importance of the diagnosis in adults with unexplained bronchiectasis.

  8. Angiotensinogen Gene Transcription in Pulmonary Fibrosis

    Science.gov (United States)

    Uhal, Bruce D.; Dang, My-Trang T.; Li, Xiaopeng; Abdul-Hafez, Amal

    2012-01-01

    An established body of literature supports the hypothesis that activation of a local tissue angiotensin (ANG) system in the extravascular tissue compartment of the lungs is required for lung fibrogenesis. Transcriptional activation of the angiotensinogen (AGT) gene is believed to be a critical and necessary step in this activation. This paper summarizes the data in support of this theory and discusses transcriptional regulation of AGT, with an emphasis on lung AGT synthesis as a determinant of fibrosis severity. Genetic data linking AGT polymorphisms to the severity of disease in Idiopathic Pulmonary Fibrosis are also discussed. PMID:22500179

  9. Angiotensinogen Gene Transcription in Pulmonary Fibrosis

    Directory of Open Access Journals (Sweden)

    Bruce D. Uhal

    2012-01-01

    Full Text Available An established body of literature supports the hypothesis that activation of a local tissue angiotensin (ANG system in the extravascular tissue compartment of the lungs is required for lung fibrogenesis. Transcriptional activation of the angiotensinogen (AGT gene is believed to be a critical and necessary step in this activation. This paper summarizes the data in support of this theory and discusses transcriptional regulation of AGT, with an emphasis on lung AGT synthesis as a determinant of fibrosis severity. Genetic data linking AGT polymorphisms to the severity of disease in Idiopathic Pulmonary Fibrosis are also discussed.

  10. The Role of PPARs in Lung Fibrosis

    Directory of Open Access Journals (Sweden)

    Heather F. Lakatos

    2007-01-01

    wound healing. PPARβ/δ agonists inhibit lung fibroblast proliferation and enhance the antifibrotic properties of PPARγ agonists. PPARγ ligands oppose the profibrotic effect of TGF-β, which induces differentiation of fibroblasts to myofibroblasts, a critical effector cell in fibrosis. PPARγ ligands, including the thiazolidinedione class of antidiabetic drugs, effectively inhibit lung fibrosis in vitro and in animal models. The clinical availability of potent and selective PPARα and PPARγ agonists should facilitate rapid development of successful treatment strategies based on current and ongoing research.

  11. The cystic fibrosis of exocrine pancreas

    DEFF Research Database (Denmark)

    Wilschanski, Michael; Novak, Ivana

    2013-01-01

    The cystic fibrosis transmembrane conductance regulator (CFTR) protein is highly expressed in the pancreatic duct epithelia and permits anions and water to enter the ductal lumen. This results in an increased volume of alkaline fluid allowing the highly concentrated proteins secreted by the acinar...... cells to remain in a soluble state. This work will expound on the pathophysiology and pathology caused by the malfunctioning CFTR protein with special reference to ion transport and acid-base abnormalities both in humans and animal models. We will also discuss the relationship between cystic fibrosis...

  12. Doxycycline Attenuated Pulmonary Fibrosis Induced by Bleomycin in Mice

    OpenAIRE

    Fujita, Masaki; Ye, Qing; Ouchi, Hiroshi; Harada, Eiji; Inoshima, Ichiro; Kuwano, Kazuyoshi; Nakanishi, Yoichi

    2006-01-01

    The administration of doxycycline prior to bleomycin in mice attenuated pulmonary fibrosis. Bronchoalveolar neutrophil influx and gelatinase activity, but not caseinolytic activity, were attenuated by doxycycline. Established fibrosis was not affected by doxycycline. Thus, doxycycline might be useful for slowing down pulmonary fibrosis by biological activity other than antibacterial activity.

  13. Living with Cystic Fibrosis: A Guide for the Young Adult.

    Science.gov (United States)

    Cystic Fibrosis Foundation, Atlanta, GA.

    Intended for the young adult with cystic fibrosis, the booklet provides information on dealing with problems and on advances in treatment and detection related to the disease. Addressed are the following topics: description of cystic fibrosis; inheritance of cystic fibrosis; early diagnosis; friends, careers, and other matters; treatment;…

  14. 78 FR 26681 - Medical Criteria for Evaluating Cystic Fibrosis

    Science.gov (United States)

    2013-05-07

    ... ADMINISTRATION RIN 0960-AF58 Medical Criteria for Evaluating Cystic Fibrosis AGENCY: Social Security....04 to evaluate claims involving cystic fibrosis in adults and children under titles II and XVI of the... information on the disability program. 2. Information for individuals with cystic fibrosis who apply...

  15. [Endomyocardial fibrosis with massive calcification of the left ventricle].

    Science.gov (United States)

    Trigo, Joana; Camacho, Ana; Gago, Paula; Candeias, Rui; Santos, Walter; Marques, Nuno; Matos, Pedro; Brandão, Victor; Gomes, Veloso

    2010-03-01

    Endomyocardial fibrosis is a rare disease, endemic in tropical countries. It is characterized by fibrosis of the endocardium that can extend to myocardium. Important calcification of the endocardium is rare with only a few cases reported in the literature. We report a case of endomyocardial fibrosis in a european caucasian patient, associated with massive calcification of left ventricle.

  16. Neutropenia relacionada con la quimioterapia en tumores sólidos. Hospital General Universitario Camilo Cienfuegos Enero 2009- 2013

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    Yania Luisa Jiménez Madrigal

    2015-09-01

    Full Text Available Se realizó un estudio descriptivo de corte transversal y retrospectivo referente al comportamiento de la neutropenia relacionada con la quimioterapia en tumores sólidos en el período comprendido Enero 2009- 2013 atendido en el servicio de oncología del Hospital General Universitario Camilo Cienfuegos de la provincia Sancti-Spíritus. La población la conformaron 230 enfermos neutropénicos relacionados con la quimioterapia. La investigación describe de forma general la relación existente entre la neutropenia con la quimioterapia, localización tumoral, entidades nosológicas asociadas, edad, sexo, tratamientos quimioterapéuticos y antibióticos más utilizados. Pudo observarse que existe alta incidencia de neutropenia relacionada con la quimioterapia en pacientes con cáncer de pulmón, el grupo más afectado fue el de 50-59 años predominando el sexo femenino con un alto índice de hipertensos asociados. Los tratamientos más utilizados fueron Etopóxido-Platino y Ceftrixone-Amikacina.

  17. Efectos de la putrescina en diferentes ciclos de selección masal divergente del maíz Harinoso Mosquera I Sin. 2 afectados por las heladas Efectos de la putrescina en diferentes ciclos de selección masal divergente del maíz Harinoso Mosquera I Sin. 2 afectados por las heladas

    Directory of Open Access Journals (Sweden)

    Córdoba Carlos Vicente

    1990-06-01

    Full Text Available

    During 1989, at the Tibaitata Research Center, an experiment plot was carried out. On cycles 6, 9, 12 and 15 of prolific and non prolific corn sub-populations which treated with 0, 1000, 2000 and 4000 Mm of putrescine. Six hours before aplication, the corn plants suffered chilling effects (-1.50 C/4 hr. As consecuence of chilling, the growth in height was suspended, the ear shoots and yield grain decreased. However putrescine application reverted the damage caused hy chilling and significative differences were observed in height and yield grain. Cicle 6 in prolific sub population was noticeable, in which 1000, 2000, and 4000 Mm of putrescine yielded 6.75, 5.24 and 5.63 Ton/ha. The Harinoso Mosquera I. Sin. 2 or cycle cero, showed a higber sensibility to chilling and the answer to putrescine application was almost negligeable.

    En 1989, en el C.I. Tibaitata, en un ensayo de bloques al azar se aplicó: 0., 1000, 2000 Y 4000 Mm de Potrescina (Put a los ciclos 6, 9, 12 Y 15 de subpoblaciones prolíficas y no prolíficas de maíz, que 6 horas antes habían sufrido los efectos de una helada (-1.50 C/4 hr. Como consecuencia del enfriamiento, el crecimiento en altura se suspendió, las mazorcas/planta florecidas que llenaron grano, y el reridimiento, disminuyeron; pero la Put aplicada revertió los daños causados por la helada, presentándose diferencias en la altura y rendimiento. Se destaca el ciclo 6 de la subpoblación prolífica, en el que con dosis de 1000, 2000 y 4000 Mm se obtuvieron rendimientos de ó.75, 5.24, y 5.ó3 Ton/ha. El maíz progenitor (ciclo 0 presentó una gran sensibilidad a la helada, y casi nula respuesta a Put.

  18. Global analysis of gene expression in pulmonary fibrosis reveals distinct programs regulating lung inflammation and fibrosis

    Science.gov (United States)

    Kaminski, Naftali; Allard, John D.; Pittet, Jean F.; Zuo, Fengrong; Griffiths, Mark J. D.; Morris, David; Huang, Xiaozhu; Sheppard, Dean; Heller, Renu A.

    2000-02-01

    The molecular mechanisms of pulmonary fibrosis are poorly understood. We have used oligonucleotide arrays to analyze the gene expression programs that underlie pulmonary fibrosis in response to bleomycin, a drug that causes lung inflammation and fibrosis, in two strains of susceptible mice (129 and C57BL/6). We then compared the gene expression patterns in these mice with 129 mice carrying a null mutation in the epithelial-restricted integrin 6 subunit (6/-), which develop inflammation but are protected from pulmonary fibrosis. Cluster analysis identified two distinct groups of genes involved in the inflammatory and fibrotic responses. Analysis of gene expression at multiple time points after bleomycin administration revealed sequential induction of subsets of genes that characterize each response. The availability of this comprehensive data set should accelerate the development of more effective strategies for intervention at the various stages in the development of fibrotic diseases of the lungs and other organs.

  19. Progressive fibrosis of the quadriceps muscle FIBROSIS PROGRESIVA DEL CUÁDRICEPS

    OpenAIRE

    Enrique Vergara-Amador; Jorge Andrés Largo González

    2011-01-01

    Background. Fibrosis of the quadriceps in children is a frequently reported pathology which is associated with antibiotics having been injected into the thigh. This study presents a series of patients having a common background of having had a single pentavalent vaccine dose or injectable vitamin K and presenting progressive fibrosis of the quadriceps muscle. Materials and methods. Seven children were found who had progressive unilateral retraction of the knee. Six of them had a background of...

  20. Correlación clinicorradiográfica de la fibrosis epidural en el síndrome poslaminectomía mediante epidurografía Clinical/radiography correlation of epidural fibrosis in postlaminectomy syndrome by epidurography

    Directory of Open Access Journals (Sweden)

    G. Cardona

    2010-05-01

    Full Text Available Introducción: La epidurografía es un recurso invasivo que evalúa deformidades y eventual ocupación del espacio epidural, además de ser un estudio fácilmente realizable, confiable, económico y que forma parte del algoritmo de manejo del síndrome poslaminectomía. Objetivo: Verificar si la localización y la extensión de la fibrosis epidural se correlacionan con el cuadro clínico presente en los pacientes con síndrome poslaminectomía. Material y métodos: Se incluyó a 30 pacientes con diagnóstico de síndrome poslaminectomía lumbar divididos en dos grupos: grupo A (n=15 pacientes con lumbalgia y dolor neuropático del miembro pélvico unilateral y grupo B (n=15 pacientes con lumbalgia con dolor neuropático bilateral. En todos los pacientes se realizó el abordaje caudal bajo control fluoroscópico en decúbito prono con aguja Tuohy #17. La epidurografía se realizó inyectando medio de contraste ioversol al 63%, acetato de metilprednisolona y lidocaína al 2%. Se relacionó la localización de la fibrosis con el cuadro clínico además de valorar la intensidad del dolor utilizando la escala visual analógica previa al procedimiento a las 2 y a las 4 semanas de realizado este. Resultados: Del total de la población, el 53,3% (16 tuvo correlación clinicorradiográfica, lo cual no se considera estadísticamente significativo (p=0,259. En relación con la evaluación de la escala visual analógica inicial se observó disminución significativa a las 2 semanas de realizado el procedimiento (p=0,0001. Conclusión: La epidurografía diagnóstica no tiene utilidad al momento de intentar correlacionar el sitio de fibrosis epidural con la presentación clínica en los pacientes con síndrome poslaminectomía.Introduction: Epidurography is an invasive technique that evaluates deformities and eventual occupation of the epidural space, besides being easy to perform, reliable and inexpensive, is part of the management algorithm of post

  1. Craniofacial morphology in children with cystic fibrosis.

    Science.gov (United States)

    Hellsing, E; Brattström, V; Strandvik, B

    1992-04-01

    Cystic fibrosis (CF) is a hereditary metabolic disorder with clinical symptoms of abnormal mucus production. This blocks the airways, gives pancreatic insufficiency, and increases sweat electrolytes. The progressive respiratory disease often leads to respiratory insufficiency and cor pulmonale. The aim of the present investigation was to examine the facial morphology in children with cystic fibrosis. The sample comprised 11 children with cystic fibrosis, who were divided in two groups, one with gastrointestinal disorders and the other with predominantly respiratory insufficiency. Eleven healthy children with normal occlusions were selected as controls. Lateral skull radiographs obtained in natural head posture were digitized, and linear and angular variables for the different groups calculated and compared statistically. The cystic fibrosis group showed open bite, decreased posterior facial height, increased mandibular and craniocervical inclination. Additionally, within the CF-group, the children with respiratory insufficiency differed more from the controls than the children with gastrointestinal disorders. Despite the small number of subjects, the facial morphology of the CF children showed a similar pattern to that of children with nasal respiratory obstruction due to enlarged adenoids or tonsils.

  2. Cystic fibrosis : terminology and diagnostic algorithms

    NARCIS (Netherlands)

    De Boeck, K; Wilschanski, M; Castellani, C; Taylor, C; Cuppens, H; Dodge, J; Sinaasappel, M

    2006-01-01

    There is great heterogeneity in the clinical manifestations of cystic fibrosis (CF). Some patients may have all the classical manifestations of CF from infancy and have a relatively poor prognosis, while others have much milder or even atypical disease manifestations and still carry mutations on eac

  3. Inhalation of antibiotics in cystic fibrosis

    NARCIS (Netherlands)

    Touw, D J; Brimicombe, R W; Hodson, M E; Heijerman, H G; Bakker, W

    1995-01-01

    Aerosol administration of antipseudomonal antibiotics is commonly used in cystic fibrosis. However, its contribution to the improvement of lung function, infection and quality of life is not well-established. All articles published from 1965 until the present time concerning the inhalation of antibi

  4. Agmatine attenuates silica-induced pulmonary fibrosis.

    Science.gov (United States)

    El-Agamy, D S; Sharawy, M H; Ammar, E M

    2014-06-01

    There is a large body of evidence that nitric oxide (NO) formation is implicated in mediating silica-induced pulmonary fibrosis. As a reactive free radical, NO may not only contribute to lung parenchymal tissue injury but also has the ability to combine with superoxide and form a highly reactive toxic species peroxynitrite that can induce extensive cellular toxicity in the lung tissues. This study aimed to explore the effect of agmatine, a known NO synthase inhibitor, on silica-induced pulmonary fibrosis in rats. Male Sprague Dawley rats were treated with agmatine for 60 days following a single intranasal instillation of silica suspension (50 mg in 0.1 ml saline/rat). The results revealed that agmatine attenuated silica-induced lung inflammation as it decreased the lung wet/dry weight ratio, protein concentration, and the accumulation of the inflammatory cells in the bronchoalveolar lavage fluid. Agmatine showed antifibrotic activity as it decreased total hydroxyproline content of the lung and reduced silica-mediated lung inflammation and fibrosis in lung histopathological specimen. In addition, agmatine significantly increased superoxide dismutase (p pulmonary nitrite/nitrate as well as tumor necrosis factor α. Collectively, these results demonstrate the protective effects of agmatine against the silica-induced lung fibrosis that may be attributed to its ability to counteract the NO production, lipid peroxidation, and regulate cytokine effects.

  5. Pirfenidone treatment in idiopathic pulmonary fibrosis

    DEFF Research Database (Denmark)

    Salih, Goran Nadir; Shaker, Saher Burhan; Madsen, Helle Dall;

    2016-01-01

    BACKGROUND: Pirfenidone was approved by the European Medicines Agency and introduced in most European countries in 2011 for treatment of idiopathic pulmonary fibrosis (IPF). OBJECTIVE: To describe the national Danish experiences of pirfenidone treatment for IPF during 30 months with respect...

  6. [Retroperitoneal fibrosis and multiple myeloma: fortuitous association?].

    Science.gov (United States)

    Sinapi, I; Caers, J; Connerotte, T; Koutaissoff, S; Lambert, M

    2010-05-01

    We report a 59-year-old man presenting with retroperitoneal fibrosis (RF) associated with IgG lambda multiple myeloma. Recent clinical and immunohistochemical findings suggest that RF might be a particular expression of plasma cell/lymphoid dyscrasia, and that this association is not merely fortuitous. We review the pathophysiological evidence supporting this hypothesis.

  7. Barriers to adherence in cystic fibrosis

    DEFF Research Database (Denmark)

    Bregnballe, Vibeke; Schiøtz, Peter Oluf

    2012-01-01

    Danish patients with cystic fibrosis aged 14 to 25 years and their parents. Conclusions: The present study showed that the majority of adolescents with CF and their parents experienced barriers to treatment adherence. Patients and parents agreed that the three most common barriers encountered lack...

  8. Nutritional assessment in children with cystic fibrosis

    Science.gov (United States)

    Optimal nutrition, including consuming 35–40% of calories (kcal) as fat, is a vital part of the management of cystic fibrosis (CF), and involves accurate assessment of dietary intake. We compared 3 methods of nutritional assessment in 8– to 14-year-old children (n=20) with CF: 1) a 24-h Dietary Reca...

  9. Zinc supplementation in children with cystic fibrosis

    Science.gov (United States)

    Cystic fibrosis (CF) leads to malabsorption of macro- and micronutrients. Symptomatic zinc deficiency has been reported in CF but little is known about zinc homeostasis in children with CF. Zinc supplementation (Zn suppl) is increasingly common in children with CF but it is not without theoretcial r...

  10. Nephrogenic systemic fibrosis: history and epidemiology

    DEFF Research Database (Denmark)

    Thomsen, Henrik S

    2009-01-01

    Nephrogenic systemic fibrosis (NSF) is a new disease; the first case was diagnosed in 1997. It took 9 years before an association between NSF and gadolinium-based contrast agents (Gd-CAs) was identified. Gadolinium has several advantages for use in relation to enhanced MRI, but it is also a toxic...

  11. The lived experience with idiopathic pulmonary fibrosis

    DEFF Research Database (Denmark)

    Overgaard, Dorthe; Kaldan, Gudrun; Marsaa, Kristoffer

    2016-01-01

    The disease course in idiopathic pulmonary fibrosis (IPF) is variable, but patients experience a progressive decline in lung function and increased symptom burden leading to death. Little is known about the patients' experience and their needs during the disease course or about the burden on family...

  12. Intestinal organoids as model for cystic fibrosis

    NARCIS (Netherlands)

    Dekkers, J.F.

    2015-01-01

    Recent advances in adult stem cell culture technology have enabled long-term in vitro expansion of intestinal organoids or ‘mini-guts’. In this thesis, we used the organoid model to develop a novel assay to measure function of CFTR, the protein mutated in subjects with cystic fibrosis (CF). This met

  13. Usefulness of an index score as a predictor of hepatic fibrosis in obese patients undergoing bariatric surgery Utilidad de un índice de puntuación como predictor de fibrosis hepática en pacientes obesos sometidos a cirugía bariátrica

    Directory of Open Access Journals (Sweden)

    R. Díez Rodríguez

    2009-08-01

    Full Text Available Objective: to evaluate the usefulness of a non-invasive clinical score to predict liver fibrosis in the steatosis associated with morbid obesity. Patients and methods: we included 88 patients, who underwent bariatric surgery in the Sanitary Area of León, Spain, and who showed a liver biopsy with steatosis greater than 5%. This is a retrospective study in which the rate of fibrosis is calculated from tests performed during the preoperative period, and is then compared to data from intraoperative hepatic biopsies. The analysis population was grouped according to the presence of advanced fibrosis in the liver biopsy (grade 3-4 or its absence (grade 0-2. The cutoff used for diagnosing advanced fibrosis was 0.676 (high cutoff point, and the cutoff point to exclude advanced fibrosis was -1.455 (low cutoff. Results: the prevalence of advanced fibrosis in the histological samples was 5.5%, and 65.9% of patients had no fibrosis. The cutoff for a low negative predictive value was 100%, and sensitivity was 100%. The cutoff point for a high positive predictive value was 1.7%, and specificity was 31.3%. Conclusions: this scoring system for morbidly obese patients eligible for bariatric surgery allows to identify those without advanced fibrosis, but cannot predict who may have advanced fibrosis.Objetivo: evaluar la utilidad de un índice de puntuación clínica no invasivo para predecir fibrosis hepática en la esteatosis asociada a la obesidad mórbida. Pacientes y métodos: se incluyeron 88 pacientes, intervenidos de cirugía bariátrica en el área sanitaria de León, que presentaron en la biopsia hepática una esteatosis mayor del 5%. Se trata de un estudio retrospectivo en el que se calculó el índice de fibrosis a partir de los datos analíticos del preoperatorio, y se comparó su resultado con los datos de la biopsia hepática intraoperatoria realizada. Para el análisis los pacientes fueron agrupados según presentaban en la biopsia hep

  14. Collagen immunostains can distinguish capsular fibrous tissue from septal fibrosis and may help stage liver fibrosis.

    Science.gov (United States)

    Chen, Wei; Rock, Jonathan B; Yearsley, Martha M; Hanje, A James; Frankel, Wendy L

    2014-01-01

    Core-needle biopsy remains essential for diagnosis of cirrhosis; however, evaluation of fibrosis in such biopsies is often challenging due to the fragmented nature of cirrhotic liver specimens. It is also common to see portions of liver capsules present in the biopsy which adds to the diagnostic challenge. The distinction between capsular/subcapsular fibrous tissue and septal fibrosis is critical to avoid potential overstaging of liver fibrosis. We compared the differential immunostaining in liver capsular and septal areas for collagens III, IV, V, VI, vitronectin, laminin, Orcein, and Trichrome in 15 whole sections of explanted cirrhotic livers and 5 simulated liver biopsies. Collagens III, IV, V, VI, Trichrome, and Orcein show distinct staining patterns in capsular fibrous tissue and septal fibrosis. Collagen IV shows strong diffuse septal staining and consistently weak to negative capsular staining. Collagens III and VI stain similar to IV for septal fibrosis, whereas collagen V, Trichrome, and Orcein show strong staining in both areas. Collagen IV, possibly with III or VI in addition to the routine Trichrome and hematoxylin and eosin stain, is useful in differentiating capsular fibrous tissue from septal fibrosis on challenging and fragmented liver biopsies.

  15. Tratamiento del herpes simple labial con láser de baja potencia

    Directory of Open Access Journals (Sweden)

    Bianka M. González

    2008-06-01

    Full Text Available Introducción: El herpes simple es causado por Herpes virus hominis, que produce una afección eruptiva aguda de uno o más grupos de vesículas sobre una base eritematosa; puede localizarse en cualquier parte del cuerpo preferentemente en la boca y los genitales precedida y acompañada de ardor y prurito.Objetivos: Se evaluó la efectividad del tratamiento con láser de baja potencia en relación al tratamiento convencional según la evolución clínica, desaparición de síntomas y signos en los pacientes con herpes simple labial. Metodología: La muestra comprendió 60 pacientes que asistieron a la consulta de dermatología por dicha entidad, en cuatro policlínicos de la capital cubana en el transcurso del año 2006. Se llevó a cabo un estudio explicativo de tipo experimental y prospectivo, donde se aplicaron dos tipos de tratamientos, el convencional con aciclovir por vía oral y tópica y el láser de baja potencia, como único tratamiento. Resultados: Hubo predominio del sexo femenino con 78.3%; el grupo de edad más afectado estaba entre 28 y 37 años (45.5%, la media del número de sesiones necesarias para la desaparición de síntomas y signos en el tratamiento convencional fue 7.3 sesiones mientras que en el tratamiento con láser fue 5.2 sesiones y se pudo evidenciar que sí existen diferencias estadísticamente significativas entre las medias de ambos tratamientos; en cuanto a la evolución clínica ambos tratamientos tuvieron mayor porcentaje de pacientes curados. Se recomienda evaluar a largo plazo la presencia de recidivas y que se utilice esta técnica como una opción de tratamiento para mejorar la calidad de vida de los pacientes afectados.

  16. Déficit en Habilidades Sociales en Niños con Trastorno por Déficit de Atención-Hiperactividad, Evaluados con la Escala basc/ Social Skills Deficits in Children Diagnosed With Attention Deficit Hyperactivity Disorder Evaluated With the BASC Escale/ Déficit em Habilidades Sociais em Crianças com Transtorno por Déficit de Atenção-Hiperatividade, Avaliados com a Escala BASC

    Directory of Open Access Journals (Sweden)

    Pedro Puentes Rozo

    2014-07-01

    Full Text Available Se analizaron las Habilidades Sociales (HS de 159 niños de ambos sexos, escolarizados, con Trastorno por Déficit de Atención-Hiperactividad (TDAH, entre los 6 y los 11 años de edad. La valoración de las HS se realizó con la escala Behavioral Assessment System for Children para padres y maestros. Los resultados mostraron que los niños con TDAH presentan puntajes más bajos en las dimensiones de habilidades sociales como escuchar, esperar turnos, reconocer señales sociales y tener capacidad de adaptabilidad. Sin embargo, en compañerismo, los participantes con TDAH combinado poseen habilidades semejantes a los no afectados. Esto replantea lo encontrado en la mayoría de los estudios en donde únicamente se evidencian deficiencias.

  17. Nutrient Status of Adults with Cystic Fibrosis

    Science.gov (United States)

    GORDON, CATHERINE M.; ANDERSON, ELLEN J.; HERLYN, KAREN; HUBBARD, JANE L.; PIZZO, ANGELA; GELBARD, RONDI; LAPEY, ALLEN; MERKEL, PETER A.

    2011-01-01

    Nutrition is thought to influence disease status in patients with cystic fibrosis (CF). This cross-sectional study sought to evaluate nutrient intake and anthropometric data from 64 adult outpatients with cystic fibrosis. Nutrient intake from food and supplements was compared with the Dietary Reference Intakes for 16 nutrients and outcomes influenced by nutritional status. Attention was given to vitamin D and calcium given potential skeletal implications due to cystic fibrosis. Measurements included weight, height, body composition, pulmonary function, and serum metabolic parameters. Participants were interviewed about dietary intake, supplement use, pulmonary function, sunlight exposure, and pain. The participants’ mean body mass index (±standard deviation) was 21.8±4.9 and pulmonary function tests were normal. Seventy-eight percent used pancreatic enzyme replacement for malabsorption. Vitamin D deficiency [25-hydroxyvitamin D (25OHD)<37.5 nmol/L] was common: 25 (39%) were deficient despite adequate vitamin D intake. Lipid profiles were normal in the majority, even though total and saturated fat consumption represented 33.0% and 16.8% of energy intake, respectively. Reported protein intake represented 16.9% of total energy intake (range 10%–25%). For several nutrients, including vitamin D and calcium, intake from food and supplements in many participants exceeded recommended Tolerable Upper Intake Levels. Among adults with cystic fibrosis, vitamin D deficiency was common despite reported adequate intake, and lipid profiles were normal despite a relatively high fat intake. Mean protein consumption was adequate, but the range of intake was concerning, as both inadequate or excessive intake may have deleterious skeletal effects. These findings call into question the applicability of established nutrient thresholds for patients with cystic fibrosis. PMID:18060897

  18. Fibrosis Quística: comparativa de tres cuestionarios de calidad de vida

    OpenAIRE

    2015-01-01

    Comprender cuáles son los determinantes de la calidad de vida relacionada con la salud (CVRS) en fibrosis quística (FQ) podría ayudar al desarrollo de intervenciones para mejorarla. Cómo refiera una persona cuál es su CVRS puede estar influido por muchas variables: demográficas, clínicas, psicológicas y sociales. Algunos de estos factores probablemente estén interrelacionados, pero es útil tenerlos en cuenta cuando se diseña, analiza e interpretan estudios que midan la CVRS, ya que pueden inf...

  19. Educar con significado o con sentido

    Directory of Open Access Journals (Sweden)

    José Joaquín García García

    2017-01-01

    Full Text Available Hoy día, tres tendencias influyen directamente en la conformación de la escuela. En primer lugar, el afán de encontrar la esencia de todo encumbró a la razón e hizo de la racionalidad el único valor a defender en las aulas. En segundo lugar, el sistema capitalista hizo lo mismo con aquello que tiene valor de uso y valor de cambio, es decir, con la mercancía, validando solo lo que puede tener una utilidad económica conocida o posible. Esto convirtió a la educación en un proceso para certificar y ganar dinero, desdibujando así su intención de formar personas. Finalmente, la visión masculina y eurocéntrica con su locura por quererlo dominar todo, y de pensar que el hombre era el dueño del planeta e inclusive la vida y el destino de los otros hombres, mutiló culturas y eliminó a la naturaleza de los currículums en los centros educativos.

  20. USO DEL TAMOXIFENO PARA TRATAMIENTO DE LA FIBROSIS SISTÉMICA NEFROGÉNICA

    Directory of Open Access Journals (Sweden)

    Enz PA

    2009-01-01

    Full Text Available La fibrosis sistémica nefrogénica (FSN es una entidad infrecuente, que se desarrolla en pacientes insuficientes renales en diálisis, vinculándose su etiopatogenia con diversos factores tales como el uso de gadolinio intravenoso, el antecedente de cirugías vasculares, etc.El Tamoxifeno, es un modulador selectivo (inhibición competitiva de los receptores estrogénicos, que posee propiedades antifibróticas las cuales se emplean para el tratamiento de entidades tales como: fibrosis retroperitoneal, esclerosis peritoneal asociada a diálisis peritoneal, tumores desmoides y en algunos casos de esclerodermia.En el presente articulo presentamos la hipótesis original de que el tamoxifeno, por su propiedades fibrinolíticas, podría ser una terapia no inmunosupresora potencialmente útil para el tratamiento de la fibrosis sistémica nefrogénica, evitándose desde ya su uso en pacientes portadores de contraindicaciones para recibirlo.

  1. Pulmonary fibrosis and emphysema: Is the emphysema type associated with the pattern of fibrosis?

    Institute of Scientific and Technical Information of China (English)

    Anastasia; Oikonomou; Paraskevi; Mintzopoulou; Argyris; Tzouvelekis; Petros; Zezos; George; Zacharis; Anastasios; Koutsopoulos; Demosthenes; Bouros; Panos; Prassopoulos

    2015-01-01

    AIM: To investigate whether the predominant emphysema type is associated with the high resolution computed tomography(HRCT) pattern of fibrosis in combined pulmonary fibrosis and emphysema(CPFE).METHODS: Fifty-three smokers with upper lobe emphysema and lower lobe pulmonary fibrosis on- HRCT-were retrospectively evaluated. Patients were stratified into 3 groups according to the predominant type of emphysema: Centrilobular(CLE), paraseptal(PSE), CLE = PSE. Patients were also stratified into 3 other groups according to the predominant type of fibrosis on HRCT: Typical usual interstitial pneumonia(UIP), probable UIP and nonspecific interstitial pneumonia(NSIP). HRCTs were scored at 5 predetermined levels for the coarseness of fibrosis(Coarseness), extent of emphysema(emphysema), extent of interstitial lung disease(Tot Ext ILD), extent of reticular pattern not otherwise specified(Ret NOS), extent of ground glass opacity with traction bronchiectasis(extG GOBx), extent of pure ground glass opacity and extent of honeycombing. HRCT mean scores, pulmonary function tests, diffusion capacity(DLCO) and systolic pulmonary arterial pressure were compared among the groups.RESULTS: The predominant type of emphysema was strongly correlated with the predominant type of fibrosis. The centrilobular emphysema group exhibited a significantly higher extent of emphysema(P P > 0.000). Although the pulmonary arterial pressure was higher in typical UIP group relative to the NSIP group, the difference was not statistically significant.CONCLUSION: In CPFE patients, paraseptal emphysema is associated more with UIP-HRCT pattern and higher extent of fibrosis than centrilobular emphysema.

  2. Caracterización de los pacientes con cirrosis hepática atendidos en Las Tunas

    Directory of Open Access Journals (Sweden)

    Alina Torreblanca Xiques

    2014-11-01

    Full Text Available Se realizó un estudio observacional descriptivo, con el objetivo de caracterizar los pacientes con cirrosis hepática (CH, atendidos en el servicio de gastroenterología del Hospital General Docente “Dr. Ernesto Guevara de la Serna”, Las Tunas, Cuba, en el período comprendido entre febrero de 2012 y febrero de 2014. Se estudiaron 55 pacientes con cirrosis hepática diagnosticada por laparoscopia. Predominaron los pacientes del grupo de edad entre 50 y 59 años, siendo el sexo masculino el más afectado. Las principales manifestaciones clínicas que se apreciaron en estos pacientes fueron la ascitis y los edemas en miembros inferiores, así como las complicaciones más frecuentemente encontradas, la ascitis y la hemorragia digestiva alta. El alcoholismo y la etiología viral por virus C se presentaron con mayor frecuencia, predominando el primero. En el mayor por ciento de los pacientes debutó la enfermedad con al menos una complicación

  3. Propuesta de intervención para fomentar la inteligencia emocional en una alumna con un trastorno del desarrollo intelectual leve de 5º de Primaria: un estudio de caso

    OpenAIRE

    Palomino-Cardona, Concepción

    2015-01-01

    La Inteligencia Emocional favorece las relaciones interpersonales y el autoconocimiento. Esto se ve afectado cuando un alumno presenta algún tipo de trastorno psicológico. Por ello el objetivo general es realizar una aproximación teórico-práctica del desarrollo de la Inteligencia Emocional en una alumna con un Trastorno del Desarrollo Intelectual leve. Para la consecución de este objetivo se ha analizado el marco legal vigente y, seguidamente, se ha elaborado un marco teórico que abarca el...

  4. Estudio de la prevalencia y posibles factores etiológicos relacionados con la Hipomineralización incisivo molar (mih) en un grupo de niños y adolescentes

    OpenAIRE

    Martínez Gómez, Teresita Patricia

    2014-01-01

    La hipomineralización incisivo molar (MIH) por sus siglas en inglés, es un defecto cualitativo del desarrollo del esmalte de uno o cuatro primeros molares permanentes, frecuentemente asociado con defecto en los incisivos. Clínicamente, se observa como una alteración en la translucides del esmalte, caracterizado por opacidades demarcadas cuyos bordes bien definidos separa claramente el esmalte afectado del normal adyacente, la zona cervical de la corona anatómica mantiene una aparaiencia y co...

  5. Stage scoring of liver fibrosis using Mueller matrix microscope

    Science.gov (United States)

    Zhou, Jialing; He, Honghui; Wang, Ye; Ma, Hui

    2016-10-01

    Liver fibrosis is a common pathological process of varied chronic liver diseases including alcoholic hepatitis, virus hepatitis, and so on. Accurate evaluation of liver fibrosis is necessary for effective therapy and a five-stage grading system was developed. Currently, experienced pathologists use stained liver biopsies to assess the degree of liver fibrosis. But it is difficult to obtain highly reproducible results because of huge discrepancy among different observers. Polarization imaging technique has the potential of scoring liver fibrosis since it is capable of probing the structural and optical properties of samples. Considering that the Mueller matrix measurement can provide comprehensive microstructural information of the tissues, in this paper, we apply the Mueller matrix microscope to human liver fibrosis slices in different fibrosis stages. We extract the valid regions and adopt the Mueller matrix polar decomposition (MMPD) and Mueller matrix transformation (MMT) parameters for quantitative analysis. We also use the Monte Carlo simulation to analyze the relationship between the microscopic Mueller matrix parameters and the characteristic structural changes during the fibrosis process. The experimental and Monte Carlo simulated results show good consistency. We get a positive correlation between the parameters and the stage of liver fibrosis. The results presented in this paper indicate that the Mueller matrix microscope can provide additional information for the detections and fibrosis scorings of liver tissues and has great potential in liver fibrosis diagnosis.

  6. Investigando con personas con dificultades de aprendizaje

    Directory of Open Access Journals (Sweden)

    Borja González Luna

    2013-12-01

    Full Text Available El artículo muestra los orígenes de lo que Walmsley (2008 denomina «investigación inclusiva». Para comprender qué se entiende por investigación inclusiva tenemos que remontarnos a los debates epistemológicos sobre las metodologías cuantitativas y cualitativas, acontecidos en la década de los 90, en torno a la revista Disability & Society. A partir de una síntesis de dichos debates, focalizados en el ámbito de la «discapacidad intelectual y del desarrollo», se exponen dos estrategias de colaboración con dicha población: a una aproximación etnográfica (de trabajo grupal, y b una aproximación biográfica (de trabajo individual. A continuación se esboza un posible diseño de trabajo de campo que intenta superar el paradigma cualitativo «clásico» con el objetivo de incluir a dicho colectivo más allá del rol de «sujetos de la investigación». Para finalizar se recoge el debate sobre la accesibilidad de los resultados de la investigación a los participantes en dichas investigaciones, y con ello la necesaria innovación en el ámbito de las «devoluciones» de los resultados, cuando se trata de incluir a personas que presentan limitaciones para la comprensión del lenguaje abstracto oral y/o escrito.

  7. Comportamiento de la cognición y comorbilidad en pacientes con infarto cerebral hospitalizados. Seychelles 2010-2011

    Directory of Open Access Journals (Sweden)

    Armando Carlos Roca Socarras

    2012-10-01

    Full Text Available Fundamento: el deterioro cognitivo y la alta comorbilidad son problemas de salud frecuentes en pacientes con enfermedad cerebrovascular. Objetivo: describir el comportamiento del deterioro cognitivo y de la comorbilidad en pacientes con infarto cerebral hospitalizados. Método: estudio descriptivo de 77 pacientes con diagnóstico de enfermedad cerebrovascular isquémica, ingresados entre abril del 2010 y diciembre del 2011, en el hospital general de Seychelles. Se analizó el comportamiento de esta enfermedad en relación con variables como: edad, presencia de enfermedades crónicas no transmisibles, deterioro cognitivo, años de estudios terminados, hemisferio cerebral afectado y tiempo de evolución. Resultados: el 68,9 % de los pacientes presentó deterior cognitivo; la edad media de estos fue de 74,2(±9,19 años frente a un 62,4(±14,2 para los que no tuvieron deterioro cognitivo. Se encontró una mayor frecuencia de enfermedades crónicas no transmisibles en los pacientes con deterioro cognitivo, con un índice de comorbilidad de Charlson en 2,11(±0,97. Se diagnosticaron con depresión 18 pacientes con deterioro cognitivo y 23 pacientes sin deterioro cognitivo. Conclusiones: más de la mitad de los pacientes hospitalizados presentó deterioro cognitivo, la mayor parte previo a la enfermedad cerebrovascular. En este grupo de pacientes predominó el sexo masculino, se observó un incremento de la edad, de la severidad de la depresión, así como de la frecuencia de enfermedades crónicas no transmisibles, e infarto de tamaño considerable, con respecto a los pacientes sin deterioro cognitivo.

  8. ROC curves in evaluation of serum fibrosis indices for hepatic fibrosis

    Institute of Scientific and Technical Information of China (English)

    Min Zheng; Wei-Min Cai; Hong-Lei Weng; Rong-Hua Liu

    2002-01-01

    AIM: Use Receiver operating characteristic (ROC) curves to find out the relationship between serum level of hyaluronic acid (HA), type Ⅲ procollagen (PCⅢ), N-terminal procollagen Ⅲ peptide (PⅢNP), laminin (LN), type Ⅳ collagen (C-Ⅳ) and hepatic fibrosis, as well as to determine their value in clinical practice.METHODS: 114 serum samples from chronic hepatitis patients were assayed for fibrosis indices including HA, PCⅢ, PⅢNP, LN and Ⅳ-C with radioimmunoassay (RIA). Liver biopsy was also performed in all these patients and the biopsy material was examined histopathologically.RESULTS: ROC curves analysis showed that area under the curve (AUC) of PⅢNP, HA, PCⅢ, C-Ⅳ and LN was 0.800,0.728, 0.727, 0.583 and 0.463, respectively. The analysis also showed that PⅢNP (r=0.452), HA (r=0.497) and PCⅢ (r=0.404) have greater diagnosis performances than C-Ⅳ (r=0.238) and LN (r=0.128) according to fibrosis staging. The sensitivity of HA plus PⅢNP was 55.1%, it was the most sensitive combination. Combined three or more than three indices that based on HA, the specificity was 100 %.Using combination assays can improve the specificity, but its sensitivity was not high. Serum fibrosis indices increased as the grade of inflammation aggravated. But only PⅢNP and PCⅢ had significant difference between G1 and G2 (PⅢNP: 13.16±8.07 VS8.32±5.09; PCⅢ: 164.22±65.69 VS 138.23±77.63). The coefficient correlation of the results of inflammation grade and fibrosis staging to HA was 0.525 and 0.553 respectively, that to PCⅢ, 0.446 and 0.412, that to LN, 0.234 and 0.194, and that to Ⅳ-C, 0.363 and 0.351, respectively.CONCLUSION: Serum fibrosis indices can indicate tendency of hepatic fibrosis, but it cannot replace liver biopsy. However, as diagnostic markers, more efficient serum fibrosis indices for the diagnosis of hepatic fibrosis need to be explored.

