MODY 是一種單基因突變導致的糖尿病，具有以下特點：通常於 25 歲前發病家族性遺傳（常染色體顯性）不依賴胰島素、胰島素分泌保留不伴有胰島素抗性或自體免疫指標| 分型 | 基因 | 臨床特徵 | 治療反應 || ------ | ----- | -------------- | ---------- || MODY 1 | HNF4A | 青年起病、口服藥反應佳 | 對磺醯脲類藥物極敏感 || MODY 2 | GCK | 穩定輕度高血糖、不易有併發症 | 通常無需治療 || MODY 3 | HNF1A | 最常見類型，進展性高血糖 | 對磺醯脲類藥物反應好 || MODY 5 | HNF1B | 合併腎囊腫、泌尿異常、不孕 | 可能需胰島素 |
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