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1.
Ann Biol Clin (Paris) ; 78(1): 7-16, 2020 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-32108583

RESUMO

Immunosuppression is a well known risk factor for the development of lymphoid pathologies. The classification of these neoplasias is becoming more precise and complex, some features being common to all immunocompromised patients, primarily the important influence of Epstein-Barr virus. Whatever the origin of the immunodepression, these lymphoid proliferations are very heterogeneous, constituting a wide range between polymorphic aspects and clearly lymphomatous morphologies indistinguishable from those observed in immunocompetent subjects. It is important to detect precisely these different categories of proliferation within each group of immunosuppression, to better individualize the prognosis and the management of patients.


Assuntos
Hospedeiro Imunocomprometido , Linfoma/etiologia , Transformação Celular Viral/fisiologia , HIV/fisiologia , Infecções por HIV/complicações , Infecções por HIV/epidemiologia , Infecções por HIV/imunologia , Infecções por HIV/patologia , Humanos , Hospedeiro Imunocomprometido/imunologia , Síndromes de Imunodeficiência/complicações , Síndromes de Imunodeficiência/epidemiologia , Imunossupressores/efeitos adversos , Linfoma/epidemiologia , Linfoma/imunologia , Transtornos Linfoproliferativos/induzido quimicamente , Transtornos Linfoproliferativos/epidemiologia , Transtornos Linfoproliferativos/imunologia , Transplante de Órgãos/efeitos adversos , Transplante de Órgãos/estatística & dados numéricos , Condicionamento Pré-Transplante/efeitos adversos , Condicionamento Pré-Transplante/estatística & dados numéricos
2.
BMC Infect Dis ; 20(1): 80, 2020 Jan 28.
Artigo em Inglês | MEDLINE | ID: mdl-31992207

RESUMO

BACKGROUND: In addition to outbreaks of nosocomial influenza, sporadic nosocomial influenza infections also occur but are generally not reported in the literature. This study aimed to determine the epidemiologic characteristics of cases of nosocomial influenza compared with the remaining severe cases of severe influenza in acute hospitals in Catalonia (Spain) which were identified by surveillance. METHODS: An observational case-case epidemiological study was carried out in patients aged ≥18 years from Catalan 12 hospitals between 2010 and 2016. For each laboratory-confirmed influenza case (nosocomial or not) we collected demographic, virological and clinical characteristics. We defined patients with nosocomial influenza as those admitted to a hospital for a reason other than acute respiratory infection in whom ILI symptoms developed ≥48 h after admission and influenza virus infection was confirmed using RT-PCR. Mixed-effects regression was used to estimate the crude and adjusted OR. RESULTS: One thousand seven hundred twenty-two hospitalized patients with severe laboratory-confirmed influenza virus infection were included: 96 (5.6%) were classified as nosocomial influenza and more frequently had > 14 days of hospital stay (42.7% vs. 27.7%, P < .001) and higher mortality (18.8% vs. 12.6%, P < .02). The variables associated with nosocomial influenza cases in acute-care hospital settings were chronic renal disease (aOR 2.44 95% CI 1.44-4.15) and immunodeficiency (aOR 1.79 95% CI 1.04-3.06). CONCLUSIONS: Nosocomial infections are a recurring problem associated with high rates of chronic diseases and death. These findings underline the need for adherence to infection control guidelines.


Assuntos
Infecção Hospitalar/epidemiologia , Influenza Humana/epidemiologia , Adolescente , Adulto , Idoso , Infecção Hospitalar/tratamento farmacológico , Feminino , Hospitalização/estatística & dados numéricos , Humanos , Síndromes de Imunodeficiência/epidemiologia , Síndromes de Imunodeficiência/virologia , Controle de Infecções , Influenza Humana/tratamento farmacológico , Influenza Humana/virologia , Tempo de Internação/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Estações do Ano , Espanha/epidemiologia , Adulto Jovem
3.
Actas dermo-sifiliogr. (Ed. impr.) ; 110(10): 841-849, dic. 2019. graf, tab
Artigo em Espanhol | IBECS | ID: ibc-185606

