Delayed onset adenosine deaminase deficiency associated with acute disseminated encephalomyelitis.
Int J Hematol
; 95(6): 692-6, 2012 Jun.
Article
em En
| MEDLINE
| ID: mdl-22447032
ABSTRACT
Acute disseminated encephalomyelitis (ADEM) is a monophasic, immune-mediated demyelinating disorder that can appear after either immunizations or, more often, infections. Magnetic resonance imaging of patients shows inflammatory lesions in the brain and spinal cord. An immune-mediated mechanism may play a role in this disease, although its precise pathogenesis remains unclear. In this study, a 2-year-old boy presented with ADEM, and he showed improvement on treatment with high-dose intravenous corticosteroids. At the age of 3 years, the presence of recurrent bronchitis, bronchiectasia, and lymphopenia suggested that the patient was suffering from combined immunodeficiency. The patient was finally diagnosed with delayed onset adenosine deaminase deficiency. Delayed onset adenosine deaminase deficiency is frequently associated with autoimmune diseases, including thyroiditis and cytopenia, both of which were observed in the patient. The ADEM in this patient may be a presentation of delayed onset adenosine deaminase deficiency.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Imunodeficiência Combinada Severa
/
Agamaglobulinemia
/
Encefalomielite Aguda Disseminada
Tipo de estudo:
Diagnostic_studies
/
Risk_factors_studies
Limite:
Child, preschool
/
Humans
/
Male
Idioma:
En
Revista:
Int J Hematol
Ano de publicação:
2012
Tipo de documento:
Article