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Sample records for afectados con fibrosis

  1. Escala de Estrés y Afrontamiento para familias con hijos afectados de Parálisis Cerebral

    OpenAIRE

    Badia Corbella, Marta; Aguado Díaz, Antonio-León

    2002-01-01

    La evaluación del estrés en las familias con hijos que tienen alguna discapacidad resulta una tarea compleja y difícil. La Escala de Estrés y Afrontamiento para familias con hijos afectados de Parálisis Cerebral (EEAF–PC) ha sido diseñada para dar respuesta a la necesidad de ofrecer un instrumento de medición del estrés para tales familias. La EEAF–PC consta de 42 ítems y el análisis factorial ha proporcionado seis factores: características de la Discapacidad, Protección, Riesgo, Retraso Ment...

  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. La ocupación laboral de los convivientes con afectados por discapacidades. Un análisis multivariable

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    Spijker, Jeroen

    2010-08-01

    Full Text Available As a result of deep demographic, health and social changes, as well as adjustments to public health policy that now seeks the maximum involvement of family members, the relationship between the role of caregiver and employment has observed many changes during the past decades. using the Spanish Survey on Disability, Deficiency and Health Status we investigate the relationship between co-residing with or caring for people with disabilities and employment using multivariate logistic regression analysis. results indicate that it is not gender but the role as caregiver that is the main determinant of being employed. it is also found that both roles become more burdensome at the expense of gender equality in homes that have to cope with the disability of one of its members.

    La relación entre el rol de cuidador y la actividad laboral ha cambiado mucho durante las últimas décadas, influida por la evolución del contexto demográfico, sanitario y sociológico, pero también por una política sociosanitaria que persigue la máxima implicación de los familiares. utilizando la Encuesta sobre Discapacidades, Deficiencias y Estado de Salud, y mediante el análisis de regresión logística multivariable, investigamos cómo se relacionan en los hogares la convivencia o cuidado de personas con discapacidad y la ocupación laboral de los convivientes. los resultados confirman que el determinante principal es la asunción del rol de cuidador y no el sexo, pero también se comprueba que los roles complementarios, masculinos y femeninos, se hacen más intensos en los hogares que deben afrontar la discapacidad de alguno de sus miembros, en detrimento de la igualación entre hombres y mujeres.

  4. Percepción de las madres con hijos afectados por malformaciones congénitas mayores: necesidad de desarrollo de un sistema de atención apropiado. Estudio cualitativo de grupos focales

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    Fernando Suárez-Obando

    2009-03-01

    Full Text Available ntroducción: Las enfermedades que son en parte o totalmente determinadas por factores genéticos, asumen paulatinamente una proporción de mayor importancia dentro del perfil epidemiológico de la población infantil, convirtiéndose en una de las principales causas de mortalidad y morbilidad. La percepción de las madres de los niños afectados por malformaciones congénitas mayores no ha sido estudiada con anterioridad en relación al sistema de salud colombiano.Objetivos: Conocer las percepciones que tienen las madres de niños afectados con malformaciones congénitas, sobre la etiología de la enfermedad de sus hijos, la atención médica recibida y las posibles consecuencias de la malformación.Métodos: Estudio cualitativo descriptivo, de tipo exploratorio a través de grupos focales con madres de niños que padecen malformaciones congénitas mayores.Resultados: Las madres identificaron los problemas de atención al niño en relación al sistema de salud, al tratamiento ambivalente y excluyente del personal de salud y la necesidad de respuestas claras sobre el futuro de los niños, así como la necesidad de establecer reformas en la atención de las personas afectadas.

  5. Estrés y afrontamiento en pacientes afectados de esclerosis múltiple. Estudio comparativo con población comunitaria

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    Lara, Silvia; Kirchner, Teresa

    2012-01-01

    La Esclerosis Múltiple (EM) es una enfermedad neurodegenerativa que lleva implícita no sólo limitaciones físicas, sino también numerosos estresores psicosociales. Los objetivos de este estudio son: 1) analizar los problemas más comúnmente narrados por afectados de EM, la intensidad de estrés que les generan, el grado de control percibido sobre ellos y las estrategias de afrontamiento que utilizan para minimizar su impacto 2) contrastar el nivel de estrés, de control sobre el estresor y el uso...

  6. 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.

     

     

  7. 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

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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.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

  8. 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

  9. Uso de neumocitos de tipo II en el tratamiento de enfermedades pulmonares asociadas con fibrosis pulmonar

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    Serrano-Mollar, Anna; Closa, Daniel; Bulbena, Oriol

    2005-01-01

    Se describe el empleo de neumocitos tipo II como agentes inhibidores de la proliferación de fibroblastos, por lo que pueden ser utilizados en la elaboración de un medicamento para el tratamientode enfermedades pulmonares que cursan con fibrosis pulmonar.

  10. Nutrición en el enfermo con fibrosis quística

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    Lopez-Legarrea, P. (Patricia); J. A. Martinez

    2010-01-01

    La fibrosis quística es una enfermedad que se asocia a una mutación de un gen localizado en el brazo largo del cromosoma 7. Esta enfermedad multisistémica afecta especialmente a los pulmones y al páncreas exocrino, lo cual supone el trastorno más influyente en la nutrición de estos pacientes. Generalmente, la suplementación con enzimas pancreáticas es necesaria como elemento terapéutico. La prevalencia de diabetes en esta enfermedad aumenta con la edad, mientras que la afectación hepática clí...

  11. Comparación entre hibridización in situ e inmunoperoxidasa indirecta para la determinación de la ubicación de pasteurella multocida en conejos afectados con enfermedad respiratoria

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    Franco Mesa, Martha Lilia

    2012-01-01

    La Pasteurella multocida es considerada un habitante normal del tracto respiratorio superior de los conejos y es el patógeno más frecuentemente aislado en la enfermedad respiratoria de esta especie, sin embargo poco se conoce acerca de su ubicación, distribución y densidad. En el presente estudio se estandarizó la técnica de hibridización in situ (HIS) para P. multocida, y se comparó esta marcación con la obtenida con técnicas de rutina como Inmunoperoxidasa Indirecta (IPI), confirmando así l...

  12. 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

  13. 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

  14. 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...

  15. 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.

  16. 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

  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. NEUMOTÓRAX ESPONTÁNEO ASOCIADO A FIBROSIS PULMONAR EN UN PACIENTE CON NEUROFIBROMATOSIS TIPO 2 Spontaneous pneumothorax associated with pulmonary fibrosis in a patient with neurofibromatosis type 2

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    Gabriel Alcalá-Cerra¹; Luis Rafael Moscote-Salazar; Carlos Fernando Lozano-Tagua; Rubén Sabogal-Barrios

    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...

  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. 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

  1. Epidemiologia molecolare di ceppi di Stenotrophomonas maltophilia isolati da pazienti con fibrosi cistica

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    Ersilia Fiscarelli

    2006-06-01

    Full Text Available Eighty-nine Stenotrophomonas maltophilia isolates were recovered from 42 cystic fibrosis patients in a paediatric hospital of Rome, Italy, during a 17-months period.Twenty patients presented repeated episodes of S. maltophilia isolates, with 2 to7 isolates per patient. The antimicrobial susceptibilities of the strains showed that trimethoprim-sulfamethoxazole was the most active antibiotic, inhibiting more than 90% of the strains, followed by levofloxacin, with 62 out of 89 isolates being sensitive (69.7%. Genetic relatedness of S. maltophilia isolates was investigated by pulsed-field gel electrophoresis (PFGE. A total of 49 well-defined distinct XbaI PFGE profiles were identified, with 39 different PFGE types encountered.Twenty-one PFGE types were represented by multiple isolates, with the most frequent being PFGE type 4 (11 isolates, PFGE type 9 and 11 (7 isolates each, and PFGE type 3 (5 isolates. Persistence of S. maltophilia infection or colonization was identified in 17 patients with repeated episodes, while genetically distinct S. maltophilia isolates from the same patient was found in 6 cases. Genetically identical or highly related isolates from different patients were observed within 8 multiple strain PFGE types.This study revealed a high genetic heterogeneity among S. maltophilia isolates recovered from cystic fibrosis patients. All together these data seem to confirm the wide environmental distribution of this pathogen. Different items of acquisition were involved both outside and inside the nosocomial settings, with the crosstransmission observed among clinical isolates of S. maltophilia among different patients probably due by multiple independent acquisitions from the environment as a main mode of transmission.

  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. Tabla de supervivencia de Pinus Halepensis afectado por incendios forestales

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    BORDON PEREZ, PABLO; Pérez-Laorga Arias, Eduardo María; Estruch Fuster, Vicente Domingo; Rodrigo Santamalia, Mª Eugenia

    2012-01-01

    En la Comunidad Valenciana se producen numerosos incendios forestales especialmente en los meses de verano, afectando principalmente a pino carrasco (Pinus halepensis Mill), que es la especie más abundante. Se ha realizado el seguimiento de pinos parcialmente afectados por el fuego en dos incendios que se produjeron en agosto de 2007 y julio de 2009. En estos árboles se midieron: altura del árbol, altura del fuste, diámetro normal, diámetro basal, espesor de corteza, altura del fuste y altura...

  4. Evolución funcional respiratoria en dos pacientes con enfisema y fibrosis pulmonar Functional respiratory evolution in two patients with emphysema and pulmonary fibrosis

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    Santiago C. Arce; Luciana Molinari; Eduardo L. De Vito

    2009-01-01

    La combinación de enfisema y fibrosis pulmonar es una condición frecuentemente subdiagnosticada. Los estudios funcionales aislados pueden generar interpretaciones inadecuadas. No hemos hallado comunicaciones de casos que documenten la evolución espirométrica de estos pacientes. Se presentan dos casos de fibrosis y enfisema combinados que permiten observar la evolución funcional a largo plazo y comprender los valores espirométricos actuales en forma más precisa. Los hallazgos más relevantes so...

  5. 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.

  6. NEUMOTÓRAX ESPONTÁNEO ASOCIADO A FIBROSIS PULMONAR EN UN PACIENTE CON NEUROFIBROMATOSIS TIPO 2 Spontaneous pneumothorax associated with pulmonary fibrosis in a patient with neurofibromatosis type 2

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    Gabriel Alcalá-Cerra¹

    2010-01-01

    Full Text Available 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 había sido reportada.Pulmonary involvement in patients with neurofibromatosis has been repetitively reported as a very rare complication in type 1 variety. It is characterized by pulmonary intersticial disease, pulmonary fibrosis and bullaes, the last with high risk of rupture. We described a case of spontaneous pneumothorax in a patient with type 2 neurofibromatosis, as consequence of pulmonary fibrotic changes. To our knowledge this association had not been reported.

  7. 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

  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. 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; María Anders; José Mella; Marcelo Amante; Federico Orozco; Ricardo Mastai

    2012-01-01

    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 enfermed...

  15. 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.

  16. Comunidades de afectados, procomún y don expandido

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    Lafuente, Antonio; Corsín Jiménez, Alberto

    2010-01-01

    El don existe. Existe, pero como tantas cosas en nuestro mundo, incluso muy importantes, parece invisible. Allí siempre habrá una mujer cuidando de un bebé, un enfermo o un anciano. En los alrededores, cualquiera que sea la dirección a donde apuntemos, habrá alguien usando la lengua, respirando aire, jugando en la calle o protestando por el ruido. Y conste que podríamos subir el tono y mencionar todo lo que tenga que ver con añorar justicia, gozar paisajes, reclamar salud o disfrutar el si...

  17. 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

  18. Excavación, traslado y reubicación de petroglifos afectados por obras públicas: la experiencia del Morrazo (Galicia).

    OpenAIRE

    Mañana-Borrazás, Patricia; Seoane-Veiga, Yolanda

    2008-01-01

    [ES] Se presentan tres actuaciones llevadas a cabo en el año 2003, con el fin de mitigar el impacto que la construcción de una carretera de alta capacidad (corredor) produciría sobre varios petroglifos de la comarca del Morrazo (Galicia). Los grabados estaban directamente afectados por las obras, por lo que se adoptó como medida correctora su excavación, extracción y traslado. Las excavaciones se plantearon en torno a las rocas grabadas, tratando de proporcionar información de su contexto arq...

  19. 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; SOLANGE CAUSSADE L; LUIS VEGA B; GISELA MOYA D; CLAUDIO CALLEJAS C; CONSUELO ARANGUIZ G; IGNACIO SÁNCHEZ D.

    2008-01-01

    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. 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.

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    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.

  1. 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

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    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.

  2. 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.

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

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    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.

  4. 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

    Directory of Open Access Journals (Sweden)

    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

  5. 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 ...

  6. Learn About Pulmonary Fibrosis

    Science.gov (United States)

    ... www.lung.org > Lung Health and Diseases > Lung Disease Lookup > Pulmonary Fibrosis Learn About Pulmonary Fibrosis Pulmonary fibrosis is ... while processing XML file."); } }); } } --> Blank Section Header Lung Disease Lookup Pulmonary Fibrosis Learn About Pulmonary Fibrosis Pulmonary Fibrosis Symptoms, ...

  7. 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

    Directory of Open Access Journals (Sweden)

    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.

  8. Living with Pulmonary Fibrosis

    Science.gov (United States)

    ... www.lung.org > Lung Health and Diseases > Lung Disease Lookup > Pulmonary Fibrosis Living With Pulmonary Fibrosis What to Expect ... while processing XML file."); } }); } } --> Blank Section Header Lung Disease Lookup Pulmonary Fibrosis Learn About Pulmonary Fibrosis Pulmonary Fibrosis Symptoms, ...

  9. 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

  10. 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

  11. 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

  12. 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

  13. Cystic Fibrosis

    Science.gov (United States)

    ... mucus and needs to spit it out. In gym class, she participates in sports but often gets ... make normal mucus, the body needs a special protein. This protein is defective in cystic fibrosis, producing ...

  14. 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

  15. Cystic fibrosis - resources

    Science.gov (United States)

    Resources - cystic fibrosis ... The following organizations are good resources for information on cystic fibrosis : Cystic Fibrosis Foundation -- www.cff.org March of Dimes -- www.marchofdimes.org/baby/cystic-fibrosis-and- ...

  16. Ceramide mediates lung fibrosis in cystic fibrosis

    OpenAIRE

    Ziobro, Regan; Henry, Brian; Edwards, Michael J.; Lentsch, Alex B.; Gulbins, Erich

    2013-01-01

    Fibrosis of the lung is one of the major clinical problems of cystic fibrosis and chronic obstructive pulmonary disease. However, the molecular mechanisms leading to pulmonary fibrosis are poorly characterized and require definition. Here, we demonstrate that chronic accumulation of ceramide in the lung contributes to the development of fibrosis in aged cystic fibrosis mice. Genetic or pharmacological normalization of ceramide in cystic fibrosis mice, which was achieved by heterozygosity of a...

  17. 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

  18. Cystic fibrosis

    OpenAIRE

    2004-01-01

    In the 1930s, when cystic fibrosis (CF) was first clearly recognised, it was a disorder that inevitably led to death in early childhood from respiratory failure and malnutrition. Since that time, antibiotic treatment and improving nutrition have brought increasing hope to sufferers from the disorder, so that increasing numbers of children have lived on into adult life. Chaired by Professor John Walker-Smith, and attended by a group of leading experts in field at the time, this transcript disc...

  19. 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 ...

  20. 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 ...

  1. Pulmonary Fibrosis Foundation

    Science.gov (United States)

    ... the most current news and updates from the Pulmonary Fibrosis Foundation. Life with PF Education & Support About PF ... open ! JOIN NOW We Imagine a World Without Pulmonary Fibrosis The Pulmonary Fibrosis Foundation mobilizes people and resources ...

  2. 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

  3. 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

    Directory of Open Access Journals (Sweden)

    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

  4. Biomarkers for liver fibrosis

    Energy Technology Data Exchange (ETDEWEB)

    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.

  5. Clinical and Epidemiological Characteristics of Patients with Condilomatosis Características clínico-epidemiológicas de pacientes afectados por condilomatosis

    Directory of Open Access Journals (Sweden)

    Graciela Caridad Cabrera Acea

    2011-08-01

    ínico-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.

  6. 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 ...

  7. Cystic fibrosis - nutritional considerations

    Science.gov (United States)

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

  8. Cystic fibrosis: case report

    International Nuclear Information System (INIS)

    Cystic fibrosis is a 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. 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.

  10. Congenital Hepatic Fibrosis

    Directory of Open Access Journals (Sweden)

    MH Antikchi

    2010-09-01

    Full Text Available Congenital hepatic fibrosis (CHF is a rare disease that primarily involves hepatobiliary and renal systems. It is characterized by hepatic fibrosis, portal hypertension and renal cystic disease. We present a 22 years old man with fever, abdominal pain, icterus and hematemesis. On complete work up of the patient and liver with kidney biopsy, the diagnosis was congenital hepatic fibrosis.

  11. 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.

  12. 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

  13. How Is Cystic Fibrosis Diagnosed?

    Science.gov (United States)

    ... page from the NHLBI on Twitter. How Is Cystic Fibrosis Diagnosed? Doctors diagnose cystic fibrosis (CF) based on ... tested to see whether the baby has CF. Cystic Fibrosis Carrier Testing People who have one normal CFTR ...

  14. 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. Print All Open All Close All Description Cystic fibrosis is an inherited disease characterized by the buildup ...

  15. 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 . ...

  16. 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.

  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. Symptoms, Diagnosis and Treatment for Pulmonary Fibrosis

    Science.gov (United States)

    ... www.lung.org > Lung Health and Diseases > Lung Disease Lookup > Pulmonary Fibrosis Pulmonary Fibrosis Symptoms, Causes & Risk Factors Pulmonary ... while processing XML file."); } }); } } --> Blank Section Header Lung Disease Lookup Pulmonary Fibrosis Learn About Pulmonary Fibrosis Pulmonary Fibrosis Symptoms, ...

  19. 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

  20. Tamoxifen for retroperitoneal fibrosis.

    OpenAIRE

    Frankart, L.; Lorge, Francis; Donckier, Julian

    1997-01-01

    Retroperitoneal fibrosis, either idiopathic or associated with aortic inflammatory aneurysm, is a chronic fibrotic disease that causes progressive obstruction of ureters and vessels around the lower abdominal aorta. Treatment is often difficult (surgery) or hazardous (steroids). We report a case of a woman with retroperitoneal fibrosis associated with aortic inflammatory aneurysm, who was successfully treated with oral tamoxifen.

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

    OpenAIRE

    José Carrón Sánchez; Javier Arza Porras

    2013-01-01

    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 l...

  2. Idiopathic Pulmonary Fibrosis

    Science.gov (United States)

    ... Explore Idiopathic Pulmonary Fibrosis What Is... How the Lungs Work Other Names Causes Signs & Symptoms Diagnosis Treatments Living With Clinical Trials Links Related Topics How the Lungs Work Lung Transplant Pulmonary Hypertension Pulmonary Rehabilitation Respiratory Failure ...

  3. ROCKing pulmonary fibrosis

    OpenAIRE

    Sheppard, Dean

    2013-01-01

    Clinical vignette: A 76-year-old man consults you for increasing shortness of breath over the past two years and an increasing requirement for home oxygen. A video-assisted thoracoscopic lung biopsy shows findings of usual interstitial pneumonitis, and he has no identifiable cause for pulmonary fibrosis, so he is considered to have idiopathic pulmonary fibrosis (IPF). His diffusing capacity for carbon monoxide (DLCO) is 45% of predicted, and his total lung capacity is 40% of predicted. Becaus...

  4. Necesidades educativas, asistenciales y sociales especiales de los niños con enfermedades minoritarias : propuestas para una atención interdisciplinar.

    OpenAIRE

    Fernández Hawrylak, María; Grau Rubio, Claudia

    2014-01-01

    Los recientes estudios sobre el colectivo del alumnado con Enfermedades Minoritarias ponen de manifiesto que tienen necesidades sanitarias, sociales y educativas, y que éstas deben ser satisfechas tanto por los correspondientes servicios sanitarios como por los servicios sociales y educativos con la finalidad de mejorar la calidad de vida de los afectados por estas enfermedades y la de sus familias. Las necesidades educativas especiales abarcan a la mayoría de las áreas curriculares y cambian...

  5. 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.

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

    OpenAIRE

    LA. Díaz Robredo; JI. Robles Sánchez

    2014-01-01

    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...

  7. 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. 

  8. 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.

  9. 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...

  10. 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...

  11. 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...

  12. Intestinal disease in cystic fibrosis.

    OpenAIRE

    Baxter, P S; Dickson, J. A.; Variend, S; Taylor, C J

    1988-01-01

    Three children with cystic fibrosis developed steatorrhoea unresponsive to changes in pancreatic supplements. The final diagnoses were chronic giardiasis, stagnant loop syndrome, and Crohn's disease. Refractory intestinal symptoms in cystic fibrosis merit further investigation.

  13. Cystic Fibrosis: Symptoms, Diagnosis, Treatment

    Science.gov (United States)

    ... please turn Javascript on. Feature: Steady Advances Against Cystic Fibrosis Symptoms, Diagnosis, Treatment Past Issues / Fall 2012 Table ... both of the baby's CFTR genes are normal. Cystic Fibrosis Carrier Testing People who have one normal and ...

  14. Sinonasal Manifestations in Cystic Fibrosis

    OpenAIRE

    Oomen, Karin P. Q.; Max M. April

    2012-01-01

    Cystic fibrosis is a genetic disease, characterized by accumulation of thickened mucous secretions in exocrine glands. Although the major clinical manifestations of the disease are pancreatic and pulmonary disease, the majority of cystic fibrosis patients will develop sinonasal manifestations as well. This paper outlines the etiology, evaluation, and management of the nasal and sinus manifestations in patients with cystic fibrosis.

  15. 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

  16. Nephrogenic systemic fibrosis

    OpenAIRE

    Braverman, Irwin M.; Cowper, Shawn

    2010-01-01

    Nephrogenic systemic fibrosis, initially called nephrogenic fibrosing dermopathy, has been strongly linked to exposure to gadolinium-based contrast media used in magnetic resonance imaging in patients with renal insufficiency. This review discusses recent advances in our understanding of the pathophysiology and clinical approach to patients with chronic kidney disease who require diagnostic imaging with gadolinium-based contrast media.

  17. 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...

  18. Nephrogenic systemic fibrosis

    DEFF Research Database (Denmark)

    Marckmann, Peter; Skov, Lone; Rossen, Kristian;

    2006-01-01

    Nephrogenic systemic fibrosis is a new, rare disease of unknown cause that affects patients with renal failure. Single cases led to the suspicion of a causative role of gadodiamide that is used for magnetic resonance imaging. This study therefore reviewed all of the authors' confirmed cases of ne...

  19. 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.

  20. Arthritis in cystic fibrosis.

    OpenAIRE

    Schidlow, D V; Goldsmith, D P; Palmer, J; Huang, N N

    1984-01-01

    We have confirmed previous observations of a transient, non-disabling recurrent arthritis in patients with cystic fibrosis. This arthritis differs from classic rheumatoid arthritis, is frequently associated with skin arthritis lesions, and its occurrence is unrelated to the severity of lung disease.

  1. 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.

  2. 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.

  3. 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

  4. 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.

  5. 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.

  6. 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.

  7. New Mechanisms of Pulmonary Fibrosis

    OpenAIRE

    Strieter, Robert M.; Mehrad, Borna

    2009-01-01

    The understanding of the pathogenesis of pulmonary fibrosis continues to evolve. The initial hypothetical model suggested chronic inflammation as the cause of pulmonary fibrosis, whereas a subsequent hypothesis posited epithelial injury and impaired wound repair as the etiology of fibrosis without preceding inflammation. Over the past decade, several concepts have led to refinement of these hypotheses. These include the following: (1) the importance of the integrity of the alveolar-capillary ...

  8. Eosinophilic activation in cystic fibrosis.

    OpenAIRE

    Koller, D. Y.; Götz, M.; Eichler, I; Urbanek, R

    1994-01-01

    BACKGROUND--The neutrophil is a potent contributor to pulmonary destruction in cystic fibrosis. Since eosinophils also possess destructive potential the involvement of eosinophils in cystic fibrosis has been investigated. METHODS--Eosinophil numbers and levels of eosinophil cationic protein (ECP), a marker of eosinophil activation, were determined in the serum of 42 patients with cystic fibrosis and in the sputum of 10 of them. To determine neutrophil activation levels of myeloperoxidase (MPO...

  9. 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.

  10. 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.

