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Sample records for afectados con fibrosis

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

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    Gomis Bataller, Manuel

    2007-01-01

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

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

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    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...

  3. Intervención con afectados de Alzheimer en fase I y atención a sus cuidadores

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    Corrales García - Morato, Elisabet

    2015-01-01

    La enfermedad de Alzheimer afecta cada día a un mayor porcentaje de la población, y su prevalencia aumenta al incrementarse la esperanza de vida. A día de hoy se desconoce la cura de dicha enfermedad, por lo que es importante seguir investigando sobre sus posibles tratamientos. En este trabajo se propone un proyecto de intervención no farmacológica que pretende trabajar distintos ámbitos de la vida diaria, no solo con la persona enferma de Alzheimer sino también con la figur...

  4. 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.

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

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    R. A ASSIS

    2002-01-01

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

  8. 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.

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

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    Tania Cruz Mariño; Ana Luz Portelles Caminero; William Áreas Zalazar; Luís Velázquez Pérez

    2010-01-01

    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...

  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. 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

  13. 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...

  14. 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.

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

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    Ana Raquel Medeiros Beck

    2007-12-01

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

  16. 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; C. Alía Alía; Marco, A.; A. López Burgos; J. de Juan Ramírez; A. Herrero Matías; J. Portilla Sogorb

    2013-01-01

    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 penit...

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

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

    2014-01-01

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

  2. 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.

  3. 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.

  4. Fibrosis retroperitoneal

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    Claudio Orlich-Castelán

    2005-07-01

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

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

    OpenAIRE

    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...

  6. Tabla de supervivencia de Pinus Halepensis afectado por incendios forestales

    OpenAIRE

    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...

  7. 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

    Directory of Open Access Journals (Sweden)

    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.

  8. Fibrosis retroperitoneal

    Directory of Open Access Journals (Sweden)

    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

  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

    Directory of Open Access Journals (Sweden)

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

    Directory of Open Access Journals (Sweden)

    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.

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

    OpenAIRE

    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...

  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

    Directory of Open Access Journals (Sweden)

    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

    OpenAIRE

    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. 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

    Directory of Open Access Journals (Sweden)

    Daniel Álvarez

    2012-02-01

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

  16. Incidencia de procesos AMD en la hidroquímica de embalses afectados en la Faja Pirítica Ibérica

    OpenAIRE

    Santisteban Fernández, María

    2015-01-01

    En Europa, los principales problemas de contaminación por metales pesados en ias aguas de ríos y embalses están asociados al drenaje ácido de mina, que tiene lugar en la Faja Pirftica Ibérica (FPI), situada en el SO Europeo, a lo largo de las provincias de Huelva y Sevilla, así como en el Sur de Portugal. La intensa actividad minera en esta zona metalogenética, ha dejado, en las cuencas de los ríos afectados un legado de 86 minas abandonadas, con sus pozos, socavones y cortas, sus innumerable...

  17. 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...

  18. Comunidades de afectados, procomún y don expandido

    OpenAIRE

    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...

  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

    OpenAIRE

    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. Ruolo dell'elastometria pulsata monodimensionale ad ultrasuoni (fibroscan) nella stadiazione della fibrosi epatica in pazienti sottoposti a trapianto ortotopico di fegato con recidiva di epatite C

    OpenAIRE

    Corradi, Francesco

    2007-01-01

    PREMESSA: La progressione della recidiva d’epatite C è accelerata nei pazienti sottoposti a trapianto di fegato e ciò ha portato alla necessità di sviluppare nuove e validate metodiche non invasive per la quantificazione e la misura della fibrosi epatica. SCOPI: Stabilire l’efficacia dell’elastometria epatica (Fibroscan®) e dei parametri sierici di fibrosi, attualmente disponibili nella pratica clinica, per predire il grado di fibrosi nei pazienti sottoposti a trapianto epatico. METODI: La co...

  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

    Directory of Open Access Journals (Sweden)

    L. Adán Merino

    2010-10-01

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

  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

    Directory of Open Access Journals (Sweden)

    Lina Manuela Jay

    2006-09-01

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

  4. Fibrose miocárdica em pacientes com cardiomiopatia hipertrófica com alto risco para morte súbita cardíaca Fibrosis miocárdica en pacientes con cardiomiopatía hipertrófica con alto riesgo para muerte súbita cardíaca Myocardial fibrosis in patients with hypertrophic cardiomyopathy and high risk for sudden death

    Directory of Open Access Journals (Sweden)

    Afonso Akio Shiozaki

    2010-04-01

    expresan la muerte súbita como primer síntoma. Trabajos recientes vienen sugiriendo que la fibrosis miocárdica puede constituirse en un importante sustrato para las arritmias ventriculares malignas, responsables de la muerte súbita en esta enfermedad. OBJETIVO: Evaluación de la prevalencia y cuantificación de la fibrosis miocárdica (FM, en pacientes con CMH con alto riesgo o recuperados de muerte súbita, portadores de cardiodesfibrilador implantable (CDI. MÉTODOS: Un total de 28 pacientes con CMH portadores de CDI fueron sometidos a la tomografía computadorizada con múltiples detectores, para la realización de la técnica de realce tardío, y evaluación de la fibrosis miocárdica. RESULTADOS: El 96% de los pacientes presentaba fibrosis miocárdica (20,38 ± 15,55 gramos y correspondía a 15,96 ± 10,20% de la masa miocárdica total. La FM fue significativamente más prevalente que los demás factores de riesgo clásicos para muerte súbita. CONCLUSIÓN: Concluimos que existe una alta prevalencia de fibrosis miocárdica en pacientes con cardiomiopatía hipertrófica de alto riesgo o recuperados de muerte súbita, como en este grupo - portadores de cardiodesfibrilador implantable. La mayor prevalencia de la fibrosis miocárdica comparada a los factores de riesgo de peor pronóstico levanta la hipótesis de que la fibrosis miocárdica pueda ser un importante sustrato potencialmente necesario en la génesis de las arritmias desencadenadoras de la muerte súbita.BACKGROUND: The stratification of risk for sudden death in hypertrophic cardiomyopathy (HCM continues to be a true challenge due to the great heterogeneity of this disease's presentation, as most individuals remain asymptomatic during their entire lives and others present sudden death as first symptom. Recent studies have suggested that myocardial fibrosis may represent an important substrate for the malignant ventricular arrhythmias, that are responsible for the cases of sudden death related to this

  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 tissue ... 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. Fibrosis retroperitoneal asociada a uso crónico de ergotamina. Tratamiento con colchicina y esteroides: Caso clínico Retroperitoneal fibrosis associated with chronic use of ergotamine: Report of one case

    OpenAIRE

    Jorge Vega; Helmuth Goecke; Mario Santamarina

    2011-01-01

    Retroperitoneal fibrosis (RPF) associated with chronic use of ergotamine is a very rare disorder. We report a 45-year-old woman who presented with a RPf after using, almost daily for 23 years, ergotamine tartrate for migraine relief. FRP presented as a chronicinflammatory state, anemia, abdominal and lumbosacral pain and a hypogastric mass. A CT-Scan showed a periaortic mass and left hydronephrosis. A percutaneous biopsy was obtained and the patient was subjected to a surgical ureterolysis an...

  8. Cystic fibrosis*

    OpenAIRE

    1985-01-01

    Cystic fibrosis represents a major health problem in developed countries. Although its distribution is worldwide, in most developing countries there is little awareness of its prevalence and little opportunity to provide treatment. The improvements in survival of cystic fibrosis patients in developed countries over the past two decades have brought mean life expectancy to 20 years or more, and many adult patients with cystic fibrosis at present are fully integrated in society and have an acce...

  9. Liver fibrosis

    OpenAIRE

    Bataller, Ramón; Brenner, David A.

    2005-01-01

    Liver fibrosis is the excessive accumulation of extracellular matrix proteins including collagen that occurs in most types of chronic liver diseases. Advanced liver fibrosis results in cirrhosis, liver failure, and portal hypertension and often requires liver transplantation. Our knowledge of the cellular and molecular mechanisms of liver fibrosis has greatly advanced. Activated hepatic stellate cells, portal fibroblasts, and myofibroblasts of bone marrow origin have been identified as major ...

  10. Fibrosis retroperitoneal asociada a uso crónico de ergotamina. Tratamiento con colchicina y esteroides: Caso clínico

    OpenAIRE

    Jorge Vega; Helmuth Goecke; Mario Santamarina

    2011-01-01

    Retroperitoneal fibrosis (RPF) associated with chronic use of ergotamine is a very rare disorder. We report a 45-year-old woman who presented with a RPf after using, almost daily for 23 years, ergotamine tartrate for migraine relief. FRP presented as a chronicinflammatory state, anemia, abdominal and lumbosacral pain and a hypogastric mass. A CT-Scan showed a periaortic mass and left hydronephrosis. A percutaneous biopsy was obtained and the patient was subjected to a surgical ureterolysis an...

  11. 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, ...

  12. 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

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    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

  14. 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 ...

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

    Directory of Open Access Journals (Sweden)

    Patricia Hernández Rodríguez

    2002-06-01

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

  16. Intervención psicoterapéutica en afectados de enfermedad de Alzheimer con deterioro leve

    OpenAIRE

    IMSERSO

    2002-01-01

    La Fundación INTRAS ha realizado un estudio cuyo objetivo es evaluar la eficacia de una intervención cognitivo-conductual, que busca ralentizar el proceso degenerativo del Alzheimer y retrasar el paso de una fase leve a una fase moderada de la enfermedad. El Programa de tratamiento combina técnicas de intervención cognitivas y conductuales que estimulan las aptitudes conservadas del paciente y entrena estrategias para subsanar las pérdidas funcionales. Se trata de un programa individualizad...

  17. 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...

  18. 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 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

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

    Directory of Open Access Journals (Sweden)

    IGNACIO SÁNCHEZ D.

    2003-01-01

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

  20. 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...

  1. [Retroperitoneal fibrosis].

    Science.gov (United States)

    Babski, Paweł; Wojtuń, Stanisław; Gil, Jerzy

    2007-05-01

    Retroperitoneal fibrosis is a rare clinical entity characterised by the presence of patologic collagen tissue in a retroperitoneal space. The fibrous mass covers abdominal organs causing their disfunctions. RPF was described at the begining of XX century but its etiology is not clear yet. Usually it causes an ureter obstuction and hydronephrosis, that is why most commonly is diagnosed by urologists and nephrologists. However, retroperitoneal fibrosis can be multifacial disease. In some patients localisation of fibrosis is atypical and manifestationns can be varied. Gastrological symptoms like jaundice, bowel obstuction, ascites can occure. Besides, some early signs of RPF are nonspecific and can imitate alarming symptoms of neoplasma, e.g.: weight loss, anemia, malaise, anorexia, fever. This force us to initiate gastrological investigation. The awareness of this disease is important. The early diagnosis and treatment improves prognosis and alows to avoid heavy complications. In typical cases radiology is often enough for diagnosis. However, histological examination is needed in many cases, especialy when patological mass is located atypical. A treatment is made up of farmacology and surgery. The first one is based on steroids, immunossuppressant and tamoxifen. Surgery is needed to eliminate organs obstruction. PMID:17679405

  2. 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 ...

  3. 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 ...

  4. Pulmonary Fibrosis Foundation

    Science.gov (United States)

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

  5. 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

  6. 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

  7. Evaluación de un programa psicoterapéutico para cuidadores de pacientes con TCA: Estudio piloto

    OpenAIRE

    Pla, Vanessa; Moreno, Eugenia; Fuentes Dura, Inmaculada; Dasí Vivó, Carmen

    2013-01-01

    El estudio tuvo como objetivo comparar el nivel de discapacidad funcional, dolor y agotamiento, el autoconcepto-autoestima, y grandes dimensiones de personalidad, en personas con y sin fibromialgia (FM). El estudio se realizó con 190 participantes, 140 afectados de FM y 50 no afectados, entre 28 y 75 años (M= 52,16; DT= 9,18), 10 varones y 180 mujeres. Se utilizaron 4 instrumentos de evaluación para medir las variables objeto de estudio: impacto de la fibromialgia, autoconcepto, autoestima y ...

  8. 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.

