Bleeding Research Articles

Clinical Course and Treatment of Patients With Apical Aneurysms Due to Hypertrophic Cardiomyopathy

BackgroundThere is controversy about risk of malignant arrhythmias and stroke in patients with apical aneurysms in hypertrophic cardiomyopathy (HCM). ObjectivesThe aim of this study was to estimate the associations of aneurysm size and major HCM risk factors with the incidence of lethal and potentially lethal arrhythmias and to estimate incidence of unexplained stroke. MethodsIn 108 patients (age 57.4 ± 13.5 years, 37% female) from 3 HCM centers, we assessed American Heart Association/American College of Cardiology guidelines risk factors and initial aneurysm size by echocardiography and cardiac magnetic resonance imaging and assessed outcomes after median 5.9 (IQR: 3.7-10.0) years. ResultsImplantable cardioverter defibrillator discharges or sudden cardiac death (SCD) occurred in 21 (19.4%) patients. Of patients with a risk factor, 55% subsequently had ventricular tachycardia (VT), ventricular fibrillation (VF), or SCD at follow-up, compared with 10% in those who did not (P < 0.001). The upper tercile of size had a 5-year cumulative risk of 35%, while the lower tercile had 5-year risk of 6% (P = 0.0046). In those with the smallest aneurysms <2 cm2 and also without risk factors VT, VF, or SCD occurred in only 2.5%. Clinical atrial fibrillation (AF) was prevalent, occurring in 49 (45%). Stroke was commonly associated with AF. Stroke without conventional cause had an incidence of 0.5%/year. Surgery in 19% was effective in reducing symptoms. VT ablation and surgery were moderately effective in preventing recurrent VT. ConclusionsRisk factors and aneurysm size were associated with subsequent VT, VF, or SCD. Patients with aneurysms in the lowest tercile of size have a low cumulative 5-year risk. Clinical AF occurred frequently. Stroke prevalence in absence of known stroke etiologies is uncommon and comparable to risk of severe bleeding.

Residual Cyst Mimicking an Aggressive Neoplasm—A Life-Threatening Condition

Odontogenic cysts are frequently encountered in clinical practice. However, residual cysts, a specific type of inflammatory odontogenic cyst, are relatively rare. These cysts may slowly expand over time, damaging surrounding soft tissues and bone, typically without posing a threat to life. We report the case of a 67-year-old man with liver failure and a cystic tumor in his right maxilla that had invaded his oral cavity and cheek, causing nasal obstruction and severe bleeding following an incisional biopsy. A computed tomography (CT) scan of the mass was nonspecific, and an initial histopathological analysis of the tissues was inconclusive due to chronic inflammation and hemorrhagic alterations, complicating the diagnostic pathway. The suspicion of a potentially aggressive malignant neoplasm and the need for immediate intervention due to bleeding necessitated a tracheotomy, tumor removal, percutaneous endoscopic gastrostomy, and transfusions of red blood cells. A subsequent histopathological examination revealed features indicative of a residual cyst. The entire lesion was excised through functional endoscopic sinus surgery. The surgical treatment was performed safely and effectively. Follow-up CT confirmed complete removal of the lesion. This case highlights a rare yet possible complication of odontogenic cysts and underscores the necessity of early diagnosis and comprehensive prophylaxis to prevent severe complications.

Evaluation of Safety and Efficacy of Emicizumab Prophylaxis in Egyptian Pediatric Patients with Hemophilia A: Single Center Cross Sectional Study.

Hemophilia A (HA) is an X-linked hereditary bleeding disorder caused by deficiency of coagulation factor VIII activity. Emicizumab is a bispecific monoclonal antibody that replaces the function of activated FVIII and prevents bleeds in patients with hemophilia A. Emicizumab is expected to reduce the risk of severe bleeds in those patients with their subsequent complications. However, data about its safety and efficacy in patients with hemophilia A is limited. We aimed to evaluate safety and efficacy of Emicizumab prophylaxis in Egyptian pediatric patients with HA. A prospective cohort study was conducted on 88 HA patient who received prophylaxis with Emicizumab. Breakthrough bleeding episodes as well as annualized bleeding rate(ABR) were reported for all patients before and after Emicizumab prophylaxis. All adverse events during prophylaxis were reported to evaluate the safety of Emicizumab. Joint bleeds were present in 94 % of the patients. 58% of them had one target joint, 36.4% had more than one target joint while 5.6% had no target joints. 17% of patients were positive for FVIII inhibitors. The median annualized joint bleeding rate (AJBR) was reduced remarkably after Emicizumab prophylaxis (36 before versus zero after Emicizumab. Also, the median ABR was 48 before Emicizumab versus zero after Emicizumab. Eight patients developed mild breakthrough bleeding episodes. The most common adverse events were local reaction at the injection sites, headache, arthralgia, fever and diarrhea. Emicizumab prophylaxis was associated with significantly lower rate of bleeding events in patients with HA with and without inhibitors. The majority of patients had zero bleeds with Emicizumab prophylaxis.

