A Japanese family of X-linked auto-immune enteropathy with haemolytic anaemia and polyendocrinopathy

Noriko Satake, M. Nakanishi, M. Okano, K. Tomizawa, A. Ishizaka, K. Kojima, M. Onodera, T. Ariga, A. Satake, Y. Sakiyama, N. Ishikawa, S. Matsumoto

Research output: Contribution to journalArticle

63 Citations (Scopus)

Abstract

Three cases of X-linked auto-immune enteropathy with haemolytic anaemia and polyendocrinopathy are described from one related Japanese kindred. Two boys had died due to severe diarrhoea accompanied by total or subtotal intestinal villous atrophy.In contrast, although one patient showed the same symptoms and had circulating IgG antibodies against enterocytes, his condition improved dramatically and he developed well following the use of cyclosporin A (CSA). CSA may be beneficial in patients with this rare disorder. Auto-immune enteropathy should be considered as a cause of protracted diarrhoea with unknown aetiology.

Original languageEnglish (US)
Pages (from-to)313-315
Number of pages3
JournalEuropean Journal of Pediatrics
Volume152
Issue number4
DOIs
StatePublished - Apr 1993
Externally publishedYes

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Hemolytic Anemia
Cyclosporine
Diarrhea
Enterocytes
Atrophy
Immunoglobulin G
Antibodies

Keywords

  • Cyclosporin A
  • Haemo lytic anaemia
  • Polyendocrinopathy
  • X-linked auto-immune enteropathy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

A Japanese family of X-linked auto-immune enteropathy with haemolytic anaemia and polyendocrinopathy. / Satake, Noriko; Nakanishi, M.; Okano, M.; Tomizawa, K.; Ishizaka, A.; Kojima, K.; Onodera, M.; Ariga, T.; Satake, A.; Sakiyama, Y.; Ishikawa, N.; Matsumoto, S.

In: European Journal of Pediatrics, Vol. 152, No. 4, 04.1993, p. 313-315.

Research output: Contribution to journalArticle

Satake, N, Nakanishi, M, Okano, M, Tomizawa, K, Ishizaka, A, Kojima, K, Onodera, M, Ariga, T, Satake, A, Sakiyama, Y, Ishikawa, N & Matsumoto, S 1993, 'A Japanese family of X-linked auto-immune enteropathy with haemolytic anaemia and polyendocrinopathy', European Journal of Pediatrics, vol. 152, no. 4, pp. 313-315. https://doi.org/10.1007/BF01956741
Satake, Noriko ; Nakanishi, M. ; Okano, M. ; Tomizawa, K. ; Ishizaka, A. ; Kojima, K. ; Onodera, M. ; Ariga, T. ; Satake, A. ; Sakiyama, Y. ; Ishikawa, N. ; Matsumoto, S. / A Japanese family of X-linked auto-immune enteropathy with haemolytic anaemia and polyendocrinopathy. In: European Journal of Pediatrics. 1993 ; Vol. 152, No. 4. pp. 313-315.
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