Competition in complementary goods: Airport handling markets and Council Directive 96/67/EC

This paper addresses the case of complementary services with vertical relations. Using the example of airport handling activities, we develop a model to investigate the effects on welfare and competitiveness of four different handling market situations. We find out that the usual Cournot result on welfare when firms compete in complementary goods is verified unless there are efficiency gaps between the firms, or if vertically related firms also compete on the same market. We also find that the presence of a horizontally integrated firm may lead to market foreclosure. Moreover, we add a few remarks on regulatory issues, where we show that regulation may be pointless or even anti-competitive. In particular, we show that Council Directive 96/67/EC, while intending to increase competition, may lead to anti-competitive situations and consumers surplus decreases.Complementary goods competition; airport handling; vertical relations.

A propósito da racionalidade camponesa: universalidade de comportamentos ou graus de racionalidade?

While studying Peasant Ractionality, one comes across to problems which mostly relate to Instrumental Rationality. This article explores such problems, and analyses those (few) approaches to Instrumental Rationality that defy the Maximization thesis. Then, some clues for the understanding of Peassant Rationality are provided.Ao estudar a Racionalidade Camponesa, depara-se, sobretudo, com problemas do âmbito da Racionalidade Instrumental. Este artigo explora tais problemas, e analisa as (poucas) abordagens da Racionalidade Instrumental quo desafiam a tese da Maximização. Finalmente, são fornecidas algumas pistas para a compreensão da Racionalidade Camponesa

A Economia da pequena agricultura no Noroeste Português : Algumas contribuições

Dissertação de Doutoramento em Economia, área de especialização em Mesoeconomia, apresentada à Faculdade de Economia da Universidade do Port

Anemia de Fanconfi — Variabilidade Fenotípica da Doença em Bulas Irmãs

Os autores apresentam o caso de uma jovem de 15 anos de idade, sexo feminino, com antecedentes de infecções do tracto urinário de repetição e com malformações congénitas esqueléticas e hipoplasia renal direita, orientada para estudo de bicitopenia (leucopenia e trombocitopenia ligeiras). Apresentava macrocitose e hemoglobina fetal aumentada. A cultura de linfócitos com diepoxibutano revelou instabilidade cromossómica característica da Anemia de Fanconi.A irmã da doente, de 9 anos de idade, sem qualquer malformação congénita detectável e sem alterações hematológicas para além de macrocitose ligeira apresentava uma instabilidade cromossómica sobreponível à da irmã

Anemia de Fanconi - variabilidade fenotípica da doença em duas irmâs

Os autores apresentam o caso de uma jovem de 15 anos de idade, sexo feminino, com antecedentes de infeções do tracto urinário de repetição e com malformações congénitas esqueléticas e hipoplasia renal direita, orientada para estudo de bicitopenia (leucopenia e trombocitopenia ligeiras). Apresentava macrocitose e hemoglobina fetal aumentada. A cultura de linfócitos com diepoxibutano revelou instabilidade cromoss6mica característica da Anemia de Fanconi. A irmã da doente, de 9 anos de idade, sem qualquer malformação congénita detectável e sem alteraç6es hematol6gicas para além de macrocitose ligeira apresentava uma instabilidade cromossómica sobreponível a da irmã

Increased red cell distribution width in Fanconi anemia: a novel marker of stress erythropoiesis

BACKGROUND: Red cell distribution width (RDW), a classical parameter used in the differential diagnosis of anemia, has recently been recognized as a marker of chronic inflammation and high levels of oxidative stress (OS). Fanconi anemia (FA) is a genetic disorder associated to redox imbalance and dysfunctional response to OS. Clinically, it is characterized by progressive bone marrow failure, which remains the primary cause of morbidity and mortality. Macrocytosis and increased fetal hemoglobin, two indicators of bone marrow stress erythropoiesis, are generally the first hematological manifestations to appear in FA. However, the significance of RDW and its possible relation to stress erythropoiesis have never been explored in FA. In the present study we analyzed routine complete blood counts from 34 FA patients and evaluated RDW, correlating with the hematological parameters most consistently associated with the FA phenotype. RESULTS: We showed, for the first time, that RDW is significantly increased in FA. We also showed that increased RDW is correlated with thrombocytopenia, neutropenia and, most importantly, highly correlated with anemia. Analyzing sequential hemograms from 3 FA patients with different clinical outcomes, during 10 years follow-up, we confirmed a consistent association between increased RDW and decreased hemoglobin, which supports the postulated importance of RDW in the evaluation of hematological disease progression. CONCLUSIONS: This study shows, for the first time, that RDW is significantly increased in FA, and this increment is correlated with neutropenia, thrombocytopenia, and highly correlated with anemia. According to the present results, it is suggested that increased RDW can be a novel marker of stress erythropoiesis in FA.info:eu-repo/semantics/publishedVersio

Spectrum of MMACHC mutations in Italian and Portuguese patients with combined methylmalonic aciduria and homocystinuria, cblC type

Methylmalonic aciduria (MMA) and homocystinuria, cblC type (MIM 277400) is the most frequent inborn error of vitamin B-12. The recent identification of the disease gene, MMACHC, has permitted preliminary genotype-phenotype correlations. We studied 24 Italian and 17 Portuguese patients with cblC defect to illustrate the spectrum of mutations in a southern European population and discuss the impact that mutation identification has on routine diagnostic procedures. Since the metabolic defect raises the serum levels of homocysteine, we also tested if variants in MTHFR-playing a key role in homocysteine remethylation pathway-could act as genetic modifier in cblC defect. We found that the c.271 dupA (accounting for 55% of the MMA CH alleles in our cohort) followed by c.394C > T (16%) and c.331C > T (9%) were the most frequent mutations. In our study we also identified a novel mutation (c.544T > C). On the other hand, the MTHFR genotype did not appear to influence age at onset, the clinical phenotype and outcome of patients with cblC defect. This study shows that mutation screening for the most common MMACH mutations occurring in early-onset forms (c.271dupA and c.331C > T) seems to have a high diagnostic yield in a southern European population with cblC defect. Although the identification of the gene defect per se does not predict completely time and severity of disease appearance, our data corroborate the importance of a molecular testing to offer accurate prenatal diagnosis to couples at high risk of having affected children. (C) 2007 Elsevier Inc. All rights reserved

Additional file 1: of Increased red cell distribution width in Fanconi anemia: a novel marker of stress erythropoiesis

Required informations about the unpublished data referred in the main manuscript. (PDF 463 kb

Origin, functional role, and clinical impact of Fanconi anemia FANCA mutations

Fanconi anemia is characterized by congenital abnormalities, bone marrow failure, and cancer predisposition. To investigate the origin, functional role, and clinical impact of FANCA mutations, we determined a FANCA mutational spectrum with 130 pathogenic alleles. Some of these mutations were further characterized for their distribution in populations, mode of emergence, or functional consequences at cellular and clinical level. The world most frequent FANCA mutation is not the result of a mutational “hot-spot” but results from worldwide dissemination of an ancestral Indo-European mutation. We provide molecular evidence that total absence of FANCA in humans does not reduce embryonic viability, as the observed frequency of mutation carriers in the Gypsy population equals the expected by Hardy-Weinberg equilibrium. We also prove that long distance Alu-Alu recombination can cause Fanconi anemia by originating large interstitial deletions involving FANCA and 2 adjacent genes. Finally, we show that all missense mutations studied lead to an altered FANCA protein that is unable to relocate to the nucleus and activate the FA/BRCA pathway. This may explain the observed lack of correlation between type of FANCA mutation and cellular phenotype or clinical severity in terms of age of onset of hematologic disease or number of malformations