RESUMO

Introducción: Desde el año 2000 se ha descrito un aumento progresivo de los casos de sífilis en diferentes ciudades europeas, sobre todo en hombres que tienen sexo con hombres (HSH). Las manifestaciones clínicas de la sífilis pueden estar influidas por la coinfección del VIH o por el antecedente de sífilis. El objetivo del presente estudio es describir las características epidemiológicas y clínicas de los casos de sífilis precoz diagnosticados en Barcelona. Métodos: Estudio retrospectivo de casos de sífilis precoz diagnosticados en Barcelona (2003-2013). Se revisaron las historias clínicas y los datos epidemiológicos y clínicos fueron recogidos de forma estructurada. Las variables se compararon según el estatus serológico del VIH y el antecedente de sífilis. Resultados: Se diagnosticaron 1.702 casos de sífilis precoz (37% primaria, 48% secundaria y 14% latente precoz), 93% de ellos en HSH. Entre ellos, el 40% presentaba una coinfección por VIH. Los casos de sífilis en pacientes VIH positivos estaban asociados con un episodio previo de sífilis (aOR, 5,2 [IC del 95%, 3,32-8,24]) y con haber tenido sexo anal desprotegido (aOR, 1,75 [IC del 95%, 1,17-2,63]). Los casos con antecedentes de sífilis se presentaron con menor frecuencia como sífilis primaria (27,5% vs. 40%) (aOR, 0,58 [IC del 95%, 0,44-0,77]). Un año después del tratamiento, la evolución clínica y serológica fue similar en los pacientes VIH positivo y negativo. Conclusiones: La epidemia de sífilis en Barcelona afecta desproporcionalmente a HSH y está estrechamente relacionada con el VIH. La forma de presentación de la sífilis está influida por el antecedente de sífilis y por el VIH, sin diferencias significativas en cuanto a la evolución un año después del tratamiento


Background: Since 2000, substantial increases in syphilis have been reported in metropolitan areas of Western countries, with increases noted among men who have sex with men (MSM). Clinical manifestations of syphilis might be influenced by concomitant VIH infection and previous episodes of syphilis. The objectives of this study were to describe the epidemiological and clinical characteristics of the cases of syphilis diagnosed in Barcelona. Methods: Retrospective study of cases with early syphilis diagnosed in the referral STI Unit of Barcelona from January 2003 to December 2013. Revision of medical records with structured collection of epidemiological and clinical data. Univariate and multivariate statistical analyses comparing the characteristics of MSM cases with and without VIH infection and with and without previous syphilis. Results: A total of 1702 cases of syphilis (37% primary, 48% secondary and 14% early latent) were diagnosed, 93% of them in MSM. Among MSM 40% were coinfected with VIH, VIH-positive cases were associated with a previous syphilis (aOR, 5.2 [95% CI, 3.32-8.24]) and with unprotected anal intercourse (aOR, 1.75 [95%CI, 1.17-2.63]). Cases with a history of syphilis presented less often with primary syphilis compared to those without it (27.5% vs. 40%) (aOR, 0.58 [95% CI, 0.44-0.77]). One year after treatment, the clinical and serological evolution were similar between VIH-positive and VIH-negative cases. Conclusion: The epidemic of syphilis in Barcelona disproportionately affects MSM and is closelly linked to VIH infection. The presentation of syphilis is influenced by VIH infection and by previous history of syphilis, without significant differences in their evolution after one year of treatment


Assuntos
Humanos , Sífilis/epidemiologia , Epidemias , Diagnóstico Precoce , Síndromes de Imunodeficiência/epidemiologia , Espanha/epidemiologia , Estudos Retrospectivos , Infecções por HIV/complicações , Microscopia , Análise Estatística , Pessoas Transgênero , Minorias Sexuais e de Gênero
4.
Genes (Basel) ; 10(11)2019 10 25.
Artigo em Inglês | MEDLINE | ID: mdl-31731573

RESUMO

Despite its inclusion in pneumococcal conjugate vaccine 13 (PCV13), Streptococcus pneumoniae serotype 3 remains a major cause of invasive pneumococcal disease in England and Wales. Previous studies have indicated that there are distinct lineages within serotype 3 clonal complex 180 and the clade distributions have shifted in recent years with the emergence of clade II. We undertook whole genome sequencing and genomic analysis of 616 serotype 3 isolates from England and Wales between 2003 and 2018, including invasive and carriage isolates. Our investigations showed that clade II has expanded since 2014 and now represents 50% of serotype 3 invasive pneumococcal disease (IPD) isolates in England and Wales. Genomic analysis of antibiotic resistance and protein antigen genes showed that distinct profiles are present within the clades which could account for the recent emergence of this clade. This investigation highlights the importance and utility of routine whole genome sequencing and its ability to identify new and emerging variation at the single nucleotide level which informs surveillance and will impact future vaccine development.


Assuntos
Evolução Molecular , Genoma Bacteriano , Síndromes de Imunodeficiência/microbiologia , Infecções Pneumocócicas/microbiologia , Sorogrupo , Streptococcus pneumoniae/genética , Antígenos de Bactérias/genética , Antígenos de Bactérias/imunologia , Inglaterra , Humanos , Síndromes de Imunodeficiência/epidemiologia , Infecções Pneumocócicas/epidemiologia , Streptococcus pneumoniae/imunologia , Streptococcus pneumoniae/isolamento & purificação , Streptococcus pneumoniae/patogenicidade , País de Gales
5.
Epidemiol Infect ; 147: e295, 2019 10 24.
Artigo em Inglês | MEDLINE | ID: mdl-31647050

RESUMO

Conditions and evidence continue to evolve related to the prediction of the prevalence of immunodeficiency-associated long-term vaccine-derived poliovirus (iVDPV) excreters, which affect assumptions related to forecasting risks and evaluating potential risk management options. Multiple recent reviews provided information about individual iVDPV excreters, but inconsistencies among the reviews raise some challenges. This analysis revisits the available evidence related to iVDPV excreters and provides updated model estimates that can support future risk management decisions. The results suggest that the prevalence of iVDPV excreters remains highly uncertain and variable, but generally confirms the importance of managing the risks associated with iVDPV excreters throughout the polio endgame in the context of successful cessation of all oral poliovirus vaccine use.


