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1. Sex, Genotype, and Liver Volume Progression as Risk of Hospitalization Determinants in Autosomal Dominant Polycystic Liver Disease

Author

Schönauer, Ria, primary, Sierks, Dana, additional, Boerrigter, Melissa, additional, Jawaid, Tabinda, additional, Caroff, Lea, additional, Audrezet, Marie-Pierre, additional, Friedrich, Anja, additional, Shaw, Melissa, additional, Degenhardt, Jan, additional, Forberger, Mirjam, additional, de Fallois, Jonathan, additional, Bläker, Hendrik, additional, Bergmann, Carsten, additional, Gödiker, Juliana, additional, Schindler, Philipp, additional, Schlevogt, Bernhard, additional, Müller, Roman-U., additional, Berg, Thomas, additional, Patterson, Ilse, additional, Griffiths, William J., additional, Sayer, John A., additional, Ambrose, John C., additional, Arumugam, Prabhu, additional, Bevers, Roel, additional, Bleda, Marta, additional, Boardman-Pretty, Freya, additional, Boustred, Christopher R., additional, Brittain, Helen, additional, Caulfield, Mark J., additional, Chan, Georgia C., additional, Elgar, Greg, additional, Fowler, Tom, additional, Giess, Adam, additional, Hamblin, Angela, additional, Henderson, Shirley, additional, Hubbard, Tim J.P., additional, Jackson, Rob, additional, Jones, Louise J., additional, Kasperaviciute, Dalia, additional, Kayikci, Melis, additional, Kousathanas, Athanasios, additional, Lahnstein, Lea, additional, Leigh, Sarah E.A., additional, Leong, Ivonne U.S., additional, Lopez, Javier F., additional, Maleady-Crowe, Fiona, additional, McEntagart, Meriel, additional, Minneci, Federico, additional, Moutsianas, Loukas, additional, Mueller, Michael, additional, Murugaesu, Nirupa, additional, Need, Anna C., additional, O’Donovan, Peter, additional, Odhams, Chris A., additional, Patch, Christine, additional, Pereira, Mariana Buongermino, additional, Perez-Gil, Daniel, additional, Pullinger, John, additional, Rahim, Tahrima, additional, Rendon, Augusto, additional, Rogers, Tim, additional, Savage, Kevin, additional, Sawant, Kushmita, additional, Scott, Richard H., additional, Siddiq, Afshan, additional, Sieghart, Alexander, additional, Smith, Samuel C., additional, Sosinsky, Alona, additional, Stuckey, Alexander, additional, Tanguy, Mélanie, additional, Taylor Tavares, Ana Lisa, additional, Thomas, Ellen R.A., additional, Thompson, Simon R., additional, Tucci, Arianna, additional, Welland, Matthew J., additional, Williams, Eleanor, additional, Witkowska, Katarzyna, additional, Wood, Suzanne M., additional, Popp, Bernt, additional, Torres, Vicente E., additional, Hogan, Marie C., additional, Somlo, Stefan, additional, Watnick, Terry J., additional, Nevens, Frederik, additional, Besse, Whitney, additional, Cornec-Le Gall, Emilie, additional, Harris, Peter C., additional, Drenth, Joost P.H., additional, and Halbritter, Jan, additional

Published
2024
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5. Clinical Implementation of an Artificial Intelligence Algorithm for Magnetic Resonance–Derived Measurement of Total Kidney Volume

Author

Theodora A. Potretzke, Panagiotis Korfiatis, Daniel J. Blezek, Marie E. Edwards, Jason R. Klug, Cole J. Cook, Adriana V. Gregory, Peter C. Harris, Fouad T. Chebib, Marie C. Hogan, Vicente E. Torres, Candice W. Bolan, Kumaresan Sandrasegaran, Akira Kawashima, Jeremy D. Collins, Naoki Takahashi, Robert P. Hartman, Eric E. Williamson, Bernard F. King, Matthew R. Callstrom, Bradley J. Erickson, and Timothy L. Kline

Subjects
General Medicine
Published
2023
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10. Utility of new image-derived biomarkers for autosomal dominant polycystic kidney disease prognosis using automated instance cyst segmentation

Author

Gregory, Adriana V., primary, Chebib, Fouad T., additional, Poudyal, Bhavya, additional, Holmes, Heather L., additional, Yu, Alan S.L., additional, Landsittel, Douglas P., additional, Bae, Kyongtae T., additional, Chapman, Arlene B., additional, Frederic, Rahbari-Oskoui, additional, Mrug, Michal, additional, Bennett, William M., additional, Harris, Peter C., additional, Erickson, Bradley J., additional, Torres, Vicente E., additional, and Kline, Timothy L., additional

Published
2023
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11. Mapa epidemiológico transversal de las ataxias y paraparesias espásticas hereditarias en España