  9. Cystic fibrosis lung disease in adult patients.

    Science.gov (United States)

    Vender, Robert L

    2008-04-01

    As the longevity of all patients with cystic fibrosis (CF) continues to increase (median 2005 survival=36.8 years), more adult patients will be receiving their medical care from nonpediatric adult-care providers. Cystic fibrosis remains a fatal disease, with more than 80% of patients dying after the age of 18 years, and most deaths resulting from pulmonary disease. The changing epidemiology requires adult-care providers to become knowledgeable and competent in the clinical management of adults with CF. Physicians must understand the influence of specific genotype on phenotypic disease presentation and severity, the pathogenic factors determining lung disease onset and progression, the impact of comorbid disease factors such as CF-related diabetes and malnutrition upon lung disease severity, and the currently approved or standard accepted therapies used for chronic management of CF lung disease. This knowledge is critical to help alleviate morbidity and improve mortality for the rapidly expanding population of adults with CF.

  10. Cyclic Nucleotide Signalling in Kidney Fibrosis

    Directory of Open Access Journals (Sweden)

    Elisabeth Schinner

    2015-01-01

    Full Text Available Kidney fibrosis is an important factor for the progression of kidney diseases, e.g., diabetes mellitus induced kidney failure, glomerulosclerosis and nephritis resulting in chronic kidney disease or end-stage renal disease. Cyclic adenosine monophosphate (cAMP and cyclic guanosine monophosphate (cGMP were implicated to suppress several of the above mentioned renal diseases. In this review article, identified effects and mechanisms of cGMP and cAMP regarding renal fibrosis are summarized. These mechanisms include several signalling pathways of nitric oxide/ANP/guanylyl cyclases/cGMP-dependent protein kinase and cAMP/Epac/adenylyl cyclases/cAMP-dependent protein kinase. Furthermore, diverse possible drugs activating these pathways are discussed. From these diverse mechanisms it is expected that new pharmacological treatments will evolve for the therapy or even prevention of kidney failure.

  11. Lymphoplasmacytic Sclerosing Pancreatitis and Retroperitoneal Fibrosis

    Directory of Open Access Journals (Sweden)

    Nigel K. F. Koo Ng

    2008-01-01

    Full Text Available Although cases of lymphoplasmacytic sclerosing pancreatitis (LSP associated with idiopathic retroperitoneal fibrosis have been reported, the association is rare. We describe a 74-year-old man who presented with obstructive jaundice and weight loss. Nineteen months earlier, he had been diagnosed with idiopathic retroperitoneal fibrosis and treated with bilateral ureteric stents. Initial investigations were suggestive of a diagnosis of LSP, however, a malignant cause could not be ruled out. He underwent an exploratory laparotomy and frozen sections confirmed the diagnosis of LSP. An internal biliary bypass was performed using a Roux loop of jejunum, and the patient made an uneventful recovery. This case illustrates the difficulty in distinguishing LSP from pancreatic carcinoma preoperatively.

  12. Painful connections: densification versus fibrosis of fascia.

    Science.gov (United States)

    Pavan, Piero G; Stecco, Antonio; Stern, Robert; Stecco, Carla

    2014-01-01

    Deep fascia has long been considered a source of pain, secondary to nerve pain receptors becoming enmeshed within the pathological changes to which fascia are subject. Densification and fibrosis are among such changes. They can modify the mechanical properties of deep fasciae and damage the function of underlying muscles or organs. Distinguishing between these two different changes in fascia, and understanding the connective tissue matrix within fascia, together with the mechanical forces involved, will make it possible to assign more specific treatment modalities to relieve chronic pain syndromes. This review provides an overall description of deep fasciae and the mechanical properties in order to identify the various alterations that can lead to pain. Diet, exercise, and overuse syndromes are able to modify the viscosity of loose connective tissue within fascia, causing densification, an alteration that is easily reversible. Trauma, surgery, diabetes, and aging alter the fibrous layers of fasciae, leading to fascial fibrosis.

  13. Inflammation and its genesis in cystic fibrosis.

    Science.gov (United States)

    Nichols, David P; Chmiel, James F

    2015-10-01

    The host inflammatory response in cystic fibrosis (CF) lung disease has long been recognized as a central pathological feature and an important therapeutic target. Indeed, many believe that bronchiectasis results largely from the oxidative and proteolytic damage comprised within an exuberant airway inflammatory response that is dominated by neutrophils. In this review, we address the longstanding argument of whether or not the inflammatory response is directly attributable to impairment of the cystic fibrosis transmembrane conductance regulator or only secondary to airway obstruction and chronic bacterial infection and challenge the importance of this distinction in the context of therapy. We also review the centrality of neutrophils in CF lung pathophysiology and highlight more recent data that suggest the importance of other cell types and signaling beyond NF-κB activation. We discuss how protease and redox imbalance are critical factors in CF airway inflammation and end by reviewing some of the more promising therapeutic approaches now under development.

  14. Macrophages in Tissue Repair, Regeneration, and Fibrosis.

    Science.gov (United States)

    Wynn, Thomas A; Vannella, Kevin M

    2016-03-15

    Inflammatory monocytes and tissue-resident macrophages are key regulators of tissue repair, regeneration, and fibrosis. After tissue injury, monocytes and macrophages undergo marked phenotypic and functional changes to play critical roles during the initiation, maintenance, and resolution phases of tissue repair. Disturbances in macrophage function can lead to aberrant repair, such that uncontrolled production of inflammatory mediators and growth factors, deficient generation of anti-inflammatory macrophages, or failed communication between macrophages and epithelial cells, endothelial cells, fibroblasts, and stem or tissue progenitor cells all contribute to a state of persistent injury, and this could lead to the development of pathological fibrosis. In this review, we discuss the mechanisms that instruct macrophages to adopt pro-inflammatory, pro-wound-healing, pro-fibrotic, anti-inflammatory, anti-fibrotic, pro-resolving, and tissue-regenerating phenotypes after injury, and we highlight how some of these mechanisms and macrophage activation states could be exploited therapeutically.

  15. Acute Exacerbations of Idiopathic Pulmonary Fibrosis

    OpenAIRE

    Collard, Harold R.; Moore, Bethany B.; Flaherty, Kevin R.; Brown, Kevin K.; Kaner, Robert J.; King, Talmadge E.; Lasky, Joseph A.; Loyd, James E.; Noth, Imre; Olman, Mitchell A.; Raghu, Ganesh; Roman, Jesse; Ryu, Jay H.; Zisman, David A.; Hunninghake, Gary W.

    2007-01-01

    The natural history of idiopathic pulmonary fibrosis (IPF) has been characterized as a steady, predictable decline in lung function over time. Recent evidence suggests that some patients may experience a more precipitous course, with periods of relative stability followed by acute deteriorations in respiratory status. Many of these acute deteriorations are of unknown etiology and have been termed acute exacerbations of IPF. This perspective is the result of an international effort to summariz...

  16. Role of endothelin in lung fibrosis

    Directory of Open Access Journals (Sweden)

    D. Abraham

    2008-12-01

    Full Text Available Accumulating evidence suggests that idiopathic pulmonary fibrosis (IPF results from lung injury primarily following an abnormal wound healing response towards epithelial cell damage. Thus, rather than resulting from chronic inflammation, as thought previously, the inflammatory response acts as a modifier of the fibrogenic response, which can also be influenced by the host’s genetic background and environmental triggers. Several key mediators, including endothelin (ET-1, have been implicated in the fibrosis and scarring associated with IPF. Elevated levels of ET-1 have been detected in bronchoalveolar lavage fluid and serum from patients with IPF and increased expression of ET-1 has been detected in small pulmonary blood vessels and macrophages. In vitro data show that ET-1 influences matrix production and degradation by promoting synthesis of collagen type I and III, inhibiting expression of matrix metalloproteinase-1 and promoting matrix remodelling. In addition, ET-1 promotes fibroblast differentiation to a myofibroblastic cell type, inducing the expression of proteins that contribute to a contractile phenotype including -smooth muscle actin. Moreover, ET-1 has been shown to initiate alveolar epithelial cell transition into fibroblast-like cells, a process termed epithelial–mesenchymal transition, and thereby contribute to pulmonary fibrosis. Furthermore, recently reported data from the BUILD (Bosentan Use in Interstitial Lung Disease-1 trial showed a trend towards a delay in time to disease progression or death following treatment with the endothelin receptor antagonist, bosentan, which was especially prominent in patients with biopsy-proven IPF. This adds to the evidence that ET-1 plays an important role in IPF. The present article examines recent evidence for the role of endothelin-1 in pulmonary fibrosis, and particularly, in the control of the function and differentiation of fibroblasts and myofibroblasts.

  17. Dental Fusion with Oral Submucous Fibrosis

    Directory of Open Access Journals (Sweden)

    Ramachandran Sudarshan

    2013-04-01

    Full Text Available Dental Fusion is developmental anomaly due to the union of two tooth germs resulting in a single tooth. It is an infrequent phenomenon but may cause caries, periodontal, cosmetic and malocclusion abnormalities. Oral Submucous Fibrosis is a chronic inflammatory disorder and a precancerous condition affecting oral mucosa causing inability to open the mouth, burning sensation and leathery consistency. This manuscript describes a case of OSMF and dental fusion. [Cukurova Med J 2013; 38(2.000: 308-310

  18. MicroRNAs in renal fibrosis

    Directory of Open Access Journals (Sweden)

    Arthur Chi-Kong Chung

    2015-02-01

    Full Text Available MicroRNAs (miRNAs are endogenous short noncoding RNAs that regulate most of important cellular processes by inhibiting gene expression through the post-transcriptional repression of their target mRNAs. . In kidneys, miRNAs have been associated in renal development, homeostasis, and physiological functions. Results from clinical and experimental animal studies demonstrate that miRNAs play essential roles in the pathogenesis of various renal diseases. Chronic kidney diseases (CKD is characterized by renal fibrosis. Transforming growth factor beta (TGF-β is recognized as a major mediator of renal fibrosis because it is able to stimulate the accumulation of extracellular matrix proteins to impair normal kidney function. Recently, emerging evidence demonstrate the relationship between TGF-β signaling and miRNAs expression during renal diseases. TGF-β regulates expression of several microRNAs, such as miR-21, miR-192, miR-200, miR-433, and miR-29. MiR-21, miR-192, and miR-433 which are positively induced by TGF-β signaling play a pathological role in kidney diseases. In contrast, members in both miR-29 and miR-200 families which are inhibited by TGF-β signaling protect kidneys from renal fibrosis by suppressing the deposition of extracellular matrix and preventing epithelial-to-mesenchymal transition, respectively. Clinically, the presence of miRNAs in blood and urine has been examined to be early biomarkers for detecting renal diseases. From experimental animal studies of CKD, targeting microRNAs also provides evidence about therapeutic potential of miRNAs during renal diseases. Now, it comes to the stage to examine the exact mechanisms of miRNAs during the initiation and progression of renal diseases. Therefore, determining the function of miRNAs in renal fibrosis may facilitate the development of both early diagnosis and treatment of renal diseases.

  19. A comparison of four fibrosis indexes in chronic HCV: Development of new fibrosis-cirrhosis index (FCI

    Directory of Open Access Journals (Sweden)

    Khaliq Saba

    2011-04-01

    Full Text Available Abstract Background Hepatitis C can lead to liver fibrosis and cirrhosis. We compared readily available non-invasive fibrosis indexes for the fibrosis progression discrimination to find a better combination of existing non-invasive markers. Methods We studied 157 HCV infected patients who underwent liver biopsy. In order to differentiate HCV fibrosis progression, readily available AAR, APRI, FI and FIB-4 serum indexes were tested in the patients. We derived a new fibrosis-cirrhosis index (FCI comprised of ALP, bilirubin, serum albumin and platelet count. FCI = [(ALP × Bilirubin / (Albumin × Platelet count]. Results Already established serum indexes AAR, APRI, FI and FIB-4 were able to stage liver fibrosis with correlation coefficient indexes 0.130, 0.444, 0.578 and 0.494, respectively. Our new fibrosis cirrhosis index FCI significantly correlated with the histological fibrosis stages F0-F1, F2-F3 and F4 (r = 0.818, p Conclusions The fibrosis-cirrhosis index (FCI accurately predicted fibrosis stages in HCV infected patients and seems more efficient than frequently used serum indexes.

  20. Multidisciplinary care in cystic fibrosis: a clinical-nutrition review Atención multidisciplinaria en la fibrosis quística: una revisión clínica y nutricional

    Directory of Open Access Journals (Sweden)

    A. Haack

    2012-04-01

    Full Text Available The multidisciplinary care, at different referral centers of cystic fibrosis, is aimed at monitoring and treating cystic fibrosis patients. Mortality attributed to this hereditary disease is high, since it affects the exocrine glands, involving multiple organs, and evolves in a chronic, progressive way. However, systemized care and the improved, shared understanding of gastroenterologists, nutritionists and pulmonologists, contribute to prolonged survival and abated morbimortality. The aim of this study is to describe the main aspects of clinical and nutritional intervention in cystic fibrosis patients so that monitoring by a multidisciplinary team is optimized and performed as early as possible. The review was carried out on articles indexed in the Medline, Lilacs, SciELO, Current Contents and Cochrane databases, finding 189 articles in Portuguese, English and Spanish, with emphasis on articles published between 2000 and 2011. Due to the scientific relevant contribution, some publications before 2000 were included totalized 77 related to the multidisciplinary care. The reviewed studies suggest that multidisciplinary care is essential for knowledge integration in order to impose permanent update of scientific information, thereby contributing to the development of intervention strategies that enhance survival and motivate the development of skills to cope with the complex treatment regimen that is necessary for cystic fibrosis treatment and prevention of related complications.La atención multidisciplinaria, en diferentes centros de referencia de la fibrosis quística, tiene por objeto el seguimiento y el tratamiento de pacientes con fibrosis quística. La mortalidad atribuidas a esta enfermedad hereditaria es alto, ya que afecta las glándulas exocrinas, afecta múltiples órganos, y evoluciona de manera crónica y progresiva. Sin embargo, la atención sistematizada y la mejor comprensión compartida de los gastroenterólogos, nutricionistas y

  1. Smoking and Pulmonary Fibrosis: Novel Insights

    Directory of Open Access Journals (Sweden)

    Katerina D. Samara

    2011-01-01

    Full Text Available The relationship between smoking and pulmonary fibrosis is under debate and intense investigation. The aim of this paper is to review the existing literature and identify further areas of research interest. Recently the negative influence of cigarette smoking on IPF outcome was highlighted, as non-smokers exhibit a better survival than ex-smokers and combined current- and ex-smokers. In patients with non-specific interstitial pneumonia (NSIP, a high prevalence of emphysema was recently demonstrated, providing an indirect support for a smoking pathogenetic hypothesis in NSIP. The coexistence of pulmonary fibrosis and emphysema has been extensively described in a syndrome termed combined pulmonary fibrosis and emphysema (CPFE. Connective tissue disorders (CTDs are a group of autoimmune diseases which affect the lung, as one of the most common and severe manifestations. However, the relationship between smoking and autoimmune disorders is still conflicting. Rheumatoid arthritis results from the interaction between genetic and environmental factors, while the best established environmental factor is tobacco smoking. Smoking has also a negative impact on the response of the RA patients to treatment. The aforementioned smoking-related implications give rise to further research questions and certainly provide one more important reason for physicians to advocate smoking cessation and smoke-free environment.

  2. Mycobacterium abscessus and Children with Cystic Fibrosis

    Science.gov (United States)

    Sermet-Gaudelus, Isabelle; Le Bourgeois, Muriel; Pierre-Audigier, Catherine; Offredo, Catherine; Guillemot, Didier; Halley, Sophie; Akoua-Koffi, Chantal; Vincent, Véronique; Sivadon-Tardy, Valérie; Ferroni, Agnès; Berche, Patrick; Scheinmann, Pierre; Lenoir, Gérard

    2003-01-01

    We prospectively studied 298 patients with cystic fibrosis (mean age 11.3 years; range 2 months to 32 years; sex ratio, 0.47) for nontuberculous mycobacteria in respiratory samples from January 1, 1996, to December 31, 1999. Mycobacterium abscessus was by far the most prevalent nontuberculous mycobacterium: 15 patients (6 male, 9 female; mean age 11.9 years; range 2.5–22 years) had at least one positive sample for this microorganism (versus 6 patients positive for M. avium complex), including 10 with >3 positive samples (versus 3 patients for M. avium complex). The M. abscessus isolates from 14 patients were typed by pulsed-field gel electrophoresis: each of the 14 patients harbored a unique strain, ruling out a common environmental reservoir or person-to-person transmission. Water samples collected in the cystic fibrosis center were negative for M. abscessus. This major mycobacterial pathogen in children and teenagers with cystic fibrosis does not appear to be acquired nosocomially. PMID:14720400

  3. [Treatment of Cystic Fibrosis with CFTR Modulators].

    Science.gov (United States)

    Tümmler, B

    2016-05-01

    Personalized medicine promises that medical decisions, practices and products are tailored to the individual patient. Cystic fibrosis, an inherited disorder of chloride and bicarbonate transport in exocrine glands, is the first successful example of customized drug development for mutation-specific therapy. There are two classes of CFTR modulators: potentiators that increase the activity of CFTR at the cell surface, and correctors that either promote the read-through of nonsense mutations or facilitate the translation, folding, maturation and trafficking of mutant CFTR to the cell surface. The potentiator ivacaftor and the corrector lumacaftor are approved in Germany for the treatment of people with cystic fibrosis who carry a gating mutation such as p.Gly551Asp or who are homozygous for the most common mutation p.Phe508del, respectively. This report provides an overview of the basic defect in cystic fibrosis, the population genetics of CFTR mutations in Germany and the bioassays to assess CFTR function in humans together with the major achievements of preclinical research and clinical trials to bring CFTR modulators to the clinic. Some practical information on the use of ivacaftor and lumacaftor in daily practice and an update on pitfalls, challenges and novel strategies of bench-to-bedside development of CFTR modulators are also provided.

  4. Opciones de tratamiento prótésico en niños con oligodoncias por displasia ectodérmica hidrótica

    OpenAIRE

    Kirenia Pieri Silva; Beatriz García Alpízar; Eduardo Enrique Castillo Betancourt; Grety de Armas Ávila

    2014-01-01

    La reposición de los dientes no solamente influye en la adecuada alimentación y fonética, sino que también mejora la sonrisa y la apariencia personal, además del aspecto positivo que produce en el estado de salud psicológico y emocional de los niños afectados. Se presentan tres casos clínicos de niños con oligodoncias asociadas a displasia ectodérmica hidrótica, atendidos en la consulta de prótesis de la Clínica Estomatológica de Especialidades de Cienfuegos. Fueron rehabilitados con prótesis...

  5. Noninvasive diagnosis of hepatic fibrosis in chronic hepatitis C

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Assessment of hepatic fibrosis is important for determining prognosis, guiding management decisions,and monitoring disease. Histological evaluation of liver biopsy specimens is currently considered the reference test for staging hepatic fibrosis. Since liver biopsy carries a small but significant risk, noninvasive tests to assess hepatic fibrosis are desirable. This editorial gives an overview on noninvasive methods currently available to determine hepatic fibrosis and their diagnostic accuracy for predicting significant fibrosis and cirrhosis in chronic hepatitis C. Based on available data, the performance of simple tests derived from routine laboratory parameters appears to be similar to that of more complex and expensive fibrosis panels. Transient elastography seems more accurate than blood tests for diagnosing cirrhosis.

  6. Role of interleukin-13 in fibrosis, particularly systemic sclerosis.

    Science.gov (United States)

    O'Reilly, Steven

    2013-01-01

    Chronic inflammation can lead to altered extracellular matrix deposition and ultimately fibrosis. Interleukin-13 (IL-13) is a cytokine that was found to promote IgE class switching and inhibit proinflammatory cytokines. However, it is now recognized as an important mediator in allergy and most importantly fibrosis. Indeed, animal studies with genetically deleted mice have demonstrated its critical role in fibrosis and although it shares over lapping functions with IL-4 it is the dominant cytokine in fibrosis. Systemic sclerosis is an autoimmune disease in which there is chronic inflammation and fibrosis. The disease is associated with a Th2 polarization and IL-13 levels are elevated both in the blood and in the skin of patients. This review will examine the role of IL-13 in driving fibrosis with a particular emphasis on systemic sclerosis as a prototypical fibrotic disease. It will highlight recent research into the role of IL-13 and how this cytokine may be targeted in systemic sclerosis.

  7. Cystic fibrosis Delta F508 heterozygotes, smoking, and reproduction

    DEFF Research Database (Denmark)

    Dahl, Morten; Tybjaerg-Hansen, A; Wittrup, H H

    1998-01-01

    Cystic fibrosis is the most common fatal autosomal recessive disease affecting Caucasian populations. It remains a puzzle how this disease is maintained at such a remarkably high incidence, however, it could be due to a reproductive advantage in cystic fibrosis heterozygotes. We tested this hypot......Cystic fibrosis is the most common fatal autosomal recessive disease affecting Caucasian populations. It remains a puzzle how this disease is maintained at such a remarkably high incidence, however, it could be due to a reproductive advantage in cystic fibrosis heterozygotes. We tested.......001). In conclusion, overall these results do not support a reproductive advantage for cystic fibrosis DeltaF508 heterozygotes. However, the data cannot totally exclude the possibility that nonsmoking DeltaF508 heterozygotes experience a reproductive advantage while smoking DeltaF508 heterozygotes experience...... the opposite, a reproductive disadvantage. Accordingly, the data suggest a previously undocumented role of smoking on fecundity among cystic fibrosis heterozygotes....

  8. Congenital hepatic fibrosis, liver cell carcinoma and adult polycystic kidneys.

    Science.gov (United States)

    Manes, J L; Kissane, J M; Valdes, A J

    1977-06-01

    In reviewing the literature, we found no liver cell carcinoma (LCC) or well-documented adult polycystic kidneys (APK) associated with congenital hepatic fibrosis (CHF). We report a 69-year-old man with CHF, LCC, APK, duplication cyst of distal portion of stomach, two calcified splenic artery aneurysms, myocardial fibrosis and muscular hypertrophy of esophagus. The LCC was grossly predunculated and microscopically showed prominent fibrosis and hyaline intracytoplasmic inclusions in the tumor cells.

  9. Assessment of liver fibrosis: Noninvasive means

    Directory of Open Access Journals (Sweden)

    Poynard Thierry

    2008-01-01

    Full Text Available Liver biopsy, owing to its limitations and risks, is an imperfect gold standard for assessing the severity of the most frequent chronic liver diseases chronic hepatitis C (HCV, B (HBV non alcoholic (NAFLD and alcoholic (ALD fatty liver diseases. This review summarizes the advantages and the limits of the available biomarkers of liver fibrosis. Among a total of 2,237 references, a total of 14 validated serum biomarkers have been identified between 1991 and 2008. Nine were not patented and five were patented. Two alternatives to liver biopsy were the most evaluated FibroTest and Fibroscan. For FibroTest, there was a total of 38 different populations including 7,985 subjects with both FibroTest and biopsy (4,600 HCV, 1,580 HBV, 267 NAFLD, 524 ALD, and 1014 mixed. For Fibroscan, there was a total of 11 published studies including 2,260 subjects (1,466 HCV, 95 cholestatic liver disease, and 699 mixed. For FibroTest, the mean diagnostic value for the diagnosis of advanced fibrosis assessed using standardized area under the ROC curves was 0.84 (95% confidence interval 0.83-0.86, without a significant difference between the causes of liver disease, hepatitis C, hepatitis B, and alcoholic or non alcoholic fatty liver disease. High-risk profiles of false negative/false positive of FibroTest, mainly Gilbert syndrome, hemolysis and acute inflammation, are present in 3% of the populations. In case of discordance between biopsy and FibroTest, half of the failures can be due to biopsy; the prognostic value of FibroTest is at least similar to that of biopsy in HCV, HBV and ALD. In conclusion this overview of evidence-based data suggests that biomarkers could be used as an alternative to liver biopsy for the first line assessment of fibrosis stage in the four most common chronic liver diseases, namely HCV, HBV, NAFLD and ALD. Neither biomarkers nor biopsy alone is sufficient for taking a definite decision in a given patient; all the clinical and biological data

  10. [Rheumatoid arthritis and combined pulmonary fibrosis and emphysema].

    Science.gov (United States)

    Fernández Casares, Marcelo; Fielli, Mariano; Cristaldo, Laura; Zárate, Lucía; Nieves Capozzi, María

    2015-01-01

    The combination of pulmonary fibrosis and emphysema is a syndrome described in the last years, which has its own characteristics and it is not only the casual association between the two entities. The idiopathic pulmonary fibrosis is the most common type of pulmonary fibrosis. However other interstitial lung diseases could be part of this syndrome. Among them is the connective tissue disease-associated interstitial lung disease. We report a case of this syndrome associated with rheumatoid arthritis. It has the peculiarity that the connective disease became overt several years after the presentation of combined pulmonary fibrosis and emphysema syndrome, which is infrequently reported in the literature.

  11. Bone marrow fibrosis in myelofibrosis: pathogenesis, prognosis and targeted strategies

    Science.gov (United States)

    Zahr, Abdallah Abou; Salama, Mohamed E.; Carreau, Nicole; Tremblay, Douglas; Verstovsek, Srdan; Mesa, Ruben; Hoffman, Ronald; Mascarenhas, John

    2016-01-01

    Bone marrow fibrosis is a central pathological feature and World Health Organization major diagnostic criterion of myelofibrosis. Although bone marrow fibrosis is seen in a variety of malignant and non-malignant disease states, the deposition of reticulin and collagen fibrosis in the bone marrow of patients with myelofibrosis is believed to be mediated by the myelofibrosis hematopoietic stem/progenitor cell, contributing to an impaired microenvironment favoring malignant over normal hematopoiesis. Increased expression of inflammatory cytokines, lysyl oxidase, transforming growth factor-β, impaired megakaryocyte function, and aberrant JAK-STAT signaling have all been implicated in the pathogenesis of bone marrow fibrosis. A number of studies indicate that bone marrow fibrosis is an adverse prognostic variable in myeloproliferative neoplasms. However, modern myelofibrosis prognostication systems utilized in risk-adapted treatment approaches do not include bone marrow fibrosis as a prognostic variable. The specific effect on bone marrow fibrosis of JAK2 inhibition, and other rationally based therapies currently being evaluated in myelofibrosis, has yet to be fully elucidated. Hematopoietic stem cell transplantation remains the only curative therapeutic approach that reliably results in resolution of bone marrow fibrosis in patients with myelofibrosis. Here we review the pathogenesis, biological consequences, and prognostic impact of bone marrow fibrosis. We discuss the rationale of various anti-fibrogenic treatment strategies targeting the clonal hematopoietic stem/progenitor cell, aberrant signaling pathways, fibrogenic cytokines, and the tumor microenvironment. PMID:27252511

  12. Postirradiation pulmonary fibrosis complicated by aspergilloma and bronchocentric granulomatosis

    Energy Technology Data Exchange (ETDEWEB)

    Makker, H.; McConnochie, K.; Gibbs, A.R. (Univ. of Wales College of Medicine, Llandough Hospital, Penarth (UK))

    1989-08-01

    An asthmatic patient requiring cortico-steroid treatment developed a pulmonary aspergilloma in an area of postmastectomy radiation fibrosis. At necropsy bronchocentric granulomatosis was also found. (author).

  13. Interstitial pulmonary fibrosis in an automobile body shop worker

    Energy Technology Data Exchange (ETDEWEB)

    Fischbein, A.; Rohl, A.N.; Suzuki, Y.; Bigman, O.

    1985-09-01

    Interstitial pulmonary fibrosis (IPF) is often of uncertain etiology and is therefore named idiopathic pulmonary fibrosis. Some occupational exposures, however, are known to cause interstitial fibrosis, asbestos and silica being well-known examples. The authors present clinical and pathological findings of a case with IPF and the results of microchemical analysis of inorganic particulate matter in the lung tissue. A very high lung burden of inorganic contaminants was found, including silica and metallic compounds. Emphasis is given to the importance of obtaining detailed occupational histories and conducting microchemical analysis of lung tissue in order to clarify etiological factors in cases with idiopathic pulmonary fibrosis.

  14. Soledad con espectador

    OpenAIRE

    2011-01-01

    Este proyecto se plantea como una investigación de las posibilidades del dibujo como una herramienta para relatar una historia propia que se empapa de realidad y de ficción con el deseo de confundir al espectador y suscitarle preguntas. Para ello me he servido de mi propia imagen como personaje central. Éste se presenta duplicado, multiplicado, repetido e interactúa consigo mismo en un espacio imaginario con intención de simular aquello que se revela en el interior de la psique. Mi trabajo se...

  15. Giochiamo con i robot

    Directory of Open Access Journals (Sweden)

    Andrea Bonarini

    2009-01-01

    Full Text Available "Giochiamo con i robot" e' un laboratorio interattivo per grandi e piccini realizzato per l'edizione 2007 del Festival della Scienza di Genova. Lungo un percorso che va dalla telerobotica alla robotica evolutiva, il laboratorio sviluppa il tema di dare intelligenza ai robot. Questo percorso, le cui tappe sono le varie installazioni, si conclude nella "bottega" dove e' possibile costruire e programmare i propri robot o smontare e modificare quelli esposti durante il percorso didattico. I visitatori sono coinvolti in attivita' ludiche grazie alle quali possonoentrare in contatto con alcune delle idee potenti della robotica,

  16. en pacientes con obesidad

    Directory of Open Access Journals (Sweden)

    Alcia María Alvarado Sánchez

    2005-01-01

    Full Text Available El objetivo de este estudio fue evaluar la eficacia de una intervención psicológica en pacientes con obesidad. Se utilizó un diseño cuasiexperimental con un grupo de estudio y un grupo control. Después de la intervención, se encontró una diferencia significativa en la reducción de peso entre los grupos. Asimismo, hubo un incremento significativo en la autoestima del grupo estudiado.

  17. Funcionando con la computadora

    OpenAIRE

    Álvarez, Eduardo; Astiz, Mercedes; Medina, Perla; Montero, Y.; Oliver, María; Rocerau, M. Cristina; VALDEZ, Guillermo; Vecino, María; Vilanova, Silvia

    2004-01-01

    En este trabajo se presenta la descripción y resultados de la segunda etapa de una experiencia planteada con el objetivo de indagar la manera en que los alumnos determinan e interpretan funciones que explican situaciones problemáticas valiéndose de una nueva forma de trabajo en el aula: la utilización de la computadora como herramienta y un programa asistente matemático. La primera etapa consistió en el desarrollo de un taller optativo con alumnos de entre 14 y 15 años de edad del Colegio Dr....

  18. Revisión y estado del arte de la corrosividad del etanol y sus mezclas con gasolina

    Directory of Open Access Journals (Sweden)

    Berlanga, C.

    2011-12-01

    Full Text Available Ethanol is currently an important source of renewable combustible for the automotive sector. It is known that traditional fuels such as gasoline have not caused corrosion problems due mainly to low miscibility with water. On the other hand, the alcohol based biofuels can contain a considerable amount of water, and in addition, partial oxidation of their components might be produced by exposition to the atmosphere, and this may lead to corrosion and subsequent failure of any metallic component in contact with the ethanol-gasoline blends. In consequence, all the industrial sectors related to this fuel, producing companies, logistics, dispensers and manufacturers of vehicles might be affected by this problem. This makes this topic very interesting, both from a scientific and an industrial point of view. This paper systematically reviews all aspects related to the corrosive behaviour of ethanol and ethanol-gasoline blends, with the aim of assessing the current state-of the-art as well as serving as a guide for further works that take the study of this subject matter.

    Actualmente, el etanol se presenta como una fuente importante de combustible renovable para el sector de la automoción. Se sabe que los carburantes tradicionales, como la gasolina, no han causado problemas de corrosión, debido principalmente a su baja miscibilidad en el agua. En cambio, los biocarburantes en base alcohol pueden contener una considerable cantidad de agua y, además, se puede producir la oxidación parcial de sus componentes por su exposición a la atmósfera. Estas reacciones pueden ocasionar corrosión y posterior fallo, de cualquier componente metálico en contacto con las mezclas etanol-gasolina. En consecuencia, todos los sectores relacionados con la industria que emplean este combustible, empresas productoras, logística, dispensadores y fabricantes de vehículos, pueden estar afectados por esta problemática. Esto hace que este tema sea muy interesante

  19. Metodología para el análisis de riesgos en establecimientos afectados por la guía CPR 15 (almacenaje de productos químicos en recipientes móviles) considerando la emisión de humos tóxicos. Aplicación a casos prácticos

    OpenAIRE

    Prieto García, Rosa Mª

    2009-01-01

    Este proyecto se centra en el estudio de los efectos de los humos emitidos durante los incendios en almacenes químicos afectados por la normativa de Accidentes Graves (SEVESO), tanto en las zonas afectadas por el incendio como en las áreas próximas al mismo. En el proyecto se realiza una introducción sobre la importancia de los humos emitidos en incendios de este tipo, analizando la normativa vigente a nivel europeo, estatal y autonómico. Incluye un análisis de accidentes ocurr...

  20. Lack of correlation between pulmonary disease and cystic fibrosis transmembrane conductance regulator dysfunction in cystic fibrosis: a case report

    Directory of Open Access Journals (Sweden)

    Cleveland Robert H

    2010-04-01

    Full Text Available Abstract Introduction Mutations in both alleles of the cystic fibrosis transmembrane conductance regulator gene result in the disease cystic fibrosis, which usually manifests as chronic sinopulmonary disease, pancreatic insufficiency, elevated sodium chloride loss in sweat, infertility among men due to agenesis of the vas deferens and other symptoms including liver disease. Case presentation We describe a pair of African-American brothers, aged 21 and 27, with cystic fibrosis. They were homozygous for a rare frameshift mutation in the cystic fibrosis transmembrane conductance regulator 3791delC, which would be expected to cause significant morbidity. Although 80% of cystic fibrosis patients are colonized with Pseudomonas aeruginosa by eight years of age, the older brother had no serum opsonic antibody titer to P. aeruginosa by age 13 and therefore would have failed to mount an effective antibody response to the alginate (mucoid polysaccharide capsule of P. aeruginosa. He was not colonized with P. aeruginosa until 24 years of age. Similarly, the younger brother was not colonized with P. aeruginosa until age 20 and had no significant lung disease. Conclusion Despite a prevailing idea in cystic fibrosis research that the amount of functional cystic fibrosis transmembrane conductance regulator predicts clinical status, our results indicated that respiratory disease severity in cystic fibrosis exhibits phenotypic heterogeneity. If this heterogeneity is, in part, genetic, it is most likely derived from genes outside the cystic fibrosis transmembrane conductance regulator locus.

  1. Current Strategies for Quantitating Fibrosis in Liver Biopsy

    Institute of Scientific and Technical Information of China (English)

    Yan Wang; Jin-Lin Hou

    2015-01-01

    Objective:The present mini-review updated the progress in methodologies based on using liver biopsy.Data Sources:Articles for study of liver fibrosis,liver biopsy or fibrosis assessment published on high impact peer review journals from 1980 to 2014.Study Selection:Key articles were selected mainly according to their levels of relevance to this topic and citations.Results:With the recently mounting progress in chronic liver disease therapeutics,comes by a pressing need for precise,accurate,and dynamic assessment of hepatic fibrosis and cirrhosis in individual patients.Histopathological information is recognized as the most valuable data for fibrosis assessment.Conventional histology categorical systems describe the changes of fibrosis patterns in liver tissue; but the simplified ordinal digits assigned by these systems cannot reflect the fibrosis dynamics with sufficient precision and reproducibility.Morphometric assessment by computer assist digital image analysis,such as collagen proportionate area (CPA),detects change of fibrosis amount in tissue section in a continuous variable,and has shown its independent diagnostic value for assessment of advanced or late-stage of fibrosis.Due to its evident sensitivity to sampling variances,morphometric measurement is feasible to be taken as a reliable statistical parameter for the study of a large cohort.Combining state-of-art imaging technology and fundamental principle in Tissue Engineering,structure-based quantitation was recently initiated with a novel proof-of-concept tool,qFibrosis.qFibrosis showed not only the superior performance to CPA in accurately and reproducibly differentiating adjacent stages of fibrosis,but also the possibility for facilitating analysis of fibrotic regression and cirrhosis sub-staging.Conclusions:With input from multidisciplinary innovation,liver biopsy assessment as a new "gold standard" is anticipated to substantially support the accelerated progress of Hepatology medicine.

  2. Current Strategies for Quantitating Fibrosis in Liver Biopsy

    Directory of Open Access Journals (Sweden)

    Yan Wang

    2015-01-01

    Full Text Available Objective: The present mini-review updated the progress in methodologies based on using liver biopsy. Data Sources: Articles for study of liver fibrosis, liver biopsy or fibrosis assessment published on high impact peer review journals from 1980 to 2014. Study Selection: Key articles were selected mainly according to their levels of relevance to this topic and citations. Results: With the recently mounting progress in chronic liver disease therapeutics, comes by a pressing need for precise, accurate, and dynamic assessment of hepatic fibrosis and cirrhosis in individual patients. Histopathological information is recognized as the most valuable data for fibrosis assessment. Conventional histology categorical systems describe the changes of fibrosis patterns in liver tissue; but the simplified ordinal digits assigned by these systems cannot reflect the fibrosis dynamics with sufficient precision and reproducibility. Morphometric assessment by computer assist digital image analysis, such as collagen proportionate area (CPA, detects change of fibrosis amount in tissue section in a continuous variable, and has shown its independent diagnostic value for assessment of advanced or late-stage of fibrosis. Due to its evident sensitivity to sampling variances, morphometric measurement is feasible to be taken as a reliable statistical parameter for the study of a large cohort. Combining state-of-art imaging technology and fundamental principle in Tissue Engineering, structure-based quantitation was recently initiated with a novel proof-of-concept tool, qFibrosis. qFibrosis showed not only the superior performance to CPA in accurately and reproducibly differentiating adjacent stages of fibrosis, but also the possibility for facilitating analysis of fibrotic regression and cirrhosis sub-staging. Conclusions: With input from multidisciplinary innovation, liver biopsy assessment as a new "gold standard" is anticipated to substantially support the accelerated

  3. Correlación clinicorradiográfica de la fibrosis epidural en el síndrome poslaminectomía mediante epidurografía Clinical/radiography correlation of epidural fibrosis in postlaminectomy syndrome by epidurography

    OpenAIRE

    Cardona, G.; J.R. Hernández-Santos; S. Tenopala; A. Jiménez-Ramos; I.M. Narváez; Torres, J C

    2010-01-01

    Introducción: La epidurografía es un recurso invasivo que evalúa deformidades y eventual ocupación del espacio epidural, además de ser un estudio fácilmente realizable, confiable, económico y que forma parte del algoritmo de manejo del síndrome poslaminectomía. Objetivo: Verificar si la localización y la extensión de la fibrosis epidural se correlacionan con el cuadro clínico presente en los pacientes con síndrome poslaminectomía. Material y métodos: Se incluyó a 30 pacientes con diagnóstico ...

  4. Co-morbidity of cystic fibrosis and celiac disease in Scandinavian cystic fibrosis patients

    DEFF Research Database (Denmark)

    Fluge, Gjermund; Olesen, Hanne Vebert; Giljam, Marita

    2009-01-01

    Background: The co-morbidity of cystic fibrosis (CF) and celiac disease (CD) has been reported sporadically since the 1960s. To our knowledge, this is the first time a systematic screening is performed in a large cohort of CF patients. Methods: Transglutaminase-IgA (TGA), endomysium-IgA (EMA...

