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1.
Clin Infect Dis ; 64(6): 767-775, 2017 03 15.
Artigo em Inglês | MEDLINE | ID: mdl-28362954

RESUMO

Background: Although prognosis of Chronic Granulomatous Disease (CGD) has greatly improved, few studies have focused on its long-term outcome. We studied the clinical course and sequelae of CGD patients diagnosed before age 16, at various adult time points. Method: Cross-sectional French nationwide retrospective study of patients screened through the National Reference Center for Primary Immunodeficiencies (CEREDIH) registry. Results: Eighty CGD patients (71 males [88.7%], 59 X-linked [73.7%], median age 23.9 years [minimum, 16.6; maximum, 59.9]) were included, Median ages at diagnosis and last follow-up were 2.52 and 23.9 years, respectively. Seven patients underwent hematopoietic stem cell transplantation. A total of 553 infections requiring hospitalization occurred in 2017 patient-years. The most common site of infection was pulmonary (31%). Aspergillus spp. (17%) and Staphylococcus aureus (10.7%) were the commonest pathogens. A total of 224 inflammatory episodes occurred in 71 patients, mainly digestive (50%). Their characteristics as well as their annual frequency did not vary before and after age 16. Main sequelae were a small adult height and weight and mild chronic restrictive respiratory failure. At age 16, only 53% of patients were in high school. After age 30 years, 9/13 patients were working. Ten patients died during adulthood. Conclusions: Adult CGD patients displayed similar characteristics and rates of severe infections and inflammatory episodes that those of childhood. The high rate of handicap has become a matter of medical and social consideration. Careful follow-up in centers of expertise is strongly recommended and an extended indication of curative treatment by HSCT should be considered.


Assuntos
Doença Granulomatosa Crônica/epidemiologia , Adolescente , Fatores Etários , Antibioticoprofilaxia , Autoimunidade , Infecções Bacterianas/tratamento farmacológico , Infecções Bacterianas/epidemiologia , Infecções Bacterianas/etiologia , Infecções Bacterianas/prevenção & controle , Criança , Pré-Escolar , Efeitos Psicossociais da Doença , Estudos Transversais , Feminino , França/epidemiologia , Doença Granulomatosa Crônica/complicações , Doença Granulomatosa Crônica/diagnóstico , Doença Granulomatosa Crônica/mortalidade , Humanos , Lactente , Recém-Nascido , Masculino , Micoses/tratamento farmacológico , Micoses/epidemiologia , Micoses/etiologia , Micoses/prevenção & controle , Fenótipo , Vigilância da População , Sistema de Registros , Estudos Retrospectivos , Análise de Sobrevida , Avaliação de Sintomas
2.
J Clin Microbiol ; 53(5): 1775-7, 2015 May.
Artigo em Inglês | MEDLINE | ID: mdl-25694520

RESUMO

We report a fatal case of acute lower respiratory tract disease with human rhinovirus C (HRV-C) as the unique cause in a 19-month-old girl with a history of repeated episodes of bronchiolitis. HRV-C type 8 nucleic acids were observed in respiratory, stool, and cerebrospinal fluid samples, and infectious virions were isolated from patient serum after inoculation onto reconstituted airway epithelia.


Assuntos
Sangue/virologia , Bronquiolite/etiologia , Infecções por Picornaviridae/diagnóstico , Infecções por Picornaviridae/virologia , Rhinovirus/isolamento & purificação , Viremia/diagnóstico , Viremia/virologia , Bronquiolite/complicações , Líquido Cefalorraquidiano/virologia , Evolução Fatal , Fezes/virologia , Feminino , Humanos , Lactente , Infecções por Picornaviridae/patologia , Sistema Respiratório/virologia , Rhinovirus/classificação , Rhinovirus/genética , Viremia/patologia , Cultura de Vírus
3.
C R Biol ; 330(11): 837-43, 2007 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-17923378

RESUMO

Innovative and fruitful studies of social bonds have been developed in recent years, although the methods used to establish the existence of a social bond between two individuals have not evolved significantly. Two types of paradigms have been currently used: the separation-reunion paradigm, which evaluates the distress caused by the disruption of the social bond, and choice paradigms, which test the specificity of the bond to a given individual. We have developed a new paradigm based on the idea that the cost an individual was ready to pay in order to gain access to a conspecific depended on the strength of the social bond between the two individuals. To test our paradigm we used mound-building mice, Mus spicilegus that present, in both males and females, a level of tolerance that differs greatly according to the degree of familiarity between the individuals. Our new method for testing social bond revealed unsuspected differences between males and females. Our results suggested that, at least in Mus spicilegus, strong social bonds were not necessary to the development of a high level of tolerance between individuals.


Assuntos
Muridae/psicologia , Apego ao Objeto , Comportamento Social , Animais , Feminino , Masculino , Comportamento de Nidação
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