Author

Ortega Suero, G., primary, Abenza Abildúa, M.J., additional, Serrano Munuera, C., additional, Rouco Axpe, I., additional, Arpa Gutiérrez, F.J., additional, Adarmes Gómez, A.D., additional, Rodríguez de Rivera, F.J., additional, Quintans Castro, B., additional, Posada Rodríguez, I., additional, Vadillo Bermejo, A., additional, Domingo Santos, Á., additional, Blanco Vicente, E., additional, Infante Ceberio, I., additional, Pardo Fernández, J., additional, Costa Arpín, E., additional, Painous Martí, C., additional, Muñoz, J.E., additional, Mir Rivera, P., additional, Montón Álvarez, F., additional, Bataller Alberola, L., additional, Gascón Bayarri, J., additional, Casasnovas Pons, C., additional, Vélez Santamaría, V., additional, López de Munain, A., additional, Fernández-Eulate, G., additional, Gazulla Abío, J., additional, Sanz Gallego, I., additional, Rojas Bartolomé, L., additional, Ayo Martín, Ó., additional, Segura Martín, T., additional, González Mingot, C., additional, Baraldés Rovira, M., additional, Sivera Mascaró, R., additional, Cubo Delgado, E., additional, Echavarría Íñiguez, A., additional, Vázquez Sánchez, F., additional, Bártulos Iglesias, M., additional, Casadevall Codina, M.T., additional, Martínez Fernández, E.M., additional, Labandeira Guerra, C., additional, Alemany Perna, B., additional, Carvajal Hernández, A., additional, Fernández Moreno, C., additional, Palacín Larroy, M., additional, Caballol Pons, N., additional, Ávila Rivera, A., additional, Navacerrada Barrero, F.J., additional, Lobato Rodríguez, R., additional, and Sobrido Gómez, M.J., additional

Published
2023
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12. Epidemiology of ataxia and hereditary spastic paraplegia in Spain: A cross-sectional study

Author

Ortega Suero, G., primary, Abenza Abildúa, M.J., additional, Serrano Munuera, C., additional, Rouco Axpe, I., additional, Arpa Gutiérrez, F.J., additional, Adarmes Gómez, A.D., additional, Rodríguez de Rivera, F.J., additional, Quintans Castro, B., additional, Posada Rodríguez, I., additional, Vadillo Bermejo, A., additional, Domingo Santos, Á., additional, Blanco Vicente, E., additional, Infante Ceberio, I., additional, Pardo Fernández, J., additional, Costa Arpín, E., additional, Painous Martí, C., additional, Muñoz García, J.E., additional, Mir Rivera, P., additional, Montón Álvarez, F., additional, Bataller Alberola, L., additional, Gascón Bayarri, J., additional, Casasnovas Pons, C., additional, Vélez Santamaría, V., additional, López de Munain, A., additional, Fernández-Eulate, G., additional, Gazulla Abío, J., additional, Sanz Gallego, I., additional, Rojas Bartolomé, L., additional, Ayo Martín, Ó., additional, Segura Martín, T., additional, González Mingot, C., additional, Baraldés Rovira, M., additional, Sivera Mascaró, R., additional, Cubo Delgado, E., additional, Echavarría Íñiguez, A., additional, Vázquez Sánchez, F., additional, Bártulos Iglesias, M., additional, Casadevall Codina, M.T., additional, Martínez Fernández, E.M., additional, Labandeira Guerra, C., additional, Alemany Perna, B., additional, Carvajal Hernández, A., additional, Fernández Moreno, C., additional, Palacín Larroy, M., additional, Caballol Pons, N., additional, Ávila Rivera, A., additional, Navacerrada Barrero, F.J., additional, Lobato Rodríguez, R., additional, and Sobrido Gómez, M.J., additional

Published
2023
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13. Bone health in autosomal dominant polycystic kidney disease (ADPKD) patients after kidney transplantation

Author

Zubidat, Dalia, primary, Hanna, Christian, additional, Randhawa, Amarjyot K., additional, Smith, Byron H., additional, Chedid, Maroun, additional, Kaidbay, Daniel-Hasan N., additional, Nardelli, Luca, additional, Mkhaimer, Yaman G., additional, Neal, Reem M., additional, Madsen, Charles D., additional, Senum, Sarah R., additional, Gregory, Adriana V., additional, Kline, Timothy L., additional, Zoghby, Ziad M., additional, Broski, Stephen M., additional, Issa, Naim S., additional, Harris, Peter C., additional, Torres, Vicente E., additional, Sfeir, Jad G., additional, and Chebib, Fouad T., additional

Published
2023
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16. OVERTURE: A Worldwide, Prospective, Observational Study of Disease Characteristics in Patients With ADPKD

Author

Perrone, Ronald D., primary, Oberdhan, Dorothee, additional, Ouyang, John, additional, Bichet, Daniel G., additional, Budde, Klemens, additional, Chapman, Arlene B., additional, Gitomer, Berenice Y., additional, Horie, Shigeo, additional, Ong, Albert C.M., additional, Torres, Vicente E., additional, Turner, A. Neil, additional, and Krasa, Holly, additional

