A Case of Recurrent Hypertriglyceridemic Acute Pancreatitis in Type 1 Congenital Generalized Lipodystrophy
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.
Congenital generalized lipodystrophy type 1Hypertriglyceridemic acute pancreatitisDiabetes mellitus
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