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Asplenia and functional hyposplenism in autoimmune polyglandular syndrome type 1

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Abstract

Asplenia was diagnosed in four patients with autoimmune polyendocrine syndrome type-I (APS-I): two children, aged 2–4 years, from the same family and two adults, the father of the two children and his cousin. We have observed a worsening in splenic function in the children during a follow-up of a few years. Patients with APS-I should be evaluated for splenic function, since splenic dysfunction has important therapeutic implications, especially in children.

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Fig. 1

Abbreviations

AIRE:

autoimmune regulator

APS-I:

autoimmune polyglandular syndrome type I

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Authors and Affiliations

  1. Department of Pediatrics B, Schneider Children’s Medical Center of Israel, Petah Tiqwa, 49202, Israel

    Uri Pollak, Vered Hoffer, Nufar Marcus, Oded Scheuerman & Ben Zion Garty

  2. Kipper Institute of Allergy & Imunology, Schneider Children’s Medical Center of Israel at Petah Tiqwa, Tel Aviv, Israel

    Nufar Marcus & Ben Zion Garty

  3. The Division of Nuclear Medicine, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel

    Zvi Bar-Sever

Authors
  1. Uri Pollak
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  2. Zvi Bar-Sever
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  3. Vered Hoffer
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  4. Nufar Marcus
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  5. Oded Scheuerman
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  6. Ben Zion Garty
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Corresponding author

Correspondence to Ben Zion Garty.

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Pollak, U., Bar-Sever, Z., Hoffer, V. et al. Asplenia and functional hyposplenism in autoimmune polyglandular syndrome type 1. Eur J Pediatr 168, 233–235 (2009). https://doi.org/10.1007/s00431-008-0735-9

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  • DOI: https://doi.org/10.1007/s00431-008-0735-9

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