Chapter Drapers and tailors. Fashion and consumption in medieval Catalonia

The range of textiles available in markets across the western Mediterranean expanded significantly during the thirteenth century. Cloth retailers, or drapers, constituted a fundamental link between merchants and consumers, using a network of local markets with specific spaces for selling cloth. They were able to sell a wide range of commodities, including Flemish and French woollens, to satisfy a growing demand. Between 1250 and 1350, there were also tailors almost everywhere, some at the permanent service of an aristocratic court, such as the kings of Aragon, but most of them worked as independent entrepreneurs offering their services in exchange for specific payments. Therefore both drapers and tailors formed small partnerships and frequently used credit in order to reach all levels of medieval society

Las Mujeres en la sociedad catalana de los siglos IX al XI

In the middle ages (9th to 11th centuries), women from the Catalan counties possessed land obtained through parental inheritance, indirect dowry (decimum), dower or other mechanisms according to Visigothic law. Nevertheless, the total amount of land under women's effective control was quite small as can be seen in the abundant 10th century evidence. Their large participation in land sales can be related to their own difficulties in achieving real economic autonomy despite their legal rights, especially during widowhood.En la edad media (de los siglos IX al XI), las mujeres de los condados catalanes poseían tierras obtenidas mediante la herencia paterna, la dote marital (decimum), el derecho de viudedad u otros mecanismos previstos por la ley visigoda. Sin embargo la cantidad de tierras bajo control efectivo de las mujeres era bastante reducido tal como puede observarse en la rica documentación del siglo X. Su amplia participación en las ventas de tierras puede relacionarse con sus dificultades en asegurarse una verdadera autonomía económica a pesar de sus derechos legales, especialmente durante la viudedad

Direct-acting antivirals for hepatitis C virus induce a rapid clinical and biochemical remission of porphyria cutanea tarda

Sporadic porphyria cutanea tarda (PCT) is strongly associated with hepatitis C virus (HCV) infection in our population. 1-4 Therapeutic options for PCT include phlebotomies and low-dose 4-aminoquinolines, which show high rates of disease remission. However, some patients with PCT may present frequent relapses attributable to resistance, intolerability or poor adherence to conventional treatments and/or persistence of risk factors. In 2014, we reported the first case of a patient with active PCT and HCV-HIV coinfection cured with direct antiviral agents (DAAs).5 Herein, we present a cohort of patients demonstrating rapid and persistent remission of PCT with DAA regimes administered for HCV infection

Urinary Porphyrin Excretion in Children is Associated with Exposure to Organochlorine Compounds

4 pages, 4 tables.-- 18941586 [PubMed].-- PMCID: PMC2569103.-- Printed version published Oct 2008.Background Hexachlorobenzene (HCB) and other organochlorines induce porphyria cutanea tarda (PCT) in animal studies. Evidence in humans, however, is contradictory. In neonates and adults from a population historically highly exposed to HCB (Flix, Catalonia, Spain), no relation with PCT or with porphyrin excretion was found.Objectives We aimed to analyze the association between urinary porphyrin excretion and exposure to HCB and other organochlorinated compounds in children 4 years of age.Methods Our birth cohort included all newborns from Flix and the five surrounding towns (where no airborne pollution occurred). Among the 68 children with porphyrins we measured in cord blood, 52 children 4 years of age provided blood to measure organochlorine compounds, hair for methylmercury, and urine for porphyrin excretion pattern.Results Quantitative porphyrin excretion was within the normal values. However, total porphyrins, coproporphyrin I (CPI), and coproporphyrin III (CPIII) adjusted to creatinine excretion increased with increasing levels of HCB, 1,1-dichloro-2,2-bis(4-chlorophenyl)ethylene (p,p′-DDE), 1,1,1-trichloro-2,2-bis(4-chlorophenyl)ethane (p,p′-DDT), and polychlorinated biphenyl congener 153 (PCB-153). We found no association with methylmercury. When we fitted multiple pollutant models, p,p′-DDE had the strongest association. We found these associations in children from both Flix and other towns, and they were independent of breast-feeding and of organochlorine and porphyrin levels at birth.Conclusion HCB at current levels did not induce porphyria or increase uroporphyrins. However, the increase of urinary coproporphyrins suggests an incipient toxic effect of the organochlorines, especially for p,p′-DDE, on the hepatic heme-synthesis pathway that differs from the major effects seen in PCT.This study was funded by the Spanish Ministry of Health (FIS-97/1102, FIS-PI041436, Red INMA G03/176, and CB06/02/0041), “Fundació La Caixa” (97/009-00 and 00/077-00), and Generalitat de Catalunya-CIRIT 1999SGR 00241.Peer reviewe

