Recombinant activated factor VII administration in a patient with congenital lack of factor VII undergoing laparoscopic hysterectomy: A case report.
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ABSTRACT: Introduction and importance
Case report of patient with congenital lack of factor VII, suffering from recurrent hematomas and massive menstrual bleedings resulting in severe anemia and multiple hospitalization.Case presentation
Patient was diagnosed with endometrial hyperplasia and not responding to hormonal treatment and substitution with recombinant factor VII was not effective to reduce the bleedings. This case describes successful laparoscopic technique of using bipolar coagulation and non-absorbable clips.Clinical discussion
We describe premedication and post-surgical management - which we had to modify from this found in very scarce literature. Despite previous vaginal deliveries without any complications during the puerperium, 20 days after the surgery patient presented with intraperitoneal bleeding after stopping rFVIIa therapy. It was treated medically without the need for re-laparoscopy.Conclusion
Laparoscopic surgery is possible in patients with lack and deficiency of FVIIa, but they need close post-operative surveillance and prolonged supplementation with recombinant FVIIa.
SUBMITTER: Dabrowski FA
PROVIDER: S-EPMC7804344 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:BACKGROUND: Diffuse alveolar haemorrhage (DAH) is a serious pulmonary complication characterised by a high mortality rate and the absence of specific treatment. The intrapulmonary administration of activated recombinant factor VII (rFVIIa) in DAH was recently published in six patients by Heslet et al with an efficient hemostatic effect. We describe two cases of DAH treated with intrapulmonary rFVIIa. METHODS: Two cases of DAH were admitted to the ICU after presenting abrupt desaturation, tachypnea, cough and haemoptysis, requiring orotracheal intubation and mechanical ventilation. The diagnosis was achieved by the bloody return during the bronchoalveolar lavage, during the procedure rFVIIa (50 mug/Kg in 50 ml of isotonic saline) was administered via the bronchoscope. RESULTS: Immediate cessation of bleeding was observed. Prior to intrapulmonary administration of rFVIIa, the FiO2 was 1, which was reduced to 0.4 24 hours later. Following the procedure, the haemostatic effect made blood transfusion superfluous. No thrombotic complications associated with administration of the drug were observed. After the intervention both cases progressed fast and was discharged from the ICU with no further episodes of bleeding. CONCLUSION: 1. Local intrabronchial deposition of DAH with rFVIIa has been shown to be effective in controlling life-threatening DAH. 2. In the case described above, no thrombotic complications were observed following the intrapulmonary administration of rFVIIa.
| S-EPMC2551590 | biostudies-other
Project description:The off-label use of recombinant activated factor VII (rFVIIa) for intractable bleeding is associated with a risk of thrombotic events. The objective of this study was to evaluate the incidence and predictors of rFVIIa-related thrombotic events and its efficacy in the reduction of transfusion requirements during various surgeries.Ninety-two cases received rFVIIa for uncontrollable bleeding despite medical and surgical hemostasis. The incidence and risk factors of thrombotic events were analyzed. Blood products transfused in the 24 h before and after rFVIIa injection were calculated. Subgroup analysis was performed to see which types of surgeries benefited most from rFVIIa.The main indication for rFVIIa administration was uncontrollable bleeding during cardiothoracic surgery followed by coagulopathy due to liver failure followed by neurosurgical procedures. Requirements of blood products after rFVIIa decreased significantly by 45 % (p = 0.012), 52 % (p = 0.0001), and 75 % (p = 0.0001) for red blood cells, plasma, and cryoprecipitate, respectively. Subgroup analysis showed that cardiothoracic surgery was the sole group that benefited from rFVIIa with a reduction in transfusion of red blood cells (p = 0.013), plasma (p = 0.0001), and cryoprecipitate (p = 0.0001). Thrombotic events occurred in 9.8 % of the cases mostly on the arterial side (89 %) and have not contributed to mortality.rFVIIa can significantly reduce transfusion requirements when given for intractable bleeding during cardiothoracic surgery at the expense of thrombotic events in approximately one tenth of the cases. Further prospective studies are necessary to study if this effect of rFVIIa is translated to a favorable outcome.
