HARMATZ, Paul : rechercher sur Z-Library

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Campagne de collecte 15 septembre 2024 – 1 octobre 2024

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Livres
Articles (196)
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1

Mucopolysaccharidosis VI pathophysiology diagnosis and treatment

Journal:

Frontiers in Bioscience

Année:

2017

Langue:

english

Fichier:

PDF, 2.88 MB

english, 2017

2

Cervical cord compression in mucopolysaccharidosis VI (MPS VI): Findings from the MPS VI Clinical Surveillance Program (CSP)

Solanki, Guirish A., Sun, Peter P., Martin, Kenneth W., Hendriksz, Christian J., Lampe, Christina, Guffon, Nathalie, Hung, Annie, Sisic, Zlatko, Shediac, Renée, Harmatz, Paul R.

Journal:

Molecular Genetics and Metabolism

Année:

2016

Langue:

english

Fichier:

PDF, 1.03 MB

english, 2016

3

Enzyme replacement therapy outcomes across the disease spectrum: findings from the mucopolysaccharidosis VI Clinical Surveillance Program

Harmatz, Paul R, Lampe, Christina, Parini, Rossella, Sharma, Reena, Teles, Elisa Leão, Johnson, Julie, Sivam, Debbie, Sisic, Zlatko

Journal:

Journal of Inherited Metabolic Disease

Année:

2019

Langue:

english

Fichier:

PDF, 1.60 MB

english, 2019

4

Mucopolysaccharidosis type VI enzyme replacement therapy outcomes across the disease spectrum: findings from the MPS VI clinical surveillance program

Harmatz, Paul, Lampe, Christina, Parini, Rossella, Sharma, Reena, Teles, Elisa Leão, Hawley, Sara, Johnson, Julie, Sivam, Debbie, Sisic, Zlatko

Journal:

Molecular Genetics and Metabolism

Année:

2018

Langue:

english

Fichier:

PDF, 52 KB

english, 2018

5

A novel Blind Start study design to investigate vestronidase alfa for mucopolysaccharidosis VII, an ultra-rare genetic disease

Harmatz, Paul, Whitley, Chester B., Wang, Raymond Y., Bauer, Mislen, Song, Wenjie, Haller, Christine, Kakkis, Emil

Journal:

Molecular Genetics and Metabolism

Année:

2018

Langue:

english

Fichier:

PDF, 307 KB

english, 2018

6

Mucopolysaccharidosis type VI enzyme replacement therapy initiated in adulthood: Findings from the MPS VI clinical surveillance program

Harmatz, Paul, Lampe, Christina, Parini, Rossella, Sharma, Reena, Teles, Elisa Leão, Hawley, Sara, Johnson, Julie, Sivam, Debbie, Sisic, Zlatko

Journal:

Molecular Genetics and Metabolism

Année:

2018

Langue:

english

Fichier:

PDF, 54 KB

english, 2018

7

Dare You Play It Safe?

Gluck, Paul, Yaron, Oded, Harmatz, Margalit

Journal:

The Physcis Teacher

Année:

2006

Langue:

english

Fichier:

PDF, 503 KB

english, 2006

8

Mucopolysaccharidosis VI

Vassili Valayannopoulos, Helen Nicely, Paul Harmatz, Sean Turbeville

Journal:

Orphanet Journal of Rare Diseases

Année:

2010

Langue:

english

Fichier:

PDF, 2.31 MB

english, 2010

9

Treatment of hip dysplasia in patients with mucopolysaccharidosis type I after hematopoietic stem cell transplantation: results of an international consensus procedure

Langereis, Eveline J, Borgo, Andrea, Crushell, Ellen, Harmatz, Paul R, van Hasselt, Peter M, Jones, Simon A, Kelly, Paula M, Lampe, Christina, van der Lee, Johanna H, Odent, Thierry, Sakkers, Ralph, S

Journal:

Orphanet Journal of Rare Diseases

Année:

2013

Langue:

english

Fichier:

PDF, 285 KB

english, 2013

10

Growth charts for individuals with mucopolysaccharidosis VI (Maroteaux-Lamy syndrome)

Quartel, Adrian, Graham, Sue, Harmatz, Paul, Lin, Ping

Journal:

Molecular Genetics and Metabolism

Année:

2014

Langue:

english

Fichier:

