Muscle Hemorrhage Research Articles

Determining the Possibilities of Reducing Visible Quality Defects in Commercial Elements of Turkey Carcasses Using an Alternative Stunning Device.

The development of the quality and commercial value of poultry meat is related to the formation of visible quality defects (hemorrhages) in muscles during the first stage of production (stunning). The production of meat with an unusual appearance for the consumers affects their purchasing decisions and, consequently, the company's economics. The aim of this research was to determine the possibility of reducing visible quality defects (large and small hemorrhages) in commercial turkey carcass elements (fillet, loin, wing) using an alternative device (AD) for the electrical stunning of animals in comparison to the quality effects obtained using the conventional device (CD) in plant X. The factors differentiating the experiment were the electrical current frequency (AD: 125, 400, 800, and 1600 Hz; CD: 50 Hz) and process time (9 and 18 s). The increase in electrical current frequency used in the alternative device stunner (own construction) resulted in changing the percentage share of defective turkey meat production. The greatest reduction of minor and severe meat defects and improvement of its quality were obtained for the alternative device at f = 800 Hz and t = 9 s-considered optimal for specific industrial conditions. Extending the time of stunning turkeys to 18 s had a positive effect on visible quality defects in the evaluated commercial elements of the carcass; however, its application in practice will depend on the efficiency of the slaughter line of the plant. A comparative analysis of the results of the impact of the frequency of electric current in the alternative device and plant X on the improvement of meat quality showed a justified need to commercialize the research results and replace the device currently used in the plant with an alternative one.

Structure, function and immune evasion strategies of aquareoviruses, with focus on grass carp reovirus

AbstractAquareoviruses (AqRVs) can infect various wild or cultured bony fishes with serious muscle and viscera haemorrhage syndrome and have caused severe economic losses worldwide. Accumulating evidences have revealed the functions of viral proteins of AqRVs in RNA transcription, virion assembly, cell attachment and/or viral replication. In addition, structural information and transcription mechanism of AqRVs have been in‐depth studied through cryo‐electron microscopy combined with three‐dimensional image reconstruction. Although far from enough, structural and functional studies of AqRVs provide substantial insights into how viral proteins coordinate the replication of AqRVs and escape the host immune response. Grass carp reovirus (GCRV) is the most pathogenic AqRV isolated from aquatic animals. The completion of GCRV lifecycle requires the participation of host proteins. So far, many host factors involved in the entry and replication of GCRV have been identified. Based on the function of viral proteins and the viral lifecycle, many vaccines and antiviral agents have been developed to prevent the replication and infection of GCRV. This review summarises the structural characteristics and the composition of core proteins of AqRVs, as well as synergistic mechanisms of structural proteins on genome transcription and virus entry of AqRVs. In addition, the review highlights our current knowledge for the infection and replication, functions of non‐structural proteins, immune escape mechanisms, as well as the vaccines and antiviral agents of AqRVs, especially for GCRV. A better understanding of structure, function and immune evasion strategies of AqRVs will offer new targets for prevention and control of AqRVs in aquaculture.

A Comparative Observational Study of Postmortem Computed Tomography and Traditional Forensic Autopsy Findings in Hanging Cases

Background: The use of advanced radiological techniques is rising in the field of forensics to supplement and validate the evidence. Postmortem Computed Tomography (PMCT) is now used as a standard procedure in many institutions to augment traditional autopsy findings. The application of PMCT in hanging cases will greatly reduce the number of invasive traditional autopsies, as hanging is the most common method of suicide in Northern parts of India. Therefore this observational study was conducted to compare PMCT with traditional autopsy in fifty hanging cases. Result: Out of 50 cases, the age of the subjects ranged between 14 years and 70 years. PMCT with the help of 3D reconstruction was able to detect external ligature marks on the neck in 92% (N=46) of cases giving a sensitivity of 92% (CI = 80.77% to 97.78%). Skin and subcutaneous tissue desiccation were identifiable in 96% (N=48) by PMCT, giving a sensitivity of 96% (CI= 86.29% to 99.51%). The muscle hemorrhage was identified in 4% (N=2) of the cases in traditional autopsy while the authors found difficulty in interpreting the muscle hemorrhage in PMCT. In detecting thyroid cartilage fracture, the sensitivity of PMCT was found to be 100% (CI= 15.81% to 100.00%) and specificity of 100% (CI=92.60% to 100.00%). In detecting hyoid bone fracture, the sensitivity was 100% (CI= 2.50% to 100.00%), and specificity of 100% (CI=92.60% to 100.00%). In addition, there was a great degree of agreement between the two observers for these findings suggesting the reproducibility of the result. Conclusions: A consistency in findings of both the PMCT and traditional autopsy was found. However, CT was unable to detect muscle hemmorhages, compared to traditional autopsy. Authors suggest the use of CT angiography to overcome this shortcoming, till then CT can only augment invasive autopsy findings not replace it

Towards achieving a haemophilia-free mind.

