En base a unos trabajos de campo desarrollados entre 1995 y 2015 en un pueblo de arroceros en el suroeste de China como artista-investigador, examino en este texto los cambios profundos que afectan a ...los territorios fronterizos rurales habitados por las minorías nacionales no-Han y, en particular, la de los Hani en el valle del río Rojo en Yunnan. ¿Cuál es el futuro para su modelo ancestral de cultivo del arroz en terrazas y cuáles son los retos a los que debe hacer frente? Mi trabajo toma un enfoque sensible a través de la fotografía, el paisaje pintado, hasta las obras de artistas chinos de arte contemporáneo, y examina las políticas patrimoniales de planificación del paisaje. Los retos de la preservación no son sólo medioambientales -biodiversidad, desarrollo sostenible-, son también identitarios -culturas vernaculares y minoritarias. Señalaré los límites y expondré el futuro.
Suite à un travail de terrain entre 1995 et 2015, dans un village de riziculteurs du Sud-Ouest de la Chine, en tant qu’artiste-chercheuse, j’examine dans ce texte les profondes mutations qui ...traversent les territoires frontaliers ruraux habités par les minorités nationales non-Han et en particulier ceux des Hani de la vallée du fleuve Rouge au Yunnan. Quel devenir pour leur modèle ancestral de riziculture irriguée en terrasse et quels sont les enjeux auxquels ils font face ? Mon travail croise une approche sensible par la photographie, le paysage peint, jusqu’aux œuvres des artistes chinois de l’art contemporain, et examine les politiques patrimoniales d’aménagement du paysage. Les enjeux de la préservation ne sont pas seulement environnementaux - biodiversité, développement durable, ils sont identitaires - cultures vernaculaires et minoritaires. J’en pointerai les limites et esquisserai le devenir.
Between 1995 and 2015, I conducted fieldwork in a rice-growing village in southwest China as an artist-researcher. In this text, I examine the profound changes taking place in rural border areas ...inhabited by non-Han minorities, and particularly those of the Hani in the Yunnan Red River Valley. What future is there for the Hani ancestral model of irrigated rice terraces, and what are the stakes? My work combines a sensitive approach through diverse artistic representations of landscapes – from classical Chinese art to the works of contemporary artists, including mine – and examines landscape planning and patrimonial policies. The conservation stakes are not only environmental – biodiversity, sustainable development – but also identity-based through vernacular and minority cultures. I will point out the limits of this landscape planning and outline its future.
Chronic kidney disease (CKD) concerns millions of individuals worldwide, with few therapeutic strategies available to date. Recent evidence suggests that the endocannabinoid system (ECS) could be a ...new therapeutic target to prevent CKD. ECS combines receptors, cannabinoid receptor type 1 (CB1R) and type 2 (CB2R), and ligands. The most prominent receptor within the kidney is CB1R, its endogenous local ligands being anandamide and 2-arachidonoylglycerol. Therefore, the present review focuses on the therapeutic potential of CB1R and not CB2R. In the normal kidney, CB1R is expressed in many cell types, especially in the vasculature where it contributes to the regulation of renal hemodynamics. CB1R could also participate to water and sodium balance and to blood pressure regulation but its precise role remains to decipher. CB1R promotes renal fibrosis in both metabolic and non-metabolic nephropathies. In metabolic syndrome, obesity and diabetes, CB1R inhibition not only improves metabolic parameters, but also exerts a direct role in preventing renal fibrosis. In non-metabolic nephropathies, its inhibition reduces the development of renal fibrosis. There is a growing interest of the industry to develop new CB1R antagonists without central nervous side-effects. Experimental data on renal fibrosis are encouraging and some molecules are currently under early-stage clinical phases (phases I and IIa studies). In the present review, we will first describe the role of the endocannabinoid receptors, especially CB1R, in renal physiology. We will next explore the role of endocannabinoid receptors in both metabolic and non-metabolic CKD and renal fibrosis. Finally, we will discuss the therapeutic potential of CB1R inhibition using the new pharmacological approaches. Overall, the new pharmacological blockers of CB1R could provide an additional therapeutic toolbox in the management of CKD and renal fibrosis from both metabolic and non-metabolic origin.
Chronic kidney disease (CKD) is one of the main long-term prognosis factors in methylmalonic acidemia (MMA), a rare disease of propionate catabolism. Our objective was to precisely address the ...clinical and biological characteristics of long-term CKD in MMA adolescent and adult patients.
In this retrospective study, we included MMA patients older than 13 years who had not received kidney and/or liver transplantation. We explored tubular functions, with special attention to proximal tubular function. We measured glomerular filtration rate (mGFR) by iohexol clearance and compared it to estimated glomerular filtration rate (eGFR) by Schwartz formula and CKD-EPI.
Thirteen patients were included (M/F = 5/8). Median age was 24 years (13 to 32). Median mGFR was 57 mL/min/1.73 m
(23.3 to 105 mL/min/1.73 m
). Ten out of 13 patients had mGFR below 90 mL/min/1.73 m
. No patient had significant glomerular proteinuria. No patient had complete Fanconi syndrome. Only one patient had biological signs suggestive of incomplete proximal tubulopathy. Four out of 13 patients had isolated potassium loss, related to a non-reabsorbable anion effect of urinary methylmalonate. Both Schwartz formula and CKD-EPI significantly overestimated GFR. Bias were respectively 16 ± 15 mL/min/1.73 m
and 37 ± 22 mL/min/1.73 m
.
CKD is a common complication of the MMA. Usual equations overestimate GFR. Therefore, mGFR should be performed to inform therapeutic decisions such as dialysis and/or transplantation. Mild evidence of proximal tubular dysfunction was found in only one patient, suggesting that other mechanisms are involved.