  9. Factores asociados a la infección tuberculosa latente en los contactos de pacientes afectados

    Directory of Open Access Journals (Sweden)

    Miquel Alsedà

    2004-04-01

    Full Text Available Fundamento: Una vez obtenidas unas cifras aceptables en la identificación y el tratamiento de los enfermos tuberculosos, se plantea una intervención para evitar la aparición de nuevos casos. Las personas con una infección tuberculosa latente (ITL reciente tienen un mayor riesgo de desarrollar una enfermedad tuberculosa. El objetivo de este estudio es evaluar la prevalencia de ITL y sus factores asociados en los contactos de pacientes tuberculosos. Métodos: Se estudiaron los contactos de estos pacientes examinados en el Centro de Prevención y Control de la Tuberculosis de Lleida en el período 1991-1997. Se recogieron factores asociados con el caso índice (demográficos, radiográficos, bacteriológicos y terapéuticos y el resultado de la prueba de la tuberculina en los contactos y sus datos demográficos. También se recogieron datos del caso índice relacionados con la infección por el VIH, el uso de drogas por vía parenteral y el consumo excesivo de alcohol. Como medida de asociación, se calcularon las odds ratio (OR crudas y ajustadas (ORa. Resultados: La prevalencia global de ITL en los contactos fue del 36,1% (780 de 2.161. En el análisis multivariante se detectó una mayor frecuencia de ITL en los contactos mayores de 14 años (ORa = 3,34; intervalo de confianza [IC] del 95%, 2,51-4,45, en los convivientes (ORa = 1,96; IC del 95%, 1,59-2,42, en los contactos de caso índice con una localización pulmonar de la enfermedad (ORa = 1,54; IC del 95%, 1,01-2,35, en los de caso índice con baciloscopia positiva en las muestras respiratorias (ORa = 1,51; IC del 95%, 1,15-1,99, en los de caso índice con patrón de cavitaciones en la radiografía de tórax (ORa = 1,27; IC del 95%, 1,01-1,61 y en los de casos índice con demora en el inicio del tratamiento (ORa = 1,31; IC del 95%, 1,05-1,62. Conclusiones: La prevalencia global de ITL en los contactos de enfermos tuberculosos es elevada. Entre los factores estudiados, la demora en el

  10. 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 ...

  11. 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 ...

  12. Cystic fibrosis - nutritional considerations

    Science.gov (United States)

    Cystic fibrosis (CF) is a life-threatening disease that causes thick, sticky mucus to build up in the lungs and digestive tract. Persons with cystic fibrosis need to eat high-calorie and high-protein ...

  13. 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

  14. 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.

  15. 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.

  16. 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 ...

  17. 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 ...

  18. 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 . ...

  19. Genetics Home Reference: retroperitoneal fibrosis

    Science.gov (United States)

    ... Understand Genetics Home Health Conditions retroperitoneal fibrosis retroperitoneal fibrosis Enable Javascript to view the expand/collapse boxes. Print All Open All Close All Description Retroperitoneal fibrosis is a disorder in which inflammation and extensive ...

  20. Reclutando profesionales con deseos de empleo.

    Directory of Open Access Journals (Sweden)

    Teresa Farfán Cabrera

    2014-01-01

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

  1. 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, ...

  2. 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

  3. 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.

  4. Imaging pulmonary fibrosis

    International Nuclear Information System (INIS)

    Localized fibrosis of the lung is usually scar tissue while diffuse pulmonary fibrosis is more often a sign of active disease. Chronic infiltrative lung disease may be classified into four categories: idiopathic pneumonitis, collagen diseases, granulomatosis (sarcoidosis), and caused by known diseases (pneumoconiosis, hypersensitivity pneumonitis, drug-induced lung disease, radiation). (authors)

  5. 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

  6. Evolución de la contaminación con elementos traza en los suelos afectados por el vertido de Aznalcóllar

    OpenAIRE

    Cabrera, Francisco; Girón Moreno, Ignacio F.; Moreno Lucas, Félix

    2007-01-01

    8 páginas, 1 tabla, 2 figuras.-- Aparece publicado en el Capítulo 2: Contaminación y recuperación de suelos.-- Trabajo presentado al II Congreso Ibérico de la Ciencia del Suelo, celebrado del 13-17 junio de 2006, en Huelva (España), como parte de la actividad científica de la Sociedad Española de la Ciencia del Suelo (SPCS) y la Sociedade Portuguesa da Ciência do Solo (SPCS).

  7. 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.

  8. Living with Cystic Fibrosis

    Science.gov (United States)

    ... Most of these centers have pediatric and adult programs or clinics. For more information about CF Care Centers, go to the Cystic Fibrosis Foundation's Care Center Network Web page. It's standard to have CF checkups every 3 ...

  9. 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 ...

  10. 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...

  11. 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...

  12. 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.

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

    OpenAIRE

    Campeño Martínez, Yoana

    2013-01-01

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

  14. Barreras de inserción al mundo laboral de los estudiantes universitarios afectados por discapacidad visual

    OpenAIRE

    Polo Sánchez, María Tamara

    2005-01-01

    En la última década asistimos a importantes transformaciones en la atención psicopedagógica relacionada con las personas afectadas por una discapacidad. La realidad de nuestros días pone de manifiesto como los discapacitados visuales pueden acceder a un sistema socioeducativo abierto a la diversidad, desde la variedad de circunstancias personales. El resultado de estas actuaciones muestra como el volumen de dichos discapacitados que se articulan en los estudios universitarios, se acrecienta s...

  15. 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 ...

  16. 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.

  17. ¿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...

  18. 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...

  19. 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...

  20. Caracterización clínica de familias costarricenses con trastorno afectivo bipolar

    OpenAIRE

    Javier Contreras-Rojas; Henriette Raventós-Vorst

    2014-01-01

    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. M...

  1. 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.

  2. Nephrogenic systemic fibrosis

    DEFF Research Database (Denmark)

    Marckmann, Peter

    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...... gadolinium-containing contrast agents used for magnetic resonance imaging have an essential causative role in most, if not all, cases of nephrogenic systemic fibrosis. One particular agent, gadodiamide, caused the majority of cases, but gadopentetate dimeglumine has also been implicated in several cases...

  3. 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...

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

    Directory of Open Access Journals (Sweden)

    José Carrón Sánchez

    2013-12-01

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

  5. 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...

  6. 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...

  7. 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.

  8. 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.

  9. 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.

  10. 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. 

  11. Adipose tissue fibrosis

    OpenAIRE

    Buechler, Christa; Krautbauer, Sabrina; Eisinger, Kristina

    2015-01-01

    The increasing prevalence of obesity causes a major interest in white adipose tissue biology. Adipose tissue cells are surrounded by extracellular matrix proteins whose composition and remodeling is of crucial importance for cell function. The expansion of adipose tissue in obesity is linked to an inappropriate supply with oxygen and hypoxia development. Subsequent activation of hypoxia inducible factor 1 (HIF-1) inhibits preadipocyte differentiation and initiates adipose tissue fibrosis. The...

  12. Radiation pneumonitis and fibrosis

    International Nuclear Information System (INIS)

    The likelihood of toxic pulmonary lesions development as the result of radiation therapy for pulmonary carcinoma and breast cancer is discussed. Two possible forms of radiation induced changes are described, namely: classical radiation pneumonitis (RP) terminating with lung fibrosis circumscribed in the radiation zone, and sporadic RP giving rise to bilateral lymphatic alveolitis and manifestations outside the irradiation field. Attention is called to the fact that chemotherapy augments the risk of toxic lung damage occurrence. A number of markers for early RP diagnosis, including lactate dehydrogenase activity, KL-6, procollagen III, transforming growth factor β, C-reactive protein and partial oxygen pressure are listed. Therapeutic possibilities in coping with RP and pulmonary fibrosis are assayed. Apart from the standard therapeutic approach using corticosteroids and azatioprin, ideas are set forth concerning the application of some antioxidants, angiotensin converting enzyme inhibitors and γ-interferon. It is pointed out that radiation pneumonitis and pulmonary fibrosis treatment has an essential practical bearing on life expectancy and quality of life in a great number of cancer patients. (author)

  13. 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 ...

  14. 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...

  15. Genetics Home Reference: congenital hepatic fibrosis

    Science.gov (United States)

    ... Home Health Conditions congenital hepatic fibrosis congenital hepatic fibrosis Enable Javascript to view the expand/collapse boxes. ... All Open All Close All Description Congenital hepatic fibrosis is a disease of the liver that is ...

  16. Análisis de la alergenicidad de componentes proteicos de soja que presentan reactividad cruzada con caseínas bovinas

    OpenAIRE

    Curciarello, Renata

    2010-01-01

    La alergia a la leche de vaca es la causa más común de alergia alimentaria en lactantes y niños, probablemente porque la LV constituye uno de los primeros antígenos alimentarios a los que resultan expuestos los niños en la primera infancia. Si bien generalmente esta situación patológica resulta transitoria, ya que la mayoría de los niños afectados desarrollan tolerancia con el tiempo, los pacientes son sometidos a tratamientos con dietas libres de LV y sus derivados. En pacientes con edades m...

  17. 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...

  18. 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...

  19. 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-...

  20. 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

  1. [Idiopathic Pulmonary Fibrosis].

    Science.gov (United States)

    Prasse, A

    2015-10-01

    Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and a disease of the elderly. Cigarette smoking and longterm exposure to substances harming alveolar epithelial cells are risk factors for the development of IPF. There is also evidence for a genetic susceptibility. IPF is defined as the idiopathic variant of Usual Interstitial Pneumonitis (UIP). Diagnosis of IPF is complex and based on the exclusion of other diseases associated with an UIP pattern. The only cure is lung transplantation. In the last years there was a breakthrough in the treatment of IPF. With pirfenidone and nintedanib there are now two compounds approved for the treatment of IPF. PMID:26444136

  2. 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.)

  3. 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, ...

  4. Serum markers of liver fibrosis

    DEFF Research Database (Denmark)

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

    2010-01-01

    BACKGROUND: Fibrosis is a central histological feature of chronic liver diseases and is characterized by the accumulation and reorganization of the extracellular matrix. The gold standard for assessment of fibrosis is histological evaluation of a percutaneous liver biopsy. Albeit a considerable......-epitopes, may be targeted for novel biochemical marker development in fibrosis. We used the recently proposed BIPED system (Burden of disease, Investigative, Prognostic, Efficacy and Diagnostic) to characterise present serological markers. METHODS: Pubmed was search for keywords; Liver fibrosis, neo...... systematic use of the neo-epitope approach, i.e. the quantification of peptide epitopes generated from enzymatic cleavage of proteins during extracellular remodeling, may prove productive in the quest to find new markers of liver fibrosis....

  5. Molecular imaging in myocardial fibrosis

    International Nuclear Information System (INIS)

    With the development of life science and medical technology, myocardial fibrosis is being increasingly recognized as a new therapeutic target for heart diseases. However, traditional methods for detection of myocardial fibrosis, such as myocardial biopsy and laboratory assay of serum metabolites or enzymes, are not satisfactory in meeting the clinical demands because of their intrinsic limitations. Molecular imaging may non-invasively and quantitatively evaluate the presence/absence, degree and turnover of myocardial fibrosis in vivo with good specificity, thus being useful for clinical assessment and intervention. Currently, the commonly used molecular imaging modalities for evaluation of myocardial fibrosis include SPECT, PET and MRI. It is hopeful that the molecular probe for targeted ultrasound technology may also be developed in the near future. This review highlights the current status and future trends of molecular imaging in myocardial fibrosis. (authors)

  6. 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.

  7. 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.

  8. 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.

  9. Evaluación del comportamiento de arsénico, cobre, plomo y zinc en suelos afectados por el vertido de la mina de Aznalcóllar (Sevilla, España)

    OpenAIRE

    Sarria Carabalí, Margarita María

    2013-01-01

    Después del accidente de la mina de Aznalcóllar, los suelos afectados fueron sometidos a medidas de remediación para disminuir la contaminación generada principalmente por As, Cu, Pb y Zn, encontrándose que después de 6 años seguían teniendo problemas (Simón et al., 2009). Por lo anterior, se hizo un muestreo en tres perfiles de suelos contaminado (SC), remediado (SR) y no contaminado (SNC, no afectado por el vertido) en el sector “Puente de las Doblas” a los diez años de ocurrido el accident...

