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1. A Multi‑Center, Open‑Label, Single‑Arm Trial to Evaluate the Efficacy, Pharmacokinetics, and Safety and Tolerability of IGSC 20% in Subjects with Primary Immunodeficiency

Author

Santamaria, Manuel, Neth, Olaf, Douglass, Jo A., Krivan, Gergely, Kobbe, Robin, Bernatowska, Ewa, Grigoriadou, Sofia, Bethune, Claire, Chandra, Anita, Horneff, Gerd, Borte, Michael, Sonnenschein, Anja, Kralickova, Pavlina, Ramón, Silvia Sánchez, Langguth, Daman, Gonzalez-Granado, Luis Ignacio, Alsina, Laia, Querolt, Montse, Griffin, Rhonda, Hames, Carrie, Mondou, Elsa, Price, Jeffrey, Sanz, Ana, and Lin, Jiang

Published
2022
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8. Correction to: A Multi-center, Open-Label, Single-Arm Trial to Evaluate the Efficacy, Pharmacokinetics, and Safety and Tolerability of IGSC 20% in Subjects with Primary Immunodeficiency

Author

Santamaria, Manuel, Neth, Olaf, Douglass, Jo A., Krivan, Gergely, Kobbe, Robin, Bernatowska, Ewa, Grigoriadou, Sofia, Bethune, Claire, Chandra, Anita, Horneff, Gerd, Borte, Michael, Sonnenschein, Anja, Kralickova, Pavlina, Ramón, Silvia Sánchez, Langguth, Daman D., Gonzalez‑Granado, Luis Ignacio, Alsina, Laia, Querolt, Montse, Griffin, Rhonda, Hames, Carrie, Mondou, Elsa, Price, Jeffrey, Sanz, Ana, and Lin, Jiang

Published
2022
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16. Surgical Emergencies in Intestinal Venous Malformations

Author

Muñoz, Martha Isabel Romo, Bueno, Alba, De La Torre, Carlos, Cerezo, Vanesa Nuñez, Rebolledo, Bryant Noriega, Cervantes, Manuel Gomez, Dore, Mariela, Gomez, Javier Jimenez, Santamaria, Manuel Lopez, Martinez, Leopoldo, and Lopez-Gutierrez, Juan Carlos

Published
2018
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18. First multivisceral transplantation in Mitchell‐Riley/Martinez‐Frias syndrome

Author

Estefanía‐Fernández, Karla, primary, Andrés, Ane, additional, Alcolea, Alida, additional, Velayos‐López, María, additional, Pastrían, Laura G., additional, Ramírez‐Amorós, Carla, additional, Gonzalez, Rocío, additional, Sarría, Marta, additional, Ramos, Esther, additional, López‐Santamaria, Manuel, additional, and Hernández, Francisco, additional

Published
2022
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19. European Liver Transplant Registry: Donor and transplant surgery aspects of 16,641 liver transplantations in children

Author

de Ville de Goyet, Jean, Baumann, Ulrich, Karam, Vincent, Adam, René, Nadalin, Silvio, Heaton, Nigel, Reding, Raymond, Branchereau, Sophie, Mirza, Darius, Klempnauer, Jürgen L, Fischer, Lutz, Kalicinski, Piotr, Colledan, Michele, Lopez Santamaria, Manuel, de Kleine, Ruben H, Chardot, Christophe, Yilmaz, Sezai, Kilic, Murat, Boillot, Olivier, di Francesco, Fabrizio, Polak, Wojciech G, Verkade, Henkjan J, European Liver, Intestine Transplant Association (ELITA), de Ville de Goyet, J, Baumann, U, Karam, V, Adam, R, Nadalin, S, Heaton, N, Reding, R, Branchereau, S, Mirza, D, Klempnauer, J, Fischer, L, Kalicinski, P, Colledan, M, Lopez Santamaria, M, de Kleine, R, Chardot, C, Yilmaz, S, Kilic, M, Boillot, O, di Francesco, F, Polak, W, Verkade, H, UCL - SSS/IREC/CHEX - Pôle de chirgurgie expérimentale et transplantation, UCL - (SLuc) Service de chirurgie et transplantation abdominale, and Center for Liver, Digestive and Metabolic Diseases (CLDM)

