Familial hyperaldosteronism type II: Description of a large kindred and exclusion of the aldosterone synthase (CYP11B2) gene

Familial hyperaldosteronism type II (FH-II) is characterized by autosomal dominant inheritance and hypersecretion of aldosterone due to adrenocortical hyperplasia or an aldosterone-producing adenoma; unlike FH type I (FH-I), hyperaldosteronism in FH-II is not suppressible by dexamethasone. Of a total of 17 FH-II families with 44 affected members, we studied a large kindred with 7 affected members that was informative for linkage analysis. Family members were screened with the aldosterone/PRA ratio test; patients with aldosterone/PRA ratio greater than 25 underwent fludrocortisone/salt suppression testing for confirmation of autonomous aldosterone secretion. Postural testing, adrenal gland imaging, and adrenal venous sampling were also performed. Individuals affected by FH-II demonstrated lack of suppression of plasma A levels after 4 days of dexamethasone treatment (0.5 mg every 6 h). All patients had neg ative genetic testing for the defect associated with FH-I, the CYP11B1/CYP11B2 hybrid gene. Genetic linkage was then examined between FH-II and aldosterone synthase (the CYP11B2 gene) on chromosome 8q. A polyadenylase repeat within the 5'-region of the CYP11B2 gene and 9 other markers covering an approximately 80-centimorgan area on chromosome 8q21-8qtel were genotyped and analyzed for linkage. Two-point logarithm of odds scores were negative and ranged from -12.6 for the CYP11B2 polymorphic marker to -0.98 for the D8S527 marker at a recombination distance (theta) of 0. Multipoint logarithm of odds score analysis confirmed the exclusion of the chromosome 8q21-8qtel area as a region harboring the candidate gene for FH-II in this family. We conclude that FH-II shares autosomal dominant inheritance and hyperaldosteronism with FH-I, but, as demonstrated by the large kindred investigated in this report, it is clinically and genetically distinct. Linkage analysis demonstrated that the CYP11B2 gene is not responsible for FH-II in this family; furthermore, chromosome 8q21-8qtel most likely does not harbor the genetic defect in this kindred.

High-level and phonological awareness abilities of children following management for supratentorial tumour: Part II

Background. Limited information is available regarding the impact of childhood tumour on the cerebral hemispheres and supratentorial cranial fossa. However, a recent study found that children managed for a tumour located in this region may demonstrate reduced general language abilities. However, the indirect or direct impact of a tumour in this region on higher-level language abilities in childhood is at present largely unknown. Materials and methods. The present study examined the higher language and phonological awareness abilities of five children treated for supratentorial tumour ranging in age from seven to fourteen years in age. Assessments included measures of receptive and expressive semantic abilities, inferencing, figurative language, and problem solving, as well as a comprehensive pre-literacy test. Results. As a group, reductions were evident in problem solving, and in the ability to receive and decode content of high-level language when compared to a group of age- and gender-matched peers. At an individual level, only two of five children managed for supratentorial tumour demonstrated language deficits. These two cases were noted to be the same children previously identified as also having general language deficits. More widespread findings were noted in phonological awareness, with four of the five children previously managed for supratentorial tumour demonstrating weaknesses in one or more areas. Conclusions. Findings demonstrated that weaknesses in general language ability in children managed for supratentorial tumour may indicate higher-level language difficulties. Language abilities beyond general measures of language should be monitored, as well as long-term consideration of phonological awareness abilities in this population.

Elevations, Goetz Residence II, Red Hill, Brisbane

Hand-drawn elevations of proposed residence.

Floor plan, Goetz Residence II, Red Hill, Brisbane

Hand-drawn plan of proposed residence.

Substrate-inhibited kinetics with catalyst deactivation in an isothermal CSTR. II. Multiple pseudosteady states and reactor failure

Analytical expressions are derived for the time and magnitude of failure of an isothermal CSTR with substrate-inhibited kinetics, caused by slow catalyst deactivation under three types of parallel and series mechanisms. Reactors operating at high space velocity are found to be most susceptible to early failure and poisoning by product is more dangerous than by reactant. The magnitude of the jump across steady states depends solely on the Langmuir-Hinshelwood kinetic parameters and a detailed analysis of reactor behavior during the jump itself is given.