  10. 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...

  11. [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

  12. 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.

  13. Neutrophils in cystic fibrosis.

    Science.gov (United States)

    Laval, Julie; Ralhan, Anjali; Hartl, Dominik

    2016-06-01

    Cystic fibrosis (CF) lung disease is characterized by chronic infection and inflammation. Among inflammatory cells, neutrophils represent the major cell population accumulating in the airways of CF patients. While neutrophils provide the first defensive cellular shield against bacterial and fungal pathogens, in chronic disease conditions such as CF these short-lived immune cells release their toxic granule contents that cause tissue remodeling and irreversible structural damage to the host. A variety of human and murine studies have analyzed neutrophils and their products in the context of CF, yet their precise functional role and therapeutic potential remain controversial and incompletely understood. Here, we summarize the current evidence in this field to shed light on the complex and multi-faceted role of neutrophils in CF lung disease. PMID:26854289

  14. 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.

  15. 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...

  16. 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

  17. 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 ...

  18. 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.

  19. 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.

  20. 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.

  1. 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 and...... accumulated in type II alveolar epithelial cells, lacking CFTR. P. aeruginosa organisms were rarely present in inflamed alveoli. CONCLUSIONS: Chronic inflammation and remodeling is present in alveolar tissues of the CF lung and needs to be addressed by anti-inflammatory therapies....

  2. 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 ...

  3. Therapeutic targets in liver fibrosis.

    Science.gov (United States)

    Fallowfield, Jonathan A

    2011-05-01

    Detailed analysis of the cellular and molecular mechanisms that mediate liver fibrosis has provided a framework for therapeutic approaches to prevent, slow down, or even reverse fibrosis and cirrhosis. A pivotal event in the development of liver fibrosis is the activation of quiescent hepatic stellate cells (HSCs) to scar-forming myofibroblast-like cells. Consequently, HSCs and the factors that regulate HSC activation, proliferation, and function represent important antifibrotic targets. Drugs currently licensed in the US and Europe for other indications target HSC-related components of the fibrotic cascade. Their deployment in the near future looks likely. Ultimately, treatment strategies for liver fibrosis may vary on an individual basis according to etiology, risk of fibrosis progression, and the prevailing pathogenic milieu, meaning that a multiagent approach could be required. The field continues to develop rapidly and starts to identify exciting potential targets in proof-of-concept preclinical studies. Despite this, no antifibrotics are currently licensed for use in humans. With epidemiological predictions for the future prevalence of viral, obesity-related, and alcohol-related cirrhosis painting an increasingly gloomy picture, and a shortfall in donors for liver transplantation, the clinical urgency for new therapies is high. There is growing interest from stakeholders keen to exploit the market potential for antifibrotics. However, the design of future trials for agents in the developmental pipeline will depend on strategies that enable equal patient stratification, techniques to reliably monitor changes in fibrosis over time, and the definition of clinically meaningful end points. PMID:21233278

  4. 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.

  5. Efectos de un programa acuático educativo dirigido al alumnado con síndrome de Angelman sobre la dimensión física: un estudio cualitativo

    OpenAIRE

    Cantallops, Jaime; Vidal, Josep; Borràs, Pere Antoni; Ponseti, Francesc Xabier; Palou, Pere

    2011-01-01

    El objetivo del presente trabajo es elaborar y aplicar un programa acuático educativo dirigido a alumnado gravemente afectado de un centro de educación especial, con la intención de ofrecer un recurso educativo que pueda contribuir a su educación integral y a la vez evaluar los beneficios observados. La investigación está motivada por la falta de estudios y experiencias que relacionan el agua como medio de aprendizaje con las personas gravemente afectadas. Se ha trabajado con una muestra de d...

  6. Efectos de un programa acuático educativo dirigido al alumnado con síndrome de Angelman sobre la dimensión física: un estudio cualitativo

    OpenAIRE

    Jaime Cantallops Ramón; Josep Vidal Conti; Pere Antoni Borràs Rotger; Francesc Xavier Ponseti Verdaguer; Pere Palou Sampol

    2016-01-01

    El objetivo del presente trabajo es elaborar y aplicar un programa acuático educativo dirigido a alumnado gravemente afectado de un centro de educación especial, con la intención de ofrecer un recurso educativo que pueda contribuir a su educación integral y a la vez evaluar los beneficios observados. La investigación está motivada por la falta de estudios y experiencias que relacionan el agua como medio de aprendizaje con las personas gravemente afectadas. Se ha trabajado con una muestra de d...

  7. 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

  8. 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...

  9. 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 ...

  10. 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 ...

  11. 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

  12. Pathogenetic mechanisms in radiation fibrosis

    International Nuclear Information System (INIS)

    Deregulation of normal regenerative responses to physical, chemical and biological toxins in susceptible individuals leads to abnormal remodelling of extracellular matrix with pathological fibrosis. Processes deregulated after radiotherapy have much in common with processes associated with fibrotic diseases affecting the heart, skin, lungs, kidneys, gastro-intestinal tract and liver. Among the secreted factors driving fibrosis, transforming growth factor beta 1 (TGFβ1) produced by a wide range of inflammatory, mesenchymal and epithelial cells converts fibroblasts and other cell types into matrix-producing myofibroblasts. Even if required for the initiation of fibrosis, inflammation and the continued stimulus of TGFβ1 may not be needed to maintain it. After myofibroblast activation, collagen production can be perpetuated independently of TGFβ1 by autocrine induction of a cytokine called connective tissue growth factor. The role of inflammation, the origins and activation of myofibroblasts as biosynthetic cells and the downstream pathways of extracellular matrix synthesis in common fibrotic states are reviewed. Oxidative stress, hypoxia and microvascular damage are also considered, before examining the same processes in the context of radiotherapy. One of the main uncertainties is the relevance of very early events, including inflammatory responses in blood vessels, to fibrosis. Despite the power of animal models, including genetic systems, the potential contribution of research based on human tissue samples has never been greater. A closer interaction between scientists researching fibrosis and radiation oncologists holds enormous promise for therapeutic advances.

  13. 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 ...

  14. 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 ...

  15. 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.)

  16. 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 ...

  17. 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.

  18. 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

  19. Quantification of portal-bridging fibrosis area more accurately reflects fibrosis stage and liver stiffness than whole fibrosis or perisinusoidal fibrosis areas in chronic hepatitis C.

    Science.gov (United States)

    Sandrini, Jérémy; Boursier, Jérôme; Chaigneau, Julien; Sturm, Nathalie; Zarski, Jean-Pierre; Le Bail, Brigitte; de Ledinghen, Victor; Calès, Paul; Rousselet, Marie-Christine

    2014-07-01

    Morphometry provides an objective evaluation of fibrosis in liver diseases. We developed an image analysis algorithm using automated thresholding and segmentation to separately quantify the areas and the fractal dimensions of portal-bridging fibrosis and perisinusoidal fibrosis in chronic hepatitis C liver biopsies. We studied 427 digitized liver biopsies and compared the automated measures of the different fibrosis compartments with (1) the Metavir F (fibrosis) and A (activity) histological scores, (2) the digitally assessed area of steatosis, and (3) the liver stiffness measured by elastography (Fibroscan). The perisinusoidal fibrosis area was higher than that of portal fibrosis in stages ≤F2; it reached its highest value in F2 stage and stabilized thereafter. The F3 stage was characterized by equal proportions of portal-bridging and perisinusoidal fibrosis, whereas portal-bridging area was predominant in cirrhosis. Measurement of portal-bridging fibrosis showed highly significantly different values between contiguous F stages; the ratio of portal-bridging fibrosis/perisinusoidal fibrosis displayed less overlap between Metavir stages than did the whole fibrosis area values. Fractal dimension showed that portal-bridging fibrosis tended to display a homogeneous surface-like spatial organization, whereas perisinusoidal fibrosis appeared more heterogeneous according to stage and curvilinear. The portal-bridging fibrosis area was low in cases with low Metavir activity and little steatosis, and became predominant with increasing activity and steatosis. Using stepwise multiple linear regression analysis, the liver stiffness was independently correlated to the portal-bridging fibrosis area (first step, P<0.001), the steatosis area (second step, P<0.001), and the Metavir A grade (third step, P=0.001), but not to the perisinusoidal fibrosis area. Automated quantification in a large cohort of chronic hepatitis C showed that perisinusoidal fibrosis progressively grew in

  20. Antifibrotic therapies to control cardiac fibrosis

    OpenAIRE

    Fan, Zhaobo; Guan, Jianjun

    2016-01-01

    Cardiac fibrosis occurs naturally after myocardial infarction. While the initially formed fibrotic tissue prevents the infarcted heart tissue from rupture, the progression of cardiac fibrosis continuously expands the size of fibrotic tissue and causes cardiac function decrease. Cardiac fibrosis eventually evolves the infarcted hearts into heart failure. Inhibiting cardiac fibrosis from progressing is critical to prevent heart failure. However, there is no efficient therapeutic approach curren...

  1. 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...

  2. 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 ...

  3. 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

  4. 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

  5. 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...

  6. Update on nephrogenic systemic fibrosis

    DEFF Research Database (Denmark)

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

    2008-01-01

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

  7. 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...

  8. 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...

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

    Directory of Open Access Journals (Sweden)

    González Rodríguez, José Manuel

    2011-01-01

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

  10. 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...

  11. [Morbus Ormond (idiopatic retroperitoneal fibrosis)].

    Science.gov (United States)

    Michaligová, A; Plank, L; Jezíková, A; Manka, V; Makovický, P; Mokán, M

    2011-05-01

    Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by the development of fibrotic tissue around the abdominal aorta and iliac arteries and often involves structures as ureters and the inferior vena cava. The age at onset of signs and symptoms is between 40-60 years, males predominane over females. In most cases the clinical manifestation is presented as compressive syndrom of ureters, therefore the first known cases were described by urologists. In this report we present the case of 37-years old male examinated for persistent fever about 38 degrees C and high inflammatory activity in spite of empiric antibiotic therapy. Positron emission tomography (PET) showed locality of high metabolic activity of fluorodeoxyglucose with maximum paraaortal left. Microscopic examination of extracted mass showed presence of fibrous and inflammatory components. With clinical presentation, imaging and histological findings we made out the diagnosis of idiopathic retroperitoneal fibrosis--morbus Ormond. PMID:21695934

  12. 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

  13. 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.)

  14. Epidemiology of idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Ley B

    2013-11-01

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

  15. [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

  16. Epidemiology of idiopathic pulmonary fibrosis

    OpenAIRE

    Ley, Brett

    2013-01-01

    Brett Ley, Harold R Collard Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California San Francisco, San Francisco, California, USA Abstract: Idiopathic pulmonary fibrosis is a chronic fibrotic lung disease of unknown cause that occurs in adults and has a poor prognosis. Its epidemiology has been difficult to study because of its rarity and evolution in diagnostic and coding practices. Though uncommon, it is likely underappreciated both in terms of it...

  17. Epidemiology of idiopathic pulmonary fibrosis

    OpenAIRE

    Ley B; Collard HR

    2013-01-01

    Brett Ley, Harold R Collard Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California San Francisco, San Francisco, California, USA Abstract: Idiopathic pulmonary fibrosis is a chronic fibrotic lung disease of unknown cause that occurs in adults and has a poor prognosis. Its epidemiology has been difficult to study because of its rarity and evolution in diagnostic and coding practices. Though uncommon, it is likely underappreciated both in terms of its...

  18. 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.

  19. 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...

  20. 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...

  1. 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...

  2. 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.

  3. 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

  4. 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

  5. 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

  6. 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 to be a...

  7. 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 to be a...

  8. 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...

  9. 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

  10. 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...

  11. 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...

  12. 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.

  13. 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...

  14. 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...

  15. 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 ...

  16. 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.

  17. 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 ...

  18. 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, ...

  19. Cystic Fibrosis Research | NIH MedlinePlus the Magazine

    Science.gov (United States)

    ... 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. Cellular basis of radiation-induced fibrosis

    International Nuclear Information System (INIS)

    Fibrosis is a common sequela of both cancer treatment by radiotherapy and accidental irradiation and has been described in many tissues including skin, lung, heart and liver. The underlying mechanisms of the radiation-induced fibrosis still remain to be resolved. In the present review we tried to illustrate the basic cellular mechanisms of radiation-induced fibrosis based on the newest findings arising from molecular radiobiology and cell biology. Based on these findings the cellular mechanism of radiation-induced fibrosis can be seen as a multicellular process involving various interacting cell systems in the target organ resulting in the fibrotic phenotype of the fibroblast/fibrocyte cell system

  1. 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

  2. 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.

