1980 …2019
If you made any changes in Pure, your changes will be visible here soon.

Research Output 1980 2019

2019

Clinical trial of laronidase in Hurler syndrome after hematopoietic cell transplantation

Polgreen, L. E., Lund, T. C., Braunlin, E. A., Tolar, J., Miller, B. S., Fung, E., Whitley, C. B., Eisengart, J., Northrop, E., Rudser, K., Miller, W. P. & Orchard, P. J., Jan 1 2019, (Accepted/In press) In : Pediatric Research.

Research output: Contribution to journalArticle

Open Access
Mucopolysaccharidosis I
Cell Transplantation
Clinical Trials
Anti-Idiotypic Antibodies
Pharmaceutical Preparations
Cell Transplantation
Screening
Neuroimaging
Intensive care units
Blood
1 Citation (Scopus)

Late Mortality after Allogeneic Blood or Marrow Transplantation for Inborn Errors of Metabolism: A Report from the Blood or Marrow Transplant Survivor Study-2 (BMTSS-2)

Wadhwa, A., Chen, Y., Holmqvist, A., Wu, J., Ness, E., Parman, M., Kung, M., Hageman, L., Francisco, L., Braunlin, E. A., Miller, W., Lund, T. C., Armenian, S., Arora, M., Orchard, P. J. & Bhatia, S., Feb 1 2019, In : Biology of Blood and Marrow Transplantation. 25, 2, p. 328-334 7 p.

Research output: Contribution to journalArticle

Inborn Errors Metabolism
Survivors
Transplantation
Bone Marrow
Transplants

Recommendations for the management of MPS IVA: Systematic evidence- A nd consensus-based guidance

Akyol, M. U., Alden, T. D., Amartino, H., Ashworth, J., Belani, K. G., Berger, K. I., Borgo, A., Braunlin, E. A., Eto, Y., Gold, J. I., Jester, A., Jones, S. A., Karsli, C., Mackenzie, W., Marinho, D. R., McFadyen, A., McGill, J., Mitchell, J. J., Muenzer, J., Okuyama, T. & 9 others, Orchard, P. J., Stevens, B., Thomas, S., Walker, R., Wynn, R., Giugliani, R., Harmatz, P., Hendriksz, C. & Scarpa, M., Jun 13 2019, In : Orphanet Journal of Rare Diseases. 14, 1, 137.

Research output: Contribution to journalArticle

Open Access
Mucopolysaccharidoses
Consensus
N-acetylglucosamine-6-sulfatase
Guidelines
Delivery of Health Care

Recommendations for the management of MPS VI: Systematic evidence- and consensus-based guidance

Akyol, M. U., Alden, T. D., Amartino, H., Ashworth, J., Belani, K. G., Berger, K. I., Borgo, A., Braunlin, E. A., Eto, Y., Gold, J. I., Jester, A., Jones, S. A., Karsli, C., Mackenzie, W., Marinho, D. R., McFadyen, A., McGill, J., Mitchell, J. J., Muenzer, J., Okuyama, T. & 9 others, Orchard, P. J., Stevens, B., Thomas, S., Walker, R., Wynn, R., Giugliani, R., Harmatz, P., Hendriksz, C. & Scarpa, M., May 29 2019, In : Orphanet Journal of Rare Diseases. 14, 1, 118.

Research output: Contribution to journalArticle

Open Access
Mucopolysaccharidosis VI
Consensus
Patient Advocacy
Delivery of Health Care
N-Acetylgalactosamine-4-Sulfatase
2018
5 Citations (Scopus)
Salamandridae
Hedgehogs
regeneration
Regeneration
Cardiac Myocytes
1 Citation (Scopus)

Duration of high-dose aspirin therapy does not affect long-term coronary artery outcomes in Kawasaki disease

Migally, K., Braunlin, E. A., Zhang, L. & Binstadt, B. A., Jun 1 2018, In : Pediatric Research. 83, 6, p. 1136-1145 10 p.

