Alagille syndrome: family studies

Alagille syndrome (AGS) is one of the major forms of chronic liver disease in childhood with severe morbidity and a mortality of 10 to 20%. It is characterised by cholestasis of variable severity with paucity of interlobular bile ducts and anomalies of the cardiovascular system, skeleton, eyes, and face. Previous studies suggest a wide variation in the expression of the disease and a high incidence of new mutations. To determine more accurately the rate of new mutations and to develop criteria for detecting the disorder in parents we systematically investigated parents in 14 families with an affected child. Clinical examination was supplemented by liver function tests, echocardiography, radiographic examination of the spine and forearm, ophthalmological assessment, and chromosome analysis. Six parents had typical anomalies in two or more systems pointing to the presence of autosomal dominant inheritance. Systematic screening of parents for the features defined in this study should improve the accuracy of genetic counselling.     

Encoded moire inspection based on a computer solid model

An investigation into a system linking a computer solid modeler to an active stereo imaging inspection station is presented. Given the computer solid model of a surface, a projected pattern can be encoded so that the reflections from the object's surface exhibit a uniformly simple pattern when the object's surface and the modeled surface are identical. Research concerning the method of generating projection patterns, as well as the sensitivity of the system, is discussed. A prototype system consisting of a computer graphics workstation; a flexible projector, and a machine vision system was used to inspect two different manufactured parts     

IEA joint trials: New lessons from old plantations

Two international trials of potential biomass species families and clones planted in 1985 have been analyzed to determine changing patterns in growth, diseases, and insects. In the Swedish trial, with close spacing and short rotations, Salix viminalis clones gave the best average yield of 17.4 Mg/Ha/yr on the first coppice rotation. In the Minnesota, USA trial, with wider spacings and a longer rotation Populus alba hybrid clones gave the best average yields of 5.5 Mg/Ha/yr. The most important disease and insect problems encountered were Septoria musiva stem cankers on some Populus clones; frost die back in some Salix clones; and the leaf blotch miner, Fenusa dohrnii, on most Alnus families. Recommendations for future international cooperation in field trials are presented.     

Pharmacodynamic modeling of the electroencephalographic effects of flumazenil in healthy volunteers sedated with midazolam

The purpose of this study was to model pharmacodynamically the reversal of midazolam sedation with flumazenil. Ten human volunteers underwent four different sessions. In session 1, individual midazolam pharmacokinetics and electroencephalographic pharmacodynamics were determined. In sessions 2 and 3, a computer-controlled infusion of midazolam with individual volunteer pharmacokinetic data was administered, targeting a plasma concentration corresponding to a light or deep level of sedation (20% or 80% of the maximal midazolam electroencephalographic effect) for a period of 210 minutes. After obtaining a stable electroencephalographic effect and constant midazolam plasma concentrations, a zero-order infusion of flumazenil was started until complete reversal of midazolam electroencephalographic effect was obtained. The flumazenil infusion was then stopped and the volunteer was allowed to resedate because of the constant midazolam drug effect. The electroencephalographic response was measured during a 180-minute period and analyzed by aperiodic analysis and fast-Fourier transforms. In session 4, a midazolam plasma concentration corresponding to a deep level of sedation was targeted for 210 minutes to examine for the possible development of acute tolerance. No flumazenil was given in session 4. For a light sedation level, with a mean midazolam plasma concentration of 160 +/- 64 ng/ml, the mean half-life of the equilibration rate constant of flumazenil reversal is 5.0 +/- 2.5 minutes, and the mean effect site concentration causing 50% of Emax is 13.7 +/- 5.8 ng/ml. For a deep level of sedation, with a mean midazolam plasma concentration of 551 +/- 196 ng/ml, the mean half-life of the equilibration rate constant is 3.9 +/- 1.5 minutes, and the mean effect site concentration causing 50% of Emax is 20.6 +/- 6.8 ng/ml. This study provides an estimate of the magnitude of the blood/central nervous system equilibration delay for flumazenil antagonism of midazolam sedation and further defines the usefulness of the electroencephalogram as a measure of midazolam pharmacodynamic effect.     

Measles, polio and tetanus toxoid antibody levels in Gambian children aged 3 to 4 years following routine vaccination

A nation-wide cross-sectional survey of 816 children 3-4 years old was carried out in The Gambia between September 1990 and July 1991 to assess the seroprevalence of antibodies against 3 diseases included in the expanded programme on immunization: measles, poliomyelitis and tetanus. Among 689 children whose records were available, 94.5% were fully immunized. Measles vaccine was administered to 97% of the children and 91% of these had detectable antibodies at the time of the survey. Antibodies against type 1 and type 3 polioviruses, after up to 6 doses of oral polio vaccine, were present in 88.1% and 89.3% of the children respectively. Ninety-seven percent of the children who had received 4 doses of diphtheria-pertussis-tetanus vaccine (DPT) and 91% of those who received 3 doses had detectable tetanus toxoid antibodies at the age of 3-4 years. This study shows that serological responses to EPI vaccines given in infancy persist at very satisfactory levels throughout early childhood.     

An analysis of the coupling of an injection laser diode to a planar LiNbO3waveguide

Calculated butt coupling efficiency between a GaAlAs double heterostructure laser and a single-mode LiNbO3Ti-diffused optical waveguide is presented in this paper as a function of the transverse displacement, the angular alignment, the longitudinal separation between the laser and the waveguide end surfaces, the radiation field of the laser diode and its dependence on the laser diode structure, and the mode profile of the waveguide and its dependence on experimental diffusion parameters. The maximum efficiency is less than 40 percent because of the mismatch between the waveguide mode and the laser radiation, caused primarily by the experimental limitations in their structures. The effect of the reflection of the waveguide end surface on the laser oscillation has also been estimated.     

The science-industry interface in the petroleum industry: Correlation of time series of indicators and their spectra,and growth modelling

Petroleum production and exploration, used as petroleum industry indicators, and accumulation of petroleum-related geoscience literature, used as a science indicator, were compared by several means to gauge the degree of interaction between science and the industry in the period 1934–1990. Methods of comparison employed were: time domain correlations and crosscorrelation; correlations of spectra using coherence and crosspower spectra, and growth-modelling of the indicators. A fifty-year exploration cycle was found, beginning about 1945. Principal features of this cycle seem to coincide with prominent features in the time series for geoscience literature, and both of these variables are correlated with petroleum production. All three variables appear to have been determined ultimately by economic and political events which affected the petroleum industry. All of them show long-period cycles which coincide with the fourth Kondratiev cycle and the beginning of the fifth Kondratiev. The longest time series used (petroleum production in the United States, 1860–1990) shows long-period cycles matching the third, fourth and fifth Kondratiev cycles.