RecruitingPhase 2Phase 3NCT05208281

A Multi-cohort Study of Safety, Efficacy, PK and PD of GNR-055 in Patients With Mucopolysaccharidosis Type II

Multicenter, Open-Label, Multi-cohort Study to Evaluate Safety, Pharmacokinetics, Pharmacodynamics, and Efficacy of Drug Product GNR 055 (JSC "GENERIUM", Russia) in Patients With Mucopolysaccharidosis Type II


Sponsor

AO GENERIUM

Enrollment

32 participants

Start Date

Nov 30, 2021

Study Type

INTERVENTIONAL

Conditions

Summary

This is phase 2/3 study to evaluate the safety, pharmacokinetics, pharmacodynamics, and efficacy of the investigational product GNR-055 in MPS II (Hunter syndrome) patients of different age groups.


Eligibility

Sex: MALE

Inclusion Criteria5

  • Signed inform consent;
  • Verified diagnosis of MPS II (Hunter syndrome);
  • Naïve patients or patients who have received standard ERT whit idursulfase products;
  • No contraindications for lumbar puncture as judged by the Investigator;
  • Willingness and ability to follow study procedures.

Exclusion Criteria4

  • Clinically pronounced hypersensitivity to ID2S or any other component of the drug product;
  • History of hematopoietic stem cell transplantation (HSCT) or bone marrow transplantation;
  • Implanted or external non-removable metal devices, a cardiac pacemaker, or other objects sensitive to the magnetic field that may pose a danger to both the wearer and the correct operation of magnetic resonance imaging (MRI) equipment;
  • Concomitant diseases and conditions that, in the Investigator's opinion, can put at risk the patient's safety during his/her participation in the study, or which will influence the safety data analysis in case of the disease/condition exacerbation during the study.

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Interventions

DRUGGNR-055 1.0-2.0-3.0 mg/kg

Weekly IV infusion (lyophilized powder) 1.0-2.0-3.0 mg/kg

DRUGGNR-055 2.0 mg/kg

Weekly IV infusion (lyophilized powder) 2.0 mg/kg

DRUGGNR-055 3.0 mg/kg

Weekly IV infusion (lyophilized powder) 3.0 mg/kg


Locations(5)

Federal State-Funded Healthcare Institution Central Clinical Hospital of the Russian Academy of Sciences (Research Institute of Pediatrics and Child Health Protection of the Central Clinical Hospital of the Russian Academy of Sciences)

Moscow, Russia

Federal State Budgetary Educational Institution of Higher Education "St. Petersburg State Pediatric Medical University" of the Ministry of Health of the Russian Federation

Saint Petersburg, Russia

V.I. Vernadsky Crimean Federal University

Simferopol, Russia

State Budgetary Healthcare Institution Republican Medical Genetic Center

Ufa, Russia

State Autonomous Healthcare Institution of the Sverdlovsk Region Regional Children's Clinical Hospital

Yekaterinburg, Russia

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NCT05208281


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