von Recklinghausen神经纤维瘤病与Albright综合征的诊断。两例报告。

Neurofibromatosis Pub Date : 1988-01-01
L Bachmann Andersen, S A Sørensen
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引用次数: 0

摘要

我们描述了两名女性,她们在童年早期被诊断为患有奥尔布赖特综合征。两人都有近亲患有神经纤维瘤病;已经确定,两人中的一个实际上患有冯雷克林豪森神经纤维瘤病,而另一个是否也患有这种疾病仍不确定。除了卡萨梅-奥-莱色素沉着的模式外,这两名患者还有一些共同的症状:反复和自发性骨折导致假关节、额部隆起和脊柱后凸。区分这两种疾病对遗传咨询很重要,因为神经纤维瘤病与奥尔布赖特综合征不同,是可遗传的。
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Diagnosis of von Recklinghausen neurofibromatosis and the Albright syndrome. Two case reports.

We describe two females who were diagnosed as suffering from the Albright syndrome early in childhood. Both have close relatives with neurofibromatosis; it has been established that one of the two, in fact, has von Recklinghausen neurofibromatosis, while it remains uncertain if the other one also has this disorder. Apart from the pattern of café-au-lait hyperpigmentation, these two patients have several symptoms in common: repeated and spontaneous bone fractures leading to pseudarthrosis, frontal bossing, and kyphoscoliosis. Distinguishing between the two diseases is important for genetic counseling since neurofibromatosis, in contrast to the Albright syndrome, is heritable.

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