PMID: 9424398Sep 1, 1996Paper

When to suspect and how to diagnose pulmonary lymphangioleiomyomatosis

Respirology : Official Journal of the Asian Pacific Society of Respirology
A NaalsundA Kolbenstvedt

Abstract

The objective of this study was to present clinical and radiological data of eight women with histologically proven lymphangioleiomyomatosis (LAM) diagnosed between 1984 and 1994, and to suggest a diagnostic strategy when LAM is suspected. A review of case reports, including results of biopsies, lung function and radiological procedures was undertaken. The mean age of the women at start of symptoms was 36 years, and the mean age at time of diagnosis 42 years. The most frequent presenting complaint was dyspnea, either in conjunction with pneumothorax (3), chylothorax (2) or on exertion (2). All patients had airflow limitation and markedly reduced gas transfer. Five patients had 16 episodes of pneumothorax. In seven patients multiple cysts were observed on the surface of the lung during thoracotomy while computerized tomography (CT) scans revealed numerous cysts evenly distributed throughout the lung parenchyma. The procedures that confirmed the diagnosis included transbronchial lung biopsy (4), open lung biopsy (2), thoracoscopy (1), thoracotomy (3) and autopsy (1). Three specimens had to be revised before the histological diagnosis was confirmed. It was concluded that the important clues to a diagnosis of LAM are recurrent epis...Continue Reading

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Citations

Dec 12, 2012·Frontiers of Medicine·Riffat MerajFrancis X McCormack
Apr 20, 2002·Transplantation·Giancarlo RoviaroMarco Maciocco
Sep 24, 2011·American Journal of Respiratory Cell and Molecular Biology·Daniel F DillingI Caroline Le Poole
Nov 7, 2009·The American Journal of Pathology·Michele Carbone
May 23, 2007·Archives of Pathology & Laboratory Medicine·Kevin O LeslieMary Beth Scholand

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