PDF, 156 KB

english, 2014

11

Global treatment response analysis of velmanase alfa long term enzyme replacement therapy for alpha-mannosidosis shows treatment benefit across ages

Harmatz, Paul, Cattaneo, Federica, Ardigò, Diego, Geraci, Silvia, Muschol, Nicole, Amraoui, Yasmina, Gil-Campos, Mercedes, Tylki-Szymanska, Anna, Wijburg, Frits, De Meirleir, Linda, Van den Hout, Joha

Journal:

Molecular Genetics and Metabolism

Année:

2018

Langue:

english

Fichier:

PDF, 58 KB

english, 2018

12

Evidence-based, expert-agreed recommendations for the management of patients with MPS IVA/VI: Recommendations to replace the specific missing enzyme

Hendriksz, Christian, Giugliani, Roberto, Harmatz, Paul, Scarpa, Maurizio

Journal:

Molecular Genetics and Metabolism

Année:

2019

Langue:

english

Fichier:

PDF, 139 KB

english, 2019

13

Dare You Play It Safe?

Gluck, Paul, Yaron, Oded, Harmatz, Margalit

Journal:

The Physcis Teacher

Année:

2006

Fichier:

PDF, 503 KB

2006

14

Assessing the impact of the five senses on quality of life in mucopolysaccharidoses

Giugliani, Roberto, Harmatz, Paul, Lin, Shuan-Pei, Scarpa, Maurizio

Journal:

Orphanet Journal of Rare Diseases

Année:

2020

Fichier:

PDF, 528 KB

2020

15

Comparison of organ dysfunction in transfused patients with SCD or β thalassemia

Elliott Vichinsky, Ellen Butensky, Ellen Fung, Mark Hudes, Elizabeth Theil, Linda Ferrell, Roger Williams, Leslie Louie, Phillip D.K. Lee, Paul Harmatz

Journal:

American Journal of Hematology

Année:

2005

Langue:

english

Fichier:

PDF, 77 KB

english, 2005

16

Morbidity and mortality in chronically transfused subjects with thalassemia and sickle cell disease: A report from the multi-center study of iron overload

Ellen B. Fung, Paul Harmatz, Meredith Milet, Samir K. Ballas, Laura De Castro, Ward Hagar, William Owen, Nancy Olivieri, Kim Smith-Whitley, Deepika Darbari, Winfred Wang, Elliott Vichinsky

Journal:

American Journal of Hematology

Année:

2007

Langue:

english

Fichier:

PDF, 190 KB

english, 2007

17

Patterns of hepatic iron distribution in patients with chronically transfused thalassemia and sickle cell disease

Nilesh R. Ghugre, Ignacio Gonzalez-Gomez, Ellen Butensky, Leila Noetzli, Roland Fischer, Roger Williams, Paul Harmatz, Thomas D. Coates, John C. Wood

Journal:

American Journal of Hematology

Année:

2009

Langue:

english

Fichier:

PDF, 190 KB

english, 2009

18

Follow-up report on the 2-year cardiac data from a deferasirox monotherapy trial

John C. Wood, Tara Glynos, Alexis Thompson, Patricia Giardina, Paul Harmatz, Barinder P. Kang, Carole Paley, Thomas D. Coates

Journal:

American Journal of Hematology

Année:

2010

Langue:

english

Fichier:

PDF, 1.04 MB

english, 2010

19

Electrocardiographic consequences of cardiac iron overload in thalassemia major

Jon Detterich, Leila Noetzli, Fred Dorey, Yaniv Bar-Cohen, Paul Harmatz, Thomas Coates, John Wood

Journal:

American Journal of Hematology

Année:

2012

Langue:

english

Fichier:

PDF, 211 KB

english, 2012

20

Threshold effect of urinary glycosaminoglycans and the walk test as indicators of disease progression in a survey of subjects with Mucopolysaccharidosis VI (Maroteaux–Lamy syndrome)

Stuart J. Swiedler, Michael Beck, Manal Bajbouj, Roberto Giugliani, Ida Schwartz, Paul Harmatz, James E. Wraith, Jane Roberts, David Ketteridge, John J. Hopwood, Nathalie Guffon, M. Clara Sá Miranda,

Journal:

American Journal of Medical Genetics Part A

Année:

2005

Langue:

english

Fichier:

PDF, 243 KB

english, 2005

21

Hepatobiliary Clearance of IgA Immune Complexes Formed in the Circulation

Paul R. Harmatz, Ronald E. Kleinman, Bruce W. Bunnell, Kurt J. Bloch, W. Allan Walker

Journal:

Année:

1982

Langue:

english

Fichier:

PDF, 604 KB

english, 1982

22

The Liver: An Integral Part of the Enteric Mucosal Immune System

Ronald E. Kleinman, Paul R. Harmatz, W. Allan Walker

Journal:

Année:

1982

Langue:

english

Fichier:

PDF, 748 KB

english, 1982

23

The Effect of Bile Duct Obstruction on the Clearance of Circulating IgA Immune Complexes

Paul R. Harmatz, Ronald E. Kleinman, Bruce W. Bunnell, Daniel T. Mcclenathan, W. Allan Walker, Kurt J. Bloch

Journal:

Année:

1984

Langue:

english

Fichier:

PDF, 559 KB

english, 1984

24

Mutational analysis of 105 mucopolysaccharidosis type VI patients

Litsa Karageorgos, Doug A. Brooks, Anthony Pollard, Elizabeth L. Melville, Leanne K. Hein, Peter R. Clements, David Ketteridge, Stuart J. Swiedler, Michael Beck, Roberto Giugliani, Paul Harmatz, James

Journal:

Année:

2007

Langue:

english

Fichier:

PDF, 294 KB

english, 2007

25

Prolactin is transported across the epithelium of the jejunum and ileum of the suckling rat

Patricia A. Gonnella, Paul Harmatz, W. Allan Walker

Journal:

Journal of Cellular Physiology

Année:

1989

Langue:

english

Fichier:

PDF, 3.38 MB

english, 1989

26

Sonographic Demonstration of Reversible Portal Vein Thrombosis Following Splenectomy in an Adolescent

Paul Harmatz, John T. Truman, Nicholas Papanicolaou, Joseph F. Simeone, Joseph T. Ferrucci Jr

Journal:

Journal of Clinical Ultrasound

Année:

1984

Langue:

english

Fichier:

PDF, 281 KB

english, 1984

27

Assessment of cardiac iron by MRI susceptometry and R2* in patients with thalassemia

Zhiyue J. Wang, Roland Fischer, Zili Chu, Donald H. Mahoney Jr., Brigitta U. Mueller, Raja Muthupillai, Ellen B. James, Rajesh Krishnamurthy, Taylor Chung, Eric Padua, Elliott Vichinsky, Paul Harmatz

Journal:

Magnetic Resonance Imaging

Année:

2010

Langue:

english

Fichier:

PDF, 560 KB

english, 2010

28

Characterization of glycated hemoglobin in diabetic patients: usefulness of electrospray mass spectrometry in monitoring the extent and distribution of glycation

Xinyi Zhang, Katalin F Medzihradszky, John Cunningham, Phillip D.K Lee, Cheryl L Rognerud, Ching-Nan Ou, Paul Harmatz, H.Ewa Witkowska

Journal:

Journal of Chromatography B: Biomedical Sciences and Applications

Année:

2001

Langue:

english

Fichier:

PDF, 319 KB

english, 2001

29

Caregiving time in sickle cell disease: Psychological effects in maternal caregivers

Judith Tedlie Moskowitz, Ellen Butensky, Paul Harmatz, Elliott Vichinsky, Melvin B. Heyman, Michael Acree, Judith Wrubel, Leslie Wilson, Susan Folkman

Journal:

Pediatric Blood & Cancer

Année:

2007

Langue:

english

Fichier:

PDF, 127 KB

english, 2007

30

Transfusional iron burden and liver toxicity after bone marrow transplantation for acute myelogenous leukemia and hemoglobinopathies

Wasil Jastaniah, Paul Harmatz, Zahra Pakbaz, Roland Fischer, Elliott Vichinsky, Mark C. Walters

Journal:

Pediatric Blood & Cancer

Année:

2008

Langue:

english

Fichier:

PDF, 207 KB

english, 2008

31

Serum ferritin underestimates liver iron concentration in transfusion independent thalassemia patients as compared to regularly transfused thalassemia and sickle cell patients

Zahra Pakbaz, Roland Fischer, Ellen Fung, Peter Nielsen, Paul Harmatz, Elliott Vichinsky