Subjects
Graft Rejection, Male, Registrie, medicine.medical_specialty, Innovative technique, Survival rate, Tissue and Organ Procurement, Adolescent, Innovative techniques, medicine.medical_treatment, Liver transplantation, Pediatric liver transplantation, Group B, Time, Liver disease, Transplant surgery, Transplantation Immunology, medicine, Living Donors, Humans, Registries, Child, COMPLICATIONS, Hepatology, business.industry, Age Factors, Infant, Graft survival, medicine.disease, Pathophysiology, Surgery, Liver Transplantation, Transplantation, Europe, RECIPIENTS, surgical procedures, operative, SURVIVAL, Female, business
Abstract

BACKGROUND & AIMS: The European Liver Transplant Registry (ELTR) has collected data on liver transplant procedures performed in Europe since 1968.APPROACH & RESULTS: Over a 50 years period (1968 - 2017), clinical and laboratory data were collected from 133 transplant centers and analyzed retrospectively (16,641 liver transplants in 14,515 children). Data were analyzed according to 3 successive periods (A: before 2000, B: 2000 to 2009, and C: since 2010), studying donor and graft characteristics, and graft outcome. The use of living donors steadily increased from A to C [A: n=296 (7%), B: n=1131 (23%) and C: n=1985 (39%); P=0.0001]. Overall, the 5-year graft survival rate has improved from 65% in group A to 75% in group B (pCONCLUSIONS: Pediatric liver transplantation has reached a high efficacy as a cure, or treatment, for severe liver disease in infants and children. Grafts that survived the first year had a half-life time similar to standard human half-life. Transplantation before or after puberty may be the pivot-point for lower long-term outcome in children. Further studies are necessary to re-visit some old concepts regarding transplant benefit (survival time) for small children, the role of recipient pathophysiology versus graft aging, and risk at transition to adult age.

Published
2022

26. Graft infusion of adipose‐derived mesenchymal stromal cells to prevent rejection in experimental intestinal transplantation: A feasibility study

Author

Andres, Ane M., primary, Stringa, Pablo, additional, Talayero, Paloma, additional, Santamaria, Monica, additional, García‐Arranz, Mariano, additional, García Gómez‐Heras, Soledad, additional, Largo‐Aramburu, Carlota, additional, Aras‐Lopez, Rosa M., additional, Vallejo‐Cremades, Maria Teresa, additional, Guerra Pastrián, Laura, additional, Vega, Luz, additional, Encinas, Jose Luis, additional, Lopez‐Santamaria, Manuel, additional, and Hernández‐Oliveros, Francisco, additional

Published
2021
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29. Ganglioneuroma: To Operate or Not to Operate

Author

Sánchez-Galán, Alba, Barrena, Saturnino, Vilanova-Sánchez, Alejandra, Martín, Sara Hernández A., Lopez-Fernandez, Sergio, García, Purificación, Lopez-Santamaria, Manuel, Martínez, Leopoldo, and Tovar, Juan A.

Published
2014
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30. Recurrence of bile salt export pump deficiency after liver transplantation

Author

Jara, Paloma, Hierro, Loreto, Martinez-Fernandez, Pilarc, Alvarez- Doforno, Rita, Yanez, Francisca, Diaz, Maria C., Camarena, Carmen, De la Vega, Angela, Frauca, Esteban, Mufloz-Bartolo, Gema, Lopez-Santamaria, Manuel, and Larrauri, Javier; Alvarez, Luis

Subjects
Cholestasis -- Care and treatment, Jaundice, Obstructive -- Care and treatment, Liver -- Transplantation, Liver -- Patient outcomes, Children -- Health aspects, Children -- Care and treatment
Abstract

The article discusses the rare phenotypic recurrence of severe bile salt export pump (BSEP) deficiency among those patients having undergone liver transplant. The main recommendation after several studies indicates that children who are undergoing liver transplantation for BSEP deficiency should be checked for circulating anti-BSEP antibodies.

