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  1. Article ; Online: Chronic neutropenia in childhood: experience of a tertiary center.

    Matushita, Letícia / Valera, Elvis Terci / Aragon, Davi Casale / Scridelli, Carlos Alberto / Roxo-Junior, Persio / de Carvalho, Luciana Martins

    Jornal de pediatria

    2024  Volume 100, Issue 3, Page(s) 311–317

    Abstract: Objective: To assess the prevalence of chronic neutropenia (CN) and the clinical profile of patients with CN aged up to 18 years, followed in the pediatric hematology, rheumatology, or immunology outpatient clinic of a tertiary medical center from May 1, ...

    Abstract Objective: To assess the prevalence of chronic neutropenia (CN) and the clinical profile of patients with CN aged up to 18 years, followed in the pediatric hematology, rheumatology, or immunology outpatient clinic of a tertiary medical center from May 1, 2018, to 30 April 2019.
    Methods: Retrospective observational study carried out by collecting data from the patient's medical charts. CN was defined as absolute neutrophil count (ANC) below 1.5 × 10
    Results: A total of 1,039 patients were evaluated; 217 (20 %) presented CN. Twenty-one (2 %) had AIN, classified as primary in 57 % of the cases. The average age at the onset of symptoms was 38.6 months. During follow-up, patients had 4.2 infections on average; frequency was higher among patients with secondary AIN (p = 003). Isolated neutropenia occurred in 43 % of the patients with AIN. Neutropenia resolved in eight (38 %) of the 21 patients with AIN within 19.6 months on average. Eight patients with secondary AIN met the criteria for Inborn Errors of Immunity.
    Conclusion: AIN prevalence was 2 %. Most cases were first evaluated by a pediatric immunologist or rheumatologist rather than a pediatric hematologist. This study highlights the need for a multidisciplinary approach involving a pediatric immunologist, rheumatologist, and hematologist.
    MeSH term(s) Humans ; Neutropenia/epidemiology ; Retrospective Studies ; Child ; Male ; Female ; Child, Preschool ; Tertiary Care Centers/statistics & numerical data ; Adolescent ; Infant ; Prevalence ; Chronic Disease ; Brazil/epidemiology ; Autoimmune Diseases/epidemiology ; Leukocyte Count
    Language English
    Publishing date 2024-01-02
    Publishing country Brazil
    Document type Journal Article ; Observational Study
    ZDB-ID 731324-x
    ISSN 1678-4782 ; 0021-7557
    ISSN (online) 1678-4782
    ISSN 0021-7557
    DOI 10.1016/j.jped.2023.12.003
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    In stock of ZB MED Cologne/Königswinter

    Zs.B 1265: Show issues Location:
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  2. Article ; Online: Proposal to Screen for Zinc and Selenium in Patients with IgA Deficiency.

    Abu Jamra, Soraya Regina / Komatsu, Camila Gomes / Barbosa, Fernando / Roxo-Junior, Persio / Navarro, Anderson Marliere

    Nutrients

    2023  Volume 15, Issue 9

    Abstract: The increase in life expectancy can be a consequence of the world's socioeconomic, sanitary and nutritional conditions. Some studies have demonstrated that individuals with a satisfactory diet variety score present a lower risk of malnutrition and better ...

