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

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 Deficiency ; genetics ; Humans

Child ; Humans ; Factor VII Deficiency

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

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

Factor VII deficiency is a rare congenital bleeding disorder characterized by episodes of spontaneous bleeding in severely affected individuals. It is rare intussusception due to submucosal hematoma in coagulation factor deficiency patient. We recently experienced an adult small bowel intussusception in a patient with factor VII deficiency. A 36-yr old female patient with coagulation factor VII deficiency who was referred to our hospital underwent emergency surgery for treatment of the small bowel intussusceptions. Emergency laparoscopy-assisted small bowel resection was performed for treatment of small bowel intussusception caused by submucosal hematoma. The patient was successfully treated with close laboratory monitoring and laparoscopy-assisted small bowel resection.
Adult ; Blood Coagulation Factors ; Emergencies ; Factor VII ; Factor VII Deficiency ; Female ; Hematoma ; Hemorrhage ; Humans ; Intussusception

Congenital factor VII deficiency is a rare autosomal-recessive bleeding disorder. Bleeding manifestations and clinical findings vary widely, ranging from asymptomatic subjects to patients with hemorrhages that may cause significant handicaps. Treatment has traditionally involved factor VII(FVII) replacement therapy using fresh frozen plasma, prothrombin complex concentrates or plasma-derived FVII concentrates. Recombinant activated FVII (NovoSeven(R)) is currently considered the first-line treatment for replacement therapy of FVII deficiency. Here we present a case of severe intracerebral and intraventricular hemorrhage in a neonate with congenital FVII deficiency.
Blood Coagulation Factors ; Factor VII ; Factor VII Deficiency ; Hemorrhage ; Humans ; Infant, Newborn ; Intracranial Hemorrhages ; Plasma ; Prothrombin

OBJECTIVETo investigate the genotypes of mutations of an inherited coagulation factor VII(F VII) deficiency pedigree.

METHODSThe diagnosis was validated by coagulant parameters. F VII gene mutations were analysed in the proband and her family members by DNA direct sequencing. The PCR fragments were cleaved by the Msp I restriction enzyme to confirm the mutations detected by sequencing was performed in this study.

RESULTSDouble heterozygous mutations at the same coding site of amino acid were detected in propositus of the pedigree: a C to T mutation at position 11348 resulting in Arg304Trp substitution combined with a G to A mutation at position 11349 resulting in Arg304Gln substitution. Her farther had a G to A mutation at position 11349 and her mother had a C to T mutation at position 11348, respectively. Both were heterozygous mutations. One of her brothers had normal genotype, the other brother and all her three offsprings had heterozygous mutations.

CONCLUSIONDouble heterozygous mutations coding the same amino acid were found in a pedigree with hereditary coagulation factor VII deficiency.

DNA Mutational Analysis ; Factor VII ; genetics ; Factor VII Deficiency ; genetics ; Female ; Heterozygote ; Humans ; Male ; Mutation ; Pedigree