Sporadic lymphangioleiomyomatosis (Q42951): Difference between revisions
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Doença pulmonar rara que resulta na proliferação de crescimento desordenado de músculo liso (leiomioma) nos pulmões inteiros, nos bronquíolos, septos alveolares, espaços perivasculares e linfáticos, levando à obstrução de pequenas vias aéreas (com formação de cistos e de pneumotórax) e dos linfáticos (levando a derrame pleural quiloso). A LAM ocorre de forma esporádica, afeta apenas mulheres, geralmente em idade reprodutiva; LAM ocorre também em pacientes que têm esclerose tuberosa. | |||
| description / en | description / en | ||
This is a rare lung disease that results in a proliferation of disorderly smooth muscle growth (leiomyoma) throughout the lungs, in the bronchioles, alveolar septa, perivascular spaces, and lymphatics, resulting in the obstruction of small airways (leading to pulmonary cyst formation and pneumothorax) and lymphatics (leading to chylous pleural effusion). LAM occurs in a sporadic form, which affects only females, usually of childbearing age; LAM also occurs in patients who have tuberous sclerosis. | |||
Revision as of 09:43, 13 August 2026
This is a rare lung disease that results in a proliferation of disorderly smooth muscle growth (leiomyoma) throughout the lungs, in the bronchioles, alveolar septa, perivascular spaces, and lymphatics, resulting in the obstruction of small airways (leading to pulmonary cyst formation and pneumothorax) and lymphatics (leading to chylous pleural effusion). LAM occurs in a sporadic form, which affects only females, usually of childbearing age; LAM also occurs in patients who have tuberous sclerosis.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | CB07.1 |
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| English | Sporadic lymphangioleiomyomatosis |
This is a rare lung disease that results in a proliferation of disorderly smooth muscle growth (leiomyoma) throughout the lungs, in the bronchioles, alveolar septa, perivascular spaces, and lymphatics, resulting in the obstruction of small airways (leading to pulmonary cyst formation and pneumothorax) and lymphatics (leading to chylous pleural effusion). LAM occurs in a sporadic form, which affects only females, usually of childbearing age; LAM also occurs in patients who have tuberous sclerosis. |
