폐림프관평활근종증 환자에서의 임신 1예
A Case of Pregnancy in Patient with Pulmonary Lymphangioleiomyomatosis
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초록
폐림프관평활근종증은 원인 불명의 매우 드문 질환으로, 평활근의 과증식을 특징으로 하며, 주로 가임기 여성에서 발생한다. 이 질환은 호흡곤란, 반복적 기흉, 유미흉, 객혈을 일으키며, 궁극적으로 호흡 부전을 초래하여 사망에 이르게 된다. 흉부촬영상 미만성의 간질 침윤 및 낭종형성을 특징으로 하며, 폐기능 검사상 잔기량의 증가와 함께 기도제한 소견을 보인다. 주로 폐경전 여성에서 나타나는 것으로 보아 호르몬적 요소가 중요한 역할을 한다고 생각되지만, 아직까지 명확한 치료는 없는 상태이다. 저자들은 폐림프관평활근종증을 진단받은 환자에서의 정상 임신을 경험하였기에, 여러 문헌 고찰과 함께 보고하는 바이다.
Abstract
Pulmonary lymphangioleiomyomatosis is a rare disorder of unknown cause and characterised by hamartomatous proliferation of smooth muscle occurring in women of reproductive age exclusively. It causes dyspnea, recurrent pneumothorax, chylothorax, hemoptysis and respiratory failure eventually. Chest radiographs show diffuse interstitial infiltrates and cysts of uniform size, and pulmonary function tests often show airflow limitation with increase in residual volume. Hormonal factors are thought to play a role because it generally affects premenopausal women, but there is no definite treatment yet. We present an unusual case of pulmonary lymphangioleiomyomatosis during normal pregnancy with a review of literature.
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