Towards achieving a haemophilia-free mind.

DosEmi study protocol: a phase IV, multicentre, open-label, crossover study to evaluate non-inferiority of pharmacokinetic-guided reduced dosing compared with conventional dosing of emicizumab in people with haemophilia A

IntroductionEmicizumab effectively prevents bleeding in people with haemophilia A (PwHA), but is a burden for national healthcare budgets and consequently may limit access. According to the drug label, dosing of...

Bleeding phenotype in nonsevere hemophilia by International Society on Thrombosis and Haemostasis bleeding assessment tool, bleeding frequency, and the joint status

BackgroundAlthough the phenotype of severe hemophilia has been well studied, there are still knowledge gaps in nonsevere hemophilia. ObjectivesThe objective of this study was to characterize the clinical bleeding phenotype in nonsevere hemophilia and its association with different factor VIII/IX assessments. MethodsThis was a cross-sectional, multicenter study to investigate the bleeding phenotype in adults with nonsevere hemophilia by the number of bleeding and joint bleeding in the past 5 years, a joint score, and the International Society on Thrombosis and Haemostasis bleeding assessment tool (ISTH-BAT). Factor levels were analyzed by 1-stage (lowest in history and at study inclusion) and chromogenic assay (at study inclusion). Patients were enrolled between March 2015 and May 2019. ResultsOf the 111 patients (86 with mild and 25 with moderate hemophilia), 57 patients (54.8%) reported any bleeding and 24 (23.1%) any joint bleeding in the past 5 years. A joint score ≥1 was found in 44 patients (41.9%), an ISTH-BAT ≥4 in 100 patients (90.1%), and an ISTH-BAT joint item ≥1 in 50 patients (45.0%). Within the ISTH-BAT, muscle and joint bleeds showed the largest difference between mild and moderate hemophilia. The lowest factor VIII/IX level in patients’ history was best associated with bleeding outcomes. Factor was inversely associated with joint bleeds (incidence rate ratio 0.88; 95% CI, 0.79-0.98), joint score, and ISTH-BAT (odds ratios from proportional odds ordinal logistic regression 0.92; 95% CI, 0.87-0.97; and 0.89; 95% CI, 0.86-0.93, respectively). ConclusionThe occurrence of joint bleeding differentiated persons with mild and moderate hemophilia. The ISTH-BAT and lowest factor in patients’ history provided valuable information of the bleeding phenotype in nonsevere hemophilia.

Mutation Analysis of F8 Gene and Phenotypic Characterization of Hemophilia A

Background Hemophilia A HA is a congenital bleeding disorder caused due to deficiency of Factor VIII clotting protein in the blood. It follows an X-linked recessive mode of inheritance.Aim In the present study clinical and haematological manifestations have been analysed and their correlation with molecular mutations in patients with haemophilia A has been tested.Methods A total of 90 HA patients were included in the study. Plasma coagulation test PT APTT and correction study factor VIII assay inhibitors screening test and quantification tests were done for all the patients. Mutation analysis was carried out to evaluate the molecular alteration in F8 gene only on exon.Results Among the 90 Hemophilia A patients included 3 3.3 were diagnosed as mild 15 16.6 as moderate and 72 80 were diagnosed as severe hemophilic patients. Positive family history was observed in 62 68.8 patients while 35 38.8 were born to consanguineous couples. Joint bleeding 88.8 was most commonly observed followed by mucocutaneous bleeding 66.6 dental bleeding 48.8 genitourinary bleeding 16.6 gastrointestinal bleeding 15.5 muscle bleeding 14.4 and intracranial bleeding 10. Around 13 14.4 HA patients developed inhibitors among which five were of low titer and eight were of high titer. Mutation analysis of 90 HA patients recorded a total of 21 mutations including 52.4 missense mutations 23.8 nonsense mutations 23.8 frameshift mutations. No novel mutation was noted.Conclusion The present study showed several missense mutations in severe HA patients. This result is a slight deviation from the reports of earlier Indian studies which could be due to difference in geographic population ethnicity and other factors. Hence a detailed genotype and phenotype screening in relation to severity and development of inhibitors is much essential for timely monitoring and appropriate treatment of Hemophilia A.