  11. Reserva cognitiva y déficit en la planificación en pacientes con esclerosis múltiple.

    OpenAIRE

    Lilia Mestas; Judith Salvador; Fernando Gordillo

    2012-01-01

    La esclerosis múltiple afecta el sistema nervioso produciendo alteraciones cognitivas. La memoria es uno de los más afectados, no obstante la naturaleza ó factor primario de alteración aún no se clarifica. Objetivo: Estudia las diferencias en el procesamiento de la información verbal en un grupo de pacientes con esta patologíaa neurológica respecto a un grupo de control sano. Para ello se analizó el desempeño neuropsicológico de 16 pacientes con esclerosis múltiple de tipo remitente-...

  12. Glosectomía parcial reconstruida con colgajo libre del radial. Propuesta de tratamiento fisioterápico

    OpenAIRE

    Almeida Santamaría, Mercedes

    2013-01-01

    El carcinoma escamoso de la lengua en estadio avanzado que es tratado quirúrgicamente con glosectomía reconstruida con colgajo libre del radial, resección de ganglios linfáticos afectados y radioterapia va a ocasionar secuelas en diferentes áreas corporales: lengua, columna cervical y miembro superior, afectándose el recorrido articular, la fuerza muscular, la sensibilidad táctil, la flexibilidad de los tejidos blandos y el drenaje linfático. El planteamiento de un tratamiento fisioterápico b...

  13. 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...

  14. Imaging from cystic fibrosis

    International Nuclear Information System (INIS)

    Cystic fibrosis (CF) is the most frequent metabolic disorder with autosomal recessive inheritance in the Caucasian population. The gene defect is located on the long arm of chromosome 7. In Germany today, the actual median survival is 37 years. The genetic defect caused by chloride anion disturbances affects multiple body systems but the morbidity and mortality is due to lung disease. The secretion of highly viscous mucus promotes viral and bacterial pulmonary infections leading to airway obstruction and consecutive destruction of the lung parenchyma. This article will review and discuss both the clinical aspects of the disease and the diagnostic methods, referring in particular to new imaging strategies. (orig.)

  15. Cystic Fibrosis: Diet and Nutrition

    Science.gov (United States)

    ... a Friend Who Cuts? Cystic Fibrosis: Diet and Nutrition KidsHealth > For Teens > Cystic Fibrosis: Diet and Nutrition Print A A A Text Size What's in ... or the flu. With the right balance of nutrition, extra fat and calories , and prescribed supplements, though, ...

  16. 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

  17. 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.

  18. 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

  19. 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...

  20. 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.

  1. 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.

  2. [Rhinosinusitis in cystic fibrosis].

    Science.gov (United States)

    Mainz, J G; Gerber, A; Arnold, C; Baumann, J; Baumann, I; Koitschev, A

    2015-11-01

    In cystic fibrosis (CF) mucociliary clearance of the entire respiratory system is impaired. This allows pathogens, such as Pseudomonas aeruginosa to persist and proliferate, which by progressive pulmonary destruction causes 90 % of premature deaths due to this inherited disease. The dramatic improvement in life expectation of patients due to intensive therapy has resulted in the inevitable but variably expressed sinonasal involvement coming into the clinical and scientific focus. Thereby, almost all CF patients reveal sinonasal pathology and many suffer from chronic rhinosinusitis. Recently, the sinonasal niche has been recognized as a site of initial and persistent colonization by pathogens. This article presents the pathophysiological background of this multiorgan disease as well as general diagnostic and therapeutic standards. The focus of this article is on sinonasal involvement and conservative and surgical options for treatment. Prevention of pathogen acquisition is an essential issue in the otorhinolaryngological treatment of CF patients. PMID:26495450

  3. 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.

  4. Proapoptotic Bid is required for pulmonary fibrosis

    OpenAIRE

    Budinger, G.R. Scott; Mutlu, Gökhan M.; Eisenbart, James; Fuller, Alyson C.; Bellmeyer, Amy A.; Baker, Christina M.; Wilson, Mindy; Ridge, Karen; Terrence A. Barrett; Lee, Vivian Y.; Chandel, Navdeep S.

    2006-01-01

    The molecular mechanisms of pulmonary fibrosis are poorly understood. Previous reports indicate that activation of TGF-β1 is essential for the development of pulmonary fibrosis. Here, we report that the proapoptotic Bcl-2 family member Bid is required for the development of pulmonary fibrosis after the intratracheal instillation of bleomycin. Mice lacking Bid exhibited significantly less pulmonary fibrosis in response to bleomycin compared with WT mice. The attenuation in pulmonary fibrosis w...

  5. 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.

  6. 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.

  7. Steady Advances Against Cystic Fibrosis

    Science.gov (United States)

    ... age 2, he grew up playing everything from football and lacrosse to ice hockey and golf. And ... Who's at Risk? Cystic fibrosis affects males and females from all racial and ethnic groups. It is ...

  8. Cystic fibrosis in premature infants

    OpenAIRE

    Lu, KD; Engmann, C.; Moya, F. (Francina); Muhlebach, M

    2011-01-01

    There are few reports of cystic fibrosis (CF) diagnosed in premature infants. We describe the clinical course of three patients, from our neonatal intensive care units, who were diagnosed with CF, and discuss the existing literature and treatment considerations.

  9. Nutritional management of cystic fibrosis.

    OpenAIRE

    A MacDonald

    1996-01-01

    Nutritional support is an integral part of the management of cystic fibrosis patients. It is arguably best provided by a qualified dietitian and nutritional care sister working in conjunction with the rest of the cystic fibrosis team. The patient's nutritional needs should be assessed, regularly reviewed, and nutritional treatment tailored to meet the changing clinical and psychosocial needs of the patient. Nutritional intervention is not without complications, and in particular attention to ...

  10. Fibrosis quística

    OpenAIRE

    Arturo Solís-Moya; José Pablo Gutiérrez-S

    2003-01-01

    La Fibrosis quística es una enfermedad multisistémica que genera la formación y acumulación de moco viscoso que afecta sobre todo a pulmones, aparato digestivo incluyendo páncreas e hígado. Anteriormente se la conocía como mucoviscidosis o fibrosis quística del páncreas.

  11. Estudio de caso: adaptaciones de acceso al currículum en un alumno de educación infantil con parálisis cerebral.

    OpenAIRE

    Tárraga Mínguez, Raúl

    2012-01-01

    Se presenta el contenido de una intervención con un alumno de educación infantil afectado de parálisis cerebral. La intervención se basa en una adaptación de acceso al currículum consistente en el diseño de tareas y actividades educativas con soporte informático accesible al alumno y adecuadas al nivel curricular propio para su edad y curso académico. Esta metodología garantiza al alumno un acceso al currículum minimizando los efectos de sus problemas de movilidad. This paper exposes the c...

  12. Presentación clínica de la Trisomía 13 Mosaico con mayor pronóstico de vida. Reporte de un caso.

    OpenAIRE

    Gabriel Abudinén A.; Alejandra Vergara V.; Alex Castet A.; Gabriela Flores F.; Ignacio Cabrera-Samith

    2013-01-01

    INTRODUCCIÓN: La trisomía del cromosoma 13, antes llamado Síndrome de Patau, es una enfermedad genética que resulta de la presencia de un cromosoma 13 supernumerario. Fue descubierta en 1960 por el Dr. Klaus Patau y actualmente es la trisomía reportada menos frecuente en la especie humana. Se suele asociar con un problema meiótico materno más que paterno y, como el síndrome de Down, el riesgo aumenta con la edad de la mujer. Los afectados mueren poco tiempo después de nacer, la mayoría a los ...

  13. 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

  14. 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

  15. 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.

  16. Mucolytics in cystic fibrosis.

    Science.gov (United States)

    Henke, Markus O; Ratjen, Felix

    2007-03-01

    Mucus accumulation in the lower airways is a key feature of cystic fibrosis (CF) lung disease. The major component of mucus in CF is not mucin derived from mucus producing cells but rather pus that includes viscous material such as polymerized DNA derived from degraded neutrophils. This has important implications for mucolytic therapy aiming to improve mucus clearance from the airways, since degradation of mucin may not be a suitable treatment strategy. In addition, thinning of secretions may not always be beneficial, since it may negatively affect certain aspects of mucus transport such as cough clearance. While inhaled N-acetylcysteine has been used as a mucolytic drug in CF for decades, there is little evidence that it has any beneficial effect. Dornase alfa has been shown to reduce pulmonary exacerbations and improve lung function and is currently the only mucolytic agent with proven efficacy in CF. Newer agents targeting other components of CF mucus, such as filamentous actin, are currently in development. Ultimately, drugs that are mucokinetic, which preserve viscoelasticity, rather than mucolytic may prove to be beneficial for CF lung disease in the future. PMID:17419975

  17. 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 ...

  18. El ejercicio como tratamiento de pacientes con claudicación intermitente de los miembros inferiores de origen vascular

    OpenAIRE

    Alberto Domenech

    2007-01-01

    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 claud...

  19. 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

  20. 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.

  1. 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

  2. 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.

  3. Cystic Fibrosis (CF) Respiratory Screen: Sputum

    Science.gov (United States)

    ... Tropical Delight: Melon Smoothie Pregnant? Your Baby's Growth Cystic Fibrosis (CF) Respiratory Screen: Sputum KidsHealth > For Parents > Cystic Fibrosis (CF) Respiratory Screen: Sputum Print A A A ...

  4. Genetics Home Reference: idiopathic pulmonary fibrosis

    Science.gov (United States)

    ... idiopathic pulmonary fibrosis. N Engl J Med. 2007 Mar 29;356(13):1317-26. Citation on PubMed ... PJ, Garcia CK. Effect of telomere length on survival in patients with idiopathic pulmonary fibrosis: an observational ...

  5. Computed tomography of cystic pancreatic fibrosis

    International Nuclear Information System (INIS)

    The computer tomographic appearances of atrophic and lipomatous degeneration of the pancreas in cystic pancreatic fibrosis are described. CT exploration of the pancreas in recommended, particularly in differential diagnostic aspects of cystic fibrosis. (orig.)

  6. Cystic Fibrosis: Prenatal Screening and Diagnosis

    Science.gov (United States)

    ... Management Education & Events Advocacy For Patients About ACOG Cystic Fibrosis: Prenatal Screening and Diagnosis Home For Patients Search ... Screening and Diagnosis FAQ171, February 2016 PDF Format Cystic Fibrosis: Prenatal Screening and Diagnosis Pregnancy What is cystic ...

  7. Serum pancreatic lipase activity in cystic fibrosis.

    OpenAIRE

    Junglee, D; Penketh, A; Katrak, A; Hodson, M.E.; Batten, J C; Dandona, P

    1983-01-01

    Patients with cystic fibrosis have been found to have abnormal serum concentrations of immunoreactive trypsin and abnormal activities of pancreatic isoamylase. A study was undertaken to discover whether activity of pancreatic lipase is also altered in cystic fibrosis. Serum from 23 patients with cystic fibrosis was assayed for immunoreactive trypsin and pancreatic lipase. Median serum pancreatic lipase activity was significantly lower in patients with cystic fibrosis than in controls, as was ...

  8. Monocyte and macrophage regulation of pulmonary fibrosis

    OpenAIRE

    Gibbons, Michael A.

    2010-01-01

    In this thesis I examined the role of circulating monocytes and lung macrophages in the pathogenesis of the early fibrotic, progressive fibrotic and resolution phases of pulmonary fibrosis. Pulmonary fibrosis with destruction of lung architecture and consequent respiratory failure and death represents a massive worldwide health burden. Although idiopathic pulmonary fibrosis (IPF) is the archetypal and most common cause of lung fibrosis, numerous respiratory diseases can prog...

  9. 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...

  10. Diagnostic Testing in Cystic Fibrosis.

    Science.gov (United States)

    Brewington, John; Clancy, J P

    2016-03-01

    Cystic Fibrosis (CF) is a rare, multisystem disease leading to significant morbidity and mortality. CF is caused by defects in the cystic fibrosis transmembrane conductance regulator protein (CFTR), a chloride and bicarbonate transporter. Early diagnosis and access to therapies provides benefits in nutrition, pulmonary health, and cognitive ability. Several screening and diagnostic tests are available to support a diagnosis. We discuss the characteristics of screening and diagnostic tests for CF and guideline-based algorithms using these tools to establish a diagnosis. We discuss classification and management of common "diagnostic dilemmas," including the CFTR-related metabolic syndrome and other CFTR-associated diseases. PMID:26857766

  11. Aplicación de Tecnosoles para la recuperación de suelos y aguas afectados por actividades de obras civiles, urbanas y minería

    OpenAIRE

    Bolaños Guerrón, Darío

    2015-01-01

    La presente tesis doctoral está desarrollada con proyectos de campo, estudios de laboratorio y análisis de proyectos de obra civil, los cuales tienen en común el diseño, formulación, elaboración y uso de Tecnosoles como herramienta de recuperación o rehabilitación de suelos y aguas contaminados o degradados. En base a los resultados de la aplicación de Tecnosoles para la restauración de la Mina de Touro, ejecutada durante varios años bajo la dirección de Felipe Macías y e...

  12. 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

  13. 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.

  14. 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.

  15. 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...

  16. 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

  17. 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

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

    OpenAIRE

    Gabriel Olveira; Casilda Olveira

    2008-01-01

    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 perce...

  19. Oesophageal subepithelial fibrosis: an extension of oral submucosal fibrosis.

    OpenAIRE

    Misra, S. P.; Misra, V; Dwivedi, M.; Gupta, S. C.

    1998-01-01

    Fifty-five patients with oral submucosal fibrosis and an equal number of patients with no evidence of the disease were studied. All patients underwent upper gastrointestinal endoscopy and any abnormality was noted. Multiple oesophageal biopsies were obtained from the upper end of the oesophagus and from any endoscopically observed abnormality. The histological changes in the two groups were assessed blindly by an experienced histopathologist. Histological abnormalities were noted in the oesop...

  20. [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).

  1. 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.

  2. Non-cystic fibrosis bronchiectasis.

    Science.gov (United States)

    Al Subie, Haya; Fitzgerald, Dominic A

    2012-05-01

    Non-cystic fibrosis (CF) bronchiectasis is said to be a declining problem in developed countries, although its prevalence in indigenous communities in Australia and New Zealand is among the highest reported in the world. Early childhood pneumonia and underlying conditions such as immunodeficiency and primary ciliary dyskinesia need to be considered in the aetiology. A high-resolution computerised tomography scan is the key investigation in children with a chronic wet cough in whom bronchiectasis is suspected. Regardless of the cause, the treatment of bronchiectasis is centred upon facilitating the clearance of airway secretions and the treatment of pulmonary exacerbations. This review aims to provide general paediatricians with an update on the presenting features, investigation and management of non-cystic fibrosis bronchiectasis. PMID:21040075

  3. [Cystic fibrosis and associated complications].

    Science.gov (United States)

    Schwarz, C; Staab, D

    2015-03-01

    Cystic fibrosis (CF) is an autosomal recessive inherited metabolic disease. The mutation is located on the long arm of chromosome 7. Due to a defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, chloride ion transport is reduced across the cell membrane. As a result, the disease can be described as an exocrinopathy. In all organs with exocrine glands, disorders occur in association with the defective chloride transport. The main impact of this defect is manifested in the lungs. Therefore, the most common cause of death is pulmonary disease with respiratory insufficiency due to recurrent infections. Unfortunately, a cure for the disease is still not available. However, new therapies that may affect the CFTR mutation more specifically give new hope for better therapeutic options in the future. The long-term goal of therapy is to develop a causal therapy for all six different mutation classes and thus for about 2000 mutations. PMID:25693903

  4. MRI in mucoviscidosis (cystic fibrosis)

    International Nuclear Information System (INIS)

    Cystic fibrosis (CF) is a multi-systemic disease with major impact on the lungs. Pulmonary manifestation is crucial for the prognosis and life expectancy of patients. Imaging modalities and lung function tests reflect the pulmonary status in these patients. The standard imaging modality for diagnosis and follow-up of pulmonary changes is chest x-ray. The gold standard for the detection of parenchymal lung changes remains high resolution computed tomography (HRCT), but this is not used routinely for CF-patients due to radiation exposure. Magnetic resonance imaging (MRI) used to be of no importance in monitoring cystic fibrosis lung disease, as shown in studies from the 1980s and early 1990s. The continuing improvement of MRI techniques, however, has allowed for an adequate application of this non-radiation method in diagnosing the major pulmonary findings in CF, in addition to the assessment of lung function. (orig.)

  5. Pharmacogenetics of cystic fibrosis treatment.

    Science.gov (United States)

    Carter, Suzanne C; McKone, Edward F

    2016-08-01

    Cystic fibrosis (CF) is genetic autosomal recessive disease caused by reduced or absent function of CFTR protein. Treatments for patients with CF have primarily focused on the downstream end-organ consequences of defective CFTR. Since the discovery of the CFTR gene that causes CF in 1989 there have been tremendous advances in our understanding of the genetics and pathophysiology of CF. This has recently led to the development of new CFTR mutation-specific targeted therapies for select patients with CF. This review will discuss the characteristics of the CFTR gene, the CFTR mutations that cause CF and the new mutation specific pharmacological treatments including gene therapy that are contributing to the dawning of a new era in cystic fibrosis care. PMID:27490265

  6. New therapies for hepatic fibrosis.

    Science.gov (United States)

    Koyama, Yukinori; Brenner, David A

    2015-09-01

    Liver fibrosis is an outcome of many chronic diseases, and often results in cirrhosis, liver failure, and portal hypertension. Liver transplantation is the only treatment available for patients with advanced stages of liver cirrhosis. Therefore, alternative methods are required to develop new strategies for anti-fibrotic therapy. Various kinds of hepatocyte injuries cause inflammatory reactions, which lead to activation of hepatic stellate cells (HSCs). Continuous liver injuries maintain these activated HSCs, and they are called as myofibroblasts. Myofibroblasts proliferate in response to various kinds of cytokines and produce extracellular matrix proteins (ECMs). Myofibroblasts undergo apoptosis and inactivation when the underlying causative etiologies are cleared. Here, we describe the current knowledge of targeting the activated HSCs as a therapeutic target for liver fibrosis. PMID:26206573

  7. Oral submucous fibrosis: an update

    OpenAIRE

    Wollina U; Verma SB; Ali FM; Patil K

    2015-01-01

    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 ca...

  8. Dendritic Cells and Liver Fibrosis

    OpenAIRE

    Rahman, Adeeb H.; Aloman, Costica

    2013-01-01

    Dendritic cells are a relative rare population of specialized antigen presenting cells that are distributed through most lymphoid and non-lymphoid tissues and play a critical role in linking the innate and adaptive arms of the immune system. The liver contains a heterogeneous population of dendritic cells that may contribute to liver inflammation and fibrosis through a number of mechanisms. This review summarizes current knowledge on the development and characterization of liver dendritic cel...

  9. 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.

  10. Finger clubbing in cystic fibrosis.

    OpenAIRE

    Pitts-Tucker, T J; Miller, M G; Littlewood, J M

    1986-01-01

    Finger clubbing was measured in 73 of 105 patients with cystic fibrosis undergoing full assessment. The sign correlated well with the chest x ray score and indices of pulmonary function and infection but not with weight, height, age, liver function, or degree of fat malabsorption. The presence of clubbing suggests appreciable pulmonary involvement. Most probably its progression indicates a deterioration in pulmonary state. In both instances increased efforts should be made to treat the infect...

  11. A case of cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Maria Alice Monti

    2009-09-01

    Full Text Available As the expected survival improves for patients with cystic fibrosis (CF, there is a growing population of adults with this disease. We describe a case of a 33-year-old woman with CF presenting with recurrent pancreatitis, malnutrition, borderline sweat test and respiratory diseases. The case report underlines the importance of diagnosis and management of CF in adults, and the important role played by the Family Physician in developing an adult care program.

  12. 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

  13. Hepatic fibrosis in Kabuki syndrome.

    Science.gov (United States)

    Nobili, Valerio; Marcellini, Matilde; Devito, Rita; Capolino, Rossella; Viola, Laura; Digilio, M Cristina

    2004-01-15

    Kabuki (Niikawa-Kuroki) syndrome (KS) is characterized by a distinctive face, mental retardation, growth deficiency, skeletal anomalies, dermatoglyphic abnormalities, palatal anomalies, congenital heart defects, and urogenital malformations. Congenital hepatic abnormalities have been sporadically described in patients with KS from the literature, consisting of extrahepatic biliary atresia, neonatal sclerosing cholangitis, and severe neonatal jaundice. We report here on an additional patient with a congenital abnormality of the liver consisting of hepatic fibrosis. To our knowledge, idiopathic congenital hepatic fibrosis has not been reported in KS. Thus, our observation expands the spectrum of liver malformations found in KS with the inclusion of hepatic fibrosis and supports the evidence that hepatic abnormalities may not be uncommon in KS. Clinician should be advised to search for the specific facial anomalies of KS in patients with syndromic congenital hepatic diseases, and KS should be added to the list of previously recognized multiple congenital anomaly syndromes with hepatic involvement. Due to the frequent association with congenital heart malformations, KS should be considered in the evaluation of patients with neonatal liver disease and cardiac malformation. Due to the expression patterns of Notch genes, involvement of the Notch signaling pathway in the development of heart and liver anomalies in KS should be considered. PMID:14699623

  14. Pathogenic mechanism in lung fibrosis

    International Nuclear Information System (INIS)

    The purpose of the study was to examine whether an interaction between two agents causing alveolar epithelial damage would produce lung fibrosis. In mouse lung, intraperitoneal injection of the antioxidant butylated hydroxytoluene causes diffuse alveolar type I cell necrosis, followed by proliferation of type II alveolar cells. In animals exposed to 70% O2 or 100-200 rad x rays during the phase of type II cell proliferation following BHT, diffuse interstitial lung fibrosis developed within 2 weeks. Quantitative analysis of the lungs for hydroxyproline showed that the interaction between BHT and O2 or x rays was synergistic. If exposure to O2 or x rays was delayed until epithelial recovery was complete, no fibrosis was seen. Abnormally high levels of lung collagen persisted up to 6 months after one single treatment with BHT and 100 rad x rays. A commonly seen form of chronic lung damage may thus be caused by an acute interaction between a bloodborne agent which damages the alveolar cell and a toxic inhalant or x rays, provided a critically ordered sequence of exposure is observed

  15. 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.

  16. Transient elastography for liver fibrosis diagnosis

    DEFF Research Database (Denmark)

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

    2009-01-01

    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...... 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...

  17. 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...... 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...

  18. 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.

  19. Secondary Retroperitoneal Fibrosis Associated with Generalized Atherosclerosis

    OpenAIRE

    Barbullushi Myftar; Pasko Nevi; Bezhani Edip; Duraku Ahmet; Rusi Reza; Hoti Klit; Bakalli Vaso; Idrizi Alma

    1999-01-01

    Retroperitoneal fibrosis is an uncommon disease that often presents in a subtle manner. Only a few cases of the combined association of generalized atherosclerosis and retroperitoneal fibrosis are reported in the recent literature, supporting the view that the condition is probably an autoimmune periaortitis. We describe a typical case of retroperitoneal fibrosis associated with generalized atherosclerosis with clinical presentation of progressive renal insufficiency, and claudication from ar...

  20. Secondary Retroperitoneal Fibrosis Associated with Generalized Atherosclerosis

    Directory of Open Access Journals (Sweden)

    Barbullushi Myftar

    1999-01-01

    Full Text Available Retroperitoneal fibrosis is an uncommon disease that often presents in a subtle manner. Only a few cases of the combined association of generalized atherosclerosis and retroperitoneal fibrosis are reported in the recent literature, supporting the view that the condition is probably an autoimmune periaortitis. We describe a typical case of retroperitoneal fibrosis associated with generalized atherosclerosis with clinical presentation of progressive renal insufficiency, and claudication from arterial compromise.

  1. Viral infection drives tissue fibrosis in vitro

    OpenAIRE

    Malizia, Andrea P; Dermot Walls; Egan, Jim J; Doran, Peter P

    2008-01-01

    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 an...

  2. BIOCONJUGATION OF OLIGONUCLEOTIDES FOR TREATING LIVER FIBROSIS

    OpenAIRE

    Ye, Zhaoyang; Hajj Houssein, Houssam S.; Mahato, Ram I.

    2007-01-01

    Liver fibrosis results from chronic liver injury due to hepatitis B and C, excessive alcohol ingestion, and metal ion overload. Fibrosis culminates in cirrhosis and results in liver failure. Therefore, a potent antifibrotic therapy is in urgent need to reverse scarring and eliminate progression to cirrhosis. Although activated hepatic stellate cells (HSCs) remains the principle cell type responsible for liver fibrosis, perivascular fibroblasts of portal and central veins as well as periductul...

