These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.


Pubmed for Handhelds

PUBMED FOR HANDHELDS

Search MEDLINE/PubMed

  • Title: [Sporadic lymphangioleiomyomatosis. Clinical and lung functional characteristics of 32 female patients].
    Author: Watz H, Oltmanns A, Kimmel S, Magnussen H, Wirtz H, Kirsten D.
    Journal: Dtsch Med Wochenschr; 2008 Apr; 133(14):705-8. PubMed ID: 18363188.
    Abstract:
    BACKGROUND AND OBJECTIVE: Lymphangioleiomyomatosis (LAM) is a rare and progressive multi-systemic disorder almost exclusively of women. Clinical and lung functional data of a substantial number of German patients have not so far been published. PATIENTS AND METHODS: In 32 women with sporadic LAM we performed the following investigations: medical history, clinical examination, lung function (spirometry, bodyplethysmography, diffusion capacity for carbon monoxide), ECG, echocardiography, and abdominal ultrasound. RESULTS: The mean age at the time of investigation was 48 (25 - 66) years, while the mean age at time of diagnosis had been 45 (21 - 61) years. The median time between onset of first symptoms and diagnosis was 8 years (1 months - 30 years). Most frequently stated first symptom was dyspnea on exertion (12/32 patients) and the most frequently stated first clinical sign was pneumothorax (12/32). Changes of hormonal status (contraception, treatment for fertility, pregnancy) occurred in 31 of the 32 patients before onset of first symptoms or clinical signs. Hormonal (16/32) and antiobstructive therapy (16/32) were the most frequently applied forms of treatment. Airway obstruction (26/32 patients), hyperinflation (20/32) and reduced diffusion capacity for carbon monoxide (24/32) were found on lung function test. Abnormal auscultation (6 patients), ECG signs of chronic cor pulmonale (4/32) and an elevated systolic pulmonary artery pressure (2 patients) were rare findings. Abdominal lesions (angiomyolipoma, 11/32 patients; lymphangioleiomyoma, 4 patients) were common. CONCLUSION: Lymphangioleiomyomatosis should be considered in women with spontaneous pneumothorax, unexplainable dyspnoea or angiomyolipoma.
    [Abstract] [Full Text] [Related] [New Search]