Davis R E
Family Nurse Practitioner Program, Millersville University, Pa., USA.
Nurse Pract. 1997 Apr;22(4):73-6, 79-81.
Neurofibromatosis is the single most common genetic disease of the neurologic system. Because neurofibromatosis type 1 (NF-1) is often diagnosed early in life, it is essential that health care providers deepen their understanding of this common genetic disorder. Children who meet particular criteria, as formulated by the National Institutes of Health, must be worked-up for NF-1 in order to initiate appropriate treatment implementation and evaluation. This article provides a review of the literature regarding the pathophysiology, clinical manifestations, and plan of treatment related to NF-1, specifically as it affects children. Clear guidelines for health care providers in primary care are outlined.
神经纤维瘤病是神经系统最常见的单基因遗传病。由于1型神经纤维瘤病(NF-1)常在生命早期被诊断出来,因此医疗保健提供者加深对这种常见遗传病的理解至关重要。符合美国国立卫生研究院制定的特定标准的儿童,必须接受NF-1的检查,以便开始适当的治疗实施和评估。本文综述了有关NF-1的病理生理学、临床表现及治疗方案的文献,特别是其对儿童的影响。文中概述了初级保健中医疗保健提供者的明确指南。