Ichthyosiform erythroderma and cardiomyopathy: report of two cases and review of the literature

We report two children with ichthyosiform erythroderma who at the ages of 9 weeks and 8 years, respectively, developed dilated cardiomyopathy, which was fatal in one and required heart transplantation in the other. A link between these conditions is considered likely, either as a primary genetic syndrome or secondary to micronutrient deficiency and/or infection. Owing to its insidious onset, cardiomyopathy may be overlooked, or symptoms attributed to the other conditions such as severe infections and failure to thrive that are common in these patients. We therefore recommend that children with congenital erythroderma are monitored closely, clinically and with echocardiography, for cardiomyopathy.     

Scalloped wings is the Lucilia cuprina Notch homologue and a candidate for the modifier of fitness and asymmetry of diazinon resistance

The Scalloped wings (Scl) gene of the Australian sheep blowfly, Lucilia cuprina, is shown to be the homologue of the Drosophila melanogaster Notch gene by comparison at the DNA sequence and genetic levels. A L. cuprina genomic fragment, which shows strong identity with the Notch (N) gene at the molecular level, hybridizes to the location of the Scl gene on polytene chromosomes. The two genes are functionally homologous; the dominant and recessive Notch-like phenotypes produced by mutations in the Scl gene allow these alleles to be classed as N-like or Abruptex-like. The Scl gene is under investigation as a candidate for the fitness and asymmetry Modifier (M) of diazinon resistance. We show that M affects the penetrance of wing and bristle phenotypes associated with two Scl alleles in a manner consistent with the M being an allele of Scl. In addition, we report a phenotypic interaction between the diazinon-resistance mutation, Rop-1, and the same alleles of Scl. We propose that the product of Rop-1, an esterase, may be involved in cell adhesion in developmental processes involving the Scl gene product.     

MILK COAGULATION CUT-TIME DETERMINATION USING ULTRASONICS

A nondestructive method of determining milk coagulation cut-time was investigated. Velocity and attenuation of ultrasonic waves through coagulating skim milk were measured after rennet addition. the ultrasonic attenuation measurements were found suitable for distinguishing the effect of different experimental variables (three levels of temperature, rennet and CaCl₂). Based on the rate of change of attenuation during coagulation, a turning point was defined at which the attenuation rate change was -0.1 neper/m/min. the cut-time was proposed to be 20 min from the time the turning point was observed. the cut-times predicted by this criterion were statistically similar to those predicted by the manual method currently used in the industry.     

Separation, purification, and properties of multiple forms of cytochrome P-450 from the liver microsomes of phenobarbital-treated mice

Four distinct cytochrome P-450 fractions (A1, A2, C1, and C2) have been separated and purified from the liver microsomes of phenobarbital-treated hybrid mice (B6D2F1/J). Fractions A2 and C2 were highly purified with specific contents of 16.5 and 17.5 nmol of cytochrome P-450/mg of protein, respectively, based on their amino acid compositions. The major hemeprotein bands of A2 and C2 have different minimum molecular weights (50,000 and 56,000, respectively) on polyacrylamide gels in the presence of sodium dodecyl sulfate. All four fractions with respect to their spectral and catalytic properties, thereby demonstrating that mouse liver microsomes from phenobarbital-treated hybrid mice contain at least four forms of cytochrome P-450.     

Degradation behavior of mechanically fastened joints of glass-reinforced crosslinked polyester immersed in hot water

This study deals with the degradation of mechanically-fastened GFRP joints immersed in hot water (80°C). The material used was randomly oriented E-glass fiber continuous strand mat with a crosslinked polyester. Three kinds of joint geometries were adopted; thickness was 3 mm, hole diameter was 6 mm, the distance from hole center to top-edge was 18 mm (3e), and specimen widths were 18 (3w), 30 (5w), and 42 mm (7w). Failure modes of original dry specimens were a function of joint geometry, The dominant failure mode of 3w3e joints was net-tension, whereas 5w3e and 7w3e joints displayed bearing failure. As degradation progressed, the dominant failure mode gradually shifted from net-tension to bearing failure. Strength reduction was marked in 5w3e and 7w3e joints, in which the dominant failure mode was bearing. Joint strength and failure mode were predicted from the combination of a macroscopic failure criterion and characteristic curves obtained from tensile testing of rectangular specimens with holes, bearing tests, and finite element analysis. Predictions agreed with experiment.