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5. Adeno-associated viral vector serotype 9–based gene therapy for Niemann-Pick disease type A

Author

Samaranch, Lluis, Pérez-Cañamás, Azucena, Soto-Huelin, Beatriz, Sudhakar, Vivek, Jurado-Arjona, Jerónimo, Hadaczek, Piotr, Ávila, Jesús, Bringas, John R, Casas, Josefina, Chen, Haifeng, He, Xingxuan, Schuchman, Edward H, Cheng, Seng H, Forsayeth, John, Bankiewicz, Krystof S, and Ledesma, María Dolores

Subjects
Medical Biotechnology, Biomedical and Clinical Sciences, Biotechnology, Pediatric, Orphan Drug, Rare Diseases, Neurosciences, Genetics, Gene Therapy, Neurodegenerative, 5.2 Cellular and gene therapies, Animals, Brain, Dependovirus, Genetic Therapy, Humans, Inflammation, Injections, Liver, Mice, Knockout, Motor Activity, Niemann-Pick Disease, Type A, Primates, Serogroup, Sphingomyelin Phosphodiesterase, Transgenes, Biological Sciences, Medical and Health Sciences, Medical biotechnology, Biomedical engineering
Abstract

Niemann-Pick disease type A (NPD-A) is a lysosomal storage disorder characterized by neurodegeneration and early death. It is caused by loss-of-function mutations in the gene encoding for acid sphingomyelinase (ASM), which hydrolyzes sphingomyelin into ceramide. Here, we evaluated the safety of cerebellomedullary (CM) cistern injection of adeno-associated viral vector serotype 9 encoding human ASM (AAV9-hASM) in nonhuman primates (NHP). We also evaluated its therapeutic benefit in a mouse model of the disease (ASM-KO mice). We found that CM injection in NHP resulted in widespread transgene expression within brain and spinal cord cells without signs of toxicity. CM injection in the ASM-KO mouse model resulted in hASM expression in cerebrospinal fluid and in different brain areas without triggering an inflammatory response. In contrast, direct cerebellar injection of AAV9-hASM triggered immune response. We also identified a minimally effective therapeutic dose for CM injection of AAV9-hASM in mice. Two months after administration, the treatment prevented motor and memory impairment, sphingomyelin (SM) accumulation, lysosomal enlargement, and neuronal death in ASM-KO mice. ASM activity was also detected in plasma from AAV9-hASM CM-injected ASM-KO mice, along with reduced SM amount and decreased inflammation in the liver. Our results support CM injection for future AAV9-based clinical trials in NPD-A as well as other lysosomal storage brain disorders.

Published
2019

10. Postoperative drift and dose-response of strabismus surgery in thyroid eye disease: predicting desired outcomes with intraoperative adjustable sutures.

Author

Huelin, Fernando J., del Valle, José María Rodriguez, Sales-Sanz, Marco, Ye-Zhu, Cristina, Díaz-Montealegre, Ana, and Muñoz-Negrete, Francisco J.

Abstract

Copyright of Canadian Journal of Ophthalmology is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Published
2024
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11. Neutrophil gelatinase-associated lipocalin is a biomarker of acute-on-chronic liver failure and prognosis in cirrhosis

Author

Ariza, X., Graupera, I., Coll, M., Solà, E., Barreto, R., García, E., Moreira, R., Elia, C., Morales-Ruiz, M., Llopis, M., Huelin, P., Solé, C., Fabrellas, N., Weiss, E., Nevens, F., Gerbes, A., Trebicka, J., Saliba, F., Fondevila, C., Hernández-Gea, V., Fernández, J., Bernardi, M., Arroyo, V., Jiménez, W., Deulofeu, C., Pavesi, M., Angeli, P., Jalan, R., Moreau, R., Sancho-Bru, P., and Ginès, P.

