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頁籤選單縮合
題 名 | Noonan Syndrome Presented with Cystic Hygroma and Chylothorax: Case Report=Noonan症候群合併囊性水瘤與乳糜胸:病例報告 |
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作 者 | 黃新純; 王慈柔; 黃崇濱; | 書刊名 | 長庚醫學 |
卷 期 | 22:2 1999.06[民88.06] |
頁 次 | 頁313-318 |
分類號 | 417.5103 |
關鍵詞 | 乳糜胸; Noonan症候群; 囊性水瘤; 羊水過多; Chylothorax; Noonan syndrome; Cystic hygroma; Polyhydramnios; |
語 文 | 英文(English) |
中文摘要 | 此病例為一位Noonan症候群之男性嬰兒,其母親在產前21週接受超音波檢查時發 現羊水過多及胎兒頸部囊性水瘤。此新生兒於 39 週時經由剖腹產分娩出,出生後發現有呼 吸窘迫,呼吸急促和乳糜胸。此病例亦顯示主肺動脈瓣肥厚、僧帽瓣、三尖瓣反流和左側輕 微水腎, 此等病徵於 6 個月大時已恢復正常,且其頸部之囊性水瘤也於出生後數日消失, 留下蹼狀頸。 一般而言,Noonan 症候群包括多樣的先天性心臟病 (多為肺動脈瓣狹窄 ), 蹼狀頸、胸部畸形和特殊的外觀,例如寬闊的額頭、寬眼距、低位耳和雙側隱睪丸等。本病 例之症狀經由外科療法 (胸部插管及睪丸固定術 ) 與餵食特殊奶粉後已改善。 |
英文摘要 | A cystic hygroma was found in a full-term (39 week-old) male newborn delivered by caesarian section. Polyhydramnios was diagnosed by sonography at 21 weeks of gestation. Clinical manifestations at birth, such as respiratory distress, tachypnea and chylothorax were successfully relieved by the insertion of chest tube and medical therapy. Other conditions, which included mild hydronephrosis of left kidney, thickening of aortic and pulmonary valves with mild mitral and tricuspid valves regurgitation, subsided within six months. The cystic hygroma regressed to a webbed neck. In general, the features and clinical manifestations of the present case resembled the criteria of Noonan syndrome including various congenital heart defects; webbed neck; chest deformity; a characteristic facial appearance comprising of a broad forehead, ocular hypertelorism, antimongoloid slant of palpebral fissures, low set ears; and bilateral undescended testis etc., suggested by Noonan and other subsequent authors. The outcome of this infant was satisfactory following medical and surgical therapy (chest tube, orchiopexy) and a special dietary regimen. |
本系統中英文摘要資訊取自各篇刊載內容。