Assuntos
Síndromes de Imunodeficiência/virologia , Poliomielite/prevenção & controle , Vacinas contra Poliovirus , Eliminação de Partículas Virais , Antivirais/uso terapêutico , Feminino , Saúde Global , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Síndromes de Imunodeficiência/tratamento farmacológico , Síndromes de Imunodeficiência/epidemiologia , Fatores Imunológicos/uso terapêutico , Masculino , Modelos Imunológicos , Poliomielite/tratamento farmacológico , Poliomielite/transmissão , Poliomielite/virologia , Prevalência , Vigilância em Saúde Pública , Medição de Risco
6.
Allergol. immunopatol ; 47(5): 437-448, sept.-oct. 2019. tab, graf
Artigo em Inglês | IBECS | ID: ibc-186518

RESUMO

Introduction and objectives: Primary immunodeficiency diseases (PIDs) are disorders associated mainly with recurrent and severe infection and an increase in susceptibility to autoimmune conditions and cancer. In Venezuela, PIDs are underdiagnosed and there is usually a delay in their diagnosis. Hence there are no data concerning the frequency and type of PIDs that occur. The aim of this study was to identify and quantify the types of PIDs that occur in Merida, a population within Venezuela. Patients or materials and methods: Following an informative program designed to alert local health professionals to the warning signs for PIDs, patients with a history of recurrent infections were referred to the Instituto de Inmunologia Clinica, Universidad de Los Andes. Results and conclusions: During the three-year period January 2014 to January 2017, thirty-two cases of PIDs were identified in pediatric patients, and 17 different types of PIDs, were identified. Predominantly antibody deficiencies were most frequent (40.6%), followed by immunodeficiencies affecting cellular and humoral immunity (21.8%), congenital defects of phagocyte (18.7%), CID with associated or syndromic features (9.3%), defects in intrinsic and innate immunity (6.4%) and diseases of immune dysregulation (3.2%). These results have important implications not only to the future approach for management of patients in our regions, but add important knowledge concerning PIDs in Latin America and worldwide


No disponible


Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Lactente , Pré-Escolar , Criança , Adolescente , Infecções/imunologia , Síndromes de Imunodeficiência , Controle de Doenças Transmissíveis , Progressão da Doença , Infecções/epidemiologia , Recidiva , Venezuela/epidemiologia , Síndromes de Imunodeficiência/epidemiologia
7.
Am J Otolaryngol ; 40(6): 102275, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31445931

RESUMO

PURPOSE: Recurrent tonsillitis in adults has a significant impact on patients' daily life and healthcare costs. Humoral immunodeficiency increases the susceptibility to recurrent infections. The purpose of this study was to investigate the prevalence and contribution of humoral immunodeficiency in adult patients with recurrent tonsillitis. MATERIAL AND METHODS: A prospective cross-sectional study conducted over 3 years duration with two groups of subjects. Group 1: included 50 normal adult subjects and group 2: included 50 adult patients with recurrent tonsillitis. Recruitment occurred in a tertiary care hospital in Egypt. Different immunoglobulins (Ig A, Ig M and Ig G isotypes) were quantitatively assessed and compared in 2 groups. Incidence of different infections was also compared in patients with humoral immunodeficiency versus patients with intact immunity. RESULTS: 4 (8%) subjects in group 1 had selective humoral Immunodeficiency versus 13 (26%) patients in group 2. Patients with recurrent tonsillitis had significantly lower mean of most assessed immunoglobulins: IgA (P = 0.002), IgM (P = 0.003), IgG (P < 0.0001), IgG1 (P < 0.0001) and IgG3 (P < 0.0001) compared to normal subjects; with no significant difference in mean of IgG2 (P = 0.395) and IgG4 (P = 0.105). Patients with humoral immunodeficiency had significantly higher incidence of tonsillitis (P < 0.0001) and rhinosinusitis (P < 0.0001) attacks compared to patients with normal immunity. CONCLUSION: Adult patients with recurrent tonsillitis may have higher prevalence of humoral immunodeficiency compared to normal subjects. These findings suggest that assessment of immune function should be undertaken routinely in these patients.


