Müferret ERGÜVÜN, Ümit AKYÜZ, Şirin GÜVEN, Fuat LALOĞLU, Olcay BİLGİÇ

Konvülziyon ve karpopedal spazm ile acil servise başvuran tip 1 otoimmün poliglandüler sendrom ve heliotropik raş birlikteliği

Adolesan dönemde hipokalsemik konvülziyon ile başuran hastalarda nadir olmakla beraber Otoimmün poliglandüler sendrom ( OPS) görülebilir. Otoimmün poliglandüler sendrom, birden fazla endokrin organın disfonksiyonu ile seyreden bir sendromdur. Üç tip OPS tanımlanmıştır. Olgu: konvülziyon ve karpopedal spazm ile başvuran 15 yaşındaki kız çocuğunda hipokalsemi nedeni ile yapılan ileri tetkikler sonucunda Tip 1 OPS tanısı konuldu. Hastada bir-iki yıldır tekrarlayan mukokütanöz kandidiazis enfeksiyon öyküsü mevcuttu. Fizik muayenesi heliotropik raş dışında normal idi.Hastanın kan kalsiyum: 5.8 mg/dl, fosfor: 11.9 mg/dl,ALP: 350 UI/L, PTH: 5.57pg/ml(N: 15-65pg/ml), 25(OH)D3:10,3 ng/ml (Normal: 19-57,6 ng/ml, antitir o globulin antikorları: 75 IU/ml (N:

Anahtar Kelimeler:

Nöbetler, Poliendokrinopatiler, otoimmün, Ergen, Kadın, Spazm, Hipokalsemi

A case of type 1 autoimmune polyglandular syndrom admitted to emergency service with convulsion and carpopedal spasm

Autoimmune polyglandular syndrome is a group of disorders in which multiple endocrine glands secrete insufficient or excess amounts of hormones. Signs and symptoms depend on which glands are affected. Three types: type I, type II, also known as Schmidt syndrome and type III have been described. The 3 major components of PGA-I are chronic mucocutaneous candidiasis, hypoparathyroidism, and autoimmune adrenal insufficiency. We present the case of a 15 year-old girl admitted to our emergency service with convulsion and carpopedal spasm. Physical examination except heliotropic rash was normal. Laboratory findings were: hypocalcemia-5.8 mg/dl, hyperphosphatemia-11.9 mg/dl, low serum PTH level-5.57 pg/ml(N: 15-65 pg/ml), low 25(OH)D3-10,3 ng/ml (Normal: 19-57,6 ng/ml), antithyroglobulin antibodies 75IU/ml(N:

Keywords:

Seizures, Polyendocrinopathies, Autoimmune, Adolescent, Female, Spasm, Hypocalcemia,

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