Published
2009

35. Routine Ultrasound Control after Successful Intussusception Reduction in Children: Is It Really Necessary?

Author

Delgado-Miguel, Carlos, García, Antonella, Delgado, Bonifacio, Muñoz-Serrano, Antonio Jesús, Miguel-Ferrero, Miriam, Barrena, Saturnino, Lopez Santamaria, Manuel, and Martinez, Leopoldo

Subjects
INTESTINAL intussusception, ENEMA, LENGTH of stay in hospitals, AGE differences
Abstract

Introduction:  Recurrence of ileocolic intussusception (ICI) has been related to residual bowel wall edema after enema reduction. Early oral tolerance has been associated with a higher risk of re-intussusception, so an imaging test (ultrasound) has traditionally been performed before restarting oral tolerance. Our aim is to analyze the cost-effectiveness of performing a routine ultrasound in patients who remain asymptomatic after successful enema reduction.Materials and Methods:  A retrospective observational study was performed in patients with ICI who underwent a successful enema reduction between 2005 and 2019 and distributed in two groups according to whether or not a routine ultrasound was performed before restarting oral tolerance: group A (ultrasound) or B (no ultrasound). We analyzed demographic, clinical and laboratory variables, length of hospital stay, and recurrence rate.Results:  We included 366 patients who presented 373 ICI episodes (165 in group A and 208 in group B), without significant differences in gender and age. Group A patients presented a higher percentage of vomiting and bloody stools than those in group B without differences in the other clinical features studied, time of evolution, or laboratory variables. Group A presented a higher length of hospital stay than group B (36 vs. 24 hours), although it was not statistically significant (p = 0.30). No statistically significant differences were observed in the recurrence rate between both groups (10.3% A vs. 10.8% B; p = 0.83).Conclusion:  Performing routine ultrasound before restarting oral tolerance in asymptomatic patients after successful ICI reduction does not decrease the risk of re-intussusception and should not be routinely encouraged. [ABSTRACT FROM AUTHOR]

Published
2021
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36. P1.49: Tuberculosis Infection after Intestinal Transplantation.

Author

Andres, Ane, primary, Sanchez-Galan, Alba, additional, Hernandez-Oliveros, Francisco, additional, Bueno, Alba, additional, Serradilla, Javi, additional, Luis Encinas, Jose, additional, Alcolea, Alida, additional, Nova-Sanchez, Miriam, additional, Ramos, Esther, additional, and Lopez-Santamaria, Manuel, additional

Published
2019
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40. Reliability of the Hirschsprung-Associated Enterocolitis Score in Clinical Practice.

Author

Dore, Mariela, Sanchez, Alejandra Vilanova, Junco, Paloma Triana, Barrena, S., De Ceano-Vivas, María, Gomez, Javier Jimenez, Moreno, Ane M. Andres, Santamaria, Manuel Lopez, Martinez, Leopoldo, Vilanova Sanchez, Alejandra, Triana Junco, Paloma, Jimenez Gomez, Javier, Andres Moreno, Ane M, and Lopez Santamaria, Manuel

Subjects
ENTEROCOLITIS, CLERGY, PEARSON correlation (Statistics), HIRSCHSPRUNG'S disease, ANTIBIOTICS, DIARRHEA, LENGTH of stay in hospitals, RESEARCH evaluation, RISK assessment, RETROSPECTIVE studies, SEVERITY of illness index, DISEASE complications
Abstract

