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題名 | The VATER Association: Analysis of Forty Six Cases Without Karyotyping=VATER聯合畸型--未經染色體檢查之46病例分析 |
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作者 | 黃麗薇; 陳銘仁; 林炫沛; 黃富源; 何文佑; 高信安; 許瓊心; 洪漢陽; 蔡淳娟; |
期刊 | 中華民國小兒科醫學會雜誌 |
出版日期 | 19950100、19950200 |
卷期 | 36:1 民84.01-02 |
頁次 | 頁30-34+68 |
分類號 | 417.133 |
語文 | eng |
關鍵詞 | VATER聯合畸型; VATER association; |
中文摘要 | 從1983年5月到1992年3月在馬偕醫院住院病人中凡具有二項或二項以上VATER聯合畸形的主要异常均列入本研究,總共有46病例。我們將主要异常發生機率與以往文獻作比較發現:在本研究中,無肛症、先天性心臟病及腎病异常是三個較常發生的主要缺陷。13個死亡病例中有5個死於心臟病并發心肺衰竭,為主要死亡原因。許多主要缺陷以外的畸形也被發現,而其中以唇顎裂及尿道下裂占較高比例。整聯而言,這些病例若能存活超過一年,其預後及發展良好。因此欠建議凡有疑似VATER聯合畸形的病例均應接受詳細的理學及實驗檢查,尤其是在心臟疾病、腎病泌尿道疾病、四肢發育及脊椎异常等,一旦有可矯正的异常應及早治療,使病患有更好的生活品質。 |
英文摘要 | Forty-six patients with two or more features of the VATER association admitted to the Mackay Memorial Hospital from May, 1983 to Mar, 1992 were retrospectively enrolled in this study. We compared the incidence of major features with that reported in the literature. Imperforate anus, congenital heart disease, and renal anomalies were the three most common major features in our study. Thirteen patients died. Heart failure was the major cause of mortality. We noted many patients associated minor features, and among them there was a relatively high ratio of cleft lip, cleft palate, and hypospadius. The overall outcome and development were good among the survivals. We suggest that children who have any congenital anomaly included in the VATER association should get a careful examination and evalution of their heart, genitourinary tract, limbs and vertebrae. Early correction if possible is indicated in such patients. |
本系統之摘要資訊系依該期刊論文摘要之資訊為主。