Published
2016
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19. Bézier curves as a total approach to measure the upper lid contour: redefining clinical outcomes in palpebral surgery

Author

Huelin, Fernando J, Sales-Sanz, Marco, Ye-Zhu, Cristina, Cruz, Antonio Augusto V, Muñoz-Negrete, Francisco J, and Rebolleda, Gema

Abstract

AimsTo define and quantify the upper lid contour by adapting Bézier curves with a newly developed software in normal subjects, assessing their reliability.MethodsFifty eyes of 50 healthy patients with no ocular pathology were included in this study from October 2020 to November 2020. All measurements were performed on Bézier curves adjusted to the upper lid contour. An original software was used to measure the radial and vertical midpupil-to-lid margin distances (MPLD), temporal-to-nasal (T/N) ratios, contour peak location and grade of superposition (GS) and asymmetry (GA) indexes. We calculated differences in the variables measured regarding age, gender or the side of the eye being assessed.ResultsThe mean Bézier curve showed an excellent level of inter-rater reliability (intraclass correlation coefficient of 0.99). The median GS index of each eyelid to the mean Bézier curve was 95.4%, 8.5 IQR, and the median GA index was 3%, 3.4 IQR. The mean contour peak location was −0.35 mm, SD 0.45. Overall, the mean central MPLD was 4.1 mm, SD 0.6. No significant differences were found between male and female patients in variables derived from Bézier curves.ConclusionBézier curves may become a very useful tool for the assessment of upper lid contour, contour peak and symmetry. GS and GA indexes, along with the T/N area ratio are potential outcomes for this purpose. All current variables can be obtained just from one single Bézier curve measurement. Our results offer an in-depth exhaustive description of these variables and their distribution in the normal population.

Published
2023
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20. Same-site trabeculectomy with mitomycin and Ologen™ following failed non-penetrating deep sclerectomy

Author

Muñoz-Negrete, FJ, Aguado-Casanova, V, Huelin, FJ, and Rebolleda, G

Abstract

Purpose To assess the effectiveness and safety of same-site trabeculectomy (TRAB) with mitomycin C (MMC) and Ologen™ (Aeon Astron Europe BV. Leiden, The Netherlands) in patients with a failed non-penetrating deep sclerectomy (NPDS).Methods A retrospective study of 24 consecutive eyes (22 patients) undergoing reintervention by same-site TRAB with at least one-year follow-up after failed NPDS. Mean visual acuity (VA), intraocular pressure (IOP) and use of glaucoma medications were compared before and one year after surgery. Early and late postoperative complications were registered. Kaplan-Meier survival analysis was performed according to four levels of success criteria.Results Overall the mean IOP reduced significantly (24.9 ± 7.1 vs. 14.4 ± 4.5 mmHg; p< 0.001), and the number of glaucoma medications (2.80 ± 1.01 vs. 0.55 ± 0.94; p< 0.001) significantly decreased, one year after surgery. The mean VA remained stable one year after surgery (p= 0.516). Hypotony, defined as IOP ≤ 5 mmHg, in the early postoperative period was observed in 62.5% of eyes, but only in 2 patients (8.33%) in the long term. The mean survival time ranged from 10 months (CI 95% 5–15) to 29 months (CI 95%: 26–32) according to the most stringent and lenient success criteria respectively.Conclusion Same-site TRAB augmented with MMC and Ologen™ may provide an effective, safe and lasting alternative following failed NPDS, especially when sparing of the conjunctiva is highly desirable. Postoperative hypotony is the most common postoperative complication.

Published
2023
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21. Phenotype and natural history of elderly onset inflammatory bowel disease: a multicentre, case-control study

Author

Manosa, M, Calafat, M, de Francisco, R, Garcia, C, Casanova, MJ, Huelin, P, Calvo, M, Tosca, J, Fernandez-Salazar, L, Arajol, C, Zabana, Y, Bastida, G, Hinojosa, J, Marquez, L, Barreiro-De-Acosta, M, Calvet, X, Monfort, D, Gomez-Garcia, MR, Rodriguez, E, Huguet, JM, Rojas-Feria, M, Hervias, D, Atienza, R, Busquets, D, Zapata, E, Duenas, C, Charro, M, Martinez-Cerezo, FJ, Plaza, R, Vazquez, JM, Gisbert, JP, Canete, F, Cabre, E, and Domenech, E

