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  1. Article: Valores normales del flujo espiratorio máximo en niños sanos de 6 a 15 años de edad de la ciudad de Puebla.

    Gómez Tello, Héctor / Gutiérrez Brito, Maricruz / Gutiérrez Sánchez, Segalix

    Revista alergia Mexico (Tecamachalco, Puebla, Mexico : 1993)

    2006  Volume 53, Issue 5, Page(s) 167–173

    Abstract: Background: The peak expiratory flow (PEF) is a clinical tool for the evaluation of flow pulmonary, integrated in many studies and guides for the management of asthma, so it is important to have references about its normal values in healthy children ... ...

    Title translation Normal values of peak expiratory flow in 6-15 year-old healthy children of Puebla's city.
    Abstract Background: The peak expiratory flow (PEF) is a clinical tool for the evaluation of flow pulmonary, integrated in many studies and guides for the management of asthma, so it is important to have references about its normal values in healthy children Puebla's city.
    Material and methods: We did an observation, descriptive, transversal and prospective study, where we evaluated to 1154 children from Puebla's city and peak expiratory flow measurements were obtained.
    Results: The mean values by stature and sex for PEF were dispersed in both sex, the average for males were of 286 L/min, females 251 L/min, finding the major average value in males, with Z = 0.6946 level to confidence of 80%, so the PEF was major in males. We compared variations; and the value of the proof of Fisher was 1.12 with a level of confidence of 90%. The lineal regression straight was: males B1 = 5.3 and B0 = -481, females B1 = 4.82, B0 = -436. The correlation between height and peak expiratory flow was: males R2 = 0.8283, females R2 = 0.7652, for the standard mistake it was calculated with the sum of square of mistakes and the number of observations corresponding for males 22.6% and females 20.3%.
    Conclusions: The values of healthy children's PEF in Puebla's city are different to other experiences. By first time we have reference values of PEF in healthy children of Puebla's city by sex and height.
    MeSH term(s) Adolescent ; Body Height ; Child ; Cross-Sectional Studies ; Female ; Humans ; Male ; Mexico ; Peak Expiratory Flow Rate ; Prospective Studies ; Reference Values ; Urban Population
    Language Spanish
    Publishing date 2006-09
    Publishing country Mexico
    Document type English Abstract ; Journal Article
    ZDB-ID 639125-4
    ISSN 0002-5151
    ISSN 0002-5151
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Human OTULIN haploinsufficiency impairs cell-intrinsic immunity to staphylococcal α-toxin.

    Spaan, András N / Neehus, Anna-Lena / Laplantine, Emmanuel / Staels, Frederik / Ogishi, Masato / Seeleuthner, Yoann / Rapaport, Franck / Lacey, Keenan A / Van Nieuwenhove, Erika / Chrabieh, Maya / Hum, David / Migaud, Mélanie / Izmiryan, Araksya / Lorenzo, Lazaro / Kochetkov, Tatiana / Heesterbeek, Dani A C / Bardoel, Bart W / DuMont, Ashley L / Dobbs, Kerry /
    Chardonnet, Solenne / Heissel, Søren / Baslan, Timour / Zhang, Peng / Yang, Rui / Bogunovic, Dusan / Wunderink, Herman F / Haas, Pieter-Jan A / Molina, Henrik / Van Buggenhout, Griet / Lyonnet, Stanislas / Notarangelo, Luigi D / Seppänen, Mikko R J / Weil, Robert / Seminario, Gisela / Gomez-Tello, Héctor / Wouters, Carine / Mesdaghi, Mehrnaz / Shahrooei, Mohammad / Bossuyt, Xavier / Sag, Erdal / Topaloglu, Rezan / Ozen, Seza / Leavis, Helen L / van Eijk, Maarten M J / Bezrodnik, Liliana / Blancas Galicia, Lizbeth / Hovnanian, Alain / Nassif, Aude / Bader-Meunier, Brigitte / Neven, Bénédicte / Meyts, Isabelle / Schrijvers, Rik / Puel, Anne / Bustamante, Jacinta / Aksentijevich, Ivona / Kastner, Daniel L / Torres, Victor J / Humblet-Baron, Stéphanie / Liston, Adrian / Abel, Laurent / Boisson, Bertrand / Casanova, Jean-Laurent

    Science (New York, N.Y.)

    2022  Volume 376, Issue 6599, Page(s) eabm6380

    Abstract: The molecular basis of interindividual clinical variability upon infection ... ...