Effect of pH on Na induced Ca deficiency

Although it is well known that high Na concentrations induce Ca deficiency in acidic conditions, the effect of high pH on this competitive mechanism is not so well understood. The effect of Ca activity ratio (CAR) and pH on the Ca uptake of mungbeans (Vigna radiata (L.) Wilczek cv. Emerald) and Rhodes grass (Chloris gayana cv. Pioneer) in Na dominated solution cultures and in soil was investigated. Changes in pH in the alkaline range were shown not to affect the critical CAR of 0.024 (corresponding to 90 % relative root length) for mungbeans grown in solution culture. Results from soil grown mungbeans confirmed those from solution culture, with a critical CAR of 0.025. A critical CAR of 0.034 was also established for soil grown Rhodes grass. The similarity of critical values established for mungbeans and Rhodes grass in solution culture and soil justifies the use of both solution culture and soil solution measurement as techniques for studying plant growth and limitations across plant species.

Mg induced Ca deficiency under alkaline conditions

Little is known about Mg induced Ca deficiency in alkaline conditions, and the relationship between Mg induced Ca deficiency and Na induced Ca deficiency. Dilute nutrient solutions (dominated by Mg) were used to investigate the effect of Ca activity ratio (CAR) on the growth of mungbeans (Vigna radiata (L.) Wilczek cv. Emerald). At pH 9.0, root growth was reduced below a critical CAR of 0.050 (corresponding to 90 % relative root length). Root growth was found to be limited more in Mg solutions than had been previously observed for Na solutions. Using a CAR equation modified with plasma membrane binding constants (to incorporate the differing antagonistic effects of Mg and Na), new critical CAR values were calculated for both Na (0.56) and Mg (0.44) dominated solutions. This modified CAR equation permits the calculation of CAR irrespective of the dominant salt present.

Effect of ionic strength and clay mineralogy on Na-Ca exchange and the SAR-ESP relationship

The relationship between sodium adsorption ratio (SAR) and exchangeable sodium percentage (ESP) for all soils has traditionally been assumed to be similar to that developed by the United States Salinity Laboratory (USSL) in 1954. However, under certain conditions, this relationship has been shown not to be constant, but to vary with both ionic strength and clay mineralogy. We conducted a detailed experiment to determine the effect of ionic strength on the Na+-Ca2+ exchange of four clay minerals (kaolinite, illite, pyrophyllite, and montmorillonite), with results related to the diffuse double-layer (DDL) model. Clays in which external exchange sites dominated (kaolinite and pyrophyllite) tended to show an overall preference for Na+, with the magnitude of this preference increasing with decreasing ESP. For these external surfaces, increases in ionic strength were found to increase preference for Na+. Although illite (2:1 non-expanding mineral) was expected to be dominated by external surfaces, this clay displayed an overall preference for Ca2+, possibly indicating the opening of quasicrystals and the formation of internal exchange surfaces. For the expanding 2:1 clay, montmorillonite, Na+-Ca2+ exchange varied due to the formation of quasicrystals (and internal exchange surfaces) from individual clay platelets. At small ionic strength and large ESP, the clay platelets dispersed and were dominated by external exchange surfaces (displaying preference for Na+). However, as ionic strength increased and ESP decreased, quasicrystals (and internal exchange surfaces) formed, and preference for Ca2+ increased. Therefore, the relationship between SAR and ESP is not constant and should be determined directly for the soil of interest.

Chemical treatment of Escherichia coli. II. Direct extraction of recombinant protein from cytoplasmic inclusion bodies in intact cells

A method is presented for the direct extraction of the recombinant protein Long-R-3-IGF-I from inclusion bodies located in the cytoplasm of intact Escherichia coli cells. Chemical treatment with 6M urea, 3 mM EDTA, and 20 mM dithiothreitol (DTT) at pH 9.0 proved an effective combination for extracting recombinant protein from intact cells. Comparable levels of Long-R-3-IGF-I were recovered by direct extraction as achieved by in vitro dissolution following mechanical disruption. However, the purity of directly extracted recombinant protein was lower due to contamination by bacterial cell components. The kinetics of direct extraction are described using a first-order equation with the time constant of 3 min. Urea appears important for permeabilization of the cell and dissolution of the inclusion body. Conversely, EDTA is involved in permeabilization of the cell wall and DTT enhances protein release. pH proved to be important with lower levels of protein release achieved at low pH values (