  3. 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

  4. 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.

  5. Niñez afectada con VIH/SIDA: Calidad de vida, funcionalidad familiar y apoyo social en cinco ciudades colombianas

    OpenAIRE

    Ana María Trejos Herrera; Mario Mosquera Vásquez; Rafael Tuesca Molina

    2009-01-01

    Objetivo: Describir calidad de vida, funcionalidad familiar y apoyo social en menores de 18 años afectados con VIH/sida y cuidadores residentes en Cali, Buenaventura, Barranquilla, Santa Marta y Cartagena. Materiales y métodos: 286 encuestas personales realizadas a 11 menores que conocían su diagnóstico de seropositivos para VIH y 275, a cuidadores de menores que aún no conocían situación de afectación para VIH; se aplicaron en instituciones de salud tanto del sector estatal como del privado,...

  6. GeoProcesamiento integrado con ArcObjects para generar afecciones por infraestructuras eléctricas de alta tensión.

    OpenAIRE

    Gutierrez Corea, Federico Vladimir; Manso Callejo, Miguel Ángel; Moreno Marimbaldo, Francisco Javier

    2012-01-01

    El presente trabajo muestra el caso de estudio de las afecciones y la Relaciones de Bienes y Derechos (RBD) afectados por nuevas infraestructuras eléctricas de transporte (obras lineales) en España. El reto consiste en ayudar al cumplimiento de los objetivos de la planificación, definida por el MITYC (revisada para 2020), para construir nuevos circuitos y subestaciones. Se ha desarrollado un Software con ArcObjects que integra/automatiza las distintas etapas del flujo de trabajo asociado: (a)...

  7. Evaluación y programas de intervención psicoeducativa en niños con tumores en el cerebelo

    OpenAIRE

    Grau Rubio, Claudia; Fortes del Valle, M. Carmen

    2003-01-01

    Se describen las secuelas neurológicas en niños diagnosticados de un tumor en el cerebelo y se ofrecen los resultados de la evaluación de 39 niños, con tumor en el cerebelo, tratados en la Unidad de Oncología Pediátrica del Hospital Infantil “La Fe” de Valencia. Asimismo, se compara la evaluación de secuelas de dos niños de educación infantil afectados de un tumor en el cerebelo: Yolanda (meduloblastoma, PNET) y Santiago (astrocitoma cerebeloso), y se describen las áreas en las que es acon...

  8. Implicaciones del tratamiento antirretroviral en la disfunción sexual del paciente con infección por el virus de la inmunodeficiencia humana.

    OpenAIRE

    Cánoves Martínez, Mª Dolores

    2007-01-01

    RESUMEN Los años 80 fueron el inicio conocido de los primeros casos de VIH y ya desde entonces, se describió el origen de la enfermedad como la consecuencia de los estilos de vida de los afectados. La información facilitada en este sentido, contribuyó a la creación de estereotipos; que con variantes, siguen hasta la actualidad; De esta forma, es habitual que los pacientes sufran además de los problemas biológicos, los psicológicos y los sociales asociados, característica ...

  9. 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...

  10. EVALUACIÓN DE LA SATISFACCIÓN DEL PROFESORADO DE CIENCIAS CON LA ADAPTACIÓN AL ESPACIO EUROPEO DE EDUCACIÓN SUPERIOR

    OpenAIRE

    Raúl Quevedo-Blasco; Tania Ariza,; Gualberto Buela-Casal

    2015-01-01

    La implantación del Espacio Europeo de Educación Superior (EEES) ha supuesto una transformación de la educación superior en España. Sin embargo, se desconoce cuál es la actitud del profesorado universitario hacia el proceso y cómo ha afectado esta reforma a sus funciones docentes y como investigador. Por ello, se evalúa el grado de satisfacción hacia distintos aspectos relacionados con el EEES. Los participantes fueron 1.958 profesores que imparten docencia en titulaciones de la rama de Cienc...

  11. SEGURIDAD E INOCUIDAD ALIMENTARIA EN HOGARES DE JORNALEROS DE FINCAS CAFETERAS CON Y SIN CERTIFICACIÓN DEL SUROESTE DE ANTIOQUIA - COLOMBIA

    OpenAIRE

    Oscar Manrique Chica; Javier Rosique Gracia

    2014-01-01

    Antecedentes: El cambio en los modelos de producción cafeteros asociados a la introducción de cafés certificados ha afectado significativamente el estudio de la calidad e inocuidad de los alimentos en las zonas cafeteras de Antioquia. Sin embargo, se espera que este tipo de productos tengan un efecto positivo en la economía, la calidad de vida y la seguridad alimentaria de los sectores relacionados con la producción. Objetivos: Determinar el efecto de la certificación de las fincas cafeteras ...

  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. Monitoring spatial and temporal dynamics of bird communities in Mediterranean landscapes affected by largewildfires = Seguimiento de la dinámica espacial y temporal de comunidades de aves en paisajes mediterráneos afectados por grandes fuegos

    OpenAIRE

    Zozaya, Elena L.; Brotons, Lluís; Herrando, Sergi; Pons, Pere; Rost, J.; Clavero, Miguel

    2010-01-01

    [ES] Se ha presentado una base de datos de aves en paisajes mediterráneos (Cataluña, NE España) afectados por incendios forestales y se ha evaluado: 1) la variabilidad espacial y temporal de la respuesta de la comunidad de aves al fuego y 2) el impacto de la vegetación antes del incendio en la composición de la comunidad de aves. La base de datos DINDIS contiene información avifaunística de todas las áreas forestales quemadas desde el año 2000 en Cataluña. Se utiliza la información disponible...

  14. 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 ...

  15. 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

  16. 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

  17. 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

  18. 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...

  19. Antibiotic allergy in cystic fibrosis

    OpenAIRE

    Parmar, J.; Nasser, S.

    2005-01-01

    Allergic reactions to antibiotics are more common in cystic fibrosis (CF) than in the general population. This in part is due to the improving survival in adults with CF and the increased use of high dose intravenous antibiotics. While some are immediate anaphylaxis type (IgE mediated) reactions, the majority are late onset and may have non-specific features such as rash and fever. Piperacillin has consistently been found to have the highest rate of reported reactions (30–50%). There is a low...

  20. 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

  1. 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.

  2. 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.

  3. 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.

  4. Imaging pulmonary fibrosis; Imagerie des fibroses pulmonaires

    Energy Technology Data Exchange (ETDEWEB)

    Brauner, M.W.; Rety, F.; Naccache, J.M.; Girard, F.; Valeyre, D.F. [Hopital Avicenne, 93 - Bobigny (France). Service de radiologie et de pneumologie

    2001-02-01

    Localized fibrosis of the lung is usually scar tissue while diffuse pulmonary fibrosis is more often a sign of active disease. Chronic infiltrative lung disease may be classified into four categories: idiopathic pneumonitis, collagen diseases, granulomatosis (sarcoidosis), and caused by known diseases (pneumoconiosis, hypersensitivity pneumonitis, drug-induced lung disease, radiation). (authors)

  5. 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.

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

    Science.gov (United States)

    Cohen-Ezra, Oranit; Ben-Ari, Ziv

    2015-03-01

    Chronic liver diseases represent a major public health problem, accounting for significant morbidity and mortality worldwide. Prognosis and management of chronic liver diseases depend on the amount of liver fibrosis. Liver biopsy has long remained the gold standard for assessment of liver fibrosis. Liver biopsy is an invasive procedure with associated morbidity, it is rarely the cause for mortality, and has a few limitations. During the past two decades, in an attempt to overcome the limitations of liver biopsy, non-invasive methods for the evaluation of liver fibrosis have been developed, mainly in the field of viral hepatitis. This review will focus on different methods available for non-invasive evaluation of liver fibrosis including a biological approach which quantifies serum levels of biomarkers of fibrosis and physical techniques which measure liver stiffness by transient elastography, ultrasound or magnetic resonance based elastography, their accuracy, advantages and disadvantages. PMID:25962254

  7. 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.

  8. 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.)

  9. 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.

  10. 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

  11. Pharmacotherapy in idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Gaurav Acharya

    2014-10-01

    Full Text Available Idiopathic pulmonary fibrosis or cryptogenic fibrosing alveolitis is a form of chronic, progressive interstitial lung disease causing scarring of lung tissue and usually affect adults. Treatment is usually aimed at controlling inflammation and thus slowing the process of fibrosis. With only few patients responding to treatment and the disease being ultimately fatal with poor progression, the underlying lesion was considered to be fibrotic rather than inflammatory. Fibrotic foci, deposition of collagen, and lack of inflammatory cells are a predominant finding. Pirfenidone and N-acetyl cysteine are the only effective pharmacotherapy available till date. Interim results of PANTHER Trial clearly indicate more risk with triple therapy. However, in Indian patients, trial of steroid therapy may be tried when there is doubt of chronic hypersensitivity pneumonitis. BIBF 1120 has also shown positive results in Phase II clinical trial and shows a positive response in deteriorating lung function. Supplemental oxygen, education of patient, pulmonary rehabilitation, and Streptococcus pneumoniae and influenza vaccine are the most important supportive care. Pulmonary rehabilitation should be used as a treatment in the majority of patients. [Int J Basic Clin Pharmacol 2014; 3(5.000: 761-763

  12. [Doppler echocardiography in endomyocardial fibrosis].

    Science.gov (United States)

    Tello, R; Cuan, V; Abundes, A; Navarro, J; García Lara, J; Astudillo, R; Ariza, H; Cuan, M

    1994-01-01

    Twelve patients with endomyocardial fibrosis with angiographic and/or histologic corroboration were studied with Doppler echocardiography with the purpose of describing the echocardiographic features and identify the affected sites. The average age was 41 years (range 16 to 59 years), 2 men and 10 women. Three patients (25%) had isolated right ventricular involvement, one patient (8%) left ventricular, 8 patients (66%) both ventricular. Our Doppler echocardiographic findings were: right atrium enlargement (91%), right ventricle outflow dilatation (83%), paradoxical septal motion (83%), left atrial enlargement (33%), mitral and tricuspid valve prolapse (50%), pericardial effusion (41%), mitral regurgitation (75%), tricuspid regurgitation (100%), apex obliteration (50%) and a restrictive type flow pattern (50%). Doppler echocardiography is a useful method for the diagnosis of endomyocardial fibrosis, the finding of normal or small ventricles associated with apex obliteration and enlarged atria, mitral or tricuspid regurgitation and a restrictive type flow pattern are characteristics of this disease. In our population, the isolated or predominantely right ventricular involvement is the most common finding as it represented 83% of the cases. PMID:7979815

  13. 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.

  14. 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.

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

    Directory of Open Access Journals (Sweden)

    Pedro Puentes Rozo

    2014-07-01

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

  17. 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. 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.

  19. Retroperitoneal fibrosis : spectrum of imaging findings

    International Nuclear Information System (INIS)

    Retroperitoneal fibrosis is a fibroproliferative process involving the retroperitoneum. It may be idiopathic or can be caused by methysergide ingestion, perianeurysmal inflammation, a leaking aneurysm, urinoma or irradiation. The symptoms and signs of retroperitoneal fibrosis are variable, and for diagnosis, imaging is therefore essential. The typical imaging findings is a fibrotic lesion in front of the lower vertebrae with ureteral obstruction. Atypical lesions, however, may occur in other parts of the retroperitoneum. The aim of this report is to describe the clinical features and various imaging findings of retroperitoneal fibrosis

  20. Familial extensive idiopathic bilateral pleural fibrosis.

    Science.gov (United States)

    Azoulay, E; Paugam, B; Heymann, M F; Kambouchner, M; Haloun, A; Valeyre, D; Battesti, J P; Tazi, A

    1999-10-01

    The authors report three sisters with bilateral isolated apical pleural fibrosis of unknown origin, which did not respond to empirical antituberculosis therapy and oral corticosteroids. The disease evolved in an unrelenting fashion producing pleural fibrosis at the lung bases and leading to the death of two sisters and to lung transplantation in the other one. There was no history of other familial disease or consanguinity. The particular features of these cases and the differences from other reports of apparently cryptogenic pleural fibrosis are outlined. PMID:10573252

  1. 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...