Published
2023
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17. Venglustat, a Novel Glucosylceramide Synthase Inhibitor, in Patients at Risk of Rapidly Progressing ADPKD: Primary Results of a Double-Blind, Placebo-Controlled, Phase 2/3 Randomized Clinical Trial

Author

Gansevoort, Ronald T., primary, Hariri, Ali, additional, Minini, Pascal, additional, Ahn, Curie, additional, Chapman, Arlene B., additional, Horie, Shigeo, additional, Knebelmann, Bertrand, additional, Mrug, Michal, additional, Ong, Albert C.M., additional, Pei, York P.C., additional, Torres, Vicente E., additional, Modur, Vijay, additional, Antonshchuk, Igor, additional, and Perrone, Ronald D., additional

Published
2023
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19. Clinical Implementation of an Artificial Intelligence Algorithm for Magnetic Resonance–Derived Measurement of Total Kidney Volume

Author

Potretzke, Theodora A., primary, Korfiatis, Panagiotis, additional, Blezek, Daniel J., additional, Edwards, Marie E., additional, Klug, Jason R., additional, Cook, Cole J., additional, Gregory, Adriana V., additional, Harris, Peter C., additional, Chebib, Fouad T., additional, Hogan, Marie C., additional, Torres, Vicente E., additional, Bolan, Candice W., additional, Sandrasegaran, Kumaresan, additional, Kawashima, Akira, additional, Collins, Jeremy D., additional, Takahashi, Naoki, additional, Hartman, Robert P., additional, Williamson, Eric E., additional, King, Bernard F., additional, Callstrom, Matthew R., additional, Erickson, Bradley J., additional, and Kline, Timothy L., additional

Published
2023
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20. Clinical Pattern of Tolvaptan-Associated Liver Injury in Trial Participants With Autosomal Dominant Polycystic Kidney Disease (ADPKD): An Analysis of Pivotal Clinical Trials

Author

Alpers, David H., primary, Lewis, James H., additional, Hunt, Christine M., additional, Freston, James W., additional, Torres, Vicente E., additional, Li, Hui, additional, Wang, Wenchyi, additional, Hoke, Molly E., additional, Roth, Sharin E., additional, Westcott-Baker, Lucas, additional, and Estilo, Alvin, additional

Published
2023
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22. PKD1 Compared With PKD2 Genotype and Cardiac Hospitalizations in the Halt Progression of Polycystic Kidney Disease Studies

Author

Berenice Gitomer, Kaleab Z. Abebe, Michel Chonchol, Alan S.L. Yu, Kristen L. Nowak, William E. Braun, Peter C. Harris, Vicente E. Torres, Ronald D. Perrone, Godela Brosnahan, Cortney N. Steele, Arlene B. Chapman, Zhiying You, and Theodore I. Steinman

Subjects
medicine.medical_specialty, PKD1, Nephrology, business.industry, Internal medicine, Genotype, Research Letter, medicine, Polycystic kidney disease, business, medicine.disease, Gastroenterology
Published
2022
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27. Assessing Risk of Rapid Progression in Autosomal Dominant Polycystic Kidney Disease and Special Considerations for Disease-Modifying Therapy

Author

Vicente E. Torres and Fouad T. Chebib

Subjects
Oncology, medicine.medical_specialty, 030232 urology & nephrology, Tolvaptan, Autosomal dominant polycystic kidney disease, Renal function, Kidney Volume, Disease, Kidney, Risk Assessment, Kidney cysts, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, Humans, 030212 general & internal medicine, Ultrasonography, urogenital system, business.industry, Age Factors, Organ Size, Polycystic Kidney, Autosomal Dominant, medicine.disease, Magnetic Resonance Imaging, medicine.anatomical_structure, Nephrology, Disease Progression, medicine.symptom, Tomography, X-Ray Computed, business, Antidiuretic Hormone Receptor Antagonists, Progressive disease, Glomerular Filtration Rate, medicine.drug
Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited cause of kidney failure, accounting for 5%-10% of cases. Predicting which patients with ADPKD will progress rapidly to kidney failure is critical to assess the risk-benefit ratio of any intervention and to consider early initiation of long-term kidney protective measures that will maximize the cumulative benefit of slowing disease progression. Surrogate prognostic biomarkers are required to predict future decline in kidney function. Clinical, genetic, environmental, epigenetic, and radiologic factors have been studied as predictors of progression to kidney failure in ADPKD. A complex interaction of these prognostic factors determines the number of kidney cysts and their growth rates, which affect total kidney volume (TKV). Age-adjusted TKV, represented by the Mayo imaging classification, estimates each patient's unique rate of kidney growth and provides the most individualized approach available clinically so far. Tolvaptan has been approved to slow disease progression in patients at risk of rapidly progressive disease. Several other disease-modifying treatments are being studied in clinical trials. Selection criteria for patients at risk of rapid progression vary widely among countries and are based on a combination of age, baseline glomerular filtration rate (GFR), GFR slope, baseline TKV, and TKV rate of growth. This review details the approach in assessing the risk of disease progression in ADPKD and identifying patients who would benefit from long-term therapy with disease-modifying agents.