    Abstract The molecular basis of interindividual clinical variability upon infection with
    MeSH term(s) Bacterial Toxins/immunology ; Cri-du-Chat Syndrome/genetics ; Cri-du-Chat Syndrome/immunology ; Endopeptidases/genetics ; Haploinsufficiency/genetics ; Haploinsufficiency/immunology ; Hemolysin Proteins/immunology ; Host-Pathogen Interactions/genetics ; Host-Pathogen Interactions/immunology ; Humans ; Immunity, Cellular/genetics ; Necrosis ; Staphylococcal Infections/genetics ; Staphylococcal Infections/immunology ; Staphylococcal Infections/pathology ; Staphylococcus aureus
    Chemical Substances Bacterial Toxins ; Hemolysin Proteins ; staphylococcal alpha-toxin ; Endopeptidases (EC 3.4.-) ; OTULIN protein, human (EC 3.4.-)
    Language English
    Publishing date 2022-06-17
    Publishing country United States
    Document type Journal Article
    ZDB-ID 128410-1
    ISSN 1095-9203 ; 0036-8075
    ISSN (online) 1095-9203
    ISSN 0036-8075
    DOI 10.1126/science.abm6380
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Latin American consensus on the supportive management of patients with severe combined immunodeficiency.

    Bustamante Ogando, Juan Carlos / Partida Gaytán, Armando / Aldave Becerra, Juan Carlos / Álvarez Cardona, Aristóteles / Bezrodnik, Liliana / Borzutzky, Arturo / Blancas Galicia, Lizbeth / Cabanillas, Diana / Condino-Neto, Antonio / De Colsa Ranero, Agustín / Espinosa Padilla, Sara / Fernandes, Juliana Folloni / García Campos, Jorge Alberto / Gómez Tello, Héctor / González Serrano, María Edith / Gutiérrez Hernández, Alonso / Hernández Bautista, Víctor Manuel / Ivankovich Escoto, Gabriele / King, Alejandra /
    Lessa Mazzucchelli, Juliana / Llamas Guillén, Beatriz Adriana / Lugo Reyes, Saul Oswaldo / Moreno Espinosa, Sarbelio / Oleastro, Matías / Otero Mendoza, Francisco / Poli Harlowe, María Cecilia / Porras, Oscar / Ramirez Uribe, Nideshda / Regairaz, Lorean / Rivas Larrauri, Francisco / Saracho Weber, Federico José / Grumach, Anete S / Staines Boone, Tamara / Tavares Costa-Carvalho, Beatriz / Yamazaki Nakashimada, Marco Antonio / Espinosa Rosales, Francisco Javier

    The Journal of allergy and clinical immunology

    2019  Volume 144, Issue 4, Page(s) 897–905

    Abstract: Severe combined immunodeficiency (SCID) represents the most lethal form of primary immunodeficiency, with mortality rates of greater than 90% within the first year of life without treatment. Hematopoietic stem cell transplantation and gene therapy are ... ...

    Abstract Severe combined immunodeficiency (SCID) represents the most lethal form of primary immunodeficiency, with mortality rates of greater than 90% within the first year of life without treatment. Hematopoietic stem cell transplantation and gene therapy are the only curative treatments available, and the best-known prognostic factors for success are age at diagnosis, age at hematopoietic stem cell transplantation, and the comorbidities that develop in between. There are no evidence-based guidelines for standardized clinical care for patients with SCID during the time between diagnosis and definitive treatment, and we aim to generate a consensus management strategy on the supportive care of patients with SCID. First, we gathered available information about SCID diagnostic and therapeutic guidelines, then we developed a document including diagnostic and therapeutic interventions, and finally we submitted the interventions for expert consensus through a modified Delphi technique. Interventions are grouped in 10 topic domains, including 123 "agreed" and 38 "nonagreed" statements. This document intends to standardize supportive clinical care of patients with SCID from diagnosis to definitive treatment, reduce disease burden, and ultimately improve prognosis, particularly in countries where newborn screening for SCID is not universally available and delayed diagnosis is the rule. Our work intends to provide a tool not only for immunologists but also for primary care physicians and other specialists involved in the care of patients with SCID.
    MeSH term(s) Consensus ; Humans ; Latin America ; Practice Guidelines as Topic ; Severe Combined Immunodeficiency/diagnosis ; Severe Combined Immunodeficiency/therapy
    Language English
    Publishing date 2019-08-13
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 121011-7
    ISSN 1097-6825 ; 1085-8725 ; 0091-6749
    ISSN (online) 1097-6825 ; 1085-8725
    ISSN 0091-6749
    DOI 10.1016/j.jaci.2019.08.002
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  4. Article ; Online: Genetic, Immunological, and Clinical Features of the First Mexican Cohort of Patients with Chronic Granulomatous Disease.

