PURPOSE: Congenital factor VII(FVII) deficiency is a rare bleeding disorder and surgery can cause excessive bleeding due to an extrinsic pathway problem. It can be diagnosed by increased PT and decreased FVII level in coagulation test. Symptom varies according to the level of FVII, but it is essential to prevent intraoperative excessive bleeding. METHODS: In this report, we described the orthognatic surgery experience in a mandibular prognathism patient with congenital F'VII deficiency, in which recombinant activated factor VII(rFVIIa) was used to manage the bleeding. Rsults: We could get a successful result without any complication and there was minimal intraoperative bleeding. CONCLUSION: The orthognathic surgery could therefore be safely performed in patients with congenital factor VII deficiency using rFVIIa.
Factor VII ; Factor VII Deficiency ; Factor VIIa ; Hemorrhage ; Humans ; Orthognathic Surgery ; Prognathism ; Recombinant Proteins

No abstract available.
Factor VII Deficiency* ; Factor VII*

Recombinant activated factor VII (rFVIIa, NovoSeven (R)) was initially developed for the treatment of bleeding in patients with hemophilia having antibodies against factor VIII or IX, and factor VII deficiency. Although the precise mode of action is still elusive and there are just several hypotheses, recently case reports have suggested a role of rFVIIa in the management of intractable or life-threatening bleeding in some non-hemophilic patients who do not respond to conventional treatments. We report the successful use of rFVIIa in a pediatric patient with intractable gastrointestinal bleeding.
Antibodies ; Factor VII Deficiency ; Factor VIIa* ; Factor VIII ; Gastrointestinal Hemorrhage* ; Hemophilia A ; Hemorrhage ; Humans

This 29 years old woman was admitted to the hospital with menstrual and dental bleeding. Physical examination revealed a pale brust on the legs. Laboratory data. - Hemograme: Hb: 7.5 g/dl, Hct: 22.7%, RBC: 2.86 x 1012/ l, WBC: 57.109/l hemostatic: PT: 23“7 (10”9), INR 5.86, aPTT: 32” (38”), PT¬mix 11”2 (10”9). Dosage of factor VII: 2.7%. Dosage of factor X: 102%. Diagnosis: congenital factor VII deficiency. Treatment: frozen plasma (15 ml/kg/day x 3 days) and provera 10 mg/dayx 4 days.
Congenital ; Factor VII Deficiency

Study on 69 patients were dead within 41 months following a partly or total gastroectomy due to the gastric glandular epithelioma in Viet Duc Hospital during January 1995 - June 1997 has shown that male/female (2/1), most of patient were lately admitted in which the abdominal tumor: 46.4%, pyloric stenosis: 20.3%, tumor perforation: 4.3% metastasis: 85.5%, diseases in phase III and phase IV: 85%. Nodal curettage and blood protein level is 2 factors that influence the survival time after operation.
Congenital ; Factor VII Deficiency

Factor VII ; Factor VII Deficiency ; Genotype ; Humans ; Pedigree

Factor VII Deficiency ; genetics ; Humans

Child ; Humans ; Factor VII Deficiency

Congenital factor VII deficiency is a rare hemorrhagic disorder, and invasive procedures are likely to cause excessive bleeding in these patients. Endoscopic submucosal dissection (ESD) has been accepted as a curative treatment modality for gastric adenoma, early gastric cancer (EGC) and any other mucosal and submucosal tumors. The most important complications of ESD are bleeding and perforation. The use of antiplatelet agents or coagulopathies are risk factors for these complications. There are only few reports of successful ESD with coagulation disorders. We report a case of a 70-year-old female patient who was diagnosed with a gastric adenoma and factor VII deficiency. The patient was successfully treated with ESD. Before ESD, recombinant Coagulation factor VIIa was injected, and the procedure was performed successfully without any complications. In conclusion, ESD can be performed successfully in patients with factor VII deficiency, when recombinant human factor VIIa is administered properly.
Adenoma* ; Aged ; Endoscopy ; Factor VII Deficiency* ; Factor VIIa ; Female ; Hemorrhage ; Hemorrhagic Disorders ; Humans ; Platelet Aggregation Inhibitors ; Risk Factors ; Stomach Neoplasms

BACKGROUND: Congenital factor VII (FVII) deficiency is a rare hemorrhagic disorder that can cause excessive bleeding during and after surgery in affected patients. The recombinant form of activated factor VII (rFVIIa, NovoSeven(R) from Novo Nordisk, Bagsvaerd, Denmark), which was developed as a second-generation bypassing agent, has recently been used in the management of bleeding for patients with congenital FVII deficiency. METHODS: We reviewed the results of 8 surgical procedures in 5 patients with congenital FVII deficiency at the Kyung Hee University Hospital, Gangdong, Seoul, Korea, between January 2008 and June 2010. We administrated rFVIIa preoperatively in six patients and postoperatively in five patients. RESULTS: Between January 2008 and June 2010 at our center, 8 operations were performed successfully and no complications were observed in the 5 patients with congenital FVII deficiency. The median level of FVII activity was 2% (range, 0.6-7%). Four orthopedic procedures, 1 tonsillectomy, and 3 dental extractions were performed. The median duration of hospitalization was 8.5 days (range, 0-15 days). rFVIIa was administered at all procedures, except the dental extraction that was performed using only antifibrinolytic agents without any replacement. No bleeding or thrombogenic complications were observed in any case. CONCLUSION: Patients with congenital FVII deficiency who require surgery can be treated efficiently and safely with rFVIIa or antifibrinolytic agents. rFVIIa was well tolerated and maintained effective hemostasis and showed good clinical outcome after the major surgery.
Antifibrinolytic Agents ; Factor VII ; Factor VIIa ; Hemorrhage ; Hemorrhagic Disorders ; Hemostasis ; Hospitalization ; Humans ; Korea ; Orthopedic Procedures ; Recombinant Proteins ; Tonsillectomy