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  1. Article ; Online: Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin.

    Sztajnbok, Flavio / Fonseca, Adriana Rodrigues / Campos, Leonardo Rodrigues / Lino, Kátia / Rodrigues, Marta Cristine Félix / Silva, Rodrigo Moulin / de Almeida, Rozana Gasparello / Perazzio, Sandro Félix / Carvalho, Margarida de Fátima Fernandes

    Advances in rheumatology (London, England)

    2024  Volume 64, Issue 1, Page(s) 28

    Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a rare genetic hyperinflammatory syndrome that occurs early in life. Macrophage activation syndrome (MAS) usually refers to a secondary form of HLH associated with autoimmunity, although there are other causes ... ...

    Abstract Hemophagocytic lymphohistiocytosis (HLH) is a rare genetic hyperinflammatory syndrome that occurs early in life. Macrophage activation syndrome (MAS) usually refers to a secondary form of HLH associated with autoimmunity, although there are other causes of secondary HLH, such as infections and malignancy. In this article, we reviewed the concepts, epidemiology, clinical and laboratory features, diagnosis, differential diagnosis, prognosis, and treatment of HLH and MAS. We also reviewed the presence of MAS in the most common autoimmune diseases that affect children. Both are severe diseases that require prompt diagnosis and treatment to avoid morbidity and mortality.
    MeSH term(s) Child ; Humans ; Macrophage Activation Syndrome/diagnosis ; Macrophage Activation Syndrome/etiology ; Lymphohistiocytosis, Hemophagocytic/diagnosis ; Lymphohistiocytosis, Hemophagocytic/complications ; Autoimmune Diseases/complications ; Diagnosis, Differential
    Language English
    Publishing date 2024-04-16
    Publishing country England
    Document type Journal Article ; Review
    ISSN 2523-3106
    ISSN (online) 2523-3106
    DOI 10.1186/s42358-024-00370-2
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Valuing tonsillitis manifestations in schoolchildren in Rio de Janeiro.

    Goldenzon, Rafaela Valentim / Lucas, Tiago Oliveira / Roiseman, Maria de Marilacc Lima / Rodrigues, Marta Cristine Félix / Fonseca, Adriana Rodrigues / Oliveira, Sheila Knupp Feitosa de / Goldenzon, Andréa Valentim

    Einstein (Sao Paulo, Brazil)

    2022  Volume 20, Page(s) eAO6342

    Abstract: Objective: To evaluate the importance attributed to tonsillitis by guardians and their level of knowledge about the disease, correlating their management with their schooling and socioeconomic profiles.: Methods: A quantitative, descriptive and ... ...

    Abstract Objective: To evaluate the importance attributed to tonsillitis by guardians and their level of knowledge about the disease, correlating their management with their schooling and socioeconomic profiles.
    Methods: A quantitative, descriptive and observational cross-sectional study involving students aged 5 to 17 years from state-owned and private schools. A questionnaire was applied on management of tonsillitis and knowledge about rheumatic fever, addressing demographic and socioeconomic data.
    Results: A total of 323 students were included, predominantly females (61.3%), from state-owned schools (77.1%), with a mean age of 9.7±0.3 years. Among the guardians, 48.6% completed high school. Among the students 75.2% had at least one episode of odynophagia in the previous 12 months. Considering the previous 2 years, 89.8% reported this symptom. There was no collection of bacteriological specimens in 67.8%, and in 83% if taking into account only the last episode, despite the search for medical care in most cases (92.6%). The use of anti-inflammatory drugs was very frequent (43.0% "always" and 42.4% "sometimes"). Among the guardians, 81.7% denied being aware of the relation between rheumatic fever and inappropriate treatment of odynophagia; 85.8% said they wished they had received more information.
    Conclusion: Although it is quite common in schoolchildren, tonsillitis is neglected by many guardians and health professionals, and the ignorance of its relation with rheumatic fever is practically universal, which may contribute to its high prevalence.
    MeSH term(s) Adolescent ; Brazil/epidemiology ; Child ; Child, Preschool ; Cross-Sectional Studies ; Female ; Humans ; Male ; Schools ; Students ; Tonsillitis/epidemiology
    Language English
    Publishing date 2022-03-14
    Publishing country Brazil
    Document type Journal Article ; Observational Study
    ZDB-ID 2418293-X
    ISSN 2317-6385 ; 2317-6385
    ISSN (online) 2317-6385
    ISSN 2317-6385
    DOI 10.31744/einstein_journal/2022AO6342
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: The respiratory tract and juvenile rheumatic diseases.

