探讨妊娠相关暴发性1型糖尿病的早期识别、临床特点、诊治和妊娠结局，以提高临床医生对其的诊疗水平，提高母胎生存率。

采用回顾性研究方法分析4例妊娠相关暴发性1型糖尿病(fulminant type 1 diabetes mellitus，FT1DM)患者的临床资料，同时以"暴发性1型糖尿病"为关键词，检索中国知识基础设施工程(CNKI)及中国期刊全文数据库在2000年1月至2024年12月期间收录的文献，初步获得相关论著及病例报告222篇，报道FT1DM患者共423例，在此基础上进一步筛选出妊娠相关性FT1DM的病例100例，并对其进行分析。

本组4例患者均呈现典型的急骤起病过程，在短时间内(1~5 d)从显著高血糖(血浆葡萄糖18.75~38.69 mmol/L)迅速发展为糖尿病酮症或酮症酸中毒，表现为极高的血浆葡萄糖与相对轻度升高的糖化血红蛋白(6.1%~6.9%)之间的"解离"现象(血浆葡萄糖/糖化血红蛋白比值≥3.3)；空腹血清C肽均<100 pmol/L；3例胰岛自身抗体阴性，1例未检测。文献检索100例妊娠相关FT1DM患者资料分析显示，妊娠相关性FT1DM多见于孕期(96%，96例)，短时间内起病(平均约3 d)。4例患者前驱症状表现为消化道症状2例，呼吸系统症状1例，神经系统症状1例；文献检索100例资料分析显示，消化道症状24例(占24%)，呼吸道症状16例(占16%)。4例患者新生儿存活3例，死胎1例；文献检索资料分析显示，妊娠相关性FT1DM总体妊娠结局极差，胎儿丢失率高达60%(以死胎为主，占54%)。

妊娠相关FT1DM起病急、症状隐匿，可导致极高的胎儿丢失率，临床医生需提高预警意识，对无糖尿病病史的孕产妇出现的任何非特异性消化道或呼吸道症状保持高度警惕，将其视为FT1DM的可能前驱信号；立即检测血浆葡萄糖、尿酮体及胰岛功能(C肽)，利用"血糖与糖化血红蛋白解离"这一关键特征进行早期鉴别；启动应急流程，多学科协作紧急救治，提升母胎生存率。

To explore the early identification, clinical characteristics, diagnosis and treatment, and pregnancy outcomes of pregnancy-related fulminant type 1 diabetes mellitus (FT1DM), in order to improve clinicians' diagnostic and therapeutic approach and increase maternal and fetal survival rates.

A retrospective analysis was conducted on the clinical data of 4 patients with pregnancy-related FT1DM. Additionally, a literature search was performed using the keyword " fulminant type 1 diabetes" in the China National Knowledge Infrastructure (CNKI) and Chinese Journal Full-text Database from January 2000 to December 2024. A total of 222 relevant articles and case reports were retrieved, including 423 patients with FT1DM. Among these, 100 cases of pregnancy-related FT1DM were further screened and analyzed.

All four patients presented with a typical acute onset, rapidly progressing from markedly elevated blood glucose (random blood glucose 18.75-38.69 mmol/L) to diabetic ketosis or ketoacidosis within a short period (1-5 days). They exhibited a " dissociation" phenomenon between extremely high random blood glucose and relatively mildly elevated glycated hemoglobin (6.1%-6.9%), with a PG/HbA1c ratio ≥3.3. Fasting serum C-peptide levels were all <100 pmol/L; pancreatic autoantibodies were negative in 3 patients and not tested in 1. Literature analysis of 100 pregnancy-related FT1DM cases showed that the condition was more common during pregnancy (96%, 96 cases), with acute onset (mean time approximately 3 days). The four patients presented with non-specific prodromal symptoms: gastrointestinal symptoms in 2 cases, respiratory symptoms in 1 case, and neurological symptoms in 1 case.Literature analysis of 100 pregnancy-related FT1DM cases showed that The most common prodromal symptoms were gastrointestinal symptoms (24%, 24 cases), followed by respiratory symptoms (16%, 16 cases). After multidisciplinary treatment, 3 neonates survived and 1 was stillborn. Literature analysis showed that the overall pregnancy outcomes were extremely poor, with a fetal loss rate as high as 60% (mainly stillbirths, accounting for 54%).

Pregnancy-related FT1DM has an acute onset and insidious symptoms, and leads to an extremely high rate of fetal loss. Clinicians should enhance their awareness and remain highly vigilant for any non-specific gastrointestinal or respiratory symptoms in pregnant women without a history of diabetes, considering them as possible prodromal signs of FT1DM. Random blood glucose, urine ketone bodies, and pancreatic function (C-peptide) should be tested immediately, and the key feature of " dissociation between blood glucose and glycated hemoglobin" should be used for early differentiation. Emergency protocols should be initiated, and multidisciplinary collaboration should be implemented to improve maternal and fetal survival.