Lymphangioleiomyomatosis associated with tuberous sclerosis complex (Q42953): 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, o que resulta na obstrução das pequenas vias aéreas (levando à formação de cistos pulmonares e pneumotórax) e dos linfáticos (levando a derrame pleural quiloso). Este diagnóstico está associado a uma doença multissistêmica genética rara que causa o crescimento de tumores não malignos no cérebro e em outros órgãos vitais como rins, coração, olhos, pulmões e pele. | |||
| 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). This diagnosis is associated with a rare multi-system genetic disease that causes non-malignant tumours to grow in the brain and on other vital organs such as the kidneys, heart, eyes, lungs, and skin. | |||
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). This diagnosis is associated with a rare multi-system genetic disease that causes non-malignant tumours to grow in the brain and on other vital organs such as the kidneys, heart, eyes, lungs, and skin.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | CB07.0 |
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| English | Lymphangioleiomyomatosis associated with tuberous sclerosis complex |
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). This diagnosis is associated with a rare multi-system genetic disease that causes non-malignant tumours to grow in the brain and on other vital organs such as the kidneys, heart, eyes, lungs, and skin. |