| S-EPMC4488183 | biostudies-literature
Project description:Recombinant activated factor VII (rFVIIa) has been used as adjunctive therapy in trauma patients with severe bleeding. However, its pharmacokinetics profile remains unknown.In two placebo-controlled studies in patients with blunt and penetrating trauma, the pharmacokinetics of rFVIIa given at an initial dose of 200 microg x kg-1 after transfusion of eight red blood cell units, followed by additional doses of 100 microg x kg-1, one and three hours later, have been studied, based on the FVII coagulant activity assay. Both non-compartment and population pharmacokinetic analyses were performed. A two-compartment, population pharmacokinetic model was used to estimate a population profile for the rFVIIa dosing regimen. Data are population means (percent coefficient of variation (CV)).Based on the two-compartment population model, the estimated pharmacokinetic parameters were: clearance 40 (30% CV) ml x kg-1 x h-1; central volume of distribution 89 (32% CV) ml x kg-1; inter-compartmental clearance 24 ml x kg-1 x h-1; and peripheral compartment volume 31 ml x kg-1. Baseline FVII coagulant activity was estimated at 0.29 (39% CV) U x ml-1, initial half-life was 0.6 (34% CV) hours, and terminal half-life 2.4 (50% CV) hours. High intra- and inter-patient variability was noted in volume of distribution and clearance, which was in part correlated with the transfusion requirements as the single significant covariate. The non-compartmental analysis led to almost identical estimates of key parameters.A high intra- and inter-patient variability was noted in the volume of distribution and clearance of rFVIIa in trauma patients with severe bleeding, mainly related with the transfusion requirements and thus blood loss and/or bleeding rate.
| S-EPMC1750999 | biostudies-other
Project description:Background and aimIntraoperative blood loss is a frequent complication of hepatic resection and orthotopic liver transplantation. Recombinant activated coagulation factor VII (rFVIIa) is a coagulation protein that induces hemostasis by directly activating factor X. There is no clear information about the prophylactic value of rFVIIa in hepatobiliary surgery, specifically in liver resection and orthotopic liver transplantation. The aim of this study was to assess the effect of rFVIIa prophylaxis to prevent mortality and bleeding resulting from hepatobiliary surgery.MethodsRelevant randomized trials were identified by searching The Cochrane Central Register of Controlled Trials in The Cochrane Library, MEDLINE, EMBASE, and Science Citation Index. Randomized clinical trials comparing different rFVIIa prophylactic schemas against placebo or no intervention to prevent bleeding in hepatobiliary surgery were included. Adults undergoing liver resection, partial hepatectomy, or orthotopic liver transplantation were included. Dichotomous data were analyzed calculating odds ratios (ORs) and 95% confidence intervals (CIs). Continuous data were analyzed calculating mean differences (MD) and 95% CIs.ResultsFour randomized controlled trials were included. There were no significant differences between rFVIIa and placebo for mortality (OR 0.96; 95% CI 0.35-2.62), red blood cell units (MD 0.32; 95% CI -0.08-0.72) or adverse events (OR 1.55; 95% CI 0.97-2.49).ConclusionsThe available information is limited, precluding the ability to draw conclusions regarding bleeding prophylaxis in hepatobiliary surgery using rFVIIa. Although an apparent lack of effect was observed in all outcomes studied, further research is needed.
| S-EPMC3144913 | biostudies-literature
Project description:Unexpected malignant tumors are a rare finding after surgery for symptomatic leiomyomas but there is little doubt that morcellation of these lesions is associated with a higher risk of iatrogenic peritoneal spread compared to women having surgery without morcellation. Thus, the FDA has issued a warning against the use of power morcellation in the majority of women undergoing myomectomy or hysterectomy for treatment of fibroids.We pressent a case report of 50year old patient with intervertebral disc degeneration and multiple uterine fibroids decided to have laparoscopic supracervical hysterectomy. 28 months later the patient cystic adnexal massas removed by laparotomy and histologically, classified as leiomyosarcoma. 19 months after this latter surgery MRI revealed a 10 x 8 x 6 cm abdominal mass attached to the liver and numerous other nodules attached to the abdominal wall. We investigated genomic and expression profiles of four samples obtained from initial surgery and the malignant tumor that appeared 16 month later by molecular inversion probe (MIP) and transcriptome array hybridization. DNA was extracted from FFPE Tissue Samples using Covaris adaptive focused acoutics (AFATM) and truXTRACTM FFPE DNA kit and subjected to array analysis using the Affymetrix OncoScan and human Clariom D platforms according to the manufacturers protocol.
