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儿童神经纤维瘤病

Neurofibromatosis in children.

作者信息

Crawford A H

出版信息

Am Fam Physician. 1978 Mar;17(3):163-70.

PMID:415586
Abstract

Neurofibromatosis occurs once in every 3,000 live births. Café au lait spots are the most common presenting lesion. Five spots with a diameter of at least 0.5 cm. should be considered diagnostic in children. Spinal deformity is the most common bony lesion. Scoliosis varies from mild nonprogressive forms to hairpin curvatures. Four types of pseudarthrosis of the tibia can be distinguished, with progressively more serious prognoses. The incidence of neoplasia greatly exceeds that of the general population.

摘要

神经纤维瘤病在每3000例活产中出现1例。牛奶咖啡斑是最常见的表现性损害。儿童中,直径至少0.5厘米的5个斑应被视为具有诊断意义。脊柱畸形是最常见的骨质损害。脊柱侧弯从轻度非进行性形式到发夹样弯曲不等。胫骨假关节可分为四种类型,预后逐渐加重。肿瘤形成的发生率大大超过一般人群。

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