    Abstract The increase in life expectancy can be a consequence of the world's socioeconomic, sanitary and nutritional conditions. Some studies have demonstrated that individuals with a satisfactory diet variety score present a lower risk of malnutrition and better health status. Zinc and selenium are important micronutrients that play a role in many biochemical and physiological processes of the immune system. Deficient individuals can present both innate and adaptive immunity abnormalities and increased susceptibility to infections. Primary immunodeficiency diseases, also known as inborn errors of immunity, are genetic disorders classically characterized by an increased susceptibility to infection and/or dysregulation of a specific immunologic pathway. IgA deficiency (IgAD) is the most common primary antibody deficiency. This disease is defined as serum IgA levels lower than 7 mg/dL and normal IgG and IgM levels in individuals older than four years. Although many patients are asymptomatic, selected patients suffer from different clinical complications, such as pulmonary infections, allergies, autoimmune diseases, gastrointestinal disorders and malignancy. Knowing the nutritional status as well as the risk of zinc and selenium deficiency could be helpful for the management of IgAD patients.
    Objectives: to investigate the anthropometric, biochemical, and nutritional profiles and the status of zinc and selenium in patients with IgAD.
    Methods: in this descriptive study, we screened 16 IgAD patients for anthropometric and dietary data, biochemical evaluation and determination of plasma and erythrocyte levels of zinc and selenium.
    Results: dietary intake of zinc and selenium was adequate in 75% and 86% of the patients, respectively. These results were consistent with the plasma levels (adequate levels of zinc in all patients and selenium in 50% of children, 25% of adolescents and 100% of adults). However, erythrocyte levels were low for both micronutrients (deficiency for both in 100% of children, 75% of adolescents and 25% of adults).
    Conclusion: our results highlight the elevated prevalence of erythrocyte zinc and selenium deficiency in patients with IgAD, and the need for investigation of these micronutrients in their follow-up.
    MeSH term(s) Adolescent ; Adult ; Child ; Humans ; Selenium ; IgA Deficiency ; Zinc ; Malnutrition ; Adaptive Immunity
    Chemical Substances Selenium (H6241UJ22B) ; Zinc (J41CSQ7QDS)
    Language English
    Publishing date 2023-04-29
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2518386-2
    ISSN 2072-6643 ; 2072-6643
    ISSN (online) 2072-6643
    ISSN 2072-6643
    DOI 10.3390/nu15092145
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  3. Article ; Online: Gammaglobulin for primary immunodeficiency

    Pérsio Roxo-Junior

    Medicina, Vol 47, Iss

    when should it be used?

    2014  Volume 1

    Abstract: Antibodies are an essential component of the adaptative immune response and hold long-term memory of the immunological experiences throughout life. Antibody defects represent approximately half of the well-known primary immunodeficiencies requiring ... ...

    Abstract Antibodies are an essential component of the adaptative immune response and hold long-term memory of the immunological experiences throughout life. Antibody defects represent approximately half of the well-known primary immunodeficiencies requiring gammaglobulin replacement therapy. On the other hand this therapy can be used for some diseases that do not belong to this group, like hyper IgM syndrome, immunodeficiency with thymoma and severe combined immunodeficiency. This therapy is a safe procedure and induces dramatic improvement in the clinical outcome of patients, reducing the risks of death and complications.
    Keywords Immunity ; Immunologic Deficiency Syndromes ; Infection ; Immunoglobulins ; Biological Therapy ; Medicine ; R
    Language Portuguese
    Publishing date 2014-03-01T00:00:00Z
    Publisher Universidade de São Paulo
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  4. Article ; Online: Bilateral Dacryoadenitis and Central Nervous System Involvement in a Child With Kimura Disease.

    Camacho, Maria Belen / Chahud, Fernando / Boasquevisques, Gustavo / Santos de Oliveira, Ricardo / Roxo-Junior, Persio / Cruz, Antonio Augusto Velasco

    Ophthalmic plastic and reconstructive surgery

    2022  Volume 38, Issue 4, Page(s) e106–e108

    Abstract: Kimura's disease (KD) is a systemic inflammatory condition characterized by lymphadenopathy and subcutaneous nodules in the head and neck region. The lesions have a distinctive histopathological pattern formed by follicular hyperplasia, eosinophilic ... ...

