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Journal of Endocrinology, Metabolism and Diabetes of South Africa

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Autoimmune polyglandular syndrome type 1 in a 12-year-old Ugandan girl

D Kibirige, F Kambugu

Abstract


Autoimmune polyglandular syndrome type 1 (APS-1), also known as autoimmune polyendocrinopathy-candidiasisectodermal dystrophy syndrome, is a very rare disorder of childhood. It is mainly characterised by the presence of at least two of the following: chronic mucocutaneous candidiasis, chronic hypoparathyroidism and autoimmune Addison’s disease. We report on the case of a 12-year-old Ugandan female patient who presented with features
that were most consistent with APS-1 (chronic mucocutaneous candidiasis and hypoparathyroidism). Significant clinical improvement was noted following oral antifungal therapy.



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