Assuntos
Imunoglobulinas/deficiência , Síndromes de Imunodeficiência/epidemiologia , Tonsilite/metabolismo , Adulto , Estudos de Casos e Controles , Estudos Transversais , Egito , Feminino , Humanos , Imunidade Humoral , Masculino , Pessoa de Meia-Idade , Prevalência , Recidiva , Tonsilite/etiologia , Adulto Jovem
8.
Indian J Pediatr ; 86(10): 885-891, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31177511

RESUMO

OBJECTIVES: To report the distribution pattern of various categories of primary immunodeficiency disorders (PIDs) in children from North India, frequency of warning signs and critical parameters for evaluation. METHODS: In this retrospective study, 528 children below 18 y of age after clinical assessment and presentation suggestive of PID were further screened by immunophenotyping for immune cell markers by flow cytometry. RESULTS: A total of 120 (23%) children were diagnosed with PID with median age at diagnosis being 2.5 y in males and 3.5 y in females and an average delay in diagnosis from onset of symptoms being approximately 5 y. Chronic lower respiratory tract infections, gastrointestinal symptoms like persistent diarrhea and failure to thrive were amongst the most common warning signs in these patients. PIDs were classified according to the International Union of Immunological Societies' (IUIS) criteria. The diagnosis of index study subjects included combined humoral and cellular immunodeficiency (29%), phagocytic defects (29%), followed by predominantly antibody deficiency (18%), innate immunity and dysregulation (17%) and other well-defined syndromes (7%). A family history of PID (23%), consanguineous marriage (8%) and previous sibling death (23%) were observed as major clinical predictors/clues for underlying PID. All children received prophylactic antibiotics and/or antifungals in addition to specific therapy for underlying immune deficiency. CONCLUSIONS: The field of PIDs in India remains largely unexplored and we are faced with various challenges in the diagnosis of PIDs due to lack of awareness as well as absence of equipped immunological laboratory support. The authors propose a methodical step-wise laboratory diagnostic approach that can facilitate early diagnosis and timely intervention of these mis/underdiagnosed disorders.


Assuntos
Doenças da Imunodeficiência Primária/epidemiologia , Adolescente , Criança , Pré-Escolar , Consanguinidade , Feminino , Citometria de Fluxo , Humanos , Imunoglobulinas/sangue , Síndromes de Imunodeficiência/diagnóstico , Síndromes de Imunodeficiência/epidemiologia , Síndromes de Imunodeficiência/terapia , Índia/epidemiologia , Masculino , Doenças da Imunodeficiência Primária/diagnóstico , Doenças da Imunodeficiência Primária/fisiopatologia , Doenças da Imunodeficiência Primária/terapia , Infecções Respiratórias/diagnóstico , Infecções Respiratórias/epidemiologia , Estudos Retrospectivos
9.
Afr Health Sci ; 19(1): 1449-1459, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31148972

RESUMO

Background: Primary immunodeficiency diseases (PID) comprise a group of more than 300 diseases that affect development and /or function of the immune system. Objectives: The aim of this study was diagnosis of PID among a suspected group of neonates and infants within the first six months of life as well as identifying the warning signs of PID characteristic to this period. Method: Fifty neonates presenting with warning signs of PID were enrolled in the study. Results: The study revealed that twenty six patients (52%) were diagnosed with Primary Immunodeficiency, T cell/combined immunodeficiency were noted as the most common PID class (88.5%) with fourteen T-B-SCID patients (70%) and six T-B+ SCID patients (30%), phagocytic disorders were estimated to be 7.7% while 3.8% were unclassified immunodeficiency. The mean age of presentation for PID group was 1.42±1.38 months with a diagnostic lag of 3.08±1.78 months. Consanguinity was positive in 76.9% of the PID group. Lower respiratory tract infections, persistent fungal infections and lymphopenia were the most significant warning signs for diagnosing PID with a p value of (0.01). Combined, lower respiratory tract infections, fungal infections and lymphopenia were 12.3 times more likely to be associated with PID. Conclusion: Focused screening in high risk neonates proved to be a valuable tool for diagnosis of PID disorders.


Assuntos
Síndromes de Imunodeficiência/diagnóstico , Síndromes de Imunodeficiência/epidemiologia , Infecções/epidemiologia , Triagem Neonatal , Imunodeficiência Combinada Severa/epidemiologia , Infecções Bacterianas/diagnóstico , Consanguinidade , Egito/epidemiologia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Prevalência , Imunodeficiência Combinada Severa/diagnóstico
10.
Respir Med ; 152: 105-111, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-31128603

