Bernstein J
Research Institute, William Beaumont Hospital, Royal Oak, Michigan 48073.
Pediatr Nephrol. 1993 Aug;7(4):490-5. doi: 10.1007/BF00857581.
Renal cysts are relatively common in tuberous sclerosis, occasionally causing severe cystic disease with renal failure. Although the imaging features and gross appearances resemble dominant polycystic kidney disease, the histopathological appearances are distinctive, perhaps unique, differing from appearances in other forms of renal cystic disease. The cysts are lined with hypertrophic and hyperplastic cells that probably cause the cysts by obstructing lumina. The renal abnormality may be inherent in tuberous sclerosis, although inconstantly expressed. Renal enlargement is sometimes the first recognized expression of tuberous sclerosis, and the occurrence of renal cysts in a child with seizures and developmental retardation leads to a strong suspicion of the diagnosis.
肾囊肿在结节性硬化症中相对常见，偶尔会导致严重的囊性疾病并伴有肾衰竭。尽管其影像学特征和大体表现类似于成人多囊肾病，但组织病理学表现却截然不同，或许是独一无二的，与其他形式的肾囊性疾病的表现有所不同。囊肿内衬有肥大和增生的细胞，这些细胞可能通过阻塞管腔而导致囊肿形成。肾脏异常可能是结节性硬化症所固有的，尽管其表达并不恒定。肾脏增大有时是结节性硬化症最早被认识到的表现，而在患有癫痫和发育迟缓的儿童中出现肾囊肿会强烈提示该诊断。