Published
2021
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28. High Prevalence of Kidney Cysts in Patients With CYP24A1 Deficiency

Author

Dawn S. Milliner, Peter J. Tebben, Theodora A. Potretzke, Andrea G. Cogal, Peter C. Harris, Yaman G. Mkhaimer, Vicente E. Torres, Fouad T. Chebib, David J. Sas, John C. Lieske, and Christian Hanna

Subjects
medicine.medical_specialty, Medullary cavity, 030232 urology & nephrology, CYP24A1 deficiency, 030204 cardiovascular system & hematology, Kidney cysts, Gastroenterology, 03 medical and health sciences, Cystic kidney disease, 0302 clinical medicine, Clinical Research, Internal medicine, medicine, kidney cyst, Cyst, Hypercalciuria, Family history, hypercalciuria, business.industry, hypercalcemia, medicine.disease, Nephrology, Nephrocalcinosis, medicine.symptom, business, Kidney disease
Abstract

Introduction Loss-of-function variants in the CYP24A1 gene cause a rare hereditary disease characterized by reduced 24-hydroxylase enzyme activity, increased serum 1,25-dihydroxycholecalciferol levels, hypercalcemia, hypercalciuria, and nephrocalcinosis and/or nephrolithiasis. Kidney cysts in patients with CYP24A1 deficiency were first reported in a single case study from our center. However, a possible association between CYP24A1 deficiency and kidney cysts has not been described. Methods Retrospective analysis of patients with confirmed or suspected CYP24A1 deficiency and available kidney imaging. Results Among 16 patients with confirmed pathogenic variants, 38% were male and 31% were children, the median age at genetic confirmation was 38 years (range 1–66), and none had a family history of cystic kidney disease. Medullary and/or corticomedullary junction cysts were present in all cases. The median age at first detected cyst was 37 years (range 3–60). The mean and median number of cysts per patient were 5.3 and 2.5 (range 1–37), respectively. Four of 5 further patients with suspected but unconfirmed pathogenic variants had cysts. The number of cysts ≥5 mm in size was above the 97.5th percentile of an age- and sex-matched control population in 55% and 67% of patients with confirmed and suspected pathogenic variants, respectively. At least 1 cyst (≥5 mm in size) was found in 80% of children with confirmed CYP24A1 deficiency. Conclusions These observations strongly suggest an association between CYP24A1 deficiency and kidney cysts. Further studies are needed to evaluate the role of CYP24A1, vitamin D metabolism, and/or hypercalciuria in cyst formation, and whether cysts exacerbate chronic kidney disease or modify nephrocalcinosis and stone risk.

Published
2021
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29. The genetic background significantly impacts the severity of kidney cystic disease in the Pkd1RC/RC mouse model of autosomal dominant polycystic kidney disease

Author

Vicente E. Torres, Ka Thao, Jessica M. Smith, Peter C. Harris, Madeline R. Martell, Diana Escobar-Zarate, Katharina Hopp, Maria V. Irazabal, Harrison H. Wells, Megan M. Constans, Jennifer Arroyo, Timothy L. Kline, and Cynthia J. Sieben

Subjects
0301 basic medicine, TRPP Cation Channels, 030232 urology & nephrology, Tolvaptan, Autosomal dominant polycystic kidney disease, Physiology, Renal function, Kidney Volume, Kidney, Kidney cysts, Article, Pathogenesis, Mice, 03 medical and health sciences, 0302 clinical medicine, Animals, Medicine, PKD1, urogenital system, business.industry, Polycystic Kidney, Autosomal Dominant, medicine.disease, Mice, Inbred C57BL, 030104 developmental biology, Nephrology, Mutation, medicine.symptom, business, Genetic Background, Progressive disease, medicine.drug
Abstract

Autosomal dominant polycystic kidney disease (ADPKD), primarily due to PKD1 or PKD2 mutations, causes progressive kidney cyst development and kidney failure. There is significant intrafamilial variability likely due to the genetic background and environmental/lifestyle factors; variability that can be modeled in PKD mice. Here, we characterized mice homozygous for the PKD1 hypomorphic allele, p.Arg3277Cys (Pkd1(RC/RC)), inbred into the BALB/cJ (BC) or the 129S6/SvEvTac (129) strains, plus F1 progeny bred with the previously characterized C57BL/6J (B6) model; F1(BC/B6) or F1(129/B6). By one-month cystic disease in both the BC and 129 Pkd1(RC/RC) mice was more severe than in B6 and continued with more rapid progression to six to nine months. Thereafter, the expansive disease stage plateaued/declined, coinciding with increased fibrosis and a clear decline in kidney function. Greater severity correlated with more inter-animal and inter-kidney disease variability, especially in the 129-line. Both F1 combinations had intermediate disease severity, more similar to B6 but progressive from one-month of age. Mild biliary dysgenesis, and an early switch from proximal tubule to collecting duct cysts, was seen in all backgrounds. Preclinical testing with a positive control, tolvaptan, employed the F1(129/B6)-Pkd1(RC/RC) line, which has moderately progressive disease and limited isogenic variability. Magnetic resonance imaging was utilized to randomize animals and provide total kidney volume endpoints; complementing more traditional data. Thus, we show how genetic background can tailor the Pkd1(RC/RC) model to address different aspects of pathogenesis and disease modification, and describe a possible standardized protocol for preclinical testing.

