Prenatal diagnosis of medium-chain acyl-CoA dehydrogenase (MCAD) deficiency in a family with a previous fatal case of sudden unexpected death in childhood

Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is a potentially fatal inherited disease with a carrier frequency of approximately 1:100 in most Caucasian populations. The disease is implicated in sudden unexpected death in childhood. A prevalent disease-causing point mutation (A985G) in the MCAD gene has been characterized, thus rendering diagnosis easy in the majority of cases. Since the clinical spectrum of MCAD deficiency ranges from death in the first days of life to an asymptomatic life, there are probably other genetic factors—in addition to MCAD mutations—involved in the expression of the disease. Thus, families who have experienced the death of a child from MCAD deficiency might have an increased risk of a seriously affected subsequent child. In such a family we have therefore performed a prenatal diagnosis on a chorionic villus sample by a highly specific and sensitive polymerase chain reaction (PCR) assay for the G985 mutation. The analysis was positive and resulted in abortion. We verified the diagnosis by direct analysis on blood spots and other tissue material from the aborted fetus and from family members.     

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.     

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.     

Hydrogen storage and distribution systems

Hydrogen storage and transportation or distribution is closely linked together. Hydrogen can be distributed continuously in pipelines or batch wise by ships, trucks, railway or airplanes. All batch transportation requires a storage system but also pipelines can be used as pressure storage system. Hydrogen exhibits the highest heating value per weight of all chemical fuels. Furthermore, hydrogen is regenerative and environment friendly. There are two reasons why hydrogen is not the major fuel of toady’s energy consumption: First of all, hydrogen is just an energy carrier. And, although it is the most abundant element in the universe, it has to be produced, since on earth it only occurs in the form of water. This implies that we have to pay for this energy, which results in a difficult economic task, because since the industrialization we are used to consuming energy for free. The second difficulty with hydrogen as an energy carrier is the low critical temperature of 33 K, i.e. hydrogen is a gas at room temperature. For mobile and in many cases also for stationary applications the volumetric and gravimetric density of hydrogen in a storage system is crucial. Hydrogen can be stored by six different methods and phenomena: high pressure gas cylinders (up to 800 bar), liquid hydrogen in cryogenic tanks (at 21 K), adsorbed hydrogen on materials with a large specific surface area (at T < 100 K), absorbed on interstitial sites in a host metal (at ambient pressure and temperature), chemically bond in covalent and ionic compounds (at ambient pressure), oxidation of reactive metals e.g. Li, Na, Mg, Al, Zn with water. These metals easily react with water to the corresponding hydroxide and liberate the hydrogen from the water. Finally, the metal hydroxides can be thermally reduced to the metals in a solar furnace.     

Mating System and Sexual Selection in the Scorpionfly Panorpa vulgaris (Mecoptera: Panorpidae)

Panorpa vulgaris   has become a model insect for testing theories of sexual selection. This contribution summarizes that which has been learned in recent years and presents new data that clearly show that the mating system of P. vulgaris is not simply a resource-defense polygyny, as has previously been thought. In P. vulgaris neither the pattern in food exploitation nor the ratio of variance in the lifetime reproductive success of the two sexes is in accordance with that expected in resource defense polygynous mating systems. Lifetime mating duration is the most important proximate determinant of male fitness. Males employing alternative mating tactics obtain copulations of varying duration in relation to the following sequence: saliva secretion  1  food offering  1  no gift. The number of salivary masses which males provide to females during their lifetime is significantly correlated with the lifetime condition index. The condition index depends on the fighting prowess of males and their ability to find food items. Thus saliva secretion of Panorpa is considered a Zahavian handicap, which can serve as an honest quality indicator used by mating females. Our results confirm four main predictions of the indicator model of the theory of sexual selection: (a) the indicator signals high ecological quality of its bearer, (b) the indicator value increases with phenotypic quality, (c) the indicator value is positively correlated with the genetic quality affecting offspring fitness in a natural selection context, and (d) the quality indicator is more costly for low- than for high-quality individuals. The evolutionary consequences of the mating pattern and the sperm competition mechanism in P. vulgaris are discussed in the context the way in which sexual selection creates and maintains sperm mixing and the evolution of a promiscuous mating system.     