Abstract

Background: Onset during old age has been reported in upto 10% of total cases of inflammatory bowel disease (IBD). Aim: To evaluate phenotypic characteristics and the use of therapeutic resources in patients with elderly onset IBD. Methods: Case-control study including all those patients diagnosed with IBD over the age of 60 years since 2000 who were followed-up for >12 months, identified from the IBD databases. Elderly onset cases were compared with IBD patients aged 18 to 40 years at diagnosis, matched by year of diagnosis, gender and type of IBD (adult-onset). Results: One thousand three hundred and seventy-four elderly onset and 1374 adult-onset cases were included (62% ulcerative colitis (UC), 38% Crohn's disease (CD)). Among UC patients, elderly onset cases had a lower proportion of extensive disease (33% vs 39%; P < 0.0001). In CD, elderly onset cases showed an increased rate of stenosing pattern (24% vs 13%; P < 0.0001) and exclusive colonic location (28% vs 16%; P < 0.0001), whereas penetrating pattern (12% vs 19%; P < 0.0001) was significantly less frequent. Regarding the use of therapeutic resources, there was a significantly lower use of corticosteroids (P < 0.0001), immunosuppressants (P < 0.0001) and anti-TNFs agents (P < 0.0001) in elderly onset cases. Regarding surgery, we found a significantly higher surgery rate among elderly onset UC cases (8.3% vs 5.1%; P < 0.009). Finally, elderly onset cases were characterised by a higher rate of hospitalisations (66% vs 49%; P < 0.0001) and neoplasms (14% vs 0.5%; P < 0.0001). Conclusions: Elderly onset IBD shows specific characteristics and they are managed differently, with a lower use of immunosuppressants and a higher rate of surgery in UC.

Published
2018

22. Characterization of systemic inflammatory response in hepatorenal syndrome in cirrhosis. A major role for il-6, TNF-alpha, and VCAM

Author

Sole, C., primary, Solà, E., additional, Morales-Ruiz, M., additional, Huelin, P., additional, Carol, M., additional, Moreira, R., additional, Graupera, I., additional, Pose, E., additional, Napoleone, L., additional, De Prada, G., additional, Ariza, X., additional, Juanola, A., additional, Fabrellas, N., additional, Jiménez, W., additional, and Ginès, P., additional

Published
2018
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23. Chronic kidney disease in patients with cirrhosis surviving an episode of acute kidney injury. Frequency and impact on patient’s outcome

Author

Bassegoda, O., primary, Huelin, P., additional, Pose, E., additional, Napoleone, L., additional, Juanola, A., additional, Moreira, R., additional, Carol, M., additional, De Prada, G., additional, Fabrellas, N., additional, Solé, C., additional, Graupera, I., additional, Solà, E., additional, and Ginès, P., additional

Published
2018
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24. Gut microbiome is profoundly altered in acute-on-chronic liver failure as evaluated by quantitative metagenomics. Relationship with liver cirrhosis severity

Author

Sole, C., primary, Llopis, M., additional, Solà, E., additional, Da Silva, K., additional, Guilly, S., additional, Le-Chatelier, E., additional, Huelin, P., additional, Moreira, R., additional, Carol, M., additional, De Prada, G., additional, Ehrlich, S., additional, Graupera, I., additional, Napoleone, L., additional, Pose, E., additional, Juanola, A., additional, Fabrellas, N., additional, Doré, J., additional, Ehrlich, D., additional, and Ginès, P., additional

Published
2018
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26. Midodrine and albumin for prevention of complications of cirrhosis in patients in the waiting list for liver transplantation. A randomized, multicenter, double-blind, placebo-controlled trial