  2. Cifrado con cubos marcados

    OpenAIRE

    Coriat, Moisés; María C. Cañadas

    2011-01-01

    Con cuatro de los 8 tetracubos y con la ayuda de la noción de cubo mínimo (cubo formado por dos tetracubos), introducimos los “cubo marcados”. A su vez, usamos esta idea para generar claves con las que cabe transmitir mensajes cifrados. Damos ejemplos de claves y mencionamos algunas posibilidades más de cifrado con dichos cubos marcados. En la introducción presentamos algunas facetas del cifrado. Después, describimos algunos policubos y prestamos especial atención a los cuatro tetracubo...

  3. 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

  4. 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.)

  5. Retroperitoneal fibrosis: a medical enigma

    International Nuclear Information System (INIS)

    Retroperitoneal fibrosis is an uncommon disease of unclear cause. It was described for the first time in English literature by Ormond, in 1948. However, the first known description of this disease was done by the French urologist Albarran, around 1905. It is characterized by chronic nonspecific inflammation of the retroperitoneum that can entrap and obstruct retroperitoneal structures, notably the ureters. This disease frequently is detected only after severe renal failure is present. However, advances in imaging techniques and increased awareness of the disease have resulted in an earlier diagnosis in unobstructed patients. As a result of the observations that showed that the only difference between an inflammatory aneurysm of the abdominal aorta and Ormond's disease is the diameter of the vessel, it had been suggested that both syndromes represent variations of the same pathology, which has been named chronic periaortitis

  6. 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...

  7. 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...

  8. Influencia de factores epidemiológicos en la progresión de la fibrosis en la hepatitis crónica C

    Directory of Open Access Journals (Sweden)

    Eduardo Vilar Gómez

    2003-10-01

    Full Text Available Aproximadamente 80 % de la hepatitis C evoluciona a la cronicidad. Factores como los epidemiológicos, virales y del huésped intervienen en la progresión de la enfermedad. Se describió el comportamiento de variables epidemiológicas asociadas a la hepatitis crónica C y se identificó la posible asociación de estas con la intensidad de la fibrosis. Se estudiaron 80 pacientes con hepatitis C. Se determinó la intensidad de la fibrosis por el sistema de puntuación METAVIR. Se agruparon en: ausencia o poca fibrosis (F0-F1 y fibrosis marcada (F2 a F4 buscando la posible asociación estadística con los factores epidemiológicos. Se halló asociación significativa entre el estimado de antigüedad de la infección, la vía de adquisición y la edad al contraerla con la intensidad de la fibrosis. La progresión de la hepatitis C puede estar determinada por algunos factores epidemiológicos que influyen de forma decisiva en la historia natural de la enfermedad.Approximately 80 % of hepatitis C evolves to chronicity. The epidemiological and viral factors as well as those of the guest take part in the progression of the disease. The behavior of the epidemiological variables associated with chronic hepatitis C was described and the possible association of these ones with the intensity of fibrosis was identified. 80 patients with hepatitis C were studied and the intensity of fibrosis was determined by the METAVIR scoring system. They were grouped into absence or little fibrosis (f0-F1 and marked fibrosis (F2-F4 seeking for the possible statistical association with the epidemiological factors. A significant association was found among the infection's oldness estimate, the way it was acquired, the patient's age on catching it and the intensity of fibrosis. The progression of hepatits C may be determined by some epidemiological factors influencing decisively the natural history of the disease.

  9. 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

  10. Pathohistological changes in fetuses with cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Đolai Matilda

    2012-01-01

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

  11. Nephrogenic systemic fibrosis: clinical picture and treatment

    DEFF Research Database (Denmark)

    Marckmann, Peter; Skov, Lone

    2009-01-01

    The classic hallmark symptoms of advanced nephrogenic systemic fibrosis (NSF) (skin thickening, hardening and hyperpigmentation, and disabling contractures in renal failure patients) in temporal association with Gd-based contrast agent (GBCA) exposure are almost pathognomonic of NSF. Less obvious...

  12. 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

  13. Imaging findings in idiopathic pelvic fibrosis

    International Nuclear Information System (INIS)

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

  14. "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

  15. 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

  16. 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.

  17. 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.

  18. Non-Invasive markers for hepatic fibrosis

    OpenAIRE

    Lal Priyanka; Baranova Ancha; Birerdinc Aybike; Younossi Zobair M

    2011-01-01

    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...

  19. 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...

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

    Directory of Open Access Journals (Sweden)

    Alina Torreblanca Xiques

    2014-11-01

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

  1. Role of Circulating Fibrocytes in Cardiac Fibrosis

    Science.gov (United States)

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

    2016-01-01

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

  2. Inhibition of SIRT2 suppresses hepatic fibrosis.

    Science.gov (United States)

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

    2016-06-01

    Liver fibrosis can progress to cirrhosis and result in serious complications of liver disease. The pathogenesis of liver fibrosis involves the activation of hepatic stellate cells (HSCs), the underlying mechanisms of which are not fully known. Emerging evidence suggests that the classic histone deacetylases play a role in liver fibrosis, but the role of another subfamily of histone deacetylases, the sirtuins, in the development of hepatic fibrosis remains unknown. In this study, we found that blocking the activity of sirtuin 2 (SIRT2) by using inhibitors or shRNAs significantly suppressed fibrogenic gene expression in HSCs. We further demonstrated that inhibition of SIRT2 results in the degradation of c-MYC, which is important for HSC activation. In addition, we discovered that inhibition of SIRT2 suppresses the phosphorylation of ERK, which is critical for the stabilization of c-MYC. Moreover, we found that Sirt2 deficiency attenuates the hepatic fibrosis induced by carbon tetrachloride (CCl4) and thioacetamide (TAA). Furthermore, we showed that SIRT2, p-ERK, and c-MYC proteins are all overexpressed in human hepatic fibrotic tissues. These data suggest a critical role for the SIRT2/ERK/c-MYC axis in promoting hepatic fibrogenesis. Inhibition of the SIRT2/ERK/c-MYC axis represents a novel strategy to prevent and to potentially treat liver fibrosis and cirrhosis. PMID:27125275

  3. 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

  4. 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

  5. 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.

  6. Caracterización del acoplamiento de una fuente de luz LED a una fibra óptica con aplicación en la terapia fotodinámica (TFD) del cáncer cérvix

    OpenAIRE

    Etcheverry, María Eugenia; Pasquale, Miguel Ángel; Garavaglia, Mario J.

    2015-01-01

    La terapia fotodinámica (TFD) se basa en la activación con luz de longitud de onda apropiada de un fotosensibilizador en presencia de oxígeno, causando la muerte de las células tumorales del tejido afectado. Cualquier fuente que emita en la zona de absorción del fotsensibilizador y capaz de penetrar suficientemente en el tejido puede usarse en la TFD. El espectro de absorción de los fotosensibilizadores actualmente utilizados presenta una banda de absorción intensa a 420 nm (Banda de Soret) y...

  7. 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...

  8. con marcos extradensos

    Directory of Open Access Journals (Sweden)

    Manuel Rodríguez

    2007-01-01

    Full Text Available El cultivo del plátano vianda al igual que el de la papaya, en la región occidental del país, son fuertemente atacados por enfermedades que hacen que las plantaciones, por el grado de deterioro que estas les producen, no duren más de un ciclo de cultivo. Sembrar una misma área, un año tras de otro con el mismo cultivo, trae consigo el establecimiento en el suelo de elementos parásitos dañinos al mismo, que pueden ser eliminados o atenuados con una rotación con otro cultivo. El presente trabajo expone los resultados experimentales de dos años de trabajo de la rotación de estos cultivos, plantados con dos marcos de plantación de alta densidad, 2 x 4 x 1 m y 3 x 1 m, con una densidad de 3333 plantas/ha y regados con la técnica de riego por goteo. Se obtuvieron rendimientos de 40,73 t/ha en plátano y de 71,42 t/ha en papaya, con una relación beneficio costo de 1,15, tasa interna de retorno de 36,27 %, valor actual neto de 21,47 y plazo de recuperación de la inversión de 3,01 años.

  9. 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.

  10. 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)

  11. 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.

  12. 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.

  13. Antibiotic allergy in cystic fibrosis.

    Science.gov (United States)

    Parmar, J S; Nasser, S

    2005-06-01

    Allergic reactions to antibiotics are more common in cystic fibrosis (CF) than in the general population. This in part is due to the improving survival in adults with CF and the increased use of high dose intravenous antibiotics. While some are immediate anaphylaxis type (IgE mediated) reactions, the majority are late onset and may have non-specific features such as rash and fever. Piperacillin has consistently been found to have the highest rate of reported reactions (30-50%). There is a low risk of cross reactions between penicillins and other non-beta-lactam classes of antibiotics in penicillin skin prick positive patients. Carbapenems should only be used with extreme caution in patients with positive skin prick tests to penicillin. However, aztreonam can be used safely in patients who are penicillin allergic with positive skin prick reactions. The aminoglycosides are a relatively uncommon cause of allergic reactions, but patients who react to one member of the family may cross react with other aminoglycosides. Desensitisation relies on the incremental introduction of small quantities of the allergen and has been used for penicillins, ceftazidime, tobramycin and ciprofloxacin and must be repeated before each course. Personalized cards should be regularly updated for patients who develop allergic reactions. Written instructions on the emergency treatment of allergic reactions should be provided to patients self-administering intravenous antibiotics at home. Further research is required to identify risk factors and predictors for antibiotic allergy. PMID:15923254

  14. 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...

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

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    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.

  16. 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.

  17. 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

  18. The Role of Catalase in Pulmonary Fibrosis

    Directory of Open Access Journals (Sweden)

    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.

  19. Acute exacerbation of airspace enlargement with fibrosis

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    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.

  20. 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

  1. 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.

  2. Microbial ecology and adaptation in cystic fibrosis airways

    DEFF Research Database (Denmark)

    Yang, Lei; Jelsbak, Lars; Molin, Søren

    2011-01-01

    Chronic infections in the respiratory tracts of cystic fibrosis (CF) patients are important to investigate, both from medical and from fundamental ecological points of view. Cystic fibrosis respiratory tracts can be described as natural environments harbouring persisting microbial communities with...

  3. 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.

  4. 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...

  5. 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...

  6. 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.

  7. 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

  8. 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...

  9. 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...

  10. 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.

  11. 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.

  12. 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;…

  13. 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

  14. 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.)

  15. 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...

  16. 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.

  17. 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.

  18. 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. PMID:26857764

  19. Cystic fibrosis, intravenous antibiotics, and home therapy.

    Science.gov (United States)

    Hammond, L J; Caldwell, S; Campbell, P W

    1991-01-01

    The survival rate of patients with cystic fibrosis has improved considerably in the last 20 years. Although not all of the factors accounting for this change are understood, aggressive nutritional management and treatment of pulmonary exacerbations certainly play a role. Home intravenous (IV) antibiotic delivery for pulmonary exacerbation has proved to be as effective as hospital treatment and offers significant advantages to the patient and family. This article examines the microbiology of pulmonary infections in patients with cystic fibrosis, as well as antimicrobial therapy, methods of IV administration, home IV therapy, and the nurse practitioner's role in this home program in the future. PMID:1990112

  20. Idiopathic pulmonary fibrosis: an Australian perspective.

    Science.gov (United States)

    Prasad, J; Holland, A E; Glaspole, I; Westall, G

    2016-06-01

    Idiopathic pulmonary fibrosis is a progressive interstitial lung disease of unknown aetiology with a dismal median survival of 3 years. Patients typically develop progressive dyspnoea and increasing exercise limitation. With a rising incidence and prevalence, an unpredictable disease course and limited treatment options, it is rapidly becoming an important public health concern. To date, lung transplantation has been the sole viable hope for treatment for those who qualify. However, the landscape of idiopathic pulmonary fibrosis management is changing, with the recent emergence of novel pharmacotherapy shown to have a favourable influence on the natural history of this disease. PMID:27257148

  1. 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

  2. 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

  3. 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 ...