  5. Co-morbidity of cystic fibrosis and celiac disease in Scandinavian cystic fibrosis patients

    DEFF Research Database (Denmark)

    Fluge, G; Olesen, Hanne Vebert; Gilljam, M

    2009-01-01

    BACKGROUND: The co-morbidity of cystic fibrosis (CF) and celiac disease (CD) has been reported sporadically since the 1960s. To our knowledge, this is the first time a systematic screening is performed in a large cohort of CF patients. METHODS: Transglutaminase-IgA (TGA), endomysium-IgA (EMA...

  6. fertilizada con diferentes abonos

    Directory of Open Access Journals (Sweden)

    Jorge Alberto Elizondo-Salazar

    2007-01-01

    Full Text Available Producción y calidad de la biomasa de morera (Morus alba fertilizada con diferentes abonos. Se llevó a cabo un experimento en la Estación Experimental “Alfredo Volio Mata” de la Universidad de Costa Rica con el fi n de evaluar la aplicación de 150 kg de N/ha/año proveniente de dos abonos orgánicos: lombriabono y compostaje; y de un fertilizante químico, sobre la producción y calidad de la biomasa de morera. El periodo experimental comprendió un ciclo de 12 meses, iniciando en julio del 2003 y fi nalizando en julio del 2004. Se utilizó una plantación de morera de 12 años de establecida con una densidad de siembra de 27.777 plantas/ ha. Se empleó un diseño de bloques completos al azar con cuatro tratamientos: dos abonos orgánicos, nitrato de amonio (33,5% N y un control. Las plantas se podaron a 0,6 m sobre el nivel del suelo al inicio del ensayo. Durante el periodo experimental, las plantas fueron podadas consecutivamente cada 90 días. Las hojas y los tallos fueron separados y analizados para determinar el contenido de materia seca y proteína cruda. La producción de materia seca fue 23% superior y el contenido de proteína cruda fue signifi cativamente mayor con el nitrógeno químico, mientras que el contenido de materia seca fue menor. No se encontraron diferencias signifi cativas entre el tratamiento control y los tratamientos orgánicos.

  7. [Berberine inhibits cardiac fibrosis of diabetic rats].

    Science.gov (United States)

    Lu, Kun; Shen, Yongjie; He, Jinfeng; Liu, Guoling; Song, Wei

    2016-10-01

    Objective To explore the effect of berberine on cardiac fibrosis of diabetic rats by observing the expressions of serum transforming growth factor-β1 (TGF-β1), connective tissue growth factor (CTGF) , collagen type 1 (Col1) and collagen type 3 (Col3) in myocardial tissues of diabetic rats after berberine treatment. Methods The diabetic model was induced by intraperitoneal injection of streptococci (STZ). Forty-three diabetic rats were randomly divided into diabetic model group (n=9), berberine treated groups of different doses [50, 100, 150 mg/(kg.d), gavage administration for 12 weeks; n=9, 9, 8 respectively], and metformin group as positive control (n=8); other 8 normal rats served as a negative control group. After the last administration, fasting blood glucose, left ventricular systolic pressure (LVSP) and left ventricular end diastolic pressure (LVEDP) were measured; rats' heart were taken to calculate the heart mass index (HMI); ELISA was used to detect the serum levels of TGF-β1 and CTGF; collagenous fibers in cardiac tissues were tested by Masson staining; collagen volume fraction (CVF) was measured by image analysis; Col1 and Col3 in cardiac tissues were determined by Western blotting. Results Compared with the normal control group, the fasting blood glucose, LVSP, LVEDP absolute value, HMI, the degree of cardiac fibrosis, the expressions of TGF-β1, CTGF, Col1 and Col3 significantly increased in the model group. All indexes mentioned above were reduced obviously in berberine treated groups of 100 and 150 mg/(kg.d). Conclusion Berberine improves cardiac fibrosis in diabetic rats through down-regulating the expressions of TGF-β1 and CTGF and reducing the synthesis and deposition of Col1 and Col3.

  8. Liver Fibrosis and Altered Matrix Synthesis

    Directory of Open Access Journals (Sweden)

    Katrin Neubauer

    2001-01-01

    Full Text Available Liver fibrosis represents the uniform response of liver to toxic, infectious or metabolic agents. The process leading to liver fibrosis resembles the process of wound healing, including the three phases following tissue injury: inflammation, synthesis of collagenous and noncollagenous extracellular matrix components, and tissue remodelling (scar formation. While a single liver tissue injury can be followed by an almost complete restitution ad integrum, the persistence of the original damaging noxa results in tissue damage. During the establishment of liver fibrosis, the basement membrane components collagen type IV, entactin and laminin increase and form a basement membrane-like structure within the space of Disse. The number of endothelial fenestrae of the sinusoids decreases. These changes of the sinusoids are called 'capillarization' because the altered structure of the sinusoids resembles that of capillaries. At the cellular level, origin of liver fibrogenesis is initiated by the damage of hepatocytes, resulting in the recruitment of inflammatory cells and platelets, and activation of Kupffer cells, with subsequent release of cytokines and growth factors. The hepatic stellate cells seem to be the primary target cells for these inflammatory stimuli, because during fibrogenesis, they undergo an activation process to a myofibroblast-like cell, which represents the major matrix-producing cell. Based on this pathophysiological mechanism, therapeutic methods are developed to inhibit matrix synthesis or stimulate matrix degradation. A number of substances are currently being tested that either neutralize fibrogenic stimuli and prevent the activation of hepatic stellate cells, or directly modulate the matrix metabolism. However, until now, the elimination of the hepatotoxins has been the sole therapeutic concept available for the treatment of liver fibrogenesis in humans.

  9. Microbiological surveillance in patients with cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Paola Gualdi

    2010-06-01

    Full Text Available Pulmonary infections in patients with cystic fibrosis (CF, are a major cause of morbidity and mortality. Prevention, diagnosis and therapy in cystic fibrosis, lead to the necessary collaboration between clinical and laboratory to identify effective strategies and appropriate solutions to address the problems inherent isolation micro-organisms, antibiotic strategies, overcoming of bacterial resistance and other problems management of these patients. The task of the microbiology laboratory and research in quickly and accurately, the agents responsible for these infectious processes, in order to isolate them from material, identify and determine their sensitivity antibiotics. A microbiological surveillance on 34 patients (13 males and 21 females with CF and related to the “Support Services Provincial Trento for the treatment of cystic fibrosis “in the period July 2005 - August 2008, was carried out. 180 Gram positive and 278 of Gram negative bacteria as well as 235 fungi wre collected. Staphylococcus aureus was the most frequently organism found in patients with CF with an incidence of 23% on 156 strains isolated, Pseudomonas aeruginosa was collected 19% of all microorganisms isolated corresponding to 131 strains, Candida albicans is the yeast often isolated with a frequency 22% equal to 149 isolates, Aspergillus fumigatus was isolated at a rate of 8%. From the data we collected and processed has been noted that the local epidemiology of CF patients reflects as reported in the scientific literature and national international consulting, both as a type microorganisms that frequency also isolated compared to age groups. Considering the score of Bartlett as discriminating respiratory fitness of the material, it has been observed that only 32 samples over 327 total (10% would materials insignificant. It follows therefore that the time of sample collection, followed by personnel (physiotherapists dedicated to CF patients, represents a crucial step

  10. [Congenital hepatic fibrosis: apropos of 12 cases].

    Science.gov (United States)

    Murga, M L; Jara, P; Díaz, M C; de la Rubia, L; Arroba, M L; Larrauri, J; Vázquez, C

    1988-02-01

    Twelve patients with congenital hepatic fibrosis have been retrospectively studied and followed for 1 to 14 years. Clinical features, hepatic function tests and biopsy have been analyzed. Presence of portal hypertension and congenital malformation have been investigated. Clinical presentations varies from newborn to nine years of age without male or female predominance. Most frequent clinical form has been hypertensive type. Cholangitic type has worse prognosis. Familiar recurrence rate is 20%. Congenital malformations are associated in 92% most frequently infantile polycystic kidney disease. Hepatic biopsy has confirmed diagnosis in all patients.

  11. Diagnosis and Management of Endomyocardial Fibrosis.

    Science.gov (United States)

    Beaton, Andrea; Mocumbi, Ana Olga

    2017-02-01

    Endomyocardial fibrosis (EMF) remains an important cause of restrictive cardiomyopathy worldwide. Patients cluster in specific geographic locations and are almost universally living in extreme poverty. Specific etiology remains elusive and is likely multifactorial. Untreated EMF has a very poor prognosis. Medical management can mitigate symptoms for a time but has no curative benefit. Early surgical interventions may improve survival but are not readily available in most EMF-endemic regions. Increased awareness, advocacy, and research are needed to further understand this neglected tropical cardiomyopathy and to improve survival of those affected.

  12. Nephrogenic systemic fibrosis: A brief review

    Directory of Open Access Journals (Sweden)

    Rajesh Waikhom

    2011-01-01

    Full Text Available Nephrogenic systemic fibrosis is a fibrosing disorder of the skin that develops in patients with advanced renal failure. It mostly presents with progressive hardening or induration of the skin of the extremities. Systemic involvement is also known to occur in this entity. Exposure to gadolinium contrast for radiological evaluation has been identified as the offending agent. The condition is progressive and can be seriously disabling. Therapeutic options are limited and not rewarding in majority of the cases. Awareness of this entity is important so that proper precautionary measures can be taken at the earliest to ameliorate the condition.

  13. Normal bone mineral density in cystic fibrosis

    OpenAIRE

    Hardin, D.; R. Arumugam; Seilheimer, D.; Leblanc, A.; Ellis, K.

    2001-01-01

    BACKGROUND—Osteoporosis has been reported as a complication of cystic fibrosis (CF).
AIMS—To measure bone mineral density (BMD) in non-acutely ill adults and bone mineral content (BMC) in children with CF.
METHODS—We analysed data from 28 adults and 13 children with CF. Corticosteroid use was minimal for the year prior to study in both groups. Dual x ray absorptiometry was used to measure total body and regional bone mineral density in adults. In children, whole body BMC was...

  14. Nephrogenic systemic fibrosis: clinical picture and treatment

    DEFF Research Database (Denmark)

    Marckmann, Peter; Skov, Lone

    2009-01-01

    The classic hallmark symptoms of advanced nephrogenic systemic fibrosis (NSF) (skin thickening, hardening and hyperpigmentation, and disabling contractures in renal failure patients) in temporal association with Gd-based contrast agent (GBCA) exposure are almost pathognomonic of NSF. Less obvious......, and the histology of deep skin biopsies. Symptomatic treatment with intensive physiotherapy and painkillers is important, but there is no known curative medical treatment. Spontaneous remission of NSF symptoms may occur with recovery of renal function after an episode of acute renal failure, or with kidney...

  15. Mycobacterium abscessus and Children with Cystic Fibrosis

    OpenAIRE

    Sermet-Gaudelus, Isabelle; Le Bourgeois, Muriel; Pierre-Audigier, Catherine; Offredo, Catherine; Guillemot, Didier; Halley, Sophie; Akoua-Koffi, Chantal; Vincent, Véronique; Sivadon-Tardy, Valérie; Ferroni, Agnès; Berche, Patrick; Scheinmann, Pierre; Lenoir, Gérard; Gaillard, Jean-Louis

    2003-01-01

    We prospectively studied 298 patients with cystic fibrosis (mean age 11.3 years; range 2 months to 32 years; sex ratio, 0.47) for nontuberculous mycobacteria in respiratory samples from January 1, 1996, to December 31, 1999. Mycobacterium abscessus was by far the most prevalent nontuberculous mycobacterium: 15 patients (6 male, 9 female; mean age 11.9 years; range 2.5–22 years) had at least one positive sample for this microorganism (versus 6 patients positive for M. avium complex), including...

  16. [Fat-soluble vitamins supplementation in patients with cystic fibrosis: is enough Aquadek's®?].

    Science.gov (United States)

    González Jiménez, David; Bousoño García, Carlos; Díaz Martin, Juan Jose; Jiménez Treviño, Santiago

    2015-04-01

    Introducción y objetivo: Conocer si la suplementación un nuevo polivitamínico (Aquadek´s®) durante 12 meses es segura y eficaz en pacientes con Fibrosis Quística (FQ). Material y Métodos: Estudio prospectivo, longitudinal y no controlado en pacientes con FQ insuficientes pancreáticos, clínicamente estables, que recibieron suplementación con Aquadek´s® (2 comprimidos masticables) durante 12 meses en lugar de su suplementación habitual. Se evaluaron niveles séricos de retinol, betacarotenos, 25 OH vitamina D y α-tocoferol un año antes, al inicio y tras un año de tratamiento. Análisis estadístico: Pruebas t para datos pareados. Resultados: Se incluyeron 28 pacientes entre 6 y 39 años (mediana 18,5 años). La suplementación con Aquadek´s® supuso un incremento en la dosis de vitaminas A y una disminución del número de comprimidos administrados. Al inicio, un 89% presentaban algún tipo de déficit vitamínico: (61% pro-Vitamina A y 54% vitamina D). Tras un año, se produjo un incremento de los niveles de betacarotenos: 160 mcg/l (IC 95% 98-222) (p.

  17. Glycyrrhizic acid alleviates bleomycin-induced pulmonary fibrosis in rats

    Directory of Open Access Journals (Sweden)

    Lili eGao

    2015-10-01

    Full Text Available Idiopathic pulmonary fibrosis is a progressive and lethal form of interstitial lung disease that lacks effective therapies at present. Glycyrrhizic acid (GA, a natural compound extracted from a traditional Chinese herbal medicine Glycyrrhiza glabra, was recently reported to benefit lung injury and liver fibrosis in animal models, yet whether GA has a therapeutic effect on pulmonary fibrosis is unknown. In this study, we investigated the potential therapeutic effect of GA on pulmonary fibrosis in a rat model with bleomycin (BLM-induced pulmonary fibrosis. The results indicated that GA treatment remarkably ameliorated BLM-induced pulmonary fibrosis and attenuated BLM-induced inflammation, oxidative stress, epithelial-mesenchymal transition and activation of tansforming growth factor-beta signaling pathway in the lungs. Further, we demonstrated that GA treatment inhibited proliferation of 3T6 fibroblast cells, induced cell cycle arrest and promoted apoptosis in vitro, implying that GA-mediated suppression of fibroproliferation may contribute to the anti-fibrotic effect against BLM-induced pulmonary fibrosis. In summary, our study suggests a therapeutic potential of GA in the treatment of pulmonary fibrosis.

  18. Gefitinib attenuates murine pulmonary fibrosis induced by bleomycin

    Institute of Scientific and Technical Information of China (English)

    WANG Ping; TIAN Qing; LIANG Zhi-xin; YANG Zhen; XU Shu-feng; SUN Ji-ping; CHEN Liang-an

    2010-01-01

    Background Gefitinib, an inhibitor of epidermal growth factor receptor (EGFR) tyrosine kinase, is an effective treatment for epithelial tumors, including non-small cell lung cancer (NSCLC), and is generally well tolerated.However, some clinical trials revealed that gefitinib exposure caused lung fibrosis, a severe adverse reaction.This study investigated the effect of gefitinib on lung fibrosis in mice.Methods We generated a mouse model of lung fibrosis induced by bleomycin to investigate the fibrotic effect of gefitinib.C57BL/6 mice were injected intratracheally with bleomycin or saline, with intragastric administration of gefitinib or saline.Lung tissues were harvested on day 14 or 21 for histology and genetic analysis.Results The histological results showed that bleomycin successfully induced lung fibrosis in mice, and gefitinib prevented lung fibrosis and suppressed the proliferation of S100A4-positive fibroblast cells.In addition, Western blotting analysis revealed that gefitinib decreased the expression of phosphorylated EGFR (p-EGFR).Furthermore, quantitative real-time PCR (qRT-PCR) demonstrated that gefitinib inhibited the accumulation of collagens Ⅰ and Ⅲ.Conclusions These results reveal that gefitinib reduces pulmonary fibrosis induced by bleomycin in mice and suggest that administration of small molecule EGFR tyrosine kinase inhibitors has the potential to prevent pulmonary fibrosis by inhibiting the proliferation of mesenchymal cells, and that targeting tyrosine kinase receptors might be useful for the treatment of pulmonary fibrosis in humans.

  19. Improved detection of cardiac fibrosis : Biomarkers and novel imaging techniques

    NARCIS (Netherlands)

    Jong, S. de

    2015-01-01

    Cardiac cells are embedded in a collagen network that provides strength in the heart against tension that occurs during contraction and relaxation. In almost every cardiac disease increased collagen (fibrosis) is observed. Fibrosis has adverse effects on cardiac pump function and increases the risk

  20. LUNG TRANSPLANTATION IN PATIENTS WITH CYSTIC-FIBROSIS

    NARCIS (Netherlands)

    MANNES, GPM; VANDERBIJ, W

    1995-01-01

    Worldwide more than 600 heart-lung or lung transplantations have been performed in patients with cystic fibrosis and end-stage respiratory disease. At the University Hospital in Groningen 10 patients with cystic fibrosis underwent bilateral sequential lung transplantation until April 1994. The 1-yea

  1. Fibrosis: a structural modulator of Sinoatrial Node physiology and dysfunction

    Directory of Open Access Journals (Sweden)

    Thomas A Csepe

    2015-02-01

    Full Text Available Heart rhythm is initialized and controlled by the Sinoatrial Node (SAN, the primary pacemaker of the heart. The SAN is a heterogeneous multi-compartment structure characterized by clusters of specialized cardiomyocytes, enmeshed within strands of connective tissue or fibrosis. Intranodal fibrosis is emerging as an important modulator of structural and functional integrity of the SAN pacemaker complex. In adult human hearts, fatty tissue and fibrosis insulate the SAN from the hyperpolarizing effect of the surrounding atria while electrical communication between the SAN and right atrium is restricted to discrete SAN conduction pathways. The amount of fibrosis within the SAN is inversely correlated with heart rate, while age and heart size are positively correlated with fibrosis. Pathological upregulation of fibrosis within the SAN may lead to tachycardia-bradycardia arrhythmias and cardiac arrest, possibly due to SAN reentry and exit block, and is associated with atrial fibrillation, ventricular arrhythmias, heart failure and myocardial infarction. In this review, we will discuss current literature on the role of fibrosis in normal SAN structure and function, as well as the causes and consequences of SAN fibrosis upregulation in disease conditions.

  2. Paciente joven con disfonía: un caso de amiloidosis poco habitual

    Directory of Open Access Journals (Sweden)

    Alicia Maria Aquino Valdovinos

    2015-08-01

    Full Text Available La amiloidosis es una enfermedad caracterizada por el depósito extracelular de fibrillas compuestas por subunidades de bajo peso molecular de una variedad de proteínas. Puede ser clasificada según su distribución y según la proteina fibrilar constituyente. La prevalencia varía según el área geográfica estudiada y el pronóstico depende del tejido u órgano afectado. Presentamos el caso de un varón jóven con disfonía de larga evolución sin otros síntomas acompañantes en cuya nasofibroscopía se observaron una tumoración en regíon laríngea. En la anatomía patológica se observó material hialino congofílico compatible con amiloide. Se realizó además una biopsia rectal donde también se observó material amiloide. Debido a la poca sintomatología y a la ausencia de compromiso de un órgano que comprometa la vida, en este paciente se optó por un manejo conservador.

  3. Evaluación de la fibrosis hepática en la hepatitis crónica por virus C mediante la aplicación prospectiva del Sabadell's NIHCED score: Sabadell's Non Invasive, Hepatitis C Related-Cirrhosis Early Detection Score Prospective evaluation of liver fibrosis in chronic viral hepatitis C infection using the Sabadell NIHCED: non-invasive hepatitis C related cirrhosis early detection index

    Directory of Open Access Journals (Sweden)

    G. Bejarano

    2009-05-01

    Full Text Available Introducción: la hepatitis crónica por VHC cursa de forma asintomática desarrollando cirrosis hepática y sus complicaciones en un 20-40% de los casos. En estudios previos se ha demostrado que la fibrosis avanzada es un factor pronóstico fundamental. El método gold standard para la valoración del grado de fibrosis es la biopsia hepática. Nuestro grupo ha validado un índice predictivo, el NIHCED (Sabadell's Non Invasive, Hepatitis C related-Cirrosis Early Detection Score, basado en datos demográficos, analíticos y ecográficos para determinar la presencia de cirrosis. Objetivo: nuestro objetivo es el de evaluar si el NIHCED predice la presencia de fibrosis avanzada en los pacientes con hepatitis crónica por virus C. Material y métodos: estudio prospectivo donde se incluyeron pacientes con hepatitis crónica por VHC. Se les realizó una biopsia hepática y el NIHCED. El grado de fibrosis se correlacionó con el valor del NIHCED mediante curva de ROC y el coeficiente de correlación de Spearman. Resultados: se incluyeron un total de 321 pacientes (ratio hombre/mujer 1,27 con una edad media de 48 ± 14 años. La biopsia hepática mostró que 131 (30,5% no tenían fibrosis o era expansión portal, mientras que 190 (69,5% tenían fibrosis avanzada o cirrosis. Para un punto de corte de 6 puntos, la sensibilidad fue del 72%, especificidad del 76,3%, VPP del 81%, VPN del 63,7% y una precisión diagnóstica del 72,5%, con un área bajo la curva fue de 0,787 y un coeficiente de correlación de Spearman de r = 0,65. Conclusiones: el NIHCED predice la presencia de fibrosis avanzada en un elevado porcentaje de pacientes sin necesidad de realizar biopsia hepática.Introduction: liver disease resulting from chronic hepatitis C virus (HCV infection follows an asymptomatic course towards cirrhosis and its complications in 20-40% of cases. Earlier studies demonstrated that advanced fibrosis is a prognostic factor. The "gold standard" for the evaluation

  4. Microdureza de caries incipientes artificiales infiltradas con resinas de baja viscosidad antes y después del termociclado

    Directory of Open Access Journals (Sweden)

    X. Zamorano Pino

    2015-12-01

    Full Text Available Introducción: En Odontología Mínimamente Invasiva, el procedimiento denominado Infiltración de caries, pretende detener la progresión de las caries incipientes mediante el sellado de las microporosidades del esmalte afectado. Este tratamiento se realiza con la aplicación de resinas de baja viscosidad. El objetivo de este trabajo fue evaluar la microdureza superficial de caries incipientes artificiales infiltradas con resinas de baja viscosidad antes y después del proceso de termociclado. Metodología: Se provocaron caries artificiales mediante la inmersión de premolares y molares sanos en una solución de ácido láctico 0,1 M (pH 4,5 mantenidos a 37º C, durante 8 semanas. Posteriormente, las muestras fueron distribuidas en 3 grupos y se infiltraron con una de las resinas estudiadas; grupo A: ICON; B: XP-Bond y grupo C: Single Bond 2. Después se realizaron las pruebas de microdureza por indentación con un durómetro, seguido de un proceso de termociclado (1.000 ciclos y se midió nuevamente la microdureza. Resultados: Los valores de microdureza (VHN obtenidos fueron: ICON= 106,9±37,1 y postermociclado= 139,13±55, XP-Bond 51,01±21,8 y postermociclado= 103,14±58 y Single Bond 2= 25,4±5,8 y postermociclado= 26,6±3,5. Discusión: Posterior al proceso de termociclado (stress térmico no se encontraron diferencias estadísticamente significativas entre los grupos infiltrados con ICON® y XP-Bond, en cambio estas diferencias sí existían con respecto del grupo tratado con Single Bond 2.

  5. Vaccines for preventing infection with Pseudomonas aeruginosa in cystic fibrosis

    DEFF Research Database (Denmark)

    Johansen, H.K.; Gøtzsche, Peter C.; Johansen, Helle Krogh

    2008-01-01

    BACKGROUND: Chronic pulmonary infection in cystic fibrosis results in progressive lung damage. Once colonisation of the lungs with Pseudomonas aeruginosa occurs, it is almost impossible to eradicate. Vaccines, aimed at reducing infection with Pseudomonas aeruginosa, have been developed. OBJECTIVES......: To assess the effectiveness of vaccination against Pseudomonas aeruginosa in cystic fibrosis. SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register using the terms vaccines AND pseudomonas (last search May 2008) and PubMed using the terms vaccin* AND cystic...... fibrosis (last search May 2008). SELECTION CRITERIA: Randomised trials (published or unpublished) comparing Pseudomonas aeruginosa vaccines (oral, parenteral or intranasal) with control vaccines or no intervention in cystic fibrosis. DATA COLLECTION AND ANALYSIS: The authors independently selected trials...

  6. Progress of Research on Organic Fibrosis with Traditional Chinese Medicine

    Institute of Scientific and Technical Information of China (English)

    Qing-Lan Wang; Xiao-Ning Wang; Ping Liu

    2016-01-01

    Fibrosis is the remodeling and repair processes of chronic injuries. There are few effective therapies. Chinese medicine formula, the main pattern of traditional Chinese medicine (TCM) in clinic, applies a multi-component, multi-target and complicated approach in the treatment of diseases, and certainly shows good comprehensive therapeutic effects on fibrosis. In this review, the clinical study, effects and mechanism of action of Fuzheng Huayu recipe in the treatment of liver, pulmonary, and renal fibrosis was analyzed and evaluated. Meanwhile, based on the understanding of TCM pathogenesis for liver cirrhosis, this review will also briefly introduce the research of different classical Chinese medicine formulae with various functions in the treatment of experimental liver fibrosis induced by different methods, including comparing the efficacy, analyzing the action characteristics and mechanism of effective formulae, exploring pathological and biological basis of TCM diagnostic and therapeutic pattern, which will contribute to the research of TCM in the treatment of organ fibrosis.

  7. New insights into cystic fibrosis-related diabetes in children.

    Science.gov (United States)

    Ode, Katie L; Moran, Antoinette

    2013-09-01

    Cystic fibrosis-related diabetes (CFRD) is the most common complication of cystic fibrosis. It is associated with significantly increased morbidity and mortality in adults and children. Adolescents with cystic fibrosis have a much higher prevalence of diabetes than any other similar age population. Glucose abnormalities that precede diabetes are even more common, especially in children younger than 10 years. The pathophysiology of glucose metabolic abnormalities is poorly understood, but insulin insufficiency is clearly the main component. Findings from animal studies have provided insight into the pathophysiology of CFRD, and imply that carbohydrate metabolic abnormalities might begin at much younger ages than was previously thought in patients with cystic fibrosis, and might be related to the basic cystic fibrosis chloride channel defect. In this Review we explore present knowledge of CFRD in children and adolescents, and new data that indicate that the pathophysiology of CFRD begins in very young patients.

  8. sistema Web con JSP

    Directory of Open Access Journals (Sweden)

    César Viloria Núñez

    2014-01-01

    Full Text Available Este artículo presenta el desarrollo de un sistema de información que permite la adquisición y la administración de información relacionada con los signos vitales como la presión arterial, la frecuencia cardiaca y respiratoria, y la saturación de oxígeno en la sangre de un paciente. La implementación del sistema se basa en una solución Web, permitiendo así que médicos especialistas puedan monitorear a sus pacientes desde cualquier punto conectado a la red en tiempo real y, al mismo tiempo, dar indicaciones críticas al personal médico que se encuentra en el lugar con el paciente.

  9. Transporte forestal con cables

    OpenAIRE

    Anaya L. Héctor J.

    2012-01-01

    La explotación forestal es un problema fundamentalmente de transporte. El apeo y la preparación de las trozas, aunque a veces presentan algunas dificultades, son operaciones fáciles de resolver comparadas con la operación de transporte la cual absorbe del 60% al 70% o más del costo total del aprovechamiento del bosque. El 30% o 40% restante es absorbido por las faenas previas de apeo y troceo.

  10. Encuentros con Elena Poniatowska

    OpenAIRE

    Uzquiza González, José Ignacio

    2008-01-01

    El autor analiza, desde su encuentro con Elena Poniatowska, la vertiente de la literatura testimonial como literatura de mujeres. Un análisis interior de la relación entre realidad y ficción, entre Elena, Jesusa o Tinísima. The author analyzes testimonial literature from the perspective of female literature through his meeting with Elena Poniatowska. An analysis of reality vs. Fiction in Elena, Jesusa and Tinisima.

  11. pacientes con falla cardiaca

    Directory of Open Access Journals (Sweden)

    Diana Marcela Achury Saldaña

    2007-01-01

    Full Text Available Objetivo: determinar la adherencia al tratamiento de pacientes con falla cardiaca hospitalizados, al aplicar un plan educativo quefomenta el autocuidado.Método: estudio cuasiexperimental (entrevistas enfermera-paciente realizado entre diciembre de 2004 y mayo de 2006, con unamuestra de 50 pacientes seleccionados por conveniencia. Se diseñó un instrumento para evaluar los comportamientos de los pacientes,con base en algunos resultados de la adherencia y sus respectivos indicadores de la taxonomía NOC (Nursing out comes classification. Laadherencia al tratamiento fue medida en dos momentos: el primero durante la hospitalización, seguido de la aplicación del plan educativoantes del alta, que proporcionaba información en el manejo de su enfermedad desde una dimensión física, psicológica y social quepromueve el autocuidado; y el segundo un mes después del alta en su domicilio.Resultados: diferencias estadísticamente significativas (P=0,0001 que demuestran cómo mediante la capacitación al paciente enel manejo de su tratamiento farmacológico y no farmacológico, el establecimiento de una sana relación entre el profesional de enfermeríay el paciente, y la participación de la familia, se logra una total adherencia al tratamiento.Conclusiones: para lograr una adherencia total del paciente con falla cardiaca al tratamiento es necesario un proceso educativo y unseguimiento continuo y personalizado que motive permanentemente al paciente y se le reconozca el papel protagónico en su cuidado y manejo de la enfermedad.

  12. Using ultrasound Nakagami imaging to assess liver fibrosis in rats.

    Science.gov (United States)

    Ho, Ming-Chih; Lin, Jen-Jen; Shu, Yu-Chen; Chen, Chiung-Nien; Chang, King-Jen; Chang, Chien-Cheng; Tsui, Po-Hsiang

    2012-02-01

    This study explored the feasibility of using the ultrasound Nakagami image to assess the degree of liver fibrosis in rats. The rat has been widely used as a model in investigations of liver fibrosis. Ultrasound grayscale imaging makes it possible to observe fibrotic rat livers in real time. Statistical analysis of the envelopes of signals backscattered from rat livers may provide useful clues about the degree of liver fibrosis. The Nakagami-model-based image has been shown to be useful for characterizing scatterers in tissues by reflecting the echo statistics, and hence the Nakagami image may serve as a functional imaging tool for quantifying rat liver fibrosis. To validate this idea, fibrosis was induced in each rat liver (n=21) by an intraperitoneal injection of 0.5% dimethylnitrosamine. Livers were excised from rats for in vitro ultrasound scanning using a single-element transducer. The backscattered-signal envelopes of the acquired raw ultrasound signals were used for Nakagami imaging. The Metavir score determined by a pathologist was used to histologically quantify the degree of liver fibrosis. It was found that the Nakagami image could be used to distinguish different degrees of liver fibrosis in rats, since the average Nakagami parameter increased from 0.55 to 0.83 as the fibrosis score increased from 0 (i.e., normal) to 4. This correlation may be due to liver fibrosis in rats involving an increase in the concentration of local scatterers and the appearance of the periodic structures or clustering of scatterers that would change the backscattering statistics. The current findings indicate that the ultrasound Nakagami image has great potential as a functional imaging tool to complement the use of the conventional B-scan in animal studies of liver fibrosis.

  13. [Management of patients with pulmonary fibrosis].

    Science.gov (United States)

    Bestaev, D V; Karateev, D E; Nasonov, E L

    2014-01-01

    Rheumatoid arthritis (RA) is a chronic autoimmune systemic disease. Its systemic manifestations include interstitial lung lesions (ILL). According to morphological studies and X-ray computed tomography, the incidence of RA-associated ILL is 60-70% which gives reason to consider pulmonary fibrosis (PF) to be the main form of lung pathology in this disease. PF is a pathological process in the lungs characterized by high mortality rate and refractoriness to therapy. It is a heterogeneous group of disorders with progressive and irreversible destruction of lung architectonics due to scarification that in the end results in organ dysfunction, disturbed gaseous exchange and respiratory distress. Changes in the interstitial lung tissue resulting from local autoimmune rheumatoid inflammation develop by the same mechanisms that underlie idiopathic pulmonary fibrosis used as a model for classification, pathogenesis and treatment of RA-associated ILL. This review is focused on the therapeutic strategy for the management of PF in the context of consensus of the American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS) and Latin American Thoracic Association (ALAT, 2010/2011).

  14. Perifollicular fibrosis: pathogenetic role in androgenetic alopecia.

    Science.gov (United States)

    Yoo, Hyeon Gyeong; Kim, Jin Sook; Lee, Se Rah; Pyo, Hyun Keol; Moon, Hyung In; Lee, Jong Hee; Kwon, Oh Sang; Chung, Jin Ho; Kim, Kyu Han; Eun, Hee Chul; Cho, Kwang Hyun

    2006-06-01

    Androgenetic alopecia (AGA) is a dihydrotestosterone (DHT)-mediated process, characterized by continuous miniaturization of androgen reactive hair follicles and accompanied by perifollicular fibrosis of follicular units in histological examination. Testosterone (T: 10(-9)-10(-7) M) treatment increased the expression of type I procollagen at mRNA and protein level. Pretreatment of finasteride (10(-8) M) inhibited the T-induced type I procollagen expression at mRNA (40.2%) and protein levels (24.9%). T treatment increased the expression of transforming growth factor-beta 1 (TGF-beta1) at protein levels by 81.9% in the human scalp dermal fibroblasts (DFs). Pretreatment of finasteride decreased the expression of TGF-beta1 protein induced by an average of T (30.4%). The type I procollagen expression after pretreatment of neutralizing TGF-beta1 antibody (10 microg/ml) was inhibited by an average of 54.3%. Our findings suggest that T-induced TGF-beta1 and type I procollagen expression may contribute to the development of perifollicular fibrosis in the AGA, and the inhibitory effects on T-induced procollagen and TGF-beta1 expression may explain another possible mechanism how finasteride works in AGA.

  15. [Nutrition, cystic fibrosis and the digestive tract].

    Science.gov (United States)

    Olveira, Gabriel; Olveira, Casilda

    2008-05-01

    The prevalence of hyponutrition in cystic fibrosis is high although it may vary according to the different studies. Detection of hyponutrition should be done by combining different methods, depending on their availability. However, the simplest and most validated criterion is to measure at each visit the weight (and height in children) in order to calculate the body mass index and categorizing hyponutrition according to absolute criteria: in adults colon disease may also condition malnourishment. In patients with cystic fibrosis, a usual high-fat diet providing 120%-150% of the recommended calories is advised. If the nutritional goals are not achieved or maintained with diet modifications, artificial supplements may be added, although the recommendation for their use has not been endorsed by solid scientific evidences. The most frequently used preparations usually are polymeric or hypercaloric. The indications for enteral (through a tube, especially gastrostomy) or parenteral nutritional support are similar to those used in other pathologies. Dietary and nutritional control should be included in a multidisciplinary program allowing the improvement of the functional capacity and the quality of life and reducing, at least from a theoretical viewpoint, the morbimortality associated to malnourishment in these patients.

  16. [Vitamin E deficiency in cystic fibrosis].

    Science.gov (United States)

    Muñoz, C; Polanco, I; Hernanz, A; Carrasco, S; Barea, I; Murga, M L; Arroba, M L; Codoceo, R

    1987-12-01

    Plasma vitamin E levels were measured by high performance liquid chromatography in 42 children with cystic fibrosis and were correlated with the following parameters: sex, age, time of follow-up, clinical evolution (Schwachman score), vitamin E/cholesterol and faecal fat excretion. All children in this study received oral alfa-tocoferol (50-100 mg daily) from the diagnosis. According to the vitamin E level patients were distributed in two groups. Group I: 27 patients (64.3%) with normal concentrations. Group II: 15 patients (35.7%) with decreases plasma levels but without clinical manifestations. Steatorrhea was present in all children except 4 patients from group I and one patient from group II. On the other hand, vitamin E/cholesterol was normal in 80% of patients with vitamin E deficiency (group II). We did not find any correlation between plasma vitamin E levels and the different clinical and biological parameters studied. Further studies should be carried out to throw more light on the mechanism underlying the pathogenesis of vitamin E deficiency in patients with cystic fibrosis.

  17. Abdominal manifestations of cystic fibrosis in children

    Energy Technology Data Exchange (ETDEWEB)

    Chaudry, Gulraiz; Navarro, Oscar M.; Levine, Daniel S.; Oudjhane, Kamaldine [University of Toronto, Department of Diagnostic Imaging, Hospital for Sick Children, Toronto, ON (Canada)

    2006-03-15

    Pulmonary complications remain the main cause of mortality in cystic fibrosis, but the presenting symptoms in children are often related to gastrointestinal or pancreaticobiliary disease. Furthermore, abdominal manifestations are now seen throughout childhood, from infancy to adolescence. The child might present in the neonatal period with meconium ileus or its attendant complications. The older child might present with distal intestinal obstruction syndrome or colonic stricture secondary to high doses of pancreatic enzyme replacement. Less-common gastrointestinal manifestations include intussusception, duodenitis and fecal impaction of the appendix. Most children also show evidence of exocrine pancreatic deficiency. Radiologically, the combination of fat deposition and pancreatic fibrosis leads to varying CT and MR appearances. A higher than normal incidence of pancreatic cysts and calcification is also seen. Decreased transport of water and chloride also increases the viscosity of bile, with subsequent obstruction of the biliary ductules. If extensive, this can progress to obstructive cirrhosis, portal hypertension and esophageal varices. Diffuse fatty infiltration, hypersplenism and gallstones are also commonly seen in these patients. We present a pictorial review of the radiological appearance of these abdominal manifestations. The conditions are dealt with individually, together with typical appearances in various imaging modalities. (orig.)

  18. Global impact of bronchiectasis and cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Margarida Redondo

    2016-09-01

    To understand variation in the aetiology, microbiology and burden of bronchiectasis and cystic fibrosis across different global healthcare systems.; Bronchiectasis is the term used to refer to dilatation of the bronchi that is usually permanent and is associated with a clinical syndrome of cough, sputum production and recurrent respiratory infections. It can be caused by a range of inherited and acquired disorders, or may be idiopathic in nature. The most well recognised inherited disorder in Western countries is cystic fibrosis (CF, an autosomal recessive condition that leads to progressive bronchiectasis, bacterial infection and premature mortality. Both bronchiectasis due to CF and bronchiectasis due to other conditions are placing an increasing burden on healthcare systems internationally. Treatments for CF are becoming more effective leading to more adult patients with complex healthcare needs. Bronchiectasis not due to CF is becoming increasingly recognised, particularly in the elderly population. Recognition is important and can lead to identification of the underlying cause, appropriate treatment and improved quality of life. The disease is highly diverse in its presentation, requiring all respiratory physicians to have knowledge of the different “bronchiectasis syndromes”. The most common aetiologies and presenting syndromes vary depending on geography, with nontuberculous mycobacterial disease predominating in some parts of North America, post-infectious and idiopathic disease predominating in Western Europe, and post-tuberculosis bronchiectasis dominating in South Asia and Eastern Europe. Ongoing global collaborative studies will greatly advance our understanding of the international impact of bronchiectasis and CF.

  19. Fibroblasts in fibrosis: novel roles and mediators

    Directory of Open Access Journals (Sweden)

    Ryan Thomas Kendall

    2014-05-01

    Full Text Available Fibroblasts are the most common cell type of the connective tissues found throughout the body and the principal source of the extensive extracellular matrix (ECM characteristic of these tissues. They are also the central mediators of the pathological fibrotic accumulation of ECM and the cellular proliferation and differentiation that occurs in response to prolonged tissue injury and chronic inflammation. The transformation of the fibroblast cell lineage involves classical developmental signaling programs and includes a surprisingly diverse range of precursor cell types—most notably, myofibroblasts that are the apex of the fibrotic phenotype. Myofibroblasts display exaggerated ECM production; constitutively secrete and are hypersensitive to chemical signals such as cytokines, chemokines, and growth factors; and are endowed with a contractile apparatus allowing them to manipulate the ECM fibers physically to close open wounds. In addition to ECM production, fibroblasts have multiple concomitant biological roles, such as in wound healing, inflammation, and angiogenesis, which are each interwoven with the process of fibrosis. We now recognize many common fibroblast-related features across various physiological and pathological protracted processes. Indeed, a new appreciation has emerged for the role of noncancerous fibroblast interactions with tumors in cancer progression. Although the predominant current clinical treatments of fibrosis involve nonspecific immunosuppressive and anti-proliferative drugs, a variety of potential therapies under investigation specifically target fibroblast biology.