Introduction:  There is a lack of an agreed Hirschsprung-associated enterocolitis (HAEC) definition. In 2009, a HAEC score was proposed for the diagnosis of HAEC episodes. Our aim was to apply the HAEC score on HAEC episodes to determine its diagnostic efficiency and whether it correlated to its severity.Methods:  Retrospective study of patients with HAEC admitted between 2000 and 2016. Episodes of HAEC were identified and the HAEC score was calculated. A cut-off of ≥ 10 according to Pastor et al and ≥ 4 according to Frykman et al were used. A Pearson's correlation coefficient was performed for outcome variable: length-of-stay (LOS).Results:  Note that 21/93 (22.6%) patients with Hirschsprung's disease presented 50 HAEC episodes with a median of 2 (1-5) episodes during an 8.3-year (2-15.6) follow-up. The most common symptoms were foul-smelling (86% [43/50]) and explosive (60% [30/50]) diarrhea. Physical findings showed a distended abdomen (76% [38/50]) and fever (60% [30/50]) with dilated bowel (82% [41/50]) and rectosigmoid cut-off (80% [40/50]) identified on X-rays. Only 34% (17/50) showed abnormal laboratory findings. Patients were admitted with a median LOS of 7 days (1-28). A HAEC score of 9 (2-16) was found, and only 50% (25/50) of patients met the initial criteria (score of 10 points). However, the use of a 4-point cut-off would have allowed the diagnosis of 98% (49/50) of the patients. A positive linear correlation was found for LOS and HAEC score (r = 0.3, p = 0.014).Conclusion:  There is no standardized definition for HAEC. The initial HAEC score cut-off is restrictive and might fail to identify milder episodes. The positive correlation with LOS and thus HAEC severity might aid in patient information and anticipation of treatment. [ABSTRACT FROM AUTHOR]

Published
2019
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41. Long-Term Results after Diversion Surgery in Extrahepatic Portal Vein Obstruction.

Author

Junco, Paloma Triana, Alvarez, Ana, Dore, Mariela, Gomez, Javier Jimenez, Galán, Alba Sánchez, Vilanova-Sánchez, Alejandra, Andres, Ane, Encinas, Jose Luis, Martinez, Leopoldo, Hernandez, Francisco, Santamaria, Manuel Lopez, Triana Junco, Paloma, Jimenez Gomez, Javier, Sánchez Galán, Alba, and Lopez Santamaria, Manuel

Subjects
PORTAL vein, PORTAL vein surgery, PORTAL hypertension, PLATELET function tests, SURGERY, HEMORRHAGE, LEUCOCYTES, SURGICAL arteriovenous shunts, BLOOD coagulation factors, VASCULAR diseases, ESOPHAGEAL varices, GASTROINTESTINAL hemorrhage, SPLEEN diseases, TREATMENT effectiveness, RETROSPECTIVE studies, LEUKOCYTE count, PLATELET count, DISEASE complications, PREVENTION
Abstract

Aim:  Extrahepatic portal vein obstruction (EHPVO) is a frequent cause of noncirrhotic portal hypertension in children. The aim of this study is to analyze long-term results after diversion surgery.Patients and Methods:  Retrospective review of EHPVO patients who underwent diversion surgery analyzing number of platelets, leukocytes, prothrombin activity, splenomegaly, and gastrointestinal bleeding 10 years after surgery.Results:  Thirty-three patients were evaluated, mostly males (64%) and presenting with gastrointestinal bleeding (46%). Mesoportal shunt (Rex) was performed in 19 patients, mesocaval in 7, distal splenorenal in 7, and proximal splenorenal in 3. While comparing mesoportal shunt to the other portosystemic shunts, an increase in platelets was found with every technique, but it was clearly higher in mesoportal shunt. The highest increase was 6 months after surgery (p = 0.0015) as well as prothrombin activity (p = 0.0003). Leukocytes level also increased without statistical significance. Spleen size (cm) and spleen size Z score (SSAZ) decreased significantly 6 months after mesoportal shunt (p = 0.0168). Before surgery, over 94% patients suffered gastrointestinal bleeding, which reduced significantly afterward with bleeding episodes in only four (12%) of them.Conclusion:  Diversion surgery in EHPVO, especially mesoportal shunt of Rex, improves hepatic function (prothrombin activity), reduces hypersplenism (platelets, leukocytes, and spleen size), and decreases gastrointestinal bleeding episodes. [ABSTRACT FROM AUTHOR]

Published
2019
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42. Prognostic Factors for Liver Transplantation in Unresectable Hepatoblastoma.