Evaluation of Wnt/β-Catenin Pathway Circulating Proteins in Children and Adolescents with Hemophilia: Correlation with the Degree of Hemophilic Arhtropathy

Introduction: The pathophysiology of hemophilic arthropathy (HAP) establishes a hallmark of heterogeneous mechanisms related with the joint degeneration which has not been fully elucidated especially in reference to the development of inflammatory and catabolic processes occurring within the articular cartilage and synovial membrane. Crucial pathways regulating bone remodeling are the Wnt/β-catenin and the Receptor Activator of Nuclear factor kB (RANK) Ligand (RANKL)/Osteoprotegerin (OPG) systems. The Wnt/β-catenin pathway plays a major role in osteoblast differentiation and calcium homoeostasis. Sclerostin (SOST) and Dickkopf-1 (DKK-1) are soluble antagonists of the Wnt/β-catenin pathway secreted solely by the osteocytes. Osteopontin (OPN) acts as a secreted protein that is referred to many aspects of bone metabolism. OPN participates in bone regulation through neurology, endocrine and immunity. In this context, we aimed to investigate the clinical significance of peripheral biomarkers implicated in HAP severity and correlate with HAP features in children and adolescents with hemophilia A and B. Patients and Methods: Οne hundred sixty two children and adolescents (mean age: 11.7 years, range 1-18.5 years) with severe, moderate and mild (n= 8) hemophilia A or B (n=11), on prophylaxis or on demand treatment (n= 6) were included in the study. Of them, 69 patients with median age of 11 years had no signs of HA (NHAP), 70 patients with median age of 14 years had arthropathy (HAP), while 23 patients with median age of 15.5 years presented with acute bleeding -hemarthrosis or muscle bleed- at the time of the biomarkers’ measurement- (AB). In all patients along with standard care laboratory evaluation, measurements of plasma circulating levels of DKK-1, RANKL, OPG, SOST and OPN were performed using immunoenzymatic techniques. Data are presented as mean ± SEM. Results: We found that a) DKK-1 levels were higher in patients with NHAP and AB compared to patients with HAP, 3.396.0±194.0 pg/mL and 3.221±282.2 pg/mL vs 2.544±172.7 pg/mL (p=0.002); b) SOST levels in patients with NHAP (192.5±27.0 pg/mL), HAP (205.5±22.3 pg/mL) and AB (188.9±30.3 pg/mL) did not differ between the groups of patients (p>0.950); c) OPG levels in patients with NHAP (9.6±0.2 pg/mL), HAP (9.3±0.3 pg/mL) and AB (8.5±0.3 pg/mL) and RANKL levels in patients with NHAP (673.0±53.9 pg/mL), HAP (717.6±53.0 pg/mL) and AB (728.0±75.0 pg/mL) did not differ between the groups of patients (p>0.148 and p>0.515, respectively); d) Similarly, OPN levels in patients with NHAP (57.6±6.9 pg/mL), HAP (65.2±7.3 pg/mL) and AB (59.4±5.9 pg/mL) did not differ between the groups of patients (p>0.822) and e) DKK-1 and OPG levels correlated significantly with FVIII/FIX levels at the time of measurement (r=-0.553, p=0.014 and r=-0.540, p=0.017, respectively). Conclusions: In this study, no significant perturbations of the crucial pathways regulating bone remodeling such as Wnt/β-catenin and RANKL/OPG systems were identified, by measuring circulating levels of key proteins involved in these pathways. Circulating DKK-1 seems to be a more sensitive biomarker indicating a potential decrease in osteoblastic activity. It appears that bone loss is rather rare in well-treated children and adolescents with hemophilic arthropathy comparing to young patients without arthropathy. Further studies are needed in order to determine how biomarkers in serum could play a prognostic role in the evolution of arthropathy from an early age in children and adolescents with hemophilia A or B, as well as the role of FVIII and FIX in bone metabolism, with the aim to ameliorate treatment strategies and prevent joint damage.

Bleeding Phenotype Does Not Appear to Correlate with Factor VIII Levels or Genetic Mutation in an Exclusive Pediatric Cohort of Hemophilia a Carriers