  20. CFTR protein repair therapy in cystic fibrosis.

    Science.gov (United States)

    Quintana-Gallego, Esther; Delgado-Pecellín, Isabel; Calero Acuña, Carmen

    2014-04-01

    Cystic fibrosis is a single gene, autosomal recessive disorder, in which more than 1,900 mutations grouped into 6 classes have been described. It is an example a disease that could be well placed to benefit from personalised medicine. There are currently 2 very different approaches that aim to correct the basic defect: gene therapy, aimed at correcting the genetic alteration, and therapy aimed at correcting the defect in the CFTR protein. The latter is beginning to show promising results, with several molecules under development. Ataluren (PTC124) is a molecule designed to make the ribosomes become less sensitive to the premature stop codons responsible for class i mutations. Lumacaftor (VX-809) is a CFTR corrector directed at class ii mutations, among which Phe508del is the most frequent, with encouraging results. Ivacaftor (VX-770) is a potentiator, the only one marketed to date, which has shown good efficacy for the class iii mutation Gly551Asp in children over the age of 6 and adults. These drugs, or a combination of them, are currently undergoing various clinical trials for other less common genetic mutations. In the last 5 years, CFTR has been designated as a therapeutic target. Ivacaftor is the first drug to treat the basic defect in cystic fibrosis, but only provides a response in a small number of patients. New drugs capable of restoring the CFTR protein damaged by the most common mutations are required.

  1. PROFILEing idiopathic pulmonary fibrosis: rethinking biomarker discovery

    Directory of Open Access Journals (Sweden)

    Toby M. Maher

    2013-06-01

    Full Text Available Despite major advances in the understanding of the pathogenesis of idiopathic pulmonary fibrosis (IPF, diagnosis and management of the condition continue to pose significant challenges. Clinical management of IPF remains unsatisfactory due to limited availability of effective drug therapies, a lack of accurate indicators of disease progression, and an absence of simple short-term measures of therapeutic response. The identification of more accurate predictors of prognosis and survival in IPF would facilitate counseling of patients and their families, aid communication among clinicians, and would guide optimal timing of referral for transplantation. Improvements in molecular techniques have led to the identification of new disease pathways and a more targeted approach to the development of novel anti-fibrotic agents. However, despite an increased interest in biomarkers of IPF disease progression there are a lack of measures that can be used in early phase clinical trials. Careful longitudinal phenotyping of individuals with IPF together with the application of novel omics-based technology should provide important insights into disease pathogenesis and should address some of the major issues holding back drug development in IPF. The PROFILE (Prospective Observation of Fibrosis in the Lung Clinical Endpoints study is a currently enrolling, prospective cohort study designed to tackle these issues.

  2. Targeting ion channels in cystic fibrosis.

    Science.gov (United States)

    Mall, Marcus A; Galietta, Luis J V

    2015-09-01

    Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause a characteristic defect in epithelial ion transport that plays a central role in the pathogenesis of cystic fibrosis (CF). Hence, pharmacological correction of this ion transport defect by targeting of mutant CFTR, or alternative ion channels that may compensate for CFTR dysfunction, has long been considered as an attractive approach to a causal therapy of this life-limiting disease. The recent introduction of the CFTR potentiator ivacaftor into the therapy of a subgroup of patients with specific CFTR mutations was a major milestone and enormous stimulus for seeking effective ion transport modulators for all patients with CF. In this review, we discuss recent breakthroughs and setbacks with CFTR modulators designed to rescue mutant CFTR including the common mutation F508del. Further, we examine the alternative chloride channels TMEM16A and SLC26A9, as well as the epithelial sodium channel ENaC as alternative targets in CF lung disease, which remains the major cause of morbidity and mortality in patients with CF. Finally, we will focus on the hurdles that still need to be overcome to make effective ion transport modulation therapies available for all patients with CF irrespective of their CFTR genotype.

  3. Imaging the Abdominal Manifestations of Cystic Fibrosis

    Directory of Open Access Journals (Sweden)

    C. D. Gillespie

    2017-01-01

    Full Text Available Cystic fibrosis (CF is a multisystem disease with a range of abdominal manifestations including those involving the liver, pancreas, and kidneys. Recent advances in management of the respiratory complications of the disease has led to a greater life expectancy in patients with CF. Subsequently, there is increasing focus on the impact of abdominal disease on quality of life and survival. Liver cirrhosis is the most important extrapulmonary cause of death in CF, yet significant challenges remain in the diagnosis of CF related liver disease. The capacity to predict those patients at risk of developing cirrhosis remains a significant challenge. We review representative abdominal imaging findings in patients with CF selected from the records of two academic health centres, with a view to increasing familiarity with the abdominal manifestations of the disease. We review their presentation and expected imaging findings, with a focus on the challenges facing diagnosis of the hepatic manifestations of the disease. An increased familiarity with these abdominal manifestations will facilitate timely diagnosis and management, which is paramount to further improving outcomes for patients with cystic fibrosis.

  4. Nephrogenic systemic fibrosis: late skin manifestations

    DEFF Research Database (Denmark)

    Bangsgaard, Nannie; Marckmann, Peter; Rossen, Kristian

    2009-01-01

    BACKGROUND: Nephrogenic systemic fibrosis (NSF) is a serious disease that occurs in patients with severe renal disease and is believed to be caused by gadolinium-containing contrast agents. A detailed description of the late skin manifestations of NSF is important to help dermatologists and nephr......BACKGROUND: Nephrogenic systemic fibrosis (NSF) is a serious disease that occurs in patients with severe renal disease and is believed to be caused by gadolinium-containing contrast agents. A detailed description of the late skin manifestations of NSF is important to help dermatologists...... confluent dermal plaques with thickening and hardening. In contrast, 3 patients presented with wrinkled, redundant skin as seen in cutis laxa. Patients with NSF had significantly poorer scores than control patients on the Daily Life Quality Index (mean [SD], 11. 4 [7.4] vs 1.5 [2. 3]; P ...: This descriptive case series of patients with NSF gives a detailed clinical picture of the skin manifestations late in the disease. It demonstrates that the clinical picture in the late stage has a varied presentation and that NSF has a significant effect on the quality of life....

  5. Imaging findings in congenital hepatic fibrosis

    Energy Technology Data Exchange (ETDEWEB)

    Akhan, Okan [Department of Radiology, Hacettepe University, School of Medicine, 06100 Ankara (Turkey)]. E-mail: akhano@tr.net; Karaosmanoglu, Ali Devrim [Department of Radiology, Hacettepe University, School of Medicine, 06100 Ankara (Turkey); Ergen, Bilge [Department of Radiology, Hacettepe University, School of Medicine, 06100 Ankara (Turkey)

    2007-01-15

    Congenital hepatic fibrosis (CHF) is a rare congenital multisystemic disorder, mostly inherited in autosomal recessive fashion, primarily affecting renal and hepatobiliary systems. Main underlying process of the disease is the malformation of the ductal plate, the embryological precursor of the biliary system, and secondary biliary strictures and periportal fibrosis ultimately leading to portal hypertension. The natural course of the disease is highly variable ranging from minimally symptomatic disease to true cirrhosis of the liver. However, in most patients the most common manifestations of the diseases that are related to portal hypertension, particularly splenomegaly and bleeding varices. Many other disease processes may co-exist with the disease including Caroli's disease, choledochal cysts and autosomal recessive polycystic kidney disease (ARPKD) reflecting the mulstisystemic nature of the disease. The associating biliary ductal disease led the authors to think that all these entities are a continuum and different reflections of the same underlying pathophysiological process. Although, conventional method of diagnosis of CHF is the liver biopsy the advent of imaging technologies and modalities, today, may permit the correct diagnosis in a non-invasive manner. Characteristic imaging features are generally present and recognition of these findings may obviate liver biopsy while preserving the diagnostic accuracy. In this article, it is aimed to increase the awareness of the practising radiologists to the imaging findings of this uncommon clinical disorder and trail the blaze for future articles relating to this issue.

  6. Lectura con adolescentes

    Directory of Open Access Journals (Sweden)

    Silvia Méndez Anchía

    2007-01-01

    Full Text Available Con base en la premisa de que la lectura de textos literarios tiene una función formadora y que esta se acentúa en la adolescencia, me propongo demostrar que el cuento “Rapunzel” puede utilizarse como estrategia para explorar algunas situaciones que los sujetos adolescentes perciben como particulares en relación con su vida, pero que se inscriben dentro de grandes problemáticas estudiadas por varias disciplinas. Para ello, he identificado, desde dos marcos de referencia (sociológico y psicoanalítico, diversas problemáticas y discursos que se desprenden de la lectura del cuento realizada por dos mujeres adolescentes, quienes respondieron una guía de lectura y participaron en una entrevista a profundidad. Concluyo que la lectura y comentario del cuento hacen posible que una serie de experiencias que los sujetos adolescentes viven como únicas (como el embarazo de una amiga, las críticas de las personas adultas y las exigencias de padres y madres, ingresen en el circuito de los conocimientos generales al relacionarlas con los discursos y problemáticas en que se inscriben (por ejemplo, el discurso de la “crisis” de la adolescencia, el enfoque de derechos humanos, el mundo fantasmático materno. Por ello, recomiendo la lectura y comentario de textos literarios como estrategia didáctica para contribuir a la elaboración de la subjetividad de personas adolescentes.

  7. Pudrición basal causada por Phytophthora capcisi en plantas de chile tratadas con vermicompost

    Directory of Open Access Journals (Sweden)

    Lidieth Uribe-Lorío

    2014-07-01

    Full Text Available El objetivo de este trabajo fue evaluar a nivel de invernadero el efecto de vermicompost sobre la incidencia y severidad de Phytophthora capcisi en plantas de chile (Capsicum annuum. Esta investigación se realizó entre febrero y marzo del 2012 en el Centro de Investigaciones Agronómicas, Sabanilla, San José, Costa Rica. Se utilizó un diseño completamente al azar con cuatro repeticiones y ocho plantas por unidad experimental en un arreglo factorial con los factores de dosis (0, 25% y 50% v/v vermicompost:suelo e inoculación (0 y 500 zoosporas por gramo de suelo. Se trasplantaron plántulas de 35 días, que fueron inoculadas dos semanas después del trasplante. La aplicación de vermicompost provocó un aumento significativo del peso fresco foliar y del peso seco foliar y radical; a mayor dosis, mayor fue el incremento. Las plantas de todos los tratamientos que incluyeron inoculación con P. capsici presentaron síntomas de la enfermedad en la raíz. Los valores de incidencia y severidad fueron mayores para el tratamiento con 50% de abono. En este tratamiento se presentaron síntomas de marchitez. La ausencia de diferencias en las variables de peso fresco y seco entre los tratamientos inoculados y sin inocular, a los que se adicionó abono al 25%, sugiere que esta dosis podría compensar el daño causado por el patógeno. Se observó una menor concentración de nutrimentos en los tejidos de las plantas inoculadas lo que indica que el daño causado a la raíz pudo haber afectado la adquisición de nutrimentos.

  8. Cementos con cenizas volantes

    Directory of Open Access Journals (Sweden)

    Ossa M., Mauricio

    1984-03-01

    additions of 20 and 30% .

    Casi la generalidad de los estudios realizados sobre cementos con adición de cenizas volantes se refieren a sus características y comportamiento en pastas, morteros y hormigones, siempre en relación con aquéllos del cemento portland. Esta vez, se desarrolló un trabajo experimental orientado a relacionar entre sí los cementos con adiciones de cenizas volantes y de puzolana natural. Para ello se fabricaron a escala de laboratorio cementos de ambos tipos, empleando como materias primas comunes clinker y yeso y, como variables, diferentes porcentajes de las dos adiciones, que cumplieron previamente los requisitos normalizados en cuanto a sus actividades puzolánicas. La calidad de los cementos fabricados resultó adecuada y concordante con la del cemento portland-puzolánico obtenido a escala industrial con los mismos clinker, yeso y puzolana natural de este estudio. Posteriormente, se determinaron las características de los cementos experimentales y se confeccionaron morteros normales para la realización de ensayos físicos y mecánicos. Los resultados de ensayos indicaron que los cementos con adición de cenizas volantes (CCV requieren menos agua para consistencia normal, presentan tiempos de fraguado mayores y expansiones en autoclave menores que los cementos con adición de puzolana (CP. Los calores de hidratación a 7 y 28 días de edad fueron aproximadamente similares para ambos tipos de cemento. En morteros normales, los cementos CCV mostraron menor retracción de secado, mayor retentividad y mayor fluidez (para igual cantidad de agua que los cementos CP. En los ensayos de exudación se observó que ésta depende más de la finura que el tipo de adición. Finalmente, los ensayos mecánicos señalaron que las resistencias a compresión y flexotracción de los morteros con cementos CCV son menores a edades inferiores que 14 días (del orden de 5 a 10% a un día de edad, pero que a partir de entonces pasan a ser mayores que las de

  9. Pancreatitis autoinmune asociada a fibrosis retroperitoneal: evolución tras dos años de seguimiento Autoimmune pancreatitis associated with retroperitoneal fibrosis: outcome after 24 months of follow-up

    Directory of Open Access Journals (Sweden)

    M. Romero

    2008-10-01

    Full Text Available Introducción: la pancreatitis autoinmune es un tipo de pancreatitis crónica caracterizado por un infiltrado linfoplasmocitario y una elevación de IgG e IgG4, que se ha descrito asociada a diversas manifestaciones extrapancreáticas y enfermedades autoinmunes, lo cual apoya la teoría de un mecanismo autoinmune fisiopatólogico de base. Caso clínico: presentamos el caso de un varón que debutó simultáneamente con una pancreatitis autoinmune asociada a fibrosis retroperitoneal y lesión de la vía biliar extrapancreática, con respuesta total tras tratamiento con corticoides durante 4 meses y ausencia de recurrencia tras 24 meses de seguimiento. Discusión: la pancreatitis autoinmune es un tipo de pancreatitis crónica que probablemente forme parte de un proceso sistémico autoinmune, cuyas manifestaciones extrapancreáticas más frecuentes son la fibrosis retroperitoneal y las lesiones de la vía biliar extrapancreática. Su correcto diagnóstico e inicio precoz del tratamiento puede favorecer la resolución completa de las lesiones, principalmente en los casos de bajo grado de actividad, con menor probabilidad de recurrencia.Introduction: autoimmune pancreatitis is a kind of chronic pancreatitis characterized by the presence of lymphoplasmacytic infiltration and severely elevated serum IgG and IgG4, which has been associated to many extrapancreatic lesions and other autoimmune disorders, leading to the theory of an autoimmune mechanism involved in the pathogenesis of this disease. Case report: we report the case of a man who simultaneously presented with autoimmune pancreatitis associated with retroperitonal fibrosis, and a lesion of the extrapancreatic bile duct, with total response to corticosteroid treatment for 4 moths and absence of recurrence after 24 months of follow-up. Discussion: autoimmune pancreatitis is a kind of chronic pancreatitis that is probably a part of a systemic autoinmune disease, with retroperitoneal fibrosis and

  10. Characterization of patient’s inflammatory pulpal diseases. Caracterización de pacientes con afecciones pulpares inflamatorias

    Directory of Open Access Journals (Sweden)

    Ana Isabel Palenque Guillemí

    2008-06-01

    grupo de edad más afectado fue 35 a 59; los dientes más afectados fueron los molares inferiores. La causa fundamental fue la caries dental; la obturación fue el tratamiento previo que más se presentó; el 46 % había asistido en más de una ocasión con el mismo proceso; la afección pulpar que predominó fue la pulpitis aguda irreversible. Conclusión: La afección pulpar más común en nuestro medio fue la pulpitis aguda irreversible y en las edades entre 35 y 59 años. 

  11. Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines.

    Science.gov (United States)

    Schwarzenberg, Sarah Jane; Hempstead, Sarah E; McDonald, Catherine M; Powers, Scott W; Wooldridge, Jamie; Blair, Shaina; Freedman, Steven; Harrington, Elaine; Murphy, Peter J; Palmer, Lena; Schrader, Amy E; Shiel, Kyle; Sullivan, Jillian; Wallentine, Melissa; Marshall, Bruce C; Leonard, Amanda Radmer

    2016-11-01

    Nutrition is integral to the care of individuals with cystic fibrosis (CF). Better nutritional status is associated with improved pulmonary function. In some individuals with CF, enteral tube feeding can be useful in achieving optimal nutritional status. Current nutrition guidelines do not include detailed recommendations for enteral tube feeding. The Cystic Fibrosis Foundation convened an expert panel to develop enteral tube feeding recommendations based on a systematic review of the evidence and expert opinion. These guidelines address when to consider enteral tube feeding, assessment of confounding causes of poor nutrition in CF, preparation of the patient for placement of the enteral feeding tube, management of the tube after placement and education about enteral feeding. These recommendations are intended to guide the CF care team, individuals with CF, and their families through the enteral tube feeding process.

  12. Triptolide Mitigates Radiation-Induced Pulmonary Fibrosis.

    Science.gov (United States)

    Yang, Shanmin; Zhang, Mei; Chen, Chun; Cao, Yongbin; Tian, Yeping; Guo, Yangsong; Zhang, Bingrong; Wang, Xiaohui; Yin, Liangjie; Zhang, Zhenhuan; O'Dell, Walter; Okunieff, Paul; Zhang, Lurong

    2015-11-01

    Triptolide (TPL) may mitigate radiation-induced late pulmonary side effects through its inhibition of global pro-inflammatory cytokines. In this study, we evaluated the effect of TPL in C57BL/6 mice, the animals were exposed to radiation with vehicle (15 Gy), radiation with TPL (0.25 mg/kg i.v., twice weekly for 1, 2 and 3 months), radiation and celecoxib (CLX) (30 mg/kg) and sham irradiation. Cultured supernatant of irradiated RAW 264.7 and MLE-15 cells and lung lysate in different groups were enzyme-linked immunosorbent assays at 33 h. Respiratory rate, pulmonary compliance and pulmonary density were measured at 5 months in all groups. The groups exposed to radiation with vehicle and radiation with TPL exhibited significant differences in respiratory rate and pulmonary compliance (480 ± 75/min vs. 378 ± 76/min; 0.6 ± 0.1 ml/cm H2O/p kg vs. 0.9 ± 0.2 ml/cm H2O/p kg). Seventeen cytokines were significantly reduced in the lung lysate of the radiation exposure with TPL group at 5 months compared to that of the radiation with vehicle group, including profibrotic cytokines implicated in pulmonary fibrosis, such as IL-1β, TGF- β1 and IL-13. The radiation exposure with TPL mice exhibited a 41% reduction of pulmonary density and a 25% reduction of hydroxyproline in the lung, compared to that of radiation with vehicle mice. The trichrome-stained area of fibrotic foci and pathological scaling in sections of the mice treated with radiation and TPL mice were significantly less than those of the radiation with vehicle-treated group. In addition, the radiation with TPL-treated mice exhibited a trend of improved survival rate compared to that of the radiation with vehicle-treated mice at 5 months (83% vs. 53%). Three radiation-induced profibrotic cytokines in the radiation with vehicle-treated group were significantly reduced by TPL treatment, and this partly contributed to the trend of improved survival rate and pulmonary density and function and the decreased severity of

  13. Monocyte Subsets in Schistosomiasis Patients with Periportal Fibrosis

    Directory of Open Access Journals (Sweden)

    Jamille Souza Fernandes

    2014-01-01

    Full Text Available A major issue with Schistosoma mansoni infection is the development of periportal fibrosis, which is predominantly caused by the host immune response to egg antigens. Experimental studies have pointed to the participation of monocytes in the pathogenesis of liver fibrosis. The aim of this study was to characterize the subsets of monocytes in individuals with different degrees of periportal fibrosis secondary to schistosomiasis. Monocytes were classified into classical (CD14++CD16−, intermediate (CD14++CD16+, and nonclassical (CD14+CD16++. The expressions of monocyte markers and cytokines were assessed using flow cytometry. The frequency of classical monocytes was higher than the other subsets. The expression of HLA-DR, IL-6, TNF-α, and TGF-β was higher in monocytes from individuals with moderate to severe fibrosis as compared to other groups. Although no differences were observed in receptors expression (IL-4R and IL-10R between groups of patients, the expression of IL-12 was lower in monocytes from individuals with moderate to severe fibrosis, suggesting a protective role of this cytokine in the development of fibrosis. Our data support the hypothesis that the three different monocyte populations participate in the immunopathogenesis of periportal fibrosis, since they express high levels of proinflammatory and profibrotic cytokines and low levels of regulatory markers.

  14. Cystic fibrosis transmembrane conductance regulator chloride channel blockers: Pharmacological, biophysical and physiological relevance

    Institute of Scientific and Technical Information of China (English)

    Paul; Linsdell

    2014-01-01

    Dysfunction of the cystic fibrosis transmembrane con-ductance regulator(CFTR) chloride channel causes cys-tic fibrosis, while inappropriate activity of this channeloccurs in secretory diarrhea and polycystic kidney dis-ease. Drugs that interact directly with CFTR are there-fore of interest in the treatment of a number of diseasestates. This review focuses on one class of small mol-ecules that interacts directly with CFTR, namely inhibi-tors that act by directly blocking chloride movementthrough the open channel pore. In theory such com-pounds could be of use in the treatment of diarrheaand polycystic kidney disease, however in practice allknown substances acting by this mechanism to inhibitCFTR function lack either the potency or specificity forin vivo use. Nevertheless, this theoretical pharmaco-logical usefulness set the scene for the developmentof more potent, specific CFTR inhibitors. Biophysically,open channel blockers have proven most useful as ex-perimental probes of the structure and function of theCFTR chloride channel pore. Most importantly, the useof these blockers has been fundamental in developing afunctional model of the pore that includes a wide innervestibule that uses positively charged amino acid sidechains to attract both permeant and blocking anionsfrom the cell cytoplasm. CFTR channels are also subjectto this kind of blocking action by endogenous anionspresent in the cell cytoplasm, and recently this blocking effect has been suggested to play a role in the physio-logical control of CFTR channel function, in particular as a novel mechanism linking CFTR function dynamically to the composition of epithelial cell secretions. It has also been suggested that future drugs could target this same pathway as a way of pharmacologically increasing CFTR activity in cystic fibrosis. Studying open channel blockers and their mechanisms of action has resulted in significant advances in our understanding of CFTR as a pharmacological target in disease states, of

  15. Asociación del anticuerpo anti-C1q con las manifestaciones clínicas, hematológicas e inmunológicas en el Lupus Eritematoso Sistémico : Estudio observacional retrospectivo de 135 casos.

    OpenAIRE

    Córica, María Emilia

    2012-01-01

    : Las concentraciones de anticuerpo anti-C1q están elevadas en los pacientes afectados de LES. Según algunos estudios la presencia de este anticuerpo está asociada a la nefropatía lúpica, sin embargo en otros estudios esta relación no se comprobó. En nuestro trabajo relacionamos la positividad del anticuerpo anti-C1q con las manifestaciones clínicas, hematológicas e inmunológicas de la enfermedad. A diferencia de estudios previos sólo pudimos establecer una relación estadísticamente significa...

  16. Ormond's disease or secondary retroperitoneal fibrosis? An overview of retroperitoneal fibrosis

    Energy Technology Data Exchange (ETDEWEB)

    Heckmann, M.; Uder, M.; Kuefner, M.A.; Heinrich, M.C. [Universitaetsklinikum Erlangen (Germany). Radiologisches Inst.

    2009-04-15

    Retroperitoneal fibrosis represents a rare inflammatory disease. About two thirds of all cases seem to be idiopathic (= Ormond's disease). The remaining one third is secondary and may be ascribed to infections, trauma, radiation therapy, malignant diseases, and the use of certain drugs. Up to 15 % of patients have additional fibrotic processes outside the retroperitoneum. The clinical symptoms of retroperitoneal fibrosis are non-specific. In sonography retroperitoneal fibrosis appears as a retroperitoneal hypoechoic mass which can involve the ureters and thus cause hydronephrosis. Intravenous urography and MR urography can demonstrate the typical triad of medial deviation and extrinsic compression of the ureters and hydronephrosis. CT and MRI are the modalities of choice for the diagnosis and follow-up of this disease. The lesion typically begins at the level of the fourth or fifth lumbar vertebra and appears as a plaque, encasing the aorta and the inferior vena cava and often enveloping and medially displacing the ureters. In unenhanced CT, retroperitoneal fibrosis appears as a mass that is isodense with muscle. When using MRI, the mass is hypointense in T1-weighted images and of variable intensity in T2-weighted images according to its stage: it may be hyperintense in early stages, while the tissue may have a low signal in late stages. After the administration of contrast media, enhancement is greatest in the early inflammatory phase and minimal in the late fibrotic phase. Dynamic gadolinium enhancement can be useful for assessing disease activity, monitoring response to treatment, and detecting relapse. To differentiate retroperitoneal masses, diffusion-weighted MRI may provide useful information. (orig.)

  17. Activated MCTC mast cells infiltrate diseased lung areas in cystic fibrosis and idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Löfdahl Claes-Göran

    2011-10-01

    Full Text Available Abstract Background Although mast cells are regarded as important regulators of inflammation and tissue remodelling, their role in cystic fibrosis (CF and idiopathic pulmonary fibrosis (IPF has remained less studied. This study investigates the densities and phenotypes of mast cell populations in multiple lung compartments from patients with CF, IPF and never smoking controls. Methods Small airways, pulmonary vessels, and lung parenchyma were subjected to detailed immunohistochemical analyses using lungs from patients with CF (20 lung regions; 5 patients, IPF (21 regions; 7 patients and controls (16 regions; 8 subjects. In each compartment the densities and distribution of MCT and MCTC mast cell populations were studied as well as the mast cell expression of IL-6 and TGF-β. Results In the alveolar parenchyma in lungs from patients with CF, MCTC numbers increased in areas showing cellular inflammation or fibrosis compared to controls. Apart from an altered balance between MCTC and MCT cells, mast cell in CF lungs showed elevated expression of IL-6. In CF, a decrease in total mast cell numbers was observed in small airways and pulmonary vessels. In patients with IPF, a significantly elevated MCTC density was present in fibrotic areas of the alveolar parenchyma with increased mast cell expression of TGF-β. The total mast cell density was unchanged in small airways and decreased in pulmonary vessels in IPF. Both the density, as well as the percentage, of MCTC correlated positively with the degree of fibrosis. The increased density of MCTC, as well as MCTC expression of TGF-β, correlated negatively with patient lung function. Conclusions The present study reveals that altered mast cell populations, with increased numbers of MCTC in diseased alveolar parenchyma, represents a significant component of the histopathology in CF and IPF. The mast cell alterations correlated to the degree of tissue remodelling and to lung function parameters. Further

  18. con problemas de aprendizaje

    Directory of Open Access Journals (Sweden)

    Claudia Jaquelina González Trujillo

    2007-01-01

    Full Text Available Un grupo de niños con diversos problemas de aprendizaje fue atendido bajo un programa de intervención de integración y adaptación social. Mejoras importantes se obtienen en competencias académicas bajo el presente programa de intervención y delimitan áreas de oportunidad para la mejora en aspectos cognitivos como el del proceso de atención e integración social. Las implicaciones de los resultados se discuten bajo un programa de apoyo psicopedagógico para la educación especial.

  19. Mayonesa con quitosano

    OpenAIRE

    Gaffrey, María Celeste

    2014-01-01

    Introducción: El quitosano es un polímero natural que se obtiene a partir de la quitina, la cual forma parte de la estructura de soporte de numerosos organismos vivos, tales como artrópodos (crustáceos e insectos), moluscos y hongos. Presenta propiedades aplicables en los alimentos, como estabilizante, emulsificante, y quelante. No puede ser digerido por los seres humanos por lo cual está considerado como una fibra dietética con un contenido calórico cero. Objetivos: Evaluar...

  20. Atender con ansiedad

    Directory of Open Access Journals (Sweden)

    Alberto Acosta

    2009-10-01

    Full Text Available Tener una personalidad ansiosa o estar ansioso en una determinada situación hace que atendamos de modo diferente a lo que acontece. Investigaciones recientes están descubriendo las relaciones específicas de la ansiedad-rasgo y de la ansiedad-estado con diferentes procesos atencionales. La intervención terapéutica para aliviar los trastornos de ansiedad, tan frecuentes en nuestra época, se va a beneficiar de este conocimiento.

  1. Creo con mis dedos

    OpenAIRE

    S??nchez Aniceto, Monta??a

    2015-01-01

    Las artes pl??sticas son muy importantes para los ni??os/as sobre todo para Educaci??n Infantil ya que promueven la creatividad mediante diferentes recursos y t??cnicas lo que favorece su motivaci??n en las competencias desde la edad temprana hasta la adolescencia. Es la primera forma que tiene el ni??o/a de expresarse en el mundo (a trav??s de los garabatos), de comunicarse, compartir sus emociones con los dem??s, creando su propio lenguaje que evolucionar?? hacia el lenguaje oral y escri...

  2. Eugenistas, pero con prudencia

    Directory of Open Access Journals (Sweden)

    Pogliano, Claudio

    1999-12-01

    Full Text Available Thinking that one could not describe eugenics like a unique movement, since numerous bound varians took place related to the geographical and cultural context, this article tries to demostrate the peculiarity of the Italian case. If already in 1889 Giuseppe Sergi wanted that the artificial selection take it to end what should make the natural, avoiding the risk of the so called «degeneration», only in the face of the First World War seems to grow the alarm for the decadent quality of the population, finding a more and more wide echo. In 1919 the Siges was born (Società italiana de genetica ed eugenica shocked under the impression of the difusse fear about the butcher the war had caused. From there from now on fastens a «nazional» direction closely related to the traditional thought and also with the new political temper. A «moderate» direction, Fascist, Catholic, that was built in consonance with the pronatalism of the regime and in rough polemic with the presumed Anglo-Saxon eugenics aberration.

    Partiendo de la base de que no se puede describir la eugénica como un movimiento unitario, ya que se produjeron numerosas variantes ligadas al contexto geográfico y cultural, este artículo intenta demostrar la peculiaridad del caso italiano. Si ya en 1889 Giuseppe Sergi deseaba que la selección artificial llevase a cabo lo que debía de hacer la natural, evitando así el riesgo de la «degeneración », sólo ante la Primera Guerra Mundial parece crecer la alarma por la decadente calidad de la población, encontrando un eco cada vez más amplio. En 1919 nació la Sige (Società italiana de genetica ed eugenica bajo la impresión del difuso temor que la carnicería bélica había provocado. De ahí en adelante prende rápidamente una dirección «nazional» que se imbrica tanto con una tradición del pensamiento como con el nuevo temple político. Una dirección «moderada» fascista, católica, que se construyó en consonancia con el

  3. Arquitectura con discurso

    OpenAIRE

    Schaposnik, Viviana

    2001-01-01

    En particular a la Carrera Arquitectura le compete un doble rol social: uno general, "educar" desde la Universidad y otro, específico, el que le es propio: dar respuesta a las necesidades planteadas por la sociedad haciéndole su lugar: construyéndolo junto con ella. Aparece la figura del "alumno de arquitectura"' nuestro destinatario específico. El alumno de arquitectura, también deberá tomar conciencia, entender, que el "espacio" que él deberá dominar a través d...

  4. Accuracy of FibroScan for diagnosing liver fibrosis

    Directory of Open Access Journals (Sweden)

    Jian ZHANG

    2011-11-01

    Full Text Available Objective To evaluate the accuracy of transient elastometry(FibroScan for the detection of liver fibrosis.Methods A total of 323 patients diagnosed with chronic liver disease based on pathological examination in the 302 Hospital of the People’s Liberation Army from April to December of 2009 were involved in the current study.Among them,141 patients were subjected to liver biopsy.Their liver function,coagulant index,B-ultrasound and blood cell count were examined clinically.Four examinations related to liver fibrosis were done on some of the patients.Meanwhile,FibroScan was used for liver stiffness measurement(LSM of every patient.The correlation between liver stiffness and the serologic index and liver fibrosis degree was analyzed.The Receive Operating Characteristic(ROC curve was adopted to analyze the accuracy of FibroScan for diagnosing liver fibrosis.Results Each serologic index was significantly correlated with liver stiffness(P < 0.001,and liver stiffness was closely related to the stage of liver fibrosis(r=0.74,P < 0.001.The statistical results of the 141 patients who underwent pathologic examination show that the areas under the ROC curve were 0.97(0.94,1.00 for patients with portal fibrosis(F1,0.96(0.93,0.99 for patients with significant fibrosis(F2,0.99(0.98,1.00 for patients with severe fibrosis(F3,and 0.97(0.94,0.99 for patients with cirrhosis(F4.The cutoff values were 4.4KPa,6.8KPa,9.7KPa,and 10.0KPa,respectively.Conclusion FibroScan is valuable for the diagnosis of liver fibrosis.It can be used as the basis for follow-up and management of patients with chronic liver diseases.

  5. Simvastatin attenuates bleomycin-induced pulmonary fibrosis in mice

    Institute of Scientific and Technical Information of China (English)

    OU Xue-mei; FENG Yu-lin; WEN Fu-qiang; HUANG Xiang-yang; XIAO Jun; WANG Ke; WANG Tao

    2008-01-01

    Background Bleomycin-induced fibrosis is extensively used to model aspects of the pathogenesis of interstitial pulmonary fibrosis. This study aimed to determine the benefic effects and mechanisms of simvastatin on bleomycin-induced pulmonary fibrosis in mice.Methods Bleomycin-induced pulmonary fibrosis mice were administered with simvastatin in different doses for 28 days.We measured inflammatory response, fibrogenic cytokines and profibrogenic markers in both bleomycin-stimulated and control lungs, and correlated these parameters with pulmonary fibrosis.Results Simvastatin attenuated the histopathological change of bleomycin-induced pulmonary fibrosis and prevented the increase of lung hydroxyproline content and collagen (Ⅰand Ⅲ) mRNA expression induced by bleomycin. Moreover,simvastatin down-regulated the increased expression of transforming growth factor-β1 (TGF-β1) and connective tissue growth factor (CTGF) induced by bleomycin at both gene and protein levels. Simultaneously, the accumulation of neutrophils and lymphocytes and the increased production of tumor necrosis factor-(] (TNF-a) in bronchial alveolar lavage fluid were inhibited by simvastatin in early inflammatory phase after bleomycin infusion. The higher dose of simvastatin was associated with a more significant reduction in these inflammatory and fibrotic parameters. Furthermore,the inactivation of p38, RhoA and Smad2/3 signaling pathways was observed during simvastatin administration.Conclusions Simvastatin attenuated bleomycin-induced pulmonary fibrosis, as indicated by decreases in Ashcroft score and lung collagen accumulation. The inhibitory effect of simvastatin on the progression of pulmonary fibrosis may be demonstrated by reducing inflammatory response and production of TGF-βI and CTGF. These findings indicate that simvastatin may be used in the treatment of pulmonary fibrosis.

  6. Impaired Lymphocyte Profile in Schistosomiasis Patients with Periportal Fibrosis

    Directory of Open Access Journals (Sweden)

    Luciana Santos Cardoso

    2013-01-01

    Full Text Available The Th2 immune response in chronic schistosomiasis is associated with the development of periportal fibrosis. However, little is known about the phenotype and activation status of T cells in the process. Objective. To evaluate the profile of T cells in schistosomiasis patients with periportal fibrosis. Methods. It was a cross-sectional study, conducted in the village of Agua Preta, Bahia, Brazil, which included 37 subjects with periportal fibrosis determined by ultrasound. Peripheral blood mononuclear cells were obtained by the Ficcol-hypaque gradient and the frequency of T cells expressing the surface markers CD28, CD69, CD25, and CTLA-4 was determined by flow cytometry. Results. The frequency of CD4+CD28+ T lymphocytes was higher in individuals with moderate to severe fibrosis compared to patients with incipient fibrosis. We did not observe any significant difference in the frequency of CD4+ T cells expressing CD69 among groups of individuals. There was also no significant difference in the frequency of CD8+ T cells expressing CD28 or CD69 among the studied groups. Individuals with moderate to severe fibrosis presented a lower frequency of CD8+ T cells, CD4+CD25high T cells, and CD4+CTLA-4+ T cells when compared to patients without fibrosis or incipient fibrosis. The frequency of CD4+CD25low cells did not differ between groups. Conclusion. The high frequency of activated T cells coinciding with a low frequency of putative Treg cells may account for the development of periportal fibrosis in human schistosomiasis.

  7. [Effects of erdosteine on inflammation and fibrosis in rats with pulmonary fibrosis induced by bleomycin].

    Science.gov (United States)

    Erden, Ersin Sükrü; Kirkil, Gamze; Deveci, Figen; Ilhan, Nevin; Cobanoğlu, Bengü; Turgut, Teyfik; Muz, Mehmet Hamdi

    2008-01-01

    We aimed to investigate the levels of some chemokines, inflammatory cell counts in bronchoalveolar lavage (BAL) fluid, histopathological changes in lung tissue, to determine the effect of erdosteine on acute inflammatory changes and fibrosis in a rat fibrosis model induced by bleomycine (BLM). Forty-five Wistar male rats were taken into the study. On day 0, intratracheal saline to control group (group 1, n= 15), intratracheal BLM 7.5 U/kg to BLM (group 2, n= 15) and erdosteine group (group 3, n= 15) was administered. In group 3, oral erdosteine (10 mg/kg/day) was applied two days before BLM. On day 0, 14, and 29th five rats in each groups were sacrificed, BAL fluid was performed. Malonyldialdehyde (MDA), macrophage inflammatory protein (MIP)-1alpha, MIP-2 levels in BAL fluid, hydroxyproline levels in lung tissue were measured. Histopathological examination was performed. When BLM group compared to erdosteine group, the levels of MDA, MIP-1alpha, MIP-2, and neutrophil counts, the hydroxyproline (OH-P) level of lung tissue were decreased in erdosteine group on acute inflammatory phase (day 14) (perdosteine group than BLM group on day 29 (p= 0.01). We conclude that erdosteine may prevent the acute lung inflammation and fibrosis by suppressing the accumulation of neutrophils, inhibition of lipid peroxydation, chemokine production, and release.

  8. Acute Exacerbations of Idiopathic Pulmonary Fibrosis

    Science.gov (United States)

    Collard, Harold R.; Moore, Bethany B.; Flaherty, Kevin R.; Brown, Kevin K.; Kaner, Robert J.; King, Talmadge E.; Lasky, Joseph A.; Loyd, James E.; Noth, Imre; Olman, Mitchell A.; Raghu, Ganesh; Roman, Jesse; Ryu, Jay H.; Zisman, David A.; Hunninghake, Gary W.; Colby, Thomas V.; Egan, Jim J.; Hansell, David M.; Johkoh, Takeshi; Kaminski, Naftali; Kim, Dong Soon; Kondoh, Yasuhiro; Lynch, David A.; Müller-Quernheim, Joachim; Myers, Jeffrey L.; Nicholson, Andrew G.; Selman, Moisés; Toews, Galen B.; Wells, Athol U.; Martinez, Fernando J.

    2007-01-01

    The natural history of idiopathic pulmonary fibrosis (IPF) has been characterized as a steady, predictable decline in lung function over time. Recent evidence suggests that some patients may experience a more precipitous course, with periods of relative stability followed by acute deteriorations in respiratory status. Many of these acute deteriorations are of unknown etiology and have been termed acute exacerbations of IPF. This perspective is the result of an international effort to summarize the current state of knowledge regarding acute exacerbations of IPF. Acute exacerbations of IPF are defined as acute, clinically significant deteriorations of unidentifiable cause in patients with underlying IPF. Proposed diagnostic criteria include subjective worsening over 30 days or less, new bilateral radiographic opacities, and the absence of infection or another identifiable etiology. The potential pathobiological roles of infection, disordered cell biology, coagulation, and genetics are discussed, and future research directions are proposed. PMID:17585107

  9. Scoliosis in cystic fibrosis - an appraisal

    Energy Technology Data Exchange (ETDEWEB)

    Paling, M.R.; Spasovsky-Chernick, M.

    1982-03-01

    An unusually high prevalence (10%) of scoliosis is described in a series of 151 patients aged four years and older with cystic fibrosis. The scolioses were of the late onset (juvenile and adolescent) type, being typically thoracic with the curve convex to the right, although there was no significant preference for either sex. No direct relationship was found between the spinal curvature and the severity or distribution of the lung disease, although the worse scolioses tended to occur in patients with relatively severe pulmonary involvement. There was no evidence of metabolic bone disease as a predisposing cause. Some indication of a familial tendency towards scoliosis was apparent, and a genetic or constitutional basis is postulated with an unknown precipitating factor.

