结节性硬化症中肝错构瘤的发病率。
Incidence of hepatic hamartomas in tuberous sclerosis.
作者信息
Jóźwiak S, Pedich M, Rajszys P, Michalowicz R
机构信息
Department of Child Neurology, Child's Health Centre, Warsaw, Poland.
出版信息
Arch Dis Child. 1992 Nov;67(11):1363-5. doi: 10.1136/adc.67.11.1363.
Hepatic hamartomas were thought to be a rare finding in patients with tuberous sclerosis. The purpose of this study was to assess their incidence in children with tuberous sclerosis and to review the literature. During 1984-90 we examined 51 children by ultrasonography; there were 25 boys and 26 girls. Their age ranged from 3 months to 18 years. Liver hamartomas were seen in 12 (23.5%) of the children, more often in girls than boys (5:1). Their incidence increased with age reaching 45% in children over the age of 10. They did not produce any symptoms of hepatic dysfunction. Our study and review of reported cases prove that hepatic hamartomas are a common finding in patients with tuberous sclerosis and may be very helpful in providing a more accurate diagnosis and consequently help in genetic counselling.
肝错构瘤曾被认为在结节性硬化症患者中是一种罕见的发现。本研究的目的是评估其在结节性硬化症儿童中的发病率并复习相关文献。在1984年至1990年期间，我们对51名儿童进行了超声检查；其中有25名男孩和26名女孩。他们的年龄从3个月到18岁不等。12名（23.5%）儿童发现有肝错构瘤，女孩比男孩更常见（比例为5:1）。其发病率随年龄增长而增加，10岁以上儿童中发病率达45%。它们未产生任何肝功能障碍症状。我们的研究以及对报告病例的复习证明，肝错构瘤在结节性硬化症患者中是一种常见发现，可能对提供更准确的诊断非常有帮助，从而有助于进行遗传咨询。
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