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自體免疫多發腺體症候羣:二病例報告

Autoimmune Polyglandular Syndrome: Report of Two Cases

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摘要


自體免疫多發腺體症候羣主要侵犯內分泌器官及非內分泌器官。本文提出兩個病例,分別是11歲與13歲的女孩。臨床上均有多食,多渴,多尿的症狀。理學檢查發現頸部均呈第二度甲狀腺腫大。實驗室方面除了高血糖之外,兩例血中的自體抗體如抗甲狀腺球蛋白抗體和抗原漿微粒抗體均為陽性。而且甲狀腺細針穿刺都有何氏細胞與淋巴球浸潤,故診斷為胰島素依賴型糖尿病合併橋本氏甲狀腺炎。此外,病例1尚且罹患多年的幼年型重症肌無力症。如此的組合十分罕見,特提出報告且參考文獻討論之。

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並列摘要


Autoimmune diseases are classified as organ-specific (eg. autoimmune endocrinopathies) or non-organ-specific (eg. collagen vascular diseases). The concurrence of multiple autoimmune endocrinopathies (with or without other nonendocrine autoimmune diseases) is common. We Present two girls, age of eleven and thirteen, respectively. Clinical features included polyphagia, polyuria, polydipsia and grade Ⅱ thyroid enlargement. Laboratory data revealed high blood sugar, thyroid autoantibodies such as antithyroglobulin antibody and antimicrosomal antibody were positive. Fine needle aspiration of thyroid gland showed infiltration of both Hurthle cells and lymphocytes. So, both cases were diagnosed as insulin-dependent diabetes mellitus associated with Hashimoto's thyroiditis. In addition, case 1 had suffered from Juvenile myasthenia gravis since early childhood. Thus we concluded that they were autoimmune polyglandular syndrome type ha (according to the classification of Neufeld and Blizzard). The majority of endocrine disorders seen in children are the result of autoimmune processes. In general, whenever one autoimmune disease is suspected or diagnosed, a search for other autoimmune disease should be made. Recognition of those interrelationships among the autoimmune endocrinopathies provides the rationale for autoantibody screening. Identification of autoantibody-positive individuals followed by appropriate endocrine testing allows the clinician to anticipate and treat disease states before their frank clinical presentation.

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