  10. Psychosocial problems in children with cystic fibrosis

    DEFF Research Database (Denmark)

    Bregnballe, V; Thastum, M; Schiøtz, P O

    2007-01-01

    AIM: To compare the well-being of children (7-14 years) with cystic fibrosis (CF) (n = 43) with the well-being of healthy controls (n = 1121). METHODS: The self-report questionnaire Beck Youth Inventories (BYI) was used to study depression, anxiety, anger, disruptive behaviour and self......-concept in children with CF. A measure of social desirability was included as well as body mass index (BMI) and percentage of predicted forced expiratory volume in one second (FEV(1)) as measures of health status. RESULTS: The children with CF did not differ from the norm group concerning depression, disruptive...... behaviour and self-concept. Young children with CF (7-10 years) and boys with CF scored significantly higher on anxiety. Girls with CF scored significantly lower on anger than controls. BMI was not associated with any of the BYI subscales. In patients aged 11-14 years, there was a significant correlation...

  11. Influenza vaccination in children with cystic fibrosis.

    Science.gov (United States)

    Patria, Maria Francesca; Longhi, Benedetta; Esposito, Susanna

    2013-04-01

    Cystic fibrosis (CF) is an inherited autosomal recessive disease characterized by progressive pulmonary damage and respiratory failure. It is known that bacterial infections play a critical role in the development of significant lung damage, whereas the role of respiratory viruses in CF pulmonary exacerbations and the relationship between viral infections and the progression of lung damage are uncertain. Health authorities throughout the world recommend influenza vaccination for CF patients. The aim of this review is to analyze the impact of seasonal and pandemic influenza on CF patients and data concerning influenza vaccination in order to assess the current situation and identify areas for future study. As data are limited, further well-constructed clinical studies of the effectiveness of influenza vaccination on the main clinical outcome measures of pulmonary function and nutritional status in patients with CF are required.

  12. Pseudomonas aeruginosa biofilms in cystic fibrosis

    DEFF Research Database (Denmark)

    Høiby, Niels; Ciofu, Oana; Bjarnsholt, Thomas

    2010-01-01

    The persistence of chronic Pseudomonas aeruginosa lung infections in cystic fibrosis (CF) patients is due to biofilm-growing mucoid (alginate-producing) strains. A biofilm is a structured consortium of bacteria, embedded in a self-produced polymer matrix consisting of polysaccharide, protein...... and DNA. In CF lungs, the polysaccharide alginate is the major part of the P. aeruginosa biofilm matrix. Bacterial biofilms cause chronic infections because they show increased tolerance to antibiotics and resist phagocytosis, as well as other components of the innate and the adaptive immune system....... As a consequence, a pronounced antibody response develops, leading to immune complex-mediated chronic inflammation, dominated by polymorphonuclear leukocytes. The chronic inflammation is the major cause of the lung tissue damage in CF. Biofilm growth in CF lungs is associated with an increased frequency...

  13. Diabetes mellitus in childhood cystic fibrosis.

    LENUS (Irish Health Repository)

    Rauf, F

    2012-02-03

    Since 1984, five patients in the cystic fibrosis (CF) clinic at Cork Regional Hospital have developed diabetes mellitus (DM) and were treated with Insulin. None had received systemic corticosteroids but two had high calorie naso-gastric feeding regimes. Two died from lung disease. A fifteen year old boy developed bilateral cataracts. In nine other paediatric CF clinics in the Republic of Ireland (total: 420 patients), three patients have DM, two receiving Insulin. Abnormal glucose tolerance is becoming more common in CF as patients survive longer. The possible role of corticosteroid treatment and intensive carbohydrate feeding regimes in development of glucose intolerance must be considered. DM in CF differs from the usual childhood DM. Regular screening and early Insulin supplementation may be beneficial.

  14. Entrevista con Geoffrey Lloyd.

    Directory of Open Access Journals (Sweden)

    Fernando Colina Pérez

    2008-01-01

    Full Text Available Helenista y también sinólogo de relieve internacional, Geoffrey E. R. Lloyd nació en Londres (1933, de padres galeses. Es un gran historiador de la ciencia y del pensamiento griegos. En 1940 fue evacuado de Londres con su madre. Sus estudios significativos comenzaron, tras algún rodeo, en el King’s College donde estudiaba su hermano. Éste sería, como su padre, médico, y él mismo dudó en estudiar esa profesión, que late en sus libros. Pero un profesor de clásicas como John Raven –que redactó, con Geoffrey Kirk, Los filósofos presocráticos–, le indujo a ocuparse de la filosofía antigua; y otro maestro, William Guthrie –a quien debemos la gran Historia de la filosofía griega–, le inició, además, en la medicina griega.

  15. con la cosecha mecanizada

    Directory of Open Access Journals (Sweden)

    Arturo Martínez Rodríguez

    2006-01-01

    Full Text Available Las investigaciones dirigidas a incrementar los indicadores de eficiencia y calidad durante la cosecha mecanizada del café, constituyen un tema de gran actualidad a nivel internacional. La determinación de las propiedades físico-mecánicas de los frutos y del sistema frutopedúnculo, es una etapa indispensable durante las investigaciones relacionadas con la cosecha mecanizada de este producto. En este trabajo se brindan los resultados sobre la determinación de un grupo de propiedades dimensionales, inerciales y elásticas del sistema fruto-pedúnculo de coffea arabica variedad Catuai en diferentes estadios de maduración, relacionadas con la cosecha mecanizada de este cultivo.Así mismo se determina el momento flector requerido para la ruptura de la unión fruto pedúnculo. Durante la investigación se emplearon técnicas de procesamiento de imágenes digitales, así como de extensometría eléctrica. Como resultado de la medición de las diferentes propiedades se apreciaron diferencias sustanciales en las características dimensionales, inerciales y elásticas de los frutos maduros y verdes, así como en el momento requerido para el desprendimiento de los frutos y en las formas en que se produce el desprendimiento.

  16. Entrevista con Patricia Ariza

    Directory of Open Access Journals (Sweden)

    Esperanza Londoño La Rotta

    2017-01-01

    Full Text Available Pensamiento, Palabra y Obra entrevista a una artista, feminista y activista política, quien como mujer y artista ha permitido pensar el arte más allá de un simple espectáculo. Toda una vida dedicada al teatro y a darle voz, a través de sus obras, a víctimas del conflicto colombiano, defensora de derechos humanos; además de hacer evidente en su vida y a través de la plataforma “Artistas por la paz”, las múltiples relaciones que se pueden establecer entre el arte, la construcción de paz y la resolución de conflictos. Hablamos en su casa, en medio del calor de la bienvenida con Patricia Ariza, directora del festival alternativo de teatro, de Mujeres en Escena y de la Corporación Colombiana de Teatro, entre otras muchas actividades que voluntariamente su espíritu libertario ha asumido. Esta entrevista se realizó antes del 2 de octubre, pero con la revisión de los acuerdos que propició el plebiscito ganado por una ínfima minoría por el no, sigue siendo vigente este planteamiento.

  17. Chemical properties of volcanic soil affected by seven-year rotations Propiedades químicas del suelo volcánico afectado por rotaciones de siete años

    Directory of Open Access Journals (Sweden)

    Juan Hirzel

    2011-06-01

    decisiones de manejo agrícola, con posibles consecuencias negativas en las propiedades físico-químicas del suelo y el ambiente. El desarrollo de información asociada a efectos en las propiedades físico-químicas del suelo en relación al uso de diferentes sistemas de rotaciones de largo plazo y sus manejos de fertilización, podrían contribuir a mejorar las decisiones de manejo agronómico en estos suelos. Se realizó un estudio que evaluó el efecto de seis rotaciones de cultivo que representan el manejo de fertilización utilizado por agricultores, que enfatiza la aplicación de N y P y eventualmente baja dosis de K, Ca y Mg, sobre las propiedades químicas de un suelo volcánico del centro-sur de Chile después de 7 años. Las propiedades químicas afectadas fueron pH, N inorgánico y K disponible, con una disminución general del pH relacionada con la fertilización usada, insuficiente en Ca, K y Mg. A su vez este suelo presentó una alta capacidad de adsorción de P (90,2-97,5%. Consecuentemente, las rotaciones de cultivo que incluyeron leguminosas forrajeras y cultivos con altos ingresos de nutrientes como remolacha generaron un efecto menos negativo en las propiedades químicas del suelo. Para prevenir efectos negativos sobre las propiedades químicas del suelo como lo indicado en este estudio, el manejo de fertilización en sistemas de rotaciones de cultivos debería considerar balances de entrada y salida de nutrientes.

  18. The pediatric NAFLD fibrosis index: a predictor of liver fibrosis in children with non-alcoholic fatty liver disease

    Directory of Open Access Journals (Sweden)

    Pietrobattista Andrea

    2009-05-01

    Full Text Available Abstract Background Liver fibrosis is a stage of non-alcoholic fatty liver disease (NAFLD which is responsible for liver-related morbidity and mortality in adults. Accordingly, the search for non-invasive markers of liver fibrosis has been the subject of intensive efforts in adults with NAFLD. Here, we developed a simple algorithm for the prediction of liver fibrosis in children with NAFLD followed at a tertiary care center. Methods The study included 136 male and 67 female children with NAFLD aged 3.3 to 18.0 years; 141 (69% of them had fibrosis at liver biopsy. On the basis of biological plausibility, readily availability and evidence from adult studies, we evaluated the following potential predictors of liver fibrosis at bootstrapped stepwise logistic regression: gender, age, body mass index, waist circumference, alanine transaminase, aspartate transaminase, gamma-glutamyl-transferase, albumin, prothrombin time, glucose, insulin, triglycerides and cholesterol. A final model was developed using bootstrapped logistic regression with bias-correction. We used this model to develop the 'pediatric NAFLD fibrosis index' (PNFI, which varies between 0 and 10. Results The final model was based on age, waist circumference and triglycerides and had a area under the receiver operating characteristic curve of 0.85 (95% bootstrapped confidence interval (CI with bias correction 0.80 to 0.90 for the prediction of liver fibrosis. A PNFI ≥ 9 (positive likelihood ratio = 28.6, 95% CI 4.0 to 201.0; positive predictive value = 98.5, 95% CI 91.8 to 100.0 could be used to rule in liver fibrosis without performing liver biopsy. Conclusion PNFI may help clinicians to predict liver fibrosis in children with NAFLD, but external validation is needed before it can be employed for this purpose.

  19. Entrevista con Juan Marichal.

    Directory of Open Access Journals (Sweden)

    - Consejo de Redacción

    1997-01-01

    Full Text Available Juan Marichal nació en Santa Cruz de Tenerife, en 1922, en el seno de una familia ligada al partido republicano insular. Muy joven, en 1935, se trasladó a Madrid, ciudad en la que vive el estallido de la guerra civil. En 1937, pasa a Valencia y luego a Barcelona; tras su exilio en 1938, prosigue sus estudios secundarios en un liceo de París, concluyéndolos en Casablanca. En 1941 emigra a México, formándose en la UNAM: fue alumno de los exiliados José Gaos y Joaquín Xirau así como del mexicano Edmundo O 'Gorman. Luego, becado en Princeton desde 1946, lo fue de América Castro, donde preparó una tesis sobre Feijoo. Apoyado en las vastas perspectivas de sus maestros, fue orientándose hada nuestra historia intelectual, desde el siglo XV hasta hoy. Su carrera profesional se ha desarrollado en los Estados Unidos (coincidiendo con Amado Alonso y con Ferrater Mora: ha sido profesor de estudios hispánicos en la Universidad de Harvard, desde 1948 hasta 1988, año en que se jubiló voluntariamente como numerario (aunque había permanecido en el Bryn Mawr College, entre 1953 y 1957. A este trabajo se suman, con todo, sus conferencias en América Latina y en España. Ha colaborado en las revistas más importantes, en este campo, de México, Nueva York, Puerto Rico, La Habana, Buenos Aires o París así como de las españolas, desde los sesenta. Juan Marichal -hoy, miembro de la Junta Directiva de los Amigos de la Residencia de Estudiantes, director del Boletín de la Institución Libre de Enseñanza y asociado al Instituto Universitario Ortega y Gasset-, reside en España desde otoño de 1989: se considera a sí mismo «voluntario en Madrid», como había dicho Alfonso Reyes en su estancia madrileña (1914-1924.

  20. Increased myocardial fibrosis and left ventricular dysfunction in Cushing's syndrome.

    NARCIS (Netherlands)

    Yiu, K.H.; Marsan, N.A.; Delgado, V.; Biermasz, N.R.; Holman, E.R.; Smit, J.W.A.; Feelders, R.A.; Bax, J.J.; Pereira, A.M.

    2012-01-01

    OBJECTIVE: Active Cushing's syndrome (CS) is associated with cardiomyopathy, characterized by myocardial structural, and ultrastructural abnormalities. The extent of myocardial fibrosis in patients with CS has not been previously evaluated. Therefore, the objective of this study was to assess myocar

  1. A patient with combined mediastinal, mesenteric and retroperitoneal fibrosis.

    Science.gov (United States)

    Graal, M B; Lustermans, F A

    1994-06-01

    We report a patient with combined mediastinal, mesenteric and retroperitoneal fibrosis who first presented with signs of a superior vena cava syndrome. She was successfully treated with corticosteroids. The aetiology, clinical picture, and possible therapy of idiopathic fibrosclerosis are discussed.

  2. [Non-invasive evaluation of liver fibrosis in hepatitis C].

    Science.gov (United States)

    de Lédinghen, V; Poynard, T; Wartelle, C; Rosenthal, E

    2008-03-01

    In 2007, the recommended FibroScan, FibroTest or liver biopsy for the initial diagnosis of fibrosis in patients with hepatitis C without co morbidities. These methods have to be interpreted according to the clinical situation, keeping in mind negative and positive false results. For FibroTest, hemolysis, Gilbert syndrome or acute inflammation can modify the result. Pre-analytical and analytical conditions of FibroTest have to be respected according to manufactory recommendations. For FibroScan, the numbers of measurements, the rate of successful measurements, and the interquartile range have to be correct. In case of suspicious results, FibroTest or FibroScan have to be done again. The liver biopsy, FibroTest, and FibroScan are less relevant for the distinction of two adjacent stages of fibrosis. However, their performances are excellent for the diagnosis of severe fibrosis or cirrhosis compared to moderate fibrosis.

  3. Contemporary use of elastography in liver fibrosis and portal hypertension

    DEFF Research Database (Denmark)

    Thiele, Maja; Kjærgaard, Maria; Thielsen, Peter

    2017-01-01

    significant portal hypertension, techniques and limitations. Four types of ultrasound elastography exist, but there is scarce evidence comparing the different techniques. The majority of experience concern transient elastography for diagnosing fibrosis and cirrhosis in patients with chronic viral hepatitis C...

  4. Attitudes of potential providers toward preconceptual cystic fibrosis carrier screening.

    NARCIS (Netherlands)

    Poppelaars, F.; Ader, H.J.; Cornel, M.C.; Henneman, L.; Hermens, R.P.M.G.; Wal, G. van der; Kate, L. ten

    2004-01-01

    To determine the attitudes of potential providers (general practitioners and Community Health Service workers) towards preconceptual cystic fibrosis (CF) carrier screening and to determine which factors are associated with a positive attitude. A survey was conducted among 200 general practitioners (

  5. Adeno-associated virus for cystic fibrosis gene therapy

    Directory of Open Access Journals (Sweden)

    S.V. Martini

    2011-11-01

    Full Text Available Gene therapy is an alternative treatment for genetic lung disease, especially monogenic disorders such as cystic fibrosis. Cystic fibrosis is a severe autosomal recessive disease affecting one in 2500 live births in the white population, caused by mutation of the cystic fibrosis transmembrane conductance regulator (CFTR. The disease is classically characterized by pancreatic enzyme insufficiency, an increased concentration of chloride in sweat, and varying severity of chronic obstructive lung disease. Currently, the greatest challenge for gene therapy is finding an ideal vector to deliver the transgene (CFTR to the affected organ (lung. Adeno-associated virus is the most promising viral vector system for the treatment of respiratory disease because it has natural tropism for airway epithelial cells and does not cause any human disease. This review focuses on the basic properties of adeno-associated virus and its use as a vector for cystic fibrosis gene therapy.

  6. Changes in Cystic Fibrosis Airway Microbiota at Pulmonary Exacerbation

    OpenAIRE

    Carmody, Lisa A.; Zhao, Jiangchao; Patrick D. Schloss; Petrosino, Joseph F.; Murray, Susan; Young, Vincent B.; Jun Z Li; LiPuma, John J.

    2013-01-01

    Rationale: In persons with cystic fibrosis (CF), repeated exacerbations of pulmonary symptoms are associated with a progressive decline in lung function. Changes in the airway microbiota around the time of exacerbations are not well understood.

  7. FACTORES SOCIO-AFECTIVOS RELACIONADOS CON LAS DIFICULTADES ESCOLARES EN NIÑAS Y NIÑOS “ESTRELLA” DEL PROGRAMA PSICOMOTRICIDAD E INTERVENCIÓN

    Directory of Open Access Journals (Sweden)

    Pilar Vargas Ramírez

    2012-07-01

    Full Text Available El presente estudio muestra los principales factores socio-afectivos relacionados con las dificultades escolares de un grupo de tres niñas y tres niños identificados como “estrella” por el Programa Psicomotricidad e Intervención. El mismo se desarrolló a través de una metodología mixta, en la que se aplicaron el Test de Figura Humana, el Dibujo Kinético de la Familia y una entrevista a la madre de cada estudiante. Los resultados revelaron la existencia de factores conflictivos familiares que afectan el estado emocional de las y los niños, generándoles sentimientos negativos consigo mismos que terminan afectado sus interacciones sociales y también su desempeño escolar.

  8. Musicalizarte con las Tics

    OpenAIRE

    Gamarra, Patricia Mabel; Velázquez, Helvecia Mercedes del Rocío; Verón, José María

    2015-01-01

    El presente trabajo hace referencia a un relato de experiencia didáctica desarrollada en el Instituto Superior de Música “Prof. Carmelo H. de Biasi” de Corrientes- Argentina, donde el área de interés de Tecnología en Educación se dirige hacia “Aplicación de las TIC en Educación/ Experiencias concretas de utilización de TIC en Educación”. En el diagnóstico realizado en la institución, se pudo observar la escasa transversalidad e interrelación con las distintas áreas curriculares como ser: l...

  9. Mirando con la cabeza

    OpenAIRE

    Corrales, Capi

    2006-01-01

    Las matemáticas y la pintura trabajan con ideas. La palabra idea viene del griego ειδω, que significa ver, mirar u observar, y de ειδοζ, que significa figura, forma, aspecto o visión. Detrás de una montaña concreta está la idea de montaña, un dibujo abstracto, unas líneas que permiten reconocer la montaña detrás de las rocas, los pinos o la nieve. La diferencia entre este árbol y árbol, entre un círculo que dibujamos en la pizarra y círculo: la diferencia entre la cosa y la idea de la cosa. E...

  10. Entrevista con Georges Duby.

    Directory of Open Access Journals (Sweden)

    - Consejo de Redacción

    1994-01-01

    Full Text Available Duby, heredero de dos grandes historiadores como Marc Bloch y Lucien Febvre, es uno de los más importantes medievalistas europeos. Fue, y sigue siendo, un motor de la importante reforma en la historia producida desde los sesenta. En sus primeros trabajos se acusa la recepción de las ideas económicas y geográficas de la mejor historiografía. Su riguroso estudio sobre la base material de la Edad Media, le permitirá luego irrumpir en la historia de las mentalidades, analizando, como decía Febvre, el utillaje mental (vocabulario, sintaxis, lugares comunes, cuadros lógicos, etc. del Medioevo. Así, el ejemplo de Mauss y LéviStrauss le anima a trabajar sobre el matrimonio, la sexualidad y ciertos sistemas del pensamiento: el primero, con su defensa de los hechos sociales totales, y el segundo, que buscaba las dimensiones simbólicas de lo social, le impulsan a trabajar sobre la ideología entendida no como mero reflejo de la situación material sino como «proyecto de acción sobre lo vivido». A su obra individual, atenta a los impulsos culturales más vivos, se suma su empuje decisivo en la realización de proyectos colectivos como la Historia de la vida privada o la Historia de las mujeres. Prácticamente, han sido traducidos todos sus libros al castellano, y han podido verse en España asimismo varios de sus programas televisivos (ha sido presidente de la SEPT, cadena de televisión cultural fundada en 1985. La amplitud de sus intereses intelectuales, transmitidos en su obra con un lenguaje a la vez muy claro y bellamente elaborado, se hace palpable también en este diálogo.

  11. Relating the disease mutation spectrum to the evolution of the cystic fibrosis transmembrane conductance regulator (CFTR.

    Directory of Open Access Journals (Sweden)

    Lavanya Rishishwar

    Full Text Available Cystic fibrosis (CF is the most common genetic disease among Caucasians, and accordingly the cystic fibrosis transmembrane conductance regulator (CFTR protein has perhaps the best characterized disease mutation spectrum with more than 1,500 causative mutations having been identified. In this study, we took advantage of that wealth of mutational information in an effort to relate site-specific evolutionary parameters with the propensity and severity of CFTR disease-causing mutations. To do this, we devised a scoring scheme for known CFTR disease-causing mutations based on the Grantham amino acid chemical difference matrix. CFTR site-specific evolutionary constraint values were then computed for seven different evolutionary metrics across a range of increasing evolutionary depths. The CFTR mutational scores and the various site-specific evolutionary constraint values were compared in order to evaluate which evolutionary measures best reflect the disease-causing mutation spectrum. Site-specific evolutionary constraint values from the widely used comparative method PolyPhen2 show the best correlation with the CFTR mutation score spectrum, whereas more straightforward conservation based measures (ConSurf and ScoreCons show the greatest ability to predict individual CFTR disease-causing mutations. While far greater than could be expected by chance alone, the fraction of the variability in mutation scores explained by the PolyPhen2 metric (3.6%, along with the best set of paired sensitivity (58% and specificity (60% values for the prediction of disease-causing residues, were marginal. These data indicate that evolutionary constraint levels are informative but far from determinant with respect to disease-causing mutations in CFTR. Nevertheless, this work shows that, when combined with additional lines of evidence, information on site-specific evolutionary conservation can and should be used to guide site-directed mutagenesis experiments by more narrowly

  12. Metformin attenuates lung fibrosis development via NOX4 suppression

    OpenAIRE

    Sato, Nahoko; Takasaka, Naoki; Yoshida, Masahiro; Tsubouchi, Kazuya; Minagawa,Shunsuke; Araya,Jun; Saito, Nayuta; Fujita, Yu; Kurita, Yusuke; KOBAYASHI, KENJI; ITO, Saburo; Hara, Hiromichi; Kadota, Tsukasa; Yanagisawa, Haruhiko; Hashimoto,Mitsuo

    2016-01-01

    Background Accumulation of profibrotic myofibroblasts in fibroblastic foci (FF) is a crucial process for development of fibrosis during idiopathic pulmonary fibrosis (IPF) pathogenesis, and transforming growth factor (TGF)-β plays a key regulatory role in myofibroblast differentiation. Reactive oxygen species (ROS) has been proposed to be involved in the mechanism for TGF-β-induced myofibroblast differentiation. Metformin is a biguanide antidiabetic medication and its pharmacological action i...

  13. Rosiglitazone attenuates pulmonary fibrosis and radiation-induced intestinal damage

    Energy Technology Data Exchange (ETDEWEB)

    Mangoni, M.; Gerini, C.; Sottili, M.; Cassani, S.; Stefania, G.; Biti, G. [Radiotherapy Unit, Clinical Physiopathology Department, University of Florence, Firenze (Italy); Castiglione, F. [Department of Human Pathology and Oncology, University of Florence, Firenze (Italy); Vanzi, E.; Bottoncetti, A.; Pupi, A. [Nuclear Medicine Unit, Clinical Physiopathology Department, University of Florence, Firenze (Italy)

    2011-10-15

    Full text of publication follows: Purpose.-The aim of the study was to evaluate radioprotective effect of rosiglitazone (RGZ) on a murine model of late pulmonary damage and of acute intestinal damage. Methods.- Lung fibrosis: C57 mice were treated with the radiomimetic agent bleomycin, with or without rosiglitazone (5 mg/kg/day). To obtain an independent qualitative and quantitative measure for lung fibrosis we used high resolution CT, performed twice a week during the entire observation period. Hounsfield Units (HU) of section slides from the upper and lower lung region were determined. On day 31 lungs were collected for histological analysis. Acute intestinal damage: mice underwent 12 Gy total body irradiation with or without rosiglitazone. Mice were sacrificed 24 or 72 h after total body irradiation and ileum and colon were collected. Results.- Lung fibrosis: after bleomycin treatment, mice showed typical CT features of lung fibrosis, including irregular septal thickening and patchy peripheral reticular abnormalities. Accordingly, HU lung density was dramatically increased. Rosiglitazone markedly attenuated the radiological signs of fibrosis and strongly inhibited HU lung density increase (60% inhibition at the end of the observation period). Histological analysis revealed that in bleomycin-treated mice, fibrosis involved 50-55% of pulmonary parenchyma and caused an alteration of the alveolar structures in 10% of parenchyma, while in rosiglitazone-treated mice, fibrosis involved only 20-25% of pulmonary parenchyma, without alterations of the alveolar structures. Acute intestinal damage: 24 h after 12 Gy of total body irradiation intestinal mucosa showed villi shortening, mucosal thickness and crypt necrotic changes. Rosiglitazone showed a histological improvement of tissue structure, with villi and crypts normalization and oedema reduction. Conclusion.- These results demonstrate that rosiglitazone displays a protective effect on pulmonary fibrosis and radiation

  14. Increased caffeine consumption is associated with reduced hepatic fibrosis

    Science.gov (United States)

    Modi, Apurva A; Feld, Jordan J; Park, Yoon; Kleiner, David E; Everhart, James E.; Liang, T. Jake; Hoofnagle, Jay H.

    2009-01-01

    Background Although coffee consumption has been associated with reduced frequency of liver disease, it is unclear whether the effect is from coffee or caffeine and whether there is an effect on hepatic fibrosis specifically. Aim To use a food-frequency instrument for dietary caffeine consumption to evaluate the relationship between caffeine intake and liver fibrosis. Methods Patients undergoing liver biopsy completed a detailed caffeine questionnaire on 3 occasions over a 6-month period. Caffeine intake was compared between patients with mild and advanced liver fibrosis (bridging fibrosis/cirrhosis). Logistic regression was used to evaluate the association between caffeine consumption and hepatic fibrosis. Results 177 patients (99 male, 104 Caucasian, 121 with chronic hepatitis C virus [HCV] infection) undergoing liver biopsy completed the caffeine questionnaire on up to three occasions. Results from repeated questionnaires were consistent. Daily caffeine consumption above the 75th percentile for the cohort (308 mg ~2.25 cups of coffee equivalents) was associated with reduced liver fibrosis (OR 0.33, 95% CI: 0.14-0.80, p=0.015) and the protective association persisted after controlling for age, sex, race, liver disease, body mass index and alcohol intake in all patients (OR 0.25, 95% CI: 0.09-0.67, p=0.006), as well as the subset with HCV infection (OR 0.19, 95% CI: 0.05-0.66, p=0.009). Despite a modest trend, consumption of caffeine from sources other than coffee or of decaffeinated coffee was not associated with reduced liver fibrosis. Conclusion A reliable tool for measurement of caffeine consumption demonstrated that caffeine consumption, particularly from regular coffee, above a threshold of approximately 2 coffee-cup equivalents per day, was associated with less severe hepatic fibrosis. PMID:20034049

  15. Vaccines for preventing infection with Pseudomonas aeruginosa in cystic fibrosis

    DEFF Research Database (Denmark)

    Johansen, Helle Krogh; Gøtzsche, Peter C

    2013-01-01

    Chronic pulmonary infection in cystic fibrosis results in progressive lung damage. Once colonisation of the lungs with Pseudomonas aeruginosa occurs, it is almost impossible to eradicate. Vaccines, aimed at reducing infection with Pseudomonas aeruginosa, have been developed.......Chronic pulmonary infection in cystic fibrosis results in progressive lung damage. Once colonisation of the lungs with Pseudomonas aeruginosa occurs, it is almost impossible to eradicate. Vaccines, aimed at reducing infection with Pseudomonas aeruginosa, have been developed....

  16. Increased PIVKA-II concentrations in patients with cystic fibrosis.

    OpenAIRE

    de Montalembert, M; Lenoir, G; Saint-Raymond, A.; Rey, J; Lefrère, J J

    1992-01-01

    Serum vitamin K concentrations and prothrombin induced by absence of vitamin K (PIVK-II) concentrations were assayed in 43 patients with cystic fibrosis. Twenty nine showed a normal PIVKA-II and vitamin K concentrations; 14 showed an increased PIVKA-II concentration, in one of whom serum vitamin K was decreased. Although their vitamin K concentrations were normal, some patients with cystic fibrosis still had an increased PIVKA-II. There was a significant correlation between PIVKA-II concentra...

  17. New insights into cystic fibrosis: molecular switches that regulate CFTR.

    Science.gov (United States)

    Guggino, William B; Stanton, Bruce A

    2006-06-01

    Cystic fibrosis transmembrane conductance regulator (CFTR), a Cl(-)-selective ion channel, is a prototypic member of the ATP-binding cassette transporter superfamily that is expressed in several organs. In these organs, CFTR assembles into large, dynamic macromolecular complexes that contain signalling molecules, kinases, transport proteins, PDZ-domain-containing proteins, myosin motors, Rab GTPases, and SNAREs. Understanding how these complexes regulate the intracellular trafficking and activity of CFTR provides a unique insight into the aetiology of cystic fibrosis and other diseases.

  18. Increased caffeine consumption is associated with reduced hepatic fibrosis.

    Science.gov (United States)

    Modi, Apurva A; Feld, Jordan J; Park, Yoon; Kleiner, David E; Everhart, James E; Liang, T Jake; Hoofnagle, Jay H

    2010-01-01

    Although coffee consumption has been associated with reduced frequency of liver disease, it is unclear whether the effect is from coffee or caffeine and whether there is an effect on hepatic fibrosis specifically. This study was undertaken to use a food-frequency instrument for dietary caffeine consumption to evaluate the relationship between caffeine intake and liver fibrosis. Patients undergoing liver biopsy completed a detailed caffeine questionnaire on three occasions over a 6-month period. Caffeine intake was compared between patients with mild and advanced liver fibrosis (bridging fibrosis/cirrhosis). Logistic regression was used to evaluate the association between caffeine consumption and hepatic fibrosis. One hundred seventy-seven patients (99 male, 104 white, 121 with chronic hepatitis C virus [HCV] infection) undergoing liver biopsy completed the caffeine questionnaire on up to three occasions. Results from repeated questionnaires were consistent. Daily caffeine consumption above the 75(th) percentile for the cohort (308 mg = approximately 2.25 cups of coffee equivalents) was associated with reduced liver fibrosis (odds ratio [OR], 0.33; 95% confidence interval [CI], 0.14-0.80; P = 0.015) and the protective association persisted after controlling for age, sex, race, liver disease, body mass index, and alcohol intake in all patients (OR, 0.25; 95% CI, 0.09-0.67; P = 0.006), as well as the subset with HCV infection (OR, 0.19; 95% CI, 0.05-0.66; P = 0.009). Despite a modest trend, consumption of caffeine from sources other than coffee or of decaffeinated coffee was not associated with reduced liver fibrosis. A reliable tool for measurement of caffeine consumption demonstrated that caffeine consumption, particularly from regular coffee, above a threshold of approximately 2 coffee-cup equivalents per day, was associated with less severe hepatic fibrosis.

  19. A practical new way to measure kidney fibrosis.

    Science.gov (United States)

    Peti-Peterdi, János

    2016-11-01

    Proper histological measurement of kidney fibrosis is essentially important in both clinical pathology and basic research using animal models of chronic kidney disease (CKD). However, standard histology techniques and their blind evaluation are cumbersome. Ranjit et al. applied an advanced optical microscopy technique for hassle-free, unbiased, and highly sensitive characterization of kidney fibrosis and tested it in a classic model of chronic kidney disease in mice. This commentary emphasizes the advantages and future promise of this new approach.

  20. Fucoidan partly prevents CCl4-induced liver fibrosis

    OpenAIRE

    Hayashi, Shinji; Itoh, Ayano; Isoda, Katsuhiro; Kondoh, Masuo; KAWASE, Masaya; Yagi, Kiyohito

    2008-01-01

    Fucoidan, a sulfated polysaccharide extracted from brown algae, has a wide range of biological activities, including anti-inflammatory, anti-viral, and anti-tumor activities. In the present study, we investigated the effects of fucoidan on CCl4-induced liver fibrosis. Administration of fucoidan reduced CCl4-induced acute and chronic liver failure. Hepatic fibrosis induced by CCl4 was also attenuated by injection of fucoidan. Damage to hepatocytes and activation of hepatic stellate cells are k...

  1. Astragalin inhibits autophagy-associated airway epithelial fibrosis

    OpenAIRE

    Cho, In-Hee; Choi, Yean-Jung; Gong, Ju-Hyun; Shin, Daekeun; Kang, Min-Kyung; Kang, Young-Hee

    2015-01-01

    Background Fibrotic remodeling of airway and lung parenchymal compartments is attributed to pulmonary dysfunction with an involvement of reactive oxygen species (ROS) in chronic lung diseases such as idiopathic pulmonary fibrosis and asthma. Methods The in vitro study elucidated inhibitory effects of astragalin, kaempferol-3-O-glucoside from leaves of persimmon and green tea seeds, on oxidative stress-induced airway fibrosis. The in vivo study explored the demoting effects of astragalin on ep...

  2. PRODUCCIÓN Y CALIDAD DE FRUTA EN CULTIVARES DE FRESA (Fragaria sp. AFECTADOS POR ESTRÉS SALINO YIELD AND FRUIT QUALITY OF SALT-STRESSED STRAWBERRY CULTIVARS (Fragaria sp.

    Directory of Open Access Journals (Sweden)

    Fánor Casierra-Posada

    2006-12-01

    Full Text Available Dado que el agua es muy buen solvente, las aguas que se usan para riego contienen algunas sales disueltas. La salinidad restringe la disponibilidad de este líquido para las plantas mediante la reducción del potencial hídrico en el suelo. La salinidad también tiene un impacto sobre la fisiología de la producción de las plantas. Es posible observar lesiones en plantas expuestas a niveles altos de salinidad. Este ensayo fue realizado para evaluar la relación entre la salinidad por NaCl, la producción y la calidad de fruta, en los cultivares de fresa ‘Chandler’, ‘Sweet Charlie’ y ‘Camarosa’ cultivados en invernadero, en materas con suelo salinizado. Se compararon cinco niveles de la sal (0, 20, 40, 60 y 80 mM de NaCl los cuales indujeron diferentes valores de conductividad eléctrica (EC (2,68; 6,08; 8,24; 9,98 y 12,62 dS.m-1, respectivamente. Los diversos niveles de EC se obtuvieron agregando el NaCl al suelo, progresivamente. La producción y la calidad de la fruta fueron afectadas negativamente, por los tratamientos probados. De igual manera, el contenido de azúcares en los frutos fue influido por los diferentes niveles de sal. Los contenidos de sólidos solubles totales y la acidez titulable en frutas se incrementaron con el aumento en la EC. Mientras que la acumulación de la materia seca en las raíces mostró un aumento proporcional con el nivel del NaCl en el substrato, los frutos presentaron una tendencia opuesta. Aunque los síntomas típicos del estrés tales como retraso del crecimiento y la producción reducida de fruta fueron observados en los cultivares de fresa evaluados, ‘Sweet Charlie’ fue menos sensible a la salinidad.Since water is a very good solvent, all irrigation waters contain some dissolved salts. Salinity restricts the availability of water to plants by lowering the total water potential in the soil. Salinity also has an impact on crop physiology and yield. Visible injury can occur at high salinity

  3. Pulmonary vaccination as a novel treatment for lung fibrosis.

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    Samuel L Collins

    Full Text Available Pulmonary fibrosis is an untreatable, uniformly fatal disease of unclear etiology that is the result of unremitting chronic inflammation. Recent studies have implicated bone marrow derived fibrocytes and M2 macrophages as playing key roles in propagating fibrosis. While the disease process is characterized by the accumulation of lymphocytes in the lung parenchyma and alveolar space, their role remains unclear. In this report we definitively demonstrate the ability of T cells to regulate lung inflammation leading to fibrosis. Specifically we demonstrate the ability of intranasal vaccinia vaccination to inhibit M2 macrophage generation and fibrocyte recruitment and hence the accumulation of collagen and death due to pulmonary failure. Mechanistically, we demonstrate the ability of lung Th1 cells to prevent fibrosis as vaccinia failed to prevent disease in Rag-/- mice or in mice in which the T cells lacked IFN-γ. Furthermore, vaccination 3 months prior to the initiation of fibrosis was able to mitigate the disease. Our findings clearly demonstrate the role of T cells in regulating pulmonary fibrosis as well as suggest that vaccinia-induced immunotherapy in the lung may prove to be a novel treatment approach to this otherwise fatal disease.

  4. The Interplay between Inflammation and Fibrosis in Kidney Transplantation

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    Irina B. Torres

    2014-01-01

    Full Text Available Serial surveillance renal allograft biopsies have shown that early subclinical inflammation constitutes a risk factor for the development of interstitial fibrosis. More recently, it has been observed that persistent inflammation is also associated with fibrosis progression and chronic humoral rejection, two histological conditions associated with poor allograft survival. Treatment of subclinical inflammation with steroid boluses prevents progression of fibrosis and preserves renal function in patients treated with a cyclosporine-based regimen. Subclinical inflammation has been reduced after the introduction of tacrolimus based regimens, and it has been shown that immunosuppressive schedules that are effective in preventing acute rejection and subclinical inflammation may prevent the progression of fibrosis and chronic humoral rejection. On the other hand, minimization protocols are associated with progression of fibrosis, and noncompliance with the immunosuppressive regime constitutes a major risk factor for chronic humoral rejection. Thus, adequate immunosuppressive treatment, avoiding minimization strategies and reinforcing educational actions to prevent noncompliance, is at present an effective approach to combat the progression of fibrosis.

  5. ¿Es la Fibromialgia un trastorno depresivo con síntomas somáticos o es un síndrome de sensibilización central con comorbilidad depresiva?

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    LA. Díaz Robredo

    Full Text Available Introducción: El objetivo de este estudio es evaluar los factores de personalidad, calidad de vida y hormonales de pacientes diagnosticados de Fibromialgia para comparar esta enfermedad con otras definidas como Síndromes de Sensibilización Central. Método: Para ello se emplearon el Personality Assessment Inventory, el Cuestionario de Impacto de Fibromialgia y muestras de cortisol salivar en un conjunto experimental de 48 personas. Resultados: Los resultados obtenidos señalan que los afectados por esta enfermedad poseen unas características de personalidad muy determinadas que no les asemejan ni con una situación de enfermedad mental psiquiátrica ni tampoco con niveles de normalidad absoluta, predominando puntuaciones altas en ansiedad, depresión y quejas somáticas, pero normales en otras como Manía, Paranoidismo o Esquizofrenia. Este patrón psicológico los hace más sensibles a los estímulos y mecanismos relacionados con el estrés. Los análisis exploratorios y confirmatorios realizados revelan que los patrones de personalidad formados por las variables psicológicas Ansiedad, Somatización y Depresión son las que más peso tienen en la calidad de vida de los pacientes y que en cambio la variable cortisol no tiene apenas influencia. Conclusiones: Las conclusiones extraídas por este estudio aconsejan buscar tratamientos terapéuticos que tengan en cuenta estas variables de personalidad señaladas, evitando su confusión con enfermedades psiquiátricas.

  6. Fibrosis is not just fibrosis - basement membrane modelling and collagen metabolism differs between hepatitis B- and C-induced injury

    DEFF Research Database (Denmark)

    Nielsen, M J; Karsdal, Morten A; Kazankov, K

    2016-01-01

    . AIM: To investigate whether differences in extracellular matrix (ECM) composition of the liver during fibrogenesis in two seemingly similar types of viral hepatitis could be reflected by differences in ECM turnover. METHODS: Utilising a cross-sectional design, we measured specific ECM protein...... fragments in plasma from 197 chronic hepatitis B (CHB) patients and 403 chronic hepatitis C (CHC) patients matched for inflammation grade and fibrosis stage. Markers of matrix metalloprotease degraded type I, III, IV and VI collagen (C1M, C3M, C4M, C6M) and type III and IV collagen formation (Pro-C3, P4NP7S...... and fibrosis only in CHC. Basement membrane collagen fragments P4NP7S and C4M were significantly higher in matched activity and fibrosis cohorts within CHB vs CHC. CONCLUSION: The main parameters to determine extracellular matrix biomarker levels are inflammation, fibrosis, and type of viral insult. Compared...