Introduction: Inherited Hemophilia A is defined as Factor VIII (FVIII) deficiency, with levels <0.40 IU/ml. Female carriers exhibit varying degrees of X chromosome inactivation, with resultant variable FVIII activity levels. Bleeding phenotype in Hemophilia A carriers has been thought to be due to low FVIII levels; however, adult studies have shown lack of correlation of FVIII levels and bleeding phenotype in some patients. Our study aimed to correlate FVIII levels with bleeding phenotype and genetic mutations in pediatric hemophilia A carriers. Methods: Retrospective medical record review of Hemophilia A carriers <18 years of age was undertaken at our center. Inclusion criteria were family history of Hemophilia A and FVIII mutation or FVIII level <0.40 IU/ml in patients without genetic testing done. Patients with other co-existing bleeding disorders were excluded. Statistical analysis was done by Chi Square/Exact tests. Results: Twenty-three pediatric Hemophilia A carriers were eligible, with median age of 12 years (range1-16). Fifteen were adolescents >10 years. Fourteen (60.9%) had FVIII level >0.40 IU/ml with 8 (57.1%) categorized as asymptomatic, and 6 (42.9%) symptomatic carriers in accordance with the new ISTH nomenclature. Nine (39.1%) patients had FVIII <0.40 IU/ml, with 8 (88.8%) categorized as mild (FVIII 0.05-0.40 IU/ml), 1 (11.1%) as moderate (FVIII 0.01-0.05 IU/ml), and none as severe (<0.01 IU/ml) Hemophilia A, as per the new nomenclature. Bleeding Scores (BS) were obtained in all patients using the ISTH Bleeding Assessment Tool (ISTH-BAT) with a median BS of 1 (range 0-7). A significant BS of >2 was present in 4/9 (44.4%) patients with FVIII <0.40 IU/ml, and 4/14 (28.6%) with FVIII >0.40 IU/ml, the difference was not statistically significant (p=0.40) (Table 1). Two symptomatic carriers with FVIII >0.4 IU/ml, had BS of 1 and 2. 5/15 adolescents (33.3%) had BS>2 (BS of 4 in 3; BS of 7 in 2). FVIII was measured with one stage assay (OSA) (N=17; 73.9%), chromogenic substrate assay (CSA) (N= 2; 8.7%) or both (N=4; 17.4%), with 1 patient's (4.3%) CSA discrepantly lower than OSA by 0.62 IU/ml and none with inverse discrepancy. Genetic testing was done in 20/23 (86.9%) patients, with FVIII missense mutations in 14 (70%), intron inversions in 4 (20%), and deletions in 2 (10%), their association with BS did not show statistically significant difference (p=0.6) (Table 2). Conclusion: Our study was done exclusively in pediatric Hemophilia A carriers, as it is important to identify children at risk of bleeding and institute preventive measures early. Unlike in adult women, higher number (one-third) of our patients experienced mucosal bleeding, and none reported joint or muscle bleeding. Our study showed lack of statistically significant association of FVIII levels with bleeding phenotype. According to the new ISTH nomenclature, 39.1% of our study patients have the diagnosis of Hemophilia A, of whom 55.6% did not have a significant BS. Of the 60.9% categorized as carriers, 28.6% had a significant BS. In addition, we found lack of statistically significant correlation of genetic mutation type with the bleeding phenotype, with none, 5% and 25% of the patients with deletion, intron inversion and missense mutation having a significant BS respectively. Follow-up/BS reassessment in our cohort is planned to monitor BS increase with age. Our study limitations include small cohort and retrospective analysis. Future pediatric studies are required to identify coagulation/genetic parameters that correlate better with bleeding phenotype, which may then help with early recognition and prompt institution of preventive therapy to pediatric Hemophilia A carriers at risk of bleeding, in order to avoid bleeding complications during adulthood. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal

Discrepancy between the Results of One-Stage and Chromogenic Factor VIII Assays: Switch in Diagnosis from Mild to Moderate Hemophilia a in Three Cases

Introduction Factor VIII (FVIII) activity can be measured with different methods including one-stage clotting assay (OSA) or chromogenic substrate assay (CSA). Some persons with mild hemophilia A (HA) have a significant discrepancy between the assays which has been previously reported. Discrepancy between these two methods, albeit less frequent, can also lead to a switch in diagnosis from mild to moderate HA. Herein, we report 3 persons with HA (PwHA) whose diagnosis changed from mild to moderate HA after obtaining FVIII levels assessed by CSA. Materials and Methods Data were extracted from the medical records of the PwHA including demographics, bleeding rates, and factor levels. FVIII activity was measured using the OSA on an ACL Top 500 with SynthaSil aPTT reagent (Instrumentation Laboratories Bedford, MA, USA). Factor VIII activity was also determined photometrically via the Chromogenix Coatest® SP4 FVIII chromogenic assay kit (bovine chromogenic FVIII activity, Diapharma Group, West Chester, OH). Results Data were collected from 3 PwHA who were previously diagnosed with mild HA yet were noted to have a bleeding phenotype worse than expected for mild HA (Table-1). Case 1 is 26 years old and had been experiencing multiple joint bleeds. His OSA FVIII level was 6.8%. He was on clotting factor concentrate (CFC) on demand. After having severe hemophilic arthropathy affecting his social life, prophylaxis with CFC was commenced. Significant improvement was observed after the initiation of prophylaxis. His CSA FVIII level was 2.7%, consistent with his bleeding phenotype, and he has been on emicizumab prophylaxis for a year without any bleeding events. Case 2 is 22 years old and had been experiencing frequent muscle bleeds. His OSA FVIII level was 6.1% and CSA FVIII level was 2.7%. He remains on CFC on demand based on his preference despite the frequent bleeding. Case 3 is 15 years old and was diagnosed with mild HA at the age of 8 after recurrent joint bleeds. His OSA FVIII level was 13.8% and CSA FVIII level was 1.5%. He has been on emicizumab prophylaxis for 10 months without any bleeding event. Conclusion While discrepancies between assays in mild hemophilia have been widely reported, there are fewer reports on an assay discrepancy changing a diagnosis from mild to moderate HA. As such, we strongly suggest performing both OSA and CSA for the diagnosis and classification of HA especially if the PwHA has more bleeding episodes than expected. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal

