Study progress of treatment for children with tuberous sclerosis complex
10.3760/cma.j.issn.2095-428X.2014.09.018
- VernacularTitle:小儿结节性硬化症治疗进展
- Author:
Fuyong JIAO
;
Li WEI
;
Jiemin WANG
;
Yadong WANG
- Publication Type:Journal Article
- Keywords:
Tuberous sclerosis complex;
Treatment;
Progress;
Child
- From:
Chinese Journal of Applied Clinical Pediatrics
2014;29(9):710-712
- CountryChina
- Language:Chinese
-
Abstract:
The tuberous sclerosis complex (TSC) in children is also named Bourneville disease,the 3 main symptoms namely epilepsy,mental retardation and facial angiofibromas.It is an autosomal dominant disease.It is an important cause of epilepsy,skin disease,and renal and pulmonary disease in children and adults.The appropriate therapy and prognosis for TSC patients are often different than that for individuals with epilepsy,renal tumors,or interstitial lung disease from other causes.In recent years,certain progress has been made in management of tuberous sclerosis,inhibitors of the mammalian target of rapamycin (mTOR) have demonstrated regression of astrocytomas,angiofibromas,and angiomyoliomas,as well as improved pulmonary function in persons with TSC.This article reviews the current therapeutic recommendations for medical and surgical management of neurologic,renal,and pulmonary manifestations of TSC.