  7. US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis: executive summary.

    Science.gov (United States)

    Floto, R Andres; Olivier, Kenneth N; Saiman, Lisa; Daley, Charles L; Herrmann, Jean-Louis; Nick, Jerry A; Noone, Peadar G; Bilton, Diana; Corris, Paul; Gibson, Ronald L; Hempstead, Sarah E; Koetz, Karsten; Sabadosa, Kathryn A; Sermet-Gaudelus, Isabelle; Smyth, Alan R; van Ingen, Jakko; Wallace, Richard J; Winthrop, Kevin L; Marshall, Bruce C; Haworth, Charles S

    2016-01-01

    Non-tuberculous mycobacteria (NTM) are ubiquitous environmental organisms that can cause chronic pulmonary infection, particularly in individuals with pre-existing inflammatory lung disease, such as cystic fibrosis (CF). Pulmonary disease (PD) caused by NTM has emerged as a major threat to the health of individuals with CF, but remains difficult to diagnose and problematic to treat. In response to this challenge, the US Cystic Fibrosis Foundation (CFF) and the European Cystic Fibrosis Society (ECFS) convened a panel of 19 experts to develop consensus recommendations for the screening, investigation, diagnosis and management of NTM-PD in individuals with CF. PICO (population, intervention, comparison, outcome) methodology and systematic literature reviews were employed to inform draft recommendations, which were then modified to achieve consensus and subsequently circulated for public consultation within the USA and European CF communities. We have thus generated a series of pragmatic, evidence-based recommendations as an initial step in optimising management for this challenging condition.

  8. Post traumatic retroperitoneal fibrosis as a cause of low-back pain Fibrosis retroperitoneal postraumática como causa de dolor lumbar

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    A.M. Hidalgo-Ovejero

    2011-12-01

    Full Text Available We present the case of a male patient with post traumatic retroperitoneal fibrosis whose main clinical expression was low-back pain. Diagnosis was established using CAT-scan and MRI, which revealed a large mass of soft tissue that almost entirely enveloped the abdominal aorta. Treatment with 40 mg of prednisone every 24 hours was established. This dose was reduced gradually, and progressive remission of clinical signs and symptoms was achieved, with a significant improvement of subsequent imaging-test results. Treatment was continued for one year. Two and a half years later the patient remains symptom-free, with no recurrence of his condition.Se presenta el caso de un paciente varón afecto de una fibrosis retroperitoneal postraumática, a cuyo diagnóstico se llegó a partir de dolor lumbar como síntoma principal. El diagnóstico se efectuó en base a los estudios mediante CT y RM, los cuales demostraron una gran masa de tejido de partes blandas que rodeaban la aorta. El tratamiento consistió en dosis de prednisona que inicialmente se instauró a 40 mg cada 24 horas, y posteriormente se fue reduciendo de forma gradual hasta la remisión de los signos y síntomas, y consecuentemente de los estudios de imagen. El tratamiento con corticoides se mantuvo durante un año. Dos años y medio el paciente está libre de síntomas sin recidiva de su proceso.

  9. Intestinal Bicarbonate Secretion in Cystic Fibrosis Mice

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    Clarke LL

    2001-07-01

    Full Text Available Gene-targeted disruption of the cystic fibrosis transmembrane conductance regulator (CFTR in mice results in an intestinal disease phenotype that is remarkably similar to bowel disease in cystic fibrosis patients. In the intestinal segment downstream from the stomach (i.e., the duodenum, CFTR plays an important role in bicarbonate secretion that protects the epithelium from acidic gastric effluent. In this report, we examine the role of CFTR in cAMP-stimulated bicarbonate secretion in the murine duodenum and the mechanisms of acid-base transport that are revealed in CFTR knockout (CF mice. Ion substitution, channel blocker and pH stat studies comparing duodena from wild-type and CF mice indicate that CFTR mediates a HCO(3(- conductance across the apical membrane of the epithelium. In the presence of a favorable cell-to-lumen HCO(3(- gradient, the CFTR-mediated HCO(3(- current accounts for about 80% of stimulated HCO(3(- secretion. Exposure of the duodenal mucosa to acidic pH reveals another role of CFTR in facilitating HCO(3(- secretion via an electroneutral, 4,4'-diisothiocyanato-stilbene-2,2' disulfonic acid (DIDS sensitive Cl(-/HCO(3(- exchange process. In CF duodenum, other apical membrane acid-base transporters retain function, thereby affording limited control of transepithelial pH. Activity of a Cl(--dependent anion exchanger provides near-constant HCO(3(- secretion in CF intestine, but under basal conditions the magnitude of secretion is lessened by simultaneous activity of a Na(+/H(+ exchanger (NHE. During cAMP stimulation of CF duodenum, a small increase in net base secretion is measured but the change results from cAMP inhibition of NHE activity rather than increased HCO(3(- secretion. Interestingly, a small inward current that is sensitive to the anion channel blocker, 5-nitro-2(3-phenylpropyl amino-benzoate (NPPB, is also activated during cAMP stimulation of the CFTR-null intestine but the identity of the current is yet to be

  10. preescolares desnutridos con madres con obesidad y sin obesidad

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    Viridiana Vanessa Conzuelo-González

    2009-01-01

    Full Text Available El primer objetivo fue conocer cuántos menores de cinco años con diferentes grados de desnutrición tienen una madre con sobrepeso/obesidad/ en una comunidad indígena que vive en extrema pobreza y bajo condiciones de migración masculina internacional. El segundo fue comparar tres variables socionutricionales (ingreso familiar, educación de la madre y adecuación nutrimental de la dieta diaria entre estos hogares y los hogares con desnutrición infantil y madres sin obesidad. Se realizó un estudio transversal (2006-2007, en la comunidad mazahua de San Francisco Tepeolulco, Municipio de Temascalcingo; que incluyó a 85 hogares integrados por preescolares con desnutrición inscritos al programa Oportunidades. Se determinó el estado nutrición de los preescolares con indicadores antropométricos y se obtuvo el IMC de las madres de estos infantes. Se aplicó una encuesta socionutricional, incluida el recordatorio de 24 horas, y complementado con la observación participante (cualitativa. Se encontró que 83% de las madres mazahuas presentaron sobrepeso u obesidad. El estado de nutrición de los preescolares con madres con obesidad presentó un porcentaje mayor de desnutrición (76%. En la variable género, se encontró que 54% de los niños con madres con obesidad tenía baja talla. Al relacionar el nivel educativo de la madre, esta variable resultó ser estadísticamente significativa (p=0.015, donde el analfabetismo está más relacionado con la desnutrición infantil que tienen madres de bajo y/o peso normal. La elevada prevalencia de hogares conformados con preescolares con desnutrición y madres con obesidad, es un síntoma más de la pobreza en zonas indígenas en México, con bajo índice de desarrollo humano.

  11. Alteraciones neuropsicológicas en pacientes con VIH e historia previa de consumo de sustancias. Un estudio preliminar

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    Amalia García-Torres

    2015-01-01

    Full Text Available El virus de la inmunodeficiencia humana (VIH puede llegar a producir alteraciones cognitivas que interfieren de forma significativa en la vida diaria de las personas que lo padecen. Estos déficits pueden incrementarse cuando existe historia previa de consumo de sustancias psicoactivas. El objetivo de este trabajo es estudiar el perfil neuropsicológico de un grupo de pacientes con VIH e historia previa de consumo de drogas y compararlo con un grupo de pacientes ex drogodependientes seronegativos. Se administró una batería neuropsicológica a una muestra de 28 sujetos espa˜noles, 14 con VIH y 14 seronegativos, donde se evaluaron los 8 dominios cognitivos que suelen estar más afectados en el VIH: atención/memoria de trabajo, velocidad de procesamiento de la información, memoria/aprendizaje, abstracción/función ejecutiva, lenguaje/verbal y habilidades motoras. Los resultados mostraron peor ejecución del grupo con VIH en todos los dominios cognitivos. El menor rendimiento neuropsicológico del grupo con VIH podría deberse presumiblemente a la acción del virus. Independientemente de cuál sea la etiología del deterioro cognitivo, lo importante es detectar los déficits neuropsicológicos de la forma más precoz posible para mejorar la calidad de vida de los pacientes mediante la intervención neuropsicológica.

  12. Serum fibrosis markers can predict rapid fibrosis progression after liver transplantation for hepatitis C.

    Science.gov (United States)

    Pungpapong, Surakit; Nunes, David P; Krishna, Murli; Nakhleh, Raouf; Chambers, Kyle; Ghabril, Marwan; Dickson, Rolland C; Hughes, Christopher B; Steers, Jeffery; Nguyen, Justin H; Keaveny, Andrew P

    2008-09-01

    Although recurrent hepatitis C virus (HCV) after liver transplantation (LT) is universal, a minority of patients will develop cirrhosis within 5 years of surgery, which places them at risk for allograft failure. This retrospective study investigated whether 2 serum fibrosis markers, serum hyaluronic acid (HA) and YKL-40, could be used to predict rapid fibrosis progression (RFP) post-LT. These markers were compared with conventional laboratory tests, histological assessment, and hepatic stellate cell activity (HSCA), a key step in fibrogenesis, as assessed by immunohistochemical staining for alpha-smooth muscle actin. Serum and protocol liver biopsy samples were obtained from 46 LT recipients at means of 5 +/- 2 (biopsy 1) and 39 +/- 6 (biopsy 2) months post-LT, respectively. RFP was defined as an increase in the fibrosis score >or= 2 from biopsy 1 to biopsy 2 (a mean interval of 33 +/- 6 months). The ability of parameters at biopsy 1 to predict RFP was compared with the areas under receiver operating characteristic curves (AUROCs). Of the 46 subjects, 15 developed RFP. Serum HA and YKL-40 performed significantly better than conventional parameters and HSCA in predicting RFP post-LT for HCV at biopsy 1, with AUROCs of 0.89 and 0.92, respectively. The accuracy of serum HA >or= 90 microg/L and YKL-40 >or= 200 microg/L in predicting RFP at biopsy 1 was 80% and 96%, respectively. In conclusion, we found that elevated levels of serum HA and YKL-40 within the first 6 months after LT accurately predicted RFP. Larger studies evaluating the role of serum HA and YKL-40 in post-LT management are warranted.

  13. Aspectos clínicos endoscópicos, relacionados con la diverticulosis de colon. Las Tunas, enero 2011 – diciembre 2012

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    Liem Fonseca Chong

    2015-12-01

    Full Text Available Se realizó un estudio descriptivo de series de casos, en el Hospital “Dr. Ernesto Guevara de la Serna” de Las Tunas, con el objetivo de caracterizar los aspectos clínicos endoscópicos de la diverticulosis del colon, en el periodo comprendido desde enero 2011 a diciembre 2012. El universo quedó constituido por todos los pacientes a los que se les realizó colonoscopia, y la muestra por todos los pacientes con diagnóstico endoscópico de enfermedad diverticular. Para la obtención de los datos primarios se revisaron los expedientes clínicos de 101 pacientes con esta enfermedad, efectuando un cuestionario, en el cual se recogieron, analizaron y tabularon las variables, utilizando como medida de resumen el porcentaje. Se procesaron los datos y se interpretaron los resultados, concluyendo que en el grupo de pacientes estudiados predominaron las mujeres de más de 65 años, con dolor abdominal como síntoma principal y las hemorroides, como enfermedad que más se asoció a esta padecimiento, siendo el colon sigmoides el más afectado.

  14. Cystic Fibrosis Heterozygote Resistance to Cholera Toxin in the Cystic Fibrosis Mouse Model

    Science.gov (United States)

    Gabriel, Sherif E.; Brigman, Kristen N.; Koller, Beverly H.; Boucher, Richard C.; Stutts, M. Jackson

    1994-10-01

    The effect of the number of cystic fibrosis (CF) alleles on cholera toxin (CT)-induced intestinal secretion was examined in the CF mouse model. CF mice that expressed no CF transmembrane conductance regulator (CFTR) protein did not secrete fluid in response to CT. Heterozygotes expressed 50 percent of the normal amount of CFTR protein in the intestinal epithelium and secreted 50 percent of the normal fluid and chloride ion in response to CT. This correlation between CFTR protein and CT-induced chloride ion and fluid secretion suggests that CF heterozygotes might possess a selective advantage of resistance to cholera.

  15. Vigas preesforzadas con refuerzo externo

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    Juan José Ferrer Narvaez

    2011-01-01

    Full Text Available Es de gran interés para la ingeniería colombiana el efecto del refuerzo externo de elementos estructurales con elementos metálicos. La Unidad de Estructuras ha venido realizando en el transcurso de esta década estudios en elementos en concreto con refuerzos externos a flexión con resultados satisfactorios. El proyecto ''Vigas preesforzadas con refuerzo externo" sirve de base en el estudio del comportamiento de este tipo de elementos estructurales y la extrapolación de los resultados obtenidos en elementos de concreto reforzado a concreto preesforzado.

  16. Expression of integrin in hepatic fibrosis and intervention of resveratrol

    Institute of Scientific and Technical Information of China (English)

    Jianye WU; Chuanyong GUO; Jun LIU; Xuanfu XUAN

    2009-01-01

    The aim of this study was to explore the expression of integrin-β1 in different stages of hepatic fibrosis and intervention of resveratrol as well as the way by which integrin-β1 promoted hepatic fibrosis. Hepatic fibrosis models of male Sprague Dawley (SD) rats were created and intragastric administration of resveratrol was given in low (40 mg/kg), middle (120mg/kg) and high (200 mg/kg) dose groups. The expression of integrin-β1, transforming growth factor-β (TGF-β) and tissue inhibitor of metalloproteinase-1 (TIMP-1) in different stages of hepatic fibrosis was detected by using RT-PCR. The expression of hexadecenoic acid (HA) and precollagen Ⅲ (pc Ⅲ) was assayed by radioimmunoassay. The expression of integrin-β1, TGF-β and TIMP-1 was determined in each group. Liver function and pathological sections of each group in different stages of hepatic fibrosis was tested to judge the therapeutic efficacy of resveratrol at different doses. The expression of integrin-β1 in normal control group was low and steady and was not increased with the development of hepatic fibrosis, but it was increased in other groups. The expression levels of integrin-β1 in the model control group (0.878±0.03, P 0.05). The expression levels of integrin-β1 and TGF-β in middle dose group and high dose group were higher than other groups (P<0.01). The expression levels of integrin-β1 and TGF-β in model control group and low dose group were lower than the normal control group (P < 0.01). The expression levels of TIMP-1 in the model control and low dose groups were higher than the other groups (P < 0.01). The expression levels of TIMP-1 in the middle dose group and the high dose group were lower than the normal control group (P<0.01). The expression of integrin-β1 existed in all stages of hepatic fibrosis of SD rats, and it was increased with the development of hepatic fibrosis. The expression of TGF-β and TIMP-1 was consistent with that ofintegrin-β1 in different stages of

  17. Liver fibrosis in chronic viral hepatitis: An ultrasonographic study

    Institute of Scientific and Technical Information of China (English)

    Rong-Qin Zheng; Qing-Hui Wang; Ming-De Lu; Shi-Bin Xie; Jie Ren; Zhong-Zhen Su; Yin-Ke Cai; Ji-Lu Yao

    2003-01-01

    AIM: To select valuable ultrasonographic predictors for the evaluation of hepatic inflammation and fibrosis degree in chronic hepatitis, and to study the value of ultrasonography in the evaluation of liver fibrosis and compensated liver cirrhosis in comparison with serology and histology.METHODS: Forty-four ultrasonographic variables were analyzed and screened using color Doppler ultrasound system in 225 patients with chronic viral hepatitis and compensated liver cirrhosis. The valuable ultrasonographic predictors were selected on the basis of a comparison with histopathological findings. The value of ultrasonography and serology in the evaluation of liver fibrosis degree and the diagnosis of compensated liver cirrhosis was also studied and compared. Meanwhile, the influencing factors on ultrasonographic diagnosis of compensated liver cirrhosis were also analyzed.RESULTS: By statistical analysis, the maximum velocity of portal vein and the degree of gall-bladder wall smoothness were selected as the valuable predictors for the inflammation grade (G), while liver surface, hepatic parenchymal echo pattern, and the wall thickness of gall-bladder were selected as the valuable predictors for the fibrosis stage (S). Three S-related independent ultrasonographyic predictors and three routine serum fibrosis markers (HA, HPCIII and CIV) were used to discriminate variables for the comparison of ultrasonography with serology. The diagnostic accuracy of ultrasonography in moderate fibrosis was higher than that of serology (P<0.01), while there were no significant differences in the general diagnostic accuracy of fibrosis as well as between mild and severe fibrosis (P<0.05). There were no significant differences between ultrasonography and serology in the diagnosis of compensated liver cirrhosis.However, the diagnostic accuracy of ultrasonography was higher in inactive liver cirrhosis and lower in active cirrhosis than that of serology (both P<0.05). False positive and false

  18. Límites, reglas, comunicación en familia monoparental Con hijos adolescentes

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    Mildred Puello Scarpati

    2014-01-01

    Full Text Available En este artículo se hace una revisión fundamentada en investigaciones a nivel local, nacional e internacional sobre los cambios que en la contemporaneidad han afectado la estructura familiar y han contribuido a nuevos tipos de familia. En este caso, el interés ha sido analizar las conclusiones de los investigadores sobre las familias monoparentales con hijos adolescentes encontrando diferencias en los resultados. Algunos investigadores señalan que este tipo de familia coloca en riesgo a los hijos adolescentes en temas de drogadicción, delincuencia; otros expresan que no se puede generalizar a todas las familias monoparentales, por esto es importante estudiar la función que desempeña el padre o la madre que lidera la familia, para favorecer el desarrollo psicológico de los hijos. Por lo anterior, se pretende estudiar en las familias monoparentales que se encuentran en la etapa vital de la adolescencia, su dinámica, especialmente los límites, reglas y comunicación interaccional como aspectos que organizan la convivencia en pro del desarrollo de sus integrantes, en especial de los adolescentes que están en la tarea vital de forjar su identidad.

  19. Entrevista con Giovanni Levi.

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    - Consejo de Redacción

    1999-01-01

    Full Text Available Nacido en Milán en 1939, Giovanni Levi, el importante y original historiador italiano, es heredero de una tradición familiar de grandes defensores de la libertad tanto cultural como política. En su práctica investigadora late siempre, de hecho, la memoria de un momento tan crítico de la historia italiana como el final del fascismo. Por añadidura, sus antepasados son judíos del Piamonte. Esta experiencia vital y las luchas sociales de los sesenta han marcado su quehacer como historiador y como estudioso. Levi, reconocido en Italia y asimismo en Europa y América, fue profesor en la Universidad de Turín y luego en la de Viterbo; actualmente es catedrático de Historia económica en Venecia, ejerciendo como profesor visitante en Francia. Su trabajo, muy creativo, aborda aspectos de la historia de la familia, la demografía y la agricultura en los siglos XVII y XVIII, pero su crítica como intelectual comprometido con el dudoso presente desborda su estricto ámbito de estudio.

  20. Características de la avifauna en un fragmento de bosque húmedo premontano afectado por ruido vehicular (Features of the avifauna in a fragment of premontane moist forest affected by vehicular noise

    Directory of Open Access Journals (Sweden)

    Jessica Nathalia Sánchez-Guzmán

    2016-09-01

    Full Text Available La contaminación acústica producida por las carreteras representa uno de los factores que afecta en mayor medida la presencia, densidad y diversidad de la avifauna. En este estudio se evaluó la asociación entre el ruido vehicular, el número de especies de aves canoras y el número de detecciones en un fragmento de bosque ripario con alta influencia urbana. Se realizaron muestreos mensuales entre septiembre y noviembre de 2014 de 6:00 a 9:00 a. m. en un fragmento de bosque húmedo premontano dentro de la Universidad del Tolima. Las aves vocalizadoras se grabaron durante diez min/hora y se registró el valor máximo de ruido (dB obtenido en dos minutos. Los cantos se analizaron empleando Audacity® y la determinación de las especies se realizó consultando a expertos y confrontando con bases de datos. Se identificaron cantos de 43 especies principalmente de la familia Thraupidae y Tyrannidae. Se registraron diferencias significativas en el número de especies (F3,47 = 4,38; p = 0,025 y el número de detecciones (F3,47 = 4,51; p = 0,02 entre meses, y en el número de especies (F3,1 = 3,14; p = 0,05 y el número de detecciones (F3,1 = 6,03; p = 0,004 entre horas. No hubo diferencias significativas en las variables en relación a la intensidad del ruido vehicular, ni en los valores de ruido en los meses y horas muestreadas. Finalmente, se evidenció un aumento en el número de detecciones a medida que se genera la transición del período seco al lluvioso, por lo cual se recomienda realizar estudios de este tipo a escala temporal anual. (Abstract. The noise pollution caused by road is one of the factors that more affect the presence, density and diversity of birds. In this study was evaluated the association between vehicular noise and, the number of species of songbirds and detections on a fragment of riparian forest with high urban influence. Monthly samplings were made between september and november of 2014 from 06:00 to 09:00 h on a

  1. Lymphatics in lymphangioleiomyomatosis and idiopathic pulmonary fibrosis

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    Souheil El-Chemaly

    2012-09-01

    Full Text Available The primary function of the lymphatic system is absorbing and transporting macromolecules and immune cells to the general circulation, thereby regulating fluid, nutrient absorption and immune cell trafficking. Lymphangiogenesis plays an important role in tissue inflammation and tumour cell dissemination. Lymphatic involvement is seen in lymphangioleiomyomatosis (LAM and idiopathic pulmonary fibrosis (IPF. LAM, a disease primarily affecting females, involves the lung (cystic destruction, kidney (angiomyolipoma and axial lymphatics (adenopathy and lymphangioleiomyoma. LAM occurs sporadically or in association with tuberous sclerosis complex (TSC. Cystic lung destruction results from proliferation of LAM cells, which are abnormal smooth muscle-like cells with mutations in the TSC1 or TSC2 gene. Lymphatic abnormalities arise from infiltration of LAM cells into the lymphatic wall, leading to damage or obstruction of lymphatic vessels. Benign appearing LAM cells possess metastatic properties and are found in the blood and other body fluids. IPF is a progressive lung disease resulting from fibroblast proliferation and collagen deposition. Lymphangiogenesis is associated with pulmonary destruction and disease severity. A macrophage subset isolated from IPF bronchoalveolar lavage fluid (BALF express lymphatic endothelial cell markers in vitro, in contrast to the same macrophage subset from normal BALF. Herein, we review lymphatic involvement in LAM and IPF.

  2. Colonic Disorders in Adult Cystic Fibrosis

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    Hugh Chaun

    2001-01-01

    Full Text Available By 1996, the median survival of patients with cystic fibrosis (CF in North America had increased to 31 years. With the markedly improved life expectancy, many CF patients are now adults. There is an associated increased risk of certain colonic disorders, and the emergence of other previously unrecognized disorders, in adult CF patients. The distal intestinal obstruction syndrome (DIOS, which is more common in older patients, is a frequent cause of abdominal pain. Intussusception may complicate DIOS; other differential diagnoses include appendiceal disease, volvolus, Crohn's disease, fibrosing colonopathy and colonic carcinoma. The diagnosis of acute appendicitis, although uncommon in patients with CF, is often delayed, and appendiceal abscess is a frequent complication. The prevalence of Crohn's disease in CF has been shown to be 17 times that of the general population. Right-sided microscopic colitis is a recently recognized entity in CF of uncertain clinical significance. Fibrosing colonopathy has been confined mostly to children with CF, attributed to the use of high strength pancreatic enzyme supplements, but it has been reported in three adults. Nine cases of carcinoma of the large intestine have been reported worldwide, associated with an apparent excess risk of digestive tract cancers in CF. Despite high carrier rates of Clostridium difficile in patients with CF, pseudomembranous colitis is distinctly rare, but severe cases complicated by toxic megacolon have been reported. In these patients, watery diarrhea is often absent. Adult CF patients with refractory or unexplained intestinal symptoms merit thorough investigations.

  3. Vitamin K status in cystic fibrosis patients

    Directory of Open Access Journals (Sweden)

    Patrycja Krzyżanowska

    2010-12-01

    Full Text Available Vitamin K belongs to the family of fat-soluble vitamins and plays an important role in hemostasis, bone metabolism and may affect cerebral sphingolipid synthesis. It is a cofactor necessary for posttranslational γ-carboxylation of glutamyl residues in selected proteins such as the osteocalcin, and procoagulation factors II, VII, IX, X. Vitamin K deficient individuals appear to have more undercarboxylated proteins, which are functionally defective. The vitamin K deficiency has been frequently documented in patients with cystic fibrosis. The main possible causes of this deficiency include: fat malabsorption due to pancreatic exocrine insufficiency, cholestatic or  noncholestatic liver disease, reduced production of vitamin K by colonic flora related to chronic antibiotic treatments, bowel resections and increased mucous accumulation in the bowel. CF patients are more prone to osteopenia, caused by chronic vitamin K shortage, than to coagulopathy. Despite available evidence, which strongly suggests that all CF patients are at risk for developing vitamin K deficiency, its supplementation doses have not been established. Recent recommendations from Europe and the UK have suggested varied doses ranging from 0.3 mg/day to 10 mg/week. Further studies, both cross sectional and longitudinal interventional, are still required to determine routine and therapeutic supplementation doses.

  4. The cystic fibrosis lower airways microbial metagenome

    Science.gov (United States)

    Moran Losada, Patricia; Chouvarine, Philippe; Dorda, Marie; Hedtfeld, Silke; Mielke, Samira; Schulz, Angela; Wiehlmann, Lutz

    2016-01-01

    Chronic airway infections determine most morbidity in people with cystic fibrosis (CF). Herein, we present unbiased quantitative data about the frequency and abundance of DNA viruses, archaea, bacteria, moulds and fungi in CF lower airways. Induced sputa were collected on several occasions from children, adolescents and adults with CF. Deep sputum metagenome sequencing identified, on average, approximately 10 DNA viruses or fungi and several hundred bacterial taxa. The metagenome of a CF patient was typically found to be made up of an individual signature of multiple, lowly abundant species superimposed by few disease-associated pathogens, such as Pseudomonas aeruginosa and Staphylococcus aureus, as major components. The host-associated signatures ranged from inconspicuous polymicrobial communities in healthy subjects to low-complexity microbiomes dominated by the typical CF pathogens in patients with advanced lung disease. The DNA virus community in CF lungs mainly consisted of phages and occasionally of human pathogens, such as adeno- and herpesviruses. The S. aureus and P. aeruginosa populations were composed of one major and numerous minor clone types. The rare clones constitute a low copy genetic resource that could rapidly expand as a response to habitat alterations, such as antimicrobial chemotherapy or invasion of novel microbes. PMID:27730195

  5. Idiopathic pulmonary fibrosis: current and future directions.

    Science.gov (United States)

    Soo, E; Adamali, H; Edey, A J

    2017-01-21

    Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and is increasingly recognised. Prior to the advent of effective therapies, achieving an early diagnosis was arguably of little prognostic consequence given IPF was considered an untreatable and uniformly fatal disease. The advent of new drug treatments has given hope for the future and raised the profile of IPF. International management guidelines highlight the critical role of radiology as part of an interstitial lung disease multidisciplinary team approach in reaching an accurate and early diagnosis of IPF. The diagnostic criteria and levels of diagnostic confidence for the radio-pathological pattern associated with the clinical syndrome of IPF, usual interstitial pneumonia (UIP), appear seemingly straightforward; however, with increasing research and recognition of radiopathological interobserver variability, limitations of this classification model are becoming increasingly apparent. This review describes ancillary radiological features, comorbidities, and emerging new entities that potentially co-exist with IPF. Beyond diagnosis radiology is developing as a key prognostic tool to inform longitudinal patient evaluation. These diagnostic and prognostic clinical challenges and the future role of radiology in IPF are discussed.

  6. Infertility Management in Men with Cystic Fibrosis

    Institute of Scientific and Technical Information of China (English)

    Nikita Rawal; Rafit Gazvani; Roger Mountford

    2009-01-01

    Cystic fibrosis (CF) is a multisystem autosomal recessive disorder. CF has a reported incidence of 1 in 2 500 and a carrier frequency of 1 in 25. It is caused by mutations in a gene located on the long arm of chromosome 7. With almost 50% of patients with CF now in the adult population, and a very high interest in future parenting (78% of men wanting children), there is an increasing need to be proactive in terms of advice about reproductive health. Discussion on reproduction should start in early adolescence and be regularly updated by medical staff. The recent advances in reproductive techniques have allowed the development of sperm aspiration coupled with intra-cytoplasmic sperm injection (ICSI). Spermatozoa can be retrieved from either the epididymis or the testes. Genetic counselling is strongly recommended for both partners when congenital bilateral aplasia of vas deferens (CBA VD) is diagnosed as there is the inevitability of transmitting a mutated CFTR gene, and an increased risk of producing an affected child with serious long-term implications. Most treatment centres, with the introduction of ICSI, routinely screen all males with azoospermia caused by obstruction (other than that caused by vasectomy) or germ cell failure. Protocols usually involve screening for 28 relatively common CFTR mutations. Further advanced testing may be necessary up to a final detection level of approximately 98.5%. Before considering any assisted reproduction treatment, it is pertinent that CF patients receive medical and psychological counselling.

  7. Sirtuin 3 Deregulation Promotes Pulmonary Fibrosis.

    Science.gov (United States)

    Sosulski, Meredith L; Gongora, Rafael; Feghali-Bostwick, Carol; Lasky, Joseph A; Sanchez, Cecilia G

    2016-08-13

    Oxidative stress leads to alveolar epithelial cell injury and fibroblast-myofibroblast differentiation (FMD), key events in the pathobiology of pulmonary fibrosis (PF). Sirtuin 3 (SIRT3) is a mitochondrial protein deacetylase regulator of antioxidant response and mitochondrial homeostasis. Here, we demonstrate reduced SIRT3 expression in the lungs of old mice compared to young mice, as well as in two murine models of PF. The analysis of the pattern of SIRT3 expression in the lungs of patients with PF revealed low SIRT3 staining within the fibrotic regions. We also demonstrated, using murine models of PF and human lung fibroblasts, that reduced SIRT3 expression in response to transforming growth factor beta 1 (TGFβ1) promotes acetylation (inactivation) of major oxidative stress response regulators, such as SOD2 and isocitrate dehydrogenase 2. Reduction of SIRT3 in human lung fibroblasts promoted FMD. By contrast, overexpression of SIRT3 attenuated TGFβ1-mediated FMD and significantly reduced the levels of SMAD family member 3 (SMAD3). Resveratrol induced SIRT3 expression and ameliorated acetylation changes induced by TGFβ1. We demonstrated that SIRT3-deficient mice are more susceptible to PF compared to control mice, and concomitantly exhibit enhanced SMAD3 expression. Collectively, these data define a SIRT3/TGFβ1 interaction during aging that may play a significant role in the pathobiology of PF.

  8. Nephrogenic Systemic Fibrosis Risk and Liver Disease

    Directory of Open Access Journals (Sweden)

    Robert F. Hanna

    2014-01-01

    Full Text Available Objective. Evaluate the incidence of nephrogenic systemic fibrosis (NSF in patients with liver disease in the peritransplant period. Materials and Methods. This IRB approved study retrospectively reviewed patients requiring transplantation for cirrhosis, hepatocellular carcinoma (HCC, or both from 2003 to 2013. Records were reviewed identifying those having gadolinium enhanced MRI within 1 year of posttransplantation to document degree of liver disease, renal disease, and evidence for NSF. Results. Gadolinium-enhanced MRI was performed on 312 of 837 patients, including 23 with severe renal failure (GFR 30. Two of 23 patients with renal failure developed NSF compared to zero NSF cases in 289 patients with GFR > 30 (0/289; P<0.003. High dose gadodiamide was used in the two NSF cases. There was no increased incidence of NSF with severe liver disease (1/71 compared to nonsevere liver disease (1/241; P=0.412. Conclusion. Renal disease is a risk factor for NSF, but in our small sample our evidence suggests liver disease is not an additional risk factor, especially if a low-risk gadolinium agent is used. Noting that not all patients received high-risk gadolinium, a larger study focusing on patients receiving high-risk gadolinium is needed to further evaluate NSF risk in liver disease in the peritransplant period.

  9. Gadolinium and nephrogenic systemic fibrosis: an update.

    Science.gov (United States)

    Weller, Alex; Barber, Joy L; Olsen, Oystein E

    2014-10-01

    Nephrogenic systemic fibrosis (NSF) is a multisystem disease seen exclusively in patients with renal impairment. It can be severely debilitating and sometimes fatal. There is a strong association with gadolinium-based contrast agents used in magnetic resonance imaging (MRI). Risk factors include renal impairment and proinflammatory conditions, e.g. major surgery and vascular events. Although there is no single effective treatment for NSF, the most successful outcomes are seen following restoration of renal function, either following recovery from acute kidney injury or following renal transplantation. There have been ten biopsy-proved pediatric cases of NSF, with no convincing evidence that children have a significantly altered risk compared with the adult population. After implementation of guidelines restricting the use of gadolinium-based contrast agents in at-risk patients, there has been a sharp reduction in new cases and no new reports in children. Continued vigilance is recommended: screening for renal impairment, use of more stable gadolinium chelates, consideration of non-contrast-enhanced MRI or alternative imaging modalities where appropriate.

  10. Nephrogenic systemic fibrosis-Implications for nephrologists

    Energy Technology Data Exchange (ETDEWEB)

    Saab, Georges [University of Missouri, Columbia School of Medicine, Department of Internal Medicine, Division of Nephrology, MA436 Health Sciences Center, One Hospital Drive, Columbia, MO 65212 (United States)], E-mail: saabg@health.missouri.edu; Abu-Alfa, Ali [Yale University School of Medicine, Department of Internal Medicine, Section of Nephrology, New Haven, CT (United States)

    2008-05-15

    Nephrogenic systemic fibrosis (NSF) is a debilitating disorder seen in-patient with advanced chronic kidney disease (CKD). Recent evidence suggests a link between NSF and the administration of gadolinium-based contrast agents (Gd-CA). In addition, other risk factors have also been suggested to facilitate the development of NSF in this population after Gd-CA. These include metabolic acidosis, high-dose erythropoietin therapy, and the altered mineral metabolism of CKD. While it is possible that these factors may increase the risk of NSF after Gd-CA exposure, they may also simply reflect conditions that increase the risk of getting exposed to Gd-CA, particularly at high doses. Furthermore, given the risk of NSF in CKD, physicians must weigh the risks of NSF versus the risk of contrast-induced nephropathy (CIN) with iodinated agents in this population. In this review, we will provide a nephrologist's perspective on these issues and the nephrologist's role in the prevention of NSF.

  11. Dermatoglyphic Patterns in Cystic Fibrosis Children

    Science.gov (United States)

    Ezzati, Atefeh; Batoei, Fereshteh; Jafari, Seyed-Ali; Kiyani, Mohammad-Ali; Mahdavi-Shahri, Naser; Ahanchian, Hamid; Tehranian, Shahrzad; Kianifar, Hamid-Reza

    2014-01-01

    Objective: It is believed that fingerprints and palm patterns may represent genetically determined congenital abnormalities in Cystic Fibrosis (CF). The main idea of this paper was to determine differences of fingerprints and palm patterns in CF and normal children. Methods: Forty-six CF children (27 males, 19 females) and 341 (113 males, 228 females) healthy individuals were recruited for this study. Fingerprint patterns, Total ridge count (TRC) of each finger, a-b ridge count, and atd angles of all participants were recorded. Asymmetry of the right and left hand for each value was determined and dissimilarity in fingerprint patterns between homologous fingers was compared using Chi-square analysis, Mann-Whitney U test and Fisher's exact test. Findings: There were significant differences in the mean TRC of the right digit IV (P=0.009), left digit III (P=0.02), left digit IV (P=0.03), and left digit V (P=0.03). Furthermore, we found significant differences in right atd angel (P=0.001), left atd angel (P=0.002), right a-b ridge (P=0.007) and left a-b ridge (P=0.001). In contrast, we found no significant differences in atd angle asymmetry, a-b ridge count asymmetry and pattern dissimilarity score between both groups (P>0.05). Conclusion: Dermatoglyphic characteristics could be used as a supplementary diagnostic method in CF children. PMID:25793070

  12. The genus Prevotella in cystic fibrosis airways.

    Science.gov (United States)

    Field, Tyler R; Sibley, Christopher D; Parkins, Michael D; Rabin, Harvey R; Surette, Michael G

    2010-08-01

    Airway disease resulting from chronic bacterial colonization and consequential inflammation is the leading cause of morbidity and mortality in patients with Cystic Fibrosis (CF). Although traditionally considered to be due to only a few pathogens, recent re-examination of CF airway microbiology has revealed that polymicrobial communities that include many obligate anaerobes colonize lower airways. The purpose of this study was to examine Prevotella species in CF airways by quantitative culture and phenotypic characterization. Expectorated sputum was transferred to an anaerobic environment immediately following collection and examined by quantitative microbiology using a variety of culture media. Isolates were identified as facultative or obligate anaerobes and the later group was identified by 16S rRNA sequencing. Prevotella spp. represented the majority of isolates. Twelve different species of Prevotella were recovered from 16 patients with three species representing 65% of isolates. Multiple Prevotella species were often isolated from the same sputum sample. These isolates were biochemically characterized using Rapid ID 32A kits (BioMérieux), and for their ability to produce autoinducer-2 and beta-lactamases. Considerable phenotypic variability between isolates of the same species was observed. The quantity and composition of Prevotella species within a patients' airway microbiome varied over time. Our results suggest that the diversity and dynamics of Prevotella in CF airways may contribute to airway disease.

  13. Asthma and cystic fibrosis: A tangled web.

    LENUS (Irish Health Repository)

    Kent, Brian D

    2014-03-01

    Successfully diagnosing concomitant asthma in people with cystic fibrosis (CF) is a challenging proposition, and the utility of conventional diagnostic criteria of asthma in CF populations remains uncertain. Nonetheless, the accurate identification of individuals with CF and asthma allows appropriate tailoring of therapy, and should reduce the unnecessary use of asthma medication in broader CF cohorts. In this review, we discuss the diagnostic challenge posed by asthma in CF, both in terms of clinical evaluation, and of interpretation of pulmonary function testing and non-invasive markers of airway inflammation. We also examine how the role of cross-sectional thoracic imaging in CF and asthma can assist in the diagnosis of asthma in these patients. Finally, we critically appraise the evidence base behind the use of asthma medications in CF populations, with a particular focus on the use of inhaled corticosteroids and bronchodilators. As shall be discussed, the gaps in the current literature make further high-quality research in this field imperative. Pediatr Pulmonol. 2014; 49:205-213. © 2014 Wiley Periodicals, Inc.

  14. Transforming growth factor-β and fibrosis

    Institute of Scientific and Technical Information of China (English)

    Franck Verrecchia; Alain Mauviel

    2007-01-01

    Transforming growth factor-β (TGF-β), a prototype of multifunctional cytokine, is a key regulator of extracellular matrix (ECM) assembly and remodeling. Specifically, TGF-β isoforms have the ability to induce the expression of ECM proteins in mesenchymal cells, and to stimulate the production of protease inhibitors that prevent enzymatic breakdown of the ECM. Elevated TGF-β expression in affected organs, and subsequent deregulation of TGF-β functions, correlates with the abnormal connective tissue deposition observed during the onset of fibrotic diseases. During the last few years, tremendous progress has been made in the understanding of the molecular aspects of intracellular signaling downstream of the TGF-β receptors. In particular, Smad proteins, TGF-β receptor kinase substrates that translocate into the cell nucleus to act as transcription factors, have been studied extensively. The role of Smad3 in the transcriptional regulation of type I collagen gene expression and in the development of fibrosis, demonstrated both in vitro and in animal models with a targeted deletion of Smad3, is of critical importance because it may lead to novel therapeutic strategies against these diseases. This review focuses on the mechanisms underlying Smad modulation of fibrillar collagen expression and how it relates to fibrotic processes.

  15. Fibrosis progresiva del cuádriceps

    OpenAIRE

    Vergara Amador, Enrique; Largo González, Jorge Andrés

    2011-01-01

    Antecedentes.Material y métodos. Se encontraron siete niños con retracción progresiva unilateral de la rodilla. Seis tenían antecedente de la aplicación de una sola dosis de vacuna a los cuatro meses de edad con contractura entre la 4ª. y 8ª. semana posterior de la aplicación, y un niño la aplicación de vitamina K al nacer.Resultados. La resonancia magnética mostró alteraciones en el vasto intermedio sin patología intraarticular. El promedio de edad de la cirugía fue de 18 meses (16-20 meses)...