Journal:

Pediatric Blood & Cancer

Année:

2007

Langue:

english

Fichier:

PDF, 172 KB

english, 2007

32

The role of enzyme replacement therapy in severe Hunter syndrome—an expert panel consensus

Joseph Muenzer, Olaf Bodamer, Barbara Burton, Lorne Clarke, Gudrun Schulze Frenking, Roberto Giugliani, Simon Jones, Maria Verónica Muñoz Rojas, Maurizio Scarpa, Michael Beck, Paul Harmatz

Journal:

European Journal of Pediatrics

Année:

2012

Langue:

english

Fichier:

PDF, 192 KB

english, 2012

33

Enzyme replacement therapy for mucopolysaccharidosis VI: evaluation of long-term pulmonary function in patients treated with recombinant humanN-acetylgalactosamine 4-sulfatase

Paul Harmatz, Zi-Fan Yu, Roberto Giugliani, Ida Vanessa D. Schwartz, Nathalie Guffon, Elisa Leão Teles, M. Clara Sá Miranda, J. Edmond Wraith, Michael Beck, Laila Arash, Maurizio Scarpa, David Ketteri

Journal:

Journal of Inherited Metabolic Disease

Année:

2010

Langue:

english

Fichier:

PDF, 239 KB

english, 2010

34

Cardiac disease in patients with mucopolysaccharidosis: presentation, diagnosis and management

Elizabeth A. Braunlin, Paul R. Harmatz, Maurizio Scarpa, Beatriz Furlanetto, Christoph Kampmann, James P. Loehr, Katherine P. Ponder, William C. Roberts, Howard M. Rosenfeld, Roberto Giugliani

Journal:

Journal of Inherited Metabolic Disease

Année:

2011

Langue:

english

Fichier:

PDF, 595 KB

english, 2011

35

Reversed papilledema in an MPS VI patient with galsulfase (Naglazyme®) therapy

Selim T. Koseoglu, Paul Harmatz, Sean Turbeville, Helen Nicely

Journal:

International Ophthalmology

Année:

2009

Langue:

english

Fichier:

PDF, 140 KB

english, 2009

36

Increased prevalence of iron-overload associated endocrinopathy in thalassaemia versus sickle-cell disease

Ellen B. Fung, Paul R. Harmatz, Phillip D.K. Lee, Meredith Milet, Rita Bellevue, Michael R. Jeng, Karen A. Kalinyak, Mark Hudes, Suruchi Bhatia, Elliott P. Vichinsky, the Multi-Centre Study of Iron Ov

Journal:

British Journal of Haematology

Année:

2006

Langue:

english

Fichier:

PDF, 108 KB

english, 2006

37

Oxidative stress and inflammation in iron-overloaded patients with β-thalassaemia or sickle cell disease

Patrick B. Walter, Ellen B. Fung, David W. Killilea, Qing Jiang, Mark Hudes, Jacqueline Madden, John Porter, Patricia Evans, Elliott Vichinsky, Paul Harmatz

Journal:

British Journal of Haematology

Année:

2006

Langue:

english

Fichier:

PDF, 533 KB

english, 2006

38

Phase Ib clinical trial of starch-conjugated deferoxamine (40SD02): a novel long-acting iron chelator

Paul Harmatz, Robert W. Grady, Paul Dragsten, Elliott Vichinsky, Patricia Giardina, Jacqueline Madden, Michael Jeng, Becky Miller, Gregory Hanson, Bo Hedlund

Journal:

British Journal of Haematology

Année:

2007

Langue:

english

Fichier:

PDF, 584 KB

english, 2007

39

Pulmonary function in thalassaemia major and its correlation with body iron stores

Eugene Y. Sohn, Leila J. Noetzli, Aakanksha Gera, Roberta Kato, Thomas D. Coates, Paul Harmatz, Thomas G. Keens, John C. Wood

Journal:

British Journal of Haematology

Année:

2011

Langue:

english

Fichier:

PDF, 105 KB

english, 2011

40

Disparity in the management of iron overload between patients with sickle cell disease and thalassemia who received transfusions

Ellen B. Fung, Paul R. Harmatz, Meredith Milet, Vinod Balasa, Samir K. Ballas, James F. Casella, Lee Hilliard, Abdullah Kutlar, Kenneth L. McClain, Nancy F. Olivieri, John B. Porter, Elliott P. Vichin

