Family and Community Medicine, University of Maryland School of Medicine, Baltimore, Maryland, USA
University of Maryland Medical Center, Baltimore, Maryland, USA.
BMJ Case Rep. 2024 Sep 19;17(9):e260369. doi: 10.1136/bcr-2024-260369.
A female of reproductive age presents to the emergency department with progressive dyspnoea due to pneumothorax. She has a history of lymphangioleiomyomatosis (LAM) diagnosed by lung biopsy 15 years ago following incidental finding of pneumothorax. Despite various procedural and medicinal treatments, she continued to have recurrent pneumothorax, with three hospital admissions over the preceding 3 months. LAM is a rare cystic lung disease affecting the lymphatic system, which most commonly affects women of childbearing age. It can be diagnosed via imaging or tissue biopsy (gold standard). Treatment can be difficult, and it often requires highly specialised care by pulmonologists and often confers significant limitations to patients' independence and quality of life. Family physicians are often part of multidisciplinary team to provide care to patients with rare chronic conditions.
一位育龄期女性因气胸导致进行性呼吸困难而到急诊科就诊。她 15 年前因气胸偶然发现后经肺活检确诊为淋巴管平滑肌瘤病（LAM）。尽管进行了各种程序和药物治疗，但她仍反复发生气胸，在过去 3 个月内有 3 次住院治疗。LAM 是一种罕见的囊性肺疾病，影响淋巴系统，最常影响育龄妇女。它可以通过影像学或组织活检（金标准）进行诊断。治疗可能很困难，通常需要肺病专家的高度专业化护理，并经常对患者的独立性和生活质量造成重大限制。家庭医生通常是多学科团队的一部分，为患有罕见慢性疾病的患者提供护理。