Prognostic analysis of destroyed-lung patients with preoperative massive hemoptysis: a 20-year retrospective study

BackgroundSurgery is the main treatment option for destroyed-lung (DL) patients with life-threatening massive hemoptysis. However, short-term and long-term surgical safety and efficacy are unclear, prompting this study.MethodsData from 124 DL patients undergoing surgery between November 2001 and January 2022 at Beijing Chest Hospital were retrospectively analyzed. Data of the DL group (82 cases) and DL + massive hemoptysis group (42 cases) were compared with regard to clinical characteristics, long-term postoperative residual lung reinfection.ResultsAs compared with DL group rates, The DL + massive hemoptysis group had greater incidence rates of postoperative complications, invasive postoperative respiratory support, long-term postoperative residual lung reinfection, and postoperative tuberculosis recurrence. Revealed risk factors for postoperative complications (Extent of lung lesion resection), postoperative invasive respiratory therapy (preoperative Hb < 9 g/L, severe intraoperative hemoptysis), and postoperative long-term residual lung reinfection (DL with massive hemoptysis).ConclusionsDL patients with massive hemoptysis had greater rate of invasive respiratory support therapy and postoperative complications. Extensive lesion removal, preoperative anaemia, severe intraoperative bleeding associated with recent postoperative complications for the patient.

Aging Influences Fracture Healing on the Cellular Level and Alters Systemic RANKL and OPG Concentrations in a Murine Model.

Clinical complications frequently follow polytrauma and bleeding fractures, increasing the risk of delayed fracture healing and nonunions, especially in aged patients. Therefore, this study examines age's impact on fracture repair with and without severe bleeding in mice. Young (17-26 weeks) and aged (64-72 weeks) male C57BL/6J mice (n=72 in total, n=6 per group) are allocated into 3 groups: the fracture group (Fx) undergoes femur osteotomy stabilized via external fixator, the combined trauma group (THFx) additionally receives pressure-controlled trauma hemorrhage (TH) and Sham animals are implanted with catheter and fixator without blood loss or osteotomy. Femoral bones are evaluated histologically 24h and 3 weeks post-trauma, while RANKL/OPG and β-CTx are measured systemically via ELISA after 3 weeks. Aging results in less mineralized bone and fewer osteoclasts within the fracture of aged mice in contrast to young groups after three weeks. Systemically, aged animals exhibit increased RANKL and OPG levels after fracture compared to their young counterparts. The RANKL/OPG ratio rises in aged Fx animals compared to young mice, with a similar trend in THFx groups. In conclusion, age has an effect during the later course of fracture healing on the cellular and systemic levels.

Efficacy and safety of aspirin plus clopidogrel versus aspirin alone in ischemic stroke or high-risk transient ischemic attack: A meta-analysis of randomized controlled trials.

