[A newborn infant with hyperventilation]. / Et nyfødt barn med hyperventilasjon.

Respiratory alkalosis is an early sign of urea cycle disorder. A high level of plasma ammonia will strengthen this suspicion. It is of great importance to transfer the infant as soon as possible to a unit capable of giving specific treatment with Na-benzoate, Na-phenylbutyrate, argininchloride and carglumic acid. The early treatment may also include haemodialysis, which is preferred over peritoneal dialysis or exchange transfusion. We here describe an infant with respiratory alkalosis within the first two days of life and a high plasma level of ammonia (> 700 micromol/L). He did not respond to conventional therapy and died 48 hours after birth in spite of specific treatment. DNA-analysis showed a gene defect in the OTC gene, c.67C >T (p.R23X), a known mutation leading to urea cycle disorder (OTC). It is important to detect carriers among older siblings and to inform the parents of the possibility of prenatal diagnostics.

Energetic mapping of Ni catalysts by detailed kinetic modeling.

Temperature-programmed desorption (TPD) of CO has been performed on supported and unsupported nickel catalysts. The unsupported Ni catalyst consists of a Ni(14 13 13) single crystal which has been studied under ultrahigh vacuum conditions. The desorption energy for CO at low CO surface coverage was found to be 119 kJ/mol, and the binding energy of C to the Ni(111) surface of the crystal was 703 kJ/mol. The supported catalysts consist of nickel supported on hydrotalcite-like compounds with three different Mg2+/Al3+ ratios. The experimental results show that for the supported Ni catalysts TPD of CO leads to desorption of both CO and CO2, with the latter being dominant. Dissociation of CO takes place, and considerable amounts of residue C are left on the surface. The residue C is removed by temperature-programmed oxidation (TPO). The results show that a low Mg2+/Al3+ ratio in the hydrotalcite precursor seems to result in more steplike sites, kinks, and defects for carbon monoxide dissociation. A detailed kinetic modeling of the TPO results based on elementary reaction steps has been conducted to give an energetic map of supported Ni catalysts. Experimental results from the ideal Ni surface fit nicely with literature values, providing useful information for identifying active sites on supported Ni catalysts.