TUBEROUS SCLEROSIS COMPLEX- RADIOLOGICAL FEATURES IN DIFFERENT BODY SYSTEMS

Veronika Apostoloska, Elizabeta Stojovska Jovanovska, Maja Jakimovska Dimitrovska, Smiljana Bundovska Kocev, Sofija Crcevska, Nacko Stavreski
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Abstract

Tuberous sclerosis (TS) is a rare genetic disorder of autosomal - dominant inheritance. Tuberous sclerosis complex is characterized by the development of benign tumors affecting different body systems who results in hamartomas involving many organs, like the brain, heart, kidneys, skin, lungs and liver. The management of these patients is often multidisciplinary, involving specialists from various fields. TSC presents at any age as a wide range of clinical and phenotypic manifestations with varying severity. The most common manifestations are facial angiofibroma, seizures, cortical tubers, cardiac rhabdomyoma, renal cysts and tumor of the kidneys. We present four patients with characteristic clinical and radiological features of multilocular tuberous sclerosis. Keywords: Tuberous sclerosis complex (TSC), tuberous sclerosis (TS), cortical tubers, angiofibromas, renal angiomyolipomas (AML).    
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结节性硬化症复合体-不同身体系统的影像学特征
摘要结节性硬化症(TS)是一种罕见的常染色体显性遗传疾病。结节性硬化症的特点是发展成影响不同身体系统的良性肿瘤,导致错构瘤累及许多器官,如大脑、心脏、肾脏、皮肤、肺和肝脏。这些患者的管理通常是多学科的,涉及来自不同领域的专家。TSC在任何年龄表现为广泛的临床和表型表现,严重程度不同。最常见的表现是面部血管纤维瘤、癫痫、皮质结节、心脏横纹肌瘤、肾囊肿和肾脏肿瘤。我们报告了四例多房结节性硬化症的临床和影像学特征。关键词:结节性硬化症(TSC),结节性硬化症(TS),皮质结节,血管纤维瘤,肾血管平滑肌脂肪瘤(AML)。,今天早上
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