Follow-up and Evaluation of the Efficacy of the Hemostatic Treatment in Congenital FVII Deficiency during Development of Inhibitor

Introduction The development of inhibitors is an extremely rare complication in patients with factor (F)VII deficiency treated with replacement therapies such as recombinant activated FVII (rFVIIa), and represents a major therapeutic challenge in the treatment of this disorder. When inhibitors result in loss of response to high doses of rFVIIa, activated prothrombin complex concentrate (aPCC) is often used as an alternative treatment for these patients. The use of high-dose rFVIIa or aPCC might be accompanied by an increased risk of thromboembolic events, so it is important monitoring properly the effect of the hemostatic treatment Here we describe the therapeutic management and follow-up of a patient with elevated inhibitor titres, resistant to rFVIIa treatment who switched to prophylaxis with aPCC, using several global coagulation tests. Methods The coagulation status of the patient and the reponse to in vitro spiking of different doses of rFVIIa or aPCC were assessed with several global tests: Prothrombine time (PT) was tested using HemosIL®RecombiPlasTin 2G. Clot formation was evaluated with Rotational Thromboelastometry (ROTEM®) using blood with CTI (to inhibit contact activation) and activated with a low amount of Tissue Factor (TF) as trigger (dilution 1:50,000 of EXTEM reagent). The thrombin generation (TG) was tested with the Calibrated Automated Thrombogram (CAT) system using plasma collected with CTI and activated with 1 pM TF plus phospholipids (PPP-Low®, Stago). Thrombus formation under flow conditions was assessed with the Total Thrombus-formation Analysis System (T-TAS®) in AR-chips coated with collagen and thromboplastin. The Area under the flow pressure curve (AUC) was calculated over 30 min after starting. Results Three year-old girl affected by severe congenital FVII deficiency (FVII 9.1%) with significant severe bleeding history (spontaneous intracranial hemorrhage at 1st month of life). Since then she is in prophylaxis treatment with rFVIIa. At 6 months of life she developed an inhibitor (9.8 BU). Despite prophylaxis she presents frequent spontaneous mucocutaneous bleedings. When she was 2 years old she needed to be admitted at hospital because of port-A-Cath infection. Due to the administration of intensive treatment with rFVIIa during admission she developed thrombosis of peripheral venous access, so prophylaxis treatment was temporary suspended. After 5 days without prophylaxis treatment, and increasing inhibitor titres, she developed a new intracranial haemorrhage, which needed intensive treatment with continuous rFVIIa infusion (90 µg/Kg/12h), immunoglobulines and rituximab for lowering inhibitor titre. Hemostatic parameters improved during the administration of these treatments and inhibitor level was reduced. After restarting prophylaxis treatment with rFVIIa (50 µg/Kg/48h), inhibitors and a hypocoagulable state were observed with all global coagulation test showing extended PT, clotting time (CT), TG Lag-time (LT) and anomalous thrombus formation (AUC) but presented very good response to low dosis (2.5 IU/Kg) of aPCC in vitro (Table-1). At the age of three years she required admission at hospital because of port-A-Cath infection again, needing its replacement by a new one after administration of high doses of rFVIIa without bleeding complications. After this, inhibitor titre raised again (> 50 BU), presenting frequent bleeding episodes (spontaneous muscle bleedings and tonsillar bleeding), and due to the clinical worsening, treatment was modified to prophylaxis with aPCC (30 UI/Kg/48h). Despite the treatment change she continued presenting bleeding episodes, global tests continued showing an hypocoagulable state, and required 10 times higher doses in vitro of aPCC (25 IU/Kg) to restore all the monitored hemostatic parameters (Table-1), probably related to the important increase in inhibitor titre. This situation led to employing higher doses of aPCC prophylaxis (60 UI/Kg/48 h) to try to avoid bleeding episodes without increasing the risk of suffering thrombotic events. Conclusions Global coagulation tests used in this study were sufficiently sensitive methods to show an abnormal hemostatic profile in FVII-deficient patients with clinical hemorrhagic symptoms with inhibitor development, supporting their use to manage the risk of bleeding or thrombosis in this life-threatening condition. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal

Clinical and laboratory presentation of von Willebrand disease: Experience from a single center in Saudi Arabia

