Assuntos
Síndrome de DiGeorge/virologia , Exantema/virologia , Vacina contra Sarampo/imunologia , Vírus do Sarampo/imunologia , Vírus do Sarampo/isolamento & purificação , Criança , Humanos , Masculino , Vacina contra Sarampo/administração & dosagem , Vacina contra Sarampo/efeitos adversos , Vacina contra Sarampo/genética , Vírus do Sarampo/genética , Pele/virologiaRESUMO
OBJECTIVE: To investigate clinicopathological features of DiGeorge syndrome (DGS). METHOD: The clinical features, histological and immunohistochemical findings were analyzed in 5 cases of DGS by autopsy. RESULTS: Five cases of DGS in male infants aged 4 days, 1 month, 7 months, 10 months, and 13 months respectively. Gross and microscopic observations revealed that thymic cortex was depleted of lymphocytes or showed few, dispersed lymphocytes. The thymic medulla showed predominantly epithelial cells with calcified Hassall bodies as well as lymphocyte depletion. T lymphocytes were also scarce in the tonsils, lymph nodes, spleen, and mucosa-associated lymphatic tissue of ileum. In addition, 3 of the 5 patients also showed parathyroid aplasia or dysplasia, and congenital hypertrophy of the ventricular septum. CONCLUSIONS: The pathological changes indicate that clinicians should be aware of defects of immune system if the infants suffer from severe infections. Pathologists should recognize the importance of abnormalities of lymphohematopoietic tissues in the diagnosis of primary immunodeficiency diseases such as DGS.
Assuntos
Síndrome de DiGeorge/patologia , Glândulas Paratireoides/patologia , Linfócitos T/patologia , Timo/patologia , Autopsia , Síndrome de DiGeorge/imunologia , Síndrome de DiGeorge/virologia , Hepatite Viral Humana/patologia , Humanos , Hipertrofia Ventricular Esquerda/patologia , Lactente , Recém-Nascido , Contagem de Linfócitos , Masculino , Pneumonia Viral/patologia , Linfócitos T/imunologiaAssuntos
Citodiagnóstico/métodos , Infecções por Citomegalovirus , Citomegalovirus/isolamento & purificação , Síndrome de DiGeorge , Células Matadoras Naturais/patologia , Citomegalovirus/genética , Infecções por Citomegalovirus/líquido cefalorraquidiano , Infecções por Citomegalovirus/complicações , Infecções por Citomegalovirus/patologia , DNA Viral/líquido cefalorraquidiano , Síndrome de DiGeorge/líquido cefalorraquidiano , Síndrome de DiGeorge/patologia , Síndrome de DiGeorge/virologia , Humanos , Lactente , Reação em Cadeia da PolimeraseRESUMO
Partial DiGeorge syndrome (pDGS) is an inherited primary immunodeficiency syndrome (incidence, 1:3000 live births) primarily affecting cellular immune function; partial, infers thymic hypoplasia with detectable circulating T-lymphocytes and adequate function. No guidelines exist regarding the recommendations for use of live viral vaccines (LVVs) in this extensive population of pediatric patients. We reviewed the experience with live viral vaccines in our cohort of patients with pDGS. Of 53 patients, 25 (47%) had received a live viral vaccine. No significant adverse events were recorded in association with administration of live viral vaccines. There was no statistically significant difference between cellular immune function at initial presentation between those patients that received live viral vaccines and those that did not. Adequate cellular immune function was documented for 15 of the 25 LVV recipients at the time of vaccine administration without significant change from baseline. These observations suggest that live viral vaccines appear safe in patients with pDGS and stable immune function.