Background: Antiplatelet therapy plays an important role in reducing the risk of stroke recurrence in patients with mild ischemic stroke or high-risk transient ischemic attack (TIA). However, data regarding the effectiveness and safety of using aspirin plus clopidogrel in dual antiplatelet therapy (DAPT) compared to aspirin alone in mild ischemic stroke is limited. Methods: PubMed/MEDLINE, Embase, Cochrane Library, and ClinicalTrials.gov were searched for randomized controlled trials (RCTs) that compared DAPT to aspirin alone started within 72 hours in mild ischemic stroke or high-risk TIA. We used a random effects model to pool risk ratios (RRs) along with 95% CIs for clinical outcomes. Results: Four RCTs with 16,547 patients were included in this study. DAPT significantly reduced the risk of recurrent stroke by 26% (RR: 0.74; 95% CI: 0.67-0.83; p < 0.00001), ischemic stroke by 28% (RR: 0.72; 95% CI: 0.65-0.80; p < 0.00001), and major adverse cardiovascular events (MACE) by 24% (RR: 0.76; 95% CI: 0.68-0.84; p < 0.00001) compared to aspirin monotherapy. However, DAPT was associated with a significantly increased risk of moderate or severe bleeding (RR: 1.88; 95% CI: 1.10-3.23; p = 0.02) compared to aspirin alone. No significant differences were observed for hemorrhagic stroke (RR: 1.77; 95% CI: 0.96-3.29; p = 0.07), all-cause mortality (RR: 1.25; 95% CI: 0.87-1.80; p = 0.23), cardiovascular mortality (RR: 1.38; 95% CI: 0.81-2.33; p = 0.23), and myocardial infarction (RR: 1.63; 95% CI: 0.77-3.46; p = 0.20). Conclusion: DAPT involving aspirin plus clopidogrel reduces stroke recurrence and MACE but can lead to an increased risk of moderate or severe bleeding compared to aspirin monotherapy. (PROSPERO ID: CRD42024499310).

Postmenopausal Vaginal Bleeding Due to Vaginal Leech Infestation: A Case Report

Introduction: Vaginal bleeding due to leech bites is a highly unusual and serious problem in postmenopausal women, potentially leading to severe complications and even death. Case Presentation: A 68-year-old rural woman, 13 years post-menopause, was admitted to a hospital in eastern Iran with a complaint of continuous vaginal bleeding with clot discharge for 12 days. During the medical examination, a dark worm moving in waves was observed in the labia minora of the vulva. The area was washed with 0.9% normal saline solution, and the leech was removed from the inner surface of the labia minora using forceps and pincers. The patient was monitored as an outpatient for 4 hours to control the bleeding and was subsequently discharged in good condition. She was advised to take iron tablets and folic acid for one month. Her test results were as follows: hemoglobin = 10 mg/dL, hematocrit = 32%, platelet count = 145,000, prothrombin time = 13 seconds, partial thromboplastin time = 35 seconds, and INR = 1.1. Her vital signs at admission were: temperature = 37.5°C, pulse rate = 100/min, blood pressure = 120/60 mmHg, and respiratory rate = 30 breaths per minute. Conclusions: Vulvar and vaginal leech bites can cause severe vaginal bleeding and anemia in postmenopausal women. To reduce complications caused by delays in diagnosis or treatment, healthcare workers should consider the possibility of vulvar and vaginal leech bites in postmenopausal rural women and individuals who use river water for drinking, bathing, or showering. Taking a detailed history and performing a thorough physical examination before any diagnostic procedure can help quickly and accurately diagnose the cause of bleeding in these patients.

Favorable safety profile of NOAC therapy in patients after tricuspid transcatheter edge-to-edge repair.

Transcatheter edge-to-edge repair for severe tricuspid regurgitation (TR) is a new treatment option (t-TEER). Data on optimal antithrombotic therapy after t-TEER in patients with an indication for anticoagulation are scarce and evidence-based guideline recommendations are lacking. We sought to investigate efficacy and safety of novel oral anticoagulation (NOAC) and vitamin-K-antagonists (VKA) in patients undergoing t-TEER. Among 78 consecutive patients with t-TEER of severe TR, 69 patients were identified with concomitant indication for oral anticoagulation. Outcomes of these patients treated with NOAC or VKA were compared over a median follow-up period of 327 (177-460) days. Despite elevated thromboembolic and bleeding risk scores (CHA2DS2-VASc 4.2 ± 1.1, HEMORR2HAGES 3.0 ± 1.0 and HAS-BLED 2.1 ± 0.8), only one major bleeding incidence occurred under NOAC therapy. The risk for overall (NOAC 8% vs. VKA group 26%, p = 0.044) and major bleeding events (NOAC 2% vs. VKA 21%, p = 0.010) was significantly lower in the NOAC compared to the VKA group. No significant difference was found between NOAC and VKA treatment in terms of mortality (NOAC 18% vs. VKA 16%, p = 0.865) or the combined endpoint of death, heart failure hospitalization, stroke, embolism, thrombosis, myocardial infarction, and severe bleeding (NOAC 48% vs. VKA 42%, p = 0.801). A comparison between apixaban (n = 27) and rivaroxaban (n = 16) treated patients revealed no significant differences between NOAC substances (all bleeding events apixaban 7% vs. rivaroxaban 13%, p = 0.638). Results of this study indicate that NOACs may offer a favorable risk-benefit profile for patients with concomitant indication for anticoagulation therapy following t-TEER.