ObjectivesThis study was aimed at assessing the clinical presentations and laboratory findings among patients diagnosed with vWD at a Saudi tertiary care unit. MethodsThis retrospective study included 189 patients with vWD who were followed up in our unit over 4 years. Clinical and laboratory data were collected and analyzed in SPSS. ResultsThe median age of the study cohort was 30 years (range 11 months–56 years). The cohort had a female preponderance, with 32.30% males and 66.70% females. Bleeding from different sites was observed, mostly from the joints and muscles (23.90%), followed by the mucus membranes (14.60%), genitourinary areas (7.70%), ecchymoses (2.80%), and gastrointestinal areas (2.80%). A total of 48% of participants presented with more than one type of bleeding. A total of 105 (58.01%) participants had type 1; 29 (16.02%) had type 2; and 47 (25.96%) had type 3 vWD. Blood tests indicated the following mean value: hemoglobin, 116 ± 25.60 gm/L; ferritin, 75.80 ± 166.80 μg/L (median 28.5); vWAg, 0.40 ± 0.27IU/ml; and vWD:RCo, 0.32 ± 0.20IU/dL. The partial thromboplastin time was prolonged in 49.20% and normal in 50.80% of participants. Platelet function analysis values were prolonged in 92.90% and normal in 7.10% of participants. Comparative analysis of the O-type and non-O blood type showed that blood type O was significantly correlated with factor VIII (p-value = 0.013), vWF:RCo (p-value = 0.004), and vWF:Ag (p-value = 0.019). ConclusionJoint and muscle bleeds were the most common clinical presentations in our cohort. Although type 1 vWD was most prevalent in our cohort, we observed a comparatively higher prevalence of type 3, possibly because of ethnic differences or referral bias. We found a significant difference between O and non-O blood type regarding FVIII and vWF:Ag, and observed a more pronounced difference for vWD activity measuresd by vWF:RCo with blood type O being the systematic factor.

Intramuscular hemorrhages in the pathway of an electric current through the body — two case reports

Intramuscular hemorrhages at autopsy can have a variety of traumatic as well as non-traumatic causes, but their recognition in electrical deaths is almost a rarity. We report on two autopsy cases of electrical fatalities, the first relating to a portion of the right upper human extremity, consisting (only) of the forearm and hand, while the other case relates to a female child who died after a high voltage electrical shock. In both cases, layered dissection of the upper limb revealed fresh intramuscular hemorrhages in the skeletal muscles that could be topographically related to the path taken by the current through the body. Externally visible electric marks were present in both cases. The hemorrhages were most likely caused by current-induced tetanic muscle contractions, producing an internal muscle trauma with rupture of fibers and bleedings. In complex situations, such as inconspicuous marks or a complete lack of visible signs on the body, the finding may be helpful in solving the case in consideration of the case history and circumstances. The vitality, topography, and pattern of the hemorrhages are discussed in the light of the available literature.

Phenotypic variation in severe hemophilia A is related to endogenous thrombin potential and plasma levels of factor VII.

Hemophilia A is a bleeding disorder caused by deficiency or low activity of circulating factor VIII characterized by prolonged blood coagulation time and often spontaneous bleeding. Patients with the severe form of the disease may present considerable heterogeneity in the occurrence of bleeding episodes and some of them have a mild hemophilia A phenotype. This study aimed to evaluate the association of biomarkers and coagulation parameters to the differential hemorrhagic profile of severe hemophilia A patients. Polymorphisms in the genes of proteins C and S, factors V and VII and prothrombin were evaluated in a group of severe hemophilia A patients with a broad spectrum of bleeding profile. Plasma levels of coagulation factors and thrombin generation were also analyzed. This study included 59 Brazilian hemophilia A patients who were allocated into low bleeding profile (LBP; n = 33) and high bleeding profile (HBP; n = 26) groups based on their joint and muscle bleeding episodes requiring treatment in the 5 years before inclusion in the study. Results evidenced that endogenous thrombin potential (ETP) and plasma factor VII levels were significantly higher in the LBP group. Results indicate a prominent importance of FVII plasma activity and endogenous thrombin potential on the differential bleeding phenotype of hemophilia A patients.

The evolution of physiotherapy in the multidisciplinary management of persons with haemophilia (PWH): A scoping review.

