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Delayed onset adenosine deaminase deficiency associated with acute disseminated encephalomyelitis.

Publication ,  Journal Article
Nakaoka, H; Kanegane, H; Taneichi, H; Miya, K; Yang, X; Nomura, K; Takezaki, S; Yamada, M; Ohara, O; Kamae, C; Imai, K; Nonoyama, S; Wada, T ...
Published in: Int J Hematol
June 2012

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.

Duke Scholars

Published In

Int J Hematol

DOI

EISSN

1865-3774

Publication Date

June 2012

Volume

95

Issue

6

Start / End Page

692 / 696

Location

Japan

Related Subject Headings

  • Severe Combined Immunodeficiency
  • Neuroimaging
  • Male
  • Magnetic Resonance Imaging
  • Lymphocyte Count
  • Immunology
  • Immunoglobulin Isotypes
  • Humans
  • Encephalomyelitis, Acute Disseminated
  • Child, Preschool
 

Citation

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Nakaoka, H., Kanegane, H., Taneichi, H., Miya, K., Yang, X., Nomura, K., … Miyawaki, T. (2012). Delayed onset adenosine deaminase deficiency associated with acute disseminated encephalomyelitis. Int J Hematol, 95(6), 692–696. https://doi.org/10.1007/s12185-012-1055-4
Nakaoka, Hideyuki, Hirokazu Kanegane, Hiromichi Taneichi, Kazushi Miya, Xi Yang, Keiko Nomura, Shunichiro Takezaki, et al. “Delayed onset adenosine deaminase deficiency associated with acute disseminated encephalomyelitis.Int J Hematol 95, no. 6 (June 2012): 692–96. https://doi.org/10.1007/s12185-012-1055-4.
Nakaoka H, Kanegane H, Taneichi H, Miya K, Yang X, Nomura K, et al. Delayed onset adenosine deaminase deficiency associated with acute disseminated encephalomyelitis. Int J Hematol. 2012 Jun;95(6):692–6.
Nakaoka, Hideyuki, et al. “Delayed onset adenosine deaminase deficiency associated with acute disseminated encephalomyelitis.Int J Hematol, vol. 95, no. 6, June 2012, pp. 692–96. Pubmed, doi:10.1007/s12185-012-1055-4.
Nakaoka H, Kanegane H, Taneichi H, Miya K, Yang X, Nomura K, Takezaki S, Yamada M, Ohara O, Kamae C, Imai K, Nonoyama S, Wada T, Yachie A, Hershfield MS, Ariga T, Miyawaki T. Delayed onset adenosine deaminase deficiency associated with acute disseminated encephalomyelitis. Int J Hematol. 2012 Jun;95(6):692–696.
Journal cover image

Published In

Int J Hematol

DOI

EISSN

1865-3774

Publication Date

June 2012

Volume

95

Issue

6

Start / End Page

692 / 696

Location

Japan

Related Subject Headings

  • Severe Combined Immunodeficiency
  • Neuroimaging
  • Male
  • Magnetic Resonance Imaging
  • Lymphocyte Count
  • Immunology
  • Immunoglobulin Isotypes
  • Humans
  • Encephalomyelitis, Acute Disseminated
  • Child, Preschool