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Biomedical subjects

M Pap

Publications and source records attributed to M Pap.

13 recordsLinked to original sources
PubMed1994

Carnitine-dependent changes of metabolic fuel consumption during long-term treatment with valproic acid.

Energy metabolism was measured in children receiving long-term treatment with valproic acid. In 8 of 10 randomly selected subjects, the resting respiratory quotient was higher than in age- and sex-matched control subjects (0.91 +/- 0.01 vs 0.87 +/- 0.01; p < 0.05). A shift was observed in fuel consumption, and a significant reduction was found in the amount of fats oxidized (0.68 +/- 0.23 vs 1.18 +/- 0.18 gm.kg-1.day-1), which was accompanied by increased utilization of carbohydrates (5.31 +/- 0.79 vs 3.81 +/- 0.39 gm.kg-1.day-1) in comparison with the control subjects. The resting total energy expenditure was not affected by the treatment. The children with an altered energy consumption pattern (n = 8) received carnitine supplementation for a month; the respiratory quotient then decreased (0.87 +/- 0.02), the oxidation of fats increased (1.42 +/- 0.25), and the consumption of carbohydrates decreased (3.87 +/- 0.79), but no changes in resting energy expenditure were observed. We conclude that carnitine depletion, a known adverse effect of valproic acid administration, may result in inhibited fatty acid oxidation, leading to a shift of substrates utilized from fats to carbohydrates.

Adolescent↗
PubMed1993

Relationship of carnitine and carnitine precursors lysine, epsilon-N-trimethyllysine, and gamma-butyrobetaine in drug-induced carnitine depletion.

Plasma concentrations and rates of urinary excretion of carnitine and some of its precursors were studied in three groups of children receiving drugs known to cause carnitine depletion. Patients in group A received pivampicillin and a molar equivalent of carnitine for 7 d. Patients in group B received pivampicillin with a 5.8-fold molar excess of carnitine for 1 wk. Patients in group C were treated chronically with valproic acid and received a molar equivalent (to valproic acid) of carnitine for 14 d. Patients in group A had markedly increased (16-fold) urinary carnitine ester excretion concomitant with diminished urinary free carnitine and gamma-butyrobetaine output and lower plasma free carnitine concentration. Supplementation with one molar equivalent of carnitine (to pivampicillin) was ineffective in preventing the reduction of plasma carnitine concentration observed with pivampicillin treatment alone. For group B patients, administration of excess carnitine resulted in a further increase (35-fold) of urinary carnitine ester output with no decrease of plasma carnitine concentration, urinary gamma-butyrobetaine, or free carnitine excretion. For patients in group C, the initially low plasma free and total carnitine concentrations and urinary output of carnitine and carnitine esters markedly increased with carnitine supplementation, but urinary excretion of gamma-butyrobetaine remained unchanged. The plasma concentrations and urinary output of L-lysine and epsilon-N-trimethyllysine remained unchanged within each group before and after treatment. A positive linear correlation was found between urinary epsilon-N-trimethyllysine and 3-methylhistidine output, indicating that the rate of epsilon-N-trimethyllysine excretion correlates with the amount of 3-methylhistidine liberated by protein turnover.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1992

The influence of religious affiliation on surname repetition in marriages in Tiszaszalka, Hungary.

The repeated-pairs of surnames in marriages (RP) approach is applied to the population of Tiszaszalka in north-eastern Hungary. The results indicate that: (1) lineage-like behaviour in mate choice results in population subdivision in both the Catholics and the Protestants of the village; (2) unlike in some other Tiszahat villages, the isonymous and the repeating unions in Tiszaszalka occur in different lineages so, in neither of these subpopulations are isonymous and repeating unions monopolised by a few lineages; (3) religious affilitation influences the mating structure of the population as measured by RP summary scores.

Choice Behavior↗
PubMed1990

Surname repetition and isonymy in northeastern Hungarian marriages.

The repeated-pair (RP) approach to surnames in married couples is a measure of population subdivision resulting from the influence of lineagelike behavior in mate choice. An excess of RP over random RP implies limitations in mate choice and a reduction of genetic variability. Here we apply the RP method to data from the rural populations of Csaroda, Tiszaadony, and Tiszavid in northeastern Hungary. The results indicate small differences between RP and random RP for Tiszavid and somewhat larger differences for Tiszaadony and Csaroda. The excess of RP over random RP in Tiszavid, however, derives primarily from marriages simultaneously isonymous and repeating in only one lineage. The discrepancy between RP and random RP implies a small reduction in genetic variability.

Consanguinity↗
PubMed1986

Severe growth failure associated with atrophic intestinal mucosa and ring chromosome 15.

Ring chromosome 15 was detected in a boy with severe growth failure and no dysmorphic features who had previously been found to have an atrophic intestinal mucosa, a finding not observed in about 300 patients with ring autosome reported so far. Of the 137 metaphases examined, 8.1% had secondary aneuploidy produced by the structural and behavioural instability of the ring. Ring derivatives could also be seen in lymphocytes after only one cycle in the culture, indicating that such cells are also generated in vivo. We observed an increased cell death rate in fibroblast culture by Trypan Blue exclusion. These results suggest that there is a continuous in vivo generation of cells with increased mortality resulting in both growth failure and atrophic intestinal mucosa in the patient. The atrophic intestinal mucosa might be a manifestation of ring instability.

Atrophy↗
PubMed1986

Prevalence of minor congenital anomalies in diabetic children.

The prevalence of 52 minor congenital anomalies (MCAs) was determined in 111 children with insulin dependent diabetes mellitus (IDDM), and in 111 healthy matched control subjects. The average MCA per person was 1.60 in diabetic children and 0.86 in the controls (p less than 0.001). The difference was exclusively due to the significantly higher proportion of subjects with 3 or more MCAs in the diabetic group (27.0 versus 9.9%; p less than 0.001). No specific MCA characteristic of IDDM was found.

Adolescent↗