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神经纤维瘤有明显的遗传因素,为神经外胚层发育异常所致,每3000次生产中约有1例得此病,男稍多于女。其特征性损害包括:皮肤咖啡色斑和沿周围神经分布的神经纤维瘤。神经纤维瘤常为孤立的或呈丛状,少数转变成肉瘤。神经纤维瘤病可伴有其他异常,包括早熟,继发于肾血管损害的高血压病或嗜铬细胞瘤和骨骼损害,如骨质侵蚀性改变,脊柱侧凸,骨骼过度生长,脊椎裂和颅裂等,神经纤维瘤病时胃肠道肿瘤较少引起注意。本文作者报告了7例神经纤维瘤病伴发胃肠道损
Neurofibromatosis has a distinct genetic factor, which is caused by abnormal development of neuroectodermal cells. About one out of every 3,000 cases of the disease is caused by the disease, with slightly more men than women. The characteristic lesions include skin macules and neurofibromas distributed along the peripheral nerves. Neurofibromas are often isolated or plexiform and a few are turned into sarcomas. Neurofibromatosis can be accompanied by other abnormalities including precocious puberty, hypertension secondary to renal vascular damage, or pheochromocytoma and skeletal lesions such as osteolytic changes, scoliosis, excessive bone growth, and spina bifida. Craniotomy, etc., gastrointestinal tumors are less noticeable in neurofibromatosis. The authors report 7 cases of neurofibromatosis with gastrointestinal lesions