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An Investigator-initiated study on rFVIIa prophylaxis in children with haemophilia A and inhibitors

Phase 1
Conditions
Hemophilia A
MedDRA version: 14.1Level: PTClassification code 10056492Term: Haemophilia A with anti factor VIIISystem Organ Class: 10010331 - Congenital, familial and genetic disorders
Therapeutic area: Diseases [C] - Blood and lymphatic diseases [C15]
Registration Number
EUCTR2009-010172-21-PL
Lead Sponsor
Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico
Brief Summary

Not available

Detailed Description

Not available

Recruitment & Eligibility

Status
ot Recruiting
Sex
Male
Target Recruitment
50
Inclusion Criteria

• Male patients with haemophilia A who have been treated with FVIII on demand or on prophylaxis and who have developed inhibitors to FVIII.
• Age < 8 years.
• = 2 years from the time of first inhibitor detection.
• High-responding inhibitors (historical peak > 5 BU/mL) and known anamnestic response in case of negative inhibitor titre.
• Patient is candidate for daily ITI treatment with doses of FVIII ranging from 50 IU/kg/day to 200 IU/kg/day.
• Maximal two bleedings in the same joint within the last 6 months before entering the study or maximal six joint bleeds in the same joint within 2 years.
• Adequate venous access for daily infusion and capable (patient or caregiver) of reconstituting and injecting the study drug (as judged by the Investigator).
• Informed consent by parents or legal guardians.
Are the trial subjects under 18? yes
Number of subjects for this age range:
F.1.2 Adults (18-64 years) no
F.1.2.1 Number of subjects for this age range
F.1.3 Elderly (>=65 years) no
F.1.3.1 Number of subjects for this age range

Exclusion Criteria

• ITI already started.
• Known or suspected hypersensitivity to the active substance, or to any of the excipients of the study drug, or to mouse, hamster or bovine protein.
• Administration of any investigational product within 30 days prior to randomisation.
• Other coagulation disorders than congenital haemophilia A.
• Family history of thrombosis at an early age (< 40 years), known thrombophilia, any previous thrombosis including catheter-related deep vein thrombosis, previous neonatal thrombosis.
• Known pseudo tumours.
• Known severe liver disease.
• Platelet count < 50,000 platelets/µL at screening.
• Surgery within one month or planned major and/or orthopaedic surgery.

Study & Design

Study Type
Interventional clinical trial of medicinal product
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
Main Objective: The objective of this study is to evaluate the efficacy and safety of a prophylactic regimen with rFVIIa in reducing the frequency of joint bleeds and the development of signs of joint damage including synovitis, as measured by the Hemophilia Joint Health Score (HJHS), in children with haemophilia A who develop high-responding inhibitors.;Secondary Objective: ;Primary end point(s): Total number of joint bleeds.;Timepoint(s) of evaluation of this end point: The last assessment of efficacy (clinical outcome) will take place at the final visit (Month 18, or earlier if the patient has achieved factor VIII recovery >= 25% or prematurely discontinues the study). For the evaluation of efficacy, clinical outcome measures (such as bleeding episodes) as of randomisation will be taken into account. Primary endpoint
Secondary Outcome Measures
NameTimeMethod
Secondary end point(s): • Number of re-bleeds in the same joint (re-bleed is defined as bleed occurring in the same joint within 72 hours of last treatment given).<br>• Time interval between haemorrhages in the same joint. <br>• Number of non-joint bleeds.<br>• Number of life threatening bleeds.<br>• Number of limb-threatening bleeds.<br>• Total number of all bleeds.<br>• Joint status evaluated by the HJHS.<br>• Days of hospitalisation, days lost from school/work (caregivers).<br>• Adherence to the assigned treatment regimen.<br>;Timepoint(s) of evaluation of this end point: The last assessment of efficacy (clinical outcome) will take place at the final visit (Month 18, or earlier if the patient achieves FVIII recovery ? 25% or prematurely discontinues the study).
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