Published
2021
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30. The STAGED-PKD 2-Stage Adaptive Study With a Patient Enrichment Strategy and Treatment Effect Modeling for Improved Study Design Efficiency in Patients With ADPKD

Author

Perrone, Ronald D., primary, Hariri, Ali, additional, Minini, Pascal, additional, Ahn, Curie, additional, Chapman, Arlene B., additional, Horie, Shigeo, additional, Knebelmann, Bertrand, additional, Mrug, Michal, additional, Ong, Albert C.M., additional, Pei, York P.C., additional, Torres, Vicente E., additional, Modur, Vijay, additional, and Gansevoort, Ronald T., additional

Published
2022
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31. Cardiovascular Outcomes in Kidney Transplant Recipients With ADPKD

Author

Chedid, Maroun, primary, Kaidbay, Hasan-Daniel, additional, Wigerinck, Stijn, additional, Mkhaimer, Yaman, additional, Smith, Byron, additional, Zubidat, Dalia, additional, Sekhon, Imranjot, additional, Prajwal, Reddy, additional, Duriseti, Parikshit, additional, Issa, Naim, additional, Zoghby, Ziad M., additional, Hanna, Christian, additional, Senum, Sarah R., additional, Harris, Peter C., additional, Hickson, LaTonya J., additional, Torres, Vicente E., additional, Nkomo, Vuyisile T., additional, and Chebib, Fouad T., additional

Published
2022
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32. Bone health in autosomal dominant polycystic kidney disease (ADPKD) patients after kidney transplantation

Author

Dalia Zubidat, Christian Hanna, Amarjyot K. Randhawa, Byron H. Smith, Maroun Chedid, Daniel-Hasan N. Kaidbay, Luca Nardelli, Yaman G. Mkhaimer, Reem M. Neal, Charles D. Madsen, Sarah R. Senum, Adriana V. Gregory, Timothy L. Kline, Ziad M. Zoghby, Stephen M. Broski, Naim S. Issa, Peter C. Harris, Vicente E. Torres, Jad G. Sfeir, and Fouad T. Chebib

Subjects
Endocrinology, Diabetes and Metabolism, Orthopedics and Sports Medicine
Published
2023
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33. Prognostic Value of Fibroblast Growth Factor 23 in Autosomal Dominant Polycystic Kidney Disease

Author

Shiqin Zhang, Kyongtae T. Bae, Vicente E. Torres, William M. Bennett, Douglas Landsittel, Frederic F. Rahbari-Oskoui, Jared J. Grantham, Jason R. Stubbs, Pengcheng Lu, Mireille El Ters, Jonathan D. Mahnken, Darren P. Wallace, Peter C. Harris, Michal Mrug, Alan S.L. Yu, and Arlene B. Chapman

Subjects
Fibroblast growth factor 23, medicine.medical_specialty, 030232 urology & nephrology, Urology, Autosomal dominant polycystic kidney disease, Renal function, Kidney Volume, 030204 cardiovascular system & hematology, lcsh:RC870-923, urologic and male genital diseases, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Clinical Research, FGF23, Medicine, ADPKD, Creatinine, business.industry, Hazard ratio, lcsh:Diseases of the genitourinary system. Urology, medicine.disease, female genital diseases and pregnancy complications, chemistry, Quartile, Nephrology, Cohort, business
Abstract

Introduction Autosomal dominant polycystic kidney disease (ADPKD) is characterized by progressive cyst growth and a loss of functioning renal mass, but a decline in glomerular filtration rate (GFR) and onset of end-stage renal disease (ESRD) occur late in the disease course. There is therefore a great need for early prognostic biomarkers in this disorder. Methods We measured baseline serum fibroblast growth factor 23 (FGF23) levels in 192 patients with ADPKD from the Consortium for Radiologic Imaging Studies of PKD (CRISP) cohort that were followed for a median of 13 years and tested the association between FGF23 levels and change over time in height-adjusted total kidney volume (htTKV), GFR, and time to the composite endpoints of ESRD, death, and doubling of serum creatinine. Results Patients in the highest quartile for baseline FGF23 level had a higher rate of increase in htTKV (0.95% per year, P = 0.0016), and faster rate of decline in GFR (difference of −1.03 ml/min/1.73 m2 per year, P = 0.005) compared with the lowest quartile, after adjusting for other covariates, including htTKV and genotype. The highest quartile of FGF23 was also associated with a substantial increase in risk for the composite endpoint of ESRD, death, or doubling of serum creatinine (hazard ratio [HR] of 2.45 in the fully adjusted model, P = 0.03). Conclusion FGF23 is a prognostic biomarker for disease progression and clinically important outcomes in ADPKD, and has additive value to established imaging and genetic biomarkers., Graphical abstract