2018-04-30 | GSE103050 | GEO
Project description:BACKGROUND:Hemophagocytic lymfohistiocytosis (HLH) is a rare, life-threatening hyperinflammation, characterized by immune system over-activation resulting in hemophagocytosis. HLH could appear as a primary disease caused by mutations of immune-regulatory genes, or develop as a result of viral or bacterial infections, or malignancy. Congenital factor VII (FVII) deficiency is a rare autosomal recessive disorder characterized by prolonged prothrombin time (PT) and low FVII, which may increase bleeding risk. CASE PRESENTATION:A 50-year-old woman was admitted for a fever persisted for 20 days, presenting with cytopenia, high hyperferritinemia, low activity of NK cells. Bone marrow aspiration showed hemophagocytosis. CT scanning found pulmonary infection. EBV and CMV were not detected. Genetic scanning did not find pathogenic mutation of a HLH NGS panel including 26 genes. This patient was treated as recommended by the HLH 2004 Guidelines. Coagulation tests identified FVII deficiency. Genetic analysis of F7 gene in the patient and her family members identified recurrent compound heterozygous F7 c.64?+?5G?>?A and c.1224 T?>?G (p.His408Gln) mutations in this patient and her brother who showed postoperative hemorrhage after surgical resection of renal cell carcinoma. Heterozygotes in this family were asymptomatic. CONCLUSIONS:To our knowledge, this is the first report of HLH in combination with congenital FVII deficiency in Chinese population.
| S-EPMC6134588 | biostudies-literature
Project description:Background and objectiveFactor VII (FVII) deficiency is probably one of the most common of the rare autosomal recessive coagulation disorders, with an estimated prevalence of l: 500000. All age groups can be affected with FVII deficiency. This study aimed to describe the demographic parameters, symptomatology, hemostatic values and the outcome of FVII deficiency.MethodsThis is a retrospective descriptive study of patients with congenital FVII deficiency over a period of seven years from (August 2008 to August 2015). The data were collected by reviewing the files for each patient diagnosed with FVII deficiency. Surgical interventions, complications and follow up visits were recorded.ResultsTwenty-four patients were included in this study, 17 females and seven males, below one year was the most common age at presentation. More than half of patients (58.3%) were diagnosed within six months of symptoms onset. The majority of patients had severe phenotype. The most common symptom was epitaxis (41.7%). Five out of 10 patients with FVII level < 1% have either mild to moderate phenotype of the disease without complications; while six out of 14 patients with FVII > 1% had at least one episode of severe bleeding. Three patients had hepatitis C; all were treated by blood products before the introduction of recombinant FVII in Iraq. The outcome of most patients (75%) was normal without complications at time of study.ConclusionClinical manifestations of FVII deficiency are variable and they are not necessarily correlated to the FVII level.