  3. The impact of emphysema in pulmonary fibrosis

    OpenAIRE

    Vincent Cottin

    2013-01-01

    Several groups have described a syndrome in which idiopathic pulmonary fibrosis (IPF) coexists with pulmonary emphysema. This comes as no surprise since both diseases are associated with a history of exposure to cigarette smoke. The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is characterised by upper lobe emphysema and lower lobe fibrosis. Physiological testing of these patients reveals preserved lung volume indices contrasted by markedly impaired diffusion capacity. The inc...

  4. Swine flu fibrosis: Regressive or progressive?

    OpenAIRE

    Nishtha Singh; Sheetu Singh; Bharat Bhushan Sharma; Virendra Singh

    2016-01-01

    Swine flu influenza had spread the world over in 2009. The main pathology was bilateral pneumonia. Majority of these cases recovered from pneumonia fully. Though in some cases, pulmonary fibrosis was reported as a sequel. However, long-term progression of such pulmonary fibrosis is uncertain. We are hereby reporting two cases of swine flu that showed residual pulmonary fibrosis. The clinical and laboratory parameters were also recorded. In both the cases, radiological shadows and spirometric ...

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

    OpenAIRE

    Huerta Grávalos, Lucía

    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...

  6. Novel biomarkers of fibrosis in Crohn's disease.

    Science.gov (United States)

    Pellino, Gianluca; Pallante, Pierlorenzo; Selvaggi, Francesco

    2016-08-15

    Fibrosis represents a major challenge in Crohn's disease (CD), and many CD patients will develop fibrotic strictures requiring treatment throughout their lifetime. There is no drug that can reverse intestinal fibrosis, and so endoscopic balloon dilatation and surgery are the only effective treatments. Since patients may need repeated treatments, it is important to obtain the diagnosis at an early stage before strictures become symptomatic with extensive fibrosis. Several markers of fibrosis have been proposed, but most need further validation. Biomarkers can be measured either in biological samples obtained from the serum or bowel of CD patients, or using imaging tools and tests. The ideal tool should be easily obtained, cost-effective, and reliable. Even more challenging is fibrosis occurring in ulcerative colitis. Despite the important burden of intestinal fibrosis, including its detrimental effect on outcomes and quality of life in CD patients, it has received less attention than fibrosis occurring in other organs. A common mechanism that acts via a specific signaling pathway could underlie both intestinal fibrosis and cancer. A comprehensive overview of recently introduced biomarkers of fibrosis in CD is presented, along with a discussion of the controversial areas remaining in this field. PMID:27574564

  7. Swine flu fibrosis: Regressive or progressive?

    Science.gov (United States)

    Singh, Nishtha; Singh, Sheetu; Sharma, Bharat Bhushan; Singh, Virendra

    2016-01-01

    Swine flu influenza had spread the world over in 2009. The main pathology was bilateral pneumonia. Majority of these cases recovered from pneumonia fully. Though in some cases, pulmonary fibrosis was reported as a sequel. However, long-term progression of such pulmonary fibrosis is uncertain. We are hereby reporting two cases of swine flu that showed residual pulmonary fibrosis. The clinical and laboratory parameters were also recorded. In both the cases, radiological shadows and spirometric values did not show deterioration. We conclude that swine flu pulmonary fibrosis is not a progressive condition. PMID:27051116

  8. Swine flu fibrosis: Regressive or progressive?

    Directory of Open Access Journals (Sweden)

    Nishtha Singh

    2016-01-01

    Full Text Available Swine flu influenza had spread the world over in 2009. The main pathology was bilateral pneumonia. Majority of these cases recovered from pneumonia fully. Though in some cases, pulmonary fibrosis was reported as a sequel. However, long-term progression of such pulmonary fibrosis is uncertain. We are hereby reporting two cases of swine flu that showed residual pulmonary fibrosis. The clinical and laboratory parameters were also recorded. In both the cases, radiological shadows and spirometric values did not show deterioration. We conclude that swine flu pulmonary fibrosis is not a progressive condition.

  9. 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 ...

  10. Breakdown in Breathing: The Complexities of Cystic Fibrosis

    Science.gov (United States)

    ... Subscribe A Breakdown in Breathing The Complexities of Cystic Fibrosis Cystic fibrosis (CF) is an inherited illness that ravages the ... B. Guggino, a researcher with the Johns Hopkins Cystic Fibrosis Center. But in CF, “the mucus gets sticky, ...

  11. Estudio de la función telomérica y de moléculas relacionadas con invasión en tumores gástricos con y sin inestabilidad en microsatélites

    OpenAIRE

    Pascua García, Irene

    2015-01-01

    El cáncer de estómago es uno de los tipos tumorales con mayor incidencia a nivel mundial, y el tercero en número de muertes por cáncer. A nivel molecular, las principales vías carcinogénicas implicadas en el desarrollo del cáncer gástrico, la vía supresora y la vía del fenotipo mutador, dan lugar al desarrollo de tumores con distintas características clínicopatológicas que confieren un pronóstico clínico diferente a los pacientes afectados. Nuestro estudio fue realizado en una población de ...

  12. 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...

  13. 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.

  14. Pulmonary complications of cystic fibrosis

    International Nuclear Information System (INIS)

    The life expectancy of patients with cystic fibrosis (CF) has steadily increased over recent decades with a corresponding increase in the frequency of complications of the disease. Radiologists are increasingly involved with managing and identifying the pulmonary complications of CF. This article reviews the common manifestations of CF lung disease as well as updating radiologists with a number of less well-known complications of the condition. Early and accurate detection of the pulmonary effects of CF are increasingly important to prevent irreversible lung damage and give patients the greatest possibility of benefiting from the new therapies becoming available, which correct the underlying defect causing CF

  15. Liver manifestations of cystic fibrosis

    International Nuclear Information System (INIS)

    Chronic liver disease is one of the major complications of cystic fibrosis (CF). Significant liver disease is seen in 13-25% of children with CF. Improved life expectancy and prolonged follow-up have favored better characterization of the hepatic manifestations of CF and allowed direct observation of an increasing number of liver-related events. Liver disease typically develops in the first decade of life, with the incidence dropping rapidly after the age of 10 years. The wide spectrum of liver disease ranging from asymptomatic gallbladder abnormalities to biliary cirrhosis will be reviewed in this article

  16. 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. PMID:27469183

  17. The Irish cystic fibrosis database.

    OpenAIRE

    Cashman, S M; Patino, A; Delgado, M G; Byrne, L; Denham, B; de Arce, M

    1995-01-01

    We have found records of 1014 Irish cystic fibrosis patients alive by December 1994, belonging to 883 families. Prevalence in the population is 1/3475 and incidence at birth 1/1461, with a gene frequency of 2.6%. Twenty percent of the patients are aged over 20 years, but at present survival rate falls rapidly after that age. We have identified 85% of the mutations on the CFTR gene in a sample of 29% of the families (506 CF chromosomes). Mutation delta F508 is found in 72% of Irish CF chromoso...

  18. 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.

  19. 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.

  20. 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.

  1. 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...

  2. Sarcoidosis in an adult with cystic fibrosis.

    OpenAIRE

    Rettinger, S D; Trulock, E. P.; MacKay, B.; Auerbach, H S

    1989-01-01

    Sarcoidosis in an adult patient with cystic fibrosis lung disease was diagnosed on the basis of pulmonary function and radiographic data. It should be considered in the differential diagnosis of new diffuse interstitial infiltrates or hilar adenopathy in a patient with cystic fibrosis; biopsy of lung, lymph node, or skin lesions and interleukin-2 receptor levels may help to obtain a diagnosis.

  3. 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

  4. Cuidados con el anciano con tos productiva.

    OpenAIRE

    Costa de Moura, María Lucia

    2005-01-01

    A partir de las informaciones y con la motivación para hacer un estudio dirigido a los cuidados con el anciano, sigue la necesidad del desarrollo de acciones pertinentes para la práctica de cuidar, o sea, la asistencia de enfermería prestada directamente al anciano, principalmente a aquellos que llegan a la unidad de salud quejándose de tos. El objeto de este estudio es la asistencia de enfermería basándome en las cuestiones que rodean la percepción del enfermero y cómo el...

  5. Caracterización clínica de familias costarricenses con trastorno afectivo bipolar

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  7. 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.

  8. Aspergillus infections in cystic fibrosis.

    Science.gov (United States)

    King, Jill; Brunel, Shan F; Warris, Adilia

    2016-07-01

    Patients with cystic fibrosis (CF) suffer from chronic lung infection and airway inflammation. Respiratory failure secondary to chronic or recurrent infection remains the commonest cause of death and accounts for over 90% of mortality. Bacteria as Staphylococcus aureus, Pseudomonas aeruginosa and Burkholderia cepacia complex have been regarded the main CF pathogens and their role in progressive lung decline has been studied extensively. Little attention has been paid to the role of Aspergillus spp. and other filamentous fungi in the pathogenesis of non-ABPA (allergic bronchopulmonary aspergillosis) respiratory disease in CF, despite their frequent recovery in respiratory samples. It has become more apparent however, that Aspergillus spp. may play an important role in chronic lung disease in CF. Research delineating the underlying mechanisms of Aspergillus persistence and infection in the CF lung and its link to lung deterioration is lacking. This review summarizes the Aspergillus disease phenotypes observed in CF, discusses the role of CFTR (cystic fibrosis transmembrane conductance regulator)-protein in innate immune responses and new treatment modalities. PMID:27177733

  9. MR evaluation of retroperitoneal fibrosis

    Energy Technology Data Exchange (ETDEWEB)

    Kamper, L.; Scharwaechter, C.; Haage, P.; Piroth, W. [HELIOS-KLINIK Wuppertal - Klinikum der Privaten Univ. Witten/Herdecke (Germany). Klinik fuer Diagnostische und Interventionelle Radiologie; Brandt, A.S.; Kukuk, S.; Roth, S. [HELIOS-KLINIK Wuppertal - Klinikum der Privaten Univ. Witten/Herdecke (Germany). Klinik fuer Urologie

    2011-08-15

    Purpose: Retroperitoneal fibrosis (RPF) is a rare fibrosing process. The relation of contrast enhancement within the fibrosis and the psoas muscle was suggested for the evaluation of disease activity and response to treatment, but no standardized method for the quantification of the disease activity is currently available. We introduce an MR parameter for the evaluation of RPF. Materials and Methods: 29 patients with untreated idiopathic RPF were examined before medical therapy and after three months of treatment. MR analyses included quotients of T 1 and T 2-signal intensities between the RPF and the psoas muscle. Furthermore, we examined the dynamic contrast enhancement of the fibrous tissue. The respective data were compared with the extent of the RPF after 3 months. Results: In the follow-up examinations we observed a significant regression in 37.9 % (n = 11) of cases, a mild regression in 48.3 % (n = 14) and a stable extent in 13.8 % (n = 4). Patients with a significantly reduced extent showed the highest mean values for the applied MR quotients in the initial examination. The lowest mean values for the respective parameters were found for the group with a stable extent. Only dynamic enhancement quotients showed statistically significant differences (p = 0.011) between the groups of response. Conclusion: We observed a significant reduction in the fibrous tissue after pharmacological treatment in patients with an elevated dynamic enhancement quotient in the initial examination. (orig.)

  10. 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.

  11. Correlation between TIMP-1 expression and liver fibrosis in two rat liver fibrosis models

    Institute of Scientific and Technical Information of China (English)

    Qing-He Nie; Ya-Fei Zhang; Yu-Mei Xie; Xin-Dong Luo; Bin Shao; Jun Li; Yong-Xing Zhou

    2006-01-01

    AIM: To evaluate serum TIMP-1 level and the correlation between TIMP-1 expression and liver fibrosis in immuneinduced and CCL4-induced liver fibrosis models in rats.METHODS: Immune-induced and CCL4-induced liver fibrosis models were established by dexamethasone (0.01 mg) and CCL4 respectively. Serum TIMP-1 level was detected with ELISA, while histopathological grade of liver biopsy was evaluated. Spearman rankcorrelation test was used to analyse the difference of the correlation between the TIMP-1 expression and hepatic fibrosis in the two fibrosis models. Furthermore,in situ hybridization was used to determine the expression difference of TIMP-1 mRNA in the two models.RESULTS: Positive correlation existed between serum TIMP-1 level of immune induced group and the histopathological stages of fibrosis liver of corresponding rats (Spearman rank-correlation test, rs = 0.812, P < 0.05), and the positivein situ hybridization signal of TIMP-1 mRNA was strong. In CCL4-induced liver fibrosis model, the correlation between the serum TIMP-1 level and the severity of hepatic fibrosis was not statistically significant(Spearman rank-correlation test, rs = 0.229, P > 0.05). And compared with immune-induced model,the positive in situ hybridization signal of TIMP-1 mRNA was weaker, while the expression variation was higher in hepatic fibrosis of the same severity.CONCLUSION: The correlations between TIMP-1 expression and liver fibrosis in two rat liver fibrosis models are different. In immune-induced model, serum TIMP-1 level could reflect the severity of liver fibrosis,while in CCL4-induced model, the correlation between the serum TIMP-1 level and the severity of hepatic fibrosis was not statistically significant.

  12. 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

  13. 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.

  14. 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.

  15. 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.

  16. 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.

  17. 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...

  18. Pulmonary changes in cystic fibrosis

    International Nuclear Information System (INIS)

    Pulmonary abnormalities in cystic fibrosis result from the obstruction of small bronchi by highly viscous mucus. Chronic obstructive lung disease and recurrent pulmonary infections result in a typical radiographic pattern later in the disease. Most patients can now be expected to survive into adulthood. The radiologist must make a careful comparison of serial films in order to detect complications early. By far the most important imaging modality is the conventional chest radiograph. CT is more sensitive for detection of structural abnormalities of the lung. Bronchography is a dangerous procedure and can lead to rapid deterioration of lung function. Lung scanning is a very sensitive method for demonstrating regional disturbances of ventilation and may reveal abnormalities earlier than conventional radiographs. In severe hemoptysis, selective bronchial arteriography with embolization of the bleeding vessel can be a life-saving procedure. (orig.)

  19. Cough in idiopathic pulmonary fibrosis.

    Science.gov (United States)

    van Manen, Mirjam J G; Birring, Surinder S; Vancheri, Carlo; Cottin, Vincent; Renzoni, Elisabetta A; Russell, Anne-Marie; Wijsenbeek, Marlies S

    2016-09-01

    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. PMID:27581827

  20. 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.

  1. Effects of inflammation and fibrosis on pulmonary function in diffuse lung fibrosis.

    OpenAIRE

    Chinet, T; Jaubert, F; Dusser, D.; Danel, C.; Chrétien, J.; Huchon, G J

    1990-01-01

    To investigate the relation between lung function and inflammation and fibrosis in patients with diffuse lung fibrosis, a study was made of untreated patients without appreciable airway obstruction (14 patients with cryptogenic fibrosing alveolitis and seven with pneumoconiosis). Quantitative assessment of inflammatory infiltration and fibrosis was carried out on open lung biopsy specimens and compared with lung volumes, carbon monoxide transfer factor (TLCO), TLCO corrected for alveolar volu...

  2. Pulmonary Function Measures Predict Mortality Differently in Idiopathic Pulmonary Fibrosis versus Combined Pulmonary Fibrosis and Emphysema

    OpenAIRE

    Schmidt, Shelley L.; Nambiar, Anoop M.; Tayob, Nabihah; Sundaram, Baskaran; Han, MeiLan K.; Gross, Barry H.; Kazerooni, Ella A.; Chughtai, Aamer R.; Lagstein, Amir; Myers, Jeffrey L.; Murray, Susan; Toews, Galen B.; Martinez, Fernando J; Flaherty, Kevin R.

    2010-01-01

    The composite physiologic index(CPI) was derived to represent the extent of fibrosis on high resolution computed tomography, adjusting for emphysema in patients with idiopathic pulmonary fibrosis(IPF). We hypothesized longitudinal change in CPI would better predict mortality than forced expiratory volume in 1 second(FEV1), forced vital capacity(FVC), or diffusing capacity for carbon monoxide(DLCO) in all patients with IPF, and especially in those with combined pulmonary fibrosis and emphysema...

  3. 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.

  4. "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. PMID:19170216

  5. Noninvasive Markers to Assess Liver Fibrosis.

    Science.gov (United States)

    Czul, Frank; Bhamidimarri, Kalyan R

    2016-07-01

    Chronic liver disease represents a major public health problem, accounting for significant morbidity and mortality worldwide. Their prognosis and management greatly depends on the amount and progression of liver fibrosis with time and the risk of development of cirrhosis. Historically, liver biopsy was considered to be the gold standard for the detection of fibrosis. Nevertheless, liver biopsy is an invasive procedure that has limitations in terms of patient acceptance, risk-benefit ratio, cost-effectiveness, and its availability in various geographic regions. Moreover, it is a questionable gold standard due to significant sampling error and intraobserver and interobserver variability. These limitations have led to the development of noninvasive techniques for assessing the presence and the degree of liver fibrosis. This review aims to revise the most recent data from the literature about noninvasive methods useful in the evaluation of liver fibrosis. PMID:27105176

  6. 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.

  7. 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

  8. Immunoreactive trypsin and neonatalscreening for cystic fibrosis

    International Nuclear Information System (INIS)

    Immunoreactive trypsin (IRT) was measured in dried blood spots from 160.822 five-day-old babies as a part of a regionwide neonatal screening program for cystic fibrosis. A second test was performed for 492 babies in whom blood IRT levels were found greater than 900 μg/l; retesting revealed persistent elevation in 55. Sweat testing confirmed cystic fibrosis in 43 babies, but results were normal in 12. During the course of this study, a total of 51 cystic fibrosis babies were identified: 43 by newborn screening, 6 because they had meconium ileus; so, early diagnosis was achieved in 49 cases out of 51. Two newborn babies did not have elevated IRT and they were missed by the screening test. Our results confirm that elevated blood IRT is characteristic of newborn babies with cystic fibrosis and show that this test has an excellent specificity (99.7%) and a good sensitivity (95%) when used as a neonatal screening test

  9. Role of NADPH Oxidases in Liver Fibrosis

    OpenAIRE

    Paik, Yong-Han; Kim, Jonghwa; Aoyama, Tomonori; De Minicis, Samuele; Bataller, Ramon; Brenner, David A

    2014-01-01

    Significance: Hepatic fibrosis is the common pathophysiologic process resulting from chronic liver injury, characterized by the accumulation of an excessive extracellular matrix. Multiple lines of evidence indicate that oxidative stress plays a pivotal role in the pathogenesis of liver fibrosis. Nicotinamide adenine dinucleotide phosphate (NADPH) oxidase (NOX) is a multicomponent enzyme complex that generates reactive oxygen species (ROS) in response to a wide range of stimuli. In addition to...

  10. Communicating with young adults with cystic fibrosis.

    OpenAIRE

    Webb, A. K.

    1995-01-01

    The care of the young adult with cystic fibrosis is complex, requiring a multidisciplinary input from different carers. Communication with and education of patients covers many areas; topics may include medical and personal problems, transplantation, survival, current scientific breakthroughs and the future. Communicating in these areas with knowledgeable young adults requires skill, tact and self-education upon the part of the cystic fibrosis team.

  11. 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.

  12. Effect of Anluohuaxian Tablet Combined with y-IFN on Schistosomal Liver Fibrosis

    Institute of Scientific and Technical Information of China (English)

    Jiaquan HUANG; Haiyan HUANG; Yuntao JIAO; Guo AI; Tiejun HUANG; Lan LI; Haijing YU; Ke MA; Fei XLAO

    2009-01-01

    The therapeutic effects of anluohuaxian tablet combined with γ-IFN on schistosomal liver fibrosis and its mechanism were studied in a murine model and clinical cases of schistosomal liver fibrosis.Fifty Kunming mice were randomly divided into 5 groups:normal control group,infection control group,anluohuaxian tablet-treated group,γ-IFN-treated group and combined treatment (anluohuaian tablet+γ-IFN) group.Pathologic changes in liver,including hepatic pigmentation and the size of schistosomal egg granuloma,were observed by HE staining after treatment for 8 weeks.The expression of the type Ⅰ and Ⅲ collagen,and TIMP-1 was detected by immunohistochemistry.TGF-β1 mRNA expression was examined by real-time fluorescent quantitative PCR.Sixty patients with schistosomal liver fibrosis were divided into treatment group and control group.The patients in treatment group were treated with anluohuaxian tablet in combination with γ-IFN for 6 months.Be-fore and after treatment,the changes of symptoms and signs,liver function,serum liver fibrosis in-dexes and imaging indexes were observed.The results showed that as compared with infection con-trol group,all forms of treatments relieved the hepatic pathological injury with apparently diminished size of schistosomal egg nodules and decreased percentage of pigmentation (P<0.05).Furthermore,the expression of collagen Ⅰ and Ⅲ,TIMP-Ⅰ,and TGF-β1 mRNA in combined treatment group was significantly decreased as compared with anluohuaxian tablet-treated and γ-IFN-treated groups (P<0.05).In the clinical observation,the serum liver fibrosis indexes,the portal vein width as well as the spleen thickness was significantly reduced in treatment group as compared with control group (P<0.05).It was concluded that the combined use of anluohuaxian tablet with γ-IFN in schistosomal liver fibrosis could protect liver function,alleviate liver fibrosis,and could be used as a choice in treating patients with schiatosomal liver fibrosis.

  13. 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.

  14. 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. PMID:26790364

  15. Simvastatin Reduces Capsular Fibrosis around Silicone Implants.

    Science.gov (United States)

    Chung, Kyu Jin; Park, Ki Rin; Lee, Jun Ho; Kim, Tae Gon; Kim, Yong-Ha

    2016-08-01

    Capsular fibrosis and contracture occurs in most breast reconstruction patients who undergo radiotherapy, and there is no definitive solution for its prevention. Simvastatin was effective at reducing fibrosis in various models. Peri-implant capsular formation is the result of tissue fibrosis development in irradiated breasts. The purpose of this study was to examine the effect of simvastatin on peri-implant fibrosis in rats. Eighteen male Sprague-Dawley rats were allocated to an experimental group (9 rats, 18 implants) or a control group (9 rats, 18 implants). Two hemispherical silicone implants, 10 mm in diameter, were inserted in subpanniculus pockets in each rat. The next day, 10-Gy of radiation from a clinical accelerator was targeted at the implants. Simvastatin (15 mg/kg/day) was administered by oral gavage in the experimental group, while animals in the control group received water. At 12 weeks post-implantation, peri-implant capsules were harvested and examined histologically and by real-time polymerase chain reaction. The average capsular thickness was 371.2 μm in the simvastatin group and 491.2 μm in the control group. The fibrosis ratio was significantly different, with 32.33% in the simvastatin group and 58.44% in the control group (P silicone implants in rats. This finding offers an alternative therapeutic strategy for reducing capsular fibrosis and contracture after implant-based breast reconstruction. PMID:27478339

  16. 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. PMID:27125275

  17. 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...

  18. 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.

  19. La participación en las actividades de ocio de los niños y adolescentes con parálisis cerebral

    OpenAIRE

    Longo Araújo de Melo, Egmar; Badia Corbella, Marta

    2009-01-01

    En este artículo se presenta una revisión sobre el tema de la participación en las actividades de ocio de los niños y adolescentes con discapacidades, centrada en la parálisis cerebral. Según los resultados, los niños y adolescentes con PC participan menos en las actividades de ocio, siendo los más gravemente afectados los que se encontraban más limitados. Además, hay evidencia de que la participación no depende solamente de aspectos intrínsecos al niño/adolescente, sino también de factores f...

  20. 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.

  1. 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.

  2. Growth factors in cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Negar Khalighi

    2016-01-01

    Full Text Available Introduction: Cystic fibrosis is one of the most common autosomal recessive diseases that affects sweat glands and mucosa. CF is a hereditary disease with annual incidence of about 2500 new cases in United Kingdom. Insulin-like growth factor-1 (IGF-1 and insulin-like growth factor binding protein-3 levels decrease in CF. The aim of this study was to assess the role of growth peptides in patients with CF. Method: We searched PubMed, Google scholar, IranMedex, and Scientific Information Database (SID in September 2012 to April 2014. We included clinical studies with available abstracts and full texts that were in English or Persian languages. Manual searching was conducted within the reference lists of articles. Two reviewers independently applied eligibility criteria, assessed quality, and extracted data.Result: The earliest study was published in 1997 and the most recent one was in 2014. Study participants were adults in 3 studies (20% and 12 studies (80% were conducted in children. Patients with CF have lower levels of IGF-1 and there is a significant correlation between IGF-1 levels and growth index in patients with CF.Conclusions: IGF-1 decreases in children with CF and might be the cause of poor growth and low body mass index in these children.

