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Zhonghua Jie He He Hu Xi Za Zhi. 2000 Jun;23(6):355-7.

[Pulmonary lymphangioleiomyomatosis].

[Article in Chinese]

Author information

1
Pulmonary Department, Zhongshan Hospital, Shanghai Medical University, Shanghai 200032, China.

Abstract

OBJECTIVE:

To improve the diagnosis and treatment of pulmonary lymphangioleiomyomatosis (PLAM).

METHODS:

Three patients with PLAM confirmed by pathological assessment were presented and relevant literatures were reviewed.

RESULTS:

PLAM is a rare pulmonary disease of unknown cause. The clinical manifestations were pneumothorax, exertional dyspnea and hemoptysis. Pulmonary function test showed obstructive or compound ventilative defect and hypoxemia. HRCT showed bilateral diffuse cystic airspaces change. Pathological features showed abnormal smooth muscle proliferation occurred along lymphatics. Lymphatics dilated and proliferated.

CONCLUSIONS:

The prognosis of PLAM is poor. There is no effective method for the treatment of this disease at present.

PMID:
11778518
[Indexed for MEDLINE]

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