Efficacy and safety of recombinant porcine factor VIII in Japanese patients with acquired hemophilia A

Acquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies inhibiting human factor VIII (hFVIII). This phase II/III open-label study evaluated the safety and efficacy of recombinant porcine factor VIII (rpFVIII, susoctocog alfa) in adults with AHA and severe bleeding episodes in Japan (NCT04580407). The initial rpFVIII dose was 200 U/kg, with subsequent doses based on clinical measures including plasma FVIII activity. The primary efficacy endpoint was the proportion of severe bleeding episodes with a positive response to rpFVIII therapy 24 h after treatment initiation. Five patients were eligible for, and completed, rpFVIII treatment (age group: 60s–80s; median hFVIII inhibitor: 52 BU/mL; porcine FVIII [pFVIII] inhibitor: 3/5 patients). The median (range) total dose/patient was 548.4 (198–1803) U/kg with a median 3.0 infusions/patient. All patients responded positively to rpFVIII therapy at 24 h regardless of baseline pFVIII inhibitor status. rpFVIII treatment was well tolerated with no adverse events of special interest such as thromboembolic events or de novo pFVIII inhibitors. This study supports the use of rpFVIII as a novel therapy in the clinical management of patients with AHA in Japan. rpFVIII was approved for treating bleeding episodes in adults with AHA in Japan in 2024.

Cervical fibroid: A diagnostic dilemma and operative challenge – one year study

: Cervical fibroids are very rare, with varying clinical presentations, and account for only 1–2% of cases. Cervical fibroids are enigmatic as they present with varying symptoms and simulate several clinical entities, for instance, a large cervical polyp, incarcerated procidentia, chronic uterine inversion, and the Ca cervix. : A two-year retrospective analysis of women diagnosed with cervical leiomyoma was conducted at Obstetrics and Gynaecology department, PGIMS Rohtak (a tertiary care institute in Northern India). A total of 24 cases diagnosed with cervical fibroid (CF) were studied. 75% of the females had vaginal bleeding, 44.6% had heavy menstrual bleeding, 33.3% had irregular bleeding, and one had postmenopausal bleeding. 41.6% had urinary symptoms; 1.5% complained of vaginal discharge; difficulty in stools (16.6%); and leiomyosarcoma (8.3%). We cannot find a clear demarcation of presenting symptoms between anterior and posterior fibroid. It was discovered that the development of malignancy and bladder and intestinal problems was linked to an increase in CF mass, while severe anaemia and vaginal bleeding were not. A cervical fibroid is mostly benign, can be present at extremes of age, and its atypical presenting symptoms pose difficulty in diagnosis. Vaginal bleeding and retention of urine are the most common symptoms. Central and supravaginal fibroids are difficult to operate. Proper pre-operative delineation of altered anatomical structure are essential for choosing the correct modality of treatment. In large cervical fibroids, suspected malignancy before surgery and hidden cervical malignancy can coexist.

Right-to-left shunt due to iatrogenic atrial septal defect manifested by aorto-caval fistula: a case report

BackgroundAn aorto-caval fistula is a rare but critical complication of abdominal aortic aneurysm (AAA) rupture, leading to high-output heart failure and increased venous pressure. The anesthetic management of such cases, particularly when complicated by an intraoperative right-to-left shunt, is seldom reported.Case presentationA 71-year-old man with a history of atrial fibrillation and catheter ablation presented with heart failure and abdominal pain, leading to cardiac arrest. Imaging revealed an AAA rupture into the inferior vena cava. During emergency surgery, severe venous bleeding was managed using intra-aortic balloon occlusion (IABO). Transesophageal echocardiography (TEE) identified a right-to-left shunt due to an iatrogenic atrial septal defect.ConclusionEarly TEE recognition and timely IABO intervention were crucial in managing this complex case, underscoring the importance of these techniques in similar emergency scenarios.