Published
2021
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34. Mineral bone disease in autosomal dominant polycystic kidney disease

Author

Franklin W. Maddux, Theodore I. Steinman, Myles Wolf, Diana George, Isidro B. Salusky, Harmut H. Malluche, Vicente E. Torres, Norma J. Ofsthun, Michel Chonchol, Tamara Isakova, Renata C. Pereira, Lorien S. Dalrymple, Jason W Stoneback, Arlene B. Chapman, Berenice Gitomer, Xuan Cai, and Zhiying You

Subjects
Adult, 0301 basic medicine, Fibroblast growth factor 23, medicine.medical_specialty, 030232 urology & nephrology, Urology, Autosomal dominant polycystic kidney disease, Kidney, urologic and male genital diseases, Article, Bone remodeling, Mice, 03 medical and health sciences, 0302 clinical medicine, Diabetes mellitus, medicine, Animals, Humans, Minerals, PKD1, urogenital system, Osteoid, business.industry, Bone fracture, Polycystic Kidney, Autosomal Dominant, medicine.disease, female genital diseases and pregnancy complications, 030104 developmental biology, Nephrology, Kidney Failure, Chronic, Bone Diseases, business, Glomerular Filtration Rate, Kidney disease
Abstract

Mice with disruption of Pkd1 in osteoblasts demonstrate reduced bone mineral density, trabecular bone volume and cortical thickness. To date, the bone phenotype in adult patients with autosomal dominant polycystic kidney disease (ADPKD) with stage I and II chronic kidney disease has not been investigated. To examine this, we characterized biochemical markers of mineral metabolism, examined bone turnover and biology, and estimated risk of fracture in patients with ADPKD. Markers of mineral metabolism were measured in 944 patients with ADPKD and other causes of kidney disease. Histomorphometry and immunohistochemistry were compared on bone biopsies from 20 patients with ADPKD with a mean eGFR of 97 ml/ min/1.73m(2) and 17 healthy individuals. Furthermore, adults with end stage kidney disease (ESKD) initiating hemodialysis between 2002–2013 and estimated the risk of bone fracture associated with ADPKD as compared to other etiologies of kidney disease were examined. Intact fibroblast growth factor 23 was higher and total alkaline phosphatase lower in patients with compared to patients without ADPKD with chronic kidney disease. Compared to healthy individuals, patients with ADPKD demonstrated significantly lower osteoid volume/bone volume (0.61 vs. 1.21%) and bone formation rate/bone surface (0.012 vs. 0.026 μm(3)/μm(2)/day). ESKD due to ADPKD was not associated with a higher risk of fracture as compared to ESKD due to diabetes (age adjusted incidence rate ratio: 0.53 (95% confidence interval 0.31, 0.74) or compared to other etiologies of kidney disease. Thus, individuals with ADPKD have lower alkaline phosphatase, higher circulating intact fibroblast growth factor 23 and decreased bone formation rate. However, ADPKD is not associated with higher rates of bone fracture in ESKD.

Published
2021
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35. Characteristics of Patients with End-Stage Kidney Disease in ADPKD

Author

Marie C. Hogan, Peter C. Harris, Reem Neal, Vicente E. Torres, Shehbaz Shukoor, Yaman G. Mkhaimer, Sarah R. Senum, Ziad Zoghby, Fouad T. Chebib, Marie E. Edwards, Sravanthi Lavu, Maria V. Irazabal, Ghaith Zaatari, Timothy L. Kline, and Lisa E. Vaughan

Subjects
Aging, medicine.medical_specialty, Characteristics of ESKD in ADPKD, Total Kidney Volume, 030232 urology & nephrology, Autosomal dominant polycystic kidney disease, Urology, Kidney Volume, 030204 cardiovascular system & hematology, 03 medical and health sciences, 0302 clinical medicine, Clinical Research, Polycystic Kidney Disease, medicine, Polycystic kidney disease, ADPKD, Macrovascular disease, Kidney, ESKD, PKD1, Vascular disease, business.industry, Gender, medicine.disease, TKV, medicine.anatomical_structure, Nephrology, business, Kidney disease
Abstract

