Reference values of various blood constituents in young minipigs.

In the Göttinger minipig, the reference values of 18 parameters in whole blood or plasma were determined. The results are expressed both as the mean value and +/- 2 SD presuming a symmetrical normal distribution and as the median value with the corresponding range. For most blood constituents, the results of the two modes of calculation were similar. Comparison of the values from young minipigs with those from children shows that these animals are well suited in this respect for experimental work related to human medicine.

An improved method for preparation of samples for the simultaneous assay of some antiepileptic drugs by gas-liquid chromatography.

A new simple extraction method, e.g., for subsequent quantitative determination of some antiepileptic drugs by GLC is described. HCl and an internal standard are added to 100 microliter of plasma or any biological fluid. This mixture is applied directly to an Extrelut column. Lipophilic substances (i.e. the antiepileptic drugs diphenylhydantoin, primidone and phenobarbital) are then extracted into 20 ml of ether and the solvent is evaporated to dryness. Direct GLC analysis of the on-column methylated drugs is performed using a P-N-detector.

Acute neonatal nonketotic hyperglycinemia: normal propionate and methylmalonate metabolism.

Propionyl-CoA carboxylase and combined methylmalonyl-CoA (MMA-CoA) racemase and -mutase activities were studied in liver and fibroblasts of two patients with the acute neonatal form of nonketotic hyperglycemia. In all experiments, these enzyme activities studied in tissues of the patients were within the range of healthy control subjects, whereas no propionyl-CoA carboxylase activity was measurable in the fibroblasts of a patient with propionic acidemia. Subcellular fractionation of liver and fibroblasts indicated that the normal amounts of MMA-CoA found after incubation of whole tissue homogenate were formed by propionyl-CoA carboxylase, a mitochondrial enzyme, and not be acetyl-CoA carboxylase, which theoretically could also be involved in the carboxylation of propionyl-CoA. From the above data as well as from clinical and biochemical observations in three patients, it was concluded that there exists a true nonketotic hyperglycinemia which is not related etiologically to the different disorders of the ketotic hyperglycinemia syndrome. True nonketotic hyperglycinemia is not associated with ketoacidosis even after loading with propionate- and MMA precursors. It must be distinguished by exclusion from mild forms of the ketotic hyperglycinemia syndrome which may present clinically as hyperglycinemia without ketosis.