RESUMO
Increased intracranial pressure developed as a terminal event in a 5-year-old boy with adrenoleukodystrophy. The CSF protein concentration was 420 mg/dL. Computed tomography showed extensive areas of decreased density in the posterior cerebral white matter. At autopsy, brain weight exceeded the expected value by 20%, and white matter water content was 87.6% (expected value, 72.5%). In rare instances, brain edema may contribute to the development of increased intracranial pressure in adrenoleukodystrophy.
Assuntos
Adrenoleucodistrofia/fisiopatologia , Esclerose Cerebral Difusa de Schilder/fisiopatologia , Pressão Intracraniana , Encéfalo/fisiopatologia , Proteínas do Líquido Cefalorraquidiano/análise , Pré-Escolar , Humanos , MasculinoRESUMO
We treated nine infants who unexpectedly developed shock, seizures, and fever, followed by diarrhea, consumption coagulopathy, and hepatorenal dysfunction. Despite vigorous treatment, three infants died and all except one of the six survivors have severe residual neurologic abnormalities. Postmortem findings included cerebral edema, white matter petechial hemorrhages, gastrointestinal hemorrhages, and fatty liver. These clinicopathologic features are similar to those previously described in 10 infants as being due to hemorrhagic shock and encephalopathy, except for the presence of fatty liver in our patients. Based on the combined experience of 19 infants, we propose diagnostic criteria for hemorrhagic shock and encephalopathy that may facilitate recognition and differentiation from other shock syndromes in infancy.
Assuntos
Encefalopatias/diagnóstico , Choque Hemorrágico/diagnóstico , Encefalopatias/patologia , Encefalopatias/terapia , Diagnóstico Diferencial , Feminino , Síndrome Hemolítico-Urêmica/diagnóstico , Humanos , Lactente , Recém-Nascido , Masculino , Síndrome de Reye/diagnóstico , Choque Hemorrágico/patologia , Choque Hemorrágico/terapia , Choque Séptico/diagnóstico , SíndromeRESUMO
This study examined the association between Ureaplasma urealyticum colonization and the development of chronic lung disease (CLD) in 93 premature infants who were treated with surfactant and who had birth weights < 1251 g. Nasopharyngeal and tracheal cultures for U. urealyticum were obtained at 2 +/- 1 and at 14 +/- 1 days after birth and were positive in 17 (18%) of 93 patients. Infants born vaginally were 4.5 times more likely to be colonized than were those born by cesarean section. Colonization with U. urealyticum was associated with 1.66 (95% confidence interval, 1.24-2.20, P = .024) times the risk of developing CLD and with a greater incidence of > or = 2+ polymorphonuclear leukocytes in the tracheal aspirate at 2 +/- 1 days of age compared with uncolonized infants (P = .025). We conclude that U. urealyticum colonization is associated with CLD even after surfactant treatment. The presence of U. urealyticum is also associated with inflammatory cells in the tracheal aspirate.