[{"data":1,"prerenderedAt":66},["ShallowReactive",2],{"q-nurse-109-1-maternal-pediatric-056":3},{"subject":4,"subjectSlug":5,"subjectFullName":4,"question":6,"related":36,"lastmod":65},"產兒科護理學","maternal-pediatric",{"id":7,"webId":8,"year":9,"session":10,"subject":4,"number":11,"stem":12,"options":13,"answer":18,"answerNote":19,"images":20,"explanation":21,"explanationDeep":22,"topics":23,"freq":26},"nurse-109-1-產兒科護理學-056","nurse-109-1-maternal-pediatric-056",109,1,56,"小銘，2 歲，罹患典型的異戊酸血症（Isovaleric Acidemia），下列敘述何者正確？",{"A":14,"B":15,"C":16,"D":17},"小銘的疾病是一種有機酸代謝異常的體染色體顯性遺傳疾病","大量的異戊酸堆積在體內，小銘的尿液會有明顯的臭腳汗味","小銘急性發作時主要的醫療處置是矯正脫水及呼吸性鹼中毒","小銘可以攝取特殊配方奶，並逐漸增加肉鹼（carnitine）攝取量","B",null,[],"本題考點在於典型異戊酸血症（Isovaleric Acidemia, IVA）的遺傳、症狀與處置。依生化病理，IVA 是白胺酸（leucine）代謝路徑上 isovaleryl-CoA dehydrogenase 缺陷所致的有機酸血症，大量異戊酸堆積會散發特徵性的「臭腳汗味（sweaty feet odor）」，故 B 正確。A 錯在遺傳模式，IVA 屬體染色體隱性遺傳而非顯性。C 錯在酸鹼判讀，急性發作因有機酸堆積造成的是代謝性酸中毒，而非呼吸性鹼中毒，處置重點為矯正酸中毒與脫水。D 的治療方向雖含特殊配方與 carnitine（肉鹼）補充，但配方應限制白胺酸攝取、肉鹼用於促進毒性代謝物排除，敘述方式與重點不正確。故答案為 B。","記憶點：IVA＝白胺酸代謝卡關＋「汗腳味」。有機酸血症（IVA、甲基丙二酸血症、丙酸血症）共通特徵是急性期陰離子間隙增大的代謝性酸中毒、高血氨與酮症。治療兩大支柱：限制前驅胺基酸（低白胺酸配方）＋補充 glycine 與 carnitine 幫助結合排除毒性代謝物。台灣新生兒篩檢已納入此類疾病，早期偵測可避免代謝危象。",[24,27,30,32,34],{"term":25,"count":26},"體染色體隱性遺傳",11,{"term":28,"count":29},"代謝性酸中毒",2,{"term":31,"count":10},"異戊酸血症",{"term":33,"count":10},"白胺酸代謝異常",{"term":35,"count":10},"汗腳味",[37,41,45,50,55,61],{"webId":38,"stem":39,"number":40,"year":9,"session":10},"nurse-109-1-maternal-pediatric-057","小春，國中 3 年級，1 年前被診斷罹患青少年憂鬱症，曾服用 fluoxetine 及 ativan 治療。最近因會考在即，課業壓力導致失眠，情緒常常不穩定，今在家忽然情緒失控，故至醫院求治，醫師表示可能憂鬱症再發，需再服藥治療。母親詢問護理師應該要怎麼做對小春最好，下列回答何者為佳？",57,{"webId":42,"stem":43,"number":44,"year":9,"session":29},"nurse-109-2-maternal-pediatric-073","王小弟腹瀉三天，每天高達 10 次，體重從 10 公斤降至 9.2 公斤，尿量也減少。請問王小弟的脫水程度為何？",73,{"webId":46,"stem":47,"number":48,"year":49,"session":29},"nurse-110-2-maternal-pediatric-007","妊娠各時期的孕婦心理反應會因生理的改變而產生變化。第一妊娠期婦女的心理反應，下列敘述何者最適當？",7,110,{"webId":51,"stem":52,"number":53,"year":54,"session":29},"nurse-111-2-maternal-pediatric-045","兒童誤食下列那種毒物時，可採用催吐處理？",45,111,{"webId":56,"stem":57,"number":58,"year":59,"session":60},"nurse-112-3-maternal-pediatric-009","陳女士，G 2 P 1 ，陰道內診結果為：「Station：floating」，其代表的意思，下列敘述何者正確？",9,112,3,{"webId":62,"stem":63,"number":60,"year":64,"session":10},"nurse-114-1-maternal-pediatric-003","陳女士月經週期規律，偶睡前服用鎮靜劑，因月經過期10天，自行購買驗孕棒進行妊娠試驗，下列敘述何者正確？",114,"2026-09-04",1788510713655]