RESUMO

BACKGROUND: By definition bronchiectasis (BE) means destructed structure of normal bronchus as a consequence of frequent bacterial infections and inflammation. In many senses, BE is a neglected orphan disease. A recent pan-European registry study, EMBARC, has been set up in order to better understand its pathophysiology, better phenotype patients, and to individualize their management. AIM: To examine the aetiology and co-morbidity of BE in the capital area in Finland. METHODS: Two hundred five patients with BE diagnosis and follow up visits between 2016 and 2017 in Helsinki University Hospital were invited to participate in the study. Baseline demographics, lung functions, imaging, microbiological, and therapeutic data, together with co-morbidities were entered into EMBARC database. Clinical characteristics, aetiologic factors, co-morbidities, and risk factors for extensive BE were explored. RESULTS: To the study included 95 adult patients and seventy nine percent of the BE patients were women. The mean age was 69 years (SD ±â€¯13). Asthma was a comorbid condition in 68% of the patients but in 26% it was estimated to be the cause of BE. Asthma was aetiological factor for BE if it had been diagnosed earlier than BE. As 41% BE were idiopathic, in 11% the disorder was postinfectious and others were associated to rheumatic disease, Alpha-1-antitrypsin deficiency, IgG deficiency and Kartagener syndrome. The most common co-morbidities in addition to asthma were cardiovascular disease (30%), gastroesophageal reflux disease (26%), overweight (22%), diabetes (16%), inactive neoplasia (15%), and immunodeficiency (12%). Extensive BE was found in 68% of BE patients in whom four or more lobes were affected. Risk factors for extensive BE were asthma (OR 2.7), asthma as aetiology for BE (OR 4.3), and rhinosinusitis (OR 3.1). CONCLUSIONS: Asthma was associated to BE in 68% and it was estimated as aetiology in every fourth patient. However, retrospectively, it is difficult to exclude asthma as a background cause in patients with asthma-like symptoms and respiratory infections. We propose asthma as an aetiology factor for BE if it is diagnosed earlier than BE. Asthma and rhinosinusitis were predictive for extensive BE.


Assuntos
Asma/complicações , Bronquiectasia/etiologia , Bronquiectasia/fisiopatologia , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Idoso , Idoso de 80 Anos ou mais , Asma/epidemiologia , Bronquiectasia/diagnóstico , Bronquiectasia/epidemiologia , Comorbidade/tendências , Feminino , Finlândia/epidemiologia , Refluxo Gastroesofágico/complicações , Refluxo Gastroesofágico/epidemiologia , Humanos , Síndromes de Imunodeficiência/complicações , Síndromes de Imunodeficiência/epidemiologia , Incidência , Síndrome de Kartagener/complicações , Síndrome de Kartagener/epidemiologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Doença Pulmonar Obstrutiva Crônica/diagnóstico , Infecções Respiratórias/complicações , Infecções Respiratórias/epidemiologia , Rinite/complicações , Rinite/epidemiologia , Fatores de Risco , Sinusite/complicações , Sinusite/epidemiologia , Deficiência de alfa 1-Antitripsina/complicações , Deficiência de alfa 1-Antitripsina/epidemiologia
11.
Eur J Haematol ; 102(6): 447-456, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30801785

RESUMO

OBJECTIVE: Despite long-standing safe and effective use of immunoglobulin replacement therapy (IgRT) in primary immunodeficiency, clinical data on IgRT in patients with secondary immunodeficiency (SID) due to B-cell lymphoproliferative diseases are limited. Here, we examine the correlation between approved IgRT indications, treatment recommendations, and clinical practice in SID. METHODS: An international online survey of 230 physicians responsible for the diagnosis of SID and the prescription of IgRT in patients with hematological malignancies was conducted. RESULTS: Serum immunoglobulin was measured in 83% of patients with multiple myeloma, 76% with chronic lymphocytic leukemia, and 69% with non-Hodgkin lymphoma. Most physicians (85%) prescribed IgRT after ≥2 severe infections. In Italy, Germany, Spain, and the United States, immunoglobulin use was above average in patients with hypogammaglobulinemia, while in the UK considerably fewer patients received IgRT. The use of subcutaneous immunoglobulin was highest in France (34%) and lowest in Spain (19%). Immunologists measured specific antibody responses, performed test immunization, implemented IgRT, and used subcutaneous immunoglobulin more frequently than physicians overall. CONCLUSIONS: The management of SID in hematological malignancies varied regionally. Clinical practice did not reflect treatment guidelines, highlighting the need for robust clinical studies on IgRT in this population and harmonization between countries and disciplines.


Assuntos
Neoplasias Hematológicas/complicações , Neoplasias Hematológicas/epidemiologia , Síndromes de Imunodeficiência/epidemiologia , Síndromes de Imunodeficiência/etiologia , Gerenciamento Clínico , Saúde Global , Humanos , Imunoglobulinas/sangue , Imunoglobulinas/imunologia , Imunoglobulinas Intravenosas/uso terapêutico , Síndromes de Imunodeficiência/diagnóstico , Síndromes de Imunodeficiência/terapia , Controle de Infecções , Infecções/etiologia , Vigilância em Saúde Pública , Resultado do Tratamento
12.
Med Mal Infect ; 49(3): 167-172, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30782449

RESUMO

Immunodeficiency in the elderly is multifactorial. The analysis of etiological factors demonstrates the major role of immunosenescence and protein-energy malnutrition (PEM) with high prevalence deficiencies in micronutrients such as vitamin D, zinc, or vitamin E in people aged above 75 years. PEM contributes to the numerous consequences of frailty syndrome, and mainly to susceptibility to infections including fungal infections, which are usually observed in immunodeficient patients. Particular attention should thus be paid to these patients. However, these peculiarities of the immune system aging and the aging-related vulnerability can lead to diagnostic delays and treatment escalation, mainly with antibiotics, as well as to a loss of time resulting in a loss of opportunity for patients. Antibiotic escalation also leads to microbiological selection pressure in frail elderly people, which can be deleterious in the long-term in case of opportunistic infections. Guidelines are mainly based on the identification and management of frailty, especially in terms of nutrition. The identification of nutritional risk, dietary management, mood vigilance, and a functional approach are the four pillars of the management strategy. These elements are part of a global geriatric assessment and care.


