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Diagnosis and treatment of a patient with gallstone ileus.

BACKGROUND: A 79-year-old white woman presented with upper abdominal pain. She had a history of rheumatoid arthritis since she was 19 years old, which was treated with prednisolone, leflunomide, diclofenac and pantoprazole. She also had factor VII deficiency. The patient had been hospitalized 2 months previously with sepsis presumed to be due to urinary infection, and was treated with antibiotics. Sonography at this time revealed a gallbladder with a monstrous thick wall and stones, and the first differential diagnosis was cholecystitis. Cholecystectomy was planned after amelioration of the patient's general state, but her general state worsened. INVESTIGATIONS: Sonography, endoscopy of the upper and lower intestine, and CT scan. DIAGNOSIS: Biliodigestive fistula and gallstone ileus. MANAGEMENT: Enterolithotomy, stenting, endoscopic retrograde cholangiopancreatography, and surgery.

Aged↗

Experience with use of recombinant activated factor VII.

Recombinant activated factor VII (rFVIIa) has recently been introduced for improving haemostasis in haemophiliac patients developing alloantibodies (inhibitors) to factor VIII (FVIII) or factor IX (FIX). We describe the successful management of haemorrhagia with rFVIIa in five different situations: an episode of surgical bleeding in a patient with haemophilia A and non-surgical haemorrhages in four patients with haemophilia A, acquired haemophilia, congenital severe FV defect and pseudo-acquired haemophilia, respectively. In each case, rFVIIa was effective and safe. There is no doubt that rFVIIa is useful in the therapeutic management of patients with antibodies to FVIII or FIX. However, the treatment is expensive and a cost-effectiveness analysis would be useful.

Adult↗

Genotype associations of factor VII gene with plasma factor VII coagulant activity and antigen levels in healthy Chinese.

A raised plasma factor VII (FVII) level is one of the risk factors for coronary artery disease. The R353Q polymorphism at codon 353 and the 10 base pair (bp) insertion (0/10 bp) polymorphism of the FVII gene have been reported to be associated with plasma FVII levels in several populations. We investigated these two polymorphisms in 209 male and 214 female healthy Chinese. The allele frequencies of 10 bp and Q were 0.036 and 0.045, respectively. Strong linkage disequilibrium was observed between these two sites (Delta = 0.85, P < 0.001). There were significant genotype associations of these two loci with FVII coagulant activity (FVIIc) and antigen (FVIIAg) levels. Heterozygous individuals had lower FVIIc and FVIIAg levels than those homozygous for the common alleles. When analyzed separately by gender, the 0/10 bp polymorphism was strongly associated with FVIIAg levels in males and females. However, both polymorphisms were significantly associated with FVIIc levels only in the females. The effect of 0/10 bp polymorphism predominated over that of the R353Q polymorphism in a two-way analysis of variance procedure. In the Chinese, the 10 bp insertion may reduce transcription of the FVII gene, leading to the decreased synthesis of FVII protein and thus FVIIc.

Adult↗

Procoagulant activity in renal transplant recipients.

Procoagulant activity (PCA) of leukocytes of renal transplant recipients was studied. This material, which activates coagulation, has previously been shown to be released from macrophages after they interact with mitogen-stimulated or antigen-stimulated T lymphocytes. Under endotoxin-free conditions, PCA of peripheral blood leukocytes, incubated for 90 min in tissue culture, was elevated in postoperative transplant recipients and in many transplant patients tested around the time of a rejection episode. The response to lipopolysaccharide added during culture was also increased in these populations. The PCA response was factor-VII-dependent when tested with washed peripheral blood mononuclear cells (PBMC), but was factor-VII-independent when tested with unwashed PBMC in their original culture medium. The results indicate a possible link between immunologic events and coagulation in transplant recipients.

Blood Coagulation Factors↗

Influence of warfarin therapy on activated factor VII clotting activity.

Oral anticoagulant therapy has been widely employed to prevent and treat a variety of thrombotic disorders, although a new generation of antithrombotic drugs, which offer inhibition of clot-bound as well as fluid-phase thrombin, has been developed and tested in several clinical trials. Although most anticoagulant responses to hydroxycoumarin compounds are well established, there are controversial evidences on their influence on activated factor VII (FVIIa). After analyzing the prothrombin time (PT) (International Normalized Ratio reference range, 0.92-1.08), factor VII clotting activity (FVII:C) (reference range, 75-130 U/dl) and activated factor VII clotting activity (FVIIa:C) (reference range, 30-110 U/l) in 46 consecutive patients on stable warfarin therapy for atrial fibrillation, a consistent trend towards decreased values of both FVII:C and FVIIa:C was observed as PT values increased. At moderate-intensity anticoagulation, with international normalized ratios between 2 and 3, the mean activities of FVII:C and FVIIa:C dropped to 28 U/dl (range, 9-61 U/dl) and 5.8 U/l (range, 1-18 U/l), respectively. Results of our investigations indicate that warfarin therapy decreases FVIIa:C, highlighting the potential benefits of oral anticoagulants in thrombotic disorders and other clinical conditions characterized by increased levels of FVIIa. Owing to the good correlation with FVIIa:C, we also hypothesize that the PT and/or FVII:C might be employed for monitoring recombinant FVIIa therapy.

Adult↗

Recombinant factor VIIa in paediatric bleeding disorders--a 2006 review.

Recombinant factor VIIa (rFVIIa) has been used in haemophilia bleeding since its introduction in 1996. It has been found to be safe and effective in the majority of patients with haemophilia who have developed inhibitors. There is increasing use of rFVIIa in many off-label bleeding conditions, but there is a paucity of randomized studies regarding the use of rFVIIa in children. This review will attempt to address and summarize the studies focusing on the role of rFVIIa in both haemophilia and non-haemophilia bleeding conditions in children. rFVIIa has been administered as both bolus and continuous infusions, and at varying doses. Furthermore, adverse events have not reportedly increased in children despite growing experience with its use in the paediatric population.

Blood Coagulation Factors↗