Full text
Available for:
IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
Patients with maple syrup urine disease (MSUD) experiencing metabolic decompensations have traditionally been treated with branched-chain amino acid (BCAA)-free mixture via oral or nasogastric ...administration routes. In some patients, enteral administration is not possible, either because the patient presents with vomiting, coma, or refuses nasogastric administration, thus intravenous (IV) BCAA-free solution is an appropriate intervention for these challenging cases.
This study aimed to evaluate the effectiveness and safety of managing metabolic decompensations by administering an IV BCAA-free solution.
This is an observational prospective study of data from MSUD patients hospitalised for decompensation episodes between 2010 and 2016 at 6 centres for rare metabolic diseases in France.
A total of 24 patients (16 males; 8 females) experiencing 126 MSUD metabolic decompensation episodes (39 in children; 87 in adults) were admitted to hospital. At presentation, mean leucine plasma concentration was ≥ 381 µmol/L in 113/126 (89.7%) episodes. Children were treated with continuous IV BCAA-free solution at doses of 0.8 to 2.0 g/kg/day, for 4.8 days and adults for 3.8 days at doses of 0.5 to 2.6 g/kg/day. In the efficacy set of 102 analysable episodes leucine concentrations were normalised (to below 381 µmol/L) in 82% (n = 18/22) of episodes in children and in 84% (n = 67/80) of episodes in adults. Mean time to leucine normalisation was 3.0 days. This was significantly (p < 0.001) shorter than the algorithmically predicted time to leucine normalisation with traditional BCAA-free mixture. Duration of hospitalisation was significantly longer for children than for adults (7.1 days in children vs 5.2 days in adults, p = 0.012). No treatment-related adverse events were reported in any patients on IV BCAA-free solution.
The IV BCAA-free solution is safe and effective in normalising leucine concentrations during MSUD decompensation episodes in both children and adults, offering a practical treatment alternative for those patients who cannot receive BCAA-free mixture via oral or nasogastric routes.
Full text
Available for:
IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
In patients receiving preoperative chemotherapy, colorectal liver metastases (CLM) are expected to demonstrate a similar behaviour because of similar organ microenvironment and tumour cell ...chemosensitivity. We focused on the occurrence of pathological and genetic heterogeneity within CLM.
Patients resected for multiple CLM between 2004 and 2011 after > three cycles of chemotherapy were included. Pathological heterogeneity was arbitrarily defined as a > 50% difference in the percentage of remaining tumour cells between individual CLM. In patients with pathological heterogeneity, the mutational genotyping (KRAS, NRAS, BRAF and PIK3CA) was determined from the most heterogeneous CLM.
Pathological heterogeneity was observed in 31 of 157 patients with multiple CLM (median = 4, range, 2-32) (19.7%). In 72.4% of them, we found a concordance of the mutation status between the paired CLM: both wild-type in 55%, and both mutated in 17.2%. We observed a discordance of the mutation status of 27.6% between CLM: one mutated and the other wild-type. The mutated CLM was the less florid one in 75% of patients with genetic heterogeneity.
Pathological heterogeneity is present in 19.7% of patients with multiple CLM. Genetic heterogeneity is present in 27.6% of patients with pathological heterogeneity. Heterogeneity could refine guide management for tissue sampling.
Maple syrup urine disease (MSUD) is a rare inborn metabolic disorder, managed with a strict protein‐restricted diet. At any time or age patients may still experience metabolic decompensations, ...requiring administration of branched chain amino acid (BCAA)‐free formula to reduce leucine levels. This retrospective observational study of 126 decompensation episodes from 54 MSUD patients treated at five centers in France and Germany from 2010 to 2016, describes episodes and outcomes for patients stratified into groups who received enteral/oral or intravenous (IV) BCAA‐free formula, and by pediatric or adult age categories. IV administration of BCAA‐free formula was required in cases of gastric intolerance (33%), refusal to undergo nasogastric tubing (31%), “emergency” (14%) or coma patients (8%), and as prophylaxis before surgery (6%). Overall, mean duration of hospitalization was 6.6 days with oral/enteral BCAA‐free formula and 5.4 days with IV formula. Leucine levels at discharge decreased by a mean of 548.5 μmol/L (69.3%) in the oral/enteral group and 657.2 μmol/L (71.3%) in the IV group. In the pediatric subgroup, there were no marked differences between administration groups on any outcome. In the adult subgroup, mean time to episode resolution was 15.8 days in the oral/enteral group and 7.7 days in the IV group (P = .008); mean duration of hospitalization was 6 days in the oral/enteral group and 4.6 days in the IV group (P = NS). Overall, seven serious adverse events in two patients were reported, of which only nausea and vomiting were treatment related.
Full text
Available for:
FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
En base a uns treballs de camp desenvolupats entre 1995 i 2015 en un poble d’arrossaires al sud-oest de la Xina com a artista-investigador, examino en aquest text els canvis profunds que afecten els ...territoris fronterers rurals habitats per les minories nacionals no-Han i, en particular, la dels Hani a la vall del riu Vermell a Yunnan. Quin és el futur per al seu model ancestral de cultiu de l’arròs en terrasses i quins són els reptes als quals ha de fer front? El meu treball pren un enfocament sensible a través de la fotografia, el paisatge pintat, fins a les obres d’artistes xinesos d’art contemporani, i examina les polítiques patrimonials de planificació del paisatge. Els reptes de la preservació no són només mediambientals -biodiversitat, desenvolupament sostenible-, són també identitaris -cultures vernaculars i minoritàries. Assenyalaré els límits i exposaré el futur.
PDF