    Domingues, Vinicius / Rodrigues, Marta Cristine Félix / Diniz, Christianne Costa / Almeida, Rozana Gasparello de / Sztajnbok, Flavio Roberto

    Revista brasileira de reumatologia

    2011  Volume 51, Issue 1, Page(s) 88–96

    Abstract: Respiratory tract disorders in the juvenile rheumatic diseases are not infrequent and can have different clinical features when compared with those in the adult diseases. The purpose of this review article is to show the main manifestations of the ... ...

    Abstract Respiratory tract disorders in the juvenile rheumatic diseases are not infrequent and can have different clinical features when compared with those in the adult diseases. The purpose of this review article is to show the main manifestations of the respiratory tract, with an emphasis on lung involvement, in the most frequent juvenile rheumatic diseases.
    MeSH term(s) Humans ; Rheumatic Diseases/complications ; Rheumatic Diseases/diagnosis ; Rheumatic Diseases/drug therapy
    Language Portuguese
    Publishing date 2011-01
    Publishing country Brazil
    Document type Journal Article ; Review
    ISSN 1809-4570
    ISSN (online) 1809-4570
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Challenges in diagnosis and treatment of a case of SAPHO syndrome in.

    Silva, Patricia Craveiro Gomes da / Oliveira, Elisa Fontenelle de / Goldenzon, Andréa Valentim / Silva, Pâmela Craveiro Gomes da / Rodrigues, Marta Cristine Felix

    Anais brasileiros de dermatologia

    2011  Volume 86, Issue 4 Suppl 1, Page(s) S46–9

    Abstract: The authors report a case of SAPHO Syndrome, in pediatric age, with a dermatological focus. This entity should be considered in patients who have pain in the anterior chest wall or other musculoskeletal symptoms, accompanied by palmoplantar pustulosis ... ...

    Abstract The authors report a case of SAPHO Syndrome, in pediatric age, with a dermatological focus. This entity should be considered in patients who have pain in the anterior chest wall or other musculoskeletal symptoms, accompanied by palmoplantar pustulosis and acne fulminans. The specific cutaneous manifestations, diagnosis and the treatment will be presented.
    MeSH term(s) Acquired Hyperostosis Syndrome/pathology ; Acquired Hyperostosis Syndrome/therapy ; Child ; Chronic Disease ; Diagnosis, Differential ; Humans ; Infant ; Male
    Language Portuguese
    Publishing date 2011-10-20
    Publishing country Spain
    Document type Case Reports ; Journal Article
    ZDB-ID 433655-0
    ISSN 1806-4841 ; 0365-0596
    ISSN (online) 1806-4841
    ISSN 0365-0596
    DOI 10.1590/s0365-05962011000700011
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 359: Show issues Location:
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  5. Article ; Online: Profile of Kawasaki disease in children referred to two pediatric rheumatology services in Rio de Janeiro, Brazil.

    Almeida, Rozana Gasparello de / Goldenzon, Andréa Valentim / Rodrigues, Marta Cristine Félix / Sztajnbok, Flávio Roberto / Elsas, Maria Ignez Capella Gaspar / Oliveira, Sheila Knupp Feitosa de

    Revista brasileira de reumatologia

    2010  Volume 50, Issue 5, Page(s) 529–538

    Abstract: Objectives: To describe a population of children diagnosed with Kawasaki's disease (KD) in pediatric rheumatology centers of Rio de Janeiro, Brazil, defining the magnitude of the delay period in diagnosing KD and initiating treatment due to confusion ... ...