Journal:

Transfusion (Wiley)

Année:

2008

Langue:

english

Fichier:

PDF, 110 KB

english, 2008

41

A Simple Model to Assess and Improve Adherence to Iron Chelation Therapy with Deferoxamine in Patients with Thalassemia

ZAHRA PAKBAZ, ROLAND FISCHER, MARSHA TREADWELL, ROBERT YAMASHITA, ELLEN B. FUNG, LISA CALVELLI, KEITH QUIROLO, DRUCILLA FOOTE, PAUL HARMATZ, ELLIOTT P. VICHINSKY

Journal:

Annals of the New York Academy of Sciences

Année:

2005

Langue:

english

Fichier:

PDF, 1.76 MB

english, 2005

42

Treatment of Hepatitis C Virus Infection in Thalassemia

ELLEN BUTENSKY, ZAHRA PAKBAZ, DRUCILLA FOOTE, MARK C. WALTERS, ELLIOTT P. VICHINSKY, PAUL HARMATZ

Journal:

Annals of the New York Academy of Sciences

Année:

2005

Langue:

english

Fichier:

PDF, 1.36 MB

english, 2005

43

Monitoring Long-Term Efficacy of Iron Chelation Treatment with Biomagnetic Liver Susceptometry

ROLAND FISCHER, ANTONIO PIGA, PAUL HARMATZ, PETER NIELSEN

Journal:

Annals of the New York Academy of Sciences

Année:

2005

Langue:

english

Fichier:

PDF, 933 KB

english, 2005

44

Validation of the Natus CO-Stat™ End Tidal BreathAnalyzer in Children and Adults

Hendrik J. Vreman, Ronald J. Wong, Paul Harmatz, Avroy A. Fanaroff, Brian Berman, David K. Stevenson

Journal:

Journal of Clinical Monitoring and Computing

Année:

1999

Langue:

english

Fichier:

PDF, 139 KB

english, 1999

45

The genetics of pheromonally mediated aggression in mice. I. Strain differences in the capacity of male urinary odors to elicit aggression

Seymour Kessler, Paul Harmatz, Sara A. Gerling

Journal:

Behavior Genetics

Année:

1975

Langue:

english

Fichier:

PDF, 294 KB

english, 1975

46

Low ceruloplasmin levels during recovery from major burn injury: Influence of open wound size and copper supplementation

John J. Cunningham, Martha K. Lydon, Rene Emerson, Paul R. Harmatz

Journal:

Année:

1996

Langue:

english

Fichier:

PDF, 719 KB

english, 1996

47

Vitamin C treatment reduces elevated C-reactive protein

Gladys Block, Christopher D. Jensen, Tapashi B. Dalvi, Edward P. Norkus, Mark Hudes, Patricia B. Crawford, Nina Holland, Ellen B. Fung, Laurie Schumacher, Paul Harmatz

Journal:

Free Radical Biology and Medicine

Année:

2009

Langue:

english

Fichier:

PDF, 499 KB

english, 2009

48

Efficacy of Sapropterin Dihydrochloride in Increasing Phenylalanine Tolerance in Children with Phenylketonuria: A Phase III, Randomized, Double-Blind, Placebo-Controlled Study

Friedrich K. Trefz, Barbara K. Burton, Nicola Longo, Mercedes Martinez-Pardo Casanova, Daniel J. Gruskin, Alex Dorenbaum, Emil D. Kakkis, Eric A. Crombez, Dorothy K. Grange, Paul Harmatz, Mark H. Lips

Journal:

The Journal of Pediatrics

Année:

2009

Langue:

english

Fichier:

PDF, 505 KB

english, 2009

49

Postisolation aggression and olfactory cues

Paul Harmatz, R. Charles Boelkins, Seymour Kessler

Journal:

Behavioral Biology

Année:

1975

Langue:

english

Fichier:

PDF, 342 KB

english, 1975

50

Effect of thermal injury on transfer of IR22 IgA myeloma protein into bile in the rat

Wolf A. Cappeller, Kurt J. Bloch, Julie Fagundes, Edward A. Carter, David Sullivan, Paul R. Harmatz

Journal:

Liver International

Année:

1992

Langue:

english

Fichier:

PDF, 720 KB

english, 1992