Infantile Hypertrophic Pyloric Stenosis Presenting as Pseudo Bartter's Syndrome and Seizures: Report of One Case

肥厚性幽門狹窄是一種嬰兒期常見的疾病。典型的病例、只需要藉由明顯的噴射性嘔吐及觸診其上腹部發現橄欖狀硬塊即可診斷，而腹部超童波也有九成以上的診斷率。本案例為一個兩個月大的男嬰，主訴有嘔吐、腹瀉且合併抽痙三天的情形；入院當時評估其有低血氯、低血鉀性驗上毒及合并高腎激素、高醛類脂血症與血壓正常的情形，這些皆符合特氏症假群(Barter's syndrome)的診斷標準。在給予適當的體液補充及矯正電解質的不平衡之後，我們為病人安排一些檢查。初次的腹部超音波檢查并未發現有明顯的病灶；而在第二次的腹部超音波上及上消化攝影則顯示有肥厚性幽門狹窄的情形。病人在接受幽門環狀肌切開術後情況良好。肥厚性幽門狹窄以偽巴特氏症候群(pesudo-Bartter's syndrome)及抽痙來表現是很少見。在文中除了對Bartter's syndrome及pesudo-Bartter's syndrome之成因與機轉做一番瞭解外，也對腹部超童波檢查的限制、上消化道攝影的適用情形及臨別診斷之重要性做一番探討。

關鍵字

僞巴特氏症候群；抽痙；嬰兒肥厚性幽門狹窄

並列摘要

We report a hypertrophic pyloric stenosis case with an unusual initial presentation of seizures and Bartter's syndrome like symptoms. This case suffered from vomiting, diarrhea and poor appetite for several days, and seizures developed after these symptoms. From laboratory tests, hypochloremic and hypokalemic metabolic alkalosis associated with hyperreninemia, hyperaldosteronism and normal blood pressure were noted. Pseudo-Bartter's syndrome was diagnosed through these clinical and laboratory tests. Although the first abdominal echo was negative, we still speculated about the peculiar symptoms of vomiting and it's relationship to pseudo-Bartter's syndrome. After all, we found the hypertrophic pyloric stenosis through an upper gastrointestinal series. From these experiences, we postulated that it's very important to put the hypertrophic pyloric stenosis into the differential diagnosis of pseudo-Batter's syndrome.

並列關鍵字

pseudo-Bartter's syndrome ； seizure disorder ； infantile hypertrophic pyloric stenosis

國際替代計量

Infantile Hypertrophic Pyloric Stenosis Presenting as Pseudo Bartter's Syndrome and Seizures: Report of One Case

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