Clinical phenotypes and genotypes of congenital fibrinogen disorder: an analysis of 16 children

To investigate the clinical phenotypes and genotypes of children with congenital fibrinogen disorder (CFD). A retrospective analysis was conducted on the clinical data of 16 children with CFD. Polymerase chain reaction was used to amplify all exons and flanking sequences of the FGA, FGB, and FGG genes, and sequencing was performed to analyze mutation characteristics. Among the 16 children, there were 9 boys (56%) and 7 girls (44%), with a median age of 4 years at the time of attending the hospital. Among these children, 9 (56%) attended the hospital due to bleeding events, and 7 (44%) were diagnosed based on preoperative examination. The children with bleeding events had a significantly lower fibrinogen activity than those without bleeding events (P<0.05). Genetic testing was conducted on 12 children and revealed a total of 12 mutations, among which there were 4 novel mutations, i.e., c.80T>C and c.1368delC in the FGA gene and c.1007T>A and C.1053C>A in the FGG gene. There were 2 cases of congenital afibrinogenemia caused by null mutations of the FGA gene, with relatively severe bleeding symptoms. There were 7 cases of congenital dysfibrinogenemia mainly caused by heterozygous missense mutations of the FGG and FGA genes, and their clinical phenotypes ranged from asymptomatic phenotype to varying degrees of bleeding. The clinical phenotypes of children with CFD are heterogeneous, and the severity of bleeding is associated with the level of fibrinogen activity, but there is a weak association between clinical phenotype and genotype.

Hepatocellular adenomas with severe intra-abdominal bleeding, related to an underlying coagulation disorder: a case report

BackgroundHepatocellular adenoma is a rare benign liver tumor. Typically, hepatocellular adenomas are solitary and are found in young women who use estrogen-containing contraceptives. The occurrence of multiple hepatocellular adenoma has been linked to higher body mass index, and as the prevalence of overweight increases, multiple hepatocellular adenomas are seen more often. An hepatocellular adenoma does not always necessitate treatment, as they can regress under conservative strategies. In incidental cases, an adenoma presents owing to bleeding, which is mostly self-limiting. If it is not, embolization of hepatic involved vessels is indicated.Case presentationIn this case report, we discuss a 42-year old Caucasian woman with multiple hepatocellular bleeds, treated by multiple endovascular procedures. After the first embolization of an adenoma in the right liver lobe, a second bleed occurred in the left lobe, necessitating additional endovascular intervention. During admittance, treatment was complicated by pulmonary embolism and a pneumonia. During follow-up, our patient was diagnosed with antiphospholipid syndrome.ConclusionHepatocellular adenoma is a rare diagnosis that requires centralized expertise. This particular case illustrates the complexity of treatment strategies for associated intra-abdominal bleeding and possible complications. Although liver adenoma is often an incidental finding, it can also result in significant morbidity. Centralization of treatment leads to expertise in managing complex treatment strategies.

Ankylosing spondylitis and heterotopic calcification: a case from the Prehispanic population of Buenavista del Norte (Tenerife, Canary Islands).

Skeletal remains of an adult (estimated age at death ≈ 27-30 years) Prehispanic male with fusion in the sacroiliac joints showed a heterotopic ossification consisting of a flat, 10 × 5 cm bone formation covering the posterior aspect of the right sacroiliac joint. The bones were recovered from a burial cave containing remains of 4 individuals, located on the side of a ravine, in Buenavista del Norte (NW corner of Tenerife, Canary Islands), at ≈ 450 m altitude. This individual was probably affected by ankylosing spondylitis (AS) that led to bony fusion of the pelvic bones with the sacrum, but several unusual features prompted us to compare the pelvis with those belonging to two modern individuals affected by AS (20th century) housed at the Department of Anatomy of the school of medicine (University of La Laguna, Tenerife). The Prehispanic individual possibly suffered a pelvic fracture that caused a hematoma which underwent ossification. Some features (periosteal reaction, formation of new foramina) may also suggest a superimposed infection. The fracture altered the pelvic structure, severely narrowing the right sciatic notch, possibly causing neural and/or vascular compromise of the lower limbs. Living in a steep mountainous environment, this individual (possibly a goatherder) probably suffered a fall, that caused the fracture complicated by a severe bleeding and pelvic deformation.