Research output: Contribution to journalArticle

Open Access
Mucocutaneous Lymph Node Syndrome
Aspirin
Coronary Vessels
Intravenous Immunoglobulins
Therapeutics
Metabolic Syndrome X
Mucopolysaccharidosis I
Cell Transplantation
Hypertension
Transplantation

Tele-Pediatric Intensive Care for Critically Ill Children in Syria

Ghbeis, M. B., Steffen, K. M., Braunlin, E. A., Beilman, G. J., Dahman, J., Ostwani, W. & Steiner, M. E., Aug 1 2018, In : Telemedicine and e-Health. 24, 8, p. 621-623 3 p.

Research output: Contribution to journalArticle

Syria
Critical Care
Critical Illness
Pediatrics
Time Management
2017
4 Citations (Scopus)
Iduronidase
Mucopolysaccharidosis I
Angiotensin Receptors
Renin-Angiotensin System
Pathology

Curricula components for entrustable professional activities for the subspecialty of pediatric cardiology

Srivastava, S., Braunlin, E., Brown, D., Cabrera, A. G., Frank, L., Glickstein, J. S., Johnston, T., Koenig, P., McDaniel, G., Printz, B., Reinking, B., Wong, P. & Carraccio, C., Mar 1 2017, In : Progress in Pediatric Cardiology. 44, p. 17-32 16 p.

Research output: Contribution to journalArticle

Cardiology
Curriculum
Pediatrics
Guidelines
Physicians
13 Citations (Scopus)
Mucopolysaccharidosis I
Hematopoietic Stem Cell Transplantation
Survival
Mortality
Incidence
2 Citations (Scopus)
Open Access
Mucopolysaccharidoses
Carotid Intima-Media Thickness
Vascular Stiffness
Pediatrics
stiffness
2016
11 Citations (Scopus)

Cardiac issues in adults with the mucopolysaccharidoses: Current knowledge and emerging needs

Braunlin, E. & Wang, R., Jan 1 2016, In : Heart. 102, 16, p. 1257-1262 6 p.

Research output: Contribution to journalReview article

Mucopolysaccharidoses
Heart Valves
Rare Diseases
Glycosaminoglycans
Lysosomal Storage Diseases
9 Citations (Scopus)

Unique medical issues in adult patients with mucopolysaccharidoses

Mitchell, J., Berger, K. I., Borgo, A., Braunlin, E. A., Burton, B. K., Ghotme, K. A., Kircher, S. G., Molter, D., Orchard, P. J., Palmer, J., Pastores, G. M., Rapoport, D. M., Wang, R. Y. & White, K., Oct 1 2016, In : European Journal of Internal Medicine. 34, p. 2-10 9 p.

Research output: Contribution to journalReview article

Mucopolysaccharidoses
Glycosaminoglycans
Metabolic Diseases
Disease Management
Life Expectancy
2014
10 Citations (Scopus)
ATP-Binding Cassette Transporters
Paraquat
Oxidative Stress
Stem Cells
Cicatrix
13 Citations (Scopus)
Mucopolysaccharidosis II
Mucopolysaccharidosis I
Carotid Intima-Media Thickness
Vascular Stiffness
Mucopolysaccharidoses
20 Citations (Scopus)

Galsulfase (Naglazyme®) therapy in infants with mucopolysaccharidosis VI

Harmatz, P. R., Garcia, P., Guffon, N., Randolph, L. M., Shediac, R., Braunlin, E. A., Lachman, R. S. & Decker, C., Jan 1 2014, In : Journal of Inherited Metabolic Disease. 37, 2, p. 277-287 11 p.

Research output: Contribution to journalArticle

Mucopolysaccharidosis VI
Glycosaminoglycans
Safety
Therapeutics
Hearing
12 Citations (Scopus)

Unexpected coronary artery findings in mucopolysaccharidosis. Report of four cases and literature review

Braunlin, E. A., Orchard, P. J., Whitley, C. B., Schroeder, L., Reed, R. C. & Manivel, J. C., Jan 1 2014, In : Cardiovascular Pathology. 23, 3, p. 145-151 7 p.

