The food consumption and assimilation were determined in the females of bank vole, Clethrionomys glareolus (S c h r e b e r, 1780) during the whole period of pregnancy and lactation (18 + 18 days). The daily food assimilation (digestion) of non-reproducing females (body weight 25 g) isl 7.5 Kcal/animal. Toward the end of gestation it reaches 23.7 Kcal/day/animal and during the maximal lactation it averages 38.9 Kcal/day for the females of the same body weight. The energy requirement of females increases by 24% during the pregnancy and 92% during the lactation. To produce a litter of 5 young and raise 4 of them to weaning a female has to assimilate additionally 364 Kcal; 75 for the pregnancy and 289 Kcal for the lactation. The increase in food intake during this period corresponds to 406 Kcal. The digestibility coefficient is high in both non-reproducing and pregnant or lactating females (88.5%). The ecological efficiency is also very high; 11.8% during the pregnancy and 14.6% during the lactation. In the models of rodent bioenergetics the correction for gestation and lactation has to be considered. These corrections are computed for whole breeding season in relation to the number of mature females in the population.

No abstract

The authors present a report on two sibling with a nearly identical phenotype mimicking peroxisomal disorder but with totally discordant biochemical findings. In an attempt to confirm the diagnosis of a peroxisomal disorder, plasma levels of very long chain fatty acids (VLCFA) and phytanic acid were determined. In addition, fibroblasts profile of VLCFA and plasmalogen levels were evaluated for Case 2. Pathomorphological examinations represented by lymph node and kidney biopsies as well as postmortem examinations of liver, adrenals and brain were performed in Case 1. The phenotypes of both siblings showed marked resemblance to those observed in children with Zellweger syndrome. The results of biochemical tests performed in Case 1, together with abundant peroxisomes in the liver and the wide spectrum of clinical abnormalities suggest pseudo-Zellweger or Zellweger-like syndromes, but no conclusive data have been found. The plasmalogen level in fibroblasts as well as the fibroblasts profile of VLCFA noted in Case 2 were within the normal range, thus excluding the possibility of a peroxisomal disorder. The striking phenotype resemblance of the siblings mimicking Zellweger, together with discordant biochemical findings, are difficult to explain on the basis of currently available tests.

No abstract

No abstract

No abstract

Abetalipoproteinaemia (ABLP) was diagnosed in a brother and sister, 9 and 13 years old, presenting with symptoms of malabsorption during the neonatal period. Both children showed most of the main clinical features of ABLP, including neurological, and ophthalmic symptoms, and mental retardation. Acanthocytosis of erythrocytes was almost complete in the affected children, while in most of the remaining 11 members of their three-generation family, it was found in less than 50% of red blood cells. Absence of apoprotein B and low concentrations of apo A-I and lipids were found only in ABLP-affected children. Among five siblings only the two affected children had ABLP-characteristic lipid storage in enterocytes. The latter features correlated better with clinical symptoms than did the acanthocytosis of erythrocytes.

No abstract