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Výstupy z registru ČNHP za rok 2024: hemofilie
The status of care for persons with haemophilia registered within CNHP registry
Sample size, valid records - This slide describes the process of records‘ validation within the registry. All patients have signed IC.
Persons with haemophilia (PWH)
Centres participating in CNHP - Centres contributing to the CNHP registry.
Basic demographics
Persons with haemophilia and inhibitors in 2024
ABR and treatment regimens in patients with inhibitor
ABR according to treatment regimen in PWH with inhibitor - (Almost all) Patients with inhibitors, are on permanent prophylaxis with emicizumab. This treatment led to minimal ABR in all HA inhibitor patients.
Demographic characteristics Haemophilia A
Severity of haemophilia A
Age at diagnosis according to severity of haemophilia A
Actual age according to severity of haemophilia A- Mean age of Czech adults with HA is over 40 years. Mean age of children with HA is around 10 years.
Hepatitis (ever) experienced - No HepC infection in children since late 90’s. None of Czech children with HA is infected with Hepatitis C.
Hepatitis (ever) experienced - There was NO NEW HepC infection in 2023.
HIV
Treatment outcomes and bleeding frequency Haemophilia A
Data from year 2024 – sample size
Frequency of bleeding requiring treatment in 2024
Location of bleeds in 2024
Location of bleeds in 2024
Preventive administration in 2024 - his figure refers to preventive factor’s administration in children with HA, applied less often, than in 2023.
Preventive administration in 2024 - This figure refers to preventive treatment in adults with HA. No significant change compared to 2023.
ABR according to treatment regimen Haemophilia A without inhibitor
Annual bleeding rate according to treatment regimen
Annual bleeding rate according to treatment regimen
ABR according to treatment regimen and age
Joint and other bleeds according to treatment regimen and age
Location and etiology of bleeds - No major difference between HA and HB bleeding. Most of bleeds are spontaneous joint bleeds, most frequently elbow.
Detailed treatment of bleeds
Location and etiology of bleeds
Detailed treatment of bleeds - Joint bleeds in children were treated with median dose of 3000 IU FIX administered in23 infusions in two days. There was no muscle bleed in 2024.
ABR according to centres Haemophilia A (PWHA)
Annual bleeding rate on permanent prophylaxis
Annual bleeding rate on permanent prophylaxis
Annual bleeding rate regardless prophylaxis
Annual bleeding rate regardless prophylaxis - Similar information for adults.
Prophylactic regimens and treatment outcomes
Prophylactic regimens and treatment outcomes
Type of treatment (subgroup of treated patients)
Type of treatment (subgroup of treated patients)
Emicizumab treatment in 2024
Demographic characteristics Haemophilia B
Severity of haemophilia B
Age at diagnosis according to severity of haemophilia B - There is no major difference in demographics between HA and HB.
Actual age according to severity of haemophilia B - There is no major difference in demographics between HA and HB, perhaps adults with HB are slightly older than those, with HA.
Hepatitis (ever) experienced - NO HepC infection in children since late 90’s. None of Czech children with HB is infected with Hepatitis C.
Hepatitis (ever) experienced - There was NO NEW HepC infection in 2024.
HIV
Treatment outcomes and bleeding frequency Haemophilia B
Data from year 2024 – sample size - See previous comment for the same slide related to HA.
Frequency of bleeding requiring treatment in 2024
Location of bleeds in 2024 - There was no CNS bleed in children with haemophilia B in 2024. 66.% of children had no bleed at all. Was 67,6% in 2023.
Location of bleeds in 2024 - Bleeding events in adults. 70.1% HB adults with no bleed (similar to 2023).
Preventive administration in 2024 - This figure refers to preventive factors administration in children with HB. This figure refers to preventive factor administration in adults with HB.
Preventive administration in 2024
ABR according to treatment regimen Haemophilia B without inhibitor
Annual bleeding rate according to treatment regimen
Annual bleeding rate according to treatment regimen
Location and etiology of bleeds
Detailed treatment of bleeds
Location and etiology of bleeds - In contrary to HA, joint bleeds are relatively more frequent in children with HB. 57,4% of all bleeds and 61,5% of joint bleeds are traumatic.
Detailed treatment of bleeds
ABR according to centres Haemophilia B (PWHB)
Annual bleeding rate on permanent prophylaxis - Not all centres treat children with HB. The median ABR is similar to HA.
Annual bleeding rate on permanent prophylaxis - Similar situation for adults with HB.
Annual bleeding rate regardless prophylaxis - This slide describes the treatment of children with HB regardless of prophylaxis in those centres, which treat PWHB.
Annual bleeding rate regardless prophylaxis - This slide describes the treatment of adults with HB regardless of prophylaxis in those centres, which treat PWHB.
Prophylactic regimens and treatment outcomes
Prophylactic regimens and treatment outcomes
Type of treatment (subgroup of treated patients) - Similar numbers of children on prophylaxis as in 2024. All of them on permanent prophy.
Type of treatment (subgroup of treated patients) - No major change compared to 2023. More adults are, however, taking the advantage of home treatment.
Treatment data and factor consumption Haemophilia A and B
Treatment
Treatment
Treatment
Comparison of treatment in years 2024 and 2023 - This table compares data between 2023 and 2024. Mainly you can see, further move from pdF to rF, mainly to EHLs
Comparison of treatment in years 2024 and 2023
Comparison of treatment in years 2024 and 2023
Comparison of treatment in years
Comparison of treatment in years - Trends in children.
Comparison of treatment in years - Same information for adults.
Consumption of drugs
Consumption of drugs
Consumption of drugs
