Determination of nutrient salts by automatic methods both in seawater and brackish water: the phosphate blank

9 páginas, 2 tablas, 2 figurasThe main inconvenience in determining nutrients in seawater by automatic methods is simply solved: the preparation of a suitable blank which corrects the effect of the refractive index change on the recorded signal. Two procedures are proposed, one physical (a simple equation to estimate the effect) and the other chemical (removal of the dissolved phosphorus with ferric hydroxide).Support for this work came from CICYT (MAR88-0245 project) and Conselleria de Pesca de la Xunta de GaliciaPeer reviewe

Shrouded by time and tile: a structural investigation for preservation of Cuba’s historic School of Ballet classroom and theatre domes

Vittorio Garatti’s School of Ballet in Havana, Cuba, is internationally acknowledged as a signifi cant architectural and cultural icon whose survival is even now jeopardized by degradation such as moisture encroachment and corrosion, excessive defl ections, and cracking of primary elements. While Garatti’s vaulted forms symbolize the idealistic egalitarianism and free expression of their time of origin (1961-1964), their current state bears the story of the ensuing decades, in which practical, social, and environmental mechanisms have each played a part. This work comprised a multi-stage structural investigation aiming to elucidate the theatre and classroom domes’ authentic structural condition and assess the behaviour and effi ciency of these unique structural systems. Archival research and site investigations supplied the domes’ construction techniques, geometry, and properties, which informed geometric and mechanical models. Detailed inspection disclosed their condition and behaviour. Then, non-linear fi nite element analysis with a concrete damaged-plasticity model simulated structural damage, yielding conclusions about their current condition

The First Case of 3-Methylcrotonyl-CoA Carboxylase (MCC) Deficiency Responsive to Biotin

3-Methylcrotonylglycinuria is an inborn error of leucine catabolism with an autosomal recessive pattern of inheritance that results from a deficiency of 3-methylcrotonyl-CoA carboxylase (MCC). We report on a nine-year-old boy with severe psychomotor retardation who developed infantile spasms at the age of three weeks. Urine analysis at the age of two years revealed massive 3-methylcrotonylglycinuria and 3-hydroxyisovaleric aciduria suggesting MCC deficiency. Carnitine serum levels were decreased. Biotin therapy led to a dramatic decrease in the frequency of seizures, disappearance of hypsarrhythmia, and near normalisation of organic aciduria. Four months later a protein-restricted diet was introduced in addition and the boy remained clinically and metabolically stable. However, severe psychomotor delay persisted, and the seizures partially reoccurred. Biochemical findings showed partial MCC deficiency in cultured fibroblasts. Molecular genetic studies revealed a heterozygote missense mutation, MCCA-R385S, converting arginine to serine in a highly conserved region of the MCCA gene. This is the first patient with MCC deficiency caused by a heterozygote mutation and who demonstrated a substantial and sustained clinical and biochemical response to therapeutic doses of biotin. Sadly, this patient again also demonstrates that the main determinant of the outcome of even easily treatable metabolic diseases is timely diagnosis