Properdin Deficiency

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Nobutaka Wakamiya - One of the best experts on this subject based on the ideXlab platform.

  • the collectins cl l1 cl k1 and cl p1 and their roles in complement and innate immunity
    Immunobiology, 2016
    Co-Authors: Soren Hansen, Katsuki Ohtani, Nobutaka Wakamiya
    Abstract:

    Both the complement system and collectins play important roles in our innate immune system. The collectins, which are characterized by their inclusion of a collagen-like region and a calcium-dependent carbohydrate recognition domain, are pattern recognition molecules and include the well characterized proteins mannan-binding lectin (MBL) and the surfactant proteins SP-A/-D. Collectin liver 1 (CL-L1), collectin kidney 1 (CL-K1) and collectin placenta 1 (CL-P1) are the most recently discovered collectins. Although their function is still under investigation, accumulating information suggests that CL-L1, CL-K1 and CL-P1 play important roles in host defense by recognizing a variety of microorganisms and interacting with effector proteins, including complement components. The recent establishment of the existence of CL-K1 in the circulation in form of heteromeric complexes with CL-L1 (known as CL-LK) and its activation of the lectin pathway via MASPs, drew new attention in the complement biology, which was further strengthened by the observed interactions between CL-P1 and CRP-C1q-factor H or Properdin. Deficiency of either CL-K1 or MASP-3 has been demonstrated in 3MC syndrome patients with developmental abnormalities, showing that lectin pathway components, regulation and/or activation are essential during the embryonic development; another feature that they most likely share CL-P1. Herein, we discuss the recent characteristics and roles of the collectins CL-L1, CL-K1 and CL-P1 in the complement system, in innate immunity and their possible association with disease development and pathogenesis.

Giménez-júlvez T. - One of the best experts on this subject based on the ideXlab platform.

  • Assessment of health information available online regarding meningococcal B vaccine recommendations
    2018
    Co-Authors: Giménez-júlvez T.
    Abstract:

    Fundamentos: La calidad de la información sanitaria en internet preocupa a gobiernos y usuarios. Nuestro objetivo fue determinar en qué medida la información disponible en la red sobre las recomendaciones de vacunación frente al meningococo B se adhiere a lo indicado por el Ministerio de Sanidad español. Métodos: Estudio transversal realizado en abril de 2017. Se evaluó la adhesión de la información sobre recomendaciones de recibir la vacuna. La información se obtuvo a través de Google utilizando veinte palabras clave. Se utilizó la prueba chi-cuadrado para estudiar la asociación entre obtener información adherida y el tipo de origen de la misma. Resultados: Se analizaron 186 enlaces web. Se detectaron recomendaciones adheridas entre el 52, 2% (97/186) de los enlaces para la indicación en personas con deficiencia de Properdina/factores terminales del complemento, y el 79, 6% para las situaciones de brotes. Vacunar a niños a partir de los dos meses de edad fue una recomendación no elaborada por el Ministerio que se detectó en el 72, 6% de los enlaces. Para cada una de las recomendaciones del Ministerio, los organismos oficiales de salud pública siempre proporcionaron información adherida. Medios de comunicación digitales aportaron con una frecuencia significativamente mayor, que las Sociedades Científicas, información adherida sobre vacunar a personas con deficiencia de Properdina/factores terminales del complemento (OR: 2, 72; IC95%: 1, 18-6, 28) y asplenia (OR: 3, 83; IC95%: 1, 66-8, 86). Conclusiones: Se evidencia una dificultad para obtener información adherida a lo indicado por la ponencia de vacunación del Ministerio de Sanidad, Servicios Sociales e Igualdad. Se debe promocionar en los usuarios la consulta de páginas web de organismos oficiales de salud pública cuando busquen información sobre esta vacuna. OBJECTIVE: The quality of health information online is a concern to governments and users. Our objective was to determine the extent to which the information available online regarding meningococcal B vaccine recommendations adhere to the guidelines of the Spanish Ministry of Health. METHODS: Cross-sectional study carried out in April 2017. The study assessed adherence of information regarding vaccine recommendations to official guidelines. The information was collected via Google with 20 keywords. The Chi-squared test was used to analyze the association between the adhered information and its origin. RESULTS: In total, 186 web links were analyzed. Adhered recommendations were found in a range of links, from 52.2% (97/186) with an indication for people with Properdin Deficiency/terminal component pathway Deficiency, to 79.6% for outbreak situations. Vaccinating children from two months of age was a recommendation not issued by the Ministry that was found in 72.6% of the links. For each of the Ministry recommendations, official public health institutions always provide information adhering to them. Digital media provided information about vaccination adhering to official guidelines with a significantly higher frequency than scientific societies in cases of people with Properdin Deficiency/terminal component pathway Deficiency (OR: 2.72; 95%CI: 1.18-6.28) and asplenia (OR: 3.83; 95%CI: 1.66-8.86). CONCLUSIONS: We have observed a difficulty to obtain adhered information. Users must be encouraged to access websites of official public health institutions when looking for information about this vaccine.OBJETIVO: La calidad de la información sanitaria en internet preocupa a gobiernos y usuarios

