Grover R, Wethers D, Shahidi S, Grossi M, Goldberg D, Davidow B
Pediatrics. 1978 May;61(5):740-9.
Since September 1974, New York State public health law has mandated that all newborn infants be tested for phenylketonuria, maple syrup urine disease, homocystinuria, histidinemia, galactosemia, adenosine deaminase deficiency, and sickle cell anemia in accordance with regulations of the state commissioner of health. During the period from May 1, 1975, to April 30, 1976, a total of 110,180 babies born in New York City were tested for these seven conditions. One year's experience with the screening program demonstrated a paucity of technological problems, low observed rate of both false-negatives and -positives, and the expected incidence of the conditions of highest prevalence, incidentally found during screening: i.e., sickle cell traits, AS and AC. What is equally apparent in reviewing this first year's experience is the extent to which the New York State law, its structure, and implementation have fallen short of the ultimate objective. The major reason for this failure is lack of funds and facilities in the areas of education, case retrieval, continuing medical care, and counseling. This report is presented with the hope that it will benefit all involved in genetic screening and especially those concerned with establishing similar programs.
自1974年9月起,纽约州公共卫生法规定,所有新生儿都必须按照州卫生专员的规定,接受苯丙酮尿症、枫糖尿症、高胱氨酸尿症、组氨酸血症、半乳糖血症、腺苷脱氨酶缺乏症和镰状细胞贫血的检测。在1975年5月1日至1976年4月30日期间,纽约市共有110,180名新生儿接受了这七种疾病的检测。筛查项目一年的经验表明,技术问题较少,假阴性和假阳性的发生率较低,并且在筛查过程中偶然发现了预期患病率最高的疾病的发病率,即镰状细胞性状,AS和AC。在回顾这第一年的经验时同样明显的是,纽约州法律及其结构和实施在多大程度上未达到最终目标。失败的主要原因是教育、病例检索、持续医疗护理和咨询领域缺乏资金和设施。本报告的提出希望能使所有参与基因筛查的人受益,尤其是那些关注建立类似项目的人。