    Blancas-Galicia, Lizbeth / Santos-Chávez, Eros / Deswarte, Caroline / Mignac, Quentin / Medina-Vera, Isabel / León-Lara, Ximena / Roynard, Manon / Scheffler-Mendoza, Selma C / Rioja-Valencia, Ricardo / Alvirde-Ayala, Alexandra / Lugo Reyes, Saul O / Staines-Boone, Tamara / García-Campos, Jorge / Saucedo-Ramírez, Omar J / Del-Río Navarro, Blanca E / Zamora-Chávez, Antonio / López-Larios, Arturo / García-Pavón-Osorio, Susana / Melgoza-Arcos, Eugenia /
    Canseco-Raymundo, María R / Mogica-Martínez, Dolores / Venancio-Hernández, Marco / Pacheco-Rosas, Daniel / Pedraza-Sánchez, Sigifredo / Guevara-Cruz, Martha / Saracho-Weber, Federico / Gámez-González, Berenise / Wakida-Kuzunoki, Guillermo / Morán-Mendoza, Ana R / Macías-Robles, Ana P / Ramírez-Rivera, Roselia / Vargas-Camaño, Eugenia / Zarate-Hernández, Carmen / Gómez-Tello, Héctor / Ramírez-Sánchez, Emmanuel / Ruíz-Hernández, Fredy / Ramos-López, Domingo / Acuña-Martínez, Héctor / García-Cruz, María L / Román-Jiménez, María G / González-Villarreal, Marina G / Álvarez-Cardona, Aristóteles / Llamas-Guillén, Beatriz A / Cuellar-Rodríguez, Jennifer / Olaya-Vargas, Alberto / Ramírez-Uribe, Nideshda / Boisson-Dupuis, Stéphanie / Casanova, Jean-Laurent / Espinosa-Rosales, Francisco J / Serafín-López, Jeanet / Yamazaki-Nakashimada, Marco / Espinosa-Padilla, Sara / Bustamante, Jacinta

    Journal of clinical immunology

    2020  Volume 40, Issue 3, Page(s) 475–493

    Abstract: Purpose: Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by an inability of phagocytes to produce reactive oxygen species, impairing their killing of various bacteria and fungi. We summarize here the 93 cases of CGD ... ...

    Abstract Purpose: Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by an inability of phagocytes to produce reactive oxygen species, impairing their killing of various bacteria and fungi. We summarize here the 93 cases of CGD diagnosed in Mexico from 2011 to 2019.
    Methods: Thirteen Mexican hospitals participated in this study. We describe the genetic, immunological, and clinical features of the 93 CGD patients from 78 unrelated kindreds.
    Results: Eighty-two of the patients (88%) were male. All patients developed bacterial infections and 30% suffered from some kind of fungal infection. Fifty-four BCG-vaccinated patients (58%) presented infectious complications of BCG vaccine. Tuberculosis occurred in 29%. Granulomas were found in 56% of the patients. Autoimmune and inflammatory diseases were present in 15% of patients. A biological diagnosis of CGD was made in 89/93 patients, on the basis of NBT assay (n = 6), DHR (n = 27), and NBT plus DHR (n = 56). The deficiency was complete in all patients. The median age of biological diagnosis was 17 months (range, 0-186 months). A genetic diagnosis was made in 83/93 patients (when material was available), corresponding to CYBB (n = 64), NCF1 (n = 7), NCF2 (n = 7), and CYBA (n = 5) mutations.
    Conclusions: The clinical manifestations in these Mexican CGD patients were similar to those in patients elsewhere. This cohort is the largest in Latin America. Mycobacterial infections are an important cause of morbidity in Mexico, as in other countries in which tuberculosis is endemic and infants are vaccinated with BCG. X-linked CGD accounted for most of the cases in Mexico, as in other Latin American countries. However, a significant number of CYBA and NCF2 mutations were identified, expanding the spectrum of known causal mutations.
    MeSH term(s) Adolescent ; Autoimmunity ; Child ; Child, Preschool ; Cohort Studies ; Female ; Genes, X-Linked ; Granulomatous Disease, Chronic/epidemiology ; Granulomatous Disease, Chronic/genetics ; Granulomatous Disease, Chronic/immunology ; Humans ; Infant ; Infant, Newborn ; Inflammation ; Male ; Mexico/epidemiology ; Mutation/genetics ; Mycobacterium/physiology ; Mycobacterium Infections/epidemiology ; NADPH Oxidase 2/genetics ; NADPH Oxidases/genetics
    Chemical Substances CYBB protein, human (EC 1.6.3.-) ; NADPH Oxidase 2 (EC 1.6.3.-) ; NADPH Oxidases (EC 1.6.3.-) ; NCF2 protein, human (EC 1.6.3.1) ; neutrophil cytosolic factor 1 (EC 1.6.3.1)
    Language English
    Publishing date 2020-02-10
    Publishing country Netherlands
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 779361-3
    ISSN 1573-2592 ; 0271-9142
    ISSN (online) 1573-2592
    ISSN 0271-9142
    DOI 10.1007/s10875-020-00750-5
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