    Abstract Kimura's disease (KD) is a systemic inflammatory condition characterized by lymphadenopathy and subcutaneous nodules in the head and neck region. The lesions have a distinctive histopathological pattern formed by follicular hyperplasia, eosinophilic infiltrates, fibrosis, and vessel proliferation. The disease may occur at all ages but predominates among young males with autoimmune dysfunctions. Visceral and orbital involvement is uncommon. We report a girl with KD who developed bilateral enlargement of the lacrimal glands and a lesion in the left lateral ventricle of the brain indistinguishable from a central nervous system neoplasia. A biopsy of both the lacrimal gland and the lateral ventricle was consistent with KD.
    MeSH term(s) Angiolymphoid Hyperplasia with Eosinophilia/diagnosis ; Central Nervous System/pathology ; Child ; Dacryocystitis/diagnosis ; Dacryocystitis/etiology ; Female ; Humans ; Kimura Disease ; Lymphadenopathy ; Male
    Language English
    Publishing date 2022-03-21
    Publishing country United States
    Document type Journal Article
    ZDB-ID 632830-1
    ISSN 1537-2677 ; 0740-9303
    ISSN (online) 1537-2677
    ISSN 0740-9303
    DOI 10.1097/IOP.0000000000002150
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    In stock of ZB MED Cologne/Königswinter

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  5. Article ; Online: Reply to "House dust mite liquid SLIT effective in atopic dermatitis, even with suboptimal dosing".

    Pimentel, Edine Coelho / Zanetti, Maria Eduarda T / Langer, Sarah Sella / Melo, Janaina Michelle L / Ferriani, Mariana P L / Trevisan Neto, Orlando / Dias, Marina Mendonça / Roxo-Junior, Pérsio / Silva, Jorgete / Aragon, Davi Casale / Arruda, L Karla

    The journal of allergy and clinical immunology. In practice

    2022  Volume 10, Issue 7, Page(s) 1937–1939

    MeSH term(s) Animals ; Antigens, Dermatophagoides ; Dermatitis, Atopic/therapy ; Dermatophagoides pteronyssinus ; Humans ; Pyroglyphidae
    Chemical Substances Antigens, Dermatophagoides
    Language English
    Publishing date 2022-07-09
    Publishing country United States
    Document type Letter ; Research Support, Non-U.S. Gov't ; Comment
    ZDB-ID 2843237-X
    ISSN 2213-2201 ; 2213-2198
    ISSN (online) 2213-2201
    ISSN 2213-2198
    DOI 10.1016/j.jaip.2022.04.022
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    In stock of ZB MED Cologne/Königswinter

    Zs.A 7086: Show issues Location:
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  6. Article ; Online: Primary immunodeficiency diseases: relevant aspects for pulmonologists.

    Roxo Júnior, Pérsio

    Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia

    2009  Volume 35, Issue 10, Page(s) 1008–1017

    Abstract: Primary immunodeficiency diseases comprise a genetically heterogeneous group of disorders that affect distinct components of the innate and adaptive immune system, such as neutrophils, macrophages, dendritic cells, complement proteins and natural killer ... ...

    Abstract Primary immunodeficiency diseases comprise a genetically heterogeneous group of disorders that affect distinct components of the innate and adaptive immune system, such as neutrophils, macrophages, dendritic cells, complement proteins and natural killer cells, as well as T and B lymphocytes. The study of these diseases has provided essential insights into the functioning of the immune system. Primary immunodeficiency diseases have been linked to over 120 different genes, abnormalities in which account for approximately 180 different forms of these diseases. Patients with primary immunodeficiency diseases are most often recognized because of their increased susceptibility to infections. However, these patients can also present with a variety of other manifestations, such as autoimmune diseases, inflammatory diseases and cancer. The purpose of this article is to update the main aspects of primary immunodeficiency diseases, especially regarding the clinical manifestations related to the diagnosis, emphasizing the need for the early recognition of warning signs for these diseases.
    MeSH term(s) Early Diagnosis ; Humans ; Immunologic Deficiency Syndromes/diagnosis ; Immunologic Deficiency Syndromes/therapy ; Pulmonary Medicine
    Language Portuguese
    Publishing date 2009-10-28
    Publishing country Brazil
    Document type Journal Article ; Review
    ZDB-ID 2223157-2
    ISSN 1806-3756 ; 1806-3713
    ISSN (online) 1806-3756
    ISSN 1806-3713
    DOI 10.1590/s1806-37132009001000010
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  7. Article: Efficacy of Probiotics in Children and Adolescents With Atopic Dermatitis: A Randomized, Double-Blind, Placebo-Controlled Study.