  4. Neutral zone and oral submucous fibrosis

    Directory of Open Access Journals (Sweden)

    Shaista Afroz

    2012-01-01

    Full Text Available Oral submucous fibrosis is a premalignant condition in which rigidity of the lip, tongue, and palate results in reduced mouth opening and tongue movement. Limited mouth opening, mucosal rigidity, and reduced salivary flow makes prosthodontic procedures difficult in these patients and affects the stability, retention, and the support of removable prostheses. The burning sensation in the mouth that these patients experience reduces the tolerance to prostheses. We report a case of oral submucous fibrosis where the conventional neutral zone technique with certain modifications was utilized to rehabilitate a completely edentulous patient with this condition.

  5. 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.

  6. 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. ...

  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. 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....

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

    Directory of Open Access Journals (Sweden)

    X. Zamorano Pino

    2015-12-01

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

  10. 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.

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

    Directory of Open Access Journals (Sweden)

    Córdoba Carlos Vicente

    1990-06-01

    Full Text Available

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

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

  12. 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...

  13. 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...

  14. 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...

  15. 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...

  16. Factores de riesgo relacionados con la conducta suicida en la infancia y adolescencia

    Directory of Open Access Journals (Sweden)

    Ariane Hernández Trujillo

    2013-12-01

    Full Text Available Se realizó un estudio observacional, descriptivo y transversal en el Hospital Infantil Norte Docente "Dr. Juan de la Cruz Martínez Maceira" de la provincia de Santiago de Cuba en el 2010, a fin de describir los factores relacionados con la conducta suicida en 26 de 31 adolescentes de 11 a 17 años, atendidos en dicha institución durante ese período. Se halló que el grupo más afectado fue el de 14-15 años, con primacía del sexo femenino. Entre los factores de riesgo predominantes figuraron, por citar los principales en la casuística: los maltratos físicos y psicológicos como las humillaciones efectuadas por los propios padres; la falta de redes de apoyo familiar en la mayoría de ellos; la ausencia de solución de sus problemas; la ingestión de medicamentos para suicidarse, fundamentalmente psicofármacos, así como la depresión y el consumo de alcohol como antecedentes patológicos familiares.

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

    Directory of Open Access Journals (Sweden)

    Alicia Maria Aquino Valdovinos

    2015-08-01

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

  18. 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...

  19. 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...

  20. Pseudomonas aeruginosa biofilms in cystic fibrosis

    DEFF Research Database (Denmark)

    Høiby, Niels; Ciofu, Oana; Bjarnsholt, Thomas

    2010-01-01

    The persistence of chronic Pseudomonas aeruginosa lung infections in cystic fibrosis (CF) patients is due to biofilm-growing mucoid (alginate-producing) strains. A biofilm is a structured consortium of bacteria, embedded in a self-produced polymer matrix consisting of polysaccharide, protein and...

  1. 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...

  2. 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 of...

  3. Inhalation of antibiotics in cystic fibrosis

    NARCIS (Netherlands)

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

    1995-01-01

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

  4. 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.

  5. MicroRNA mimicry blocks pulmonary fibrosis

    Science.gov (United States)

    Montgomery, Rusty L; Yu, Guoying; Latimer, Paul A; Stack, Christianna; Robinson, Kathryn; Dalby, Christina M; Kaminski, Naftali; van Rooij, Eva

    2014-01-01

    Over the last decade, great enthusiasm has evolved for microRNA (miRNA) therapeutics. Part of the excitement stems from the fact that a miRNA often regulates numerous related mRNAs. As such, modulation of a single miRNA allows for parallel regulation of multiple genes involved in a particular disease. While many studies have shown therapeutic efficacy using miRNA inhibitors, efforts to restore or increase the function of a miRNA have been lagging behind. The miR-29 family has gained a lot of attention for its clear function in tissue fibrosis. This fibroblast-enriched miRNA family is downregulated in fibrotic diseases which induces a coordinate increase of many extracellular matrix genes. Here, we show that intravenous injection of synthetic RNA duplexes can increase miR-29 levels in vivo for several days. Moreover, therapeutic delivery of these miR-29 mimics during bleomycin-induced pulmonary fibrosis restores endogenous miR-29 function whereby decreasing collagen expression and blocking and reversing pulmonary fibrosis. Our data support the feasibility of using miRNA mimics to therapeutically increase miRNAs and indicate miR-29 to be a potent therapeutic miRNA for treating pulmonary fibrosis. PMID:25239947

  6. 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.

  7. 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

  8. 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 acina...... (CF) and pancreatitis, and outline present and potential therapeutic approaches in CF treatment relevant to the pancreas....

  9. [Macrolides, Pseudomonas aeruginosa and cystic fibrosis].

    Science.gov (United States)

    Guillot, M; Amiour, M; El Hachem, C; Harchaoui, S; Ribault, V; Paris, C

    2006-10-01

    Long-term low dose azithromycin treatment in cystic fibrosis patients with chronic Pseudomonas aeruginosa infection is safe and reduces the decline in lung function, the number of acute exacerbations and improves nutritional status; underlying efficacy mechanisms are multiple and synergistic. PMID:17370396

  10. Nephrogenic systemic fibrosis: history and epidemiology

    DEFF Research Database (Denmark)

    Thomsen, Henrik S

    2009-01-01

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

  11. 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

  12. The role of apoptosis in pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    B. D. Uhal

    2008-12-01

    Full Text Available Apoptosis has been defined as "gene-directed cellular self-destruction" and is an active process that is tightly regulated by a number of gene products, which promote or block cell death. Apoptotic death can be triggered by a wide variety of stimuli and, importantly, not all cells necessarily undergo apoptosis in response to the same stimulus. Abnormal regulation of apoptosis has been implicated in a wide range of diseases and approaches to modifying apoptosis represent important future therapeutic strategies. Idiopathic pulmonary fibrosis (IPF is a progressive and relentless disease involving scarring of the lung, which has been recognised as the most lethal interstitial lung disease. In the lungs of IPF patients, increased epithelial apoptosis, together with decreased apoptosis of myofibroblasts, represents persistent findings (particularly in areas of collagen deposition supporting an interaction between altered apoptosis and the pathogenesis of the disease. Data from human tissues and animal models are refining current knowledge of the processes involved in this pathogenesis. This has challenged the dogma that IPF is purely a disease of unresolved inflammation by emphasising the central roles played by the alveolar epithelial cell and myofibroblasts and, as part of that role, the importance of altered apoptosis. Evidence suggests blockade of epithelial cell apoptosis can prevent subsequent collagen deposition, and induction of myofibroblast apoptosis, at least theoretically, would be expected to resolve ongoing fibrosis. These two concepts raise the prospect of therapeutic intervention aimed at modifying apoptosis and, thus, fibrosis in idiopathic pulmonary fibrosis.

  13. 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

  14. Serum hyaluronic acid as a marker of hepatic fibrosis

    International Nuclear Information System (INIS)

    To determine the serum hyaluronic acid (HA) levels as biochemical marker of hepatic fibrosis and cirrhosis and correlate it with the degree of hepatic fibrosis and cirrhosis. This study was performed on 100 patients of chronic liver disease whose liver biopsies had been carried out. Fifty healthy controls were also included in the study. Routine liver function tests, hepatitis serology and serum hyaluronic acid levels were carried out on patients and controls. Liver biopsy of 100 patients revealed that 21 were in stage 0 fibrosis, 38 in stage 1 fibrosis, 26 in stage 3 fibrosis and 15 in stage 4 fibrosis. Mean Serum HA (mean +- SD) concentration in patients were 189 +- 98 mg/L vs. 21 +- 10 mg/L of healthy controls. The difference observed was statistically significant (p < 0.001). Patients in stage 4 fibrosis had significantly higher (p <0.001) mean serum HA concentration as compared to other stages of liver fibrosis. Diagnostic accuracy of serum HA at marginally elevated level of 60 mg/L determined the sensitivity 78.4 %, specificity 80.9%, positive predicted value 93.9% and negative predicted value of 50%. Serum HA is a useful non-invasive marker of liver fibrosis. There is a strong positive correlation between serum HA levels and degree of liver fibrosis. The concentration of serum HA rises according to progression of liver fibrosis and levels are highest in patients with liver cirrhosis. (author)

  15. 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

  16. 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...

  17. 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)...

  18. fertilizada con diferentes abonos

    Directory of Open Access Journals (Sweden)

    Jorge Alberto Elizondo-Salazar

    2007-01-01

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

  19. 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.

  20. Estudio de los cambios, durante el proceso de restauración, en la materia orgánica, la biomasa microbiana y la actividad biológica de suelos degradados enmendados con lodos de EDAR sometidos a diferentes pos-tratamientos

    OpenAIRE

    Marando, Graciela

    2013-01-01

    En España, la legislación minera obliga, desde el año 1982, a que todas las actividades mineras realicen trabajos de restauración con el fin de devolver al entorno aquellos terrenos afectados por las actividades extractivas. En la presente tesis doctoral se ha comprobado la aplicabilidad de lodos de depuradora que han sido sometidos a secado térmico o a compostaje, como fuente de materia orgánica (MO) y nutrientes para sustratos minerales de restauración (residuos de extracción y de triturac...

  1. 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.

  2. 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.

  3. Contemos con la publicidad

    OpenAIRE

    Muñoz Santonja, José

    1995-01-01

    En este artículo se presentan algunas propuestas para utilizar la publicidad en clases de Matemáticas, como apoyo didáctico a algunos temas que hay que analizar en el currículum de esta materia a lo largo del curso. Se plantean, al mismo tiempo, una serie de actividades para realizar con la publicidad en el ámbito de la prensa escrita.

  4. Los experimentos con ajusticiados.

    OpenAIRE

    Bert, Paul

    2011-01-01

    Los experimentos realizados con ajusticiados se multiplican y son muy pocos los decapitados que escapan a las investigaciones más o menos preparadas y ejecutadas. Es conveniente indicar a los médicos y también al público lo que puede ser interesante intentar, lo que es inútil y sobretodo lo que no se puede permitir.

  5. 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. 

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

    OpenAIRE

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

    2010-01-01

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

  7. 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.

  8. European Cystic Fibrosis Society Standards of Care

    DEFF Research Database (Denmark)

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

    2014-01-01

    therapies, approaches to care and indeed data recording. The quality of care for individuals with CF has become a focus at several levels: patient, centre, regional, national and international. This paper reviews the quality management and improvement issues at each of these levels with particular reference......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...... to indicators of health, the role of CF Centres, regional networks, national health policy, and international data registration and comparisons....

  9. 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.

  10. 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

  11. 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 ...

  12. 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...

  13. 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...

  14. 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...

  15. 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...

  16. 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...

  17. 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...

  18. 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 ...

  19. 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.)

  20. 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...

  1. Towards Systems Biology of Human Pulmonary Fibrosis

    OpenAIRE

    Studer, Sean M.; Kaminski, Naftali

    2007-01-01

    The integrated effect of multiple pathways, molecules, genetic polymorphisms, environmental stimuli, and possible infection determines the lung phenotype in idiopathic pulmonary fibrosis (IPF), a chronic progressive and often lethal lung disease. Systems biology approaches aim to provide a systemwide view of biological process using computational tools and high-throughput technologies. Although much of the analysis of genome-level transcriptional high-resolution profiles of IPF was reductioni...

  2. 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

  3. 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...

  4. Pseudocyst formation in retroperitoneal fibrosis relapse

    OpenAIRE

    Jansen, I.; Hendriksz, T.R.; Bommel, E. F. H.

    2010-01-01

    We describe a 45-year-old male patient with recurrent retroperitoneal fibrosis (RPF), in whom a pseudocyst in the peri-aortic fibrotic mantle was diagnosed. Without any intervention other than oral treatment with tamoxifen, the pseudocyst showed significant regression. Although rare, pseudocyst formation may sometimes appear in RPF and may mimic other benign and malignant conditions. This is the first paper to describe pseudocyst formation in an RPF relapse.

  5. 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

  6. Reversible airway obstruction in cystic fibrosis.

    OpenAIRE

    Ormerod, L P; Thomson, R A; Anderson, C. M.; Stableforth, D. E.

