抽象的
肺淋巴管霉素症(LAM)是一种影响女性的罕见疾病,可以导致严重的呼吸损害。由于首次报道了Bourneville的肺结核硬化(TS),因此两个实体之间的惊人相似之处使许多人相信LAM是TS的Forme Fruste。这提出了报道,即血管肌脂肪碱本身,稀有肿瘤的稀有肿瘤在40-80%的TS患者中报告,并在15-30%的林患者中发生。回顾性图表审查由14名患者进行,该患者介绍了我们的机构,其诊断为LAM。我们试图记录临床表现,特别是脱果肿瘤的发病率和位置,以进一步支持林是TS的Forme Fruste的假设。12名患者患有前辈症状和两种绝经后症。确诊在组织学上(n = 12)和/或通过胸部的计算断层扫描(CT)扫描确认(n = 12)。成像调查显示14名患者中的12例(86%)中的脱毛肿瘤。14名患者中的八个(57%)患有肾肿瘤,与血管植物(五个患者中的双侧)一致。只有一名患者患有肾脏症状(侧翼疼痛和血尿病)。 All had normal serum creatinine, one had a reduced creatinine clearance. Extrathoracic nonrenal tumours were discovered in the pancreas, adrenals and uterus, findings previously unreported in LAM. In summary, the incidence of extrathoracic tumours in lymphangioleiomyomatosis patients is much higher than previously reported in the literature. This increased association supports the theory that lymphangioleiomyomatosis and tuberous sclerosis represent part of a spectrum of a similar disease process.