Author

Solà, E., primary, Solé, C., additional, Simón-Talero, M., additional, Martín-Llahí, M., additional, Castellote, J., additional, Garcia-Martínez, R., additional, Moreira, R., additional, Torrens, M., additional, Márquez, P., additional, Fabrellas, N., additional, Prada, G.D., additional, Huelin, P., additional, Ventura, M., additional, Manríquez, M., additional, Nazar, A., additional, Ariza, X., additional, Suñé, P., additional, Graupera, I., additional, Pose, E., additional, Colmenero, J., additional, Pavesi, M., additional, Guevara, M., additional, Xiol, X., additional, Córdoba, J., additional, Vargas, V., additional, and Ginès, P., additional

Published
2017
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27. New diagnostic algorithm of acute kidney injury in cirrhosis that includes categorization of stage 1 and assessment of urine NGAL. Relevance for the differential diagnosis and clinical outcomes

Author

Huelin, P., primary, Elia, C., additional, Solà, E., additional, Solé, C., additional, Moreira, R., additional, Carol, M., additional, Fabrelles, N., additional, Risso, À., additional, Albertos, S., additional, Glavin, E., additional, de Prada, G., additional, Pose, E., additional, Graupera, I., additional, Morales, M., additional, and Ginès, P., additional

Published
2017
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28. Prevalence of liver steatosis in subjects with risk factors for nonalcoholic fatty liver disease in primary care using controlled attenuation parameter (CAP). A population-based study

Author

Fabrellas, N., primary, Hernandez, R., additional, Solà, E., additional, Coiduras, A., additional, Ramos, P., additional, Graupera, I., additional, Martin, N., additional, Graells, T., additional, Simon, C., additional, Duaso, I., additional, Garcia, M., additional, Larrañaga, A., additional, Perez, A., additional, Sole, C., additional, Huelin, P., additional, Moreira, R., additional, De Prada, G., additional, and Ginés, P., additional

Published
2017
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29. Adipocyte fatty acid binding protein in cirrhosis: increased hepatic gene expression and plasma levels and correlation with clinical outcomes

Author

Graupera, I., primary, Coll, M., additional, Pose, E., additional, Elia, C., additional, Piano, S., additional, Solà, E., additional, Blaya, D., additional, Huelin, P., additional, Solé, C., additional, Moreira, R., additional, de Prada, G., additional, Fabrelles, N., additional, Morales, M., additional, Sancho-Bru, P., additional, and Ginès, P., additional

Published
2017
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30. Phenotypic characteristics and use of therapeutic resources in elderly-onset inflammatory bowel disease: A multicentre, case control study

Author

Manosa, M., Calafat, M., de Francisco, R., GARCIA, C., Casanova, M. J., Huelin, P., Zabana, Y., Calvo, M., Minguez, M., Ruiz, A., Bastida, G., Hinojosa, J., Marquez, L., Barreiro-de-Acosta, M., Calvet, X., Monfort, D., Gomez, R., Rodriguez, E., Huguet, J. M., Rojas, M., Hervas, D., Atienza, R., Busquets, D., Zapata, E., Duenas, C., Charro, M., Martinez-Cerezo, F. J., Plaza, R., Vazquez, J. M., Gisbert, J. P., Cabre, E., and Domenech, E.

Published
2014

34. Management of haemostatic alterations and associated disorders in cirrhosis in Spain: A national survey.

Author

Fortea, José Ignacio, Puente, Ángela, Ezcurra, Iranzu, Cuadrado, Antonio, Arias-Loste, María Teresa, Cabezas, Joaquín, Llerena, Susana, Iruzubieta, Paula, Rodríguez-Lope, Carlos, Huelin, Patricia, Casafont, Fernando, Fábrega, Emilio, and Crespo, Javier