    de Andrade, Paula Danielle Santa Maria Albuquerque / Maria E Silva, Jorgete / Carregaro, Vanessa / Sacramento, Laís Amorim / Roberti, Luciana Rodrigues / Aragon, Davi Casale / Carmona, Fabio / Roxo-Junior, Pérsio

    Frontiers in nutrition

    2022  Volume 8, Page(s) 833666

    Abstract: Objective: To evaluate the clinical efficacy of a mixture of probiotics (Lactobacillus and Bifidobacterium) in children and adolescents with atopic dermatitis (AD) and the effects on sensitization, inflammation, and immunological tolerance.: Methods: ...

    Abstract Objective: To evaluate the clinical efficacy of a mixture of probiotics (Lactobacillus and Bifidobacterium) in children and adolescents with atopic dermatitis (AD) and the effects on sensitization, inflammation, and immunological tolerance.
    Methods: In this double-blind, randomized, placebo-controlled clinical trial, we enrolled 60 patients aged between 6 months and 19 years with mild, moderate, or severe AD, according to the criteria proposed by Hanifin and Rajka. Patients were stratified to receive one gram per day of probiotics or placebo for 6 months. The primary outcome was a decrease in SCORing Atopic Dermatitis (SCORAD). Secondary outcomes were to assess the role of probiotics on the use of topical and oral medicines (standard treatment), serum IgE levels, skin prick test (SPT), and tolerogenic and inflammatory cytokines. Background therapy was maintained.
    Results: Forty patients completed the study (24 probiotics, 16 placebo). After treatment for six months, the clinical response was significantly better in the probiotics group; the SCORAD decreased [mean difference (MD) 27.69 percentage points; 95% confidence interval (CI), 2.44-52.94], even after adjustment for co-variables (MD 32.33 percentage points; 95%CI, 5.52-59.13), especially from the third month of treatment on. The reduction of the SCORAD in probiotic group persisted for three more months after the treatment had been discontinued, even after adjustment for co-variables (MD 14.24 percentage points; 95%CI, 0.78-27.70). Patients in the probiotics group required topical immunosuppressant less frequently at 6 and 9 months. No significant changes were found for IgE levels, SPT and cytokines.
    Conclusions: Children and adolescents with AD presented a significant clinical response after 6 months with a mixture of probiotics (Lactobacillus rhamnosus, Lactobacillus acidophilus, Lactobacillus paracasei, and Bifidobacterium lactis. However, this clinical benefit is related to treatment duration. Probiotics should be considered as an adjuvant treatment for AD.
    Language English
    Publishing date 2022-01-26
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2776676-7
    ISSN 2296-861X
    ISSN 2296-861X
    DOI 10.3389/fnut.2021.833666
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  8. Article ; Online: Imunodeficiências primárias

    Pérsio Roxo Júnior

    Jornal Brasileiro de Pneumologia, Vol 35, Iss 10, Pp 1008-

    aspectos relevantes para o pneumologista Primary immunodeficiency diseases: relevant aspects for pulmonologists

    2009  Volume 1017

    Abstract: As imunodeficiências primárias são um grupo de doenças geneticamente heterogêneas que afetam diferentes componentes da imunidade inata e adaptativa, como neutrófilos, macrófagos, células dendríticas, proteínas do sistema complemento, células natural ... ...