Research output: Contribution to journalArticle

Mucopolysaccharidoses
Mucopolysaccharidosis I
Glycosaminoglycans
Coronary Vessels
Coronary Artery Disease
2013
59 Citations (Scopus)

Anaesthesia and airway management in mucopolysaccharidosis

Walker, R., Belani, K. G., Braunlin, E. A., Bruce, I. A., Hack, H., Harmatz, P. R., Jones, S., Rowe, R., Solanki, G. A. & Valdemarsson, B., Mar 1 2013, In : Journal of Inherited Metabolic Disease. 36, 2, p. 211-219 9 p.

Research output: Contribution to journalArticle

Mucopolysaccharidoses
Airway Management
Anesthetics
Anesthesia
Airway Obstruction
41 Citations (Scopus)

A Randomized, Double-Blind Trial of Lisinopril and Losartan for the Treatment of Cardiomyopathy in Duchenne Muscular Dystrophy

Allen, H. D., Flanigan, K. M., Thrush, P. T., Dvorchik, I., Yin, H., Canter, C., Connolly, A. M., Parrish, M., McDonald, C. M., Braunlin, E., Colan, S. D., Day, J., Darras, B. & Mendell, J. R., Jan 1 2013, In : PLoS Currents. DEC, ecurrents.md.2cc69a1dae4be7dfe2bcb420024ea865.

Research output: Contribution to journalArticle

Lisinopril
Losartan
Duchenne Muscular Dystrophy
Cardiomyopathies
Angiotensin Receptor Antagonists
6 Citations (Scopus)

Cardiac ultrasound findings in infants with severe (hurler phenotype) untreated mucopolysaccharidosis (MPS) type i

Schroeder, L., Orchard, P. J., Whitley, C. B., Berry, J. M., Tolar, J., Miller, W. P. & Braunlin, E. A., Jan 1 2013, JIMD Reports. Springer, p. 87-94 8 p. (JIMD Reports; vol. 10).

Research output: Chapter in Book/Report/Conference proceedingChapter

Mucopolysaccharidoses
Ultrasonics
Mucopolysaccharidosis I
Phenotype
Aortic Valve
51 Citations (Scopus)

Clinical overview and treatment options for non-skeletal manifestations of mucopolysaccharidosis type IVA

Hendriksz, C. J., Al-Jawad, M., Berger, K. I., Hawley, S. M., Lawrence, R., Mc Ardle, C., Summers, C. G., Wright, E. & Braunlin, E. A., Mar 1 2013, In : Journal of Inherited Metabolic Disease. 36, 2, p. 309-322 14 p.

Research output: Contribution to journalReview article

Mucopolysaccharidosis IV
Tooth Abnormalities
Tracheomalacia
Quality of Life
Uterine Cervical Dysplasia
10 Citations (Scopus)

Endothelial function in children and adolescents with mucopolysaccharidosis

Kelly, A. S., Metzig, A. M., Steinberger, J. & Braunlin, E. A., Mar 1 2013, In : Journal of Inherited Metabolic Disease. 36, 2, p. 221-225 5 p.

Research output: Contribution to journalArticle

Mucopolysaccharidoses
Enzyme Replacement Therapy
Hematopoietic Stem Cell Transplantation
Mucopolysaccharidosis II
Hyperemia
44 Citations (Scopus)

Enzyme replacement therapy for mucopolysaccharidosis VI: Long-term cardiac effects of galsulfase (Naglazyme®) therapy

Braunlin, E., Rosenfeld, H., Kampmann, C., Johnson, J., Beck, M., Giugliani, R., Guffon, N., Ketteridge, D., Sá Miranda, C. M., Scarpa, M., Schwartz, I. V., Leão Teles, E., Wraith, J. E., Barrios, P., Dias Da Silva, E., Kurio, G., Richardson, M., Gildengorin, G., Hopwood, J. J., Imperiale, M. & 3 others, Schatz, A., Decker, C. & Harmatz, P., Mar 1 2013, In : Journal of Inherited Metabolic Disease. 36, 2, p. 385-394 10 p.