  3. Respiratory Conditions Update: Cystic Fibrosis.

    Science.gov (United States)

    Pritchard, Lyle L

    2016-09-01

    Cystic fibrosis (CF) is an autosomal recessive genetic disease that occurs in approximately 1 in 2,500 white live births. It is less common in nonwhite individuals. A dysfunctional epithelial chloride channel leads to excessively thick mucus affecting multiple organ systems. Common issues include mucous plugging of the airway, lung inflammation, chronic pulmonary infections, intestinal malabsorption, and malnutrition. Universal screening of newborns for CF is recommended in many countries. CF can be diagnosed based on clinical evidence of disease along with genetic testing or other laboratory evidence of chloride channel dysfunction. Pulmonary system dysfunction causes the most morbidity and mortality. Pulmonary function testing is the primary modality used to monitor CF progression. Therapies include chest physiotherapy, mucolytics, antibiotics, anti-inflammatory drugs, targeted therapies, and vaccines. Dysfunction of the exocrine pancreas and gastrointestinal tract leads to malabsorption, malnutrition, and intestinal obstruction. Nutrition should be optimized with adequate calories, pancreatic enzymes, and appropriate dietary supplements. Complications, including acute pulmonary exacerbations, gastrointestinal conditions, chronic rhinosinusitis, CF-related diabetes, osteoporosis, infertility, and psychosocial issues, must be managed. At the appropriate time, lung transplantation and end-of-life issues must be addressed. PMID:27576234

  4. Pneumothorax and idiopathic pulmonary fibrosis

    International Nuclear Information System (INIS)

    We evaluated the relation between the severity of idiopathic pulmonary fibrosis (IPF) and the incidence of pneumothorax on computed tomography (CT) images. In this retrospective study, we evaluated the presence of pneumothorax in 56 consecutive patients who died of IPF from the initial CT to death. We quantitatively analyzed a total of 207 CT images and measured the volume of the normal pattern (N-pattern) and each lesion pattern on the initial CT and their serial changes. The effects of pneumothorax and clinical and CT features on survival were evaluated using Cox regression analysis. Pneumothorax occurred in 17 of 56 patients. Comparison of the pneumothorax (+) and (-) groups showed the initial vital capacity (VC) was lower (P=0.005) and the follow-up period was shorter (P=0.03) in the former group. The decrease in the N-pattern volume in the pneumothorax (+) group was significantly faster than in the pneumothorax (-) group (P=0.013). Cox regression analyses identified a rapid decrease in N-pattern volume (P=0.008) and a rapid decrease in VC (P=0.002), but not pneumothorax, as significant predictors of poor survival. Pneumothorax in IPF patients is associated with lower VC and rapid deterioration of CT findings. The findings suggest that pneumothorax is a complication of advanced IPF. (author)

  5. 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.

  6. 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....

  7. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

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    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

  10. 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

  11. 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.

  12. Bioconjugation of oligonucleotides for treating liver fibrosis.

    Science.gov (United States)

    Ye, Zhaoyang; Houssein, Houssam S Hajj; Mahato, Ram I

    2007-01-01

    Liver fibrosis results from chronic liver injury due to hepatitis B and C, excessive alcohol ingestion, and metal ion overload. Fibrosis culminates in cirrhosis and results in liver failure. Therefore, a potent antifibrotic therapy is urgently needed to reverse scarring and eliminate progression to cirrhosis. Although activated hepatic stellate cells (HSCs) remain the principle cell type responsible for liver fibrosis, perivascular fibroblasts of portal and central veins as well as periductular fibroblasts are other sources of fibrogenic cells. This review will critically discuss various treatment strategies for liver fibrosis, including prevention of liver injury, reduction of inflammation, inhibition of HSC activation, degradation of scar matrix, and inhibition of aberrant collagen synthesis. Oligonucleotides (ODNs) are short, single-stranded nucleic acids, which disrupt expression of target protein by binding to complementary mRNA or forming triplex with genomic DNA. Triplex forming oligonucleotides (TFOs) provide an attractive strategy for treating liver fibrosis. A series of TFOs have been developed for inhibiting the transcription of alpha1(I) collagen gene, which opens a new area for antifibrotic drugs. There will be in-depth discussion on the use of TFOs and how different bioconjugation strategies can be utilized for their site-specific delivery to HSCs or hepatocytes for enhanced antifibrotic activities. Various insights developed in individual strategy and the need for multipronged approaches will also be discussed. PMID:18154454

  13. 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.

  14. 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.

  15. ATRIAL FIBROSIS IS A MORPHOLOGICAL BASIS OF ATRIAL FIBRILLATION

    OpenAIRE

    DRAPKINA O.M.; A. V. Emelyanov

    2015-01-01

    Mechanisms of atrial fibrosis including the role of serotonin in the development of this lesion in patients with atrial fibrillation are presented. New approaches to the treatment of atrial fibrillation aimed at atrial fibrosis reduction are discussed.

  16. [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.

  17. 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.

  18. Matrix Remodeling in Pulmonary Fibrosis and Emphysema.

    Science.gov (United States)

    Kulkarni, Tejaswini; O'Reilly, Philip; Antony, Veena B; Gaggar, Amit; Thannickal, Victor J

    2016-06-01

    Pulmonary fibrosis and emphysema are chronic lung diseases characterized by a progressive decline in lung function, resulting in significant morbidity and mortality. A hallmark of these diseases is recurrent or persistent alveolar epithelial injury, typically caused by common environmental exposures such as cigarette smoke. We propose that critical determinants of the outcome of the injury-repair processes that result in fibrosis versus emphysema are mesenchymal cell fate and associated extracellular matrix dynamics. In this review, we explore the concept that regulation of mesenchymal cells under the influence of soluble factors, in particular transforming growth factor-β1, and the extracellular matrix determine the divergent tissue remodeling responses seen in pulmonary fibrosis and emphysema. PMID:26741177

  19. Vitamin K and the management of patients with cystic fibrosis.

    OpenAIRE

    Durie, P R

    1994-01-01

    OBJECTIVE: To assess the advisability of routine vitamin K supplementation in patients with cystic fibrosis (CF). DATA SOURCES: Studies identified through a MEDLINE search with the use of MeSH terms vitamin K, cystic fibrosis, PIVKA-II (protein induced by vitamin K absence-II), coagulation abnormality and cystic fibrosis, and hepatic disorder and cystic fibrosis. STUDY SELECTION: Six articles published between January 1981 and December 1992 were selected: one general review of vitamin K in in...

  20. Nitric oxide metabolites in cystic fibrosis lung disease

    OpenAIRE

    Grasemann, H; Ioannidis, I.; Tomkiewicz, R; de Groot, H.; Rubin, B; Ratjen, F

    1998-01-01

    Although the activity of nitric oxide (NO) synthases are increased in lung tissue of patients with cystic fibrosis, the concentrations of nasal and exhaled NO have recently been found to be decreased in cystic fibrosis. This could either be due to reduced NO formation or metabolism of NO within airway fluids. In this study, the stable NO metabolites, nitrate and nitrite, were determined in the saliva and sputum of 18 stable cystic fibrosis patients, 21 cystic fibrosis pat...

  1. 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.

  2. Dyspnea perception in cystic fibrosis patients

    Directory of Open Access Journals (Sweden)

    B. Ziegler

    2013-09-01

    Full Text Available We evaluated dyspnea perception in cystic fibrosis patients compared with normal subjects, during an inspiratory resistive loading test and 6-min walk test. We also evaluated the correlation between dyspnea scores induced by resistive loads and by the 6-min walk test. In this prospective, cross-sectional study, 31 patients with cystic fibrosis (≥15 years of age and 31 age-, gender-, and ethnicity-matched healthy volunteers (20 females and 11 males per group underwent inspiratory resistive loading, spirometry, and the 6-min walk test. As the magnitude of the inspiratory loads increased, dyspnea scores increased (P<0.001, but there was no difference between groups in dyspnea score (P=0.654. Twenty-six (84% normal subjects completed all the resistive loads, compared with only 12 (39% cystic fibrosis patients (P<0.001. Dyspnea scores were higher after the 6-min walk test than at rest (P<0.001, but did not differ between groups (P=0.080. Post-6-min walk test dyspnea scores correlated significantly with dyspnea scores induced by resistive loads. We conclude that dyspnea perception induced in cystic fibrosis patients by inspiratory resistive loading and by 6-min walk test did not differ from that induced in normal subjects. However, cystic fibrosis patients discontinued inspiratory resistive loading more frequently. There were significant correlations between dyspnea perception scores induced by inspiratory resistance loading and by the 6-min walk test. This study should alert clinicians to the fact that some cystic fibrosis patients fail to discriminate dyspnea perception and could be at risk for delay in seeking medical care.

  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. 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...

  5. Acute Scedosporium apiospermum Endobronchial Infection in Cystic Fibrosis.

    Science.gov (United States)

    Padoan, Rita; Poli, Piercarlo; Colombrita, Domenico; Borghi, Elisa; Timpano, Silviana; Berlucchi, Marco

    2016-06-01

    Fungi are known pathogens in cystic fibrosis patients. A boy with cystic fibrosis boy presented with acute respiratory distress. Bronchoscopy showed airways obstruction by mucus plugs and bronchial casts. Scedosporium apiospermum was identified as the only pathogen. Bronchoalveolar lavage successfully resolved the acute obstruction. Plastic bronchitis is a new clinical picture of acute Scedosporium endobronchial colonization in cystic fibrosis patients. PMID:26967814

  6. 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.

  7. 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;…

  8. Endocytosis and intracellular protein degradation in cystic fibrosis fibroblasts

    International Nuclear Information System (INIS)

    Normal rates of pinocytosis of [3H]sucrose were measured in cystic fibrosis fibroblasts, and were not affected by the addition of cystic fibrosis serum. Bulk protein degradation (a significant proportion of which occurs intralysosomally following autophagy) and its regulation by growth state were apparently identical in normal and cystic fibrosis cultures. (Auth.)

  9. Centrilobular emphysema combined with pulmonary fibrosis results in improved survival

    OpenAIRE

    Todd Nevins W; Jeudy Jean; Lavania Sachin; Franks Teri J; Galvin Jeffrey R; Deepak Janaki; Britt Edward J; Atamas Sergei P

    2011-01-01

    Abstract Background We hypothesized that, in patients with pulmonary fibrosis combined with emphysema, clinical characteristics and outcomes may differ from patients with pulmonary fibrosis without emphysema. We identified 102 patients who met established criteria for pulmonary fibrosis. The amount of emphysema (numerical score) and type of emphysema (centrilobular, paraseptal, or mixed) were characterized in each patient. Clinical characteristics, pulmonary function tests and patient surviva...

  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. 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.

  12. 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.

  13. Cellular and molecular mechanisms in kidney fibrosis

    Science.gov (United States)

    Duffield, Jeremy S.

    2014-01-01

    Fibrosis is a characteristic feature of all forms of chronic kidney disease. Deposition of pathological matrix in the interstitial space and within the walls of glomerular capillaries as well as the cellular processes resulting in this deposition are increasingly recognized as important factors amplifying kidney injury and accelerating nephron demise. Recent insights into the cellular and molecular mechanisms of fibrogenesis herald the promise of new therapies to slow kidney disease progression. This review focuses on new findings that enhance understanding of cellular and molecular mechanisms of fibrosis, the characteristics of myofibroblasts, their progenitors, and molecular pathways regulating both fibrogenesis and its resolution. PMID:24892703

  14. 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...

  15. 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. PMID:26857767

  16. Qulaity of Life in Cystic Fibrosis Children

    OpenAIRE

    Hamid-Reza Kianifar; Banafsheh Bakhshoodeh; Paria Hebrani; Fatemeh Behdani

    2013-01-01

    Objective: Cystic fibrosis (CF) is a chronic, multisystem genetic disease with a wide variability in clinical severity. The measurement of quality of life in CF provides additional information about the impact of this disease. This article tries to assess quality of life (QoL) in children and adolescents with CF and to compare it with control group.Methods: Patients 2-18 years old with admission diagnosis of cystic fibrosis entered the study. QoL was observed in CF patients and compared with ...

  17. 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

  18. 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.

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

    OpenAIRE

    Cebrián Pinar, Mónica

    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...

  20. Papel del sistema angiotensina en la fisiopatología de la fibrosis pulmonar

    OpenAIRE

    Molina Molina, María

    2007-01-01

    [spa] La Fibrosis Pulmonar Idiopática (FPI) es la enfermedad pulmonar intersticial difusa mas frecuente y con peor pronóstico, la supervivencia media desde el diagnostico es de 3-4 años. No existe en la actualidad ningún tratamiento efectivo. La hipótesis fisiopatologica actualmente aceptada es que una lesión o lesiones de la célula epitelial alveolar provocaría la apoptosis de estas células y su activación, la síntesis y secreción de diversos mediadores profibroticos, que, a su vez, provocar...

  1. Non-Invasive Evaluation of Cystic Fibrosis Related Liver Disease in Adults with ARFI, Transient Elastography and Different Fibrosis Scores

    OpenAIRE

    Karlas, Thomas; Neuschulz, Marie; Oltmanns, Annett; Güttler, Andrea; Petroff, David; Wirtz, Hubert; Mainz, Jochen G.; Mössner, Joachim; Berg, Thomas; Tröltzsch, Michael; Keim, Volker; Wiegand, Johannes

    2012-01-01

    Background Cystic fibrosis-related liver disease (CFLD) is present in up to 30% of cystic fibrosis patients and can result in progressive liver failure. Diagnosis of CFLD is challenging. Non-invasive methods for staging of liver fibrosis display an interesting diagnostic approach for CFLD detection. Aim We evaluated transient elastography (TE), acoustic radiation force impulse imaging (ARFI), and fibrosis indices for CFLD detection. Methods TE and ARFI were performed in 55 adult CF patients. ...

  2. 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.

  3. 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.

  4. 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.

  5. 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.

  6. 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.

  7. Efectos de las cenizas y la repelencia al agua en suelos afectados por incendios forestales en ecosistemas mediterráneos = Ash and water repellency efects on soil hydrology in fire-afected Mediterranean ecosystems

    OpenAIRE

    Berenguer Bodí, Mercedes

    2012-01-01

    El fuego es un factor ecológico natural en la Tierra y ha actuado con diferente intensidad desde hace 400 millones de años. En condiciones de clima mediterráneo el fuego es un factor recurrente que las sociedades humanas han controlado desde hace milenios. Sin embargo, tras medio siglo de abandono agrícola y ganadero, la gestión del monte mediterráneo mediante el uso del fuego es inexistente. Además, con la recuperación de la vegetación, el fuego afecta ahora a una densa y continua masa fores...

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

    OpenAIRE

    Andersson, Cecilia K; Andersson Sjöland, Annika; Mori, Michiko; Hallgren, Oskar; Pardo, Annie; Eriksson, Leif; Bjermer, Leif; Löfdahl, Claes-Göran; Selman, Moises; Westergren-Thorsson, Gunilla; Erjefält, Jonas

    2011-01-01

    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 usin...

  9. Cellular Mechanisms of Tissue Fibrosis. 7. New insights into the cellular mechanisms of pulmonary fibrosis

    OpenAIRE

    Barkauskas, Christina E.; Paul W. Noble

    2014-01-01

    Idiopathic pulmonary fibrosis (IPF) is a devastating disease characterized by severe and progressive scar formation in the gas-exchange regions of the lung. Despite years of research, therapeutic treatments remain elusive and there is a pressing need for deeper mechanistic insights into the pathogenesis of the disease. In this article, we review our current knowledge of the triggers and/or perpetuators of pulmonary fibrosis with special emphasis on the alveolar epithelium and the underlying m...

  10. Fibrosis of Two: Epithelial Cell-Fibroblast Interactions in Pulmonary Fibrosis

    OpenAIRE

    Sakai, Norihiko; Tager, Andrew M.

    2013-01-01

    Idiopathic pulmonary fibrosis (IPF) is characterized by the progressive and ultimately fatal accumulation of fibroblasts and extracellular matrix in the lung that distorts its architecture and compromises its function. IPF is now thought to result from wound-healing processes that, although initiated to protect the host from injurious environmental stimuli, lead to pathological fibrosis due to these processes becoming aberrant or over-exuberant. Although the environmental stimuli that trigger...

  11. Combined pulmonary fibrosis and emphysema (CPFE): an entity different from emphysema or pulmonary fibrosis alone

    OpenAIRE

    Lin, Huijin; Jiang, Shanping

    2015-01-01

    Chronic obstructive pulmonary disease (COPD) and idiopathic interstitial pneumonias (IIP), with different radiological, pathological, functional and prognostic characteristics, have been regarded as separate entities for a long time. However, there is an increasing recognition of the coexistence of emphysema and pulmonary fibrosis in individuals. The association was first described as a syndrome by Cottin in 2005, named “combined pulmonary fibrosis and emphysema (CPFE)”, which is characterize...

  12. 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...

  13. 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...

  14. 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

  15. Statement on Exercise Testing in Cystic Fibrosis

    NARCIS (Netherlands)

    Hebestreit, Helge; Arets, Hubertus G M; Aurora, Paul; Boas, Steve; Cerny, Frank; Hulzebos, Erik H J; Karila, Chantal; Lands, Larry C; Lowman, John D; Swisher, Anne; Urquhart, Don S

    2015-01-01

    This statement summarizes the information available on specific exercise test protocols and outcome parameters used in patients with cystic fibrosis (CF) and provides expert consensus recommendations for protocol and performance of exercise tests and basic interpretation of results for clinicians. T

  16. 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...

  17. 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...

  18. Exercise and Immune Function in Cystic Fibrosis

    NARCIS (Netherlands)

    van Leeuwen, P.B.

    2013-01-01

    In this thesis, we investigated the association between exercise, infection and inflammation in patients with Cystic Fibrosis (CF), since they have been described to be interconnected.Patients with CF spend a lot of time in physical exercises to maintain physical fitness, since exercise capacity dec

  19. Respiratory bacterial infections in cystic fibrosis

    DEFF Research Database (Denmark)

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

    2013-01-01

    PURPOSE OF REVIEW: 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...... respiratory tract (nasal sampling) should be investigated and both infection sites should be treated....

  20. Prognosis in Cystic Fibrosis: Trends and Predictors

    NARCIS (Netherlands)

    Slieker, M.G.

    2008-01-01

    Cystic fibrosis (CF) is a multisystem disease affecting the digestive system, sweat glands, and the reproductive tract, but progressive lung disease continues to be the major cause of morbidity and mortality. Patients develop chronic infection of the respiratory tract with a characteristic array of

  1. 4246 Hypercoagulability promotes atrial fibrosis and fibrillation

    NARCIS (Netherlands)

    Schotten, Ulrich; Verheule, Sander; De Jong, Anne-Margreet; De Boer, Hetty; Maass, Alexander H.; Lau, Dennis H.; Rienstra, Michiel; Kamphuisen, Pieter W.; Ten Cate, Hugo; Crijns, Harry J.G.; Van Gelder, Isabelle C.; Van Zonneveld, Anton J.; Spronk, Henri

    2014-01-01

    Introduction: Atrial fibrillation (AF) induces a hypercoagulable state. Coagulation factors provoke pro-fibrotic, pro-hypertrophic, and pro-inflammatory responses in a variety of tissues by stimulation of protease-activated receptors. We studied whether hypercoagulability causes atrial fibrosis and

  2. Potent effects of dioscin against liver fibrosis

    Science.gov (United States)

    Zhang, Xiaoling; Han, Xu; Yin, Lianhong; Xu, Lina; Qi, Yan; Xu, Youwei; Sun, Huijun; Lin, Yuan; Liu, Kexin; Peng, Jinyong

    2015-01-01

    We previously reported the promising effects of dioscin against liver injury, but its effect on liver fibrosis remains unknown. The present work investigated the activities of dioscin against liver fibrosis and the underlying molecular mechanisms. Dioscin effectively inhibited the cell viabilities of HSC-T6, LX-2 and primary rat hepatic stellate cells (HSCs), but not hepatocytes. Furthermore, dioscin markedly increased peroxisome proliferator activated receptor-γ (PPAR-γ) expression and significantly reduced a-smooth muscle actin (α-SMA), transforming growth factor-β1 (TGF-β1), collagen α1 (I) (COL1A1) and collagen α1 (III) (COL3A1) levels in vitro. Notably, dioscin inhibited HSCs activation and induced apoptosis in activated HSCs. In vivo, dioscin significantly improved body weight and hydroxylproline, laminin, α-SMA, TGF-β1, COL1A1 and COL3A1 levels, which were confirmed by histopathological assays. Dioscin facilitated matrix degradation, and exhibited hepatoprotective effects through the attenuation of oxidative stress and inflammation, in addition to exerting anti-fibrotic effects through the modulation of the TGF-β1/Smad, Wnt/β-catenin, mitogen-activated protein kinase (MAPK) and mitochondrial signaling pathways, which triggered the senescence of activated HSCs. In conclusion, dioscin exhibited potent effects against liver fibrosis through the modulation of multiple targets and signaling pathways and should be developed as a novel candidate for the treatment of liver fibrosis in the future. PMID:25853178

  3. Surgical management of pneumothorax in cystic fibrosis.

    OpenAIRE

    Seddon, D. J.; Hodson, M E

    1988-01-01

    Twenty seven adults with cystic fibrosis who had had either a surgical pleurodesis or pleurectomy for the management of pneumothorax were studied. There were no significant differences in postoperative respiratory function, incidence of recurrent pneumothorax, or incidence of major postoperative complications between the two groups.

  4. Cystic fibrosis, are we missing in India?

    Directory of Open Access Journals (Sweden)

    Pramila Menon

    2012-09-01

    Full Text Available Introduction: This study was undertaken to evaluate and improve the availability and accessibility of CF services in Maharashtra, Pune,India. Material and methods: Tool used was a questionnaire (Annexure I which was prevalidated by the departmental faculty with 15 items which included experience regarding CF patients in relation to knowledge about diagnosis and prognosis of CF patients, availability about diagnostic and management services of CF wasadministered to the pediatricians who participated in the training workshop at Pune Regional centre, Aundh, Pune, Maharashtra, India. ( n = 40. Results: 92.5% agreed that Cystic fibrosis (CF, is now a pan ethnic disease. 100% accepted that the factors that are associated with decreased survival in CF patients from developing countries are early age of onset of symptoms, severe malnutrition at the time of diagnosis and frequent episodes of pneumonia. Severemalnutrition, not responding to nutritional therapy , neonatal meconium ileus and recurrent pneumonia , 87.5% 100% and 85% suspect cystic fibrosis respectively. 90% do not have facilities for diagnosis of cystic fibrosis like sweat chloride test and 87.5% felt absence of facilities makes the diagnosis of cystic fibrosis difficult. 90% agreed that the education of pediatricians about the disease, can improve the quality of life and survival in CF. Conclusion: Inadequacy of diagnostic services for CF may be reason for missing CF cases in India and we need to sensitize the health professionals for CF diagnosis and develop better diagnostic and patient support services for CF.

  5. 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

  6. 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...

  7. 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...

  8. Mouse models of liver fibrosis mimic human liver fibrosis of different etiologies.

    Science.gov (United States)

    Martínez, Allyson K; Maroni, Luca; Marzioni, Marco; Ahmed, Syed T; Milad, Mena; Ray, Debolina; Alpini, Gianfranco; Glaser, Shannon S

    2014-12-01

    The liver has the amazing capacity to repair itself after injury; however, the same processes that are involved in liver regeneration after acute injury can cause serious consequences during chronic liver injury. In an effort to repair damage, activated hepatic stellate cells trigger a cascade of events that lead to deposition and accumulation of extracellular matrix components causing the progressive replacement of the liver parenchyma by scar tissue, thus resulting in fibrosis. Although fibrosis occurs as a result of many chronic liver diseases, the molecular mechanisms involved depend on the underlying etiology. Since studying liver fibrosis in human subjects is complicated by many factors, mouse models of liver fibrosis that mimic the human conditions fill this void. This review summarizes the general mouse models of liver fibrosis and mouse models that mimic specific human disease conditions that result in liver fibrosis. Additionally, recent progress that has been made in understanding the molecular mechanisms involved in the fibrogenic processes of each of the human disease conditions is highlighted. PMID:25396098

  9. 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.