    1980-01-01

    Fourteen (29%) of 48 children with cystic fibrosis had a greater than 15% improvement in forced expiratory volume in one second, or in forced vital capacity after inhalation of salbutamol. All these children were atopic (one or more positive prick tests) and had a significantly higher mean serum IgE than either non-atopic subjects or those atopic subjects without airways reversibility (p less than 0.02). Half of those with airways reversibility had or subsequently developed the clinical pictu...

  7. 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

  8. Idiopathic pulmonary fibrosis: a historical review.

    OpenAIRE

    Homolka, J.

    1987-01-01

    Hamman and Rich are generally considered to have been the first to describe idiopathic pulmonary fibrosis (IPF) as a new clinical and pathological entity. However, several earlier reports in the German-language literature described autopsy findings consistent with IPF from a contemporary point of view. The author discusses these and later reports in a review of the history, diagnosis and treatment of the disease.

  9. 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 ...

  10. 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...

  11. 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

  12. [Molecular epidemiology of cystic fibrosis in Tunisia].

    Science.gov (United States)

    Messaoud, T; Bel Haj Fredj, S; Bibi, A; Elion, J; Férec, C; Fattoum, S

    2005-01-01

    Cystic fibrosis is the most frequent autosomal recessive genetic disease in North European population. This pathology seems to not be rare in Tunisia. On another hand, development of molecular biology techniques has largely contributed to implement the study of the different mutations in the CFTR gene where over 1,300 mutations were reported. Herein, we describe the strategy used to detect molecular defects responsible of cystic fibrosis on 390 children (383 families) in Tunisian population. Several techniques were performed for genotype diagnosis: DNA extraction was from peripheral blood. Polymerase chain reaction (PCR) and polyacylamide gel electrophoresis, and reverse dot blot procedures were used to detect known point mutations. Denaturant gradient gel electrophoresis (DGGE) were used in a next step searching for the unknown point mutations that are later identified by automated sequencing on ABIprism 310. This strategy allowed us to detect 17 different mutations located on the different exons of the CFTR gene. The most frequent was the F508del (50.74%) followed by three other mutations (G542X, W1282X and N1303K) known to be common in the Mediterranean area. For mutations (T665S, 2766 del8, F1166C, L1043R) were exclusively found, up to now, in the Tunisian population. Our results permitted to establish cystic fibrosis mutations and their distribution in Tunisia and to implement an appropriate prevention program of these diseases through the genetic council and prenatal diagnosis. PMID:16330381

  13. 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.

  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. 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

  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. Subcutaneous fibrosis after whole neck irradiation

    International Nuclear Information System (INIS)

    Purpose: To identify the risk factors for moderate to severe subcutaneous fibrosis after whole neck irradiation. Methods and Materials: We analyzed 233 cases of patients who had undergone whole neck irradiation with 4-MV X-ray or 8-10-MeV electrons, or both, and had been followed with regard to their skin condition for at least 1 year. The prescribed dose to the whole neck ranged from 19.2 to 72.4 Gy (median 50). The skin-absorbed dose was specified as that at a depth of 4.1 mm (d4.1-mmdepth), and a biologically equivalent dose (BED) of d4.1-mmdepth was also estimated (BED1.8 4.1-mmdepth). Results: Univariate analysis revealed that previous neck dissection, concurrent chemotherapy, corticosteroid administration as a part of chemotherapy, fractionation, and BED1.8 4.1-mmdepth were significant prognostic variables. Multivariate analysis showed that BED1.8 4.1-mmdepth and previous neck dissection were the only prognostic variables for moderate to severe subcutaneous fibrosis. Conclusion: A high dose to a 4.1-mm depth of the skin and a history of neck dissection were identified as the predominant risk factors for moderate to severe subcutaneous fibrosis after whole neck irradiation. A subcutaneous dose should be considered in radiotherapy treatment planning involving the whole neck, especially in cases in which patients have undergone previous neck dissection

  19. 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

  20. 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

  1. 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.

  2. 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.

  3. 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...

  4. 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 ...

  5. 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...

  6. 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....

  7. 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...

  8. 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...

  9. 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...

  10. 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...

  11. Computed tomography findings in liver fibrosis and cirrhosis

    OpenAIRE

    Huber, Adrian Thomas; Ebner, Lukas Michael; Montani, Matteo; Semmo, Nasser; Roy, Choudhury; Heverhagen, Johannes; Christe, Andreas

    2014-01-01

    Abstract PRINCIPLES: Computed tomography (CT) is inferior to the fibroscan and laboratory testing in the noninvasive diagnosis of liver fibrosis. On the other hand, CT is a frequently used diagnostic tool in modern medicine. The auxiliary finding of clinically occult liver fibrosis in CT scans could result in an earlier diagnosis. The aim of this study was to analyse quantifiable direct signs of liver remodelling in CT scans to depict liver fibrosis in a precirrhotic stage. METHO...

  12. 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.

  13. 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...

  14. 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...

  15. Historia editorial con moraleja

    Directory of Open Access Journals (Sweden)

    José Luis de Diego

    2012-06-01

    Full Text Available Cuando lo conocí, R. ya tendría cuarenta. Un hombre de esos con el pelo como un cepillo, que anda bronceado todo el año, que ostenta ropa de marca y que cuando entra a un boliche estira el cogote, ansioso por conocer a alguien y que otros lo reconozcan a él. Había hecho una buena carrera en Económicas y la influencia decisiva de un profesor le dio la oportunidad de hacer un posgrado en el exterior en algo que llaman Business Process Management, y que ignoro prolijamente qué diablos es, pero c...

  16. Eugenistas, pero con prudencia

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    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

  17. con problemas de aprendizaje

    Directory of Open Access Journals (Sweden)

    Claudia Jaquelina González Trujillo

    2007-01-01

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

  18. 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...

  19. Arquitectura con discurso

    OpenAIRE

    Schaposnik, Viviana

    2001-01-01

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

  20. 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...

  1. Con-tacto

    OpenAIRE

    Arietti, María Luz; Baeza, María Elena; Enriori, Adriana Amalia

    2009-01-01

    Proyecto de Educación Sexual con modalidad de Taller realizado en todos los años de la Escuela Normal “R.J.Cárcano” de Monte Caseros Corrientes, durante el ciclo lectivo 2008. Áreas: Ciencias Naturales, Educación Física, Artística y Matemática. Se realizó el diagnóstico a partir de una encuesta anónima e individual, para trabajar preconceptos sobre Educación sexual que nos permite indagar las ideas previas y representaciones que los alumnos poseen sobre pubertad, desarrollo, caracteres sec...

  2. Pulsaciones con ondas sonoras

    OpenAIRE

    Beléndez Vázquez, Augusto; Álvarez López, Mariela Lázara; Beléndez Vázquez, Tarsicio; Bernabeu Pastor, José Guillermo; Bleda Pérez, Sergio; Calzado Estepa, Eva María; Campo Bagatín, Adriano; Dale Valdivia, Roberto; Durá Domenech, Antonio; Fernández Varó, Elena; Gallego Rico, Sergi; Hernández Prados, Antonio; Marco Tobarra, Amparo; Márquez Ruiz, Andrés; Martín García, Agapito

    2010-01-01

    El objetivo de esta práctica es la demostración del efecto de interferencia que se produce al superponerse varias ondas sonoras. Para ello se emplearán dos diapasones con sus cajas de resonancia, un martillo, un micrófono y un ordenador. Los diapasones nos servirán para crear dos señales sonoras de dos únicas frecuencias. El martillo se empleará para golpear los diapasones y así hacerlos vibrar. El micrófono lo emplearemos para captar el sonido generado por los diapasones e introducirlo en el...

  3. 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

  4. 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

  5. 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

  6. Dynamics of allograft fibrosis in pediatric liver transplantation.

    Science.gov (United States)

    Venturi, C; Sempoux, C; Quinones, J A; Bourdeaux, C; Hoyos, S P; Sokal, E; Reding, R

    2014-07-01

    Progressive liver allograft fibrosis (LAF) is well known to occur long term, as shown by its high prevalence in late posttransplant liver biopsies (LBs). To evaluate the influence of clinical variables and immunosuppression on LAF progression, LAF dynamic was assessed in 54 pediatric liver transplantation (LT) recipients at 6 months, 3 and 7 years post-LT, reviewing clinical, biochemical data and protocol LBs using METAVIR and the liver allograft fibrosis score, previously designed and validated specifically for LAF assessment. Scoring evaluations were correlated with fibrosis quantification by morphometric analysis. Progressive LAF was found in 74% of long-term patients, 70% of whom had unaltered liver enzymes. Deceased grafts showed more fibrosis than living-related grafts (p = 0.0001). Portal fibrosis was observed in correlation with prolonged ischemia time, deceased grafts and lymphoproliferative disease (p = 0.001, 0.006 and 0.012, respectively). Sinusoidal fibrosis was correlated with biliary complications (p = 0.01). Centrilobular fibrosis was associated with vascular complications (p = 0.044), positive autoantibodies (p = 0.017) and high gamma-globulins levels (p = 0.028). Steroid therapy was not associated with reduced fibrosis (p = 0.83). LAF could be viewed as a dynamic process with mostly progression along the time. Peri- and post-LT-associated factors may condition fibrosis development in a specific area of the liver parenchyma. PMID:24934832

  7. 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

  8. 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.

  9. 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.

  10. ¿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.

  11. 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...

  12. 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...

  13. Entrevista con Geoffrey Lloyd.

    Directory of Open Access Journals (Sweden)

    Fernando Colina Pérez

    2008-01-01

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

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

    Directory of Open Access Journals (Sweden)

    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.

  15. 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.

  16. 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

  17. 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.

  18. 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.

  19. 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.

  20. 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) and...

  1. 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) and...

  2. 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,...

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

    Directory of Open Access Journals (Sweden)

    M. Romero

    2008-10-01

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

  4. 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.

  5. 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

  6. Autoimmune Pancreatitis Associated with Retroperitoneal Fibrosis

    Directory of Open Access Journals (Sweden)

    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.

  7. 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

  8. Eureka! Ets a target for fibrosis!

    Science.gov (United States)

    Leask, Andrew

    2011-12-01

    The oncogenic Ets family of transcription factors is now recognized to play a key role in fibroblasts as it controls the expression of a variety of pro-fibrotic genes, including the induction of CCN2 by transforming growth factor β. A recent report (Baran et al., Am J Respir Cell Mol Biol. 2011 May 11) shows that mice containing a version of ets2 that is incapable of being phosphorylated are resistant to bleomycin-induced lung fibrosis. This latter paper is the subject of this commentary. PMID:21748431

  9. 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 < .001). CONCLUSIONS......BACKGROUND: Nephrogenic systemic fibrosis (NSF) is a serious disease that occurs in patients with severe renal disease and is believed to be caused by gadolinium-containing contrast agents. A detailed description of the late skin manifestations of NSF is important to help dermatologists and...

  10. 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

  11. 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.

  12. 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

  13. 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

  14. 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...

  15. 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

  16. 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.

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

    Directory of Open Access Journals (Sweden)

    Lili eGao

    2015-10-01

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

  18. 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.

  19. 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....

  20. 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... we published in the Federal Register on February 4, 2013 (78 FR 7968). We use Listings 3.04 and 103.04 to evaluate claims involving cystic fibrosis in adults and children under titles II and XVI of...

  1. 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

  2. Fibrosis Assessment in Nonalcoholic Fatty Liver Disease (NAFLD) in 2016.

    Science.gov (United States)

    Kaswala, Dharmesh H; Lai, Michelle; Afdhal, Nezam H

    2016-05-01

    Nonalcoholic fatty liver disease (NAFLD) is a spectrum of liver pathologies characterized by hepatic steatosis with a history of little to no alcohol consumption or secondary causes of hepatic steatosis. The prevalence of NAFLD is 20-25 % of the general population in the Western countries and is associated with metabolic risk factors such as obesity, diabetes mellitus, and dyslipidemia. The spectrum of disease ranges from simple steatosis to nonalcoholic steatohepatitis, fibrosis, and cirrhosis. Advanced fibrosis is the most significant predictor of mortality in NAFLD. It is crucial to assess for the presence and degree of hepatic fibrosis in order to make therapeutic decisions and predict clinical outcomes. Liver biopsy, the current gold standard to assess the liver fibrosis, has a number of drawbacks such as invasiveness, sampling error, cost, and inter-/intra-observer variability. There are currently available a number of noninvasive tests as an alternative to liver biopsy for fibrosis staging. These noninvasive fibrosis tests are increasingly used to rule out advanced fibrosis and help guide disease management. While these noninvasive tests perform relatively well for ruling out advanced fibrosis, they also have limitations. Understanding the strengths and limitations of liver biopsy and the noninvasive tests is necessary for deciding when to use the appropriate tests in the evaluation of patients with NAFLD. PMID:27017224

  3. 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...