Author

Junco, Paloma Triana, Cano, Esther María, Dore, Mariela, Gomez, Javier Jimenez, Galán, Alba Sánchez, Vilanova-Sánchez, Alejandra, Andres, Ane, Encinas, Jose Luis, Martinez, Leopoldo, Hernandez, Francisco, Santamaria, Manuel Lopez, Triana Junco, Paloma, Jimenez Gomez, Javier, Sánchez Galán, Alba, and Lopez Santamaria, Manuel

Subjects
DESMOID tumors, LIVER transplantation, ADENOMATOUS polyposis coli, TUMORS in children, DIAGNOSIS, LOW birth weight, CANCER cells, CANCER invasiveness, COMBINED modality therapy, DEAD, PREMATURE infants, LIVER tumors, LONGITUDINAL method, METASTASIS, MOTHERHOOD, ORGAN donors, PARENTING, PROGNOSIS, QUESTIONNAIRES, SMOKING, SURVIVAL analysis (Biometry), RETROSPECTIVE studies, SURGERY
Abstract

Aim:  Hepatoblastoma is the most frequent hepatic tumor in children, and its initial presentation will affect treatment and prognosis. The aim of this study is to evaluate long-term results of liver transplantation in children with unresectable hepatoblastoma.Patients and Methods:  This is a retrospective review of patients with hepatoblastoma who underwent liver transplantation, analyzing risk factors, tumor presentation, treatment, and long-term survival to identify prognostic factors.Results:  Thirty-one patients underwent liver transplantation in the context of unresectable hepatoblastoma, mainly males (67%) and with risk factors such as prematurity (12.9%), maternal smoking (6.5%), and familial adenomatous polyposis (3.2%). Most frequent presentation was multifocal PRETEXT III (51.6%) and PRETEXT IV (45.2%), with metastasis at diagnosis in 12.9% and vascular involvement in 54.8%.Twenty-one patients received a living-donor (67.7%) and 10 a cadaveric graft (32.2%), at 31.7 months of age (5-125). Most transplants were primary, and only two were performed as rescue therapy after an attempt of surgical resection of the tumor.Overall survival 1 and 5 years after transplantation were 93.3% ± 4.6% and 86.4% ± 6.3%, respectively. We could not find any statistically significant differences between risk factors, tumor presentation, type of graft, or type of transplant.Conclusion:  Liver transplantation has increased hepatoblastoma survival in unresectable tumors. Probably due to these good results, we have not been able to find significant prognostic factors in this cohort. [ABSTRACT FROM AUTHOR]

Published
2019
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45. Afectividad y disidencias de la facticidad.

Author

García, José Ordóñez, Coordinación, Vieira, Ignacio, Santamaría, Manuel, De Bravo Delorme, Cristián, Gilabert, Fernando, de Mingo Rodríguez, Alicia Mª, Moreno-Márquez, César, Ribas, José Ángel Rodríguez, de Jesús Fernández Mora, Vicente, Periñán, Juan José Garrido, Uche, Miguel Grijalba, Ordóñez-García, José, Martín, Francisco Romero, García, José Ordóñez, Vieira, Ignacio, Santamaría, Manuel, De Bravo Delorme, Cristián, Gilabert, Fernando, de Mingo Rodríguez, Alicia Mª, Moreno-Márquez, César, Ribas, José Ángel Rodríguez, de Jesús Fernández Mora, Vicente, Periñán, Juan José Garrido, Uche, Miguel Grijalba, Ordóñez-García, José, and Martín, Francisco Romero

Published
2023

47. Neonatal Intrathoracic Gastric Volvulus in Marfan's Syndrome

Author

Serradilla, Javier, additional, Bueno, Alba, additional, Torre, Carlos, additional, Gamarra, Eduardo, additional, Romo, Martha, additional, Nava Hurtado de Saracho, Francisco, additional, Barrial, María, additional, Cervantes, Manuel, additional, and Santamaria, Manuel, additional

Published
2018
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48. Nuss Procedure for a Patient with Negative Haller Index

Author

Dore, Mariela, additional, Triana Junco, Paloma, additional, De La Torre, Carlos, additional, Vilanova-Sánchez, Alejandra, additional, Bret, Monserrat, additional, Gonzalez, Gaspar, additional, Nuñez Cerezo, Vanesa, additional, Jimenez Gomez, Javier, additional, Luis Encinas, Jose, additional, Hernandez, Francisco, additional, Martínez Martínez, Leopoldo, additional, and Lopez Santamaria, Manuel, additional

Published
2018
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49. Surgical Emergencies in Intestinal Venous Malformations

