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頁籤選單縮合
題名 | Hyper-IgM Syndrome Complicated with Interstitial Pneumonia and Peritonitis=免疫不全併高值IgM症候群合併間質性肺炎和腹膜炎 |
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作者姓名(中文) | 黃群峰; 王志祿; 黃永豐; 謝凱生; 楊崑德; | 書刊名 | 長庚醫學 |
卷期 | 26:1 2003.01[民92.01] |
頁次 | 頁53-59 |
分類號 | 417.594 |
關鍵詞 | 低丙種球蛋白血症; 免疫不全併高值IgM症候群; CD40結合體; 間質性肺炎; 腹膜炎; Hypogammaglobulinemia; Hyper-IgM syndrome; CD40 ligand; Interstitial pneumonia; Peritonitis; |
語文 | 英文(English) |
中文摘要 | 免疫不全併高值IgM症候群是一種少見的疾病,為CD40結合體基因突變所致,其血清IgM值常偏高或正常,而IgG、IgA及IgE則偏低。本文報告一例疑似肺囊多引起間質性肺炎的病童,經流速細胞計數法證實為免疫不全併高值IgM症候群。經過靜脈注射免疫球蛋白及磺胺類抗生素治療後,肺炎情況明顯戶善。隨後卻併發腹膜炎並且接受手術治療,病理顯示腸淋巴增生現象。回顧相關文獻得知免疫不全併高值IgM症候群合併腹膜炎極其少見,但由本病例可知腸淋巴增生所導致的腹膜炎仍是免疫不全併高值IgM症候群可能的併發症。 |
英文摘要 | Hyper-IgM syndrome (HIM) is a rare disorder resulting from mutation in the CD40 lig and (CD40L) gene. This defect is associated withnormal or elevated serum level of IgM and with low to undetectable levels of serum IgG, IgA, IgE. This case of HIM with CD40L deficiency was proven by flow cytometry but initially presented as interstitial pneumonia. Was highly suggested. After intravenous immunoglobulin and trimethoprim-sulfamethoxazole treatment, his lung condition improved. However, pertoinit developed and surgical intervention was performed. Ileum perforation and intestinal lymphorproliferation from a pathologic specimen were noted. Although peritonitis is extremently rare in patients with HIM, this report indicates that peritonitis which results from intestinal lymphoproliferation may amy be a manifestation of HIM. |
本系統之摘要資訊系依該期刊論文摘要之資訊為主。