  • Evaluación de la información sanitaria disponible en Internet sobre las recomendaciones de vacunación frente al Meningococo B
    2018
    Co-Authors: Giménez-júlvez T.
    Abstract:

    Fundamentos. La calidad de la información sanitaria en internet preocupa a gobiernos y usuarios. Nuestro objetivo fue determinar en qué medida la información disponible en la red sobre las recomendaciones de vacunación frente al meningococo B se adhiere a lo indicado por el Ministerio de Sanidad español. Métodos. Estudio transversal realizado en abril de 2017. Se evaluó la adhesión de la información sobre recomendaciones de recibir la vacuna. La información se obtuvo a través de Google utilizando veinte palabras clave. Se utilizó la prueba chi-cuadrado para estudiar la asociación entre obtener información adherida y el tipo de origen de la misma. Resultados. Se analizaron 186 enlaces web. Se detectaron recomendaciones adheridas entre el 52, 2% (97/186) de los enlaces para la indicación en personas con deficiencia de Properdina/factores terminales del complemento, y el 79, 6% para las situaciones de brotes. Vacunar a niños a partir de los dos meses de edad fue una recomendación no elaborada por el Ministerio que se detectó en el 72, 6% de los enlaces. Para cada una de las recomendaciones del Ministerio, los organismos oficiales de salud pública siempre proporcionaron información adherida. Medios de comunicación digitales aportaron con una frecuencia significativamente mayor, que las Sociedades Científicas, información adherida sobre vacunar a personas con deficiencia de Properdina/factores terminales del complemento (OR: 2, 72; IC95%: 1, 18-6, 28) y asplenia (OR: 3, 83; IC95%: 1, 66-8, 86). Conclusiones. Se evidencia una dificultad para obtener información adherida a lo indicado por la ponencia de vacunación del Ministerio de Sanidad, Servicios Sociales e Igualdad. Se debe promocionar en los usuarios la consulta de páginas web de organismos oficiales de salud pública cuando busquen información sobre esta vacuna. Background. The quality of health information online is a concern to governments and users. Our objective was to determine the extent to which the information available online regarding meningococcal B vaccine recommendations adhere to the guidelines of the Spanish Ministry of Health. Methods. Cross-sectional study carried out in April 2017. The study assessed adherence of information regarding vaccine recommendations to official guidelines. The information was collected via Google with 20 keywords. The Chi-squared test was used to analyze the association between the adhered information and its origin. Results. In total, 186 web links were analyzed. Adhered recommendations were found in a range of links, from 52.2% (97/186) with an indication for people with Properdin Deficiency/termial component pathway Deficiency, to 79.6% for outbreak situations. Vaccinating children from two months of age was a recommendation not issued by the Ministry that was found in 72.6% of the links. For each of the Ministry recommendations, official public health institutions always provide information adhering to them. Digital media provided information about vaccination adhering to official guidelines with a significantly higher frequency than scientific societies in cases of people with . Properdin Deficiency/terminal component pathway Deficiency (OR: 2.72; 95%CI: 1.186.28) and asplenia (OR: 3.83; 95%CI: 1.66-8.86). Conclusions. We have observed a difficulty to obtain adhered information. Users must be encouraged to access websites of official public health institutions when looking for information about this vaccine

Soren Hansen - One of the best experts on this subject based on the ideXlab platform.

  • the collectins cl l1 cl k1 and cl p1 and their roles in complement and innate immunity
    Immunobiology, 2016
    Co-Authors: Soren Hansen, Katsuki Ohtani, Nobutaka Wakamiya
    Abstract:

    Both the complement system and collectins play important roles in our innate immune system. The collectins, which are characterized by their inclusion of a collagen-like region and a calcium-dependent carbohydrate recognition domain, are pattern recognition molecules and include the well characterized proteins mannan-binding lectin (MBL) and the surfactant proteins SP-A/-D. Collectin liver 1 (CL-L1), collectin kidney 1 (CL-K1) and collectin placenta 1 (CL-P1) are the most recently discovered collectins. Although their function is still under investigation, accumulating information suggests that CL-L1, CL-K1 and CL-P1 play important roles in host defense by recognizing a variety of microorganisms and interacting with effector proteins, including complement components. The recent establishment of the existence of CL-K1 in the circulation in form of heteromeric complexes with CL-L1 (known as CL-LK) and its activation of the lectin pathway via MASPs, drew new attention in the complement biology, which was further strengthened by the observed interactions between CL-P1 and CRP-C1q-factor H or Properdin. Deficiency of either CL-K1 or MASP-3 has been demonstrated in 3MC syndrome patients with developmental abnormalities, showing that lectin pathway components, regulation and/or activation are essential during the embryonic development; another feature that they most likely share CL-P1. Herein, we discuss the recent characteristics and roles of the collectins CL-L1, CL-K1 and CL-P1 in the complement system, in innate immunity and their possible association with disease development and pathogenesis.

