28. June - 1. July 2012
12th International symposium on MPS and related diseases
Noordwijkerhout, The Netherlands

Scientific program

Thursday 28. June 2012
15:00         
Registration
17:00
Words of Welcome
Hanka Meutgeert, chair of the Dutch patient and family organization for metabolic disorders
Ans van der Ploeg & Frits Wijburg, on behalf of the scientific committee
17:15
        
Regenerative medicine: promises for future treatment
Christine Mummery, the Netherlands
18:30 Welcome reception
Snack & Drinks

Friday 29. June 2012
07:00 - 08:45   
Non-Skeletal Manifestations of MPS IVA Breakfast Symposium
BioMarin Pharmaceutical Inc.

Theme: Musculoskeletal disease and Mucopolysaccharidoses
09:00 Session 1 - scientific & patient program
Bones and joints: defining the problem
Chairs: Calogera Simonaro & Michael Beck
09:00
The patient’s story - short movie
09:05
      
General overview of bone and cartilage tissue; growth and metabolism en clinical issues
Ans van de Ploeg & Arnold Reuser,
the Netherlands
09:35
      
Challenges in treating musculoskeletal disease in MPSs
Katherine Ponder, USA

10:05
Practical approaches to musculoskeletal disease in MPSs
Bianca Link, Germany


10:30
Coffee break


11:00
Session 2 - scientific program
Musculosketal disease: pathophysiology and treatment
Chairs: Joseph Muenzer & Katherine Ponder
11:00
ERT and the musculoskeletal disease in MPSs: results and challenges
Simon Jones, UK
11:15
       
MPS IX: clinical presentation and lessons from mice
Barbara Triggs-Raine, Canada
11:35
Activation of the TLR4 pathway in the MPS: potentials for therapy
Calogera Simonaro, USA


12:00
General discussion
Oral presentations
12:10
Pathogenesis of vertebral bone disease in the cervical spines of mucopolysaccharidosis I dogs
Joseph Chiaro, USA
12:20
      
Diagnosis of mucopolysaccharidosis IVA: a comprehensive algorithm encompassing clinical, radiographic, and clinical laboratory data
Chris Hendriksz, UK



12:30
Lunch


12:30 Lunch symposium - Genzyme



Theme: The central nervous system (CNS) and Mucopolysaccharidoses
13:30
Session 3 - scientific & patient program
The Brain in MPSs and other LSDs: defining the problems and approaches for therapy
Chairs: Roberto Giugliani & Frits Wijburg
13:30
The patient’s story - Short movie
13:35
     

Overview of CNS disease in MPS
Maurizio Scarpa, Italy
14:05
        
Challenges in treating the brain in MPSs
David Begley, United Kingdom


14:30
Emerging gene therapy strategies for lysosomal storage diseases
Beverly Davidson, USA


15:00
Tea break


15:30
Session 4- scientific program
The Brain in MPSs and other LSDs
Chairs: Jean Michel Heard & Brian Bigger
15:30
      
Bringing brain therapies to the bedside for MPS
Patricia Dickson, USA
15:55
      
Biomarkers for brain disease: lessons learned from other degenerative brain diseases
Niklas Mattson, Sweden
16:20
     
Exploring brain structure and function in MPS disorders
Elsa Shapiro, USA
Oral presentations
16:45
Injection of rhSGSH into the spinal CSF in MPS IIIA dogs ameliorates neuropathology in brain
Kim Hemsley, Australia

16:55
Long Circulating Enzyme Replacement Therapy
Shunji Tomatsu, USA
17:05
  
Heparan sulfate in plasma and GAGs in urine correlate with disease severity in Sanfilippo disease
Jessica de Ruijter, the Netherlands


17:15
Drinks & snacks & poster walks


Saturday 30. June 2012
09:00          Session 5 - scientific & patient program
Novel approaches for treatment and early diagnosis
Chairs: George Ruiter & John Hopwood
09:00
      
Intrathecal enzyme therapy for MPS II and III
Joseph Muenzer, USA
09:20
      
Genistein: results of clinical trials
Frits Wijburg, the Netherlands
09:40
      
Genistein: animal studies and a new trial
Brian Bigger, United Kingdom
10:00
      
Animal models and innovative therapies for brain disease
Kim Hemsley, Australia
10:20
General discussion


