先天性全身型脂肪营养不良1型并反复发作高三酰甘油血症性急性胰腺炎1例  

A Case of Recurrent Hypertriglyceridemic Acute Pancreatitis in Type 1 Congenital Generalized Lipodystrophy

作　　者：周泉秀 陈晓红 ZHOU Quanxiu;CHEN Xiaohong(Medical Department,Jianghan University,Wuhan 430056,China;Department of Endocrinology and Metabolism,Wuhan Children’s Hospital,Wuhan 430015,China)

机构地区：[1]江汉大学医学院,湖北武汉430056 [2]武汉儿童医院遗传代谢内分泌科,湖北武汉430015

出　　处：《标记免疫分析与临床》2024年第7期1368-1372,共5页Labeled Immunoassays and Clinical Medicine

基　　金：武汉市卫健委面上重点项目(编号:WX20B13)。

摘　　要：目的分析1例先天性全身型脂肪营养不良1型(CGL 1型)患儿合并反复发作高三酰甘油血症性急性胰腺炎(HTG-AP)的临床特点。方法回顾性分析武汉儿童医院收治的1例CGL 1型患儿的临床资料、实验室检查、影像学结果和治疗情况,以提高对CGL1型和HTG-AP的认识。结果女性患者,14岁,因急性腹痛入院,患儿全身皮下脂肪缺乏,合并难以控制的糖尿病、严重的高三酰甘油血症以及反复发作的急性胰腺炎。经常规治疗后,腹痛很快缓解,三酰甘油可迅速下降至安全范围,血糖仍控制不佳。结论对于难以控制的糖尿病、严重的高三酰甘油血症及胰岛素抵抗,应考虑CGL 1型可能,严格的生活管理、积极控制代谢并发症,有助于预防HTG-AP的发生、加重或复发。Objective To analyze the clinical characteristics of a case of recurrent hypertriglyceridemic acute pancreatitis(HTG-AP)in a patient with type 1 congenital generalized lipodystrophy(CGL 1).Methods A retrospective analysis of the clinical data,laboratory tests,imaging results,and treatment of a patient with CGL 1 admitted to Wuhan Children’s Hospital was conducted to gain a better understanding of CGL 1 and HTG-AP.Results A 14-year-old female patient presented with acute abdominal pain was studied.The patient exhibited generalized subcutaneous fat loss,accompanied by poorly controlled diabetes mellitus,severe hypertriglyceridemia,and recurrent acute pancreatitis.Following standard treatment,abdominal pain was rapidly relieved,and triglyceride levels quickly decreased to a safe range,while blood glucose remained poorly controlled.Conclusion For poorly controlled diabetes mellitus,severe hypertriglyceridemia,and insulin resistance,CGL 1 should be considered.Accordingly,the strict lifestyle management and active control of metabolic complications can help prevent the occurrence,exacerbation,or recurrence of HTG-AP.

关 键 词：先天性全身型脂肪营养不良1型 高三酰甘油血症性急性胰腺炎 糖尿病 

分 类 号：R657.51[医药卫生—外科学]