Polyglandular autoimmune type i syndrome with hypoparathyroidism, chronic mucocutaneous candidiasis and intestinal malabsorption
Hayashida, C.Y.; Toledo, S.P.; Barros, M.T.; Ezabella, M.C.; Laudanna, A.A.
Revista do Hospital das Clinicas 45(1): 24-28
1990
ISSN/ISBN: 0041-8781 PMID: 2133167 Document Number: 356375
The occurrence of chronic mucocutaneous candidiasis accompanying polyglandular autoimmune syndrome type I is reported in a female aged 13. Apart the candidiasis, since the age of 3, she had convulsions beginning at 6, cataract at 9, teeth abnormalities, and basal ganglia calcifications. Laboratory data confirmed the diagnosis of hypoparathyroidism. This picture was accompanied by intestinal malabsorption, leading to a state of progressive malnutrition, with intense hypoalbuminemia and anemia. Although the pathophysiology of malabsorption, in these cases, is still not clear, the therapeutic response to pancreatin, in the present case, suggested pancreatic insufficiency, reinforced by the normal d-xylose test and the small intestinal biopsy with inexpressive result.