Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Hecimovic, A; Jakopovic, M; Pavlisa, G; Jankovic, M; Vukic-Dugac, A; Redzepi, G; Brcic, L; Samarzija, M; Gupta, N.
SUCCESSFUL TREATMENT OF PULMONARY AND LYMPHATIC MANIFESTATIONS OF LYMPHANGIOLEIOMYOMATOSIS WITH SIROLIMUS.
Lymphology. 2015; 48(2):97-102
Web of Science
PubMed
- Co-Autor*innen der Med Uni Graz
- Brcic Luka
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- Abstract:
- Lymphangioleiomyomatosis (LAM) is a rare, progressive, diffuse cystic lung disease predominantly affecting women of child bearing age. Recently treatment with sirolimus was shown to stabilize lung function decline and improve quality of life in patients with LAM. We treated three premenopausal women suffering from LAM manifesting as diffuse cystic lung disease, chylous effusions, and lymphangioleioyomas with sirolimus (1-3 mg a day; sirolimus trough levels 2.9-8.5 ng/ml). All three patients had a remarkable response to sirolimus, with resolution of effusions, improvement in lung function and shrinking of abdominal lymphangioleiomyomas. Our case series further complements the literature in that sirolimus is a safe and effective treatment for LAM and its lymphatic manifestations.
- Find related publications in this database (using NLM MeSH Indexing)
- Adult -
- Antineoplastic Agents - therapeutic use
- Biomarkers, Tumor - analysis
- Biopsy -
- Female -
- Humans -
- Immunohistochemistry -
- Lung Neoplasms - chemistry
- Lung Neoplasms - diagnosis
- Lung Neoplasms - drug therapy
- Lymphangioleiomyomatosis - diagnosis
- Lymphangioleiomyomatosis - drug therapy
- Lymphangioleiomyomatosis - metabolism
- Respiratory Function Tests -
- Retroperitoneal Neoplasms - chemistry
- Retroperitoneal Neoplasms - diagnosis
- Retroperitoneal Neoplasms - drug therapy
- Sirolimus - therapeutic use
- Tomography, X-Ray Computed -
- Treatment Outcome -
- Find related publications in this database (Keywords)
- lymphangioleimyomatosis
- lymphangioleiomyoma
- chylous ascites
- cylothorax
- pleural effusion
- sirolimus
- MILES trial