  10. Imaging of intestinal fibrosis: current challenges and future methods.

    Science.gov (United States)

    Stidham, Ryan W; Higgins, Peter Dr

    2016-08-01

    Crohn's disease (CD) activity assessments are dominated by inflammatory changes without discrete measurement of the coexisting fibrotic contribution to total bowel damage. Intestinal fibrosis impacts the development of severe structural complications and the overall natural history of CD. Measuring intestinal fibrosis is challenging and existing methods of disease assessment are unable to reliably distinguish fibrosis from inflammation. Both the immediate clinical need to measure fibrosis for therapeutic decision-making and the near-future need for tools to assess pipeline anti-fibrotic medications highlight the demand for biomarkers of fibrosis in CD. Developing non-invasive technologies exploit changes in intestinal perfusion, mechanical properties, and macromolecular content to provide quantitative markers of fibrosis. In this review of existing and experimental technologies for imaging intestinal fibrosis, we discuss the expanding capabilities of quantitative MR and ultrasound imaging, encouraging developments in non-invasive elastography, and emerging novel methods including photoacoustic imaging. PMID:27536361

  11. Modeling the mechanical properties of liver fibrosis in rats.

    Science.gov (United States)

    Zhu, Ying; Chen, Xin; Zhang, Xinyu; Chen, Siping; Shen, Yuanyuan; Song, Liang

    2016-06-14

    The progression of liver fibrosis changes the biomechanical properties of liver tissue. This study characterized and compared different liver fibrosis stages in rats in terms of viscoelasticity. Three viscoelastic models, the Voigt, Maxwell, and Zener models, were applied to experimental data from rheometer tests and then the elasticity and viscosity were estimated for each fibrosis stage. The study found that both elasticity and viscosity are correlated with the various stages of liver fibrosis. The study revealed that the Zener model is the optimal model for describing the mechanical properties of each fibrosis stage, but there is no significant difference between the Zener and Voigt models in their performance on liver fibrosis staging. Therefore the Voigt model can still be effectively used for liver fibrosis grading. PMID:27017300

  12. 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

    OpenAIRE

    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

    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...

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

    OpenAIRE

    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

    2015-01-01

    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 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)...

  14. 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

  15. Biopsy-controlled liver fibrosis staging using the enhanced liver fibrosis (ELF score compared to transient elastography.

    Directory of Open Access Journals (Sweden)

    Kristin Wahl

    Full Text Available BACKGROUND AND AIMS: Chronic liver diseases are characterized by inflammatory and fibrotic liver injuries that often result in liver cirrhosis with its associated complications such as portal hypertension and hepatocellular carcinoma. Liver biopsy still represents the reference standard for fibrosis staging, although transient elastography is increasingly used for non-invasive monitoring of fibrosis progression. However, this method is not generally available and is associated with technical limitations emphasizing the need for serological biomarkers staging of liver fibrosis. The enhanced liver fibrosis (ELF score was shown to accurately predict significant liver fibrosis in different liver diseases, although extracellular matrix components detected by this score may not only mirror the extent of liver fibrosis but also inflammatory processes. METHODS: In this prospective biopsy-controlled study we evaluated the utility of the ELF score in comparison to transient elastography to predict different stages of fibrosis in 102 patients with chronic liver diseases. RESULTS: Both techniques revealed similar area under receiver operating characteristic curve values for prediction of advanced fibrosis stages. Compared to transient elastography, the ELF score showed a broader overlap between low and moderate fibrosis stages and a stronger correlation with inflammatory liver injury. CONCLUSIONS: Both the ELF score as well as transient elastography allowed for high quality fibrosis staging. However, the ELF score was less discriminative in low and moderate fibrosis stages and appeared more strongly influenced by inflammatory liver injury. This should be considered when making clinical interpretations on the basis of ELF score values.

  16. 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

  17. Funciones con Microsoft Excel

    OpenAIRE

    Castillo, Dalia Imelda; Estrada, Ana Luisa; Hernández, Brenda Amalia

    2009-01-01

    En este documento se presenta el desarrollo de algunas actividades que se trabajaron con estudiantes de primer semestre de la Universidad Autónoma de Nayarit; utilizando la hoja de cálculo Excel en el tema de visualización de funciones, para la materia de lenguaje y pensamiento matemático. Ya que la tecnología ha adquirido un papel muy importante en el proceso enseñanza-aprendizaje, nos ofrece un medio para que el estudiante explore, analice, verifique y desarrolle habilidades que se serán út...

  18. 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...

  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. 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

  1. Conversando con... BENEDETTA TAGLIABUE

    OpenAIRE

    Torres, Ana; Cabanes, Miguel

    2011-01-01

    Esta entrevista se realiza en el marco del XIII Congreso Internacional de Expresión Gráfica Arquitectónica realizado en la Escuela Técnica Superior de Arquitectura de Valencia los días 27 al 30 de Mayo de 2010.Benedetta Tagliabue es, en la actualidad, una de las arquitectas con mayor prestigio en el panorama internacional. El Pabellón de España para la Expo de Shanghai 2010, es una de sus últimas obras más representativas, en el que se acentúa y desarrolla un conjunto de características arqui...

  2. 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. 

  3. 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.

  4. 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.

  5. 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.

  6. 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.

  7. 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.

  8. 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.

  9. Nasal Eosinophilic Angiocentric Fibrosis with Orbital Extension.

    Science.gov (United States)

    Faramarzi, Mohammad; Dadgarnia, Mohammad Hossein; Moghimi, Mansour; Sharouny, Hadi; Behniafard, Nasim

    2015-09-01

    Eosinophilic angiocentric fibrosis (EAF) is an extremely rare, chronic, benign, idiopathic disorder that mostly affects the upper respiratory tract, particularly the nasal cavity, and features progressive submucosal perivascular fibrosis. To the best of our knowledge, only seven cases of EAF with orbital involvement have been reported. We report a case of sinonasal EAF with orbital extension that presented with left nasolacrimal duct obstruction. A 35-year-old man presented with left epiphora, proptosis, anterolateral globe displacement and nasal obstruction. Endoscopic sinus examination showed a firm, gritty, creamy, yellow, fibrous, adherent mass of maxillary sinus. Diagnosis was established with histopathological examination of excisional biopsy of the lesion. Although EAF is very rare, it should be considered in the differential diagnosis of lesions of upper airway tract, particularly the nasal cavity. Biopsy is necessary for diagnosis and treatment planning. Resecting of the involved tissues completely is essential for prevention of recurrence. PMID:25601283

  10. Congenital Fibrosis of the Extraocular Muscles

    Directory of Open Access Journals (Sweden)

    Leyla Niyaz

    2014-08-01

    Full Text Available Congenital fibrosis of the extraocular muscles (CFEOM is a rare disorder characterized by hereditary non-progressive restrictive strabismus and blepharoptosis. Although most of the cases are bilateral and isolated, some patients may have systemic findings. CFEOM is divided into three groups as CFEOM 1, 2, and 3 according to the phenotype. Primary responsible genes are KIF21A for CFEOM type 1 and 3 and PHOX2A/ARIX gene for CFEOM type 2. Studies suggest that abnormal innervation of the extraocular muscles is the cause of muscle fibrosis. Early treatment is important because of the risk of amblyopia. Surgery is the primary treatment option for strabismus and blepharoptosis. (Turk J Ophthalmol 2014; 44: 312-5

  11. 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.

  12. [Noncirrhotic liver fibrosis after chronic arsenic poisoning].

    Science.gov (United States)

    Piontek, M; Hengels, K J; Borchard, F; Strohmeyer, G

    1989-10-27

    A 67-year-old woman with portal hypertension, splenomegaly without portal vein thrombosis, leucopenia and thrombocytopenia of splenic origin had repeated episodes of life-threatening haemorrhage from esophageal varices. Since childhood she had suffered from psoriasis and had been treated over a period of 15 years with Fowler's solution (in all about 25 g of arsenic trioxide). She had the characteristic skin lesions of arsenical poisoning-palmar hyperkeratoses and two basal cell carcinomas on the trunk. Histological examination of a wedge biopsy from the liver showed definite structural changes with fibrosis around the central veins and in the portal tracts. There was no evidence of cirrhotic alteration. The hepatocytes were normal by light microscopy and electron microscopy. This case of noncirrhotic hepatic fibrosis is considered to have been caused by chronic arsenical poisoning.

  13. 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.

  14. Entrevista con Geoffrey Lloyd.

    OpenAIRE

    Fernando Colina Pérez; Mauricio Jalón

    2008-01-01

    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,...

  15. Acute exacerbation of airspace enlargement with fibrosis

    OpenAIRE

    Tomoyuki Kakugawa; Kazuhiro Tabata; Daiki Ogawara; Tomoshi Tsuchiya; Shintaro Hara; Noriho Sakamoto; Yuji Ishimatsu; Kazuto Ashizawa; Takeshi Nagayasu; Junya Fukuoka; Shigeru Kohno

    2014-01-01

    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 ...

  16. Objective cough frequency in Idiopathic Pulmonary Fibrosis

    OpenAIRE

    Key, Angela L; Holt, Kimberley; Hamilton, Andrew; Smith, Jaclyn A; Earis, John E

    2010-01-01

    Background Cough is a common presenting symptom in patients with Idiopathic Pulmonary Fibrosis (IPF). This study measured cough rates in IPF patients and investigated the association between cough and measures of health related quality of life and subjective cough assessments. In addition, IPF cough rates were related to measures of physiological disease severity and compared to cough rates in health and other respiratory conditions. Methods Nineteen IPF patients, mean age 70.8 years ± 8.6, f...

  17. Peripheral Blood Biomarkers in Idiopathic Pulmonary Fibrosis

    OpenAIRE

    Vij, Rekha; Noth, Imre

    2012-01-01

    In this article, we review the evidence for peripheral blood biomarkers in idiopathic pulmonary fibrosis (IPF), a life-threatening fibrotic lung disease of unknown etiology. We focus on selected biomarkers present in peripheral blood, as they are easy to obtain, can be measured longitudinally, and have the greatest likelihood of achieving clinical utility. This article concentrates on biomarkers with mechanistic plausibility that may be directly involved in the development of IPF, including K...

  18. Cellular and molecular mechanisms in kidney fibrosis

    OpenAIRE

    Duffield, Jeremy S.

    2014-01-01

    Fibrosis is a characteristic feature of all forms of chronic kidney disease. Deposition of pathological matrix in the interstitial space and within the walls of glomerular capillaries as well as the cellular processes resulting in this deposition are increasingly recognized as important factors amplifying kidney injury and accelerating nephron demise. Recent insights into the cellular and molecular mechanisms of fibrogenesis herald the promise of new therapies to slow kidney disease progressi...

  19. Substance P modulates colitis-associated fibrosis.

    Science.gov (United States)

    Koon, Hon Wai; Shih, David; Karagiannides, Iordanes; Zhao, Dezheng; Fazelbhoy, Zafeer; Hing, Tressia; Xu, Hua; Lu, Bao; Gerard, Norma; Pothoulakis, Charalabos

    2010-11-01

    Substance P (SP) and the neurokinin-1 receptor (NK-1R) are involved in the development of colitis and mucosal healing after colonic inflammation. We studied whether SP modulates colonic fibrosis by using a chronic model of trinitrobenzenesulfonic acid (TNBS)-induced colitis in wild-type (WT) and NK-1R-deficient (NK-1R KD) mice. We found increased mRNA expression levels of collagen, vimentin, and the fibrogenic factors transforming growth factor β1 and insulin-like growth factor 1 in the chronically inflamed colons of WT mice treated with repeated intracolonic TNBS administrations. Fibrosis in TNBS-treated mice was also evident immunohistochemically by collagen deposition in the colon. Treatment of TNBS-exposed WT mice with the NK-1R antagonist CJ-12255 reduced colonic inflammation, colonic fibrosis, fibroblast accumulation, and expression levels of the fibrogenic factors. NK-1R knockout mice chronically exposed to TNBS had similar colonic inflammation compared with WT, but reduced colonic fibrosis, fibroblast accumulation, and expression levels of fibrogenic factors. Immunohistochemical staining also showed co-localization of NK-1R with fibroblasts in inflamed colons of mice and in colonic mucosa of patients with Crohn's disease. Exposure of human colonic CCD-18Co fibroblasts to SP (10 nmol/L) increased cell migration. SP stimulated collagen synthesis in CCD-18Co fibroblasts in the presence of transforming growth factor β1 and insulin-like growth factor 1, and this effect was reduced by Akt inhibition. Thus, SP, via NK-1R, promotes intestinal fibrogenesis after chronic colitis by stimulating fibrotic responses in fibroblasts. PMID:20889569

  20. Cardiac manifestations of idiopathic pulmonary fibrosis

    OpenAIRE

    Agrawal, Abhinav; Verma, Isha; Shah, Varun; Agarwal, Abhishek; Sikachi, Rutuja R

    2016-01-01

    Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, parenchymal disease of the lung with an estimated prevalence of 14–43 per 100,000. Patient usually presents with coughing and exertional dyspnea, which can lead to acute respiratory failure. IPF has been associated with various co-morbidities such as lung cancer, emphysema, obstructive sleep apnea (OSA), GERD and multiple cardiovascular consequences. The cardiovascular manifestations of IPF include pulmonary hypertension, heart fa...

  1. Targeting Fibrosis in Duchenne Muscular Dystrophy

    OpenAIRE

    Zhou, Lan; Lu, Haiyan

    2010-01-01

    Duchenne muscular dystrophy (DMD) is the most common genetic muscle disease affecting 1 in 3,500 live male births. It is an X-linked recessive disease caused by a defective dystrophin gene. The disease is characterized by progressive limb weakness, respiratory and cardiac failure and premature death. Fibrosis is a prominent pathological feature of muscle biopsies from patients with DMD. It directly causes muscle dysfunction and contributes to the lethal DMD phenotype. Although gene therapy an...

  2. Exacerbations in cystic fibrosis: 2 · Prevention

    OpenAIRE

    Bell, Scott C; Robinson, Philip J

    2007-01-01

    The life span of people with cystic fibrosis (CF) has increased dramatically over the past 50 years. Many factors have contributed to this improvement. Respiratory exacerbations of CF lung disease are associated with the need for hospitalisation and antibiotic treatment, reduction in the quality of life, fragmented sleep and mortality. A number of preventive treatment strategies have been developed to reduce the frequency and severity of respiratory exacerbations in CF including mucolytic age...

  3. Cystic Fibrosis in a Hispanic Adolescent

    OpenAIRE

    Lin, Jenny H.; Collaco, Joseph M.; Paranjape, Shruti M.

    2013-01-01

    We describe the presentation of a Hispanic adolescent with chronic respiratory symptoms and poor growth that led to a diagnosis of cystic fibrosis (CF) based on an indeterminate sweat chloride result and DNA sequence analysis that revealed a single new frameshift mutation, Nt3878insATCAG, which results in a premature stop codon in exon 20 of the CFTR gene. This case, highlighted by the identification of a deleterious, disease-causing mutation, illustrates the importance of maintaining both a ...

  4. MR imaging of pancreas in cystic fibrosis

    International Nuclear Information System (INIS)

    The pancreatic regions of 18 patients with cystic fibrosis were analyzed with a 1.5 Tesla MR unit. Signal intensity of the pancreas was correlated with clinical data and ultrasound. A hyperintense pancreas on T1-weighted image was consistent with fatty replacement of pancreatic insufficiency. A pancreas of normal soft tissue intensity was found in two asymptomatic and one symptomatic patient. A very hypointense pancreas on any pulse sequence was considered to be an intermediate stage of pancreatic degeneration. (orig.)

  5. 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.

  6. Intraluminal fibrosis in interstitial lung disorders.

    OpenAIRE

    Basset, F.; Ferrans, V J; Soler, P; T. Takemura; Y. Fukuda(Miyagi University of Education); Crystal, R G

    1986-01-01

    The histopathologic and ultrastructural features of intraluminal organizing and fibrotic changes were studied in open lung biopsies and autopsy specimens from 373 patients with interstitial lung disorders, including hypersensitivity pneumonitis (n = 44), idiopathic pulmonary fibrosis (n = 92), collagen-vascular diseases (n = 20), chronic eosinophilic pneumonia (n = 10), pulmonary histiocytosis X (n-90), pulmonary sarcoidosis (n = 62), pneumoconioses (n = 25), Legionnaire's disease (n = 5), dr...

  7. 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

  8. Liver Fibrosis: From Pathogenesis to Novel Therapies.

    Science.gov (United States)

    Weiskirchen, Ralf; Tacke, Frank

    2016-01-01

    Chronic liver injury is accompanied by a dysbalanced scarring process, termed fibrosis. This process is mainly driven by chronic inflammation and an altered activity of a multitude of different chemokines and cytokines, resulting in the infiltration by immune cells (especially macrophages) and increase of matrix-expressing cell types. These processes might lead to cirrhosis representing the end-stage of fibrosis. Recent clinical studies comprising patients successfully treated for viral hepatitis showed that liver fibrogenesis and even cirrhosis may be reverted. The hepatic capacity to remodel scar tissue and to revert into a normal liver follows specific mechanistic principles that include the termination of chronic tissue damage, shifting the cellular bias from inflammation to resolution, initiation of myofibroblast apoptosis or senescence and, finally, fibrinolysis of excess scar tissue. The plurality of molecular and cellular triggers involved in initiation, progression and resolution of hepatic fibrogenesis offers an infinite number of therapeutic possibilities. For instance, inflammatory macrophages can be targeted via inhibition of chemokine CCL2 or its receptor CCR2 (e.g., by cenicriviroc) as well as by transfer of restorative macrophage subsets. Another target is galectin-3 that acts at various stages along the continuum from acute to chronic inflammation. Profibrogenic cytokines (e.g., transforming growth factor-β), matricellular proteins (e.g., CCN1/CYR61) or signaling pathways involved in fibrogenesis offer further possible targets. Other options are the application of therapeutic antibodies directed against components involved in biogenesis or remodeling of connective tissue such as lysyl oxidase-like-2 or synthetic bile acids like obeticholic acid that activate the farnesoid X receptor and was antifibrotic in a phase 2 study (FLINT trial). Factors affecting the gut barrier function or the intestinal microbiome further expanded the repertoire of drug

  9. Managing comorbidities in idiopathic pulmonary fibrosis

    OpenAIRE

    Fulton BG; Ryerson CJ

    2015-01-01

    Blair G Fulton,1 Christopher J Ryerson1,2 1Department of Medicine, 2Centre for Heart Lung Innovation, University of British Columbia, Vancouver, BC, Canada Abstract: Major risk factors for idiopathic pulmonary fibrosis (IPF) include older age and a history of smoking, which predispose to several pulmonary and extra-pulmonary diseases. IPF can be associated with additional comorbidities through other mechanisms as either a cause or a consequence of these diseases. We review the literature rega...

  10. Vitamin D Deficiency in Cystic Fibrosis

    OpenAIRE

    Hall, William B.; Sparks, Amy A.; Aris, Robert M

    2010-01-01

    Cystic Fibrosis is the most common inherited genetic respiratory disorder in the Western World. Hypovitaminosis D is almost universal in CF patients, likely due to a combination of inadequate absorption, impaired metabolism, and lack of sun exposure. Inadequate levels are associated with the high prevalence of bone disease or osteoporosis in CF patients, which is associated with increased morbidity including fractures, kyphosis, and worsening pulmonary status. Treatment goals include regular ...

  11. 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.

  12. Host Responses in Tissue Repair and Fibrosis

    Science.gov (United States)

    Duffield, Jeremy S.; Lupher, Mark; Thannickal, Victor J.

    2013-01-01

    Myofibroblasts accumulate in the spaces between organ structures and produce extracellular matrix (ECM) proteins, including collagen I. They are the primary “effector” cells in tissue remodeling and fibrosis. Previously, leukocyte progenitors termed fibrocytes and myofibroblasts generated from epithelial cells through epithelial-to-mesenchymal transition (EMT) were considered the primary sources of ECM-producing myofibroblasts in injured tissues. However, genetic fate mapping experiments suggest that mesenchyme-derived cells, known as resident fibroblasts, and pericytes are the primary precursors of scar-forming myofibroblasts, whereas epithelial cells, endothelial cells, and myeloid leukocytes contribute to fibrogenesis predominantly by producing key fibrogenic cytokines and by promoting cell-to-cell communication. Numerous cytokines derived from T cells, macrophages, and other myeloid cell populations are important drivers of myofibroblast differentiation. Monocyte-derived cell populations are key regulators of the fibrotic process: They act as a brake on the processes driving fibrogenesis, and they dismantle and degrade established fibrosis. We discuss the origins, modes of activation, and fate of myofibroblasts in various important fibrotic diseases and describe how manipulation of macrophage activation could help ameliorate fibrosis. PMID:23092186

  13. Smoking and Pulmonary Fibrosis: Novel Insights

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    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.

  14. Progress in therapies for cystic fibrosis.

    Science.gov (United States)

    De Boeck, Kris; Amaral, Margarida D

    2016-08-01

    Standard follow-up and symptomatic treatment have allowed most patients with cystic fibrosis to live to young adulthood. However, many patients still die prematurely from respiratory insufficiency. Hence, further investigations to improve these therapies are important and might have relevance for other diseases-eg, exploring how to increase airway hydration, how to safely downscale the increased inflammatory response in the lung, and how to better combat lung infections associated with cystic fibrosis. In parallel, development of modulators that target the underlying dysfunction in the cystic fibrosis transmembrane conductance regulator (CFTR) is fast moving forward. Existing treatments are specific to certain mutations, or mutation class, in CFTR. An effective, although not yet entirely corrective, treatment is available for patients with class III mutations, and a treatment with modest effectiveness is available for patients who are homozygous for Phe508del, albeit at a very high cost. Corrective treatments that are non-specific to mutation class and thus applicable to all patients-eg, gene therapy, cell-based therapies, and activation of alternative ion channels that bypass CFTR-are being explored, but they are still in early stages of development. In view of the large number of patients with very rare mutations, a plan to advance personalised biomarkers to predict treatment effect is also being investigated and validated. PMID:27053340

  15. Eosinophilic Angiocentric Fibrosis of the Nasal Septum

    Directory of Open Access Journals (Sweden)

    Yunchuan Li

    2013-01-01

    Full Text Available Background. Eosinophilic angiocentric fibrosis (EAF is a rare benign condition of unknown aetiology that causes stenosis of the upper respiratory tract. It is most commonly found at the nasal septum and sinus mucosa causing mucosal thickening and nasal obstructive symptoms. The diagnosis is mainly based on characteristic histologic findings. Case Report. A 27-year-old young woman presented with a slow growing mass at her anterior nasal septum for over eight years. She complained of persistent nasal obstruction, epistaxis, sometimes diffused facial pain, and chronic headache. 3 years ago, the tumor was partially resected for ventilation and a nasal septum perforation was left. Imaging findings indicated soft-tissue thickening of the anterior part of septum and adjacent lateral nasal walls. Pathological examination showed numerous inflammatory cells infiltrates containing eosinophils, fibroinflammatory lesion with a whorled appearance fibrosis which typically surrounded vessels. A diagnosis of eosinophilic angiocentric fibrosis was made. All laboratory tests were unremarkable. Skin prick test was positive. The tumor-like lesion was totally resected. Conclusions. EAF is a rare benign and progressive disorder causing destruction. Combined with radiological imaging of EAF historical findings contribute to the diagnosis. It is important to prevent tumor from recurrence by total resection of the lesion.

  16. Promising Therapy Candidates for Liver Fibrosis

    Directory of Open Access Journals (Sweden)

    Ping eWang

    2016-02-01

    Full Text Available Liver fibrosis is a wound-healing process in response to repeated and chronic injury to hepatocytes and/or cholangiocytes. Ongoing hepatocyte apoptosis or necrosis lead to increase in ROS production and decrease in antioxidant activity, which recruits inflammatory cells from the blood and activate hepatic stellate cells changing to myofibroblasts. Injury to cholangiocytes also recruits inflammatory cells to the liver and activates portal fibroblasts in the portal area, which release molecules to activate and amplify cholangiocytes. No matter what origin of myofibroblasts, either hepatic stellate cells or portal fibroblasts, they share similar characteristics, including being positive for -smooth muscle actin and sproducing extra cellular matrix. Based on the extensive pathogenesis knowledge of liver fibrosis, therapeutic strategies have been designed to target each step of this process, including hepatocyte apoptosis, cholangiocyte proliferation, inflammation, and activation of myofibroblasts to deposit extracellular matrix, yet the current therapies are still in early-phase clinical development. There is an urgent need to translate the molecular mechanism of liver fibrosis to effective and potent reagents or therapies in human.

  17. Molecular mechanisms in progressive idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Steele, Mark P; Schwartz, David A

    2013-01-01

    There is clear evidence that environmental exposures and genetic predisposition contribute to the pathogenesis of idiopathic pulmonary fibrosis (IPF). Cigarette smoking increases the risk of developing IPF several-fold, as do other exposures such as metal-fume and wood-dust exposure. Occupations that increase the risk of IPF are agricultural work, hairdressing, and stone polishing, supporting the role of environmental exposure in disease pathogenesis. Genetic predisposition to IPF is evident from its familial aggregation and the fact that pulmonary fibrosis develops in several rare genetic disorders. Mutations in surfactant proteins lead to pulmonary fibrosis and are associated with endoplasmic reticulum stress in alveolar type II epithelial cells. Mutations in telomerase have been found in several families with IPF, and shortened telomeres are found in sporadic cases of IPF. A common variant in mucin 5B predisposes to both familial and sporadic IPF and is present in the majority of cases, indicating sporadic IPF occurs in those with genetic predisposition. PMID:23020878

  18. Presentación clínica de la Trisomía 13 Mosaico con mayor pronóstico de vida. Reporte de un caso.

    Directory of Open Access Journals (Sweden)

    Gabriel Abudinén A.

