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马凡氏综合症(Marfan Syndrome) ,又名蜘蛛脚样指(Arachnodactyly) 。是一种先天性显性优势遗传性疾病。其主要症状为:1.全身长管骨过长,尤其是手指(趾) 细长,状如蜘蛛脚样。2.眼部病变,以晶状体半脱位最常见(约占80％) ,此外尚可能合并有轴性高度近视、斜视、大角膜、球状晶体,黄斑缺损及视网膜脱离等。3.约有40-60％的患者合并有先天性心血管系统疾患。马凡氏(1896年) 首先报道一例,以后逐有较多的病例的发现,文献报道有一次多达400例者。本病在国内报道较少,张氏(1951) 首先报道二例,以后也有少数病例报道。我院于1974年以来发现两例,兹将其中病历较完整的一例报道如下: 患儿郑××,男,8岁。于1976年1月16日因近来视力下降来院就诊。父母发现:患儿在出生2～3岁后手指(趾) 逐渐细长显目。4～5岁时视远物不清。全身消瘦,但食欲良
Marfan Syndrome, also known as Arachnodactyly. Is a congenital dominant dominant genetic disease. The main symptoms are: 1. The body long bones are too long, especially the fingers (toes) slender, shaped like a spider feet. 2. Ocular lesions, the most common subluxation of the lens (about 80%), in addition may still be combined with axial high myopia, strabismus, the cornea, spherulites, macular defects and retinal detachment. 3. About 40-60% of patients with congenital cardiovascular system disorders. Marfan (1896) first reported an example, after more cases were found, reported in the literature as many as 400 cases. Less reported in the country, Zhang (1951) first reported two cases, but also a small number of cases reported. Two cases were found in our hospital since 1974, and one of the more complete cases of medical records is hereby reported as follows: The children Zheng X, male, 8 years old. On January 16, 1976 due to the recent decline in vision to hospital. Parents found: children born after the age of 2 to 3 fingers (toe) gradually slender conspicuous. 4 to 5 years old as distant objects unclear. Whole body weight loss, but good appetite