Clinical Manifestations and Imaging Diagnostic Value of Tuberous Sclerosis Accompanied by Multiple Organs Injury
- VernacularTitle:伴多脏器损害的结节性硬化症临床表现与影像学诊断价值
- Author:
Jianguo YIN
;
Xiaohong LIU
;
Jijun NING
;
Yan YANG
;
Xiong JIA
- Publication Type:Journal Article
- Keywords:
tuberous sclerosis;
angiofibroma;
harmatoma
- From:
Journal of Practical Radiology
1996;0(04):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To discuss the clinical manifestations and imaging diagnostic value of tuberous sclerosis accompanied by multi-organ injuries.Methods 2 cases in our hospital and 27 cases of tuberous sclerosis from the published reports were collected.The clinical manifestations and imaging features as well as the extent and type of multi-organ lesion were analyzed.Results Among the 29 patients,17 were male and 12 were female.The averaged age was 16.8.The clinical manifestations included:with family history in 6 , epilepsies in 25 (15 with mental retardation),symmetric angiofibroma in the face in 22 (3 with similar changes in the else body surface),fibromas beneath toenail or fingernail in 4, hyperdactylia of thumb in 2 and abdominal masses in 7 (4 with hematuria), tuberous scleroses beneath ependymal layer in 27,17 patients with kidney diseases,9 involved liver,5 involved spleen.Conclusion Multiple organs can be involved by tuberous sclerosis.The calcification of ependymal lyer and harmatoma of kidney are the most valuable in imaging diagnosis of the disease.