    2013-04-01

    Full Text Available INTRODUCCIÓN: La trisomía del cromosoma 13, antes llamado Síndrome de Patau, es una enfermedad genética que resulta de la presencia de un cromosoma 13 supernumerario. Fue descubierta en 1960 por el Dr. Klaus Patau y actualmente es la trisomía reportada menos frecuente en la especie humana. Se suele asociar con un problema meiótico materno más que paterno y, como el síndrome de Down, el riesgo aumenta con la edad de la mujer. Los afectados mueren poco tiempo después de nacer, la mayoría a los 3 meses de edad. Entre el 80-90% de los fetos con el síndrome no llegan a término. PRESENTACIÓN DEL CASO: Se presenta el caso de un recién nacido (RN con diagnóstico de trisomía 13, asociado a malformaciones características de la trisomía, destacando la Tetralogia de Fallot y la laringotraqueomalacia. Al nacimiento, evoluciona con múltiples complicaciones secundarias a su patología de base, interfiriendo con la evolución y pronóstico de la enfermedad. El pronóstico de vida se relaciona claramente con la gravedad de las malformaciones cerebrales, renales y cardiacas; que a su vez se relacionan con el grado de alteración cromosómica que presenta el individuo, siendo la menos complicada el mosaicismo, como se describirá más adelante. DISCUSIÓN: Últimamente la visibilidad de los casos de trisomía 13 han aumentado por la mayor práctica en el diagnóstico de este mismo y además de su sobrevida por las nuevas intervenciones que se han descubierto en la medicina.

  19. 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.

  20. Signaling pathways in the epithelial origins of pulmonary fibrosis.

    Science.gov (United States)

    Hardie, William D; Hagood, James S; Dave, Vrushank; Perl, Anne-Karina T; Whitsett, Jeffrey A; Korfhagen, Thomas R; Glasser, Stephan

    2010-07-15

    Pulmonary fibrosis complicates a number of disease processes and leads to substantial morbidity and mortality. Idiopathic pulmonary fibrosis (IPF) is perhaps the most pernicious and enigmatic form of the greater problem of lung fibrogenesis with a median survival of three years from diagnosis in affected patients. In this review, we will focus on the pathology of IPF as a model of pulmonary fibrotic processes, review possible cellular mechanisms, review current treatment approaches and review two transgenic mouse models of lung fibrosis to provide insight into processes that cause lung fibrosis. We will also summarize the potential utility of signaling pathway inhibitors as a future treatment in pulmonary fibrosis. Finally, we will present data demonstrating a minimal contribution of epithelial-mesenchymal transition in the development of fibrotic lesions in the transforming growth factor-alpha transgenic model of lung fibrosis. PMID:20676040

  1. Multiple pulmonary hyalinizing granulomas associated with systemic idiopathic fibrosis.

    Science.gov (United States)

    Kuramochi, S; Kawai, T; Yakumaru, K; Mikata, A; Torikata, C; Kasuga, Y; Fujiwara, T

    1991-05-01

    A 41-year-old man with progressive nodular infiltration of the lung of about 2 years' duration died of cardiac and respiratory failure. Autopsy revealed bilateral multiple pulmonary hyalinizing granulomas (PHGs) diagnosed on the basis of the characteristic dense hyaline collagen bundles with nonspecific inflammatory infiltration. Constrictive pericarditis, retroperitoneal fibrosis, mediastinal fibrosis, fibrous thickening of the peritoneal and pleural surfaces, and fibrosis of soft tissue of the neck, flank, and hepatic hilar region were present, therefore, a diagnosis of systemic idiopathic fibrosis was made. The patient had anti-thyroglobulin and anti-thyroid microsomal antibodies and lymphocytic thyroiditis. The inflammatory process of PHG of the present case was active and the clinical course was progressive. PHG seems to be a lesion belonging to the systemic idiopathic fibrosis complex. Immunologic abnormalities may be related to PHG and systemic idiopathic fibrosis. PMID:1714226

  2. 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.

  3. Pregnancy and cystic fibrosis: Approach to contemporary management.

    Science.gov (United States)

    Geake, James; Tay, George; Callaway, Leonie; Bell, Scott C

    2014-12-01

    Over the previous 50 years survival of patients with cystic fibrosis has progressively increased. As a result of improvements in health care, increasing numbers of patients with cystic fibrosis are now considering starting families of their own. For the health care professionals who look after these patients, the assessment of the potential risks, and the process of guiding prospective parents through pregnancy and beyond can be both challenging and rewarding. To facilitate appropriate discussions about pregnancy, health care workers must have a detailed understanding of the various important issues that will ultimately need to be considered for any patient with cystic fibrosis considering parenthood. This review will address these issues. In particular, it will outline pregnancy outcomes for mothers with cystic fibrosis, issues that need to be taken into account when planning a pregnancy and the management of pregnancy for mothers with cystic fibrosis or mothers who have undergone organ transplantation as a result of cystic fibrosis. PMID:27512443

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

    Directory of Open Access Journals (Sweden)

    LA. Díaz Robredo

    2014-06-01

    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.

  5. preescolares desnutridos con madres con obesidad y sin obesidad

    Directory of Open Access Journals (Sweden)

    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.

  6. Capillary remodeling in bleomycin-induced pulmonary fibrosis.

    OpenAIRE

    Schraufnagel, D. E.; Mehta, D.; Harshbarger, R.; Treviranus, K.; Wang, N. S.

    1986-01-01

    Lung fibrosis is a process in which collagen is laid down and the delicate capillary-alveolar relationship is disturbed. The architectural changes which occur in the capillaries, a main element of the oxygen transferring unit, are difficult to illustrate without a three-dimensional tool, such as scanning electron microscopy. Therefore, a scanning electron microscopic study was undertaken to show the capillary changes of lung fibrosis. Fibrosis was induced in rats by intratracheal instillation...

  7. Immunohistochemical localisation of advanced glycation end products in pulmonary fibrosis.

    OpenAIRE

    Matsuse, T.; Ohga, E.; Teramoto, S.; Fukayama, M; Nagai, R.; Horiuchi, S; Ouchi, Y.

    1998-01-01

    AIM: To investigate the presence and distribution of advanced glycation end products (AGE) in pulmonary fibrosis. METHODS: Lung tissue samples obtained from seven necropsy cases with idiopathic pulmonary fibrosis and seven with normal pulmonary parenchyma were examined immunohistochemically with a monoclonal antibody specific for AGE: 6D12. We also tested three cases with diffuse alveolar damage. RESULTS: All the specimens from cases with pulmonary fibrosis and diffuse alveolar damage showed ...

  8. Atrial Fibrosis and the Mechanisms of Atrial Fibrillation

    OpenAIRE

    Everett, Thomas H; Olgin, Jeffrey E.

    2006-01-01

    Atrial fibrillation (AF) is commonly associated with congestive heart failure (CHF), and CHF has been shown to be associated with atrial structural remodeling resulting in fibrosis. This atrial interstitial fibrosis has been seen in patients with CHF and animal models of pacing induced heart failure. With atrial fibrosis, conduction abnormalities result in an increase in AF vulnerability. The mechanism of AF that is associated with CHF is still under debate as both focal and reentrant mechani...

  9. Triptolide ameliorates colonic fibrosis in an experimental rat model

    OpenAIRE

    Tao, Qingsong; Wang, Baochai; Zheng, Yu; Li, Guanwei; Ren, Jianan

    2015-01-01

    Triptolide is known to exert anti-inflammatory and immunomodulatory activities; however, its impact on intestinal fibrosis has not been previously examined. Based on our previous studies of the suppressive activity of triptolide on human colonic subepithelial myofibroblasts and the therapeutic efficacy of triptolide in Crohn’s disease, it was hypothesized that triptolide may have beneficial effects on intestinal fibrosis. In the present study, colonic fibrosis was induced in rats by 6 weekly ...

  10. Hypertonic Saline in Treatment of Pulmonary Disease in Cystic Fibrosis

    OpenAIRE

    Reeves, Emer P; Kevin Molloy; Kerstin Pohl; McElvaney, Noel G

    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....

  11. Pulmonary abnormalities in obligate heterozygotes for cystic fibrosis.

    OpenAIRE

    Davis, P B; Vargo, K

    1987-01-01

    Parents of children with cystic fibrosis have been reported to have a high prevalence of increased airway reactivity, but these studies were done in a select young, healthy, symptomless population. In the present study respiratory symptoms were examined in 315 unselected parents of children with cystic fibrosis and 162 parents of children with congenital heart disease (controls). The cardinal symptom of airway reactivity, wheezing, was somewhat more prevalent in cystic fibrosis parents than i...

  12. Combined Pulmonary Fibrosis and Emphysema Syndrome: A Review

    OpenAIRE

    Jankowich, Matthew D.; Rounds, Sharon I. S.

    2012-01-01

    There is increasing clinical, radiologic, and pathologic recognition of the coexistence of emphysema and pulmonary fibrosis in the same patient, resulting in a clinical syndrome known as combined pulmonary fibrosis and emphysema (CPFE) that is characterized by dyspnea, upper-lobe emphysema, lower-lobe fibrosis, and abnormalities of gas exchange. This syndrome frequently is complicated by pulmonary hypertension, acute lung injury, and lung cancer. The CPFE syndrome typically occurs in male smo...

  13. Morphometric and Visual Evaluation of Fibrosis in Renal Biopsies

    OpenAIRE

    Farris, Alton B.; Adams, Catherine D.; Brousaides, Nicole; Della Pelle, Patricia A.; Collins, A. Bernard; Moradi, Ellie; Smith, R. Neal; Grimm, Paul C.; Colvin, Robert B.

    2011-01-01

    Interstitial fibrosis is an outcome measure of increasing importance in clinical trials of both renal transplantation and native disease, but data on the comparative advantages of fibrosis measurement methods are limited. We compared four morphometric techniques and contrasted these with two visual fibrosis-scoring methods on trichrome-stained slides. Two morphometric methods included whole-slide digital images: collagen III immunohistochemistry and a new technique using trichrome and periodi...

  14. Myocardial fibrosis in desmin-related hypertrophic cardiomyopathy

    OpenAIRE

    Dai Qinyi; Hong Daojun; Zhang Zhaoqi; He Yi; Jiang Tengyong

    2010-01-01

    Abstract Desmin-related myopathy (DRM) is known to cause different types of cardiomyopathy. Late gadolinium enhancement cardiovascular magnetic resonance (CMR) has been shown to identify fibrosis in ischemic and non-ischemic cardiomyopathies. We present a rare case of desmin-related hypertrophic cardiomyopathy, CMR revealed fibrosis in the lateral wall of the left ventricle. CMR is superior to conventional echocardiography for the detection of myocardial fibrosis in desmin-related cardiomyopa...

  15. 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

  16. 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.

  17. 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

  18. Characterizing Mucous Cell Remodeling in Cystic Fibrosis: Relationship to Neutrophils

    OpenAIRE

    Hays, Steven R.; John V Fahy

    2006-01-01

    Rationale: Relatively few studies have characterized mucous cells or mucins in detail in cystic fibrosis (CF), and the relationship between mucous cell abnormalities and neutrophilic inflammation is uncertain.

  19. [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.

  20. Inhibition of the Unfolded Protein Response Mechanism Prevents Cardiac Fibrosis

    Science.gov (United States)

    Jung, Joanna; Dyck, Jason R. B.; Lopaschuk, Gary D.; Agellon, Luis B.; Michalak, Marek

    2016-01-01

    Background Cardiac fibrosis attributed to excessive deposition of extracellular matrix proteins is a major cause of heart failure and death. Cardiac fibrosis is extremely difficult and challenging to treat in a clinical setting due to lack of understanding of molecular mechanisms leading to cardiac fibrosis and effective anti-fibrotic therapies. The objective in this study was to examine whether unfolded protein response (UPR) pathway mediates cardiac fibrosis and whether a pharmacological intervention to modulate UPR can prevent cardiac fibrosis and preserve heart function. Methodology/Principal Findings We demonstrate here that the mechanism leading to development of fibrosis in a mouse with increased expression of calreticulin, a model of heart failure, stems from impairment of endoplasmic reticulum (ER) homeostasis, transient activation of the unfolded protein response (UPR) pathway and stimulation of the TGFβ1/Smad2/3 signaling pathway. Remarkably, sustained pharmacologic inhibition of the UPR pathway by tauroursodeoxycholic acid (TUDCA) is sufficient to prevent cardiac fibrosis, and improved exercise tolerance. Conclusions We show that the mechanism leading to development of fibrosis in a mouse model of heart failure stems from transient activation of UPR pathway leading to persistent remodelling of cardiac tissue. Blocking the activation of the transiently activated UPR pathway by TUDCA prevented cardiac fibrosis, and improved prognosis. These findings offer a window for additional interventions that can preserve heart function. PMID:27441395

  1. Genetic modifiers of nutritional status in cystic fibrosis1234

    OpenAIRE

    Bradley, Gia M; Blackman, Scott M.; Christopher P Watson; Doshi, Vishal K.; Cutting, Garry R

    2012-01-01

    Background: Improved nutrition early in life is associated with better pulmonary function for patients with cystic fibrosis (CF). However, nutritional status is poorly correlated with the CFTR genotype.

  2. 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

    2016-06-01

    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

  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. Proteins and renal fibrosis: low-protein diets induce Kruppel-like factor-15, limiting renal fibrosis

    OpenAIRE

    Wang, Yanlin; Mitch, William E.

    2011-01-01

    The mechanisms underlying beneficial influences of low-protein diets on the progressive nature of chronic kidney disease are incompletely understood. Gao et al. identify a downregulation of Kruppel-like factor-15 (KLF15) associated with increased renal fibrosis. The KLF15 downregulation and renal fibrosis are reversed with dietary protein restriction, especially when ketoacids are added. The results suggest that KLF15 may play a role in suppressing renal fibrosis and could contribute to the b...

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

    OpenAIRE

    Cleveland Robert H; García Christopher; Asher Daniel; Cannon Carolynn L; Levy Hara; Pier Gerald B; Knowles Michael R; Colin Andrew A

    2010-01-01

    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 homoz...

  6. 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.

  7. 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

  8. 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.

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

    Institute of Scientific and Technical Information of China (English)

    MinZheng; Wei-MinCai; Hong-LeiWeng; Rong-HuaLiu

    2002-01-01

    AIM:Use Receiver operating characteristic(ROC) curves to find out the relationship between serum level of hyaluronic acid(HA),typeⅢ procollagen (PCⅢ),N-terminal procollagen Ⅲ peptied(PⅢNP),iaminin(LN),type Ⅳ collagen(C-Ⅳ)and hepatic fibrosis,as well as to determined their value in clinical practice.

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

    DEFF Research Database (Denmark)

    Fluge, G; Olesen, H V; 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...

  11. 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...

  12. 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

  13. 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

  14. 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.

  15. 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

  16. Autoimmune Pancreatitis Associated with Retroperitoneal Fibrosis

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    Ohkawa M

    2005-05-01

    Full Text Available CONTEXT: Autoimmune pancreatitis is sometimes associated with other autoimmune diseases. We have presented two cases of autoimmune pancreatitis with retroperitoneal fibrosis and compared our findings with those found in the literature. CASE 1: A 71-year-old male developed anorexia and weight loss. Abdominal ultrasonography (US and computed tomography (CT showed diffuse swelling of the pancreas and the peritoneal soft tissue surrounding the aorta, associated with right hydronephrosis. Endoscopic retrograde pancreatography showed narrowing of the main pancreatic duct. He was diagnosed as having autoimmune pancreatitis associated with retroperitoneal fibrosis and underwent steroid therapy. After 3 weeks, a follow-up CT showed a marked reduction in the size of both the pancreas and retroperitoneal mass. CASE 2: A 62-year-old male was admitted to another hospital complaining of obstructive jaundice. Abdominal CT and US showed swelling of the pancreas. Endoscopic retrograde cholangiopancreatography demonstrated stenosis of the lower bile duct and narrowing of the main pancreatic duct. With the diagnosis of pancreatic head carcinoma, a choledochojejunostomy and a gastrojejunostomy were performed. Histological examination of the biopsy of the pancreatic mass revealed marked fibrosis with lymphoplasmacytic infiltration. One year later, a retroperitoneal mass was detected on follow-up CT. He was treated with prednisolone for two years. Recurrence of retroperitoneal mass with left hydronephrosis occurred 18 months later. There was no sign of recurrence of the autoimmune pancreatitis. He was again treated with prednisolone, and the retroperitoneal mass was gradually reduced. CONCLUSIONS: A total of 7 cases including the present cases have been reported. All were middle-aged males. Steroid therapy was effective for both the pancreatic and the retroperitoneal masses.

  17. Calentamiento global con Scratch y escuelas eficientes con Arduino

    OpenAIRE

    Ainzua Cemborain, José Ignacio

    2014-01-01

    Este trabajo final de máster está formado por dos proyectos con metodología de aprendizaje basado en proyectos (ABP). El primero de ellos se realiza en la asignatura de Tecnología y en coordinación con la asignatura de Ciencias Naturales, y el segundo únicamente para Tecnología. En la primera parte del proyecto se analiza la metodología ABP utilizada y se compara con la tradicional. Posteriormente se estudian las tres herramientas utilizadas en este proyecto como son; Scratch, Scratch for...

  18. 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.

  19. Cystic Fibrosis: Microbiology and Host Response.

    Science.gov (United States)

    Zemanick, Edith T; Hoffman, Lucas R

    2016-08-01

    The earliest descriptions of lung disease in people with cystic fibrosis (CF) showed the involvement of 3 interacting pathophysiologic elements in CF airways: mucus obstruction, inflammation, and infection. Over the past 7 decades, our understanding of CF respiratory microbiology and inflammation has evolved with the introduction of new treatments, increased longevity, and increasingly sophisticated laboratory techniques. This article reviews the current understanding of infection and inflammation and their roles in CF lung disease. It also discusses how this constantly evolving information is used to inform current therapeutic strategies, measures and predictors of disease severity, and research priorities. PMID:27469179

  20. Cystic Fibrosis Diagnosis and Newborn Screening.

    Science.gov (United States)

    Rosenfeld, Margaret; Sontag, Marci K; Ren, Clement L

    2016-08-01

    The diagnosis of cystic fibrosis (CF) has evolved over the past decade as newborn screening has become universal in the United States and elsewhere. The heterogeneity of phenotypes associated with CF transmembrane conductance regulator (CFTR) dysfunction and mutations in the CFTR gene has become clearer, ranging from classic pancreatic-insufficient CF to manifestations in only 1 organ system to indeterminate diagnoses identified by newborn screening. The tools available for diagnosis have also expanded. This article reviews the newest diagnostic criteria for CF, newborn screening, prenatal screening and diagnosis, and indeterminate diagnoses in newborn-screened infants and symptomatic adults. PMID:27469178

  1. Substance P Modulates Colitis-Asscociated Fibrosis

    OpenAIRE

    Koon, Hon Wai; Shih, David; Karagiannides, Iordanes; Zhao, Dezheng; Fazelbhoy, Zafeer; Hing, Tressia; Xu, Hua; Lu, Bao; Gerard, Norma; Pothoulakis, Charalabos

    2010-01-01

    Substance P (SP) and the neurokinin-1 receptor (NK-1R) are involved in the development of colitis and mucosal healing after colonic inflammation. We studied whether SP modulates colonic fibrosis by using a chronic model of trinitrobenzenesulfonic acid (TNBS)-induced colitis in wild-type (WT) and NK-1R-deficient (NK-1R KD) mice. We found increased mRNA expression levels of collagen, vimentin, and the fibrogenic factors transforming growth factor β1 and insulin-like growth factor 1 in the chron...

  2. Targeting the Root Cause of Cystic Fibrosis.

    Science.gov (United States)

    Trescott, Laura; Holcomb, Joshua; Spellmon, Nicholas; Mcleod, Cathy; Aljehane, Leala; Sun, Fei; Li, Chunying; Yang, Zhe

    2015-01-01

    Cystic Fibrosis (CF) is a serious genetic condition caused by CF transmembrane conductance regulator (CFTR) mutation. CF patients have shortened lifespan due to airway obstruction, infection, and end-stage lung failure. However, recent development in CF therapy suggests a brighter future for CF patients. Targeting specific CFTR mutations aims to potentiate the channel gating activity of impaired CFTR and restore protein trafficking to the plasma membrane. Gene therapy introduces correct CFTR gene into the affected airway epithelium leading to the functional expression of CFTR in CF patients. This review will sum up the current status in CF-cause targeting therapy. PMID:25316272

  3. 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.

  4. Nontuberculous Mycobacterial Infections in Cystic Fibrosis.

    Science.gov (United States)

    Martiniano, Stacey L; Nick, Jerry A; Daley, Charles L

    2016-03-01

    Nontuberculous mycobacteria (NTM) are important emerging cystic fibrosis (CF) pathogens, with estimates of prevalence ranging from 6% to 13%. Diagnosis of NTM disease in patients with CF is challenging, as the infection may remain indolent in some, without evidence of clinical consequence, whereas other patients suffer significant morbidity and mortality. Treatment requires prolonged periods of multiple drugs and varies depending on NTM species, resistance pattern, and extent of disease. The development of a disease-specific approach to the diagnosis and treatment of NTM infection in CF patients is a research priority, as a lifelong strategy is needed for this high-risk population. PMID:26857770

  5. Nutrition and Growth in Cystic Fibrosis.

    Science.gov (United States)

    Lusman, Sarah; Sullivan, Jillian

    2016-08-01

    Close attention to nutrition and growth is essential in caring for children with cystic fibrosis (CF). Growth and nutritional status should be monitored as part of routine CF care. Children with CF should achieve growth and nutritional status comparable with that of well-nourished children without CF. Children with CF are at risk for nutritional deficiencies. Optimal nutritional and growth status may be difficult to attain in this population given risk of insufficient caloric intake and likelihood of increased caloric expenditure. Various methods to attain optimal nutritional status may be used, including oral supplementation, behavioral treatment, pharmacotherapy, and enteral nutrition. PMID:27469181

  6. Vitamin D Deficiency in Cystic Fibrosis

    Directory of Open Access Journals (Sweden)

    William B. Hall

    2010-01-01

    Full Text Available Cystic Fibrosis is the most common inherited genetic respiratory disorder in the Western World. Hypovitaminosis D is almost universal in CF patients, likely due to a combination of inadequate absorption, impaired metabolism, and lack of sun exposure. Inadequate levels are associated with the high prevalence of bone disease or osteoporosis in CF patients, which is associated with increased morbidity including fractures, kyphosis, and worsening pulmonary status. Treatment goals include regular monitoring 25 hydroxyvitamin D (25OHD levels with aggressive treatment for those with levels <75 nmol/L (<30 ng/mL. More research is needed to determine optimal supplementation goals and strategies.

  7. 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.

  8. 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

  9. 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.

  10. OX40L blockade protects against inflammation-driven fibrosis.

    Science.gov (United States)

    Elhai, Muriel; Avouac, Jérôme; Hoffmann-Vold, Anna Maria; Ruzehaji, Nadira; Amiar, Olivia; Ruiz, Barbara; Brahiti, Hassina; Ponsoye, Matthieu; Fréchet, Maxime; Burgevin, Anne; Pezet, Sonia; Sadoine, Jérémy; Guilbert, Thomas; Nicco, Carole; Akiba, Hisaya; Heissmeyer, Vigo; Subramaniam, Arun; Resnick, Robert; Molberg, Øyvind; Kahan, André; Chiocchia, Gilles; Allanore, Yannick

    2016-07-01

    Treatment for fibrosis represents a critical unmet need, because fibrosis is the leading cause of death in industrialized countries, and there is no effective therapy to counteract the fibrotic process. The development of fibrosis relates to the interplay between vessel injury, immune cell activation, and fibroblast stimulation, which can occur in various tissues. Immunotherapies have provided a breakthrough in the treatment of immune diseases. The glycoprotein OX40-OX40 ligand (OX40L) axis offers the advantage of a targeted approach to costimulatory signals with limited impact on the whole immune response. Using systemic sclerosis (SSc) as a prototypic disease, we report compelling evidence that blockade of OX40L is a promising strategy for the treatment of inflammation-driven fibrosis. OX40L is overexpressed in the fibrotic skin and serum of patients with SSc, particularly in patients with diffuse cutaneous forms. Soluble OX40L was identified as a promising serum biomarker to predict the worsening of lung and skin fibrosis, highlighting the role of this pathway in fibrosis. In vivo, OX40L blockade prevents inflammation-driven skin, lung, and vessel fibrosis and induces the regression of established dermal fibrosis in different complementary mouse models. OX40L exerts potent profibrotic effects by promoting the infiltration of inflammatory cells into lesional tissues and therefore the release of proinflammatory mediators, thereafter leading to fibroblast activation. PMID:27298374

  11. Typing of Pseudomonas aeruginosa strains in Norwegian cystic fibrosis patients

    DEFF Research Database (Denmark)

    Fluge, G; Ojeniyi, B; Høiby, N;

    2001-01-01

    OBJECTIVES: Typing of Pseudomonas aeruginosa isolates from Norwegian cystic fibrosis (CF) patients with chronic Pseudomonas lung infection in order to see whether cross-infection might have occurred. METHODS: Isolates from 60 patients were collected during the years 1994-98, and typed by pulsed...... between cystic fibrosis patients has occurred....

