This report describes the isolation and culture of venous endothelial cells from the umbilical cord of an obligate heterozygote for homocystinuria. The effect of different sulphur-containing amino acids on the viability and function of these cells was studied and compared with cultured normal endothelial cells. When endothelial cells were cultured in the presence of methionine (10 mmol/l) or homocystine (10 mmol/l), differences occurred between the viability and function of the heterozygote and normal cells in terms of 51Cr release and ability to prevent platelet adherence. The Cr release corrected for spontaneous release increases for the heterozygote cells after incubation/for 21 h in the presence of methionine to 81.3% (control cells, range: 0-23.3%, n = 5) and in the presence of homocystine to 141% (control cells, range: 13.5-55.2%, n = 5). The total number of platelets that adhere to confluent monolayers increases for heterozygote cells cultured in the presence of methionine to 0.98 X 10(7) platelets cm-2 (normal cells, range: 0.56-0.72 X 10(7) platelets cm-2) and in the presence of homocystine to 1.41 X 10(7) platelets cm-2 (normal cells, range: 0.94-1.06 X 10(7) platelets cm-2). Both normal and control cells were sensitive to homocysteine. This study/indicates for the first time what vascular endothelial cells, derived from an obligate heterozygote, are (partly) deficient in cysthathionine synthase and are more susceptible to methionine- and homocystine-mediated injury than normal endothelial cells. Consequently, in homocystinuria, due to dysfunction of the endothelial cells, toxic sulphur-containing amino acids may accumulate in these cells, causing injury of these cells.
A field trial was conducted on 36 farms in the Netherlands to compare the fertilizing capacity of fresh and frozen-thawed boar spermatozoa. Four-hundred and fifty-one sows were artificially inseminated with semen than that had been frozen and thawed according to the Beltsville Method or diluted in Kiev extender and inseminated on the day of collection. Twelve boars of the Dutch Landrace and Dutch Large White breeds were used. Farrowing rates, total number of pigs per litter and number of live pigs per litter were higher (P less than .0001) for sows inseminated with fresh semen than for sows inseminated with frozen-thawed semen (79.1%, 10.6 and 9.9 vs 47.0%, 7.4 and 7.1, respectively). Farrowing rates for sows inseminated with frozen-thawed semen were higher when semen from Dutch Large White boars was used than when semen from Dutch Landrace boars was used (58.6 vs 40.9%); the pattern reversed for insemination with fresh semen (76.5 vs 81%). Boar differences based on farrowing rate ranged from 62 to 92% for fresh semen and from 29 to 72% from frozen semen. There was no inseminator effect or farm effect on farrowing rate. On the basis of these results, frozen semen used for artificial insemination under practical circumstances can be expected to result in a farrowing rate about 30 percentage points lower and a litter size about three pigs smaller than does fresh semen.
Inhibition of growth and adrenal suppression are reported following the use of intranasal beta-methasone (0.1%) in a 9-year-old boy with cystic fibrosis and nasal polyps and in a 3-year-old girl with allergic rhinitis. On stopping treatment catch-up growth occurred and adrenal function returned to normal.
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