Gaucher sunum 9 kasım
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|www.genzyme.com
Erik Familial Hypercholesterolemia USA
Dr. Ertan Çam
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Femurdaki Patolojik Değişikliklerin Örnekleri
Hemorrajik İnfarktlar
Nekroz
Kortikal Skleroz
Osteoskleroz
Kortikal kemik kaybı
Erlenmeyer flask Deformitesi
Clinical Involvement
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5 Hematologtan sadece 1’i Gaucher hastalığının 6 ortak işaret ve semptomları bulunan hastada Gaucher hastalığından şüphelenmiştir.
Leukaemia Lymphoma MultiplMyelom
Gaucher Hastalığı
Kronik granülosit lösemi
Kanama hastalığı
Cevap yok
Hek
imle
rin o
ranı
0%
10%
20%
30%
40%
50%
60%
70%
80%
90%
100%
Market Research: Gaucher's disease Awareness Amongst Hematologists, August 2005, Holden Pearmain, Ltd., N=406 : US – 136, Canada – 50, Brazil – 50, Argentina – 50, Spain – 50, Japan – 50, Australia – 20
Gaucher Hastalığı TeşhisiAyrıcı Tanı
Hekimlerin; 42 yaşında erkek hastada anemi, trombositopeni, hepatomegali, splenomegali, akut kemik ağrısı ve kronik kemik ağrısı bulguları sonucu tanıları
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Bacterial DNA
Plasmid
Culture Medium
Bacterium
Protein
CHO Host Cell
Rekombinant Glikoserobrosidaz’ın Üretimi: Cerezyme
Human glucocerebrosidase
gene
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40Ver 1 Jan 2010
8,1
16,6
37,3
05
10152025303540
>2 yıl
AVN
İnsi
dans
Ora
nı(H
er 1
,000
kiş
i-yıl)
Avasküler nekrozun , tanı ve Cerezyme’ a başlamaya göre görülme oranı
<2 yılTanı ve Cerezyme tedavisi arasındaki
zaman aralığı
Cerezyme ile erken başlama ve Avasküler Nekroz insidansı
Cerezyme öncesi
Mistry PK et al., Br J Haematol 2009; 147: 561-570. Klinik Veriler
AVN gelişme riski, tanı sonrası Cerezyme tedavisine ilk 2 yıl içerisinde başlayan hastalarda , tanıdan 2 yıl sonra tedaviye başlayanlarla karşılaştırıldığında %41oranında
daha azdır.
-8.5
n=2700
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İletişim Bilgileri
Murat AktaşBölge Bilimsel Tanıtım Sorumlusu
(Clinical Science Associate) Gaucher & Mps-I
Genzyme0 533 580 72 77
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