European Specialist Porphyria Laboratories: Diagnostic Strategies, Analytical Quality, Clinical Interpretation, and Reporting As Assessed by an External Quality Assurance Program

BACKGROUND: The porphyrias are a group of rare metabolic disorders whose diagnosis depends on identification of specific patterns of porphyrin precursor and porphyrin accumulation in urine, blood, and feces. Diagnostic tests for porphyria are performed by specialized laboratories in many countries. Data regarding the analytical and diagnostic performance of these laboratories are scarce. METHODS: We distributed 5 sets of multispecimen samples from different porphyria patients accompanied by clinical case histories to 18–21 European specialist porphyria laboratories/centers as part of a European Porphyria Network organized external analytical and postanalytical quality assessment (EQA) program. The laboratories stated which analyses they would normally have performed given the case histories and reported results of all porphyria-related analyses available, interpretative comments, and diagnoses. RESULTS: Reported diagnostic strategies initially showed considerable diversity, but the number of laboratories applying adequate diagnostic strategies increased during the study period. We found an average interlaboratory CV of 50% (range 12%–152%) for analytes in absolute concentrations. Result normalization by forming ratios to the upper reference limits did not reduce this variation. Sixty-five percent of reported results were within biological variation–based analytical quality specifications. Clinical interpretation of the obtained analytical results was accurate, and most laboratories established the correct diagnosis in all distributions. CONCLUSIONS: Based on a case-based EQA scheme, variations were apparent in analytical and diagnostic performance between European specialist porphyria laboratories. Our findings reinforce the use of EQA schemes as an essential tool to assess both analytical and diagnostic processes and thereby to improve patient care in rare diseases

Evaluation of metabolic changes in acute intermittent porphyria patients by targeted metabolomics

Acute intermittent porphyria (AIP) is an inherited rare hepatic disorder due to mutations within the hydroxymethylbilane gene. AIP patients with active disease overproduce aminolevulinic acid (ALA) and porphobilinogen (PBG) in the liver which are exported inducing severe neurological attacks. Different hepatic metabolic abnormalities have been described to be associated with this condition. The goal of this research was to explore the metabolome of symptomatic AIP patients by state-of-the art liquid chromatography-tandem mass spectrometry (LC-MS/MS). A case versus control study including 18 symptomatic AIP patients and 33 healthy controls was performed. Plasmatic levels of 51 metabolites and 16 ratios belonging to four metabolic pathways were determined. The results showed that the AIP patients presented significant changes in the two main areas of the metabolome under study: (a) the tryptophan/kynurenine pathway with an increase of tryptophan in plasma together with increase of the kynurenine/tryptophan ratio; and (b) changes in the tricarboxylic acid cycle (TCA) including increase of succinic acid and decrease of the fumaric acid/succinic acid ratio. We performed a complementary in vitro study adding ALA to hepatocytes media that showed some of the effects on the TCA cycle were parallel to those observed in vivo. Our study confirms in plasma previous results obtained in urine showing that AIP patients present a moderate increase of the kynurenine/tryptophan ratio possibly associated with inflammation. In addition, it also reports changes in the mitochondrial TCA cycle that, despite requiring further research, could be associated with an energy misbalance due to sustained overproduction of heme-precursors in the liver.This research was funded by Instituto de Salud Carlos III FEDER (grant number PI14/00147), Generalitat de Catalunya (research team grant number 2014SGR692) and Spanish Health National System (contract number CPII16/00027 for Oscar J Pozo).Peer ReviewedPostprint (published version