Abstract

Abstract Background Knowledge of haematological abnormalities in cirrhosis has greatly improved in recent years. Aims To evaluate how Spanish Digestive Disease specialists manage haemostatic alterations and associated disorders in patients with cirrhosis. Methods All members of the Spanish Association for the Study of the Liver and Spanish Society of Digestive Pathology were invited to fill in a web-based questionnaire. Results 135 professionals, 93 hepatologists and 42 non-hepatologists responded to the survey. The concept of rebalanced haemostasis was known by 74.8% of them. Most specialists corrected the INR and thrombocytopenia before invasive procedures with moderate risk of bleeding or major surgery and in severe gastrointestinal bleeding. The threshold of platelets and, especially, INR used to administer blood products varied greatly. Pharmacological prophylaxis of venous thromboembolism prevailed, but it was highly dependent on the INR and platelet figures. Most participants initiated anticoagulation regardless of the degree of portal vein thrombosis, even in patients ineligible for transplantation. In potential candidates, only 56% maintained it indefinitely or until liver transplantation. No major differences between hepatologists and non-hepatologists were found. Conclusions A significant variability and certain deviation from current guidelines was observed among Spanish Digestive Disease specialists regarding management of haemostatic alterations and associated disorders in cirrhosis. [ABSTRACT FROM AUTHOR]

Published
2019
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37. Systemic Inflammatory Response Profile in Acute-on-Chronic Liver Failure and its Relationship with Prognosis

Author

Sole, C., primary, Sola, E., additional, Morales-Ruiz, M., additional, Fernàndez, G., additional, Huelin, P., additional, Graupera, I., additional, Moreira, R., additional, De Prada, G., additional, Ariza, X., additional, Pose, E., additional, Fabrellas, N., additional, Kalko, S., additional, Jiménez, W., additional, and Ginès, P., additional

Published
2016
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38. High Levels of Monocyte Chemotactic Protein-1 (MCP-1) are Associated with Readmission Rate and Mortality in Decompensated Cirrhosis

Author

Graupera, I., primary, Solà, E., additional, Moreira, R., additional, Fabrellas, N., additional, Solé, C., additional, Huelin, P., additional, de Prada, G., additional, Pose, E., additional, Ariza, X., additional, Risso, A., additional, Albertos, S., additional, Morales, M., additional, Jiménez, W., additional, and Ginés, P., additional

Published
2016
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39. Lipocalin-2 Gene and its Protein Neutrophil Gelatinase-Associated Lipocalin are Biomarkers of Acute-On-Chronic Liver Failure

Author

Graupera, I., primary, Coll, M., additional, Llopis, M., additional, Ariza, X., additional, Solà, E., additional, Huelin, P., additional, Solé, C., additional, Pose, E., additional, Millán, C., additional, Moreira, R., additional, de Prada, G., additional, Blaya, D., additional, Rodrigo, D., additional, Perea, L., additional, Aguilar, B., additional, Sancho-Bru, P., additional, and Ginés, P., additional

Published
2016
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41. P1336 : Treatment of type 1 hepatorenal syndrome with terlipressin infusion adjusted according to hemodynamic response. The ameliorate study

Author

Huelin, P., primary, Rodriguez, E., additional, Poca, M., additional, Martin-Llahi, M., additional, Elia, C., additional, Moreira, R., additional, Solè, C., additional, Cirera, I., additional, Sanchez, J., additional, Vargas, V., additional, Soriano, G., additional, Arroyo, V., additional, Sola, E., additional, and Gines, P., additional

Published
2015
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42. P1316 : Effects of long-term administration of midodrine and albumin in the prevention of complications in patients with cirrhosis on waiting list for liver transplantation. A randomized, multicenter, double-blind, placebo-controlled trial. macht study

Author

Sola, E., primary, Simon Talero, M., additional, Martin-Llahi, M., additional, Castellote, J., additional, Nazar, A., additional, Solè, C., additional, Ventura, M., additional, Cela, R., additional, Fabrellas, N., additional, Torrens, M., additional, Lopez, E., additional, de Prada, G., additional, Huelin, P., additional, Suñé, P., additional, Guevara, M., additional, Xiol, X., additional, Vargas, V., additional, Arroyo, V., additional, and Gines, P., additional

Published
2015
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43. P0216 : Influence of previous decompensation of cirrhosis on hyperdynamic circulation and response to β-blockers in primary prophylaxis of variceal bleeding

