Document Detail

Delayed onset adenosine deaminase deficiency associated with acute disseminated encephalomyelitis.
MedLine Citation:
PMID:  22447032     Owner:  NLM     Status:  Publisher    
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.
Hideyuki Nakaoka; Hirokazu Kanegane; Hiromichi Taneichi; Kazushi Miya; Xi Yang; Keiko Nomura; Shunichiro Takezaki; Masafumi Yamada; Osamu Ohara; Chikako Kamae; Kohsuke Imai; Shigeaki Nonoyama; Taizo Wada; Akihiro Yachie; Michael S Hershfield; Tadashi Ariga; Toshio Miyawaki
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-3-24
Journal Detail:
Title:  International journal of hematology     Volume:  -     ISSN:  1865-3774     ISO Abbreviation:  -     Publication Date:  2012 Mar 
Date Detail:
Created Date:  2012-3-26     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9111627     Medline TA:  Int J Hematol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Department of Pediatrics, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, 2630 Sugitani, Toyama, Toyama, 930-0194, Japan.
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