Haemophilia is a rare congenital bleeding disorder, and the most common manifestation is spontaneous bleeding in muscles and joints. Despite the benefits linked to recent and dramatic pharmacological advances at least in high income setting, many patients still develop musculoskeletal dysfunctions during their lifetime, which must be managed by physiotherapists in the frame of a multidisciplinary team. The aim of the scoping review is to map the available evidence by providing an overview on the past and present physiotherapy scenario in persons with haemophilia (PWH). The review was conducted according to the guidelines of the PRISMA extension for scoping reviews. Scientific articles on physiotherapy and sport interventions for PWH published from 1960 up to September 2021 have been included. Search was conducted on the e-databases PubMed and PEDro without restrictions for the study design. Sixty eight articles were included, 52 related to rehabilitation and preventive physiotherapy, 16 to sport. The results have been reported in chronological order and divided into two categories: (1) rehabilitation and preventive physiotherapy; (2) sport activities. This is the first scoping review on physiotherapy in haemophilia, based on the existing evidence on this topic which allowed us to underline how the role of the physiotherapist changed over time. Historically this specialist did intervene only after an acute bleed or surgical operation, but now he has a pivotal role in the multidisciplinary team that acts to improve from birth the quality of life of the PWH. His activity is also closely intertwined with sport promotion and supervision.

Assessment of quality of life by functional independence score in hemophilic patients: a single-center experience

BackgroundHemophilia is an inherited condition that is caused by the deficiency of clotting factors in the blood. The most common presentations of hemophilia are hemarthrosis and muscle hemorrhage. The severity of the bleeding episodes can vary from mild to severe resulting in pain and swelling of the joints and the muscles. Repeated musculoskeletal bleeding can result in hemophilic arthropathies with subsequent joint degeneration and deformity. Our study aims to clinically assess the quality of life in hemophilic patients in terms of disease severity and morbidity in our community using the Functional Independence Score for Hemophilia (FISH).ResultsOur cross-sectional study which involved 64 hemophilia patients revealed that the most affected joints were the weight-bearing large joints (knees, elbows, ankles) and this was associated with a lower FISH score. While the total FISH score showed a significant positive correlation with the factor activity level, the average FISH score was 21.11 ± 4.5, and the score was slightly lower in severe hemophilia compared to mild-moderate disease.ConclusionWe concluded that there is a significant decline in the functional ability of hemophilia patients having disease affecting the knee and elbow joints. Moreover, squatting was significantly reduced in patients with severe hemophilia. The quality of patients’ life can be improved by early and regular physiotherapy and regular administration of the prophylactic factor.

Spontaneous atypical muscle bleeding of the anterior abdominal wall in patient with COVID-19 – case report

COVID-19 is an unpredictable disease that can lead to multiorgan dysfunctions. There is a high frequency of venous and arterial thrombosis, among other symptoms,. Spontaneous bleeding in COVID-19 patients has also been described, but rarely, whether or not they are on anticoagulant therapy. We report a case of a 65-year-old female COVID-19 patient treated in our hospital. During the hospitalisation, she experienced sudden, severe pain in the lower part of the abdomen and had signs of hemorrhagic shock. CT of the abdomen and pelvis revealed a spontaneous giant haematoma of the anterior abdominal wall. A surgical procedure was done. We identified spontaneous bleeding in the muscles of the anterior abdominal wall. The patient recovered well. Rapid diagnosis and timely intervention are crucial to ensure a good patient outcome.

An observational study of haemophilia patients attending a tertiary care centre in West Bengal

Background: Haemophilia is the most common inherited coagulation disorders transmitted by X-linked recessive fashion affecting the males and females are the carriers of the disease. Haemophilia is distributed worldwide and has heterogeneous presentation depending on its severity starting from neonatal period. Knowledge of spectrum of the presentation of haemophilia helps in early diagnosis and planning of management. Aims and Objectives: This study aims to evaluate the socio-demographic profile of the patients, clinical presentations, epidemiological profiles, and the outcomes of the hemophilic patients in a tertiary care teaching hospital in West Bengal. Materials and Methods: This study was a prospective, observational, single center study conducted in the Pediatrics haemophilia treatment center at Midnapore Medical College, Paschim Medinipore from May 2020 to April 2021. Detailed history was taken and recorded in a predesigned pro forma Case Record Form. Analyses were done only after completion of CRF of the last patient. Statistical analyses were performed using SPSS version 20.0 software. Continuous variables were compared by Paired t-test. Dichotomous events were analyzed using the Fisher’s exact test and Chi-squared Test. Statistical significance was defined as P&lt;0.05. Results: Mean age of children was 93.64 months (SD 6.38). All children of haemophilia were male. This study noted that majority of patients of haemophilia were staying at rural area (67.19%) and rest at urban area (32.81%). BMI of majority children was within normal limit (51.56%), followed by underweight children (32.81%), overweight children (12.50%), and obese child (3.13%). Factor assay showed that 51 children (79.69%) had severe factor deficiency and 13 children (20.31%) had moderate factor deficiency. Positive family history was seen in 41 children (64.06%), and rest 23 children (35.94%) had no family history of haemophilia or any other bleeding disorder. Majority of patients (26 patients) came with their first bleed during infancy (40.63%), 21 children presented between the age of &gt;12 months to ≤24 months (32.81%). Bruises were the most common presentation (37.50%), followed by joint bleeding (31.25%), muscle bleed (10.94%), and gum bleeding (7.81%). Conclusion: Facility of counseling for children of hemophilia is required at regular interval (trimester wise/semester wise) with the aim to enhance the knowledge of personal care, treatment, and to boost up themselves for their academics, activities, etc.

