European Journal of Pediatrics

, Volume 152, Issue 4, pp 313–315 | Cite as

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

  • N. Satake
  • M. Nakanishi
  • M. Okano
  • K. Tomizawa
  • A. Ishizaka
  • K. Kojima
  • M. Onodera
  • T. Ariga
  • A. Satake
  • Y. Sakiyama
  • N. Ishikawa
  • S. Matsumoto
Immunology/Allergology

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.

Key words

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

Abbreviations

CSA

cyclosporin A

AIHA

auto-immune haemolytic anaemia

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Copyright information

© Springer-Verlag 1993

Authors and Affiliations

  • N. Satake
    • 1
  • M. Nakanishi
    • 1
  • M. Okano
    • 2
  • K. Tomizawa
    • 1
  • A. Ishizaka
    • 1
  • K. Kojima
    • 1
  • M. Onodera
    • 1
  • T. Ariga
    • 1
  • A. Satake
    • 2
  • Y. Sakiyama
    • 1
  • N. Ishikawa
    • 2
  • S. Matsumoto
    • 1
  1. 1.Department of PaediatricsHokkaido University School of MedicineSapporoJapan
  2. 2.Department of PaediatricsKitami Red Cross HospitalKitamiJapan

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