    Abstract As imunodeficiências primárias são um grupo de doenças geneticamente heterogêneas que afetam diferentes componentes da imunidade inata e adaptativa, como neutrófilos, macrófagos, células dendríticas, proteínas do sistema complemento, células natural killer e linfócitos B e T. O estudo dessas doenças tem fornecido importantes entendimentos sobre o funcionamento do sistema imune. Mais de 120 diferentes genes já foram identificados, cujas anormalidades são responsáveis aproximadamente 180 diferentes formas de imunodeficiências primárias. Pacientes com imunodeficiências primárias são frequentemente reconhecidos pela sua elevada suscetibilidade a infecções; porém, esses pacientes podem apresentar também várias outras manifestações, como doenças autoimunes, doenças inflamatórias e câncer. O propósito deste artigo é atualizar os principais aspectos das imunodeficiências primárias, especialmente em relação às manifestações clínicas relacionadas ao diagnóstico, enfatizando a necessidade do reconhecimento precoce dos sinais de alerta para essas doenças. Primary immunodeficiency diseases comprise a genetically heterogeneous group of disorders that affect distinct components of the innate and adaptive immune system, such as neutrophils, macrophages, dendritic cells, complement proteins and natural killer cells, as well as T and B lymphocytes. The study of these diseases has provided essential insights into the functioning of the immune system. Primary immunodeficiency diseases have been linked to over 120 different genes, abnormalities in which account for approximately 180 different forms of these diseases. Patients with primary immunodeficiency diseases are most often recognized because of their increased susceptibility to infections. However, these patients can also present with a variety of other manifestations, such as autoimmune diseases, inflammatory diseases and cancer. The purpose of this article is to update the main aspects of primary immunodeficiency diseases, especially regarding the clinical manifestations related to the diagnosis, emphasizing the need for the early recognition of warning signs for these diseases.
    Keywords Infecções respiratórias ; Ativação do complemento ; Síndromes de imunodeficiência ; Fagócitos ; Imunoglobulinas ; Respiratory tract infections ; Complement activation ; Immunologic deficiency syndromes ; Phagocytes ; Immunoglobulins ; Diseases of the respiratory system ; RC705-779 ; Specialties of internal medicine ; RC581-951 ; Internal medicine ; RC31-1245 ; Medicine ; R ; DOAJ:Internal medicine ; DOAJ:Medicine (General) ; DOAJ:Health Sciences
    Language Portuguese
    Publishing date 2009-10-01T00:00:00Z
    Publisher Sociedade Brasileira de Pneumologia e Tisiologia
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  9. Article: A Novel Homozygous

    Barreiros, Lucila A / Segundo, Gesmar R S / Grumach, Anete S / Roxo-Júnior, Pérsio / Torgerson, Troy R / Ochs, Hans D / Condino-Neto, Antonio

    Frontiers in pediatrics

    2018  Volume 6, Page(s) 230

    Abstract: We report a novel ... ...

    Abstract We report a novel homozygous
    Language English
    Publishing date 2018-08-20
    Publishing country Switzerland
    Document type Case Reports
    ZDB-ID 2711999-3
    ISSN 2296-2360
    ISSN 2296-2360
    DOI 10.3389/fped.2018.00230
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  10. Article: Corrigendum: A Novel Homozygous

    Barreiros, Lucila A / Segundo, Gesmar R S / Grumach, Anete S / Roxo-Júnior, Pérsio / Torgerson, Troy R / Ochs, Hans D / Condino-Neto, Antonio

    Frontiers in pediatrics

    2018  Volume 6, Page(s) 358

    Abstract: This corrects the article DOI: 10.3389/fped.2018.00230.]. ...

    Abstract [This corrects the article DOI: 10.3389/fped.2018.00230.].
    Language English
    Publishing date 2018-11-23
    Publishing country Switzerland
    Document type Journal Article ; Published Erratum
    ZDB-ID 2711999-3
    ISSN 2296-2360
    ISSN 2296-2360
    DOI 10.3389/fped.2018.00358
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