10:30
Coffee break


11:00
Session 6 - scientific program
Novel approaches for treatment and early diagnosis challenges and solutions
Chairs: Arnold Reuser & Vassili Valayannopoulos
11:00
Gene Therapy for Sanfilippo syndrome
Jean Michel Heard, France
11:20
      
Gene therapy for MPS VI
Alberto Auricchio, Italy
11:40
Gene therapy by transfecting autologous hematopoietic stem cells
Alessandra Biffi, Italy
Oral presentations
12:00
      
A single systemic rAAV9-hNAGLU gene delivery: normalized survival and permanent restoration of NAGLU activity in the CNS, PNS and somatic tissues in MPS IIIB mice
Haiyan Fu, USA
12:10
Lentiviral SGSH expression from the human CD11b promoter in transplanted haematopoietic stem cells fully corrects behaviour and neuropathology of Mucopolysaccharidosis IIIA mice
Alexander Langford-Smith, United Kingdom
12:20
Heparan Sulphate Inhibits CXCL12-mediated Hematopoietic Cell Migration and Engraftment in a Mouse Model of Mucopolysaccharidosis I
Brian Bigger, United Kingdom


12:30
Lunch


12:30
Lunch symposium - Shire


13:30
Session 7 - scientific & patient program
Drug pricing and access to treatment
Chairs: Ans van de Ploeg & Alasdair Parker
13:30
Orphan drug development: successes and challenges
Bert Leufkens, the Netherlands
13:50
      
Perspectives of patients and families
Christine Lavery, United Kingdom
14:10
The doctor’s perspective
Carla Hollak, the Netherlands
14:30
     
Perspective of regulating authorities
Wim Goettsch, the Netherlands
14:50
      
Cost effectiveness and orphan drugs
Michael Schlander, Germany
15:10
General discussion


15:30
Tea break


16:00
Session 8 - scientific & patient program
Updates on treatment outcomes
Chairs: Maurizio Scarpa & David Begley
16:00
MPS I
Roberto Giugliani, Brazil
16:15
      
MPS II
Michael Beck, Germany
16:30
MPS IV
Chris Hendriksz, United Kingdom
16:45
      
MPS VI
Vassili Valayannopoulos, France
Oral presentations
17:00 Anti-idursulfase Antibody and the Efficacy of Enzyme Replacement Therapy in Mucopolysaccharidosis Type II Patients
Akemi Tanaka, Japan
17:10 Long-term Experience with Enzyme Replacement Therapy (ERT) in MPS II Patients with a Severe (Neuronopathic) Phenotype: an International Case Series
Christina Lampe, Germany
17:20
International multicenter study to identify predictors of long term outcome of Hurler syndrome patients after successful hematopoietic stem cell transplantation
Jaap Jan Boelens, the Netherlands
17:30
 
Lysosomal storage, neurodegeneration and brain inflammation in the mouse model of MPS III type C
Alexey Pshezhetsky, Canada


17:40
Drinks & snacks & poster walks


19:00
Congress dinner


 

Sunday 1. July 2012
09:00         
       
MPS 2012 lectureMPSs: experiences from the past and lessons for the future
scientific & patient program
John Hopwood, Australia


09:40
Short break


09:45
Late breaking news - scientific program
Chairs: Lorne Clarke and Ben Poorthuis
09:45
     
Modulating cellular clearance in lysosomal storage diseases
Andrea Ballabio, Italy
10:00
      
MPS IIIE - a novel mucopolysaccharidosis caused by deficiency of Arylsulfatase G
Thomas Dierks, Germany
10:15
    
ERT for Alpha Mannosidosis
Line Borgwardt, Denmark
10:30
      
Differential Proteomic and Genome-wide Expression Studies of the Developing MPS Growth Plate Identifies Profound and Complex Alterations of Chondrocyte Homeostasis
Jonathan Heppner, Canada
10:45
       
Rapid CNS Correction in MPS IIIA Mice Using Systemic Delivery of Self-complementary AAV9 and AAVrh74 SGSH Vectors
Douglas McCarty, USA


11:00
Coffee break


11:30
Poster prizes
11:50
Presentation MPS 2013
International Symposium - Brazil
12:00
Closing remarks and farewell
12:15
End of the meeting