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Introduction: A paucity of data exists on the incidence, diagnosis and treatment of

Running title: FVII inhibitors in congenital FVII deficiency: the STER [55 characters; maximum 60 characters and spaces]2 SummaryCongenital factor VII (FVII) deficiency is a rare bleeding disorder, and few anecdotal reports are available regarding FVII inhibitors. Using data from the Seven Treatment Evaluation Registry (STER; 225 patients, 312 treatments), we have prospectively evaluated the occurrence of FVII inhibitors using a standardized method over a period of 8 years. The central laboratory screened 115 paired samples; FVII inhibitors were detected in 3/115 (2.6%) patients (one de novo inhibitor and two pre-existing inhibitors). A fourth inhibitor was detected in a patient who had been screened locally. All four patients had high-responding inhibitors (10-72 BU) and had previously received factor replacement therapy. In three patients, the inhibitors appeared before the age of 6 months during prophylaxis following central nervous system or gastrointestinal bleeds; however, treatments were continued without apparent loss of efficacy. In one patient, an inhibitor developed in adulthood after replacement therapy for minor surgery. No anaphylactoid reactions or renal complications were reported during or after prophylaxis. In conclusion, inhibitor development is rare in patients with congenital FVII deficiency (2%), with an incidence similar to that in patients with haemophilia B. FVII inhibitors display immunological (anamnesis) and kinetic features similar to those in patients with haemophilia. Summary word count: 198 words (maximum 200 words)

This is a non-controlled experimental prospective clinical study that evaluates the satisfactory results in the chemical synovectomy (synoviorthesis) with oxytetracycline clorhydrate (Emicine, Lab. Pfizer Ltda, Guarulhos, Sao Paulo, Brazil) in recurrence haemarthrosis in different joints, demonstrating that it is an effective method in the treatment of these recurrent haemarthrosis in haemophilia. 84 patients of whom 77 concluded the full course of treatment. 82 joints were injected. The dosage injected was 5 cm(3) of the drug (25 mg) in 5 cm(3) of anaesthesia for the knee, 2 cm(3) with 1 cm(3) of anaesthesia for the elbow and 1 cm(3) plus 1 cm(3) of anaesthesia for the ankle. These injections were administered once weekly with a reinforcement in 1 month. In case of failure the same can be administered repeatedly. Subjective parameters included pain, range of movement and use of the joint involved. Pain decreased from a mean of 6.5 to 0.9 (Likert scale). Range of movement increased from 5.9 to 9 and joint use increased from 5.9 to 9.2. Objective parameters included joint diameter and range of movement. Range of movement for flexion and extension improved from 72.2 and 149.2 to 73.7 and 167, respectively, for the knees. From 57.3 and 160 to 66.6 and 170, respectively, for the shoulder. And, from 22.7 and 10.8 to 34 and 18.6, respectively, for the ankle. This procedure has multiple advantages such as immediate therapeutic effect, short period of treatment, easy technique, much less AHF coverage (30% above coagulation level), less costly than radiocolloid treatment, which make it a perfect alternative treatment for developing countries.