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Wiskott-Aldrich氏症候群患者之牙科考量-病例報告

Dental Considerations for the Patient with Wiskott-Aldrich Syndrome: Report of a Case

摘要


Wiskott-Aldrich氏症候群是一種X染色體隱性的性聯遺傳的罕見疾病,患者最主要的3個臨床症狀為血小板數量低下、濕疹、和免疫力缺乏。患者多為男性,通常在2~3個月大時便出現症狀。大多數患者在兒童時期便會死亡,除非骨髓移植成功才能有較高的存活率。本病例報告將討論一位罹患Wiskott-Aldrich氏症候群之11歲男孩,即將於小兒科接受造血細胞移植。我們將就Wiskott-Aldrich氏症候群之臨床症狀、牙科照護之血液值方面考量、及造血細胞移植前之牙科考量,做一文獻回顧及討論。

並列摘要


Wiskott-Aldrich syndrome is a rare, x-linked recessive hereditary disease, mostly affecting male patients. The common expressions of this disease include a triad: thrombocytopenia, eczema, and immunodeficiency. Those who are affected often have symptoms by the age of 2-3 months old, and mostly die in their childhood unless bone marrow transplantation was successfully performed. This case report reviewed the literatures and described an 11-year-old male patient with Wiskott-Aldrich syndrome, who is going to receive hematopoietic cell transplantation. We discussed the clinical symptoms and signs of Wiskott-Aldrich syndrome, hematological considerations for dental care, and dental procedures before hematopoietic cell transplantation.

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