eISSN: 1644-4124
ISSN: 1426-3912
Central European Journal of Immunology
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4/2011
vol. 36
 
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abstract:

Clinical immunology
Problem of asplenia in a patient with autoimmune polyglandular syndrome type 1

Edyta Heropolitańska-Pliszka
,
Bożena Mikołuć
,
Elżbieta Rowińska
,
Barbara Pietrucha
,
Radosław Motkowski
,
Przemysław Westfal
,
Elżbieta Piskorska
,
Ewa Bernatowska

(Centr Eur J Immunol 2011; 36 (4): 256-261)
Online publish date: 2011/12/24
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Autoimmune polyglandular syndrome (APS) type 1 is a rare autosomal recessive endocrinopathy characterized by the presence of two out of three major component diseases: chronic candidiasis, chronic hypoparathyroidism and autoimmune adrenal insufficiency. Other endocrine and nonendocrine conditions such as thyroid autoimmune disease, malabsorption or ectodermal dystrophy can be present. We report a case of a 15-year-old boy, who simultaneously presented chronic oral candidiasis, adrenal insufficiency and hypoparathyroidism. Additionally, the patient suffered from gastrointestinal involvement (malabsorption probably caused by secretory failure of exocrine pancreas) and asplenia.

At the age of 14 he developed leucoplakia lesions. The boy was properly treated for endocrine diseases as well as asplenia and chronic candidiasis.

Asplenia occurs very rarely in APS type 1, however for asplenic patients the risk of infection with encapsulated bacteria is higher so they require special treatment to prevent it.
keywords:

asplenia, autoimmune polyglandular syndrome, autoimmunity

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