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題名 | Langerhans Cell Histiocytosis--A 10-Year Review=Langerhans細胞組織球增殖症--十年之回顧 |
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作者 | 夏文傑; 徐山靜; 朱惠瑜; 遲景上; | 書刊名 | 中華民國小兒科醫學會雜誌 |
卷期 | 35:5 民83.09-10 |
頁次 | 頁385-390 |
分類號 | 415.96 |
關鍵詞 | Letter-siwe病; Hand-schuller-christian病; 嗜伊紅性肉芽腫; 組織球增殖症X; Letterer-siwe disease; Hand-schuller-christian disease; Eosinophilic granuloma; Histiocytosis X; |
語文 | 英文(English) |
中文摘要 | 從1982年十月至1992年十月,針對17位經由電子顯微鏡發現Birbeck顆粒,确定診斷為Langerhans細胞組織球增殖症,作一回顧性之研究。其包括了男性11位,女性6位,年齡由5個月至17歲不等(平均6歲)。臨床表徵非常多樣化,包括有發燒、疼痛、骨病灶、肺病灶、不正常齒列、尿崩症、口腔潰瘍、流耳水、皮疹、貧血、血小板低下、淋巴腺腫大、以及肝脾腫大。在骨病灶方面,以侵犯頭骨及股骨最多。我們主要治療方法有用術切除,放射線治療,以及化學療法。較差的預後因子包括有較早發病年紀,較多器官系統被侵犯,器官功能不正常,以及臨床診斷為Letterer-Siwe者。雖然Langerhans細胞組織球增殖症不是一種罕見疾病,但是它值得進一步之研究,以探討其神秘之病因。 |
英文摘要 | Hospital records of seventeen patients (11 males & 6 females) with Langerhans cell histiocytosis, confirmed by electron microscopic demonstration of Birbeck granules, were studied retrospectively from October 1982 to October 1992 at Taichung Veterans General Hospital. The ages at presentation ranged from 5 months to 17 years (a median of 6 years). The clinical features were protean and included fever, pain, bony lesions, lung lesions, abnormal dentition, diabetes insipidus, oral ulcer, otorrhea, dermatitis, anemia, thrombocytopenia, lymphadenopathy, and hepatosplenomegaly. Skull and femur were the most common sites of bony lesions. The main therapeutic modalities were excision, radiotherapy, and chemotherapy with vincristine and prednisolone. The young age at presentation, several involved organ systems, presence of organ dysfunction, and clinical diagnosis with Letterer-Siwe disease were poor prognostic factors. Although Langerhans cell histiocytosis is not a rare disease, the cause is still unknown. It needs further research to disclose the mystery. |
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