Department of Medicine, University of California-San Diego and Veterans' Affairs San Diego Healthcare System, CA, USA.
Acta Pharmacol Sin. 2012 Feb;33(2):155-72. doi: 10.1038/aps.2011.153.
Rare adipose disorders (RADs) including multiple symmetric lipomatosis (MSL), lipedema and Dercum's disease (DD) may be misdiagnosed as obesity. Lifestyle changes, such as reduced caloric intake and increased physical activity are standard care for obesity. Although lifestyle changes and bariatric surgery work effectively for the obesity component of RADs, these treatments do not routinely reduce the abnormal subcutaneous adipose tissue (SAT) of RADs. RAD SAT likely results from the growth of a brown stem cell population with secondary lymphatic dysfunction in MSL, or by primary vascular and lymphatic dysfunction in lipedema and DD. People with RADs do not lose SAT from caloric limitation and increased energy expenditure alone. In order to improve recognition of RADs apart from obesity, the diagnostic criteria, histology and pathophysiology of RADs are presented and contrasted to familial partial lipodystrophies, acquired partial lipodystrophies and obesity with which they may be confused. Treatment recommendations focus on evidence-based data and include lymphatic decongestive therapy, medications and supplements that support loss of RAD SAT. Associated RAD conditions including depression, anxiety and pain will improve as healthcare providers learn to identify and adopt alternative treatment regimens for the abnormal SAT component of RADs. Effective dietary and exercise regimens are needed in RAD populations to improve quality of life and construct advanced treatment regimens for future generations.
罕见脂肪组织疾病(RADs),包括多发性对称性脂肪瘤病(MSL)、脂肪水肿和德库姆病(DD),可能被误诊为肥胖症。生活方式的改变,如减少热量摄入和增加身体活动,是肥胖症的标准治疗方法。虽然生活方式的改变和减重手术对 RADs 中的肥胖成分有效,但这些治疗方法并不能常规减少 RADs 的异常皮下脂肪组织(SAT)。RAD SAT 可能是由于 MSL 中棕色干细胞群体的生长和继发性淋巴功能障碍,或者由于脂肪水肿和 DD 中的原发性血管和淋巴功能障碍导致的。RAD 患者不能仅通过热量限制和增加能量消耗来减少 SAT。为了提高对 RADs 与肥胖症的区分,本文提出了 RADs 的诊断标准、组织病理学和病理生理学,并与家族性部分脂肪营养不良、获得性部分脂肪营养不良和肥胖症进行了对比,这些疾病可能与 RADs 混淆。治疗建议侧重于循证数据,包括淋巴引流治疗、支持 RAD SAT 减少的药物和补充剂。随着医疗保健提供者学会识别和采用 RADs SAT 异常的替代治疗方案,相关的 RAD 疾病,包括抑郁、焦虑和疼痛,将得到改善。RAD 人群需要有效的饮食和运动方案,以提高生活质量,并为后代制定先进的治疗方案。