Assuntos
Idoso Fragilizado , Fragilidade/imunologia , Síndromes de Imunodeficiência/etiologia , Síndromes de Imunodeficiência/terapia , Idoso , Idoso de 80 Anos ou mais , Idoso Fragilizado/estatística & dados numéricos , Fragilidade/epidemiologia , Fragilidade/terapia , Avaliação Geriátrica , Humanos , Sistema Imunitário/fisiologia , Síndromes de Imunodeficiência/epidemiologia , Fatores de Risco
13.
Allergol. immunopatol ; 47(1): 38-42, ene.-feb. 2019. graf
Artigo em Inglês | IBECS | ID: ibc-180769

RESUMO

Introduction: Disseminated BCG infections among other complications of Bacillus Calmette-Guérin (BCG) vaccine are rare and have occurred in children with immunodeficiency disorders such as mendelian susceptibility to mycobacterial disease (MSMD) which could be due to defects in some elements of IL-12/IFN-γ axis. MSMD-causing mutations have been identified in 10 genes during the last two decades. Among them, mutations in the IL12Rβ1 and IFN gamma R1 genes constitute about 80% of recorded cases of MSMD syndrome. The aim of this study was to investigate IL-12RBeta1 and IFN- gammaR1 deficiencies in patients with disseminated BCG infection. Methods: This study was performed on 31 children with disseminated BCG infections who referred to children's medical center. Whole blood cell culture was performed in presence of BCG, IL-12 and IFN- gamma stimulators. The supernatants were assayed for IFN-gamma and IL-12p70 by ELISA method. In order to evaluate IL12Rbeta1 and IFN- gammaR1 receptors expression, flow cytometry staining was performed on the patients’ T-cells stimulated with PHA. Results: Flow cytometry staining of 31 Iranian patients with disseminated BCG infections with the average age of 43 months showed lack of the expression of IL-12RBeta1 and IFN- gamma R1 genes in PHA-T-cells of the nine and one patients, respectively in whom the incomplete production of IFN- gamma and IL-12 was reported by ELISA. Among these 10 patients, eight cases had related parents (80%). Conclusion: It is recommended that to avoid BCG complications, screening be performed for MSMD before BCG inoculation in individuals with positive family history of primary immunodeficiency diseases and inhabitants of areas with high frequency of consanguinity


No disponible


Assuntos
Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Vacina BCG/imunologia , Síndromes de Imunodeficiência/epidemiologia , Mutação/genética , Infecções por Mycobacterium/epidemiologia , Receptores de Interferon/genética , Interleucina-12/genética , Linfócitos T/imunologia , Células Cultivadas , Predisposição Genética para Doença , Imunização , Síndromes de Imunodeficiência/genética , Síndromes de Imunodeficiência/imunologia , Interferon gama/metabolismo , Interleucina-12/metabolismo , Irã (Geográfico)/epidemiologia , Infecções por Mycobacterium/genética , Infecções por Mycobacterium/imunologia
14.
Crit Rev Oncol Hematol ; 133: 149-162, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30661651

RESUMO

Neutropenia is a dangerous and potentially fatal condition that renders patients vulnerable to recurrent infections. Its severity is commensurate with the absolute count of neutrophil granulocytes in the circulation. In paediatric patients, neutropenia can have many different aetiologies. Primary causes make up but a small portion of the whole and are relatively unknown. In the past decades, a number of genes has been discovered that are responsible for congenital neutropenia. By perturbation of mitochondrial energy metabolism, vesicle trafficking or synthesis of functional proteins, these mutations cause a maturation arrest in myeloid precursor cells in the bone marrow. Apart from these isolated forms, congenital neutropenia is associated with a multiplicity of syndromic diseases that includes among others: oculocutaneous albinism, metabolic diseases and bone marrow failure syndromes. Congenital neutropenia is a primary immunodeficiency disease that is associated with recurrent bacterial infections, auto-inflammatory and auto-immune phenomena, haematological malignancy and neuro-psychiatric manifestations. The aim of this review is to give a comprehensive overview of the most recent literature concerning the clinical, aetiological and genetic features of congenital neutropenia and the syndromes in which it might be encountered.


