Oakeshott Pippa, Hunt Gillian M
Department of Community Health Sciences, St George's Hospital Medical School, London SW17 0RE.
Br J Gen Pract. 2003 Aug;53(493):632-6.
Doctors need reliable data on outcome in order to help parents faced with difficult decisions about termination of an affected pregnancy or treatment after birth.
To determine survival, health and lifestyle at the mean age of 30 years in a complete cohort of adults born with open spina bifida.
Prospective cohort study.
Well-documented cohort of 117 consecutive cases of open spina bifida whose backs were closed non-selectively at birth between 1963 and 1971.
Survivors (age range = 26 to 33 years) were surveyed by postal questionnaire and telephone interview. The main outcome measures were the health, independence and lifestyle of the survivors in terms of living in the community, driving a car and working in open employment.
Ascertainment was 100%. Sixty (51%) had died, mainly the most disabled. Of the 57 survivors, 84% had a cerebrospinal fluid (CSF) shunt, 70% had an IQ of 80 or more, 37% lived independently in the community, 39% drove a car, 30% could walk more than 50 metres and 26% were in open employment. However one-third (19) still needed daily care, three were on respiratory support, two were blind, two had diabetes mellitus, and one was on dialysis. Mortality, disability and achievement reflected the neurological deficit that had been recorded in infancy in terms of sensory level. Attainment and independence were reduced in those who had needed revision of CSF shunt.
The survivors in this unselected cohort showed a wide range of outcome from apparent normality to very severe disability. This reflected both the extent of their original neurological deficit and events in the history of their CSF shunt.
医生需要可靠的预后数据，以帮助面临终止受影响妊娠或出生后治疗等艰难决策的父母。
确定一组完整的成年开放性脊柱裂患者在平均30岁时的生存情况、健康状况和生活方式。
前瞻性队列研究。
1963年至1971年间出生时背部非选择性闭合的117例连续开放性脊柱裂病例的详细记录队列。
通过邮寄问卷和电话访谈对幸存者（年龄范围为26至33岁）进行调查。主要结局指标是幸存者在社区生活、开车和从事公开就业方面的健康状况、独立性和生活方式。
随访率为100%。60人（51%）已死亡，主要是残疾最严重的患者。在57名幸存者中，84%有脑脊液（CSF）分流管，70%智商在80或以上，37%在社区独立生活，39%开车，30%能行走超过50米，26%从事公开就业。然而，三分之一（19人）仍需要日常护理，3人需要呼吸支持，2人失明，2人患有糖尿病，1人需要透析。死亡率、残疾程度和成就反映了婴儿期记录的感觉平面方面的神经功能缺损。脑脊液分流管需要翻修的患者其成就和独立性较低。
这个未经过筛选的队列中的幸存者显示出从明显正常到非常严重残疾的广泛预后范围。这既反映了他们最初神经功能缺损的程度，也反映了他们脑脊液分流管治疗史中的事件。