Alteration in Rhizosphere Soil Properties of Afforested <Emphasis Type="Italic">Rhamnus lycioides</Emphasis> Seedlings in Short-Term Response to Mycorrhizal Inoculation with <Emphasis Type="Italic">Glomus intraradices</Emphasis> and Organic Amendment

The reestablisment of autochthonous plant species is an essential strategy for recovering degraded areas under semiarid conditions. A field experiment was carried out to assess the short-term effect of two reafforestation methods involving mycorrhizal inoculation and compost addition on soil quality parameters and Rhamnus lycioides seedling growth. The nutrient content (NPK) and enzymatic activities (dehydrogenase, urease, protease-BAA, acid phosphatase and β-glucosidase) increased and bulk density decreased in the rhizosphere soil with the organic amendment. Biomass C of rhizosphere soil increased by at least 240% with respect to the control soil after mycorrhizal inoculation and the combination of compost addition + mycorrhizal inoculation. Both mycorrhizal inoculation and composted organic residue addition increased R. lycioides seedling growth in the same proportion. In the short term, we conclude that the application of both reafforestation methods not only enhances the establishment of R. lycioides seedlings, but also improves soil quality.     

Book reviews

Making Cities Work: the role of local government in the urban environment. Richard Gilbert, Don Stevenson, Herbert Girardet & Richard Stren, 1996, London, Earthscan, 203pp. ISBN 1 85383 354 1, £13.95 pbk

Environmental Policy in an International Context, Vols 1-3: Perspectives, Conflicts and Prospects. P. Glasbergen & A. Blowers (Series Eds) London, Arnold

Rhetoric and Reality in Environmental Policy. M. Wintle & R. Reeve (Eds), 1994, Aldershot, Avebury Studies in Green Research, 154 pp. ISBN 1 85628 927 3

FutureNatural: Nature, Science, Culture. G. Roberston et al. (Eds) 1996, London, Routledge, ISBN 0415 070139, £40.00 hbk

Environmental Policy with Political Integration: the European Union and the United States. New horizons in environmental economics. John B. Braden, Henk Folmer & Thomas S. Ulen (Eds), 1996, Cheltenham, Elgar Publishing, 477 pp. ISBN 1 85898 217 0     

Marine protected areas and local coastal conservation and management in Hong Kong

This paper focuses on the role of marine parks and reserves in the conservation of Hong Kong's coastal environment. This environment is under serious threat as a result of pollution of marine waters and habitat loss associated with urbanisation, large-scale reclamation projects, and inadequate treatment and disposal of sewage and industrial effluents. The paper discusses whether in a highly urbanised and stressed environment like Hong Kong, which currently lacks a conservation strategy and an overall framework for handling sustainability issues, small marine parks and reserves can be effective in helping to conserve local biodiversity. The paper also suggests that Hong Kong should adopt a broader regional perspective on its coastal conservation and management problems, a perspective that recognises the significance for the territory of the rapid urban and industrial development taking place in neighbouring Guangdong Province. This will require more extensive and focused liaison with Chinese agencies.     

A New Modus Operandi for the Agricultural Economics Profession

Agricultural economics has, until the 1990s, enjoyeda reputation for relevance and usefulness to theagri-food industry and policy-makers. Thatreputation has been jeopardized by a growinginfatuation with models and quantification, and aconcomitant underemphasis placed on many complexproblems and issues of society. An illustrativeexample is explored, using agriculturalactivity-related damage to the natural resourcebase, environment and ecology. Agriculturaleconomists are urged to respond by broadening theirterms of reference and joining forces with otherdisciplines.