Assuntos
Síndromes de Imunodeficiência , Neutropenia/congênito , Criança , Síndrome Congênita de Insuficiência da Medula Óssea , Humanos , Síndromes de Imunodeficiência/complicações , Síndromes de Imunodeficiência/diagnóstico , Síndromes de Imunodeficiência/epidemiologia , Síndromes de Imunodeficiência/genética , Neutropenia/complicações , Neutropenia/diagnóstico , Neutropenia/epidemiologia , Neutropenia/genética , Fenótipo
15.
PLoS One ; 14(1): e0210353, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30633778

RESUMO

Seasonal influenza is a cause of hospitalization, especially in people with underlying disease or extreme age, and its severity may differ depending on the types and subtypes of circulating viruses. We investigated the factors associated with ICU admission or death in hospitalized patients with severe laboratory-confirmed influenza according to the viral type and subtype. An observational epidemiological study was carried out in patients aged ≥18 years from 12 Catalan hospitals between 2010 and 2016. For each reported case we collected demographic, virological and clinical characteristics. A mixed-effects logistic regression model was used to estimate crude and adjusted ORs. 1726 hospitalized patients were included: 595 (34.5%) were admitted to the ICU and 224 (13.0%) died. Lower ICU admission was associated with age ≥75 years in all influenza types and subtypes and with age 65-74 years for type A. In contrast, the 65-74 and ≥75 years age groups were associated with an increased risk of death in all types and subtypes, especially for type B (aOR 27.42, 95% CI: 4.95-151.93 and 15.96; 95% CI: 3.01-84.68). The comorbidity most closely associated with severe outcomes was immune deficiency, which was associated with death for type B (aOR 9.02, 95% CI: 3.05-26.69) and subtype A(H1N1)pdm09 (aOR 3.16, 95% CI: 1.77-5.66). Older age was a differential factor for ICU admission and death: it was associated with lower ICU admission but a risk factor for death. The comorbidity with the closest association with death was immune deficiency, mainly in influenza type B patients.


Assuntos
Influenza Humana/epidemiologia , Influenza Humana/virologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Comorbidade , Cuidados Críticos , Feminino , Hospitalização , Humanos , Síndromes de Imunodeficiência/epidemiologia , Vírus da Influenza A Subtipo H1N1 , Vírus da Influenza A Subtipo H3N2 , Vírus da Influenza B , Influenza Humana/mortalidade , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Razão de Chances , Fatores de Risco , Espanha/epidemiologia , Adulto Jovem
16.
Clin Exp Immunol ; 195(1): 132-138, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30216434

RESUMO

Good's syndrome (thymoma and hypogammaglobulinaemia) is a rare secondary immunodeficiency disease, previously reported in the published literature as mainly individual cases or small case series. We use the national UK-Primary Immune Deficiency (UKPID) registry to identify a large cohort of patients in the UK with this PID to review its clinical course, natural history and prognosis. Clinical information, laboratory data, treatment and outcome were collated and analysed. Seventy-eight patients with a median age of 64 years, 59% of whom were female, were reviewed. Median age of presentation was 54 years. Absolute B cell numbers and serum immunoglobulins were very low in all patients and all received immunoglobulin replacement therapy. All patients had undergone thymectomy and nine (12%) had thymic carcinoma (four locally invasive and five had disseminated disease) requiring adjuvant radiotherapy and/or chemotherapy. CD4 T cells were significantly lower in these patients with malignant thymoma. Seventy-four (95%) presented with infections, 35 (45%) had bronchiectasis, seven (9%) chronic sinusitis, but only eight (10%) had serious invasive fungal or viral infections. Patients with AB-type thymomas were more likely to have bronchiectasis. Twenty (26%) suffered from autoimmune diseases (pure red cell aplasia, hypothyroidism, arthritis, myasthenia gravis, systemic lupus erythematosus, Sjögren's syndrome). There was no association between thymoma type and autoimmunity. Seven (9%) patients had died. Good's syndrome is associated with significant morbidity relating to infectious and autoimmune complications. Prospective studies are required to understand why some patients with thymoma develop persistent hypogammaglobulinaemia.


Assuntos
Doenças Autoimunes/epidemiologia , Linfócitos B/imunologia , Síndromes de Imunodeficiência/imunologia , Infecções/epidemiologia , Timoma/epidemiologia , Agamaglobulinemia , Idoso , Estudos de Coortes , Feminino , Humanos , Síndromes de Imunodeficiência/epidemiologia , Síndromes de Imunodeficiência/mortalidade , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Prospectivos , Sistema de Registros , Índice de Gravidade de Doença , Análise de Sobrevida , Reino Unido/epidemiologia
17.
Pediatr Pulmonol ; 54(2): 194-199, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30575324