Matrix elements of U(2n) generators in a multishell spin-orbit basis. II. The two-shell nonzero shift ACCs and U(2n) generator MEs

This is the second in a series of articles whose ultimate goal is the evaluation of the matrix elements (MEs) of the U(2n) generators in a multishell spin-orbit basis. This extends the existing unitary group approach to spin-dependent configuration interaction (CI) and many-body perturbation theory calculations on molecules to systems where there is a natural partitioning of the electronic orbital space. As a necessary preliminary to obtaining the U(2n) generator MEs in a multishell spin-orbit basis, we must obtain a complete set of adjoint coupling coefficients for the two-shell composite Gelfand-Paldus basis. The zero-shift coefficients were obtained in the first article of the series. in this article, we evaluate the nonzero shift adjoint coupling coefficients for the two-shell composite Gelfand-Paldus basis. We then demonstrate that the one-shell versions of these coefficients may be obtained by taking the Gelfand-Tsetlin limit of the two-shell formulas. These coefficients,together with the zero-shift types, then enable us to write down formulas for the U(2n) generator matrix elements in a two-shell spin-orbit basis. Ultimately, the results of the series may be used to determine the many-electron density matrices for a partitioned system. (C) 1998 John Wiley & Sons, Inc.

Improving wheat simulation capabilities in Australia from a cropping systems perspective - II. Testing simulation capabilities of wheat growth

To simulate cropping systems, crop models must not only give reliable predictions of yield across a wide range of environmental conditions, they must also quantify water and nutrient use well, so that the status of the soil at maturity is a good representation of the starting conditions for the next cropping sequence. To assess the suitability for this task a range of crop models, currently used in Australia, were tested. The models differed in their design objectives, complexity and structure and were (i) tested on diverse, independent data sets from a wide range of environments and (ii) model components were further evaluated with one detailed data set from a semi-arid environment. All models were coded into the cropping systems shell APSIM, which provides a common soil water and nitrogen balance. Crop development was input, thus differences between simulations were caused entirely by difference in simulating crop growth. Under nitrogen non-limiting conditions between 73 and 85% of the observed kernel yield variation across environments was explained by the models. This ranged from 51 to 77% under varying nitrogen supply. Water and nitrogen effects on leaf area index were predicted poorly by all models resulting in erroneous predictions of dry matter accumulation and water use. When measured light interception was used as input, most models improved in their prediction of dry matter and yield. This test highlighted a range of compensating errors in all modelling approaches. Time course and final amount of water extraction was simulated well by two models, while others left up to 25% of potentially available soil water in the profile. Kernel nitrogen percentage was predicted poorly by all models due to its sensitivity to small dry matter changes. Yield and dry matter could be estimated adequately for a range of environmental conditions using the general concepts of radiation use efficiency and transpiration efficiency. However, leaf area and kernel nitrogen dynamics need to be improved to achieve better estimates of water and nitrogen use if such models are to be use to evaluate cropping systems. (C) 1998 Elsevier Science B.V.

Copper(II) complexes of mono- and di-nucleating hexaamines

The potentially hexadentate polyamines N,N,N',N'-tetrakis(2-aminoethyl)ethane-1,2-diamine (L-1) and the octamethylated analogue N,N,N',N'-tetrakis(2-dimethylaminoethyl)ethane 1,2-diamine (L-2) have been complexed with copper(II) and the crystal structures of their complexes determined. A trigonal-bipyramidal co-ordination geometry for [Cu(HL1)][ClO4](3) was found where one aminoethyl arm is not co-ordinated. By contrast, a dinuclear structure of formula [(H2O)Cu(L-2)Cu(OH)](3+) was determined for the N-methylated analogue, where the hexaamine acts as a bridging ligand between the two square-pyramidal metal centres. Electronic and EPR spectroscopy are both consistent with these structures being maintained in solution.

A novel tricopper(II) complex of a polyamine alcohol

The trinuclear copper(II) complex of a new polyamino alcohol ligand has been isolated; it exhibits a structure similar to that found at the active site of ascorbate oxidase.