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Paul Harmatz

University of California, San Francisco · US
Area of research
Physiology · Rheumatology
Research interest
Research interests include Lysosomal Storage Disorders Research, Glycogen Storage Diseases and Myoclonus, Hemoglobinopathies and Related Disorders, and Iron Metabolism and Disorders.
h-index
64
citations
14,350
works
519
NIH funding
primary concept
email

Recent publications

Long-term efficacy and safety of arimoclomol in Niemann-Pick disease type C: Final results of the phase 2/3 NPC-002 48-month open-label extension trial
Molecular Genetics and Metabolism 2025cited by 1position: middledoi
Clinical characteristics and real-world outcomes in patients with mucopolysaccharidosis II over 18 years: final report of the Hunter Outcome Survey
Molecular Genetics and Metabolism 2025cited by 1position: middledoi
Persistent growth-promoting effects of vosoritide in children with achondroplasia are accompanied by improvements in physical and social aspects of health-related quality of life
Genetics in Medicine 2024cited by 17position: middledoi
Sustained growth-promoting effects of vosoritide in children with achondroplasia from an ongoing phase 3 extension study
Med 2024cited by 16position: middledoi
Vosoritide therapy in children with achondroplasia aged 3−59 months: a multinational, randomised, double-blind, placebo-controlled, phase 2 trial
The Lancet Child & Adolescent Health 2023cited by 60position: middledoi
Intrauterine enzyme replacement therapies for lysosomal storage disorders: Current developments and promising future prospects
Prenatal Diagnosis 2023cited by 22position: middledoi
First-in-human in vivo genome editing via AAV-zinc-finger nucleases for mucopolysaccharidosis I/II and hemophilia B
Molecular Therapy 2022cited by 80position: firstdoi
Growth parameters in children with achondroplasia: A 7-year, prospective, multinational, observational study
Genetics in Medicine 2022cited by 27position: middledoi
Safe and persistent growth-promoting effects of vosoritide in children with achondroplasia: 2-year results from an open-label, phase 3 extension study
Genetics in Medicine 2021cited by 93position: middledoi
Evaluation of the long-term treatment effects of intravenous idursulfase in patients with mucopolysaccharidosis II (MPS II) using statistical modeling: data from the Hunter Outcome Survey (HOS)
Orphanet Journal of Rare Diseases 2021cited by 27position: middledoi
Once-daily, subcutaneous vosoritide therapy in children with achondroplasia: a randomised, double-blind, phase 3, placebo-controlled, multicentre trial
The Lancet 2020cited by 194position: middledoi
C-Type Natriuretic Peptide Analogue Therapy in Children with Achondroplasia
New England Journal of Medicine 2019cited by 215position: middledoi
Recommendations for the management of MPS IVA: systematic evidence- and consensus-based guidance
Orphanet Journal of Rare Diseases 2019cited by 114position: middledoi
Efficacy and Safety of Asfotase Alfa in Infants and Young Children With Hypophosphatasia: A Phase 2 Open-Label Study
The Journal of Clinical Endocrinology & Metabolism 2019cited by 75position: middledoi
Recommendations for the management of MPS VI: systematic evidence- and consensus-based guidance
Orphanet Journal of Rare Diseases 2019cited by 62position: middledoi
Comprehensive long‐term efficacy and safety of recombinant human alpha‐mannosidase (velmanase alfa) treatment in patients with alpha‐mannosidosis
Journal of Inherited Metabolic Disease 2018cited by 56position: middledoi
Enzyme replacement therapy with velmanase alfa (human recombinant alpha-mannosidase): Novel global treatment response model and outcomes in patients with alpha-mannosidosis
Molecular Genetics and Metabolism 2018cited by 49position: firstdoi
Observational Prospective Natural History of Patients with Sanfilippo Syndrome Type B
The Journal of Pediatrics 2018cited by 40position: middledoi
Health Related Quality of Life, Disability, and Pain in Alpha Mannosidosis
Journal of Inborn Errors of Metabolism and Screening 2018cited by 9position: middledoi
Monitoring guidance for patients with hypophosphatasia treated with asfotase alfa
Molecular Genetics and Metabolism 2017cited by 128position: middledoi
Ten years of the Hunter Outcome Survey (HOS): insights, achievements, and lessons learned from a global patient registry
Orphanet Journal of Rare Diseases 2017cited by 64position: middledoi
The effect of galsulfase enzyme replacement therapy on the growth of patients with mucopolysaccharidosis VI (Maroteaux-Lamy syndrome)
Molecular Genetics and Metabolism 2017cited by 47position: firstdoi
Long-term endurance and safety of elosulfase alfa enzyme replacement therapy in patients with Morquio A syndrome
Molecular Genetics and Metabolism 2016cited by 68position: lastdoi
Impact of long‐term elosulfase alfa treatment on respiratory function in patients with Morquio A syndrome
Journal of Inherited Metabolic Disease 2016cited by 35position: lastdoi
Impact of elosulfase alfa in patients with morquio A syndrome who have limited ambulation: An open‐label, phase 2 study
American Journal of Medical Genetics Part A 2016cited by 34position: firstdoi
Neurocognition across the spectrum of mucopolysaccharidosis type I: Age, severity, and treatment
Molecular Genetics and Metabolism 2015cited by 67position: middledoi
Safety and physiological effects of two different doses of elosulfase alfa in patients with morquio a syndrome: A randomized, double‐blind, pilot study
American Journal of Medical Genetics Part A 2015cited by 39position: lastdoi
Long-term velaglucerase alfa treatment in children with Gaucher disease type 1 naïve to enzyme replacement therapy or previously treated with imiglucerase
Molecular Genetics and Metabolism 2015cited by 38position: middledoi
Efficacy and safety of enzyme replacement therapy with BMN 110 (elosulfase alfa) for Morquio A syndrome (mucopolysaccharidosis IVA): a phase 3 randomised placebo‐controlled study
Journal of Inherited Metabolic Disease 2014cited by 211position: middledoi
International guidelines for the management and treatment of Morquio A syndrome
American Journal of Medical Genetics Part A 2014cited by 138position: middledoi

Grants

No grants ingested yet.

Frequent collaborators

Christian J. Hendriksz · University of Manchester14 papers (2012–2018) · 13 papers (2012–2025)Barbara K. Burton · Northwestern University12 papers (2012–2025)Simon Jones · New York University11 papers (2012–2018) · 10 papers (2012–2018)Julian Raiman · Hospital for Sick Children7 papers (2014–2025)Christina Lampe · Johannes Gutenberg University Mainz6 papers (2012–2017) · 6 papers (2013–2017) · 6 papers (2012–2025) · 6 papers (2013–2017)Michael Beck · Johannes Gutenberg University Mainz6 papers (2012–2017) · 5 papers (2012–2016)Maurizio Scarpa · University of Padua5 papers (2012–2017) · 5 papers (2012–2016)John James Mitchell · McGill University Health Centre5 papers (2013–2016)Kenneth I. Berger · New York University5 papers (2014–2016) · 4 papers (2013–2016)Gregory M. Pastores · New York University4 papers (2012–2015)Eugen Mengel · Johannes Gutenberg University Mainz4 papers (2013–2025)Heather Lau · Yale University4 papers (2014–2022)