Inheritance of quantitative expression of erythrocyte glucose-6-phosphate dehydrogenase activity in the Negro—a twin study

Studies have been conducted on eight sets of monozygous and nine sets of dizygous female Negro twins, both members of whom were heterozygous for G-6-PD deficiency. Twins were studied both by assay of erythrocytic G-6-PD activity and by the methemoglobin elution test (MET). The MET is a procedure which identifies histochemically cells with appreciable G-6-PD activity and permits accurate determination of the percentage of such cells in heterozygotes. Monozygous twins showed significantly less within-pair variation than dizygous twins with both the MET and G-6-PD assay.Concerning the significantly greater agreement in MET results in monozygous twins than dizygous twins, our present working hypothesis is that X-chromosomal inactivation in the Negro female is genetically controlled, rather than random. However, certain alternate hypotheses allowing for random X-inactivation have not been excluded; these include somatic cell selection after random X-inactivation, and cell exchange between identical twins in utero/it. Studies in nontwin related heterozygotes now underway should help differentiate among these various possibilities.In addition to the studies on 17 pairs of female twins heterozygous for G-6-PD deficiency, 26 pairs of nondeficient female Negro twins have been studied by G-6-PD assay. Within-pair variation in monozygous twins was significantly less than within-pair variation in dizygous twins in all cases. The genetic influences detected with the G-6-PD assay in the female twins could theoretically be due to nonrandom X-inactivation, to genetically determined quantitative differences in enzyme activity (e.g., isoalleles), or to both. By appropriate calculations, based on the MET results, we have factored out the effects of X-inactivation on overall enzyme activity in the heterozygous deficient twins. After removal of the effect of X-inactivation, monozygous twins heterozygous for enzyme deficiency continue to show significantly less within-pair variation than dizygous twins. This finding indicates significant genetic influences on quantitative G-6-PD activity other than X-inactivation and other than the deficiency allele. This conclusion has been strengthened by studies on male twins where X-inactivation is not present.Supported by USPHS research grants AM-09381, HE-17544, AM-09919, and HE-03341, by USPHS Career Development Award 1-K3-AM-7959 (Dr. Brewer) and by U.S.A.E.C. Contract (11-1)-1552.     

The Microflora of Frozen Passionfruit Nectar Base

A considerable and varied microflora is introduced into passionfruit nectar base under present methods of production. In spite of the great acidity of the nectar base (pH range: 2.8 to 3.2), the high sucrose concentration (approximately 50%), and storage at -20 C, remnants of the microflora persist for a year or longer. During storage, however, there is a steady and gradual decrease, until after about 18 months the microflora is near to extinction. Sample regression lines show straight-line slopes for this diminution in numbers.

A battery of nine media was used to grow a representative aerobic flora. Purified cultures of isolates were identified to genera. Yeasts were the most numerous organisms in all samples, followed by molds, bacteria, and streptomycetes. The bacteria were the first group to disappear during storage. No fecal streptococci or gram-negative bacilli were found in any samples.

     

INTRAVENOUS NUTRITION—A Clinical Evaluation of a 50 Per Cent Dextrose in Water Solution,Containing 1 mg. of Hydrocortisone per 100 ml

A 50 per cent dextrose in water solution, containing 1 mg. of hydrocortisone per 100 ml., was used successfully in 70 patients for intravenous nutritional maintenance and repletion. There were no adverse systemic effects during or following 216 infusions. The only undesirable local reaction was the rare occurrence of pain in the arm when the concentrated solutions were given too rapidly. Glycosuria was minimal if the infusion rate did not exceed 0.85 gm. of glucose per kilogram of body weight per hour, particularly if 50 units of insulin were added to each 550 ml. bottle of 50 per cent dextrose. In patients without significantly elevated serum potassium content, 30 mEq. of potassium chloride, acetate or phosphate was added to each bottle to prevent hypokalemia.Preliminary observations suggest that this new solution may be given safely intravenously, without need for cutdowns or plastic catheters, if the needle is carefully inserted and well immobilized in the arm vein and the duration of the infusion is not too prolonged. Further studies on the effect of such high caloric supplementation plus protein hydrolysates in parenteral nutritional repletion and maintenance are indicated.