CAJ | 학술논문

目的探讨获得性部分性脂肪萎缩疾病(APL)患者的临床特点、病程及对治疗的反应.方法回顾分析1991年至2012年北京协和医院诊断的3例APL患者的临床治疗及实验室检查资料,并进行长期随访.结果 3例患者均为女性,起病年龄均为10岁左右,诊断年龄20～ 62岁,随访2～ 12年.3例患者均有上半身脂肪明显减少下半身脂肪不变或增加,均患有糖尿病,有明显高胰岛素血症、高甘油三酯血症及脂肪肝,最严重患者甘油三酯达48.32 mmol/L.2例患者补体C3降低.2例患者有正常月经,其中1例患者正常妊娠.例3患者(65岁)有轻度颈动脉内中膜增厚.3例患者均无肾小球肾炎,均未合并其他自身免疫疾病,均无糖尿病微血管并发症.采用以吡格列酮为基础的药物治疗,血糖控制基本满意,甘油三酯可基本控制于正常范围,但患者上半身脂肪萎缩情况无明显改善.结论 APL患者通过以吡格列酮为基础的治疗,可以获得基本良好的代谢控制,但是脂肪萎缩情况无明显改善.
목적 탐토획득성부분성지방위축질병(APL)환자적림상특점、병정급대치료적반응.방법 회고분석1991년지2012년북경협화의원진단적3례APL환자적림상치료급실험실검사자료,병진행장기수방.결과 3례환자균위녀성,기병년령균위10세좌우,진단년령20～ 62세,수방2～ 12년.3례환자균유상반신지방명현감소하반신지방불변혹증가,균환유당뇨병,유명현고이도소혈증、고감유삼지혈증급지방간,최엄중환자감유삼지체48.32 mmol/L.2례환자보체C3강저.2례환자유정상월경,기중1례환자정상임신.례3환자(65세)유경도경동맥내중막증후.3례환자균무신소구신염,균미합병기타자신면역질병,균무당뇨병미혈관병발증.채용이필격렬동위기출적약물치료,혈당공제기본만의,감유삼지가기본공제우정상범위,단환자상반신지방위축정황무명현개선.결론 APL환자통과이필격렬동위기출적치료,가이획득기본량호적대사공제,단시지방위축정황무명현개선.
Objective To investigate the clinical characteristics,cause of disease and response to treatment on acquired partial lipodystrophy(APL).Methods From 1991 to 2012,3 Chinese APL patients were studied.Results 3 patients of APL were all female,onset of disease were all around 10 years old,and the age of diagnosis were 20-62 years old.They were followed 2-12 years and now they are 25-65 years old.The subcutaneous adipose tissue was significantly decreased in upper body and was not affected or increased in the lower body in all 3 patients.Diabetes was diagnosed in all 3 patients and they were all with hyperinsulinemia,hypertriglyceridemia and fatty liver.Complement C3 were lower than lower limit of normal in 2 patients.Two patients had normal menstruation,one patient had normal pregnancy.The 3 patients were treated on the basis of pioglitazone,blood glucose was well controlled and serum triglyceride decreased to normal,but the severely decreased subcutaneous adipose tissue in upper body didn't recover.Conclusion With treatment on the basis of pioglitazone,3 APL patients can be well controlled metabolically,but the severely decreased subcutaneous adipose tissue in upper body couldn't recover.