Research output: Contribution to journalArticle

Mucopolysaccharidosis VI
Enzyme Replacement Therapy
Hypertrophy
Aortic Valve Insufficiency
Heart Valves
23 Citations (Scopus)

Noncanonical EF-hand motif strategically delays Ca2+ buffering to enhance cardiac performance

Wang, W., Barnabei, M. S., Asp, M. L., Heinis, F. I., Arden, E., Davis, J., Braunlin, E., Li, Q., Davis, J. P., Potter, J. D. & Metzger, J. M., Mar 1 2013, In : Nature Medicine. 19, 3, p. 305-312 8 p.

Research output: Contribution to journalArticle

EF Hand Motifs
Parvalbumins
Biological systems
Cations
Buffers
2012
2 Citations (Scopus)

Cardiac Involvement in the Mucopolysaccharide Disorders

Braunlin, E. A., Jan 13 2012, Pediatric Cardiovascular Medicine: Second Edition. Wiley Blackwell, p. 982-991 10 p.

Research output: Chapter in Book/Report/Conference proceedingChapter

Glycosaminoglycans
4 Citations (Scopus)

Increased longevity and metabolic correction following syngeneic BMT in a murine model of mucopolysaccharidosis type i

Wolf, D. A., Lenander, A. W., Nan, Z., Braunlin, E. A., Podetz-Pedersen, K. M., Whitley, C. B., Gupta, P., Low, W. C. & McIvor, R. S., Sep 1 2012, In : Bone Marrow Transplantation. 47, 9, p. 1235-1240 6 p.

Research output: Contribution to journalArticle

Iduronidase
Mucopolysaccharidoses
Mucopolysaccharidosis I
Glycosaminoglycans
Brain
2011
104 Citations (Scopus)

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

Braunlin, E. A., Harmatz, P. R., Scarpa, M., Furlanetto, B., Kampmann, C., Loehr, J. P., Ponder, K. P., Roberts, W. C., Rosenfeld, H. M. & Giugliani, R., Dec 1 2011, In : Journal of Inherited Metabolic Disease. 34, 6, p. 1183-1197 15 p.

Research output: Contribution to journalReview article

Mucopolysaccharidoses
Heart Diseases
Glycosaminoglycans
Enzyme Replacement Therapy
Ventricular Function
7 Citations (Scopus)

Clear cells in the atrioventricular valves of infants with severe human mucopolysaccharidosis (Hurler syndrome) are activated valvular interstitial cells

Braunlin, E., Tolar, J., MacKey-Bojack, S., Masinde, T., Krivit, W. & Schoen, F. J., Sep 1 2011, In : Cardiovascular Pathology. 20, 5, p. 315-321 7 p.

Research output: Contribution to journalArticle

Mucopolysaccharidoses
Mucopolysaccharidosis I
Myofibroblasts
Glycosaminoglycans
Staining and Labeling

Delayed Arterial Switch Operation for D-Transposition of the Great Arteries and Glucocorticoid Remediable Aldosteronism

Arain, N., Braunlin, E., Louis, J. S. & Bryant, R., Jan 1 2011, In : World Journal for Pediatric and Congenital Heart Surgery. 2, 2, p. 316-317 2 p.

Research output: Contribution to journalReview article

Transposition of Great Vessels
Hyperaldosteronism
Antihypertensive Agents
Heart Ventricles
Hypertension
2010
56 Citations (Scopus)

Mechanical circulatory support in patients with heart failure secondary to transposition of the great arteries

Joyce, D. L., Crow, S. S., John, R., St. Louis, J. D., Braunlin, E. A., Pyles, L. A., Kofflin, P. & Joyce, L. D., Nov 1 2010, In : Journal of Heart and Lung Transplantation. 29, 11, p. 1302-1305 4 p.