    Abstract Objectives: To describe a population of children diagnosed with Kawasaki's disease (KD) in pediatric rheumatology centers of Rio de Janeiro, Brazil, defining the magnitude of the delay period in diagnosing KD and initiating treatment due to confusion with common childhood febrile illnesses and the impact of this delay on the frequency of coronary sequels.
    Methods: Data analysis from hospital records summarized in a dedicated form, including name, gender, age, date of first recorded clinical signs, date of admission to the specialty service, information about symptoms, clinical evolution, intravenous immunoglobulin (IVIG) use and coronary sequels.
    Results: Of 125 patients, 63% were males. 40% were under 2 years at diagnosis. Average lapse between earliest signs and KD diagnosis was 12 days (mean fever duration, 14 d). Only 22.4% had a diagnosis of KD before entering the specialty service. For the remainder, initial hipotheses included: bacterial (60%) and viral infections (12%), rheumatological diseases (4%) and adverse vaccination reactions (1.6%). Hence, prevalent febrile illnesses of childhood were major confounding factors. For records (85.6%) mentioning treatment, 46.7% reported IVIG treatment, beginning after day 10 in 23 cases (21.5%). 20 patients (16%) presented coronary sequels, 9 of which were diagnosed late, including 3 given IVIG after day 10, and 6 given no IVIG. We found no significant association between the frequency of coronary sequels and: a) sex; b) age; c) clinical criteria; d) initiation of IVIG treatment (before or after day 10).
    Conclusions: Common febrile illnesses of childhood often confound the diagnosis of KD.
    MeSH term(s) Brazil ; Child ; Child, Preschool ; Delayed Diagnosis ; Female ; Health Facilities ; Humans ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome/diagnosis ; Mucocutaneous Lymph Node Syndrome/drug therapy ; Retrospective Studies ; Urban Health
    Keywords covid19
    Language Portuguese
    Publishing date 2010-09
    Publishing country Brazil
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2223192-4
    ISSN 1809-4570 ; 0482-5004
    ISSN (online) 1809-4570
    ISSN 0482-5004
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article: Indicacaões e efeitos adversos das drogas anti-TNFalpha em reumatologia pediátrica: experiencia de um unico centro.

    de Oliveira, Sheila Knupp Feitosa / de Almeida, Rozana Gasparello / Fonseca, Adriana Rodrigues / Rodrigues, Marta Cristine Félix / Sztajnbok, Flavio / Diniz, Christianne

    Acta reumatologica portuguesa

    2007  Volume 32, Issue 2, Page(s) 139–150

    Abstract: Objective: To report the efficacy and adverse events with the use of anti-TNFalpha agents in pediatric patients with rheumatic diseases.: Patients and methods: Retrospective, observational clinical case series of patients with rheumatic diseases ... ...

    Title translation Indications and adverse events with the use of anti-TNFalpha agents in pediatric rheumatology: experience of a single center.
    Abstract Objective: To report the efficacy and adverse events with the use of anti-TNFalpha agents in pediatric patients with rheumatic diseases.
    Patients and methods: Retrospective, observational clinical case series of patients with rheumatic diseases refractory to the conventional treatment. Infliximab and etanercept were the the drugs used.
    Results: Thirty patients received anti-TNFalpha therapy: Juvenile Idiopathic Arthritis (18), chronic idiopathic anterior uveitis (2), juvenile dermatomyositis (4), Blau syndrome (1), relapsing polychondritis (2), CINCA syndrome (1), and microscopic polyangiitis (1). Twenty (66,6%) patients used infliximab exclusively, 2 (6,6%) etanercept and 8 (26,6%) both drugs. The response with infliximab was good in 9 patients, partial in 12 and poor in 5. Two patients were not evaluated for the short period of treatment. The response with etanercept was good in 7, partial in 2 and poor in 1. The best benefits were observed in JIA patients with polyarticular arthritis, psoriatic arthritis and enthesitis-related arthritis. Adverse effects occurred in 15 patients (50%). Suspension of the treatment due to adverse events occurred in 7. The most frequent infusion reactions with the infliximab were cough and nausea. Infectious complications occurred in 2 patients receiving etanercept and in 7 receiving infliximab (one death due to sepsis). No patients developed tuberculosis.
    Conclusion: Infliximab and etanercept are effective in some subtypes of juvenile idiopathic arthritis and other rheumatic diseases refractory to conventional therapy. Although frequent, adverse events could be controlled most of the time.
    MeSH term(s) Adolescent ; Adult ; Antibodies, Monoclonal/adverse effects ; Antibodies, Monoclonal/therapeutic use ; Antirheumatic Agents/adverse effects ; Antirheumatic Agents/therapeutic use ; Child ; Child, Preschool ; Etanercept ; Female ; Humans ; Immunoglobulin G/adverse effects ; Immunoglobulin G/therapeutic use ; Infliximab ; Male ; Receptors, Tumor Necrosis Factor/therapeutic use ; Retrospective Studies ; Rheumatic Diseases/drug therapy ; Tumor Necrosis Factor-alpha/antagonists & inhibitors
    Chemical Substances Antibodies, Monoclonal ; Antirheumatic Agents ; Immunoglobulin G ; Receptors, Tumor Necrosis Factor ; Tumor Necrosis Factor-alpha ; Infliximab (B72HH48FLU) ; Etanercept (OP401G7OJC)
    Language Portuguese
    Publishing date 2007-04
    Publishing country Portugal
    Document type English Abstract ; Journal Article
    ZDB-ID 442031-7
    ISSN 0303-464X
    ISSN 0303-464X
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.B 2113: Show issues Location:
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