Ligature strangulation in 2001–2020 in the materials of the Jagiellonian University Department of Forensic Medicine, Kraków

Aim: The aim of the study was to analyze and compare the most common post-mortem changes which can be found in the body after ligature strangulation and compare it with the most recent literature. Materials and methods: autopsy protocols from years 2001–2020 gathered in the Department of Forensic Medicine in Kraków were investigated. 37 cases of certain and possible strangulation were found and analysed. Prevalence of ligature strangulation, age, sex, information concerning perpetrator and post-mortem changes were considered. Results: we found and analysed 37 cases, however only in 16 cases the certain cause of death was ligature strangulation. in the remaining 18 cases the mechanism of death was not precisely defined. in 3 cases advanced late post-mortem changes disturbed establishment of certain identification. Women constituted 87.5% of all victims, while men 12.5%. among 16 certain ligature strangulations we analysed the most important post-mortem changes. Both subconjunctival hemorrhagic petechiae and facial skin petechiae were observed in 93.8% of the cases, laryngeal or hyoid fracture in 56.3%, and hemorrhages in neck muscles were detected in 87.5% of the cases. Conclusions: The most frequent post-mortem changes after ligature strangulation include subconjunctival petechiae, facial skin petechiae and hemorrhages in the neck muscles which are convergent with the data found in literature. The autopsy may not indicate the circumstance of strangulation.

The Emergence of Dry Needling in Stroke Rehabilitation

The management of stroke, acute revascularization and rehabilitation techniques have taken a giant leap in the current decade. The presence of neurological deficits, spasticity and pain are the common limiting factors in post-stroke patients. The various rehabilitation measures in stroke are focused on improving the trio. Spasticity, in a post-stroke patient, is disabling and can be managed using oral pharmacological agents, botulinum toxin, intrathecal baclofen. The use of needle therapy, dry needling, which was commonly used in the management of myofascial pain syndromes has emerged useful in the management of spasticity. Further recent evidence has shown it to be useful in the other dimensions of stroke rehabilitation. Dry Needling is a skilled intervention, that is performed by trained personnel using a filiform needle. It can be performed superficially or deep. The speculative mechanisms by which dry needling reduces spasticity include inducing stretch in the muscle and afferent modulation of the sensory information from the muscle. It improves blood flow and oxygenation, reduces pain through central and peripheral mechanisms. (1)&#x0D; The use of dry needling in neurology has evolved from its use in the management of myofascial pain to spasticity in spastic tetra paresis and stroke rehabilitation. In post-stroke patients, dry needling has been used in the management of post-stroke spasticity, balance, post-stroke pain. In the management of spasticity, dry needling using ‘pistoning technique’ in the lower limbs (gastrocnemius, tibialis posterior) and upper limbs (shoulder, elbow and wrist) revealed improved scores in modified modified Ashworth scale (MMAS) that translated to improved gait and range of upper limb motion respectively. The dry needling was performed for 1 minute in each muscle studied. However, the number of sessions varied from a single session to 8-week therapy between studies. In the management of balance, the dry needling of the involved tibialis posterior muscle improved the balance as measured by single-leg stance, balance component of Fugl-Meyer scale and dynamic posturography. The improvement in the above measures is also shown to improve the functional outcome of post-stroke patients in Barthel index, 10-metre walk test, timed up and go test. The dry needling is shown to reduce pain including post-stroke shoulder pain by reducing the pain threshold. The above effects of dry needling were also evident in ultrasound assessment of the intervened gastrocnemius muscle that showed improved muscle length, reduced mean muscle thickness and angle of pennation. (1-3)&#x0D; The dry needling has also been studied in post-stroke rehabilitation as a component of the multimodal approach. The use of dry needling in conjunction with electrical stimulation of the muscle is shown to produce persistent improvement in the tone of the muscle. The adverse events noted following the use of dry needling include post interventional muscle soreness, bleeding, bruise and pain during the intervention. Major side effects such as loss of consciousness are rare and hence dry needling serves as a safe therapeutic option in the rehabilitation of post-stroke patients. (1-3)&#x0D; Hence dry needling is a safe, cost-effective technique that can be used in the management of post-stroke spasticity, balance, pain that translates to improved functional outcomes. However, further evidence is required to establish the efficacy of this promising technique in the management of post-stroke rehabilitation.