Research output: Contribution to journalArticle

Transposition of Great Vessels
Heart Failure
Heart-Assist Devices
Catheterization
Heart Diseases
21 Citations (Scopus)

Pre-transplant risk factors affecting outcome in Hurler syndrome

Orchard, P. J., Milla, C., Braunlin, E., Defor, T., Bjoraker, K., Blazar, B. R., Peters, C., Wagner, J. & Tolar, J., Jul 1 2010, In : Bone Marrow Transplantation. 45, 7, p. 1239-1246 8 p.

Research output: Contribution to journalArticle

Mucopolysaccharidosis I
Transplants
Enzyme Replacement Therapy
Intubation
Bronchiolitis
5 Citations (Scopus)
Heart-Assist Devices
False Aneurysm
Myocarditis
Aorta
Heart Failure
1 Citation (Scopus)

Targeted Sprouty1 overexpression in cardiac myocytes does not alter myocardial remodeling or function

Charles, N. J., Huebert, R. C., Lee, S., Adhikari, N., Polster, S., Rider, J. E., Braunlin, E. A., Mariash, A., Robledo, M., Schuweiler, D. & Hall, J. L., Sep 1 2010, In : Molecular and cellular biochemistry. 342, 1-2, p. 57-62 6 p.

Research output: Contribution to journalArticle

Cardiac Myocytes
Mitogen-Activated Protein Kinases
Tissue
Ventricular Myosins
Up-Regulation
2009
5 Citations (Scopus)
Mucopolysaccharidosis I
Matrix Metalloproteinases
Heart Diseases
Matrix Metalloproteinase 12
Acoustic Microscopy
2008
105 Citations (Scopus)

Enhancing efficacy of stem cell transplantation to the heart with a PEGylated fibrin biomatrix

Zhang, G., Hu, Q., Braunlin, E. A., Suggs, L. J. & Zhang, J. J., Jun 1 2008, In : Tissue Engineering - Part A.. 14, 6, p. 1025-1036 12 p.

Research output: Contribution to journalArticle

Hepatocyte Growth Factor
Stem Cell Transplantation
Stem cells
Fibrin
Injections
2007
13 Citations (Scopus)

The host immune response is essential for the beneficial effect of adult stem cells after myocardial ischemia

Tolar, J., Wang, X., Braunlin, E., McElmurry, R. T., Nakamura, Y., Bell, S., Xia, L., Zhang, J., Hu, Q., Panoskaltsis-Mortari, A., Zhang, J. & Blazar, B. R., Apr 1 2007, In : Experimental Hematology. 35, 4, p. 682-690 9 p.

Research output: Contribution to journalArticle

Adult Stem Cells
Myocardial Ischemia
Stem Cells
Myocardial Infarction
Mental Competency
2006
76 Citations (Scopus)

Cardiac Findings After Enzyme Replacement Therapy for Mucopolysaccharidosis Type I

Braunlin, E. A., Berry, J. M. & Whitley, C. B., Aug 1 2006, In : American Journal of Cardiology. 98, 3, p. 416-418 3 p.

Research output: Contribution to journalArticle

Mucopolysaccharidosis I
Enzyme Replacement Therapy
Heart Valves
Iduronidase
Hematopoietic Stem Cell Transplantation
35 Citations (Scopus)
Mucopolysaccharidosis I
Therapeutics
A73025
Aortic Valve
Coronary Vessels
2004
7 Citations (Scopus)

Thyroid dysfunction after pediatric cardiac surgery

Lynch, B. A., Brown, D. M., Herrington, C. & Braunlin, E. A., Jan 1 2004, In : Journal of Thoracic and Cardiovascular Surgery. 127, 5, p. 1509-1511 3 p.