RESUMO

BACKGROUND: The role of viruses in children with respiratory tract infections and humoral immunodeficiencies has hardly been studied. We have evaluated these infections in children with humoral immunodeficiencies who required immunoglobulin replacement therapy, considering their relationship with symptoms, lung function, bacterial co-infection, and outcomes. METHODS: We conducted a prospective case-control study during a 1-year period, including children with humoral immunodeficiencies receiving immunoglobulin replacement therapy. For each patient, at least one healthy family member was included. Respiratory samples for viral detection were taken every 1-3 months, and in case of respiratory tract infections. Symptoms questionnaires were filled biweekly. Spirometry and sputum culture were performed in every episode. RESULTS: Sixty-six episodes were analyzed in 14 patients (median age 12 years; IQR 7-17), identifying 18 respiratory viruses (27.3%), being rhinovirus the most frequently isolated one (12/18; 66%). Positive viral episodes were associated with clinical symptoms (89% vs 43%), more frequent antibiotic treatment (44% vs 15%) or hospital admission (22% vs 0%) than negative ones. Patients with positive viral detection showed impaired lung function, with lower FEV1 and FVC values. CONCLUSIONS: In our experience, viral respiratory tract infections can cause significant respiratory symptoms and impaired lung function, in children with HID, despite immunoglobulin replacement therapy. These patients could benefit from the monitoring of viral infections, as these may be a gateway for ongoing lung damage.


Assuntos
Imunoglobulinas/uso terapêutico , Síndromes de Imunodeficiência , Infecções Respiratórias , Viroses , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Humanos , Síndromes de Imunodeficiência/tratamento farmacológico , Síndromes de Imunodeficiência/epidemiologia , Síndromes de Imunodeficiência/fisiopatologia , Masculino , Testes de Função Respiratória , Infecções Respiratórias/tratamento farmacológico , Infecções Respiratórias/epidemiologia , Infecções Respiratórias/fisiopatologia , Espanha/epidemiologia , Viroses/tratamento farmacológico , Viroses/epidemiologia , Viroses/fisiopatologia
18.
Allergol Immunopathol (Madr) ; 47(1): 38-42, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30268380

RESUMO

INTRODUCTION: Disseminated BCG infections among other complications of Bacillus Calmette-Guérin (BCG) vaccine are rare and have occurred in children with immunodeficiency disorders such as mendelian susceptibility to mycobacterial disease (MSMD) which could be due to defects in some elements of IL-12/IFN-γ axis. MSMD-causing mutations have been identified in 10 genes during the last two decades. Among them, mutations in the IL12Rß1 and IFNγR1 genes constitute about 80% of recorded cases of MSMD syndrome. The aim of this study was to investigate IL-12Rß1 and IFN-γR1 deficiencies in patients with disseminated BCG infection. METHODS: This study was performed on 31 children with disseminated BCG infections who referred to children's medical center. Whole blood cell culture was performed in presence of BCG, IL-12 and IFN-γ stimulators. The supernatants were assayed for IFN-γ and IL-12p70 by ELISA method. In order to evaluate IL12Rß1 and IFN-γR1 receptors expression, flow cytometry staining was performed on the patients' T-cells stimulated with PHA. RESULTS: Flow cytometry staining of 31 Iranian patients with disseminated BCG infections with the average age of 43 months showed lack of the expression of IL-12Rß1 and IFN-γR1 genes in PHA-T-cells of the nine and one patients, respectively in whom the incomplete production of IFN-γ and IL-12 was reported by ELISA. Among these 10 patients, eight cases had related parents (80%). CONCLUSION: It is recommended that to avoid BCG complications, screening be performed for MSMD before BCG inoculation in individuals with positive family history of primary immunodeficiency diseases and inhabitants of areas with high frequency of consanguinity.


Assuntos
Vacina BCG/imunologia , Síndromes de Imunodeficiência/epidemiologia , Mutação/genética , Infecções por Mycobacterium/epidemiologia , Receptores de Interferon/genética , Receptores de Interleucina-12/genética , Linfócitos T/imunologia , Células Cultivadas , Criança , Pré-Escolar , Feminino , Predisposição Genética para Doença , Humanos , Imunização , Síndromes de Imunodeficiência/genética , Síndromes de Imunodeficiência/imunologia , Lactente , Interferon gama/metabolismo , Interleucina-12/metabolismo , Irã (Geográfico)/epidemiologia , Masculino , Infecções por Mycobacterium/genética , Infecções por Mycobacterium/imunologia , Linhagem
19.
Infection ; 47(1): 87-93, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30194635

RESUMO

BACKGROUND: Primary immunodeficiency (PID) in adults is rare and mostly revealed by infections. MATERIAL AND METHODS: Adults without predisposing factors who were admitted to an intensive care unit (ICU) for infection were screened for PID. RESULTS: Six PID cases were diagnosed, mostly revealed by encapsulated bacterial infections. CONCLUSION: Investigation of PID after ICU discharge should be considered to improve early detection.


Assuntos
Infecções Bacterianas/epidemiologia , Síndromes de Imunodeficiência/epidemiologia , Adulto , Infecções Bacterianas/imunologia , Infecções Bacterianas/microbiologia , Feminino , França/epidemiologia , Hospitalização , Humanos , Síndromes de Imunodeficiência/microbiologia , Unidades de Terapia Intensiva , Masculino , Programas de Rastreamento , Pessoa de Meia-Idade , Prevalência , Estudos Prospectivos , Estudos Retrospectivos , Adulto Jovem
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