  12. 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.

  13. Liver fibrosis caused by choledocholith to regress after biliary drainage

    Institute of Scientific and Technical Information of China (English)

    Zuo-Bing Chen; Shu-Sen Zheng; Guo-Zhi Hu; Yuan Gao; Chen-Yan Ding; Yun Zhang; Xue-Hong Zhao; Lin-Mei Ni

    2005-01-01

    AIM: To study the correlation between liver fibrosis severity and biliary drainage in patients with choledocholith.METHODS: A follow-up study on seven patients with liver fibrosis due to choledocholith was made. The data, including biochemical tests (aspartate aminotransferase, alanine aminotransferase) and liver histological features before and after biliary drainage, were collected and studied. The fibrosis severity was scored on a scale from 0 to 3, with 0 denoting none, 1 portal and periportal fibrosis, 2 the presence of numerous fiber septa, and 3 cirrhosis. The average liver fibrosis severity scores of the first and second biopsy were compared with statistical method.RESULTS: The first, second liver fibrosis severity scores of these seven patients were 2,1; 2,1; 1,0; 1,1; 2,1; 2,1;1,0 respectively. The results showed that the average liver fibrosis severity score of the second liver biopsy decreased significantly compared with the first liver biopsy (n = 7, t = 4.25, P<0.05).CONCLUSION: Liver fibrosis due to choledocholith may regress after biliary drainage.

  14. Infectious and nutritional mechanisms in children with cystic fibrosis

    OpenAIRE

    Diaconu Ramona; Bozomitu Laura; Anton Emil; Popovici Paula; Anton Carmen; Timofte Daniel; Alin Ciobica; Moraru Evelina

    2015-01-01

    Cystic fibrosis is a polymorphic disease characterized by severe genetic dysfunctions. Besides the complex genetic background, most patients with cystic fibrosis also have increased susceptibility to infections and and their nutritional status is affected. Chronic pulmonary infection and gastrointestinal or nutritional abnormalities are characteristics of this disorder. Of our selected 56 subjects, 21.28% presented a pulmonary condition, and 28.57% digestiv...

  15. New insights into pulmonary inflammation in cystic fibrosis

    OpenAIRE

    Rao, S; Grigg, J

    2006-01-01

    Persistent lower airway infection with inflammation is the major cause of morbidity and mortality in cystic fibrosis. This review examines the recent advances in the understanding of airway inflammation in cystic fibrosis, and focuses on the evidence that pulmonary inflammation is, under some circumstances, disassociated from infection, and the potential implications for therapeutic intervention.

  16. 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.

  17. 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...

  18. Myocardial fibrosis in patients with myotonic dystrophy type 1

    DEFF Research Database (Denmark)

    Petri, Helle; Ahtarovski, Kiril Aleksov; Vejlstrup, Niels;

    2014-01-01

    , echocardiography and Holter-monitoring are useful but insufficient. Cardiovascular magnetic resonance (CMR) can provide additional information of which myocardial fibrosis may be relevant. The purpose of this study was to describe the prevalence of myocardial fibrosis in patients with DM1 assessed by CMR......, and the association between myocardial fibrosis and abnormal findings on ECG, Holter-monitoring and echocardiography. METHODS: We selected 30 unrelated patients with DM1: 18 patients (10 men, mean age 51 years) with, and 12 patients (7 men, mean age 41 years) without abnormal findings on ECG and Holter......-monitoring. Patients were evaluated with medical history, physical examination, ECG, Holter-monitoring, echocardiography and CMR. RESULTS: Myocardial fibrosis was found in 12/30 (40%, 9 men). The presence of myocardial fibrosis was associated with the following CMR-parameters: increased left ventricular mass (median...

  19. Cystic fibrosis with normal sweat chloride concentration: case report

    Directory of Open Access Journals (Sweden)

    Silva Filho Luiz Vicente Ferreira da

    2003-01-01

    Full Text Available Cystic fibrosis is a genetic disease usually diagnosed by abnormal sweat testing. We report a case of an 18-year-old female with bronchiectasis, chronic P. aeruginosa infection, and normal sweat chloride concentrations who experienced rapid decrease of lung function and clinical deterioration despite treatment. Given the high suspicion ofcystic fibrosis, broad genotyping testing was performed, showing a compound heterozygous with deltaF508 and 3849+10kb C->T mutations, therefore confirming cystic fibrosis diagnosis. Although the sweat chloride test remains the gold standard for the diagnosis of cystic fibrosis, alternative diagnostic tests such as genotyping and electrophysiologic measurements must be performed if there is suspicion of cystic fibrosis, despite normal or borderline sweat chloride levels.

  20. Pseudomonal infection in cystic fibrosis: the battle continues.

    Science.gov (United States)

    Elkin, Sarah; Geddes, Duncan

    2003-12-01

    Pseudomonas aeruginosa lung infection is the major cause of morbidity and mortality in patients with cystic fibrosis. Infection usually begins in childhood and is responsible for respiratory failure and death in most patients with cystic fibrosis. The organism triggers an exuberant chronic inflammatory reaction which damages the airways and leads to progressive loss of lung function. Over the last decade significant advances have been made in the understanding of the pathophysiology of cystic fibrosis airways disease. These should assist the development of new and better therapies to treat this pathogen. This review provides an overview of pseudomonal infection in cystic fibrosis, including mechanisms by which the bacteria may colonize the cystic fibrosis airway, persistence of pseudomonal infection and the biofilm mode of growth. Available treatments and possible novel approaches to therapy will be discussed. PMID:15482158

  1. 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.

  2. 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.

  3. Fibrosis and progression of autosomal dominant polycystic kidney disease (ADPKD).

    Science.gov (United States)

    Norman, Jill

    2011-10-01

    The age on onset of decline in renal function and end-stage renal disease (ESRD) in autosomal polycystic kidney disease (ADPKD) is highly variable and there are currently no prognostic tools to identify patients who will progress rapidly to ESRD. In ADPKD, expansion of cysts and loss of renal function are associated with progressive fibrosis. Similar to the correlation between tubulointerstitial fibrosis and progression of chronic kidney disease (CKD), in ADPKD, fibrosis has been identified as the most significant manifestation associated with an increased rate of progression to ESRD. Fibrosis in CKD has been studied extensively. In contrast, little is known about the mechanisms underlying progressive scarring in ADPKD although some commonality may be anticipated. Current data suggest that fibrosis associated with ADPKD shares at least some of the "classical" features of fibrosis in CKD (increased interstitial collagens, changes in matrix metalloproteinases (MMPs), over-expression of tissue inhibitor of metalloproteinase-1 (TIMP-1), over-expression of plasminogen activator inhibitor-1 (PAI-1) and increased transforming growth factor beta (TGFβ) but that there are also some unique and stage-specific features. Epithelial changes appear to precede and to drive interstitial changes leading to the proposal that development of fibrosis in ADPKD is biphasic with alterations in cystic epithelia precipitating changes in interstitial fibroblasts and that reciprocal interactions between these cell types drives progressive accumulation of extracellular matrix (ECM). Since fibrosis is a major component of ADPKD it follows that preventing or slowing fibrosis should retard disease progression with obvious therapeutic benefits. The development of effective anti-fibrotic strategies in ADPKD is dependent on understanding the precise mechanisms underlying initiation and progression of fibrosis in ADPKD and the role of the intrinsic genetic defect in these processes. This article is

  4. 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.

  5. 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.

  6. 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.

  7. 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.

  8. 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.

  9. [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).

  10. 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. PMID:26115565

  11. Natural Killer cells and liver fibrosis

    Directory of Open Access Journals (Sweden)

    Frank eFasbender

    2016-01-01

    Full Text Available In the 40 years since the discovery of Natural Killer (NK cells it has been well established that these innate lymphocytes are important for early and effective immune responses against transformed cells and infections with different pathogens. In addition to these classical functions of NK cells, we now know that they are part of a larger family of innate lymphoid cells and that they can even mediate memory-like responses. Additionally, tissue resident NK cells with distinct phenotypical and functional characteristics have been identified. Here we focus on the phenotype of different NK cell subpopulations that can be found in the liver and summarize the current knowledge about the functional role of these cells with a special emphasis on liver fibrosis. NK cell cytotoxicity can contribute to liver damage in different forms of liver disease. However, NK cells can limit liver fibrosis by killing hepatic stellate cell-derived myofibroblasts, which play a key role in this pathogenic process. Therefore, liver NK cells need to be tightly regulated in order to balance these beneficial and pathological effects.

  12. PROFILEing idiopathic pulmonary fibrosis: rethinking biomarker discovery.

    Science.gov (United States)

    Maher, Toby M

    2013-06-01

    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. PMID:23728868

  13. Imaging: how to recognise idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Anand Devaraj

    2014-06-01

    Full Text Available It is well known that high-resolution computed tomography (HRCT is an essential component of the diagnostic pathway in idiopathic pulmonary fibrosis (IPF. Honeycombing, a common feature of IPF seen on HRCT, is crucial for an accurate diagnosis. Unfortunately, identification of honeycombing is not always straightforward, and there is some disagreement regarding its imaging features. It can be difficult to distinguish honeycombing from traction bronchiectasis and emphysema, although several imaging characteristics can be helpful. Recently, there has been an interest in expanding the use of HRCT beyond diagnosis for disease monitoring and prognostication, and several studies have provided valuable contributions in this regard. Traction bronchiectasis and the extent of fibrosis, for example, have been reported to be powerful prognostic predictors for mortality. Finally, considering the difficulties in diagnosis of “possible usual interstitial pneumonia”, clinicians should always be aware that clinical factors must be considered together with HRCT in order to reach an accurate diagnosis and provide appropriate treatment.

  14. 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.

  15. Pirfenidone treatment of idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Ye Gan

    2011-02-01

    Full Text Available Ye Gan1,2, Erica L Herzog2, Richard H Gomer31Department of Medicine, Central South University, Changsha, Hunan, China; 2Department of Medicine, Yale University School of Medicine, New Haven, CT, USA; 3Department of Biology, Texas A&M University, College Station, TX, USAAbstract: Idiopathic pulmonary fibrosis (IPF is a discrete clinicopathologic entity defined by the presence of usual interstitial pneumonia on high-resolution CT scan and/or open lung biopsy and the absence of an alternate diagnosis or exposure explaining these findings. There are currently no FDA-approved therapies available to treat this disease, and the 5-year mortality is ~80%. The pyridone derivative pirfenidone has been studied extensively as a possible therapeutic agent for use in this deadly disease. This review will present the unique clinical features and management issues encountered by physicians caring for IPF patients, including the poor response to conventional therapy. The biochemistry and preclinical efficacy of pirfenidone will be discussed along with a comprehensive review of the clinical efficacy, safety, and side effects and patient-centered foci such as quality of life and tolerability. It is hoped that this information will lend insight into the complex issues surrounding the use of pirfenidone in IPF and lead to further investigation of this agent as a possible therapy in this devastating disease.Keywords: pirfenidone, fibrosis, clinical trials 

  16. [CYSTIC FIBROSIS: CARE OF THE LUNG DISEASE].

    Science.gov (United States)

    Hubert, Dominique

    2015-10-01

    (Rh-DNase) and/or hydration (hypertonic saline) nebulisations, Moreover, treatment with inhaled antibiotics is indicated (tobramycin, colistine or aztreonam lysine) for chronic lung infection with Pseudomonas aeruginosa (PA). The treatment regimen also includes bronchodilators for bronchospasms and azithromycin. Regular physical activity is recommended. A treatment potentiating the CFTR protein, ivacaftor, is now indicated for patients with a class 3 mutation. Initial bronchial infection with PA must be treated as soon as possible in order to eradicate the pathogen. Pulmonary exacerbations require antibiotic courses, either orally or intravenously for PA infection. Complications require hospitalisation, with thoracic chest tube placement for a pneumothorax or bronchial artery embolisation for massive hemoptysis. Oxygen therapy and non-invasive ventilation with a nasal mask become necessary when respiratory insufficiency progresses, justifying the initiation of the lung transplant process. Lung disease affects the prognosis of cystic fibrosis, therefore its management in cystic fibrosis centres is of utmost importance. Maintenance treatment mainly relies on daily chest physiotherapy, which can be facilitated by mucolytic PMID:26749716

  17. 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.

  18. Managing comorbidities in idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Fulton BG

    2015-09-01

    Full Text Available Blair G Fulton,1 Christopher J Ryerson1,2 1Department of Medicine, 2Centre for Heart Lung Innovation, University of British Columbia, Vancouver, BC, Canada Abstract: Major risk factors for idiopathic pulmonary fibrosis (IPF include older age and a history of smoking, which predispose to several pulmonary and extra-pulmonary diseases. IPF can be associated with additional comorbidities through other mechanisms as either a cause or a consequence of these diseases. We review the literature regarding the management of common pulmonary and extra-pulmonary comorbidities, including chronic obstructive pulmonary disease, lung cancer, pulmonary hypertension, venous thromboembolism, sleep-disordered breathing, gastroesophageal reflux disease, coronary artery disease, depression and anxiety, and deconditioning. Recent studies have provided some guidance on the management of these diseases in IPF; however, most treatment recommendations are extrapolated from studies of non-IPF patients. Additional studies are required to more accurately determine the clinical features of these comorbidities in patients with IPF and to evaluate conventional treatments and management strategies that are beneficial in non-IPF populations. Keywords: interstitial lung disease, management, idiopathic pulmonary fibrosis, comorbidities

  19. 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.)

  20. 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.

  1. Abdominal manifestations of cystic fibrosis in children

    International Nuclear Information System (INIS)

    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.)

  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. 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

  4. Colagenosis con afectación dermatológica en edades pediátricas en Las Tunas, 2010 - 2012

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    Katiuska Tamayo Mariño

    2014-08-01

    Full Text Available El estudio de las enfermedades del colágeno en edades pediátricas es relativamente joven y no se cuenta en Las Tunas con archivos que permitan evaluar su comportamiento, por ello se realizó un estudio descriptivo, transversal y prospectivo en el período comprendido entre los años 2010 al 2012, para caracterizar a los pacientes ingresados con estas enfermedades en el hospital pediátrico Mártires de Las Tunas. Inicialmente se determinaron las conectivopatias diagnosticadas con más frecuencia y luego se estudiaron las variables: sexo, edad, color de la piel, procedencia geográfica y antecedentes patológicos familiares de colagenosis, Los resultados fueron expuestos en tablas y expresados en porcientos. Predominaron la artritis idiopática juvenil, seguido de la fiebre reumática y la morfea. El sexo más frecuente fue el femenino, el grupo de edad más afectado fueron los adolescentes mayores de 13 años, el color de la piel que prevaleció fue el blanco y predominaron los pacientes sin antecedentes patológicos familiares de colagenosis

  5. Monocyte Subsets in Schistosomiasis Patients with Periportal Fibrosis

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    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.

  6. Effects of Heterogeneous Diffuse Fibrosis on Arrhythmia Dynamics and Mechanism.

    Science.gov (United States)

    Kazbanov, Ivan V; ten Tusscher, Kirsten H W J; Panfilov, Alexander V

    2016-01-01

    Myocardial fibrosis is an important risk factor for cardiac arrhythmias. Previous experimental and numerical studies have shown that the texture and spatial distribution of fibrosis may play an important role in arrhythmia onset. Here, we investigate how spatial heterogeneity of fibrosis affects arrhythmia onset using numerical methods. We generate various tissue textures that differ by the mean amount of fibrosis, the degree of heterogeneity and the characteristic size of heterogeneity. We study the onset of arrhythmias using a burst pacing protocol. We confirm that spatial heterogeneity of fibrosis increases the probability of arrhythmia induction. This effect is more pronounced with the increase of both the spatial size and the degree of heterogeneity. The induced arrhythmias have a regular structure with the period being mostly determined by the maximal local fibrosis level. We perform ablations of the induced fibrillatory patterns to classify their type. We show that in fibrotic tissue fibrillation is usually of the mother rotor type but becomes of the multiple wavelet type with increase in tissue size. Overall, we conclude that the most important factor determining the formation and dynamics of arrhythmia in heterogeneous fibrotic tissue is the value of maximal local fibrosis. PMID:26861111

  7. 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

  8. Brote de shigellosis con 146 casos relacionado con una feria Shigellosis outbreak with 146 cases related to a fair

    Directory of Open Access Journals (Sweden)

    Juan Castell Monsalve

    2008-02-01

    Full Text Available Fundamento: El 3 de septiembre de 2005, el Servicio de Salud Pública de Ciudad Real conoció la existencia de 20 casos de gastroenteritis en el municipio de Daimiel. Se inició una investigación, con los objetivos de determinar las causas y establecer medidas de control. La mayor parte de los afectados eran jóvenes que habían visitado la feria de la localidad. Métodos: Se llevó a cabo un estudio descriptivo y otro analítico de casos y controles. En el descriptivo se incluyeron todas las variables de interés disponibles en las historias clínicas de los pacientes. Se diseñó un estudio de casos y controles apareado por edad (más o menos 5 años, sexo y asistencia a la feria, que incluyó a 65 casos y a 65 controles. Se llevaron a cabo inspecciones de establecimientos y toma de muestras clínicas y de alimentos. Resultados: Se notificaron 196 casos, de los cuales 146 fueron confirmados. La curva epidémica sugiere una fuente común de corta duración. El estudio de casos y controles apareado muestra una asociación con el consumo de patatas con salsa en uno de los chiringuitos (odds ratio [OR] = 20,56; intervalo de confianza [IC] del 95%, 6,15-75,93; p Background: On September 3, 2005, the Ciudad Real Public Health Service (Spain received a report of 20 cases of gastroenteritis in the municipality of Daimiel. We conducted an investigation to determine the cause or causes of the outbreak and to implement control measures. Most of the cases involved young people who visited the municipality's fair. Methods: We carried out a descriptive study and an analytic case-control study. In the descriptive study, all variables of interest available in the medical records were included. In the case-control study, each case was matched with a control by age (plus or minus 5 years, gender, and attendance at the fair. Sixty-five cases and 65 controls were finally included in the study. Samples of foods and stools from food handlers were taken. Results

  9. Cellular Mechanisms of Tissue Fibrosis. 3. Novel mechanisms of kidney fibrosis

    OpenAIRE

    Campanholle, Gabriela; Ligresti, Giovanni; Gharib, Sina A; Duffield, Jeremy S.

    2013-01-01

    Chronic kidney disease, defined as loss of kidney function for more than three months, is characterized pathologically by glomerulosclerosis, interstitial fibrosis, tubular atrophy, peritubular capillary rarefaction, and inflammation. Recent studies have identified a previously poorly appreciated, yet extensive population of mesenchymal cells, called either pericytes when attached to peritubular capillaries or resident fibroblasts when embedded in matrix, as the progenitors of scar-forming ce...

  10. Cellular Mechanisms of Tissue Fibrosis. 6. Purinergic signaling and response in fibroblasts and tissue fibrosis

    OpenAIRE

    Lu, David; Insel, Paul A.

    2013-01-01

    Tissue fibrosis occurs as a result of the dysregulation of extracellular matrix (ECM) synthesis. Tissue fibroblasts, resident cells responsible for the synthesis and turnover of ECM, are regulated via numerous hormonal and mechanical signals. The release of intracellular nucleotides and their resultant autocrine/paracrine signaling have been shown to play key roles in the homeostatic maintenance of tissue remodeling and in fibrotic response post-injury. Extracellular nucleotides signal throug...

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

    International Nuclear Information System (INIS)

    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.)

  12. Combined Pulmonary Fibrosis and Emphysema Alters Physiology but Has Similar Mortality to Pulmonary Fibrosis Without Emphysema

    OpenAIRE

    Jankowich, Matthew D.; Rounds, Sharon

    2010-01-01

    Studies have described individuals with combined pulmonary fibrosis and emphysema (CPFE), with preserved lung volumes, significant reductions in gas exchange, and high prevalence of pulmonary hypertension. While physiologic changes in CPFE are well documented, there is little mortality data in the CPFE population compared to appropriate controls. A study was performed to determine the features and outcomes of a group of individuals with imaging and/or pathologic evidence of CPFE to determine ...

  13. 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.)

  14. 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.

  15. Liver and spleen volume variations in patients with hepatic fibrosis

    Institute of Scientific and Technical Information of China (English)

    Peng Liu; Peng Li; Wen He; Li-in Zhao

    2009-01-01

    AIM: To study the liver and spleen volume variations in hepatic fibrosis patients at different histopathological stages.METHODS: Multidetector computed tomography (MDCT) scan was performed in 85 hepatic fibrosis patients. Liver volume (LV) and spleen volume (SV) were measured. Fifteen healthy individuals served as a control group (S0). The patients were divided into stage 1 (S1) group ( n = 34), stage 2 (S2) group ( n = 25), stage 3 (S3) group ( n = 16), and stage 4 (S4)group ( n = 10) according to their histopathological stage of liver fibrosis.RESULTS: The LV and standard LV(SLV)had a tendency to increase with the severity of fibrosis, but no statistical difference was observed in the 5 groups (LV: F = 0.245, P = 0.912; SLV: F = 1.902,P = 0.116). The SV was gradually increased with the severity of fibrosis, and a statistically significant difference in SV was observed among the 5 groups( P < 0.01). The LV/SV ratio and SLV/SV ratio were gradually decreased with the aggravation of hepatic fibrosis, and statistically significant differences in both LV/SV and SLV/SV were found among the 5 groups ( P< 0.01).CONCLUSION: The absence of obvious LV reduction in patients with chronic liver disease may be a morphological index of patients without liver cirrhosis. The SV is related to the severity of fibrosis, and the spleen of patients with advanced fibrosis is enlarged evidently. The LV/SV ratio and SLV/SV ratio are of a significant clinical value in the diagnosis of advanced liver fibrosis.

  16. Accuracy of FibroScan for diagnosing liver fibrosis

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    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.

  17. 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.

  18. 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

  19. Cellular mechanisms of tissue fibrosis. 6. Purinergic signaling and response in fibroblasts and tissue fibrosis.

    Science.gov (United States)

    Lu, David; Insel, Paul A

    2014-05-01

    Tissue fibrosis occurs as a result of the dysregulation of extracellular matrix (ECM) synthesis. Tissue fibroblasts, resident cells responsible for the synthesis and turnover of ECM, are regulated via numerous hormonal and mechanical signals. The release of intracellular nucleotides and their resultant autocrine/paracrine signaling have been shown to play key roles in the homeostatic maintenance of tissue remodeling and in fibrotic response post-injury. Extracellular nucleotides signal through P2 nucleotide and P1 adenosine receptors to activate signaling networks that regulate the proliferation and activity of fibroblasts, which, in turn, influence tissue structure and pathologic remodeling. An important component in the signaling and functional responses of fibroblasts to extracellular ATP and adenosine is the expression and activity of ectonucleotideases that attenuate nucleotide-mediated signaling, and thereby integrate P2 receptor- and subsequent adenosine receptor-initiated responses. Results of studies of the mechanisms of cellular nucleotide release and the effects of this autocrine/paracrine signaling axis on fibroblast-to-myofibroblast conversion and the fibrotic phenotype have advanced understanding of tissue remodeling and fibrosis. This review summarizes recent findings related to purinergic signaling in the regulation of fibroblasts and the development of tissue fibrosis in the heart, lungs, liver, and kidney. PMID:24352335

  20. Ciclofosfamida oral metronómica en combinación con prednisona tras la progresión a docetaxel en pacientes afectados de un cáncer de próstata metastásico resistente a la castración (CPRC).

