Iniencephaly is not uniformly fatal

Iniencephaly is an uncommon, but not rare, lesion involving a fusion between the posterior occipital bone and the cervical spine. Many cases are associated with other cranio-cervical abnormalities and anencephaly, and most are associated with other structural abnormalities. The prognosis is thought to be dismal. We describe a male infant, one of twins, who was diagnosed prenatally as having iniencephaly. The child was born without complication and has grown and thrived. This is the fourth patient with long-term survival with iniencephaly. We recommend that non-directive counselling be applied when presenting the findings of iniencephaly to parents.     

Prenatal diagnosis using deletion studies in Duchenne muscular dystrophy

Accurate carrier testing and prenatal diagnosis in Duchenne muscular dystrophy (DMD) families is facilitated when an Xp21 deletion is found to be segregating within a family. We discuss the results of the DNA testing in two families, one in which DNA from affected males was available for study and the other in which no DNA from an affected male was available. Factors complicating the counselling of DMD deletion families are outlined.     

Cystic hygroma: Prenatal diagnosis and genetic counselling

Six cases of cystic hygromas detected during second trimester ultrasound examination are reported: 4 fetuses (67 per cent) had a 45, X karyotype, 1 fetus had trisomy 18, 1 fetus had a normal karyotype (46,XX) and at autopsy multiple anomalies were observed. In the latter case the family history suggested an autosomal recessive pattern of inheritance. In order to reach a definite diagnosis and give proper genetic counselling when a fetus is found to have cystic hygroma, a fetal karyotype as well as a family and reproductive history should be obtained.     

Is maternal alpha-fetoprotein screening still of value in a low-risk area for neural tube defects?

Estimation of maternal serum alpha-fetoprotein (AFP) was used as a screening method for the detection of neural tube defects (NTDs) in 6344 women over three years. Of 88 (1.4 per cent) who had one or more serum AFP levels equal to, or greater than, 2.5 multiples of the median (MoM) for the relevant gestational age, 43 (0.68 per cent) underwent amniocentesis. There were eight NTDs. Four of these were screened by serum AFP, and all cases of spina bifida had serum AFP levels greater than 3.0 MoM, including one small open defect which was not seen on ultrasound. The other four cases of NTD, which were not screened, were identified by ultrasound. Of 64 singleton pregnancies 32 (50 per cent) had serum AFP levels between 2.5 and 3.0 MoM, and low birthweight (⪕2500 g) occurred in 29 per cent. Because of improvements in ultrasound techniques and the apparent falling incidence of NTD, the role of serum AFP as the primary screening procedure should be regularly reviewed. Effective screening is dependent on mothers booking early.     

Sonographic diagnosis of cystic adenomatoid malformation in-utero

Routine ultrasound examination at 30 weeks gestation revealed an intrapulmonary cystic mass in an otherwise normal fetus. Following delivery at term, the diagnosis of congenital cystic adenomatoid malformation of the right lung was confirmed, and an elective right middle lobectomy successfully performed at nine days of age.     

Prenatal diagnosis for the detection of down syndrome: Why are so few eligible women tested?

Postpartum women ≧ 33 years were interviewed about their attitudes to and knowledge and use of prenatal diagnosis. Overall, 68 per cent had heard of prenatal diagnosis; nevertheless, only 30 per cent of those ≧ 35 had actually been tested. The only significant difference between eligible women who were tested and those who were not was maternal age. Of those tested, half requested it for themselves; conversely, only two-thirds of women requesting the procedure actually received it. Among women not tested, 82 per cent were never offered the procedure by the physician. Expressed attitudes to prenatal diagnosis were strongly positive among all women, with 75 per cent continuing to want testing after learning both their age-specific risk of having an affected child and the possible risks of amniocentesis. The data document a potential demand for amniocentesis far in excess of current use and present service facilities. They suggest, moreover, that underuse may reflect professional hesitation and underreferral more than consumer lack of demand or reluctance to be tested.     

湿式氧化技术处理炼油厂碱渣废水

考察了湿式氧化法处理运行过程 ,以及相关参数的控制方案。在实现装置出水、废气达标排放的前提下 ,稳定控制装置运行是可以实现的。如后续有酸化除酚工艺和SBR工艺进行再处理 ,则应选用温度 (14 5± 5 )℃ ,压力控制在 0 6～ 0 .7MPa的湿式氧化法     

Application of the analytic hierarchy process to select characterization and risk-based decision-making and management methods for hazardous waste sites

Environmental managers at U.S. Department of Defense (DoD) installations overseas are faced with the challenge of managing contaminated sites at these installations with little information on the extent of contamination or the risk posed by the site. In this regard, DoD managers overseas encounter a situation quite similar to the situation faced by decision makers in the U.S. who are managing brownfields. Innovative site characterization and risk-based decision-making methods, which are currently being developed for expeditious application at brownfield sites in the U.S., may also be appropriate for application at overseas DoD sites. In this paper, the analytic hierarchy process (AHP) is used by DoD decision makers to evaluate and rank innovative site characterization technologies and risk-based decision-making and management methods, for use at installations in Korea. Results indicate that for sites with high potential risk the decision makers preferred site characterization technologies that produce data of high quality and a method that can be used to establish credible risk-based remediation goals. This study provides a framework for applying characterization technologies and risk management to poorly characterized contaminated sites in developing countries, where resources for remedial actions may be limited.     

邢台煤矿矿井水的综合治理及利用

随着开采深度及范围的扩大，邢台煤矿井下高压出水点越来越多，既浪费了大量的排水费用，又对岩溶水环境和水资源造成了严重破坏。针对上述情况，用四种不同的方法分别进行了封堵和重新利用，使矿井减少了４８０ｍ＾３／ｈ的无效排水，经济效益和环保效益均十分明显。