Research output: Contribution to journalArticle

Thoracic Surgery
Thyroid Gland
Pediatrics
23 Citations (Scopus)

Troponin I level in a hemolytic uremic syndrome patient with severe cardiac failure

Askiti, V., Hendrickson, K., Fish, A. J., Braunlin, E. A. & Sinaiko, A. R., Mar 1 2004, In : Pediatric Nephrology. 19, 3, p. 345-348 4 p.

Research output: Contribution to journalArticle

Hemolytic-Uremic Syndrome
Troponin I
Heart Failure
Platelet Transfusion
Troponin
2003
32 Citations (Scopus)
Mucolipidoses
Bone Marrow
Transplants
Dysostoses
Lysosomal Storage Diseases
77 Citations (Scopus)

Usefulness of bone marrow transplantation in the Hurler syndrome

Braunlin, E. A., Stauffer, N. R., Peters, C. H., Bass, J. L., Berry, J. M., Hopwood, J. J. & Krivit, W., Oct 1 2003, In : American Journal of Cardiology. 92, 7, p. 882-886 5 p.

Research output: Contribution to journalArticle

Mucopolysaccharidosis I
Glycosaminoglycans
Bone Marrow Transplantation
Coronary Vessels
2001
39 Citations (Scopus)

Coronary artery patency following long-term successful engraftment 14 years after bone marrow transplantation in the hurler syndrome

Braunlin, E. A., Rose, A. G., Hopwood, J. J., Candel, R. D. & Krivit, W., Nov 1 2001, In : American Journal of Cardiology. 88, 9, p. 1075-1077 3 p.

Research output: Contribution to journalArticle

Vascular Patency
Mucopolysaccharidosis I
Fatal Outcome
Preschool Children
Bone Marrow Transplantation
21 Citations (Scopus)

Open-lung biopsy guides therapy in children

Jaklitsch, M. T., Linden, B. C., Braunlin, E. A., Bolman, R. M. & Foker, J. E., Jul 3 2001, In : Annals of Thoracic Surgery. 71, 6, p. 1779-1785 7 p.

Research output: Contribution to journalArticle

Biopsy
Lung
Extracorporeal Membrane Oxygenation
Pulmonary Hypertension
Mechanical Ventilators
2000
7 Citations (Scopus)

Radiological case of the month

Braunlin, E. A., Krivit, W., Burke, B. A., Rocchini, A. P., Foker, J. E. & Whitley, C. B., Jan 1 2000, In : Archives of Pediatrics and Adolescent Medicine. 154, 8, p. 841-842 2 p.

Research output: Contribution to journalArticle

Mucopolysaccharidosis I
Aortic Coarctation
Bone Marrow Transplantation
1998
14 Citations (Scopus)

Usefulness of surveillance endomyocardial biopsy after pediatric cardiac transplantation

Braunlin, E. A., Shumway, S. J., Bolman, R. M., McDonald, K. M., Ring, W. S., Olivari, M. T. & Nakhleh, R. E., Jun 1 1998, In : Clinical Transplantation. 12, 3, p. 184-189 6 p.

Research output: Contribution to journalArticle

Heart Transplantation
Pediatrics
Biopsy
Immunosuppression
Transplantation
1995
55 Citations (Scopus)

Antibody response of pediatric solid organ transplant recipients to immunization against influenza virus

Mauch, T. J., Crouch, N. A., Freese, D. K., Braunlin, E. A., Dunn, D. L. & Kashtan, C. E., Jan 1 1995, In : The Journal of Pediatrics. 127, 6, p. 957-960 4 p.

Research output: Contribution to journalArticle

Orthomyxoviridae
Antibody Formation
Immunization
Pediatrics
Transplants
15 Citations (Scopus)

Echocardiographic-Guided Internal Jugular Venous Cannulation in Children With Heart Disease

Etheridge, S. P., Berry, J. M., Krabill, K. A. & Braunlin, E. A., Jan 1 1995, In : Archives of Pediatrics & Adolescent Medicine. 149, 1, p. 77-80 4 p.

Research output: Contribution to journalArticle

Catheterization
Jugular Veins
Heart Diseases
Neck
Pediatrics