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Greenhouse Gas Reduction Pathways: In the UNFCCC Process up to 2025
Meeting the EU objective of limiting global average temperature increase to 2 degrees Celsius above pre-industrial levels requires a peak in global greenhouse gas emissions within the next two decades. This means that early participation of developing countries in global emission control is needed, even under a significant strengthening of the commitments of Annex I countries under the Kyoto Protocol. The study has shown that it is possible to design a set of consistent rules for the attribution of the long-term emission endowments of the different world regions. The gains from participating in global emission trading and from reduced air pollution damage and/or abatement costs does substantially enhance, from a developing country perspective, the attractiveness of an early participation in a regime based on greenhouse gas reduction pathways, provided that the level and the form of their commitment is well designed so as to minimise economic risks
Biochemical-diagnosis of a Fatal Case of Gunther Disease in a Newborn With Hydrops-fetalis
The birth of a male baby was induced at 32 weeks. In utero, the child presented, inter alia, signs of hydrops, hepatosplenomegaly and anaemia. Two in utero transfusions for correction of the anaemia were performed at 28 and 29 weeks, respectively. The baby rapidly presented respiratory distress with mixed acidosis. Three hours after birth, pink urine was excreted. Signs of icterus necessitated phototherapy, after which photosensitivity occurred. Erythrocytes were fluorescent under long-wavelength UV light. The baby died 24 hours after birth, displaying severe acidosis, a diffuse haemorrhagic syndrome, and repeated bradycardia which did not respond to isoprenaline.
The analysis of porphyrins in urine, blood and faeces of the baby gave the following results: 1) uroporphyrin (I and III isomeric series) was increased in urine and faeces, with traces in erythrocytes and plasma; 2) heptacarboxyporphyrin I was found mainly in urine and much less in erythrocytes, plasma and faeces; 3) coproporphyrin I was increased in urine, erythrocytes, plasma and faeces, and 4) 5-aminolaevulinic acid and porphobilinogen in urine and plasma were within the reference ranges.
Determination of the enzymes of haem biosynthesis in erythrocytes and lymphocytes showed that both parents possessed only 50% of the normal activity of cosynthase.
A previously described point mutation in codon 73 was observed in one parent. Fatal cases of neonatal Gunther's disease are extremely rare and such an observation, according to our knowledge, is probably one of the first described
Proximal deletion of chromosome 21 confirmed by in situ hybridization and molecular studies.
Foetal blood sampling was performed at 35 weeks of gestation due to abnormal foetal ultrasound findings. There was apparent monosomy 21 (45,XX,-21) in all mitoses analyzed. The infant died at 37 weeks during delivery. Examination disclosed facial anomalies, clubfeet, hypoplasia of the left urogenital tract, agenesis of corpus callosum, ventricular dilatation, and heterotopias. Reevaluation of the karyotype showed an unbalanced translocation t(1;21) (q44;q22.11) which resulted from a maternal balanced translocation. These findings were confirmed by fluorescence in situ hybridization and molecular studies with chromosome 21 specific markers. The latter showed a proximal deletion of the maternally derived chromosome 21 including all loci from centromere down to the D21S210 locus. This case illustrates the need for complementary cytogenetic and molecular investigations in cases of apparent monosomy 21.Case ReportsJournal ArticleResearch Support, Non-U.S. Gov'tReviewinfo:eu-repo/semantics/publishe