| S-EPMC6994885 | biostudies-literature
Project description:IntroductionOur aim was to develop consensus guidelines for use of recombinant activated factor VII (rFVIIa) in massive hemorrhage.MethodsA guidelines committee derived the recommendations using clinical trial and case series data identified through searches of available databases. Guidelines were graded on a scale of A-E according to the strength of evidence available. Consensus was sought among the committee for each recommendation.ResultsA recommendation for use of rFVIIa in blunt trauma was made (grade B). rFVIIa might also be beneficial in post-partum hemorrhage (grade E), uncontrolled bleeding in surgical patients (grade E) and bleeding following cardiac surgery (grade D). rFVIIa could not be recommended for use: in penetrating trauma (grade B); prophylactically in elective surgery (grade A) or liver surgery (grade B); or in bleeding episodes in patients with Child-Pugh A cirrhosis (grade B). Efficacy of rFVIIa was considered uncertain in bleeding episodes in patients with Child-Pugh B and C cirrhosis (grade C). Monitoring of rFVIIa efficacy should be performed visually and by assessment of transfusion requirements (grade E), while thromboembolic adverse events are a cause for concern. rFVIIa should not be administered to patients considered unsalvageable by the treating medical team.ConclusionsThere is a rationale for using rFVIIa to treat massive bleeding in certain indications; however, only adjunctively to the surgical control of bleeding once conventional therapies have failed. Lack of data from randomized, controlled clinical trials, and possible publication bias of the case series data, limits the strength of the recommendations that can be made.
| S-EPMC1750973 | biostudies-literature
Project description:BackgroundThere are various surgical approaches of hysterectomy for benign indications. This study aimed to compare vaginal hysterectomy (VH) and laparoscopic hysterectomy (LH) with respect to their complications and operative outcomes.MethodsWe selected randomised controlled trials that compared VH with LH for benign gynaecological indications. We included studies published after January 2000 in the following databases: Medline, EMBASE, and CENTRAL (The Cochrane Library). The primary outcome was comparison of the complication rate. The secondary outcomes were comparisons of operating time, blood loss, intraoperative conversion, postoperative pain, length of hospital stay and duration of recuperation. We used Review Manager 5.3 software to perform the meta-analysis.ResultsEighteen studies of 1618 patients met the inclusion criteria. The meta-analysis showed no differences in overall complications, intraoperative conversion, postoperative pain on the day of surgery and at 48 h, length of hospital stay and recuperation time between VH and LH. VH was associated with a shorter operating time and lower postoperative pain at 24 h than LH.ConclusionsWhen both surgical approaches are feasible, VH should remain the surgery of choice for benign hysterectomy.
| S-EPMC6591934 | biostudies-literature
Project description:OBJECTIVE:To compare the effects of laparoscopic hysterectomy and abdominal hysterectomy in the abdominal trial, and laparoscopic hysterectomy and vaginal hysterectomy in the vaginal trial. DESIGN:Two parallel, multicentre, randomised trials. SETTING:28 UK centres and two South African centres. PARTICIPANTS:1380 women were recruited; 1346 had surgery; 937 were followed up at one year. Primary outcome Rate of major complications. RESULTS:In the abdominal trial laparoscopic hysterectomy was associated with a higher rate of major complications than abdominal hysterectomy (11.1% v 6.2%, P = 0.02; difference 4.9%, 95% confidence interval 0.9% to 9.1%) and the number needed to treat to harm was 20. Laparoscopic hysterectomy also took longer to perform (84 minutes v 50 minutes) but was less painful (visual analogue scale 3.51 v 3.88, P = 0.01) and resulted in a shorter stay in hospital after the operation (3 days v 4 days). Six weeks after the operation, laparoscopic hysterectomy was associated with less pain and better quality of life than abdominal hysterectomy (SF-12, body image scale, and sexual activity questionnaires). In the vaginal trial we found no evidence of a difference in major complication rates between laparoscopic hysterectomy and vaginal hysterectomy (9.8% v 9.5%, P = 0.92; difference 0.3%, -5.2% to 5.8%), and the number needed to treat to harm was 333. We found no evidence of other differences between laparoscopic hysterectomy and vaginal hysterectomy except that laparoscopic hysterectomy took longer to perform (72 minutes v 39 minutes) and was associated with a higher rate of detecting unexpected pathology (16.4% v 4.8%, P = < 0.01). However, this trial was underpowered. CONCLUSIONS:Laparoscopic hysterectomy was associated with a significantly higher rate of major complications than abdominal hysterectomy. It also took longer to perform but was associated with less pain, quicker recovery, and better short term quality of life. The trial comparing vaginal hysterectomy with laparoscopic hysterectomy was underpowered and is inconclusive on the rate of major complications; however, vaginal hysterectomy took less time.
| S-EPMC314503 | biostudies-literature