[{"data":1,"prerenderedAt":64},["ShallowReactive",2],{"q-nurse-107-1-maternal-pediatric-063":3},{"subject":4,"subjectSlug":5,"subjectFullName":4,"question":6,"related":33,"lastmod":63},"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-107-1-產兒科護理學-063","nurse-107-1-maternal-pediatric-063",107,1,63,"有關罹患甲基丙二酸血症（Methylmalonic Acidemia）的患童，下列敘述何者錯誤？",{"A":14,"B":15,"C":16,"D":17},"是一種有機酸代謝異常的體染色體隱性遺傳疾病","適時補充葡萄糖靜脈輸液及碳酸鈉，避免酸中毒症狀","大量甲基丙二酸、丙酸等有機酸堆積會造成楓糖漿尿症","未治療會造成嚴重的代謝性酸血症、酮酸血症、昏迷","C",null,[],"本題考點是甲基丙二酸血症(Methylmalonic Acidemia)的疾病特性,要找錯誤敘述。依其代謝機轉,此症因酵素缺陷使甲基丙二酸、丙酸等『有機酸』堆積,屬有機酸血症;而『楓糖漿尿症』是另一種疾病,肇因於支鏈胺基酸代謝障礙,病因不同,故 C 錯誤,選 C。選項 A 正確:此症為有機酸代謝異常的體染色體隱性遺傳病。選項 B 正確:急性期補充葡萄糖靜脈輸液供能,並給碳酸氫鈉矯正酸中毒。選項 D 正確:未治療會導致嚴重代謝性酸血症、酮酸血症甚至昏迷。錯項 C 把兩種先天代謝異常混為一談。","先天代謝異常分類記憶:甲基丙二酸血症與丙酸血症屬『有機酸血症』,堆積的是有機酸;楓糖漿尿症(MSUD)屬『胺基酸代謝異常』,堆積的是支鏈胺基酸與其酮酸,尿液帶楓糖漿甜味。兩者雖都可致代謝性酸中毒與昏迷,但生化缺陷與飲食管理不同(MSUD 限支鏈胺基酸;有機酸血症限特定蛋白質前驅物並可能需維生素 B12)。急性期共通處置:停蛋白、給葡萄糖抗分解代謝、碳酸氫鈉糾酸。本題以『把 A 病說成會造成 B 病』的張冠李戴手法設錯項。",[24,27,29,31],{"term":25,"count":26},"體染色體隱性遺傳",11,{"term":28,"count":10},"甲基丙二酸血症有機酸血症",{"term":30,"count":10},"楓糖漿尿症為胺基酸代謝異常",{"term":32,"count":10},"代謝性酸中毒處置",[34,38,43,48,53,58],{"webId":35,"stem":36,"number":37,"year":9,"session":10},"nurse-107-1-maternal-pediatric-064","小芳，3 歲，腦瘤手術住院，媽媽對於小芳 7 歲的哥哥在學校變得易怒、打同學感到煩心，下列措施何者不適當？",64,{"webId":39,"stem":40,"number":41,"year":9,"session":42},"nurse-107-2-maternal-pediatric-080","陳小弟，因腦瘤（brain tumor）接受手術，下列護理措施何者錯誤？",80,2,{"webId":44,"stem":45,"number":46,"year":47,"session":42},"nurse-108-2-maternal-pediatric-014","照顧處於過渡期之待產婦，下列何者為最適當的護理措施？",14,108,{"webId":49,"stem":50,"number":51,"year":52,"session":42},"nurse-109-2-maternal-pediatric-052","下列何者不屬於新生兒篩檢的項目？",52,109,{"webId":54,"stem":55,"number":56,"year":57,"session":10},"nurse-111-1-maternal-pediatric-016","下列何者為枕後位（OP）第一、二產程延長的主要機轉？",16,111,{"webId":59,"stem":60,"number":61,"year":62,"session":10},"nurse-112-1-maternal-pediatric-050","有關不同年齡層兒童口服用藥，下列敘述何者正確？",50,112,"2026-09-04",1788510703856]