Introduction Cystic expansion damaging the parenchyma is thought to lead to end-stage kidney disease (ESKD) in autosomal dominant polycystic kidney disease (ADPKD). Here we characterized genotypic and phenotypic attributes of ADPKD at time of ESKD. Methods This is a retrospective cross-sectional study of patients with ADPKD with ESKD evaluated at Mayo Clinic with available abdominal computed tomography (CT) or magnetic resonance imaging (MRI). Kidney volumes were measured (total kidney volume adjusted for height [HtTKV]), Mayo Image Class (MIC) calculated, ADPKD genotype determined, and clinical and laboratory features obtained from medical records. Results Differences in HtTKV at ESKD were associated with patient age and sex; older patients and women had smaller HtTKV at ESKD. HtTKV at ESKD was observed to be 12.3% smaller with each decade of age (P < 0.01); but significant only in women (17.8%, P < 0.01; men 6.9%, P = 0.06). Patients with onset of ESKD at 61 years had different characteristics, with a shift from youngest to oldest in male to female enrichment, MIC from 1D/1E to 1B/1C, likely fully penetrant PKD1 mutations from 95% to 42%, and presence of macrovascular disease from 8% to 40%. Macrovascular disease was associated with smaller kidneys in female patients. Conclusion HtTKV at ESKD was smaller with advancing age in patients with ADPKD, particularly in women. These novel findings provide insight into possible underlying mechanisms leading to ESKD, which differ between younger and older individuals. Cystic growth is the predominant mechanism in younger patients with ESKD, whereas aging-related factors, including vascular disease, becomes potentially important as patients age., Graphical abstract

Published
2021
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36. Salt, water, and vasopressin in polycystic kidney disease

Author

Vicente E. Torres

Subjects
0301 basic medicine, medicine.medical_specialty, Vasopressin, Vasopressins, 030232 urology & nephrology, Autosomal dominant polycystic kidney disease, Renal function, Kidney, urologic and male genital diseases, 03 medical and health sciences, 0302 clinical medicine, Copeptin, Internal medicine, medicine, Polycystic kidney disease, Humans, Sodium Chloride, Dietary, Salt intake, reproductive and urinary physiology, urogenital system, business.industry, Glycopeptides, Water, Polycystic Kidney, Autosomal Dominant, medicine.disease, female genital diseases and pregnancy complications, 030104 developmental biology, medicine.anatomical_structure, Endocrinology, Nephrology, Disease Progression, business, Biomarkers, Glomerular Filtration Rate, Kidney disease
Abstract

Excessive salt intake and vasopressin may promote cyst growth in autosomal dominant polycystic kidney disease and glomerular filtration rate (GFR) decline in chronic kidney disease. Kramers et al. confirmed the effects of salt in a large autosomal dominant polycystic kidney disease cohort. Copeptin mediated 72% and 25% of the salt effects on GFR decline and kidney growth. Kidney growth did not significantly contribute to the copeptin-mediated salt effect on GFR. Differences in kidney growth and GFR decline trajectories and factors other than cyst growth contributing to GFR decline may explain the divergent effects.

Published
2020
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38. Kidney Cysts in Hypophosphatemic Rickets With Hypercalciuria: A Case Series

Author

Hanna, Christian, primary, Potretzke, Theodora A., additional, Chedid, Maroun, additional, Rangel, Laureano J., additional, Arroyo, Jennifer, additional, Zubidat, Dalia, additional, Tebben, Peter J., additional, Cogal, Andrea G., additional, Torres, Vicente E., additional, Harris, Peter C., additional, Sas, David J., additional, Lieske, John C., additional, Milliner, Dawn S., additional, and Chebib, Fouad T., additional

Published
2022
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39. Urinary human papilloma virus infection and bladder cancer risk: A systematic review and a prisma-compliant meta-analysis

Author

Garcia-Rojo, D., primary, Muñoz-Rodriguez, J., additional, Centeno, C., additional, Dominguez, A., additional, Prera, A., additional, Abad, C., additional, Vicente, E., additional, Gonzalez-Sala, J.L., additional, Hannaoui, N., additional, Capdevila, M., additional, De Verdonces, L., additional, Ferran, A., additional, Pla, C., additional, Azuara, P., additional, Cuadrench, S., additional, Zegri, M.E., additional, and Prats, J., additional

Published
2022
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41. Monoallelic IFT140 pathogenic variants are an important cause of the autosomal dominant polycystic kidney-spectrum phenotype

Author

Senum, Sarah R., primary, Li, Ying (Sabrina) M., additional, Benson, Katherine A., additional, Joli, Giancarlo, additional, Olinger, Eric, additional, Lavu, Sravanthi, additional, Madsen, Charles D., additional, Gregory, Adriana V., additional, Neatu, Ruxandra, additional, Kline, Timothy L., additional, Audrézet, Marie-Pierre, additional, Outeda, Patricia, additional, Nau, Cherie B., additional, Meijer, Esther, additional, Ali, Hamad, additional, Steinman, Theodore I., additional, Mrug, Michal, additional, Phelan, Paul J., additional, Watnick, Terry J., additional, Peters, Dorien J.M., additional, Ong, Albert C.M., additional, Conlon, Peter J., additional, Perrone, Ronald D., additional, Cornec-Le Gall, Emilie, additional, Hogan, Marie C., additional, Torres, Vicente E., additional, Sayer, John A., additional, and Harris, Peter C., additional

