Inhibitor development and substitution therapy in a developing country: Turkey

Küçük Resim Yok

Tarih

1998

Dergi Başlığı

Dergi ISSN

Cilt Başlığı

Yayıncı

Blackwell Science Ltd

Erişim Hakkı

info:eu-repo/semantics/closedAccess

Özet

Prevalence of inhibitor in developing countries, such as Turkey, where fresh frozen plasma (FFP) is still in use due to high cost of concentrates, is unknown. To determine the frequency of inhibitors in Turkish haemophiliacs exposed to blood products, 53 haemophilia A patients (age range 1-20; median: 11 years) and 12 haemophilia B patients (age range 3-20; median: 10 years), were evaluated; 31 haemophilia A patients (23 severe) received plasma-derived concentrates and 22 patients (10 severe) only FFP. No haemophilia B patients developed inhibitor, compared with seven of 53 (13%) haemophilia A patients, all with a severe defect (7/33; 21%) and treated with concentrates (7/23; 30%), whereas severe patients treated with FFP showed a lower risk to develop inhibitors (0/10, P = 0.07). Inhibitors were detected after 8-125 exposure days (median: 52). Intermediate-purity concentrates and pasteurization seemed to be linked with a higher risk of inhibitor compared to high-purity concentrates and solvent-detergent inactivation for seven patients with inhibitor. In four of seven inhibitor patients low-dose concentrate was administered at 25 IU kg(-1) twice weekly and inhibitor disappeared in 1-4 months. This regimen might be recommended for immune tolerance in developing countries for its lower cost.

Açıklama

Anahtar Kelimeler

developing countries, factor concentrates, fresh frozen plasma, haemophilia, inhibitors

Kaynak

Haemophilia

WoS Q Değeri

N/A

Scopus Q Değeri

Cilt

4

Sayı

2

Künye