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SHR Neuro Krebs Kardio Lipid Stoffw Microb

Gouw, SC; van den Berg, HM; Fischer, K; Auerswald, G; Carcao, M; Chalmers, E; Chambost, H; Kurnik, K; Liesner, R; Petrini, P; Platokouki, H; Altisent, C; Oldenburg, J; Nolan, B; Garrido, RP; Mancuso, ME; Rafowicz, A; Williams, M; Clausen, N; Middelburg, RA; Ljung, R; van der Bom, JG; PedNet and Research of Determinants of INhibitor development (RODIN) Study Group.
Intensity of factor VIII treatment and inhibitor development in children with severe hemophilia A: the RODIN study.
Blood. 2013; 121(20): 4046-4055. Doi: 10.1182/blood-2012-09-457036 [OPEN ACCESS]
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Abstract:
The objective of this study was to examine the association of the intensity of treatment, ranging from high-dose intensive factor VIII (FVIII) treatment to prophylactic treatment, with the inhibitor incidence among previously untreated patients with severe hemophilia A. This cohort study aimed to include consecutive patients with a FVIII activity < 0.01 IU/mL, born between 2000 and 2010, and observed during their first 75 FVIII exposure days. Intensive FVIII treatment of hemorrhages or surgery at the start of treatment was associated with an increased inhibitor risk (adjusted hazard ratio [aHR], 2.0; 95% confidence interval [CI], 1.3-3.0). High-dose FVIII treatment was associated with a higher inhibitor risk than low-dose FVIII treatment (aHR, 2.3; 95% CI, 1.0-4.8). Prophylaxis was only associated with a decreased overall inhibitor incidence after 20 exposure days of FVIII. The association with prophylaxis was more pronounced in patients with low-risk F8 genotypes than in patients with high-risk F8 genotypes (aHR, 0.61, 95% CI, 0.19-2.0 and aHR, 0.85, 95% CI, 0.51-1.4, respectively). In conclusion, our findings suggest that in previously untreated patients with severe hemophilia A, high-dosed intensive FVIII treatment increases inhibitor risk and prophylactic FVIII treatment decreases inhibitor risk, especially in patients with low-risk F8 mutations.
Find related publications in this database (using NLM MeSH Indexing)
Adolescent -
Adult -
Blood Coagulation Factor Inhibitors - blood
Chemoprevention - adverse effects
Child -
Cohort Studies -
Dose-Response Relationship, Drug -
Factor VIII - administration & dosage
Hemophilia A - blood
Hemorrhage - blood
Humans -
Risk Factors -
Severity of Illness Index -
Young Adult -

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