PDF(Pubmed)
Abstract：
Lymphangioleiomyomatosis(LAM) is a slow progressive, rare cystic lung disease in women of reproductive age, associated with infiltration of the lung by atypical smooth muscle like cells, leading to the cystic destruction of the lung parenchyma. As LAM exclusively affects women of childbearing age, it can arise or exacerbate during pregnancy. Many patients with LAM are discouraged from pregnancy, although there is not much objective evidence effect on fertility. Patients diagnosed with LAM during pregnancy experience worse outcomes, so the safety of pregnancy is a vexing problem. What was worse, treatment strategies are limited on the effects of LAM on pregnancy outcomes. Pregnancy could be considered in LAM patients. Successful delivery in women with LAM depends on the condition of the LAM, which is in turn dependent on obstetricians and respiratory physicians. In this review, we describe the epidemiology, pathogenesis, diagnosis, clinical features and the treatment strategies of LAM during pregnancy.
摘要：
淋巴管平滑肌瘤病（LAM）是一种缓慢进展的，育龄妇女罕见的囊性肺病，与非典型平滑肌样细胞浸润肺有关，导致肺实质的囊性破坏。由于LAM只影响育龄妇女，它可以在怀孕期间出现或加剧。许多LAM患者不鼓励怀孕，虽然对生育影响的客观证据不多。怀孕期间被诊断为LAM的患者的结局较差，所以怀孕的安全是一个令人烦恼的问题。更糟糕的是，治疗策略仅限于LAM对妊娠结局的影响.LAM患者可以考虑怀孕。LAM妇女的成功分娩取决于LAM的状况，这又取决于产科医生和呼吸医生。在这次审查中,我们描述了流行病学，发病机制，诊断,妊娠LAM的临床特点及治疗策略。
