METHODS: Patients chose to continue treatment with nonacog beta pegol in either one of two once-weekly prophylaxis arms (10IU/kg or 40IU/kg), or an on-demand arm (40IU/kg for mild/moderate bleeds; 80IU/kg for severe bleeds). The primary objective was to evaluate immunogenicity; key secondary objectives included assessing safety and haemostatic efficacy in the treatment and prevention of bleeds.
RESULTS: Seventy-one patients received prophylaxis or on-demand treatment. No patient developed an inhibitor and no safety concerns were identified. The success rate for the treatment of reported bleeds was 94.6%; most (87.9%) resolved with one injection. The median annualised bleeding rate for patients on prophylaxis was 1.36 (interquartile range [IQR] 0.00-2.23) and 1.00 (IQR 0.00-2.03) for the 10 and 40IU/kg treatment arms, respectively. The mean FIX activity trough achieved for 10 and 40IU once weekly was 9.8% and 21.3%, respectively. Fourteen patients on prophylaxis underwent 23 minor surgical procedures; haemostatic perioperative outcomes for all of those evaluated were 'excellent' or 'good'.
CONCLUSIONS: Nonacog beta pegol showed a favourable tolerability profile (with no safety issues identified) with good prophylactic protection and control of bleeding in previously treated adult and adolescent haemophilia B patients.
CASE PRESENTATION: We described a 43 years old male who presented with a right mastoid swelling, nine years after a right retro-sigmoid craniotomy and excision for a cerebellopontine angle meningioma. He also had multiple cranial neuropathies involving trigeminal, facial and vestibulocochlear nerves. Temporal bone CT and MRI showed features suggestive of cholesterol granuloma with extensive bony erosions. He was treated with surgical excision and drainage where bone wax residues were found intraoperatively. Histopathological analysis of the lesion confirmed the diagnosis of cholesterol granuloma. Post-operatively, the mastoid swelling resolved and his recovery was uneventful.
CONCLUSION: Our case showed that CG could manifest as a complication of bone wax usage in a neurosurgical procedure. Even though further study is needed to draw a definitive conclusion on this theory, we believe this paper will contribute to the current literature as it is the only reported case of cholesterol granuloma with bone wax as the possible causative agent. This is important so that surgeons are aware of this potential complication and use this haemostatic agent more judiciously.
SUMMARY: Background rVIII-SingleChain is a novel B-domain truncated recombinant factor VIII (rFVIII) comprised of covalently bonded FVIII heavy and light chains, demonstrating a high binding affinity to von Willebrand factor. Objectives This phase III study investigated the safety, efficacy and pharmacokinetics of rVIII-SingleChain in previously treated pediatric patients < 12 years of age with severe hemophilia A. Patients/Methods Patients could be assigned to prophylaxis or on-demand therapy by the investigator. For patients assigned to prophylaxis, the treatment regimen and dose were based on the bleeding phenotype. For patients receiving on-demand therapy, dosing was guided by World Federation of Hemophilia recommendations. The primary endpoint was treatment success, defined as a rating of 'excellent' or 'good' on the investigator's clinical assessment of hemostatic efficacy for all treated bleeding events. Results The study enrolled 84 patients (0 to < 6 years, n = 35; ≥ 6 to < 12 years, n = 49); 81 were assigned to prophylaxis and three to an on-demand regimen. Patients accumulated a total of 5239 exposure days (EDs), with 65 participants reaching > 50 EDs. In the 347 bleeds treated and evaluated by the investigator, hemostatic efficacy was rated as excellent or good in 96.3%. The median annualized spontaneous bleeding rate was 0.00 (Q1, Q3: 0.00, 2.20), and the median annualized bleeding rate was 3.69 (Q1, Q3: 0.00, 7.20) across all prophylaxis regimens. No participant developed an inhibitor. Conclusions rVIII-SingleChain is a novel rFVIII molecule showing excellent hemostatic efficacy and a favorable safety profile in a clinical study in children < 12 years of age with severe hemophilia A.
AIM: Our present paper has been written to disclose the statistical counts on the number of vWD cases reported from 2011 to 2013.