  4. 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.

  5. 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.

  6. A Case of Autoimmune Pancreatitis Associated with Retroperitoneal Fibrosis

    Directory of Open Access Journals (Sweden)

    Koushiro Ohtsubo

    2007-05-01

    Full Text Available Context Autoimmune pancreatitis is characterized by diffuse enlargement of the pancreas, diffuse irregular narrowing of the main pancreatic duct, severe lymphoplasmacytic infiltration and fibrosis of the pancreas. Retroperitoneal fibrosis may occasionally be associated with autoimmune pancreatitis. Case report We report a 77-year-old man with autoimmune pancreatitis associated with retroperitoneal fibrosis. Abdominal ultrasonography and computed tomography demonstrated diffuse enlargement of the pancreas and a capsule-like rim. Furthermore, a retroperitoneal mass was recognized anterior to the abdominal aorta. Antinuclear antibody, IgG and IgG4 values were elevated. Therefore, this patient was diagnosed as having autoimmune pancreatitis associated with retroperitoneal fibrosis. We performed steroid therapy using prednisolone. After 4 weeks, both IgG and IgG4 values decreased and both the swelling of the pancreas and also the retroperitoneal mass were obviously diminished. Conclusion This is a rare case of autoimmune pancreatitis associated with retroperitoneal fibrosis.

  7. 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...

  8. 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.

  9. Myocardial fibrosis in patients with myotonic dystrophy type 1

    DEFF Research Database (Denmark)

    Petri, Helle; Ahtarovski, Kiril Aleksov; Vejlstrup, Niels; Vissing, John; Witting, Nanna; Køber, Lars; Bundgaard, Henning

    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...

  10. 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

  11. 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

  12. 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.

  13. Qulaity of Life in Cystic Fibrosis Children

    Directory of Open Access Journals (Sweden)

    Hamid-Reza Kianifar

    2013-04-01

    Full Text Available 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 control group.Findings: Based on children's reports, significant differences between the CF patients and control group were noted for emotional, physical, social, school performance, and total scores (P<0.05. Based on parents’ reports, quality of life score in CF patients from the physical point of view as well as social and total scores were decreased (P<0.05.Conclusion: QoL in CF patients seems to be low, and therapy programs should take into account the suggestive perceived quality of life.

  14. 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.

  15. 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

  16. 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.

  17. 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.

  18. 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.

  19. 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 

  20. Medical Therapy in Idiopathic Pulmonary Fibrosis.

    Science.gov (United States)

    Antoniou, Katerina M; Wuyts, Wim; Wijsenbeek, Marlies; Wells, Athol U

    2016-06-01

    Medical therapy for idiopathic fibrosis remains controversial. Idiopathic pulmonary fibrosis (IPF) was uniformly a disease that progressed inexorably, typically leading to death within 3 to 5 years from onset of symptoms. Until recently, lung transplantation was the only effective transplant option. Within the past decade, several placebo-controlled trials failed to show benefit in patients with IPF. However, within the past 2 years, two novel antifibrotic agents (pirfenidone and nintedanib) were approved by the Food and Drug Administration (FDA) in the United States and European Medicines Agency (EMA) based upon pivotal studies that showed benefit (specifically slowing of the rate of disease progression) with both agents. Short-term outcomes (12 months) showed less deterioration of physiological parameters (e.g., change in forced vital capacity), although survival benefit has not convincingly been established with either agent. Nonetheless, these agents bring a glimmer of hope to patients with this deadly disease. The appropriate indications for initiating therapy, best candidates for therapy, and possible role for combination therapy remain controversial. Additional studies using agents that attenuate or abrogate profibrotic cytokines and chemokines may provide even further improvement in the future. PMID:27231861

  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. 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.)

  3. 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.

  4. Imaging findings in congenital hepatic fibrosis

    International Nuclear Information System (INIS)

    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

  5. Cystic fibrosis chronic rhinosinusitis: A comprehensive review

    Science.gov (United States)

    Chaaban, Mohamad R.; Kejner, Alexandra; Rowe, Steven M.

    2013-01-01

    Background: Advances in the care of patients with cystic fibrosis (CF) have improved pulmonary outcomes and survival. In addition, rapid developments regarding the underlying genetic and molecular basis of the disease have led to numerous novel targets for treatment. However, clinical and basic scientific research focusing on therapeutic strategies for CF-associated chronic rhinosinusitis (CRS) lags behind the evidence-based approaches currently used for pulmonary disease. Methods: This review evaluates the available literature and provides an update concerning the pathophysiology, current treatment approaches, and future pharmaceutical tactics in the management of CRS in patients with CF. Results: Optimal medical and surgical strategies for CF CRS are lacking because of a dearth of well-performed clinical trials. Medical and surgical interventions are supported primarily by level 2 or 3 evidence and are aimed at improving clearance of mucus, infection, and inflammation. A number of novel therapeutics that target the basic defect in the cystic fibrosis transmembrane conductance regulator channel are currently under investigation. Ivacaftor, a corrector of the G551D mutation, was recently approved by the Food and Drug Administration. However, sinonasal outcomes using this and other novel drugs are pending. Conclusion: CRS is a lifelong disease in CF patients that can lead to substantial morbidity and decreased quality of life. A multidisciplinary approach will be necessary to develop consistent and evidence-based treatment paradigms. PMID:24119602

  6. 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

  7. autorregulado con estudiantes universitarios

    Directory of Open Access Journals (Sweden)

    Jairo Andrés Montes

    2005-01-01

    Full Text Available El propósito del presente estudio es describir la forma en la que se presentan los procesos de aprendizaje autorregulado con un grupo de estudiantes (22 estudiantes de tercer semestre de Psicología de la PUJ, Cali, en el evento de preparación para la presentación un examen. Asimismo se describen las correlaciones que ocurren entre las distintas fases de dicho proceso de autorregulación del aprendizaje. Para conseguir los objetivos propuestos se ha hecho uso de una observación de desempeño en tiempo real, es decir, de la observación durante una sesión de preparación de examen de los estudiantes, en la cual se emplearon protocolos verbales para dar cuenta de lo que «pasaba por su mente» mientras estudiaban. Una entrevista semi-estructurada y una prueba objetiva. Los resultados fueron analizados a la luz del modelo mixto de procesamiento de información y constructivismo abordado por Winne(1998. Como resultado se encontró una relación significativa entre los niveles de desempeño en el proceso de ARR y el resultado del examen. Igualmente se encontraron bajos niveles de regulación en una parte importante de la muestra y un desfase significativo entre conocimiento declarativo de ARR y desempeño en el mismo

  8. Comparativa de métodos de descontaminación de suelos afectados por hidrocarburos. Aplicación a la obra del AVE de Málaga

    OpenAIRE

    Herrero Peña, María José

    2016-01-01

    El objetivo de esta tesis, va a ser la investigación y desarrollo de tratamientos de biorremediación para conseguir la recuperación de los terrenos contaminados situados en el tramo del tren de alta velocidad entre Córdoba y Málaga, reduciendo de esta forma los residuos enviados a vertederos. Para ello, se va a investigar y a desarrollar una tecnología innovadora de descontaminación in situ de suelos con altas concentraciones de hidrocarburos, basándonos en el landfarming y como principal av...

  9. 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.

  10. 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.

  11. 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...

  12. 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

  13. Monocyte Subsets in Schistosomiasis Patients with Periportal Fibrosis

    Directory of Open Access Journals (Sweden)

    Jamille Souza Fernandes

    2014-01-01

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

  14. 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

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

    Directory of Open Access Journals (Sweden)

    B. Madrigal

    2008-08-01

    Full Text Available Comunicamos un caso de mucormicosis intestinal en un hombre de 46 años de edad, diagnosticado de enfermedad de Hodgkin clásica, estadio IV-B. Durante la primera fase de la quimioterapia, sufrió una hemorragia digestiva masiva secundaria a una úlcera yeyunal por zigomicosis tipo mucor, diagnosticada por biopsia endoscópica. El paciente fue tratado con antifúngicos y resección quirúrgica del intestino afectado. En la cirugía, se apreció una doble perforación yeyunal cubierta. El estudio anatomopatológico de la pieza confirmó el diagnóstico previo. Tras un año de seguimiento, el paciente está recuperado y su linfoma de Hodgkin en remisión completa. Tras una extensa revisión de la literatura, según nuestro conocimiento, este es el segundo caso publicado en la literatura de mucormicosis intestinal en un paciente con linfoma de Hodgkin.We report a case of intestinal mucormycosis in a 46-year-old male diagnosed with classical Hodgkin's disease, IV-B stage. During the first phase of chemotherapy he had a massive digestive bleeding event secondary to a jejunal ulcer, and zygomicosis mucor-type was diagnosed by endoscopic biopsy. The patient was treated with antifungal drugs and surgical resection of the intestine involved. At surgery a double covered perforation of the jejunum was seen. Pathological examination confirmed the previous diagnosis. After one year of follow-up the patient is doing well, and his lymphoma is on remission. To our best knowledge this is the second case of intestinal mucormycosis in a patient with Hodgkin's lymphoma reported in the medical literature.

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

    Directory of Open Access Journals (Sweden)

    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

  17. 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.

  18. Blood Gene Expression Profiling of Breast Cancer Survivors Experiencing Fibrosis

    International Nuclear Information System (INIS)

    Purpose: To extend knowledge on the mechanisms and pathways involved in maintenance of radiation-induced fibrosis (RIF) by performing gene expression profiling of whole blood from breast cancer (BC) survivors with and without fibrosis 3-7 years after end of radiotherapy treatment. Methods and Materials: Gene expression profiles from blood were obtained for 254 BC survivors derived from a cohort of survivors, treated with adjuvant radiotherapy for breast cancer 3-7 years earlier. Analyses of transcriptional differences in blood gene expression between BC survivors with fibrosis (n = 31) and BC survivors without fibrosis (n = 223) were performed using R version 2.8.0 and tools from the Bioconductor project. Gene sets extracted through a literature search on fibrosis and breast cancer were subsequently used in gene set enrichment analysis. Results: Substantial differences in blood gene expression between BC survivors with and without fibrosis were observed, and 87 differentially expressed genes were identified through linear analysis. Transforming growth factor-β1 signaling was identified as the most significant gene set, showing a down-regulation of most of the core genes, together with up-regulation of a transcriptional activator of the inhibitor of fibrinolysis, Plasminogen activator inhibitor 1 in the BC survivors with fibrosis. Conclusion: Transforming growth factor-β1 signaling was found down-regulated during the maintenance phase of fibrosis as opposed to the up-regulation reported during the early, initiating phase of fibrosis. Hence, once the fibrotic tissue has developed, the maintenance phase might rather involve a deregulation of fibrinolysis and altered degradation of extracellular matrix components.

  19. Accuracy of FibroScan for diagnosing liver fibrosis

    Directory of Open Access Journals (Sweden)

    Jian ZHANG

    2011-11-01

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

  20. 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.

  1. 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...

  2. 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...

  3. 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.)

  4. 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.)

  5. 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 ...

  6. 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

  7. 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)

  8. 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.

  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 in...... children with CF. A measure of social desirability was included as well as body mass index (BMI) and percentage of predicted forced expiratory volume in one second (FEV(1)) as measures of health status. RESULTS: The children with CF did not differ from the norm group concerning depression, disruptive...... behaviour and self-concept. Young children with CF (7-10 years) and boys with CF scored significantly higher on anxiety. Girls with CF scored significantly lower on anger than controls. BMI was not associated with any of the BYI subscales. In patients aged 11-14 years, there was a significant correlation...

  10. 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

  11. 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

  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. 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

  15. 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

  16. 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.

  17. 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.

  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. 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.

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

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    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