  16. Treatment and education reduce the severity of schistosomiasis periportal fibrosis

    Directory of Open Access Journals (Sweden)

    Paula Carolina Valenca Silva

    2013-07-01

    Full Text Available Introduction This study evaluates the factors associated with the development of severe periportal fibrosis in patients with Schistosoma mansoni. Methods A cross-sectional study was conducted from April to December 2012 involving 178 patients infected with S. mansoni who were treated in the Hospital das Clínicas of Pernambuco, Brazil. Information regarding risk factors was obtained using a questionnaire. Based on the patients' epidemiological history, clinical examination, and upper abdomen ultrasound evaluation, patients were divided into 2 groups: 137 with evidence of severe periportal fibrosis and 41 patients without fibrosis or with mild or moderate periportal fibrosis. Univariate and multivariate analyses were conducted using EpiInfo software version 3.5.5. Results Illiterate individuals (30.1% and patients who had more frequent contact with contaminated water in towns in the Zona da Mata of Pernambuco (33.2% were at greater risk for severe periportal fibrosis. Based on multivariate analysis, it was determined that an education level of up to 11 years of study and specific prior treatment for schistosomiasis were preventive factors for severe periportal fibrosis. Conclusions The prevailing sites of the severe forms of periportal fibrosis are still within the Zona da Mata of Pernambuco, although there has been an expansion to urban areas and the state coast. Specific treatment and an increased level of education were identified as protective factors, indicating the need for implementing social, sanitary, and health education interventions aimed at schistosomiasis to combat the risk factors for this major public health problem.

  17. TGF-β/Smad signaling in renal fibrosis

    Directory of Open Access Journals (Sweden)

    Xiao-Ming eMeng

    2015-03-01

    Full Text Available TGF-β (transforming growth factor-β is well identified as a central mediator in renal fibrosis. TGF-β initiates canonical and non-canonical pathways to exert multiple biological effects. Among them, Smad signaling is recognized as a major pathway of TGF- signaling in progressive renal fibrosis. During fibrogenesis, Smad3 is highly activated, which is associated with the down-regulation of an inhibitory Smad7 via an ubiquitin E3-ligases-dependent degradation mechanism. The equilibrium shift between Smad3 and Smad7 leads to accumulation and activation of myofibroblasts, overproduction of ECM (extracellular matrix, and reduction in ECM degradation in the diseased kidney. Therefore, overexpression of Smad7 has been shown to be a therapeutic agent for renal fibrosis in various models of kidney diseases. In contrast, another downstream effecter of TGF-β/Smad signaling pathway, Smad2, exerts its renal protective role by counter-regulating the Smad3. Furthermore, recent studies demonstrated that Smad3 mediates renal fibrosis by down-regulating miR-29 and miR-200 but up-regulating miR-21 and miR-192. Thus, overexpression of miR-29 and miR-200 or down-regulation of miR-21 and miR-192 is capable of attenuating Smad3-mediated renal fibrosis in various mouse models of chronic kidney diseases. Taken together, TGF-/Smad signaling plays an important role in renal fibrosis. Targeting TGF-β/Smad3 signaling may represent a specific and effective therapy for chronic kidney diseases associated with renal fibrosis.

  18. Gene Expression Patterns Associated With Histopathology in Toxic Liver Fibrosis.

    Science.gov (United States)

    Ippolito, Danielle L; AbdulHameed, Mohamed Diwan M; Tawa, Gregory J; Baer, Christine E; Permenter, Matthew G; McDyre, Bonna C; Dennis, William E; Boyle, Molly H; Hobbs, Cheryl A; Streicker, Michael A; Snowden, Bobbi S; Lewis, John A; Wallqvist, Anders; Stallings, Jonathan D

    2016-01-01

    Toxic industrial chemicals induce liver injury, which is difficult to diagnose without invasive procedures. Identifying indicators of end organ injury can complement exposure-based assays and improve predictive power. A multiplexed approach was used to experimentally evaluate a panel of 67 genes predicted to be associated with the fibrosis pathology by computationally mining DrugMatrix, a publicly available repository of gene microarray data. Five-day oral gavage studies in male Sprague Dawley rats dosed with varying concentrations of 3 fibrogenic compounds (allyl alcohol, carbon tetrachloride, and 4,4'-methylenedianiline) and 2 nonfibrogenic compounds (bromobenzene and dexamethasone) were conducted. Fibrosis was definitively diagnosed by histopathology. The 67-plex gene panel accurately diagnosed fibrosis in both microarray and multiplexed-gene expression assays. Necrosis and inflammatory infiltration were comorbid with fibrosis. ANOVA with contrasts identified that 51 of the 67 predicted genes were significantly associated with the fibrosis phenotype, with 24 of these specific to fibrosis alone. The protein product of the gene most strongly correlated with the fibrosis phenotype PCOLCE (Procollagen C-Endopeptidase Enhancer) was dose-dependently elevated in plasma from animals administered fibrogenic chemicals (P < .05). Semiquantitative global mass spectrometry analysis of the plasma identified an additional 5 protein products of the gene panel which increased after fibrogenic toxicant administration: fibronectin, ceruloplasmin, vitronectin, insulin-like growth factor binding protein, and α2-macroglobulin. These results support the data mining approach for identifying gene and/or protein panels for assessing liver injury and may suggest bridging biomarkers for molecular mediators linked to histopathology.

  19. Myofibroblasts and lung fibrosis induced by carbon nanotube exposure.

    Science.gov (United States)

    Dong, Jie; Ma, Qiang

    2016-11-04

    Carbon nanotubes (CNTs) are newly developed materials with unique properties and a range of industrial and commercial applications. A rapid expansion in the production of CNT materials may increase the risk of human exposure to CNTs. Studies in rodents have shown that certain forms of CNTs are potent fibrogenic inducers in the lungs to cause interstitial, bronchial, and pleural fibrosis characterized by the excessive deposition of collagen fibers and the scarring of involved tissues. The cellular and molecular basis underlying the fibrotic response to CNT exposure remains poorly understood. Myofibroblasts are a major type of effector cells in organ fibrosis that secrete copious amounts of extracellular matrix proteins and signaling molecules to drive fibrosis. Myofibroblasts also mediate the mechano-regulation of fibrotic matrix remodeling via contraction of their stress fibers. Recent studies reveal that exposure to CNTs induces the differentiation of myofibroblasts from fibroblasts in vitro and stimulates pulmonary accumulation and activation of myofibroblasts in vivo. Moreover, mechanistic analyses provide insights into the molecular underpinnings of myofibroblast differentiation and function induced by CNTs in the lungs.In view of the apparent fibrogenic activity of CNTs and the emerging role of myofibroblasts in the development of organ fibrosis, we discuss recent findings on CNT-induced lung fibrosis with emphasis on the role of myofibroblasts in the pathologic development of lung fibrosis. Particular attention is given to the formation and activation of myofibroblasts upon CNT exposure and the possible mechanisms by which CNTs regulate the function and dynamics of myofibroblasts in the lungs. It is evident that a fundamental understanding of the myofibroblast and its function and regulation in lung fibrosis will have a major influence on the future research on the pulmonary response to nano exposure, particle and fiber-induced pneumoconiosis, and other human

  20. REFORZAMIENTO DE UNA ESTRUCTURA INDUSTRIAL CON DOS TIPOS DE DISIPADORES DE ENERGÍA

    Directory of Open Access Journals (Sweden)

    R. Aguiar

    2016-12-01

    Full Text Available Se presenta en este artículo el reforzamiento de un silo de almacenamiento de harina afectado por el terremoto del 16 de abril de 2016 en Ecuador, que tuvo una magnitud de momento de 7.8. Inmediatamente después del terremoto se colocó fibra de carbono en los elementos afectados y la mampostería de bloques fue cambiada con paredes livianas, de tal forma de seguir laborando. Se realizó un estudio de peligrosidad sísmica y se determinó dos espectros de peligro sísmico uniforme para 975 y 2475 años de período de retorno. Para reforzar esta estructura se propone colocar diagonales de acero en el subsuelo, de tal manera que las cargas que se generan van directamente a la cimentación. En la planta baja se colocará disipadores de energía tipo Shear-Link y en la siguiente planta alta disipadores TADAS. La estructura reforzada, fue analizada para los espectros obtenidos en Manta del terremoto del 16 de abril y para los dos sismos de peligro sísmico uniforme indicados. This paper presents the reinforcement of a flour storage silo affected by the earthquake of April 16, 2016, in ECUADOR, which had a moment magnitude of 7.8. Immediately after the earthquake carbon fiber was placed in the affected elements and the block masonry was changed with light walls in such a way to continue working. A seismic hazard study was carried out and two uniform seismic hazard spectra were determined for 975 and 2475 years of return period. To reinforce this structure it is proposed to place steel diagonals in the subsoil, in such a way that the loads that are generated go directly to the foundation. On the ground floor will be placed Shear Link power dissipators and on the next floor high TADAS heat sinks. The reinforced structure was analyzed for the spectra obtained in Manta from the earthquake of April 16 and for the two earthquakes of uniform seismic hazard indicated.

  1. Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Nico Derichs

    2013-03-01

    Full Text Available Cystic fibrosis (CF is caused by genetic mutations that affect the cystic fibrosis transmembrane conductance regulator (CFTR protein. These mutations can impact the synthesis and transfer of the CFTR protein to the apical membrane of epithelial cells, as well as influencing the gating or conductance of chloride and bicarbonate ions through the channel. CFTR dysfunction results in ionic imbalance of epithelial secretions in several organ systems, such as the pancreas, gastrointestinal tract, liver and the respiratory system. Since discovery of the CFTR gene in 1989, research has focussed on targeting the underlying genetic defect to identify a disease-modifying treatment for CF. Investigated management strategies have included gene therapy and the development of small molecules that target CFTR mutations, known as CFTR modulators. CFTR modulators are typically identified by high-throughput screening assays, followed by preclinical validation using cell culture systems. Recently, one such modulator, the CFTR potentiator ivacaftor, was approved as an oral therapy for CF patients with the G551D-CFTR mutation. The clinical development of ivacaftor not only represents a breakthrough in CF care but also serves as a noteworthy example of personalised medicine.

  2. Entrevista con Alberto Tenenti.

    Directory of Open Access Journals (Sweden)

    - Consejo de Redacción

    1995-01-01

    Full Text Available Gran especialista en historia moderna, Alberto Tenenti nació en Viareggio, en 1924. Tras realizar estudios superiores en Italia, trabajó en el Centre National de la Recherche Scientifique varios años, asesorado por Lucien Febvre. Ha dirigido el Archivo del Estado de Brescia; y, más tarde, ha enseñado en París, desde una cátedra en la École Pratique des Hautes Études en Sciences Sociales (VI Sección, alIado de Braudel. Su Il senso della morte e l'amore della vita nel Rinascimento, de 1957, es una obra maestra sobre los orígenes de la sensibilidad moderna: sin olvidar el naciente vitalismo, estudia el desarrollo de dos motivos, el del ars moriendi, que tiene su evolución propia desde 1350 hasta su difusión impresa, y el de lo macabro, que refleja la crisis de conciencia del siglo XV y adquiere «unas dimensiones desconocidas y verdaderamente anormales». En este libro sobre un problema clave como la muerte, apela de modo notable a la iconografía: Tenenti ha recordado que la cultura tradicional, eclesiástica sobre todo, percibió un mayor peligro en la capacidad de reflexión autónoma y de crítica de los hombres de letras, que en las renovaciones radicales de los artistas. Numerosos trabajos de conjunto realizados por él han perseguido una historia global: Los fundamentos del mundo moderno; Florencia en la época de los Medicis; La formación del mundo moderno; El Renacimiento; el primero de ellos estaba firmado con un historiador de su misma generación, R. Romano, estudioso de las relaciones comerciales en la época moderna en Europa y en la América española. Tenenti ha publicado monografías (Venezia e i corsari, 1961, colecciones de artículos (Credence, ideologie, libertinismi tra medioevo ed eta moderna, 1978; Stato: un'idea, una logica. Dal comune italiano all'assolutismo francese, 1987 y editado a clásicos como Il libri della famiglia de L. B. Alberti, 1969. Es también especialista en temas económicos, como el del

  3. Puentes con vigas pretensadas

    Directory of Open Access Journals (Sweden)

    Editorial, Equipo

    1965-07-01

    Full Text Available This paper describes one of the three bridges which Hidrocivil, S. A., has built in Catalonia (northern Spain, over the river Ripoll. The other two bridges are very similar to this one, both in construction and design, and show only minor adjustments to the local topography. The contracting firm proposed several alterations in the prefabrication and constructional procedure, in relation to the initial project, and these changes were accepted. The main feature of these projects is the use of prestressed beams, built at the workshop in sections, and joined together by means of sixty 7 mm cables in each beam. As the shear forces are more acute at the joints, the end of each section has a kind of diaphragm, to provide a large contact area, and hence greater surface to transmit the shear forces. The methods of construction are also of interest. Briefly, they involve building the bridge piles, and use these to support a provisional structure with transversal movement. This provisional structure, in turn, served as platform for two bridge cranes, which lifted the girders to their final location. After the first span was completed, the deck was concreted and the auxiliary structure pushed forward to the next span, to repeat the same operations. This arrangement saved the use of provisional framework.En este trabajo se describe uno de los tres puentes que Hidrocivil, S. A., ha construido.—previo concurso— en la región catalana; concretamente, el que salva el río Ripoll. Los otros dos no han sido objeto de descripción general por ser muy similares, en lo que a ejecución y concepción se refiere, con la única variante que presentan las características topográficas locales. La empresa propuso ciertas variantes— que fueron aceptadas— en la prefabricación y métodos de construcción. El interés de estas obras se centra en el empleo de vigas pretensadas, prefabricadas en taller por trozos, y solidarizados en el mismo mediante las operaciones

  4. Entrevista con Bernard Vincent.

    Directory of Open Access Journals (Sweden)

    Fernando Colina Pérez

    2006-01-01

    Full Text Available Nacido en París (1941, Bernard Vincent es un historiador de renombre en Europa y América del Sur. Consiguió su agregación universitaria en 1966, y desde sus inicios se centró en la Historia Moderna, particularmente en grupos marginales de la España de los siglos XVI-XVII. Ha residido en nuestro país muchos años. Vincent fue miembro de la sección científica de la Casa de Velázquez inicialmente (1968-1971, algo más tarde director de estudios (1977-1978, y a continuación secretario general (1978-1982 de dicha institución. Asimismo ha encabezado el programa de cooperación francoespañol en ciencias sociales (1993-1996. Por otra parte, ha enseñado en la Universidad de París VII, en varias etapas de su vida; pero, sobre todo, ha pertenecido siempre a centros superiores de investigación: Centre National de la Recherche Scientifique (1976-1978, y a la École des Hautes Études en Sciences Sociales, en donde ha sido director de estudios desde 1988 –era doctor de Estado ya en 1986–, y luego responsable de la Sección de Historia, desde 1996 hasta hoy. Sus colaboraciones con las universidades y centros investigadores españoles han sido constantes hasta el presente. Por añadidura, ha sido miembro del Consejo Nacional de las Universidades en Francia, en lo relativo a la historia moderna y contemporánea (1987-1988, 1992-1995, y es desde hace años miembro de la madrileña Academia de la Historia.

  5. The association between indirect bilirubin levels and liver fibrosis due to chronic hepatitis C virus infection.

    Science.gov (United States)

    Cengiz, Mustafa; Yılmaz, Guldal; Ozenirler, Seren

    2014-08-01

    We proposed to evaluate the association between serum indirect bilirubin levels and liver fibrosis in patients with chronic hepatitis C (CHC) genotype 1b. Biopsy proven CHC genotype 1b patients' demographics, clinical and histopathological characteristics were evaluated. Logistic regression analysis was done to evaluate the clinical, laboratory and demographic features of the histologically proven liver fibrosis in CHC patients. A total of 112 biopsy proven CHC genotype 1b patients were enrolled into the study. Liver fibrosis scores were measured by using Ishak fibrosis scores and were divided into two groups; fibrosis scores ≤ 2 were categorized as mild fibrosis, 82 patients (73.2%), whereas fibrosis scores >2 were categorized as advanced fibrosis group, 30 patients (26.8%). Patients with advanced fibrosis had lower indirect bilirubin levels than the mild fibrosis group (0.28 ± 0.02 mg/dl vs. 0.44 ± 0.032 mg/dl, pbilirubin level was negatively correlated with advanced fibrosis scores (r=-0.416 and pbilirubin level was an independent predicting factor of advanced liver fibrosis (OR: 0.001, 95% CI: 0.0-0.005, pbilirubin levels and advanced liver fibrosis caused by CHC genotype 1b.

  6. The Immune Interplay between Thyroid Papillary Carcinoma and Hepatic Fibrosis.

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    Nidal Muhanna

    Full Text Available A high prevalence of thyroid papillary cancer was reported in hepatitis-C-virus (HCV positive patients. However, the mechanistic role of hepatic-fibrosis in thyroid malignancy progressions is still unclear.We aimed to study the immune-modulatory interactions between thyroid papillary carcinoma and hepatic-fibrosis.Hepatic-fibrosis was induced in nude-nu-male mice by intra-peritoneal administration of carbon-tetrachloride. To induce thyroid-tumor, a thyroid papillary carcinoma cell line (NPA was injected subcutaneously in the backs. Fibrotic profile was estimated by α-smooth-muscle-actin (αSMA expression in liver tissue extracts using western-blots and RT-PCR. Intra-hepatic NK cells were isolated and stained for NK activity (CD107a by flow cytometry. Liver histopathology (H&E staining, thyroid tumor mass and serum alanine aminotransferase (ALT, serum vascular endothelial growth factor (VEGF and free-T4 levels were also assessed.Ex-vivo: NPA cells were co-cultured with intra-hepatic NK cells isolated from fibrotic mice with/without the tumor were analyzed for CFSE-proliferations. Both tumor groups (with/without hepatic-fibrosis excreted higher serum free T4 levels. Hepatic-fibrosis increased tumor weight and size and serum free-T4 levels. In addition, tumor induction increased liver injury (both hepatic-fibrosis, necro-inflammation and serum ALT levels. In addition, tumor-bearing animals with hepatic-fibrosis had increased NK activity. NPA tumor-bearing animals increased fibrosis in spite of increased NK activity; probably due to a direct effect through increased serum free-T4 excretions. Serum VEGF levels were significantly increased in the fibrotic- bearing tumor groups compared to the non-fibrotic groups. In-vitro, NK cells from fibrotic tumor-bearing animals reduced proliferation of NPA cells. This decrease is attributed to increase NK cells activity in the fibrotic animals with the NPA tumors.Our results propose that NK cells although were

  7. Therapy of experimental NASH and fibrosis with galectin inhibitors.

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    Peter G Traber

    Full Text Available Non-alcoholic steatohepatitis (NASH and resultant liver fibrosis is a major health problem without effective therapy. Some data suggest that galectin-3 null mice are resistant to the development of NASH with fibrosis. We examined the ability of two complex carbohydrate drugs that bind galectin-3, GM-CT-01 and GR-MD-02, to treat NASH with fibrosis in a murine model. GR-MD-02 treatment resulted in marked improvement in liver histology with significant reduction in NASH activity and collagen deposition. Treatments seemed also to improve both glomerulopathy and interstitial fibrosis observed in kidneys. The improvement in liver histology was evident when animals were treated early in disease or after establishment of liver fibrosis. In all measures, GM-CT-01 had an intermediate effect between vehicle and GR-MD-02. Galectin-3 protein expression was increased in NASH with highest expression in macrophages surrounding lipid laden hepatocytes, and reduced following treatment with GR-MD-02, while the number of macrophages was unchanged. Treatment with GR-MD-02 also reduced the expression of pathological indicators including iNOS, an important TH1 inflammatory mediator, CD36, a scavenger receptor for lipoproteins on macrophages, and α-smooth muscle actin, a marker for activated stellate cells which are the primary collagen producing cells in liver fibrosis. We conclude that treatment with these galectin-3 targeting drugs improved histopathological findings of NASH and markedly reduced fibrosis in a murine model of NASH. While the mechanisms require further investigation, the treatment effect is associated with a reduction of galectin-3 expressed by activated macrophages which was associated with regression of NASH, including hepatocellular fat accumulation, hepatocyte ballooning, intra-portal and intra-lobular inflammatory infiltrate, and deposition of collagen. Similar effects were found with GM-CT-01, but with approximately four-fold lower potency than

  8. The development of the pediatric NAFLD fibrosis score (PNFS to predict the presence of advanced fibrosis in children with nonalcoholic fatty liver disease.

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    Naim Alkhouri

    Full Text Available BACKGROUND: Noninvasive hepatic fibrosis scores that predict the presence of advanced fibrosis have been developed and validated in adult patients with NAFLD. The aims of our study were to assess the utility of commonly used adult fibrosis scores in pediatric NAFLD and to develop a pediatric specific fibrosis score that can predict advanced fibrosis. METHODS: Consecutive children with biopsy-proven NAFLD were included. Fibrosis was determined by an experienced pathologist (F0-4. Advanced fibrosis was defined as fibrosis stage ≥ 3. The following adult fibrosis scores were calculated for each child: AST/ALT ratio, AST/platelet ratio index (APRI, NAFLD fibrosis score (NFS, and FIB-4 Index. Multivariable logistic regression analysis was performed to build a new pediatric model for predicting advanced fibrosis. RESULTS: Our cohort consisted of 242 children with a mean age of 12.4 ± 3.1 years and 63% were female. 36 (15% subjects had advanced fibrosis. APRI and FIB-4 were higher in patients with advanced fibrosis compared to those with fibrosis stage 0-2; however, AST/ALT ratio and NFS were not different between the two groups. We used our data to develop a new model to predict advanced fibrosis which included: ALT, alkaline phosphatase, platelet counts and GGT. The multivariable logistic regression model (z was defined as follows: z = 1.1+(0.34*sqrt(ALT+(0.002*alkaline phosphatase - (1.1*log(platelets - (0.02*GGT. This value was then converted into a probability distribution (p with a value between 0 to 100 by the following formula: p = 100 × exp(z/[1+exp(z]. The AUCROC for this model was 0.74 (95% CI: 0.66, 0.82. This was found to be significantly better than APRI, NAFLD Fibrosis Score and FIB-4 Index. CONCLUSION: Noninvasive hepatic fibrosis scores developed in adults had poor performance in diagnosing advanced fibrosis in children with NAFLD. We developed a new pediatric NAFLD fibrosis score with improved performance characteristics.

  9. Matrices con entradas enteras e inversa con entradas enteras

    OpenAIRE

    Mora, Walter

    2004-01-01

    Algunos artículos publicados en The American Mathematical Monthly discuten acerca de la construcción de matrices con entradas enteras, valores propios enteros y vectores propios con componentes enteras, en particular en [1] se hace una construcción que además permite construir, de manera sencilla, matrices con entradas enteras cuya inversa también tiene entradas enteras. Este artículo trata de estas últimas construcciones e incluye software en Java para generar y modificar ejemplos y para hac...

  10. Estrogen reduces CCL4- induced liver fibrosis in rats

    Institute of Scientific and Technical Information of China (English)

    Jun-Wang Xu; Jun Gong; Xin-Ming Chang; Jin-Yan Luo; Lei Dong; Zhi-Ming Hao; Ai Jia; Gui-Ping Xu

    2002-01-01

    AIM: Chronic liver diseases, such as fibrosis or cirrhosis,are more common in men than in women. This genderdifference may be related to the effects of sex hormones onthe liver. The aim of the present work was to investigatethe effects of estrogen on CCL4-induced fibrosis of the liverin rats.METHODS: Liver fibrosis was induced in male, female andovariectomized rats by CCL4 administration. All the groupswere treated with estradiol(1 mg/kg) twice weekly. Andtamoxifen wasgiven to male fibrosis model. At the end of 8weeks, all therats were killed to study serum indicators andthe livers.RESULTS: Estradiol treatment reduced aspartateaminotransferase(AST), alanine aminotransferase (ALT),hyaluronic acid(HA) and type IV collagen(CIV) in sera,suppressed hepatic collagen content, decreased the areas ofhepatic stellate cells (HSC) positive for α-smooth muscle actin(α-SMA), and lowered the synthesis of hepatic type I collagensignificantly in both sexes and ovariectomy fibrotic rats inducedby CCL4 administration. Whereas, tamoxifen had the oppositeeffect. The fibrotic response of the female liver to CCL4treatment was significantly weaker than that of male liver.CONCLUSION: Estradiol reduces CCL4-induced hepaticfibrosis in rats. The antifibrogenic role of estrogen in theliver may be one reason for the sex associated differencesin the progression from hepatic fibrosis to cirrhosis.

  11. Antifibrotic effect of heparin on liver fibrosis model in rats

    Institute of Scientific and Technical Information of China (English)

    Binita; Shah; Gaurang; Shah

    2012-01-01

    AIM: To evaluate the effect of chronic thrombin inhibition by heparin on experimentally induced chronic liver injury (liver fibrosis) in rats. METHODS: Chronic liver injury (liver fibrosis) was induced in Wistar rats by oral administration of carbon tetrachloride (CCl 4 ) for 7 wk, an animal model with persistent severe hepatic fibrosis. Intravenous administration of the thrombin antagonist (heparin) started 1 wk after the start of CCl 4 intoxication for 6 wk. After completion of treatment (7 wk), markers of hepatic dysfunction were measured and changes evaluated histopathologically. RESULTS: Higher serum glutamate oxaloacetate transaminase (SGOT), serum glutamate pyruvate transaminase (SGPT), alkaline phosphatase (ALP), total, direct and indirect bilirubin levels, as well as lower fibrinogen levels, were found in CCl 4 intoxicated rats. Heparin, silymarin and combination of drug (heparin and silymarin) treatment for 6 wk prevented a rise in SGOT, SGPT, ALP, total, direct and indirect bilirubin levels and improved fibrinogen levels. Deterioration in hepatic function determined by the fibrosis area was retarded, as evident from hepatic histopathology. Total protein levels were not changed in all groups.CONCLUSION: Heparin, a thrombin antagonist, preserved hepatic function and reduced severity of hepatic dysfunction/fibrogenesis. Combination of heparin and silymarin produced additional benefits on liver fibrosis.

  12. Extracellular Matrix Molecular Remodeling in Human Liver Fibrosis Evolution.

    Directory of Open Access Journals (Sweden)

    Andrea Baiocchini

    Full Text Available Chronic liver damage leads to pathological accumulation of ECM proteins (liver fibrosis. Comprehensive characterization of the human ECM molecular composition is essential for gaining insights into the mechanisms of liver disease. To date, studies of ECM remodeling in human liver diseases have been hampered by the unavailability of purified ECM. Here, we developed a decellularization method to purify ECM scaffolds from human liver tissues. Histological and electron microscopy analyses demonstrated that the ECM scaffolds, devoid of plasma and cellular components, preserved the three-dimensional ECM structure and zonal distribution of ECM components. This method has been then applied on 57 liver biopsies of HCV-infected patients at different stages of liver fibrosis according to METAVIR classification. Label-free nLC-MS/MS proteomics and computation biology were performed to analyze the ECM molecular composition in liver fibrosis progression, thus unveiling protein expression signatures specific for the HCV-related liver fibrotic stages. In particular, the ECM molecular composition of liver fibrosis was found to involve dynamic changes in matrix stiffness, flexibility and density related to the dysregulation of predominant collagen, elastic fibers and minor components with both structural and signaling properties. This study contributes to the understanding of the molecular bases underlying ECM remodeling in liver fibrosis and suggests new molecular targets for fibrolytic strategies.

  13. Gene delivery in peritoneal dialysis related peritoneal fibrosis research

    Institute of Scientific and Technical Information of China (English)

    LI Xie-jia; SUN Lin; XIAO Li; LIU Fu-you

    2012-01-01

    Objective To summarize the development of gene delivery vectors in peritoneal fibrosis research and discuss the feasibility and superiority of lentiviral vectors.Data sources The data in this article were collected from PubMed database with relevant English articles published from 1995 to 2011.Study selection Articles regarding the gene therapy in peritoneal fibrosis research using non-viral vectors,adenoviral vectors,ratroviral vectors,and lentiviral vectors were selected.Data were mainly extracted from 60 articles,which are listed in the reference section of this review.Results Non-viral vector-mediated gene delivery (including naked DNA for ex vivo,oligonucleotides,ultrasoundcontrast agent mediated naked gene delivery,etc.) and viral vector-mediated gene delivery (including adenovirus,helper-dependant adenovirus,and retrovirus vectors) have been successfully applied both in the mechanistic investigation and the potential prevention and treatment of peritoneal fibrosis.Conclusions Peritoneal fibrosis is a major complication of peritoneal dialysis (PD).Recently,the wide use of the gene delivery technique made it possible to access and further research peritoneal fibrosis.The use of lentiviral vector is expected to be widely used in PD research in the future due to its advantages in gene delivery.

  14. New Effective Treatment of Liver Fibrosis by Chinese Herbal Medicine

    Institute of Scientific and Technical Information of China (English)

    张国梁

    2002-01-01

    @@ Liver fibrosis is an abnormal proliferation pathologic process of intrahepatic fibrous connective tissue that occurs after liver cells have been necrotized and stimulated by inflammatory factors. It is called fibrosis when the pathological change is mild, and liver cirrhosis when the change becomes so severe as to reconstruct the liver lobuli to form pseudolobuli and nodule(1). Liver fibrosis is an important pathological characteristic of chronic hepatopathy and the chief intermediate link to further develop of liver cirrhosis. No ideal remedy for treatment of chronic hepatitic cirrhosis has been found so far. Although some drugs, such as colchicine and penicillamine, had been reported to have the effect of fibrosis inhibition, their clinical application is still limited for the rather severe toxic-side effects. Certain progress have been made from the clinical and experimental studies on anti-fibrosis treatment by traditional Chinese medicine (TCM) carried out widely in China in recent ten years. And here is a general review of the drugs used.

  15. Reversal of hepatic fibrosis: pathophysiological basis of antifibrotic therapies

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    Ismail MH

    2011-07-01

    Full Text Available Mona H Ismail1, Massimo Pinzani21Department of Internal Medicine, Division of Gastroenterology, King Fahad University Hospital, Al-Khobar, Saudi Arabia; 2Dipartimento di Medicina Interna Center for Research, High Education and Transfer, Università degli Studi di Firenze, Florence, ItalyAbstract: Chronic liver injuries of different etiologies eventually lead to fibrosis, a scarring process associated with increased and altered deposition of extracellular matrix in the liver. Progression of fibrosis has a major worldwide clinical impact due to the high number of patients affected by chronic liver disease which can lead to severe complications, expensive treatment, a possible need for liver transplantation, and death. Liver fibrogenesis is characterized by activation of hepatic stellate cells and other extracellular matrix producing cells. Liver fibrosis may regress following specific therapeutic interventions. Other than removing agents causing chronic liver damage, no antifibrotic drug is currently available in clinical practice. The extent of liver fibrosis is variable between individuals, even after controlling for exogenous factors. Thus, host genetic factors are considered to play an important role in the process of liver scarring. Until recently it was believed that this process was irreversible. However, emerging experimental and clinical evidence is starting to show that even cirrhosis in its early stages is potentially reversible.Keywords: liver fibrosis, cirrhosis, fibrogenesis, antifibrotic agents

  16. Chronic hepatitis C and fibrosis: evidences for possible estrogen benefits

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    Liana Codes

    2007-06-01

    Full Text Available The main injury caused by hepatitis C virus is the hepatic fibrosis, as a result of a chronic inflammatory process in the liver characterized by the deposit of components from the extracellular matrix. The fibrosis development leads to the modification of the hepatic architecture, of the hepatocellular function and to irregularities in the microcirculation. The tissue remodeling process observed in fibrosis has stellate cells, located at the space of Disse, as main acting agents. These cells, in response to a harmful stimulus, undergo phenotypic changes from non-proliferating cells to proliferating cells that express a- smooth-muscle actin (a-SMA, a process called as transdifferentiation. There are evidences that the oxidative stress is involved in the chronic liver disease and serves as bond between the injury and the hepatic fibrosis. A number of studies suggest that the estrogen, at physiological levels, presents an antifibrogenic action probably through an antioxidant effect, decreasing the levels of lipid peroxidation products in the liver and blood, thus inhibiting the myofibroblastic transformation of stellate cells and contributing for gender-associated differences in relation to the fibrosis development. The aim of this paper was to describe data from literature concerning the interaction between chronic hepatitis C and estrogens, pregnancy, use of oral contraceptives, menopause and hormone reposition therapy.

  17. Mechanisms and Biomarkers of Apoptosis in Liver Disease and Fibrosis

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    Jayashree Bagchi Chakraborty

    2012-01-01

    Full Text Available Liver fibrosis and cirrhosis are a major cause of morbidity and mortality worldwide. Development of the fibrotic scar is an outcome of chronic liver diseases of varying aetiologies including alcoholic liver disease (ALD nonalcoholic liver disease (NAFLD including non-alcoholic steatohepatitis (NASH viral hepatitis B and C (HBV, HCV. The critical step in the development of scar is activation of hepatic stellate cells (HSCs, which become the primary source of extracellular matrix. Aberrant apoptosis is a feature of chronic liver diseases and is associated with worsening stages of fibrosis. However, apoptosis is also the main mechanism promoting the resolution of fibrosis, and spontaneous or targeted apoptosis of HSC is associated with regression of fibrosis in animal models and patients with chronic liver disease. Given the importance of apoptosis in disease progression and resolution, there is much interest in precisely delineating the mechanisms involved and also developing biomarkers that accurately reflect the underlying pathogenesis. Here, we review the mechanisms driving apoptosis in development of liver disease and use of apoptosis -related biomarkers to aid in clinical diagnosis. Finally, we will also examine the recent literature regarding new insights into mechanisms involved in apoptosis of activated HSCs as possible method of fibrosis regression.

  18. SECs (Sinusoidal Endothelial Cells), Liver Microenvironment, and Fibrosis

    Science.gov (United States)

    Natarajan, Vaishaali; Harris, Edward N.

    2017-01-01

    Liver fibrosis is a wound-healing response to chronic liver injury such as alcoholic/nonalcoholic fatty liver disease and viral hepatitis with no FDA-approved treatments. Liver fibrosis results in a continual accumulation of extracellular matrix (ECM) proteins and paves the way for replacement of parenchyma with nonfunctional scar tissue. The fibrotic condition results in drastic changes in the local mechanical, chemical, and biological microenvironment of the tissue. Liver parenchyma is supported by an efficient network of vasculature lined by liver sinusoidal endothelial cells (LSECs). These nonparenchymal cells are highly specialized resident endothelial cell type with characteristic morphological and functional features. Alterations in LSECs phenotype including lack of LSEC fenestration, capillarization, and formation of an organized basement membrane have been shown to precede fibrosis and promote hepatic stellate cell activation. Here, we review the interplay of LSECs with the dynamic changes in the fibrotic liver microenvironment such as matrix rigidity, altered ECM protein profile, and cell-cell interactions to provide insight into the pivotal changes in LSEC physiology and the extent to which it mediates the progression of liver fibrosis. Establishing the molecular aspects of LSECs in the light of fibrotic microenvironment is valuable towards development of novel therapeutic and diagnostic targets of liver fibrosis. PMID:28293634

  19. TINF2 Gene Mutation in a Patient with Pulmonary Fibrosis

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    T. W. Hoffman

    2016-01-01

    Full Text Available Pulmonary fibrosis is a frequent manifestation of telomere syndromes. Telomere gene mutations are found in up to 25% and 3% of patients with familial disease and sporadic disease, respectively. The telomere gene TINF2 encodes an eponymous protein that is part of the shelterin complex, a complex involved in telomere protection and maintenance. A TINF2 gene mutation was recently reported in a family with pulmonary fibrosis. We identified a heterozygous Ser245Tyr mutation in the TINF2 gene of previously healthy female patient that presented with progressive cough due to pulmonary fibrosis as well as panhypogammaglobulinemia at age 52. Retrospective multidisciplinary evaluation classified her as a case of possible idiopathic pulmonary fibrosis. Telomere length-measurement indicated normal telomere length in the peripheral blood compartment. This is the first report of a TINF2 mutation in a patient with sporadic pulmonary fibrosis, which represents another association between TINF2 mutations and this disease. Furthermore, this case underlines the importance of telomere dysfunction and not telomere length alone in telomere syndromes and draws attention to hypogammaglobulinemia as a manifestation of telomere syndromes.

  20. Nephrogenic systemic fibrosis; Nephrogene systemische Fibrose

    Energy Technology Data Exchange (ETDEWEB)

    Samtleben, W. [Klinikum Grosshadern der Ludwig-Maximilians-Universitaet Muenchen, Schwerpunkt Nephrologie,Medizinische Klinik und Poliklinik I, Muenchen (Germany)

    2007-09-15

    A scleromyxedema-like disease was recognized in 1997. In 2000 this disorder was first published and termed nephrogenic fibrosing dermopathy because all patients had advanced renal failure. In 2006 it was discovered that the patients had a history of a preceding contrast-enhanced magnetic resonance imaging (MRI). All patients had acute or chronic severe renal insufficiency with a glomerular filtration rate (GFR) <30 ml/min per 1.73 m{sup 2}. So far a total of about 215 patients with this new skin disorder have been reported to international registries. The skin thickening has a typical histology and begins in the peripheral extremities and progresses proximally, including also the abdominal wall and the head in some patients. NSF involves not only the skin, but also the muscles and other organs (e.g., lungs, heart, eyes) in some patients. Therefore the term nephrogenic systemic fibrosis (NSF) was introduced. Skin fibrosis and sclerosis are usually progressive with disabling contractures of involved joints (knees, hands, feet). NSF may be lethal in up to 28% of patients. Spontaneous remissions are rare. No generally accepted treatment is available. So far, the pathogenesis is not well understood. One hypothesis supposes a role of gadolinium liberated from the contrast agents. As patients with acute or chronic advanced renal failure (GFR <30 ml/min per 1.73 m{sup 2}) including those with hepatorenal dysfunctions are at high risk to develop NSF after exposure to gadolinium-based contrast agents, contrast-enhanced MRI should be avoided in this group and alternative diagnostic procedures should be used whenever possible. (orig.) [German] Ein neues, Skleromyxoedem-aehnliches Krankheitsbild wurde erstmals 1997 beobachtet und im Jahre 2000 publiziert. Es trat nur bei Patienten mit eingeschraenkter Nierenfunktion (glomerulaere Filtrationsrate [GFR] <30 ml/min/1,73 m{sup 2}) auf, weshalb der Terminus nephrogene fibrosierende Sklerose gewaehlt wurde. Erst 2006 konnte ein

  1. Airway Clearance Devices for Cystic Fibrosis

    Science.gov (United States)

    2009-01-01

    Executive Summary Objective The purpose of this evidence-based analysis is to examine the safety and efficacy of airway clearance devices (ACDs) for cystic fibrosis and attempt to differentiate between devices, where possible, on grounds of clinical efficacy, quality of life, safety and/or patient preference. Background Cystic fibrosis (CF) is a common, inherited, life-limiting disease that affects multiple systems of the human body. Respiratory dysfunction is the primary complication and leading cause of death due to CF. CF causes abnormal mucus secretion in the airways, leading to airway obstruction and mucus plugging, which in turn can lead to bacterial infection and further mucous production. Over time, this almost cyclical process contributes to severe airway damage and loss of respiratory function. Removal of airway secretions, termed airway clearance, is thus an integral component of the management of CF. A variety of methods are available for airway clearance, some requiring mechanical devices, others physical manipulation of the body (e.g. physiotherapy). Conventional chest physiotherapy (CCPT), through the assistance of a caregiver, is the current standard of care for achieving airway clearance, particularly in young patients up to the ages of six or seven. CF patients are, however, living much longer now than in decades past. The median age of survival in Canada has risen to 37.0 years for the period of 1998-2002 (5-year window), up from 22.8 years for the 5-year window ending in 1977. The prevalence has also risen accordingly, last recorded as 3,453 in Canada in 2002, up from 1,630 in 1977. With individuals living longer, there is a greater need for independent methods of airway clearance. Airway Clearance Devices There are at least three classes of airway clearance devices: positive expiratory pressure devices (PEP), airway oscillating devices (AOD; either handheld or stationary) and high frequency chest compression (HFCC)/mechanical percussion (MP

  2. Cystic Fibrosis in the African Diaspora.

    Science.gov (United States)

    Stewart, Cheryl; Pepper, Michael S

    2017-01-01

    Identifying mutations that cause cystic fibrosis (CF) is important for making an early, unambiguous diagnosis, which, in turn, is linked to better health and a greater life expectancy. In patients of African descent, a molecular diagnosis is often confounded by the fact that the majority of investigations undertaken to identify causative mutations have been conducted on European populations, and CF-causing mutations tend to be population specific. We undertook a survey of published data with the aim of identifying causative CF mutations in patients of African descent in the Americas. We found that 1,584 chromosomes had been tested in only 6 countries, of which 876 alleles (55.3%) still remained unidentified. There were 59 mutations identified. Of those, 41 have been shown to cause CF, 17 have no associated functional studies, and one (R117H) is of varying clinical consequence. The most common mutations identified in the patients of African descent were: ΔF508 (29.4% identified in the United States, Colombia, Brazil, and Venezuela); 3120 + 1G>A (8.4% identified in Brazil, the United States, and Colombia); G85E (3.8% identified in Brazil); 1811 + 1.6kbA>G (3.7% identified in Colombia); and 1342 - 1G>C (3.1% identified in the United States). The majority of the mutations identified (81.4%) have been described in just one country. Our findings indicate that there is a need to fully characterize the spectrum of CF mutations in the diaspora to improve diagnostic accuracy for these patients and facilitate treatment.