Author

Bueno, Alba, primary, De La Torre, Carlos, primary, Cerezo, Vanesa, primary, Rebolledo, Bryant, primary, Cervantes, Manuel, primary, Dore, Mariela, primary, Gomez, Javier, primary, Santamaria, Manuel, primary, Martinez, Leopoldo, primary, Lopez-Gutierrez, Juan, primary, and Romo Muñoz, Martha, additional

Published
2017
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50. Effect of consumption of tree tomato juice (Cyphomandra betacea) on lipid profile and glucose concentrations in adults with hyperlipidemia, Ecuador

Author

Salazar-Lugo, Raquel, Barahona, Amparito, Ortiz, Katherine, Chávez, Cynthia, Freire, Paola, Méndez, Jimmy, Bermeo, Bélgica, Santamaria, Manuel, Salas, Hilda, and Oleas, Mariana

Subjects
tomate de árbol, hypoglycemic, lipid profile, hipoglicemiante, Cyphomandra betacea, hyperlipidemia, hiperlipidemia, tree tomato, Ecuador, perfil lipídico
Abstract

En este trabajo se evaluó el efecto del consumo del jugo de tomate de árbol (Cyphomandra betacea) sobre parámetros nutricionales y bioquímicos en 54 voluntarios (44 mujeres y 10 hombres) con edades 45±8 años de Ecuador. Al inicio se les realizó una evaluación nutricional y bioquímica, luego se les invitó a consumir el jugo (100g de fruto en 150 ml de agua) diariamente durante 6 semanas. Finalizado este tiempo, se procedió a realizar la evaluación nutricional y bioquímica nuevamente. La evaluación nutricional determinó 67% de obesidad abdominal. Disminuyendo a 53% después del consumo del jugo de tomate de árbol. El 87% de los voluntarios antes del tratamiento, mostraron hipercolesterolemia; 40,7% y 46,3% tenían los triglicéridos y el LDL elevados y 18,5% valores de glucosa entre 98-130mg/dL. Valores que disminuyeron significativamente en este grupo después de la toma del jugo En general, tanto en voluntarios con y sin sobrepeso se observó disminución significativa de colesterol total, LDL y glucosa; sin cambios significativos en los valores de HDL. El consumo del jugo no afectó la actividad de las enzimas alanina-aminotransferasa ni aspartato-aminotransferasa, tampoco las concentraciones de creatinina, urea y ácido úrico; ni la presión arterial, demostrando que no afecta la función hepática ni renal. Estos resultados indican que el consumo del jugo de tomate de árbol, durante seis semanas, parece ejercer un efecto hipolipemiante y moduladora en el metabolismo de la glucosa y colocan a C betacea como uno de los frutos andinos con alto potencial nutraceutico. Sin embargo, estos aspectos deben ser investigados con más detalles. In this work the effect of consumption of tree tomato juice (Cyphomandra betacea) was evaluated on nutritional and biochemical parameters in 54 volunteers (44 women and 10 men) aged 45 ± 8 years-Ecuador. A nutritional and biochemical evaluation was performed in volunteers; then they were invited to drink tree tomato juice (100g of fruit in 150 ml of water) daily for 6 weeks. Finished these 6 weeks, volunteers were nutritional and biochemical evaluated again. 67% abdominal obesity was found, decreasing at 53% after drinking the established dosage of juice. 87% of the volunteers before treatment, showed hypercholesterolemia, 40.7% and 46.3% had hypertriglyceridemia and elevated LDL, respectively and 18.5% glucose concentrations between 98-130 mg/dL. These values decrease significantly in this group after drinking tomato juice. In general, Total Cholesterol, LDL and glucose concentrations decrease significantly after drinking tree tomato juice in all the voluntaries with or without overweight. There is no change in HDL concentrations. The consumption of tree tomato juice did not affect the activity of alanine aminotransferase or aspartate aminotransferase enzymes either creatinine, urea and uric acid concentrations, neither blood pressure suggesting that does not affect renal or liver function. These results indicate that consumption of tree tomato juice for six weeks appears to have a lipid-lowering and modulating effect on glucose metabolism, suggesting C betacea as one of the high Andean fruits nutraceutical potential. However, this issue should be investigated in more detail.

Published
2016

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