Diagnosis and treatment for 2 cases of tuberous sclerosis complex with pulmonary lymphangioleiomyomatosis / 中南大学学报(医学版)
Journal of Central South University(Medical Sciences)
; (12): 771-776, 2016.
Article
in Chinese
| WPRIM (Western Pacific)
| ID: wpr-814965
Responsible library:
WPRO
ABSTRACT
To improve the diagnosis and treatment for tuberous sclerosis complex (TSC) with pulmonary lymphangioleiomyomatosis, a retrospective analysis was performed based on the clinical data of 2 patients with such disease. Both of them have typical thin-walled cystic lesion throughout the lung field, renal angioleiomyolipoma, and various degrees of skin lesions. Central nervous system is involved in one patient. Lesions in the lung and kidney in one patient were improved significantly after 5 months of rapamycin treatment. The clinical phenotypes were diverse in TSC patients. The CT imaging showed typical characteristics when the lung was invaded by the tumor. When a patient was diagnosed as pulmonary lymphangioleiomyomatosis, we should pay attention to the clinical screening of TSC. Rapamycin is an effective and safe treatment for this disease.
Full text:
Available
Database:
WPRIM (Western Pacific)
Main subject:
Tuberous Sclerosis
/
Retrospective Studies
/
Lymphangioleiomyomatosis
/
Kidney
/
Lung
/
Lung Neoplasms
Type of study:
Diagnostic study
/
Observational study
Limits:
Humans
Language:
Chinese
Journal:
Journal of Central South University(Medical Sciences)
Year:
2016
Document type:
Article