    OpenAIRE

    Morales Barrera, Rafael; Galcerán, Joan Carles

    2011-01-01

    Estudi retrospectiu que avalua eficàcia i tolerabilitat de ciclofosfamida oral metronòmica (COM) més prednisona en CPRC com a segona línia de tractament després de la progressió a docetaxel. Este estudio retrospectivo evalúa eficacia y tolerabilidad de ciclofosfamida oral metronómica más prednisona en CPRC como segunda línea de tratamiento tras la progresión a docetaxel.

  1. con mala calidad de vida

    Directory of Open Access Journals (Sweden)

    Agustín Martín-Rodríguez

    2007-01-01

    Full Text Available En este estudio ex post facto se ha analizado si los familiares de pacientes con mala calidad de vida presentan diferencias en las variables clínicas de personalidad y relaciones familiares en función de que el paciente haya estado o no ingresado en una Unidad de Cuidados Intensivos. Seleccionamos dos grupos: 29 familiares de pacientes traumatizados graves transcurridos cuatro años de su ingreso en una UCI de Traumatología y con mala calidad de vida (debido a secuelas físicas y/o psicológicas tras el ingreso, tales como traumatismos craneoencefálicos, politraumatismos y tetraplejias traumáticas y 32 familiares de pacientes con mala calidad de vida con cuatro años de evolución de su enfermedad física (hipertensión, diabetes, artritis reumatoide y síndrome de intestino irritable que no han estado ingresados en la UCI. Para alcanzar nuestro objetivo empleamos una Encuesta Psicosocial y los siguientes instrumentos: Cuestionario de Análisis Clínico, Escala de Clima Social en la Familia y Escala de Adaptación Psicosocial de la Enfermedad. Los resultados mostraron que los familiares de pacientes con mala calidad de vida que estuvieron ingresados en la UCI hace cuatro años, presentan diferencias significativas en las variables agitación y expresividad comparados con los familiares de pacientes con mala calidad de vida que no han estado ingresados en la UCI.

  2. Quantitative immunoassays for diagnosis and carrier detection in cystic fibrosis

    International Nuclear Information System (INIS)

    Quantitative immunoprecipitation and immunoradiometric assays have been developed for a protein present in the serum of cystic fibrosis homozygotes, and to a lesser extent in the serum of heterozygotes. When tested on a panel of sera from 14 cystic fibrosis patients, 29 heterozygotes and 23 controls, the immunoprecipitation assay allowed correct assignments to be made on 94% of occasions with one batch of antiserum and 95% with another. With the same panel of sera, the immunoradiometric assay allowed 94% correct assignments. It is suggested that such accuracy is the maximum that can be expected in the present state of knowledge of cystic fibrosis. (author)

  3. Encuentro con el tiempo: Adagio con variaciones de Alfredo Aracil

    Directory of Open Access Journals (Sweden)

    Rosa María Rodríguez Hernández

    2012-01-01

    Full Text Available Examinando analíticamente la obra sinfónica Adagio con variaciones de Aracil, estudiaremos los principales componentes para concluir con una evaluación de los mismos. A través de la cronología de la obra de Aracil175, observamos la importancia que adquiere en su trayectoria la memoria; su pensamiento va siempre unido a ésta. Uno de sus recursos principales es la cita; es Adagio con variaciones donde claramente observaremos la memoria lejana e inmediata al tiempo: Wagner es el punto de referencia al pasado, Wolf impulsa hacia el presente, y, Aracil advierte el devenir en cada una de sus variaciones.

  4. 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.

  5. The diffusing capacity in adult cystic fibrosis.

    Science.gov (United States)

    Espiritu, J D; Ruppel, G; Shrestha, Y; Kleinhenz, M E

    2003-06-01

    The value of adjusting the diffusing capacity for the lung volume has been demonstrated in a large number of patients with other lung diseases but has not been validated in patients with cystic fibrosis (CF). Pulmonary function test results on a cohort of 52 adult CF patients were analyzed to determine whether the diffusing capacity of carbon monoxide by single breath method (DLCO(SB)) when adjusted for alveolar volume (V(A)%), correlated with the severity of pulmonary dysfunction. The DLCO(SB) remained within the reference range except in those with severe lung impairment (61.88 +/- 15.48%). DLCO(SB) has a significant (P V/Q relationship due to claustration in CF. PMID:12814143

  6. Paediatric nasal polyps in cystic fibrosis.

    Science.gov (United States)

    Mohd Slim, Mohd Afiq; Dick, David; Trimble, Keith; McKee, Gary

    2016-01-01

    Patients with cystic fibrosis (CF) are at increased risk of nasal polyps. We present the case of a 17-month-old Caucasian patient with CF who presented with hypertelorism causing cycloplegic astigmatism, right-sided mucoid discharge, snoring and noisy breathing. Imaging suggested bilateral mucoceles in the ethmoid sinuses. Intraoperatively, bilateral soft tissue masses were noted, and both posterior choanae were patent. Polypectomy and bilateral mega-antrostomies were performed. Histological examination revealed inflammatory nasal polyposis typical of CF. The role of early functional endoscopic sinus surgery (FESS) in children with CF nasal polyposis remains questionable as the recurrence rate is higher, and no improvement in pulmonary function has been shown. Our case, however, clearly demonstrates the beneficial upper airway symptom relief and normalisation of facial appearance following FESS in a child with this condition. PMID:27329094

  7. Cardiac manifestations of idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Agrawal, Abhinav; Verma, Isha; Shah, Varun; Agarwal, Abhishek; Sikachi, Rutuja R

    2016-05-01

    Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, parenchymal disease of the lung with an estimated prevalence of 14-43 per 100,000. Patient usually presents with coughing and exertional dyspnea, which can lead to acute respiratory failure. IPF has been associated with various co-morbidities such as lung cancer, emphysema, obstructive sleep apnea (OSA), GERD and multiple cardiovascular consequences. The cardiovascular manifestations of IPF include pulmonary hypertension, heart failure, coronary artery disease, cardiac arrhythmias & cardiac manifestations of drugs used to treat IPF. This review will outline evidence of the association between IPF and cardiovascular conditions and attempt to provide insights into the underlying pathophysiology. We also discuss the impact of these cardiovascular diseases on patients with IPF including increased morbidity and mortality. PMID:27195188

  8. Nephrogenic systemic fibrosis: late skin manifestations

    DEFF Research Database (Denmark)

    Bangsgaard, Nannie; Marckmann, Peter; Rossen, Kristian;

    2009-01-01

    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.......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...

  9. 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...... 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...... between FEV(1) and self-concept as well as a significant inverse correlation between FEV(1) and anxiety. CONCLUSIONS: Younger children with CF and boys with CF were more anxious than the healthy controls, and girls with CF expressed less anger than their healthy peers. Effects sizes however were small...

  10. [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 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.

  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. Current status of nephrogenic systemic fibrosis

    International Nuclear Information System (INIS)

    Nephrogenic systemic fibrosis (NSF) occurs in patients with advanced chronic kidney disease (CKD) or acute renal failure, most commonly following exposure to gadolinium-based contrast agents (GBCAs). NSF can be debilitating and associated with increased mortality. The putative association of NSF with GBCAs prompted the development of guidelines to limit the use of these contrast agents in at-risk patients. Indeed, the incidence of NSF has decreased dramatically following application of these guidelines, which appears to be the only effective means of decreasing NSF incidence. Thus, increasing clinician awareness of these updated guidelines is important. The present review introduces and compares updated guidelines for GBCA use and discusses the latest advances in the understanding of the pathogenic mechanisms and treatment of NSF

  13. Scoliosis in cystic fibrosis - an appraisal

    International Nuclear Information System (INIS)

    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. (orig.)

  14. Chronic Rhinosinusitis in Patients with Cystic Fibrosis.

    Science.gov (United States)

    Hamilos, Daniel L

    2016-01-01

    Chronic rhinosinusitis (CRS) is highly prevalent in patients with cystic fibrosis (CF) and accounts for significant morbidity and contribution to CF lung disease. Mutations of the cystic fibrosis transmembrane regulator gene occur with increased prevalence in patients with CRS without CF, suggesting some contribution to CRS pathophysiology. Nasal polyps (NPs) occur with increased prevalence in patients with CF of all ages and have a more neutrophilic appearance with fewer eosinophils and increased submucosal glandular elements in comparison to NPs from patients without CF. Mainstays of medical treatment include isotonic saline irrigations and topical intranasal glucocorticoids, with some evidence that topical intranasal glucocorticoids reduce NP size. Although inhaled hypertonic saline (7%) has been widely studied as a mucolytic agent for CF lung disease, there are no reports of its use in CF CRS. Mucolytics have also not been studied as a treatment for CRS in CF, and most evidence does not support their use for CF lung disease. Nasally nebulized dornase alfa (recombinant human deoxyribonuclease) following sinus surgery shows promise for treatment. Other unproven therapies include addition of baby shampoo to isotonic saline to potentially thin mucus and help prevent biofilm formation. There are no data to support the use of low-dose oral macrolide antibiotics or the use of prophylactic oral antibiotics for CRS in patients with CF. However, there is some support for the use of topical antibiotics, including colistimethate sodium or tobramycin, administered as a sinus irrigation or antral lavage in patients following sinus surgery when susceptible bacteria are cultured. Key components of CF sinus surgical management include extensive surgery to ensure that the maxillary, frontal, sphenoid, and ethmoid sinuses are all widely opened with smoothing of bony overhangs to prevent mucus retention and bacterial recolonization, postoperative meticulous daily nasal irrigations

  15. 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.

  16. Entrevista con Hans Robert Jauss.

    Directory of Open Access Journals (Sweden)

    - Consejo de Redacción

    1997-01-01

    Full Text Available El importante teórico y crítico literario alemán nació en 1921. Tras el largo paréntesis de la segunda guerra mundial, Jauss realizará sus estudios superiores, a partir de 1948, en Heidelberg, licenciándose en filología románica. Se doctora pronto con una tesis, publicada en 1955, sobre el tiempo y el recuerdo en la obra capital de Marcel Proust, En busca del tiempo perdido, y se habilita en 1957 con un trabajo sobre la epopeya animal en la Edad Media. A continuación, escribe un conjunto de estudios sobre la alegoría, y codirige con el gran especialista Erich Kohler una monumental historia de la literatura medieval, campo al que ha dedicado otros artículos, a la par que proseguía sus investigaciones iniciales.

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

    Directory of Open Access Journals (Sweden)

    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.

  18. Acoso psicológico en el trabajo y psicopatología: un estudio con el LIPT-60 y el SCL 90-R

    Directory of Open Access Journals (Sweden)

    JOSÉ LUIS GONZÁLEZ DE RIVERA Y REVUELTA

    2006-01-01

    Full Text Available Los autores revisan los estudios sobre Acoso Psicológico en el Trabajo APT (mobbing o adult bullying realizados en el Instituto de Psicoterapia e Investigación Psicosomática de Madrid en los últimos 6 años, que incluyen la descripción del Síndrome de Acoso Institucional, la elaboración del LIPT-60, versión expandida y mejorada del Leymann Inventory of Psychological Terrorization, la determinación del perfil de psicopatología de las víctimas de APT con el SCL90R y de sus relaciones con las dinámicas de acoso y la puesta a punto de un procedimiento terapéutico especifico. Los afectados de acoso en el trabajo presentan psicopatologia significativa, con elevaciones en el total de síntomas y en las dimensiones de somatización, depresión, obsesión-compulsión, hostilidad e ideación paranoide con respecto a la media de la población psiquiátrica. La proporción de solteros en la muestra de APT es notablemente más elevada que en la población general. El nivel socioeconómico correlaciona de manera inversa con el grado de afectación psicopatológica. La relación inversa entre las dimensiones psicopatológicas de ideación paranoide y de sensibilidad interpersonalsugiere que las estrategias de acoso psicológico en el trabajo tienen un efecto paranógeno, independiente de la susceptibilidad interpersonal o de la tendencia a interpretar de manera negativa las actuaciones ajenas.Los autores revisan los estudios sobre Acoso Psicológico en el Trabajo APT (mobbing o adult bullying realizados en el Instituto de Psicoterapia e Investigación Psicosomática de Madrid en los últimos 6 años, que incluyen la descripción del Síndrome de Acoso Institucional, la elaboración del LIPT-60, versión expandida y mejorada del Leymann Inventory of Psychological Terrorization, la determinación del perfil de psicopatología de las víctimas de APT con el SCL90R y de sus relaciones con las dinámicas de acoso y la puesta a punto de un procedimiento

  19. [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.

  20. Impairment of Alveolar Macrophage Transcription in Idiopathic Pulmonary Fibrosis

    OpenAIRE

    Ren, Ping; Rosas, Ivan O.; MacDonald, Sandra D.; Wu, Hai-Ping; Billings, Eric M; Gochuico, Bernadette R.

    2007-01-01

    Rationale: Alveolar macrophages are inflammatory cells that may contribute to the pathogenesis of idiopathic pulmonary fibrosis (IPF), which is characterized by excessive alveolar aggregation of cells and extracellular matrix proteins.

  1. 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...... allowing for gender and the potential confounders of age, income, and education. Finally, after allowing for these covariates, number of children in DeltaF508 heterozygotes decreased with increasing extent of smoking (trend test: P = 0.003), while the opposite was true for noncarriers (trend test: P

  2. Inflammation in Achromobacter xylosoxidans infected cystic fibrosis patients

    DEFF Research Database (Denmark)

    Hansen, C. R.; Pressler, T.; Nielsen, K. G.;

    2010-01-01

    BACKGROUND: Achromobacter xylosoxidans infection may cause conspicuous chronic pulmonary inflammation in cystic fibrosis (CF) patients similar to Pseudomonas aeruginosa and the Burkholderia cepacia complex (Bcc). Evolution in lung function was compared in chronically infected patients. Cytokine...

  3. Gastrointestinal transit times and motility in patients with cystic fibrosis

    DEFF Research Database (Denmark)

    Hedsund, Caroline; Gregersen, Tine; Jønsson, Iben;

    2012-01-01

    OBJECTIVE: Patients with cystic fibrosis (CF) often suffer from gastrointestinal (GI) dysfunction including obstructive symptoms, malabsorption and pain, but the underlying pathophysiology remains obscure. AIM: To compare GI motility and transit times in CF patients and healthy controls. MATERIAL...

  4. Modular microfluidic system as a model of cystic fibrosis airways

    DEFF Research Database (Denmark)

    Skolimowski, Maciej; Weiss Nielsen, Martin; Abeille, Fabien;

    2012-01-01

    A modular microfluidic airways model system that can simulate the changes in oxygen tension in different compartments of the cystic fibrosis (CF) airways was designed, developed, and tested. The fully reconfigurable system composed of modules with different functionalities: multichannel peristaltic...... pumps, bubble traps, gas exchange chip, and cell culture chambers. We have successfully applied this system for studying the antibiotic therapy of Pseudomonas aeruginosa, the bacteria mainly responsible for morbidity and mortality in cystic fibrosis, in different oxygen environments. Furthermore, we...... have mimicked the bacterial reinoculation of the aerobic compartments (lower respiratory tract) from the anaerobic compartments (cystic fibrosis sinuses) following an antibiotic treatment. This effect is hypothesised as the one on the main reasons for recurrent lung infections in cystic fibrosis...

  5. 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....... 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...... March 2015). We previously searched PubMed using the terms vaccin* AND cystic fibrosis (last search 30 May 2013). SELECTION CRITERIA: Randomised trials (published or unpublished) comparing Pseudomonas aeruginosa vaccines (oral, parenteral or intranasal) with control vaccines or no intervention in cystic...

  6. Immunisation in the current management of cystic fibrosis patients

    DEFF Research Database (Denmark)

    Malfroot, Anne; Adam, Georgios; Ciofu, Oana;

    2005-01-01

    Although no special recommendations exist, clearly patients with cystic fibrosis (CF) can benefit from immunisation. We reviewed the literature regarding vaccination in CF and other chronic diseases. CF subjects should follow national immunisation programmes without delay to obtain optimal...

  7. 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

  8. The role of gastroesophageal reflux in idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Raghu, Ganesh

    2003-08-18

    Fibroblast foci are indicative of idiopathic pulmonary fibrosis and appear to be a cellular attempt to repair the damaged alveolus. Although this progressive, often fatal, clinical syndrome is thought to be dependent on alveolar injury of unknown origin, significant clinical and preclinical evidence points to gastric acid as a causative harmful agent. Graded instillation of various forms of acid in several animal models resulted in aspiration-induced lung injury, including pulmonary fibrosis in pigs. Moreover, compelling clinical data suggest that a high percentage of patients with idiopathic pulmonary fibrosis also experience abnormal esophageal acid exposure, without necessarily experiencing the typical symptoms of gastroesophageal reflux disease (GERD). Aggressive, long-term therapeutic trials of patients with GERD and evaluation of the therapeutic effects on pulmonary disease will allow determination of the real influences of abnormal esophageal acid exposure in the development of idiopathic pulmonary fibrosis. PMID:12928077

  9. 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.

  10. Changes in Cystic Fibrosis Airway Microbiota at Pulmonary Exacerbation

    OpenAIRE

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

  11. Cystic fibrosis in a retro-positive child

    OpenAIRE

    Praveen Sharma; N Arthi; Sagar Bhattad; Kim Vaiphei

    2015-01-01

    We present a rare association of cystic fibrosis and retro positivity in a grossly malnutrited child. The child had pulmonary, pancreatic and colonic manifestations with superadded herpes simplex virus interstitial pneumonia and lymphocytic meningitis.

  12. Sarcoidosis in two patients with cystic fibrosis: a fortuitous association?

    OpenAIRE

    Cooper, T J; Day, A. J.; Weller, P H; Geddes, D. M.

    1987-01-01

    Two cases of cystic fibrosis complicated by sarcoidosis are described. Possible pathogenetic interactions between the two diseases are discussed. The diagnosis of sarcoidosis in patients with chronic pulmonary inflammation is difficult, and if overlooked may lead to inappropriate treatment.

  13. Association between Pulmonary Function and Sputum Biomarkers in Cystic Fibrosis

    OpenAIRE

    Mayer-Hamblett, Nicole; Aitken, Moira L.; Accurso, Frank J.; Kronmal, Richard A.; Konstan, Michael W.; Burns, Jane L.; Sagel, Scott D.; Ramsey, Bonnie W.

    2007-01-01

    Rationale: Sputum biomarkers of infection and inflammation are noninvasive measures that enable quantification of the complex pathophysiology of cystic fibrosis (CF) lung disease. Validation of these biomarkers as correlates of disease severity is a key step for their application.

  14. AEROSOL DEPOSITION AS A FUNCTION OF AIRWAY DISEASE: CYSTIC FIBROSIS

    Science.gov (United States)

    Progressive lung disease associated with cystic fibrosis (CF) is a continuous interaction of the processes of airway obstruction, infection and inflammation. ecent literature has suggested that the manifestation of CF could compromise the successful administration of pharmacologi...

  15. Hydrator Therapies for Chronic Bronchitis. Lessons from Cystic Fibrosis.

    Science.gov (United States)

    Bennett, William D; Henderson, Ashley G; Donaldson, Scott H

    2016-04-01

    Patients with the chronic bronchitis form of chronic obstructive pulmonary disease and cystic fibrosis share similar clinical features, including mucus obstruction of airways and the development of chronic/recurrent airways infections that often manifest as disease exacerbations. There is growing evidence that these diseases may have parallels in disease pathogenesis as well, including cystic fibrosis transmembrane conductance regulator dysfunction, mucus dehydration, and defective mucociliary clearance. As progress is made in the development of therapies that target the basic defects that lead to cystic fibrosis lung disease, it is possible that similar approaches could also benefit patients with chronic bronchitis. A deeper understanding of how tobacco smoke and other triggers of chronic bronchitis actually lead to disease, and exploration of the concept that therapies that restore cystic fibrosis transmembrane conductance regulator function, mucus hydration, and/or mucociliary clearance may benefit patients with chronic bronchitis, hold the prospect of significant progress in treating this prevalent disease. PMID:27115955

  16. Measurement of alveoli wall fibrosis using image processing

    International Nuclear Information System (INIS)

    Lung disease can be estimated by observing the morphological conditions of alveoli and pulmonary blood vessels. Respiratory functions of lung are affected by fibrosis of alveoli wall and hypertrophy of the blood vessels. It is, therefore, important to measure the grade of fibrosis and hypertrophy for diagnosis of the lung disease. In this study, a system based on image processing technique is developed to measure the grade of fibrosis, and the result is well-correlated with the diagnostic judgement of the medical doctor. It is mentioned that by examining the correlation between the alveoli wall fibrosis and the hypertrophy of the blood vessels, discrimination of different types of lung disease becomes possible. (author)

  17. 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.

  18. Tratamiento con implantes Leader-Nano en paciente con oligodoncia

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    Salvador Javier Santos Medina

    2015-03-01

    Full Text Available Los implantes dentales de titanio han revolucionado el mundo de la rehabilitación desde su surgimiento. De manera particular, el empleo de implantes de carga inmediata acorta el tiempo quirúrgico y protésico, con el consiguiente bienestar estético. Se presenta el caso de una paciente femenina de 32 años de edad, con antecedentes de oligodoncia de ambos incisivos laterales superiores y portadora de prótesis parcial acrílica. Fue atendida por el equipo multidisciplinario de implantes en la Clínica Estomatológica Docente “3 de Octubre” y se le realizó tratamiento de rehabilitación integral con implantes Leader-Nano y prótesis fija con corona acrílica sobre dichos implantes. La implantología fue satisfactoria en la paciente; la mejoría estética y funcional, así como la satisfacción de la paciente, fueron los principales logros obtenidos

  19. Microbiología pulpar de dientes íntegros con lesiones apicales de origen idiopático

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    Patricia Rodríguez

    2008-03-01

    Full Text Available Introducción: Los cambios periapicales denominados lesiones, en dientes con integridad coronal completa y sin antecedentes de trauma, no presentan una etiología clara. Objetivo: Determinar la presencia de microorganismos en el tejido pulpar clarifica las causas de su muerte y el consiguiente daño a los tejidos periodontalesMateriales y métodos: Se seleccionaron 23 dientes, en personas con rangos de edad entre 10 y 39 años. Las muestras se tomaron con puntas de papel y limas Nº 0.8 (estériles, se transportaron en VMGA III, se procesaron en las siguientes 24 horas de tomada la muestra y se sembraron en agar brucella. Resultados: Los dientes más afectados fueron los centrales superiores 43.8%. De los 23 dientes estudiados, en 20 se observó crecimiento microbiológico. Se identificaron los siguientes microorganismos: Fusobacterium spp., 25%; Eubacte-rium spp., 15%; Peptostreptococcus spp., 10%; Campylobacter spp., 10%; bacilos entéricos gram negativos, 10%; Porphyromonas gingivalis, 10%; Prevotella intermedia, 5%; Eikenellia corrodens, 5%; Dialister pneumosintes, 5%; y levaduras en 5%. No hubo evidencias de crecimiento de Actinomyces actinomycetemcomitans, Tanerella forsythensis ni de estreptococo b hemolítico.Discusión y conclusiones: El tejido pulpar sano es estéril, la lesión sobre él causa inflamación degeneración, muerte pulpar y contaminación bacteriana. Los resultados en el presente estudio determinaron claramente la presencia de micro-organismos en lesiones apicales cerradas de origen endodóntico. De igual forma se evidencia que gran parte de los microorganismos que se encontraron son considerados periodontopatógenos lo que puede igualmente sugerir manejo compartido entre tratamiento endodóntico, periodontal y farmacológico.

  20. Reserva cognitiva y déficit en la planificación en pacientes con esclerosis múltiple.

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    Lilia Mestas

    2012-01-01

    Full Text Available

    La esclerosis múltiple afecta el sistema nervioso produciendo alteraciones cognitivas. La memoria es uno de los más afectados, no obstante la naturaleza ó factor primario de alteración aún no se clarifica. Objetivo: Estudia las diferencias en el procesamiento de la información verbal en un grupo de pacientes con esta patologíaa neurológica respecto a un grupo de control sano. Para ello se analizó el desempeño neuropsicológico de 16 pacientes con esclerosis múltiple de tipo remitente-recurrente y un grupo de control equiparado en las variables sociodemográficas. A los dos grupos se les aplic? el Test de Aprendizaje Verbal España-Complutense, y se realizó una comparación entre diversos índices de la prueba, además de un análisis de correlaciones entre las variables socio-demográficas del grupo experimental para identificar algún factor protector. Resultados: el grupo de esclerosis múltiple obtuvo rendimientos inferiores en los índices analizados respecto al de control, además de una correlación positiva entre años de escolaridad y el inicio de la enfermedad y entre el inicio de la enfermedad y la ocupación. Los datos encontrados parecen indicar que los pacientes con esclerosis múltiple tienen poca capacidad de planificación, que incide de manera directa en su desempeño en tareas de memoria y aprendizaje.