RESUMO
Disseminated disease caused by non-tuberculous, environmental mycobacteria (EM) reflects impaired host immunity. Disseminated disease caused by Mycobacterium scrofulaceum has primarily been reported in patients with AIDS. Moreover, observing M. scrofulaceum as the agent of localized disease in childhood has become increasingly rare. We report the first case of disseminated disease caused by M. scrofulaceum in a child with inherited interferon-gamma receptor 1 (IFN-gammaR1) complete deficiency. As in this case, mycobacterial bone infections in IFN-gammaR1 deficiency can sometimes mimic the clinical picture of chronic recurrent multifocal osteomyelitis.
Assuntos
Síndromes de Imunodeficiência/complicações , Infecções por Mycobacterium não Tuberculosas , Mycobacterium scrofulaceum/isolamento & purificação , Receptores de Interferon/deficiência , Tuberculose Osteoarticular , Pré-Escolar , Pé/microbiologia , Pé/patologia , Mãos/microbiologia , Mãos/patologia , Humanos , Perna (Membro)/microbiologia , Perna (Membro)/patologia , Masculino , Infecções por Mycobacterium não Tuberculosas/microbiologia , Infecções por Mycobacterium não Tuberculosas/patologia , Tuberculose Osteoarticular/microbiologia , Tuberculose Osteoarticular/patologia , Receptor de Interferon gamaRESUMO
We present a case of a 4-month-old girl referred to the emergency department with a provisional diagnosis of acute life-threatening event with a recent episode of heart block and a history of long-lasting fever. Soon after admission, the child suddenly deteriorated rapidly; she became pulseless with complete heart block and died despite intensive resuscitation efforts. Postmortem examination showed coronary arteritis with aneurysmal dilatation and coronary thrombosis, revealing atypical Kawasaki disease. With this case presentation, we discuss the importance of early recognition and treatment of atypical and/or incomplete forms of Kawasaki disease, which are most common in young infants and may lead, if untreated, to coronary artery abnormalities with a potential for myocardial infarctions, aneurysm formation, and sudden death. In addition, the relevance of postmortem examination in a case of sudden and undiagnosed infant death is underlined.
Assuntos
Doença das Coronárias/patologia , Síndrome de Linfonodos Mucocutâneos/fisiopatologia , Doença das Coronárias/etiologia , Evolução Fatal , Feminino , Humanos , Lactente , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnósticoRESUMO
Congenital varicella syndrome refers to the spectrum of fetal anomalies associated with maternal varicella zoster virus (VZV) infection during the first trimester of pregnancy. The syndrome is rare and the risk to the fetus uncertain. We describe an unusual case of congenital varicella syndrome in which hydrocephalus was the main consequence and likely represented VZV reactivation in utero.
