Preface

The Nordic Hemophilia Council commissioned the work on Practical Nordic Guideline for diagnosis and management of FVII deficiency. A working group with representatives from six hemophilia treatment centers in four Nordic countries was formed. The work was partly based on the Ph.D. thesis by Hoa Tran, PhD, Oslo, Norway, and owing to the high prevalence of severe factor VII deficiency in Norway and the accumulated expertise, the work was supervised by professor Pål André Holme, Oslo, Norway. The final document was assembled and finalized by Eva Zetterberg, Malmö, Sweden and Ole Halfdan Larsen, Aarhus, Denmark. A hearing version was distributed to all relevant treatment centers in the Nordic countries prior to publication.

Literature searches were performed by the individual subgroups. However, factor VII deficiency is a rare bleeding disorder, with very limited data available. Hence, the quality of evidence supporting the recommendations are generally weak, and using a systematic rating system in this setting was not found relevant. However, when clinical decisions cannot be based on hard evidence, the present practical consensus guideline on what is considered state-of-the art treatment in the Nordic Countries may hopefully be a helpful tool tailoring treatment in factor VII deficiency patients.

In 2025 the original text was revised and updated by seven experts in bleeding disorders representing seven centers (see Home).