MATERIAL AND METHODS: This article is based on sociodemographic data, diagnoses and laboratory findings of vWD in Malaysia. A total of 92 patients were reported to have vWD in Malaysia from 2011 to 2013.
RESULTS: Sociodemographic-analysis revealed that 60% were females, 63% were of the Malay ethnicity, 41.3% were in the 19-44 year old age group and 15.2% were from Sabah, with the East region having the highest registered number of vWD cases. In Malaysia, most patients are predominately affected by vWD type 1 (77.2%). Factor 8, von Willebrand factor: Antigen and vWF: Collagen-Binding was the strongest determinants in the laboratory profiles of vWD.
CONCLUSION: This report has been done with great interest to provide an immense contribution from Malaysia, by revealing the statistical counts on vWD from 2011-2013.
OBJECTIVES: The primary aim of this review was to investigate the effect of fibrinogen concentrate in postoperative blood loss in adult surgical patients.
DESIGN: Systematic review and meta-analysis.
DATA SOURCES: Databases of MEDLINE, EMBASE and CENTRAL were searched from their start date until July 2019.
ELIGIBILITY CRITERIA: All randomized clinical trials comparing intravenous fibrinogen concentrate and placebo in adult surgical patients were included, regardless of type of surgery. Observational studies, case reports, case series and non-systematic reviews were excluded.
RESULTS: Thirteen trials (n = 900) were included in this review. In comparison to placebo, fibrinogen concentrate significantly reduced the first 12-hour postoperative blood loss, with a mean difference of -134.6 ml (95% CI -181.9 to -87.4). It also significantly increased clot firmness in thromboelastometry (FIBTEM) with a mean difference of 2.5 mm (95%CI 1.1 to 3.8). No significant differences were demonstrated in the adverse events associated with fibrinogen concentrate use, namely incidence of thromboembolism, myocardial infarction and acute kidney injury.
CONCLUSIONS: In this meta-analysis of 13 randomized trials, low level of evidence and substantial heterogeneity with small sample size limit strong recommendation on the use of fibrinogen concentrate in adult surgical patients. However, its use is tolerable without any notable adverse events.
TRIAL REGISTRATION: CRD42019149164.
METHODS: An electronic search was performed via PubMed, SCOPUS, Google Scholar, and Cochrane from inception to June 2022. The included trials were evaluated according to the recommendations of the Cochrane Handbook for Systematic Reviews. The primary outcome assessed was the intraoperative bleeding score of the surgical field. The mean arterial pressure, duration of the surgery, amount of blood loss and surgeon's satisfaction score were assessed as the secondary outcomes. The risk of bias for each study was evaluated using the Cochrane risk of bias tool.
RESULTS: A total of 254 records were identified after removal of duplicates. Based on the title and abstract 246 records were excluded, leaving seven full texts for further consideration. Five records were excluded following full text assessment. Three trials with a total of 212 patients were selected. Hot saline irrigation was superior to control in the intraoperative bleeding score (MD - 0.51, 95% CI - 0.84 to - 0.18; P < 0.001; I2 = 72%; very low quality of evidence) and surgeon's satisfaction score (RR 0.18, 95% CI 0.09 to 0.33; P < 0.001; I2 = 0%; low quality of evidence). The duration of surgery was lengthier in control when compared to HSI (MD - 9.02, 95% CI - 11.76 to - 6.28; P < 0.001; I2 = 0; very low quality of evidence). The volume of blood loss was greater in control than HSI (MD - 56.4, 95% CI - 57.30 to - 55.51; P < 0.001; I2 = 0%; low quality of evidence). No significant difference between the two groups for the mean arterial pressure was noted (MD - 0.60, 95% CI - 2.17 to 0.97; P = 0.45; I2 = 0%; low quality of evidence).
CONCLUSIONS: The practice of intranasal HSI during ESS is favorable in controlling intraoperative bleeding and improving the surgical field quality. It increases the surgeon's satisfaction, reduces blood loss, shortens operative time and has no effect on intraoperative hemodynamic instability.
TRIAL REGISTRATION: PROSPERO registration number: CRD42019117083.