Published
2022
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45. Next-Generation 3D Modelling Software for Personalised Surgery Decision-Making in Perihilarcholangiocarcinoma: Results of a Multi-Institutional Cohort Study

Author

Lopez-Lopez, V., primary, Gomez-Perez, B., additional, De Vicente, E., additional, Jimenez-Galanes, S., additional, Sabater, L., additional, Lang, H., additional, Conesa, A. Lopez, additional, Brusadin, R., additional, Melendez, R., additional, Santiago, M.J. Castro, additional, Crespo, M.J., additional, Cayuela, V., additional, and Robles Campos, R., additional

Published
2022
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47. Erythropoietin and Fibroblast Growth Factor 23 in Autosomal Dominant Polycystic Kidney Disease Patients

Author

Wei Wang, Godela Brosnahan, Theodore I. Steinman, Berenice Gitomer, Kyongtae T. Bae, Renata C. Pereira, Zhiying You, Vicente E. Torres, Arlene B. Chapman, Ronald D. Perrone, Michel Chonchol, Isidro B. Salusky, Kristen L. Nowak, and Mark R. Hanudel

Subjects
Fibroblast growth factor 23, 0303 health sciences, medicine.medical_specialty, business.industry, 030232 urology & nephrology, Autosomal dominant polycystic kidney disease, medicine.disease, 03 medical and health sciences, 0302 clinical medicine, Endocrinology, Nephrology, Erythropoietin, Internal medicine, Research Letter, medicine, business, 030304 developmental biology, medicine.drug
Published
2019
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48. Long-term trajectory of kidney function in autosomal-dominant polycystic kidney disease

Author

Chengli Shen, Kyongtae T. Bae, Alan S.L. Yu, Arlene B. Chapman, Douglas Landsittel, Jared J. Grantham, Michal Mrug, Peter C. Harris, Vicente E. Torres, William M. Bennett, Larry T. Cook, Frederic F. Rahbari-Oskoui, and Tiange Shi

Subjects
Adult, Male, 0301 basic medicine, medicine.medical_specialty, TRPP Cation Channels, Time Factors, 030232 urology & nephrology, Autosomal dominant polycystic kidney disease, Urology, Renal function, Kidney Volume, Kidney, urologic and male genital diseases, Models, Biological, Article, Young Adult, 03 medical and health sciences, 0302 clinical medicine, medicine, Polycystic kidney disease, Humans, PKD1, urogenital system, business.industry, Surrogate endpoint, Polycystic Kidney, Autosomal Dominant, medicine.disease, female genital diseases and pregnancy complications, 030104 developmental biology, medicine.anatomical_structure, Nephrology, Mutation, Disease Progression, Kidney Failure, Chronic, Female, business, Glomerular Filtration Rate, Kidney disease
Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by cyst and kidney growth, which is hypothesized to cause loss of functioning renal mass and eventually end-stage kidney disease. However, the time course of decline in glomerular filtration rate (GFR) is poorly defined. The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease study is a 14-year observational cohort study of 241 adults with ADPKD. As an estimate of the rate of kidney growth, participants were stratified into 5 subclasses based on baseline age and magnetic resonance imaging measurements of total kidney volume (TKV) according to the method of Irazabal. GFR trajectories spanning over four decades of life were reconstructed and fitted using mixed polynomial models, which were validated using data from the HALT-PKD study. GFR trajectories were nonlinear, with a period of relative stability in most participants, followed by accelerating decline. The shape and slope of these trajectories were strongly associated with baseline Irazabal class. Patients with PKD1 mutations had a steeper GFR decline than patients with PKD2 mutations or with no detected mutation, largely mediated by the effect of genotype on Irazabal class. Thus, GFR decline in ADPKD is nonlinear, and its trajectory throughout adulthood can be predicted from a single measurement of kidney volume. These models can be used for clinical prognostication, clinical trial design, and patient selection for clinical interventions. Our findings support a causal link between growth in kidney volume and GFR decline, adding support for the use of TKV as a surrogate endpoint in clinical trials.

Published
2019
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49. PKD1 Compared With PKD2 Genotype and Cardiac Hospitalizations in the Halt Progression of Polycystic Kidney Disease Studies

Author

Steele, Cortney, primary, You, Zhiying, additional, Gitomer, Berenice Y., additional, Brosnahan, Godela M., additional, Abebe, Kaleab Z., additional, Braun, William E., additional, Chapman, Arlene B., additional, Harris, Peter C., additional, Perrone, Ronald D., additional, Steinman, Theodore I., additional, Torres, Vicente E., additional, Yu, Alan S.L., additional, Chonchol, Michel, additional, and Nowak, Kristen L., additional

Published
2021
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