Hemofilik çocuklarda inhibitör gelişme sıklığı ve inhibitörlü hastaların izlemi
Küçük Resim Yok
Tarih
1998
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Pıhtılaşma faktörleri konsantrelerinin aşırı pahalı olması ve sosyal güvenlik sorunları nedeniyle taze dondurulmuş plazmanın (TDP) önemli oranda kullanıldığı Türkiye gibi gelişmekte olan ülkelerde inhibitor gelişim sıklığı bilinmemektedir. Kan ürünleri ile tedavi edilen 63'ü hemofili-A (1-20 yaş, ortanca 11 yaş), 17'si hemofili-B (3-20 yaş, ortanca 10 yaş), biri faktör V eksikliği olan 81 çocuk üç yıl süreyle inhibitor gelişimi yönünden izleme alındı. Kırk hemofili-A hastası faktör konsantresi (26'sı ağır), 23 hastada sadece TDP (11'i ağır) kullanıyordu, inhibitor testleri Bethesda yöntemi ile uygulandı. Hiçbir hemofili-B'li hastada inhibitor oluşmazken, 63 hemofili-A hastasından sekizinde (% 13) inhibitor saptandı. Bunların tamamı konsantre kullanılan (8/26; %31), ağır tip (8/37; %22) hastalardı. TDP kullanan ağır hemofilik vakalarda inhibitor gelişimi saptanmadı (0/11) (p=0.07). inhibitor 8-125 kullanım günü sonrası (ortanca 54 gün) saptandı. Ara saflıkta ve pastörize konsantre kullananlarda daha sık inhibitor gelişimi izlendi. Proflaksi grubundaki dört inhibitörlü hastanın (1, 2, 3 ve 5 Bethesda ünitesi-BÜ) tamamında 1-4 ay içinde tedavi kesilmeksizin (haftada 2 kez, 25 iÜ/kg dozda) inhibitor spontan olarak kayboldu. Yüksek titrajlı iki hastada (56 ve 11 BU) aynı protokolle immün tolerans tedavisi uygulandı. Kanama epizotlarının tedavisinde kullanılan protrombin kompleksi ile 10 kanama atağının altısında (%60) hemostaz sağlandı.
Inhibitor prevalence in developing countries such as Turkey, where fresh frozen plasma (FFP) is still in use due to the high cost of concentratres, is unknown, To determine the frequency of inhibitors in Turkish hemophiliacs exposed to blood products, 63 hemophilia A patients (age range 1-20; median 11 yrs) and 17 hemophilia B patients (age range 3-20; median 10 yrs), and one patient with factor V deficiency were evaluated. Forty hemophilia A patients (26 severe) received plasma-derived concentrates and 23 patients (11 severe) only FFP. No hemophilia B patients developed inhibitors, compared to eight of 63 (13%) hemophilia A patients, all with a severe defect (8/37,22%) and treated with concentrates (8/26,31%). On the other hand, severe patients treated with FFP showed a lower risk to develop inhibitors (0/11, p=0.07). Inhibitors were detected after eight to 125 exposure days (median: 54). For eight patients with inhibitor, intermediate purity and pasteurization seemed to be linked with a higher risk of inhibitor compared to high purity and solvent-detergent inactivation. In four of eight inhibitor patients, low-dose concentrate was administered at 25 ID/kg twice weekly and inhibitors (1, 2, 3, 5 Bethesda unit-BU) disappeared in one to four months in the prophylaxis group. A low-dose immune tolerance regimen might be recommended for immune tolerance in developing countries because of its lower cost. In two high inhibitor (56 and 11 BU) patients in the episodic treatment group, an immune tolerance regimen was used. Hemostasis was obtained using prothrombin complex concentrate in 60 percent of bleeding episodes in patients with inhibitor.
Inhibitor prevalence in developing countries such as Turkey, where fresh frozen plasma (FFP) is still in use due to the high cost of concentratres, is unknown, To determine the frequency of inhibitors in Turkish hemophiliacs exposed to blood products, 63 hemophilia A patients (age range 1-20; median 11 yrs) and 17 hemophilia B patients (age range 3-20; median 10 yrs), and one patient with factor V deficiency were evaluated. Forty hemophilia A patients (26 severe) received plasma-derived concentrates and 23 patients (11 severe) only FFP. No hemophilia B patients developed inhibitors, compared to eight of 63 (13%) hemophilia A patients, all with a severe defect (8/37,22%) and treated with concentrates (8/26,31%). On the other hand, severe patients treated with FFP showed a lower risk to develop inhibitors (0/11, p=0.07). Inhibitors were detected after eight to 125 exposure days (median: 54). For eight patients with inhibitor, intermediate purity and pasteurization seemed to be linked with a higher risk of inhibitor compared to high purity and solvent-detergent inactivation. In four of eight inhibitor patients, low-dose concentrate was administered at 25 ID/kg twice weekly and inhibitors (1, 2, 3, 5 Bethesda unit-BU) disappeared in one to four months in the prophylaxis group. A low-dose immune tolerance regimen might be recommended for immune tolerance in developing countries because of its lower cost. In two high inhibitor (56 and 11 BU) patients in the episodic treatment group, an immune tolerance regimen was used. Hemostasis was obtained using prothrombin complex concentrate in 60 percent of bleeding episodes in patients with inhibitor.
Açıklama
Anahtar Kelimeler
Genel ve Dahili Tıp, Pediatri
Kaynak
Çocuk Sağlığı ve Hastalıkları Dergisi
WoS Q Değeri
Scopus Q Değeri
Cilt
41
Sayı
3
