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題 名 | Tuberous Sclerosis with Visceral Organ Involvement=結節硬化症對內臟器官的侵犯 |
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作 者 | 林宛菁; 沈淵瑤; 邱南昌; 何啟生; 李盈良; 陳銘仁; 蔡政道; | 書刊名 | 臺灣兒科醫學會雜誌 |
卷 期 | 40:5 民88.09-10 |
頁 次 | 頁305-308+357 |
分類號 | 417.5 |
關鍵詞 | 結節硬化症; 內臟器官; 心臟; 腎臟; 小兒科; Tuberous sclerosis; Visceral organ; Cardiac rhabdomyoma; Polycystic kidney; |
語 文 | 英文(English) |
中文摘要 | 結節硬化症是皮膚神經最常見的疾病之一,其特徵在於良性腫瘤可侵犯多個內臟 器官。原因則是田為在胚胎期,外胚層的分化發生問題,導致神經外胚層及皮膚的外胚層都 發生病變。最近的理論認為以中胚層的誘發也有相當關係。因此,這個疾病常發現侵犯丙臟 器官,所以我們進行這個研究,統計近十年的病例進行分析研究。以觀察結節硬化症對內臟 器宮的發生率。我們共有三十例診斷為結節硬化症,包括十七名男孩及十三名女孩。侵犯到心 臟共有十例(40%),六名年齡小於一歲(mean age 1.6±2.2years),有兩名是新生兒:一名是 產前超音波檢查發現有心臟質塊;另一名是因為心搏過快,超音波檢查有心臟橫紋肌瘤 〈Rhabdomyoma〉而接受外科手術。四例侵犯到腎臟(18.1%),其中三名小於五歲(mean age 5.2±5.2 years)。 此十年的研究及追蹤。我們並沒有發現死亡病例。心臟是結節硬化症最早侵犯的內臟器 官。而腎臟是在較晚期發現。吾人建議臨床醫師在治療結節硬化症病患者時,必須要考慮這 些常見的內臟器官的侵犯。 |
英文摘要 | This study is to determine the incidence of visceral organ involvement in tuberous sclerosis (TS). We reviewed 30 cases of TS diagnosed between 1987 to 1997. There were 17 males and l3 females, ages ranged from one day old to 17 years old. Among the 30 cases, 25 patients had seizures and skin manifestations; 24 had cerebral tubercles; 10 had heart involvement (9 rhabdomyoma, I dilated cardiomyopathy); 4 had kidney involvement (3 polycystic kidney disease, I renal hamartoma): and 3 had retinal astrocytic hamartoma. Based on our study, the most common visceral organs involved were the heart and kidney. Among the ten patients with cardiac rhabdomyoma, six were less than I year old (mean age 1.6 ± 2.2 years old). One newborn presented with a cardiac mass diagnosed by prenatal sonography and another newborn, noted to have tachycardia after birth, underwent surgical intervention due to impending heart failure. Four patients had kidney abnormalities: three were less than 5 years old (mean age 5.2 ± 5.2 years). During this l0 year period, there was no mortality seen among patients with visceral organ involvement. We suggest that clinicians treating patients with TS should not overlook the visceral organs, especially heart and kidney, which, if involved can have significant morbidity. |
本系統中英文摘要資訊取自各篇刊載內容。