International Niemann–Pick Disease Alliance

Clinical trials with results moving on to next steps with regulators/HTA

Disease Study Drug Route Sponsor Trial Name Trial sites with enrolled patients Clinicaltrails.gov  reference # Current status
NPC Adrabetadex 2-hydroxyproply-beta cyclodextrin Intrathecal Mandos Health Adrabetadex to Treat Niemann-Pick Type C1 (NPC1) Disease Australia, France,
Germany,
New Zealand,
Singapore,
Switzerland,
Turkiye
United Kingdom,
USA
NCT04958642 NDA submitted to FDA
NPC Adrabetadex
2-hydroxyproply-beta cyclodextrin
IV Washington University Study of IV VTS-270 for infantile liver disease associated with NPC St Louis,
Missouri,
USA
NCT03471143 Study completed
NPC Arimoclomol/Miplyffatm oral Zevra Therapeutics Arimoclomol Prospective Study in Participants Diagnosed With Niemann-Pick Disease Type C Denmark, France,
Italy,
Poland, Germany, Spain,  Switzerland,
United Kingdom
USA
NCT02612129 Approved by FDA September 2024.
Further regulatory submissions including EMA pendin
NPC N-acetyl-L-leucine/Aqneursatm oral IntraBio A Pivotal Study of N-Acetyl-L-Leucine on Niemann-Pick Disease Type C Denmark, France,
Italy, Poland, Germany, Spain, Switzerland,, United Kingdom,
USA
NCT05163288 Approved by FDA September 2024.
NDA submitted to EMA.  Further regulatory submissions pending
ASMD Olipudase Alfa/Xenpozymetm IV Sanofi Efficacy, Safety, Pharmacodynamic, and Pharmacokinetics Study of Olipudase Alfa in Patients With Acid Sphingomyelinase Deficiency (ASCEND) Argentina, Australia, Belgium, Brazil, Bulgaria, Chile, France, Germany, Italy, Japan, Netherlands, Portugal, Spain, Tunisia, Turkey, UK, USA NCT02004691 Regulatory approval received from FDA, EMA and others, global submissions ongoing,
Not all regulatory approvals are accompanied by HTA approvals for funding
ASMD Olipudase Alfa/Xenpozymetm IV Sanofi Safety, Tolerability, PK, and Efficacy Evaluation of Repeat Ascending Doses of Olipudase Alfa in Pediatric Patients <18 Years of Age With Acid Sphingomyelinase Deficiency (ASCEND-Peds) Brazil,
France,
Germany,
Italy,
United Kingdom
USA
NCT02292654 As above
ASMD Olipudase Alfa/Xenpzymetm IV Sanofi Data Analysis of Adult and Pediatric Participants With Acid Sphingomyelinase Deficiency (ASMD) on Early Access to Olipudase Alfa in France (OPERA) France NCT05359276 Study complete

Further resources to understand clinical trials and drug development:

What patients need to know about clinical trials – resource from the FDA https://www.fda.gov/patients/clinical-trials-what-patients-need-know/basics-about-clinical-trials

The drug development process – resource from the FDA https://www.fda.gov/patients/learn-about-drug-and-device-approvals/drug-development-process

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