  3. The enigma of nephrogenic systemic fibrosis.

    Science.gov (United States)

    Farlow, Jonathan T

    2007-01-01

    Nephrogenic systemic fibrosis is a severely physically disabling phenomenon experienced by a specific subset of patients with renal disease. Several of the 200 reported cases worldwide are currently treated within the author's facility. My colleagues and I have witnessed first-hand the life altering effects of NSF on our patients' physical, psychological, and social health, not to mention the unseen effects on family dynamics and changes in the interpretation and function of individual roles. Outside of the ESRD community, NSF is a largely unknown entity, likely due to the infrequency of incidence and relatively cohorted population. Despite the infrequency, we in the nephrology specialty must spread awareness of this condition and share our common knowledge and experiences to help those suffering from NSF to maintain their quality of life. In collaboration with the Centers for Disease Control (CDC), the Yale University International Center for NSF Research has established a website (www.icnfdr.org) for reporting of new cases of NSF/NFD. Until a causative agent is irrefutably identified, it is of the utmost importance that new cases be reported immediately. We expend a great deal of effort and gain valuable experience in helping our patients live their lives as richly as possible. If we as professional nurses share our knowledge and experience in managing the life-altering effects of NSF, our patients as a collective whole will benefit. It is, therefore, our responsibility to collaborate within and outside of the profession to reduce the number of new cases of NSF and help existing patients live their lives with the highest quality standard possible.

  4. Evidence for a Cystic Fibrosis Enteropathy.

    Directory of Open Access Journals (Sweden)

    Marlou P M Adriaanse

    Full Text Available Previous studies have suggested the existence of enteropathy in cystic fibrosis (CF, which may contribute to intestinal function impairment, a poor nutritional status and decline in lung function. This study evaluated enterocyte damage and intestinal inflammation in CF and studied its associations with nutritional status, CF-related morbidities such as impaired lung function and diabetes, and medication use.Sixty-eight CF patients and 107 controls were studied. Levels of serum intestinal-fatty acid binding protein (I-FABP, a specific marker for enterocyte damage, were retrospectively determined. The faecal intestinal inflammation marker calprotectin was prospectively studied. Nutritional status, lung function (FEV1, exocrine pancreatic insufficiency (EPI, CF-related diabetes (CFRD and use of proton pump inhibitors (PPI were obtained from the medical charts.Serum I-FABP levels were elevated in CF patients as compared with controls (p<0.001, and correlated negatively with FEV1 predicted value in children (r-.734, p<0.05. Faecal calprotectin level was elevated in 93% of CF patients, and correlated negatively with FEV1 predicted value in adults (r-.484, p<0.05. No correlation was found between calprotectin levels in faeces and sputum. Faecal calprotectin level was significantly associated with the presence of CFRD, EPI, and PPI use.This study demonstrated enterocyte damage and intestinal inflammation in CF patients, and provides evidence for an inverse correlation between enteropathy and lung function. The presented associations of enteropathy with important CF-related morbidities further emphasize the clinical relevance.

  5. Practical management of Idiopathic Pulmonary Fibrosis.

    Science.gov (United States)

    Kishaba, Tomoo

    2015-07-22

    Idiopathic Pulmonary Fibrosis (IPF) is relentless progressive interstitial lung disease (ILD) of unknown etiology. Main pathogenesis is aberrant recovery of epithelial injury and collagen deposition. Majority of IPF patients have been elderly men with smokers. However, there are important differential diagnosis such as fibrotic non-specific interstitial pneumonia (NSIP), Connective Tissue Disease (CTD) associated ILD, chronic hypersensitivity pneumonia (CHP). Clinical point of view, non-productive cough and progressive exertional dyspnea are main symptoms. In addition, scalene muscle hypertrophy, fine crackles and finger clubbing are key findings. Serum marker such as lactate deydrogenase (LDH), Krebs von den Lungeng-6 (KL-6) are sensitive for ILD detection and activity. Pulmonary function test and 6 minute walk test (6MWT) are quite meaningful physiological examination. Serial change of forced vital capacity 6MWT distance predict mortality of IPF. International IPF guideline published recently and highlighted on the importance of high resolution computed tomography (HRCT) findings. Key findings of IPF are honeycombing, traction bronchiectasis and subpleural reticular opacity. IPF is chronic progressive disease. Therefore, tracing disease behavior is crucial and unifying clinical, physiological, imaging information over time provide useful information for physicians.In management, many candidate agent failed to have positive result. Pirfenidone which is anti-fibrotic agent showed to slow the decline of vital capacity and prevent of acute exacerbation. Molecular agent such as nintedanib is promising agent for prevention of progression of IPF. In this review, we review the clinical information of IPF and IPF guideline. Lastly, we show the clinical algorithm of this devastated disease.

  6. con el aborto provocado

    Directory of Open Access Journals (Sweden)

    José Luis Redondo Calderón

    2008-01-01

    Full Text Available Las vacunas de células diploides humanas (WI-38, MRC-5 tienen un origen éticamente objetable, dado que dichas células proceden de abortos provocados. Entre ellas destacan vacunas empleadas contra rubéola, sarampión, parotiditis, rabia, poliomielitis, viruela, hepatitis A, varicela y herpes zóster. Actualmente se encuentran en desarrollo otras vacunas cultivadas en células (293, PER.C6 transformadas mediante virus, procedentes de abortos. Entre ellas hay vacunas contra la gripe, virus respiratorio sincitial, parainfl uenza, HIV, virus del Nilo Occidental, virus Ébola, Marburg y Lassa, hepatitis B y C, glosopeda, encefalitis japonesa, dengue, tuberculosis, carbunco, peste, tétanos y paludismo. También con igual origen se trabaja en la elaboración de anticuerpos monoclonales y otras proteínas, terapia génica y genómica. Existe la tecnología necesaria para producir todo lo descrito sin recurrir a abortos provocados. Debe indicarse en los prospectos de vacunas y otros productos el origen de las células empleadas. Debe facilitarse el acceso a las vacunas existentes no cultivadas en células procedentes de abortos provocados. Debe potenciarse la investigación de opciones en aquellos casos en los que no exista una vacuna no originada en células procedentes de abortos provocados. Debe potenciarse la elaboración de anticuerpos monoclonales y de otras proteínas, así como la terapia génica y la genómica sin recurrir a células procedentes de abortos provocados. No sería consecuente rechazar productos obtenidos a partir de células troncales embrionarias y aceptar los originados en células procedentes de abortos provocados. Se debe evitar que la biotecnología basada en el aborto provocado invada todos los terrenos de la medicina.

  7. Celecoxib treatment reduces peritoneal fibrosis and angiogenesis and prevents ultrafiltration failure in experimental peritoneal dialysis

    NARCIS (Netherlands)

    Fabbrini, Paolo; Schilte, Margot N.; Zareie, Mammad; ter Wee, Piet M.; Keuning, Eelco D.; Beelen, Robert H. J.; van den Born, Jaap

    2009-01-01

    Background. Daily peritoneal exposure to peritoneal dialysis fluid (PDF) induces severe morphological alterations including fibrosis and angiogenesis that lead to a loss of peritoneal ultrafiltration (UF) capacity. Since cyclooxygenase (COX)-2 is involved in fibrosis and angiogenesis, we investigate

  8. Comparing new treatments for idiopathic pulmonary fibrosis--a network meta-analysis.

    LENUS (Irish Health Repository)

    Loveman, Emma

    2015-01-01

    The treatment landscape for idiopathic pulmonary fibrosis, a devastating lung disease, is changing. To investigate the effectiveness of treatments for idiopathic pulmonary fibrosis we undertook a systematic review, network meta-analysis and indirect comparison.

  9. DeltaF508 heterozygosity in cystic fibrosis and susceptibility to asthma

    DEFF Research Database (Denmark)

    Dahl, Morten; Tybjaerg-Hansen, A; Lange, P;

    1998-01-01

    Cystic fibrosis is a recessive disorder mainly characterised by lung disease. We tested the hypothesis that individuals heterozygous for the common cystic fibrosis deltaF508 mutation are at risk of obstructive pulmonary disease....

  10. Signaling in fibrosis: TGF-β, WNT, and YAP/TAZ converge

    NARCIS (Netherlands)

    Piersma, Bram; Bank, Rudolf; Boersema, Miriam

    2015-01-01

    Chronic organ injury leads to fibrosis and eventually organ failure. Fibrosis is characterized by excessive synthesis, remodeling, and contraction of extracellular matrix produced by myofibroblasts. Myofibroblasts are the key cells in the pathophysiology of fibrotic disorders and their differentiati

  11. Sex Disparities in Effects of Cystic Fibrosis-Related Diabetes on Clinical Outcomes: A Matched Study

    Directory of Open Access Journals (Sweden)

    Robert J Miller

    2008-01-01

    Full Text Available BACKGROUND: Cystic fibrosis-related diabetes (CFRD is an increasingly prevalent comorbidity factor for patients with cystic fibrosis (CF. CFRD has been associated with an accelerated decline in clinical parameters and an increased mortality rate.

  12. Genetics Home Reference: hereditary fibrosing poikiloderma with tendon contractures, myopathy, and pulmonary fibrosis

    Science.gov (United States)

    ... Fibrosis National Heart, Lung, and Blood Institute (NHLBI): Pulmonary Function Tests National Heart, Lung, and Blood Institute (NHLBI): What Is Pulmonary Rehabilitation? Pulmonary Fibrosis Foundation: Treatment Options General Information ...

  13. El ejercicio como tratamiento de pacientes con claudicación intermitente de los miembros inferiores de origen vascular

    Directory of Open Access Journals (Sweden)

    Alberto Domenech

    2007-01-01

    Full Text Available La aterosclerosis de los miembros inferiores es una entidad que por sí misma, a causa del dolor isquémico, genera una gran limitación en los individuos afectados, con riesgo de pérdida de la extremidad afectada.Estudios recientes demuestran que el ejercicio programado es una excelente herramienta para el tratamiento, tanto del síntoma como de los factores de riesgo que generan la enfermedad.Numerosos trabajos prospectivos y aleatorizados han mostrado el beneficio del entrenamiento en la claudicación intermitente versus otras estrategias, invasivas o no.Los programas de caminata han logrado éxito cuando han sido de una duración no menor de seis meses, con al menos tres sesiones semanales de más de treinta minutos.El ejercicio produce además un descenso significativo en el riesgo cardiovascular en esta población de pacientes, ya que al mismo tiempo este método es terapéutico en la hipertensión arterial, la dislipidemia, el tabaquismo, la diabetes, la obesidad, el estrés, etc.A todo paciente con claudicación severa se le debería brindar la oportunidad de mejorar con el ejercicio aeróbico de marcha, en lugar de la revascularización como primera opción terapéutica de la claudicación crónica. No hacerlo lo privaría de la posibilidad de una mejoría significativa e incluso de la desaparición del síntoma.

  14. Experiencia en reconstrucción auricular en cáncer de piel con colgajo en "quesadilla"

    Directory of Open Access Journals (Sweden)

    C. Gutiérrez Gómez

    Full Text Available La reconstrucción auricular es una de las más difíciles ya que implica reproducir las sofisticadas y delicadas formas del pabellón auricular. Cuando hay que resecar piel en la oreja por un cáncer cutáneo y dejamos expuesto el cartílago, sin pericondrio, suele suceder que al colocar injertos no hay una integración adecuada de los mismos por las caprichosas formas y relieves del pabellón auricular; cuando es necesario resecar el pericondrio estamos obligados a cubrir el defecto con un colgajo y no con un simple injerto. Frente a esta dificultad técnica, diseñamos un colgajo ricamente vascularizado que preserva el cartílago no afectado con una buena cubierta y al mismo tiempo respeta la anatomía de la oreja. Para la cobertura del cartílago auricular anterior usamos un colgajo fasciocutáneo posterior que se asemeja a un plato típico de la cocina mexicana que llamamos "quesadilla", donde el cartílago por su color blanco recuerda el queso y el gran colgajo fasciocutaneo recuerda la tortilla que cubre al queso. Este colgajo incluye la piel enrollada del hélix, que en un segundo tiempo retornará a su lugar de origen anatómico mediante una z-plastía asimétrica. Presentamos, de entre una serie de 13 pacientes con carcinoma de pabellón auricular, 2 casos resueltos mediante esta técnica.

  15. Esclerosis sistémica complicada con síncope y bloqueo AV completo

    OpenAIRE

    Francisco Femenía; Mauricio Arce; Martín Arrieta

    2010-01-01

    La esclerosis sistémica es una compleja enfermedad que afecta el tejido conectivo, el sistema vascular y el sistema inmunológico, y se caracteriza por fibrosis cutánea y de órganos viscerales. Los bloqueos de rama y los hemibloqueos se presentan en el 25 a 75% de los casos y constituyen predictores independientes de mortalidad. Los bloqueos auriculoventriculares de segundo o tercer grado son muy raros. Presentamos el caso de una mujer de 47 años de edad, con diagnóstico de esclerosis sistémic...

  16. Influencia de los tratamientos microabrasivos sobre la resistencia adhesiva en dientes con fluorosis

    Directory of Open Access Journals (Sweden)

    Paola Mena Silva

    2015-12-01

    Full Text Available La búsqueda de tratamientos mínimamente invasivos y altamente estéticos para pacientes con fluorosis, ha llevado a buscar mecanismos para solucionar esta problemática, mediante la utilización de resinas como la primera opción. Sin embargo el principal problema del tratamiento, se centra en la poca adhesión debido a las características estructurales del esmalte fluorótico, en grados de fluorosis TF 1 al 3, el ácido no actúa correctamente, causando ausencia de grabado principalmente entre las estrías de Retzius, en las rugosidades y espacios adamantinos ocupados por detritus de materia orgánica y cúmulos de placa dentobacteriana, siendo necesario efectuar técnicas microabrasivas y desproteinizantes como paso previo a los procesos adhesivos. Así, el presente estudio pretendió determinar la resistencia adhesiva en dientes con fluorosis grados TF 2 y 3, empleando tres diferentes técnicas de abrasión adamantina, se seleccionaron 48 dientes divididos en 3 grupos de 16 muestras cada uno, 8 con grado TF2 y 8 con grado TF3. Las superficies del esmalte fueron preparadas mediante la técnica “microabrasión modificada”, la “técnica de microabrasión” y “Opalustre”, todos los grupos recibieron desproteinización con hipoclorito de sodio al 5% por un minuto. Posterior a lo cual fueron realizados los procesos adhesivos mediante sistema adhesivo y resina compuesta, sometiéndose posteriormente a pruebas de cizallamiento. Los resultados analizados mediante testes de ANOVA y T de Student no determinaron una diferencia estadísticamente significativa entre los grupos, la facilidad en los protocolos de aplicación debe ser considerada en la elección de la técnica, sin embargo resulta importante su ejecución en dientes afectados por fluorosis.

  17. PUDRICIÓN BASAL CAUSADA POR Phytophthora capsici EN PLANTAS DE CHILE TRATADAS CON VERMICOMPOST

    Directory of Open Access Journals (Sweden)

    Lidieth Uribe-Lor\\u00EDo

    2014-01-01

    Full Text Available Pudrición basal causada por Phytophthora capcisi en plantas de chile tratadas con vermicompost. El objetivo de este trabajo fue evaluar a nivel de invernadero el efecto de vermicompost sobre la incidencia y severidad de Phytophthora capcisi en plantas de chile (Capsicum annuum. Esta investigación se realizó entre febrero y marzo del 2012 en el Centro de Investigaciones Agronómicas, Sabanilla, San José, Costa Rica. Se utilizó un diseño completamente al azar con cuatro repeticiones y ocho plantas por unidad experimental en un arreglo factorial con los factores de dosis (0, 25% y 50% v/v vermicompost:suelo e inoculación (0 y 500 zoosporas por gramo de suelo. Se trasplantaron plántulas de 35 días, que fueron inoculadas dos semanas después del trasplante. La aplicación de vermicompost provocó un aumento significativo del peso fresco foliar y del peso seco foliar y radical; a mayor dosis, mayor fue el incremento. Las plantas de todos los tratamientos que incluyeron inoculación con P. capsici presentaron síntomas de la enfermedad en la raíz. Los valores de incidencia y severidad fueron mayores para el tratamiento con 50% de abono. En este tratamiento se presentaron síntomas de marchitez. La ausencia de diferencias en las variables de peso fresco y seco entre los tratamientos inoculados y sin inocular, a los que se adicionó abono al 25%, sugiere que esta dosis podría compensar el daño causado por el patógeno. Se observó una menor concentración de nutrimentos en los tejidos de las plantas inoculadas lo que indica que el daño causado a la raíz pudo haber afectado la adquisición de nutrimentos.

  18. CTGF is a central mediator of tissue remodeling and fibrosis and its inhibition can reverse the process of fibrosis.

    Science.gov (United States)

    Lipson, Kenneth E; Wong, Carol; Teng, Yuchin; Spong, Suzanne

    2012-01-01

    CTGF is a secreted matricellular protein with very complex biology. It has been shown to modulate many signaling pathways leading to cell adhesion and migration, angiogenesis, myofibroblast activation, and extracellular matrix deposition and remodeling, which together lead to tissue remodeling and fibrosis. It has been reported in the literature that inhibition of CTGF expression by siRNA prevents CCl4-induced liver fibrosis and can reverse fibrosis when administered after significant collagen deposition is observed. A monoclonal antibody to CTGF that is currently in clinical development (FG-3019) has demonstrated the ability to reverse vascular stiffening and improve cardiac function in a rat model of diabetic complications. FG-3019 has also exhibited activity in a murine radiation-induced pulmonary fibrosis model. When FG-3019 was administered to mice after a significant radiation-induced increase in lung density could be observed by CT imaging, the density of the lungs was observed to decrease over the period during which the antibody was administered and to remain stable after therapy had ceased. When considered together, these data indicate that inhibition of CTGF can prevent and reverse the process of fibrosis.

  19. Perfil del trabajador con discapacidad

    OpenAIRE

    CAPGEMINI; FUNDACIÓN ADECCO

    2014-01-01

    Capgemini y la Fundación Adeco analizan y reflexionan sobre empleo de las personas con discapacidad. A pesar de los avances que se están registrando en este ámbito, la situación laboral de las personas con discapacidad aún está lejos de alcanzar las cotas deseadas de normalización: mientras que la tasa de actividad de las personas sin discapacidad se sitúa en el 76,3%, la de las que sí la tienen desciende bruscamente hasta el 36,5%. En otras palabras, un 63,4% de las personas con discapacidad...

  20. Atomic Structure of the Cystic Fibrosis Transmembrane Conductance Regulator.

    Science.gov (United States)

    Zhang, Zhe; Chen, Jue

    2016-12-01

    The cystic fibrosis transmembrane conductance regulator (CFTR) is an anion channel evolved from the ATP-binding cassette (ABC) transporter family. In this study, we determined the structure of zebrafish CFTR in the absence of ATP by electron cryo-microscopy to 3.7 Å resolution. Human and zebrafish CFTR share 55% sequence identity, and 42 of the 46 cystic-fibrosis-causing missense mutational sites are identical. In CFTR, we observe a large anion conduction pathway lined by numerous positively charged residues. A single gate near the extracellular surface closes the channel. The regulatory domain, dephosphorylated, is located in the intracellular opening between the two nucleotide-binding domains (NBDs), preventing NBD dimerization and channel opening. The structure also reveals why many cystic-fibrosis-causing mutations would lead to defects either in folding, ion conduction, or gating and suggests new avenues for therapeutic intervention.

  1. Hypertonic saline in treatment of pulmonary disease in cystic fibrosis.

    LENUS (Irish Health Repository)

    Reeves, Emer P

    2012-01-01

    The pathogenesis of lung disease in cystic fibrosis is characterised by decreased airway surface liquid volume and subsequent failure of normal mucociliary clearance. Mucus within the cystic fibrosis airways is enriched in negatively charged matrices composed of DNA released from colonizing bacteria or inflammatory cells, as well as F-actin and elevated concentrations of anionic glycosaminoglycans. Therapies acting against airway mucus in cystic fibrosis include aerosolized hypertonic saline. It has been shown that hypertonic saline possesses mucolytic properties and aids mucociliary clearance by restoring the liquid layer lining the airways. However, recent clinical and bench-top studies are beginning to broaden our view on the beneficial effects of hypertonic saline, which now extend to include anti-infective as well as anti-inflammatory properties. This review aims to discuss the described therapeutic benefits of hypertonic saline and specifically to identify novel models of hypertonic saline action independent of airway hydration.

  2. Fibroscan ® : A noninvasive test of liver fibrosis assessment

    Directory of Open Access Journals (Sweden)

    Al-Ghamdi Abdullah

    2007-01-01

    Full Text Available Determination of the extent of progress of hepatic fibrosis is important in clinical practice, where it may reflect the severity of liver disease and predict response to treatment. Percutaneous liver biopsy is the gold standard for grading and staging of liver disease. However, liver biopsy is an invasive procedure with certain unavoidable risks and complications. Several methods have been studied in an attempt to reach a diagnosis of cirrhosis by noninvasive means. Fibroscan ® has been designed to quantify liver fibrosis by means of elastography and found to have reasonably good sensitivity and specificity patterns, especially in patients with advanced fibrosis and can be used as an alternative to liver biopsy.

  3. Vaccines for preventing infection with Pseudomonas aeruginosa in cystic fibrosis

    DEFF Research Database (Denmark)

    Johansen, Helle Krogh; Gøtzsche, Peter C

    2015-01-01

    BACKGROUND: Chronic pulmonary infection in cystic fibrosis results in progressive lung damage. Once colonisation of the lungs with Pseudomonas aeruginosa occurs, it is almost impossible to eradicate. Vaccines, aimed at reducing infection with Pseudomonas aeruginosa, have been developed...... in the vaccine group and 91 (1 severe) in the control group. In this large trial of a vaccine developed against flagella antigens, antibody titres against the epitopes contained in the vaccine were higher in the vaccine group compared to the placebo group (P Vaccines against....... This is an update of a previously published review. OBJECTIVES: To assess the effectiveness of vaccination against Pseudomonas aeruginosa in cystic fibrosis. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register using the terms vaccines AND pseudomonas (last search 30...

  4. Combined pulmonary fibrosis and emphysema: an increasingly recognized condition

    Directory of Open Access Journals (Sweden)

    Olívia Meira Dias

    2014-06-01

    Full Text Available Combined pulmonary fibrosis and emphysema (CPFE has been increasingly recognized in the literature. Patients with CPFE are usually heavy smokers or former smokers with concomitant lower lobe fibrosis and upper lobe emphysema on chest HRCT scans. They commonly present with severe breathlessness and low DLCO, despite spirometry showing relatively preserved lung volumes. Moderate to severe pulmonary arterial hypertension is common in such patients, who are also at an increased risk of developing lung cancer. Unfortunately, there is currently no effective treatment for CPFE. In this review, we discuss the current knowledge of the pathogenesis, clinical characteristics, and prognostic factors of CPFE. Given that most of the published data on CPFE are based on retrospective analysis, more studies are needed in order to address the role of emphysema and its subtypes; the progression of fibrosis/emphysema and its correlation with inflammation; treatment options; and prognosis.

  5. Adoptive transfer of macrophages ameliorates renal fibrosis in mice.

    Science.gov (United States)

    Nishida, Masashi; Okumura, Yasuko; Fujimoto, Shin-Ichiro; Shiraishi, Isao; Itoi, Toshiyuki; Hamaoka, Kenji

    2005-06-24

    We performed adoptive transfer of bone marrow-derived (BM) macrophages following pharmacological depletion of leukocytes in a mouse model of unilateral ureteral obstruction (UUO). Treatment with cyclophosphamide (CPM) caused marked decrease in the numbers of F4/80-positive interstitial macrophages as well as in peripheral blood leukocyte counts, and adoptive transfer of BM macrophages to CPM-treated mice resulted in significant increase in the numbers of interstitial macrophages both at day 5 and at day 14 after UUO. At day 5 after UUO, no significant change was observed in the degree of renal interstitial fibrosis either by treatment with CPM or with CPM+macrophage. However, at day 14 after UUO, treatment with CPM caused significant increase in the degree of interstitial fibrosis, and adoptive macrophage transfer to these mice attenuated this enhancement in renal fibrosis. Our result suggests the role of infiltrating macrophages on facilitating tissue repair at late stage of UUO.

  6. An atypical presentation of cystic fibrosis: a case report

    Directory of Open Access Journals (Sweden)

    Joshi Deepak

    2008-06-01

    Full Text Available Abstract Introduction The presentation of cystic fibrosis is dependant upon which organs are affected. Common presentations include chronic respiratory infections and malabsorption. Patients with atypical disease tend to present late in childhood or as adults. Eye manifestations of cystic fibrosis are less well known. Case presentation A 14-year-old Caucasian boy presented with tiredness and difficulty seeing at night, over a period of 6 months. Good vision was only described in bright conditions. There was no history of jaundice, steatorrhea or diarrhoea. Conclusion This is the first reported case of newly diagnosed cystic fibrosis-related liver disease in a teenage boy, whose presenting symptom was night blindness secondary to vitamin A deficiency.

  7. Hypertonic Saline in Treatment of Pulmonary Disease in Cystic Fibrosis

    Directory of Open Access Journals (Sweden)

    Emer P. Reeves

    2012-01-01

    Full Text Available The pathogenesis of lung disease in cystic fibrosis is characterised by decreased airway surface liquid volume and subsequent failure of normal mucociliary clearance. Mucus within the cystic fibrosis airways is enriched in negatively charged matrices composed of DNA released from colonizing bacteria or inflammatory cells, as well as F-actin and elevated concentrations of anionic glycosaminoglycans. Therapies acting against airway mucus in cystic fibrosis include aerosolized hypertonic saline. It has been shown that hypertonic saline possesses mucolytic properties and aids mucociliary clearance by restoring the liquid layer lining the airways. However, recent clinical and bench-top studies are beginning to broaden our view on the beneficial effects of hypertonic saline, which now extend to include anti-infective as well as anti-inflammatory properties. This review aims to discuss the described therapeutic benefits of hypertonic saline and specifically to identify novel models of hypertonic saline action independent of airway hydration.

  8. Nephrogenic systemic fibrosis and gadolinium-based contrast media

    DEFF Research Database (Denmark)

    Thomsen, Henrik S; Morcos, Sameh K; Almén, Torsten

    2012-01-01

    PURPOSE: To update the guidelines of the Contrast Media Safety Committee (CMSC) of the European Society of Urogenital Radiology (ESUR) on nephrogenic systemic fibrosis and gadolinium-based contrast media. AREAS COVERED: Topics reviewed include the history, clinical features and prevalence...... of nephrogenic systemic fibrosis and the current understanding of its pathophysiology. The risk factors for NSF are discussed and prophylactic measures are recommended. The stability of the different gadolinium-based contrast media and the potential long-term effects of gadolinium in the body have also been...... reviewed. KEY POINTS : • Clinical features, risk factors and prevention of nephrogenic systemic fibrosis are reviewed • Patients with GFR below 30 ml/min/1.73 m ( 2 ) have increased risk of developing NSF • Low stability gadolinium contrast media show the strongest association with NSF • Following...

  9. Combined pulmonary fibrosis and emphysema in hypersensitivity pneumonitis.

    Science.gov (United States)

    Soumagne, Thibaud; Pana-Katatali, Héloïse; Degano, Bruno; Dalphin, Jean-Charles

    2015-12-21

    Combined pulmonary fibrosis and emphysema is a distinct syndrome reported in patients who smoke. A 72-year-old, never-smoking female dairy farmer was referred for progressive dyspnoea on exertion, basal crackles on auscultation, normal spirometry and normal lung volumes but decreased diffusing capacity of the lung for carbon monoxide, centrilobular emphysema in the upper zones of the lungs and diffuse infiltrative lung disease in the lower zones on high-resolution CT scan. Bronchoalveolar lavage differential cell count showed 35% lymphocytosis, and precipitating antibodies for Wallemia sebi, Trichoderma species and Cladosporium sphaerospermum were identified. The diagnosis of farmer's lung disease with combined pulmonary fibrosis and emphysema was retained. This case highlights for the first time that hypersensitivity pneumonitis should be suspected in the setting of combined pulmonary fibrosis and emphysema in non-smoking patients.

  10. Cystic fibrosis and pregnancy: counseling, obstetrical management and perinatal outcome.

    Science.gov (United States)

    Grigoriadis, Charalampos; Tympa, Aliki; Theodoraki, Kassiani

    2015-03-01

    The progress in research of in vitro fertilization and fetal-maternal medicine allows more women and men, with fertility problems due to cystic fibrosis, to have a baby. In the majority of cases, pregnancy in women with cystic fibrosis results in favorable maternal and fetal outcomes. However, the incidence of preterm delivery, intrauterine growth restriction, caesarean section and deterioration of the maternal health are increased. Pre-pregnancy counseling is a crucial component of overall obstetric care, especially in women with poor pulmonary function. Additionally, closer monitoring during pregnancy with a multidisciplinary approach is required. The value of serial ultrasound scans and fetal Doppler assessment is important for the control of maternal and fetal wellbeing, as well as for the definition of the appropriate timing of delivery. In this article, clinical issues of pregnant women with cystic fibrosis are reviewed; counseling, obstetrical management and perinatal outcomes are being discussed.

  11. Night blindness in a teenager with cystic fibrosis.

    LENUS (Irish Health Repository)

    Roddy, Marie Frances

    2011-12-01

    This article describes the case of a 16-year-old boy with cystic fibrosis who presented with difficulty seeing in the dark. He had a history of bowel surgery at birth, and he developed cystic fibrosis liver disease and osteopenia during his teenage years. He always had good lung function. When his serum vitamin A level was checked, it was undetectable in sample. He was diagnosed with night blindness and commenced on high-dose vitamin A. His symptoms resolved within 3 days. However, it took over 1 year for his vitamin A level to return to normal. This case emphasizes the importance of monitoring vitamin levels in cystic fibrosis to detect deficiency and prevent long-term consequences, and it highlights the challenges encountered during the course of night blindness treatment.

  12. Molecular pathways: connecting fibrosis and solid tumor metastasis.

    Science.gov (United States)

    Cox, Thomas R; Erler, Janine T

    2014-07-15

    Pathologic organ fibrosis is a condition that can affect all major tissues and is typically ascribed to the excessive accumulation of extracellular matrix components, predominantly collagens. It typically leads to compromise of organ function and subsequent organ failure, and it is estimated that 45% of deaths in the developed world are linked to fibrotic disease. Fibrosis and cancer are known to be inextricably linked; however, we are only just beginning to understand the common and overlapping molecular pathways between the two. Here, we discuss what is known about the intersection of fibrosis and cancer, with a focus on cancer metastasis, and highlight some of the exciting new potential clinical targets that are emerging from analysis of the molecular pathways associated with these two devastating diseases.

  13. Modulation of surgical fibrosis by microbial zwitterionic polysaccharides

    Science.gov (United States)

    Ruiz-Perez, Begonia; Chung, Doo R.; Sharpe, Arlene H.; Yagita, Hideo; Kalka-Moll, Wiltrud M.; Sayegh, Mohamed H.; Kasper, Dennis L.; Tzianabos, Arthur O.

    2005-11-01

    Bacterial carbohydrates have long been considered T cell-independent antigens that primarily induce humoral immune responses. Recently, it has been demonstrated that bacterial capsules that possess a zwitterionic charge motif can activate CD4+ T cells after processing and presentation by antigen-presenting cells. Here we show that these zwitterionic polysaccharides can prevent T helper 1-mediated fibrosis by signaling for the release of IL-10 from CD4+ T cells in vivo. IL-10 production by these T cells and their ability to prevent fibrosis is controlled by the inducible costimulator (ICOS)-ICOS ligand pathway. These data demonstrate that the interaction of the zwitterionic polysaccharides with T cells results in modulation of surgical fibrosis in vivo and suggest a previously undescribed approach to "harnessing" T cell function to prevent inflammatory tissue disorders in humans. IL-10 | microbial polysaccharides | inducible costimulator

  14. Autophagy: A new therapeutic target for liver fibrosis

    Institute of Scientific and Technical Information of China (English)

    2015-01-01

    Hepatic fibrosis is a wound-healing response to liverinjury and the result of imbalance of extracellular matrix(ECM) accumulation and degradation. The relentless production and progressive accumulation of ECM canlead to end-stage liver disease. Although significantprogress has been achieved in elucidating the mechanismsof fibrogenesis, effective anti-fibrotic strategiesare still lacking. Autophagy is an intracellular process ofself-digestion of defective organelles to provide materialrecycling or energy for cell survival. Autophagy hasbeen implicated in the pathophysiology of many humandisorders including hepatic fibrosis. However, the exactrelationships between autophagy and hepatic fibrosisare not totally clear and need further investigations.A new therapeutic target for liver fibrosis could bedeveloped with a better understanding of autophagy.

  15. Epithelial to mesenchymal transition in the progression of tubulointerstitial fibrosis

    Institute of Scientific and Technical Information of China (English)

    LI Min-xia; LIU Bi-cheng

    2007-01-01

    Objective To review the mechanisms of epithelial to mesenchymal transition (EMT) and its role in the progression of tubulointerstitial fibrosis.Data sources The data used in this review were obtained mainly from the studies of EMT reported from 2000-2006.Study selection Relevant articles on studies of EMT in tubulointerstitial fibrosis were selected. Data were mainly extracted from the 45 articles listed in the reference section of this review.Results The process of EMT has gained wide recognition as candidate mechanism in progression of chronic fibrotic disorders. New markers were identified and facilitate the observation of EMT. EMT is regulated by many factors through activation of kinase-dependent signaling cascades. Recent findings suggest that EMT is a reversible process, which can be controlled by factors for their epithelial inducing activities.Conclusion Remarkable progresses of EMT research have been made recently. Preventing or reversing EMT is a promising strategy against renal fibrosis.

  16. Mechanisms of the noxious inflammatory cycle in cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Freyssinet Jean-Marie

    2009-03-01

    Full Text Available Abstract Multiple evidences indicate that inflammation is an event occurring prior to infection in patients with cystic fibrosis. The self-perpetuating inflammatory cycle may play a pathogenic part in this disease. The role of the NF-κB pathway in enhanced production of inflammatory mediators is well documented. The pathophysiologic mechanisms through which the intrinsic inflammatory response develops remain unclear. The unfolded mutated protein cystic fibrosis transmembrane conductance regulator (CFTRΔF508, accounting for this pathology, is retained in the endoplasmic reticulum (ER, induces a stress, and modifies calcium homeostasis. Furthermore, CFTR is implicated in the transport of glutathione, the major antioxidant element in cells. CFTR mutations can alter redox homeostasis and induce an oxidative stress. The disturbance of the redox balance may evoke NF-κB activation and, in addition, promote apoptosis. In this review, we examine the hypotheses of the integrated pathogenic processes leading to the intrinsic inflammatory response in cystic fibrosis.

  17. Mucormicosis yeyunal en paciente con linfoma de Hodgkin Jejunal mucormycosis in a patient with Hodgkin's lymphoma

    Directory of Open Access Journals (Sweden)

    B. Madrigal

    2008-08-01

    Full Text Available Comunicamos un caso de mucormicosis intestinal en un hombre de 46 años de edad, diagnosticado de enfermedad de Hodgkin clásica, estadio IV-B. Durante la primera fase de la quimioterapia, sufrió una hemorragia digestiva masiva secundaria a una úlcera yeyunal por zigomicosis tipo mucor, diagnosticada por biopsia endoscópica. El paciente fue tratado con antifúngicos y resección quirúrgica del intestino afectado. En la cirugía, se apreció una doble perforación yeyunal cubierta. El estudio anatomopatológico de la pieza confirmó el diagnóstico previo. Tras un año de seguimiento, el paciente está recuperado y su linfoma de Hodgkin en remisión completa. Tras una extensa revisión de la literatura, según nuestro conocimiento, este es el segundo caso publicado en la literatura de mucormicosis intestinal en un paciente con linfoma de Hodgkin.We report a case of intestinal mucormycosis in a 46-year-old male diagnosed with classical Hodgkin's disease, IV-B stage. During the first phase of chemotherapy he had a massive digestive bleeding event secondary to a jejunal ulcer, and zygomicosis mucor-type was diagnosed by endoscopic biopsy. The patient was treated with antifungal drugs and surgical resection of the intestine involved. At surgery a double covered perforation of the jejunum was seen. Pathological examination confirmed the previous diagnosis. After one year of follow-up the patient is doing well, and his lymphoma is on remission. To our best knowledge this is the second case of intestinal mucormycosis in a patient with Hodgkin's lymphoma reported in the medical literature.

  18. Phagocytosis of Pseudomonas aeruginosa by polymorphonuclear leukocytes and monocytes: effect of cystic fibrosis serum.

    OpenAIRE

    Thomassen, M J; Demko, C A; Wood, R.E.; Sherman, J. M.

    1982-01-01

    It has been shown previously that serum from chronically infected patients with cystic fibrosis inhibits the phagocytosis of Pseudomonas aeruginosa by both normal and cystic fibrosis alveolar macrophages. In the present study, the ability of peripheral monocytes and polymorphonuclear leukocytes from normal volunteers and cystic fibrosis patients to phagocytize P. aeruginosa was shown not to be inhibited in the presence of serum from cystic fibrosis patients.

  19. Draft genome sequences of four Achromobacter ruhlandii strains isolated from cystic fibrosis patients

    Science.gov (United States)

    Rodrigues, Elenice RA; Rocha, Géssica A; Ferreira, Alex G; Leão, Robson S; Albano, Rodolpho M; Marques, Elizabeth A

    2016-01-01

    Achromobacter species are being increasingly isolated from the respiratory tract of cystic fibrosis patients. Recent reports indicate that Achromobacter ruhlandii is a potential human pathogen in cystic fibrosis-related infections. Here we report the draft genome of four A. ruhlandii strains isolated from cystic fibrosis patients in Brazil. This report describes A. ruhlandii as a potential opportunistic pathogen in cystic fibrosis and provides a framework to for additional enquires into potential virulence factors and resistance mechanisms within this species. PMID:27812598

  20. Losartan attenuates paraquat-induced pulmonary fibrosis in rats.

    Science.gov (United States)

    Guo, F; Sun, Y B; Su, L; Li, S; Liu, Z F; Li, J; Hu, X T; Li, J

    2015-05-01

    Paraquat (PQ) is one of the most widely used herbicides in the world and can cause pulmonary fibrosis in the cases with intoxication. Losartan, an angiotensin II type 1 receptor antagonist, has beneficial effects on the treatment of fibrosis. The aim of this study was to examine the effect of losartan on pulmonary fibrosis in PQ-intoxicated rats. Adult male Sprague Dawley rats (n = 32, 180-220 g) were randomly assigned to four groups: (i) control group; (ii) PQ group; (iii) PQ + losartan 7d group; and (iv) PQ + losartan 14d group. Losartan treatment (intragastrically (i.g.), 10 mg/kg) was performed for 7 and 14 days after a single i.g. dose of 40 mg/kg PQ. All rats were killed on the 16th day, and hematoxylin-eosin and Masson's trichrome staining were used to examine lung injury and fibrosis. The levels of hydroxyproline and transforming growth factor β1 (TGF-β1), matrix metallopeptidase 9 (Mmp9), and tissue inhibitor of metalloproteinase 1 (TIMP-1) messenger RNA (mRNA) expression and relative expression levels of collagen type I and III were also detected. PQ caused a significant increase in hydroxyproline content, mRNA expression of TGF-β1, Mmp9, and TIMP-1, and relative expression levels of collagen type I and III ( p losartan significantly decreased the amount of hydroxyproline and downregulated TGF-β1, Mmp9, and TIMP-1 mRNA and collagen type I and III expressions ( p losartan could markedly reduce such damage and prevent pulmonary fibrosis. The results of this study indicated that losartan could reduce lung damage and prevent pulmonary fibrosis induced by PQ.