Katsuki Ohtani - One of the best experts on this subject based on the ideXlab platform.

  • the collectins cl l1 cl k1 and cl p1 and their roles in complement and innate immunity
    Immunobiology, 2016
    Co-Authors: Soren Hansen, Katsuki Ohtani, Nobutaka Wakamiya
    Abstract:

    Both the complement system and collectins play important roles in our innate immune system. The collectins, which are characterized by their inclusion of a collagen-like region and a calcium-dependent carbohydrate recognition domain, are pattern recognition molecules and include the well characterized proteins mannan-binding lectin (MBL) and the surfactant proteins SP-A/-D. Collectin liver 1 (CL-L1), collectin kidney 1 (CL-K1) and collectin placenta 1 (CL-P1) are the most recently discovered collectins. Although their function is still under investigation, accumulating information suggests that CL-L1, CL-K1 and CL-P1 play important roles in host defense by recognizing a variety of microorganisms and interacting with effector proteins, including complement components. The recent establishment of the existence of CL-K1 in the circulation in form of heteromeric complexes with CL-L1 (known as CL-LK) and its activation of the lectin pathway via MASPs, drew new attention in the complement biology, which was further strengthened by the observed interactions between CL-P1 and CRP-C1q-factor H or Properdin. Deficiency of either CL-K1 or MASP-3 has been demonstrated in 3MC syndrome patients with developmental abnormalities, showing that lectin pathway components, regulation and/or activation are essential during the embryonic development; another feature that they most likely share CL-P1. Herein, we discuss the recent characteristics and roles of the collectins CL-L1, CL-K1 and CL-P1 in the complement system, in innate immunity and their possible association with disease development and pathogenesis.

Giménez Júlvez, Mª Teresa - One of the best experts on this subject based on the ideXlab platform.

  • Evaluación de la información sanitaria disponible en internet sobre las recomendaciones de vacunación frente al meningococo B
    2018
    Co-Authors: Hernández García Ignacio, Giménez Júlvez, Mª Teresa
    Abstract:

    ABSTRACT Background. The quality of health information online is a concern to governments and users. Our objective was to determine the extent to which the information available online regarding meningococcal B vaccine recommendations adhere to the guidelines of the Spanish Ministry of Health. Methods. Cross-sectional study carried out in April 2017. The study assessed adherence of information regarding vaccine recommendations to official guidelines. The information was collected via Google with 20 keywords. The Chi-squared test was used to analyze the association between the adhered information and its origin. Results. In total, 186 web links were analyzed. Adhered recommendations were found in a range of links, from 52.2% (97/186) with an indication for people with Properdin Deficiency/terminal component pathway Deficiency, to 79.6% for outbreak situations. Vaccinating children from two months of age was a recommendation not issued by the Ministry that was found in 72.6% of the links. For each of the Ministry recommendations, official public health institutions always provide information adhering to them. Digital media provided information about vaccination adhering to official guidelines with a significantly higher frequency than scientific societies in cases of people with Properdin Deficiency/terminal component pathway Deficiency (OR: 2.72; 95%CI: 1.18-6.28) and asplenia (OR: 3.83; 95%CI: 1.66-8.86). Conclusions. We have observed a difficulty to obtain adhered information. Users must be encouraged to access websites of official public health institutions when looking for information about this vaccine.RESUMEN Fundamentos. La calidad de la información sanitaria en internet preocupa a gobiernos y usuarios. Nuestro objetivo fue determinar en qué medida la información disponible en la red sobre las recomendaciones de vacunación frente al meningococo B se adhiere a lo indicado por el Ministerio de Sanidad español. Métodos. Estudio transversal realizado en abril de 2017. Se evaluó la adhesión de la información sobre recomendaciones de recibir la vacuna. La información se obtuvo a través de Google utilizando veinte palabras clave. Se utilizó la prueba chi-cuadrado para estudiar la asociación entre obtener información adherida y el tipo de origen de la misma. Resultados. Se analizaron 186 enlaces web. Se detectaron recomendaciones adheridas entre el 52,2% (97/186) de los enlaces para la indicación en personas con deficiencia de Properdina/factores terminales del complemento, y el 79,6% para las situaciones de brotes. Vacunar a niños a partir de los dos meses de edad fue una recomendación no elaborada por el Ministerio que se detectó en el 72,6% de los enlaces. Para cada una de las recomendaciones del Ministerio, los organismos oficiales de salud pública siempre proporcionaron información adherida. Medios de comunicación digitales aportaron con una frecuencia significativamente mayor, que las Sociedades Científicas, información adherida sobre vacunar a personas con deficiencia de Properdina/factores terminales del complemento (OR: 2,72; IC95%: 1,18-6,28) y asplenia (OR: 3,83; IC95%: 1,66-8,86). Conclusiones. Se evidencia una dificultad para obtener información adherida a lo indicado por la ponencia de vacunación del Ministerio de Sanidad, Servicios Sociales e Igualdad. Se debe promocionar en los usuarios la consulta de páginas web de organismos oficiales de salud pública cuando busquen información sobre esta vacuna