Author

Alvarado, E.A., primary, Pavel, O., additional, Ardèvol, A., additional, Graupera, I., additional, Huelin, P., additional, Colomo, A., additional, Martinez, M., additional, Escajadillo, N.C., additional, Ríos, M.A., additional, Guarner Aguilar, C., additional, and Villanueva, C., additional

Published
2015
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46. PS-016 - Midodrine and albumin for prevention of complications of cirrhosis in patients in the waiting list for liver transplantation. A randomized, multicenter, double-blind, placebo-controlled trial

Author

Solà, E., Solé, C., Simón-Talero, M., Martín-Llahí, M., Castellote, J., Garcia-Martínez, R., Moreira, R., Torrens, M., Márquez, P., Fabrellas, N., Prada, G.D., Huelin, P., Ventura, M., Manríquez, M., Nazar, A., Ariza, X., Suñé, P., Graupera, I., Pose, E., Colmenero, J., Pavesi, M., Guevara, M., Xiol, X., Córdoba, J., Vargas, V., and Ginès, P.

Published
2017
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48. P606 Phenotypic characteristics and use of therapeutic resources in elderly-onset inflammatory bowel disease: A multicentre, case-control study

Author

Mañosa, M., primary, Calafat, M., additional, de Francisco, R., additional, Garcia, C., additional, Casanova, M.J., additional, Huelin, P., additional, Zabana, Y., additional, Calvo, M., additional, Mínguez, M., additional, Ruiz, A., additional, Bastida, G., additional, Hinojosa, J., additional, Márquez, L., additional, Barreiro-de-Acosta, M., additional, Calvet, X., additional, Monfort, D., additional, Gómez, R., additional, Rodríguez, E., additional, Huguet, J.M., additional, Rojas, M., additional, Hervas, D., additional, Atienza, R., additional, Busquets, D., additional, Zapata, E., additional, Dueñas, C., additional, Charro, M., additional, Martínez-Cerezo, F.J., additional, Plaza, R., additional, Vázquez, J.M., additional, Gisbert, J.P., additional, Cabré, E., additional, and Domènech, E., additional

Published
2014
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49. The effect of density on short rotation Populus sp. plantations in the Mediterranean area

Author

Cañellas, I. [0000-0002-9716-7776], Ciria, Pilar [0000-0002-4798-1550], Sixto Blanco, Hortensia Concepción [0000-0001-7188-4438], Gea Izquierdo, Guillermo [0000-0003-0148-3721], Cañellas, Isabel, Huelin, P., Hernández, María José, Ciria, Pilar, Calvo, R., Saz-Sánchez, Miguel Ángel, Sixto Blanco, Hortensia Concepción, Gea Izquierdo, Guillermo, Cañellas, I. [0000-0002-9716-7776], Ciria, Pilar [0000-0002-4798-1550], Sixto Blanco, Hortensia Concepción [0000-0001-7188-4438], Gea Izquierdo, Guillermo [0000-0003-0148-3721], Cañellas, Isabel, Huelin, P., Hernández, María José, Ciria, Pilar, Calvo, R., Saz-Sánchez, Miguel Ángel, Sixto Blanco, Hortensia Concepción, and Gea Izquierdo, Guillermo

Abstract

In this study, the effect of density on poplar short-rotation forestry plantations was analyzed in the context of maximizing biomass production under Mediterranean conditions. Data from 12 experimental sites in Spain with densities ranging from 6666 to 33,333 cuttings·ha -1 were used. At the end of the first year, biomass production from the different poplar plantations differed significantly among sites and densities. Furthermore, after one year of growth, biomass production was found to increase exponentially with plot density. However, after three years of growth, at the end of the first rotation, significant differences were not found. When we analyzed the variables that affect the production, we found that weed control and therefore a reduction in competition explained much of the variability. The greatest biomass production was achieved in high density plots in more southern latitudes where high spring temperatures benefit trees growing in well-watered soils. Considering on the one hand the scope of current weed control methods and on the other, the cost of plantation and/or the difficulties associated with management and mechanisation, plantation densities of more than 15,000 plants ha -1 would not be recommendable. © 2012 Elsevier Ltd.

Published
2012

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