A thermal cross-linking approach to developing a reinforced elastic chitosan cryogel for hemostatic management of heavy bleeding

Uncontrolled hemorrhage stands as the primary cause of potentially preventable deaths following traumatic injuries in both civilian and military populations. Addressing this critical medical need requires the development of a hemostatic material with rapid hemostatic performance and biosafety. This work describes the engineering of a chitosan-based cryogel construct using thermo-assisted cross-linking with α-ketoglutaric acid after freeze-drying. The resulting cryogel exhibited a highly interconnected macro-porous structure with low thermal conductivity, exceptional mechanical properties, and great fluid absorption capacity. Notably, assessments using rabbit whole blood in vitro, as well as rat liver volume defect and femoral artery injury models simulating severe bleeding, showed the remarkable hemostatic performance of the chitosan cryogel. Among the cryogel variants with different chitosan molecular weights, the 150 kDa one demonstrated superior hemostatic efficacy, reducing blood loss and hemostasis time by approximately 73 % and 63 % in the hepatic model, and by around 60 % and 68 %, in the femoral artery model. Additionally, comprehensive in vitro and in vivo evaluations underscored the good biocompatibility of the chitosan cryogel. Taken together, these results strongly indicate that the designed chitosan cryogel configuration holds significant potential as a safe and rapid hemostatic material for managing severe hemorrhage.

Intraoperative severe gastric venous congestion during total pancreatectomy with replaced common hepatic artery: a case report

BackgroundGastric venous congestion (GVC) is one of the complications of total pancreatectomy (TP). Here, we report a case of intraoperative severe GVC during TP with a replaced common hepatic artery (RCHA).Case presentationA 65-year-old female patient was diagnosed with intraductal papillary mucinous carcinoma. Her CHA branched from the superior mesenteric artery as RCHA. She underwent subtotal stomach preserving TP. The tumor was resected with splenic artery (SpA) and total gastric vein transections. Severe GVC and bleeding from the stomach tube occurred intraoperatively. A strong pulsation was observed in the left gastric artery (LGA), and we suspected an increased blood flow from the celiac artery (CeA) to the LGA after SpA resection. Total gastrectomy (TG) was then performed to control the severe GVC-related bleeding. The patient was discharged without complications 19 days postoperatively.ConclusionTP with RCHA may increase the risk of severe GVC due to increased blood flow from CeA to LGA.

Can mechanical heart valves perform similarly to tissue valves? An in vitro study

Current surgical aortic valve (AV) replacement options include bioprosthetic and mechanical heart valves (MHVs), each with inherent limitations. Bioprosthetic valves offer superior hemodynamics but suffer from durability issues, typically initiating deterioration within 7–8 years. MHVs, while durable, necessitate lifelong anticoagulation therapy, presenting risks such as severe bleeding and thromboembolic events. The need for anticoagulants is caused by non-physiological flow through the hinge area during the closed phase and large spikes of regional backflow velocity (RBV) during the closing phase that produces high shear events. This study introduces the iValve, a novel MHV designed to combine the hemodynamic benefits of bioprosthetic valves with the durability of MHVs without requiring anticoagulation. The iValve features eye-like leaflets, a saddle-shaped housing, and an optimized hinge design to enhance blood flow and minimize thrombotic risk. Fabricated using 6061-T6 aluminum and polyether ether ketone (PEEK), twelve iValve iterations were evaluated for their opening and closing dynamics. The reported top-performing prototypes demonstrated competitive performance against industry standards. The proposed iValve prototype exhibited a mean RBV of −4.34 m/s with no spikes in RBV, performing similarly to bioprosthetic valves and significantly outperforming existing MHVs. The iValve’s optimized design showed a 7–10% reduction in closing time and a substantial decrease in RBV spikes, potentially reducing the need for anticoagulation therapy. This study highlights the iValve’s potential to revolutionize prosthetic heart valve technology by offering a durable, hemodynamically superior solution that mitigates the drawbacks of current MHVs.

A case of severe bleeding stomal varices in a cirrhotic patient: managed by transjular intrahepatic portosystemic shunts and obliteration of collaterals

A case of severe bleeding stomal varices in a cirrhotic patient: managed by transjular intrahepatic portosystemic shunts and obliteration of collaterals

Prevalence and characterization of anti-VWF antibodies in a population of patients with type 3 VWD

Abstractvon Willebrand disease (VWD) is an inherited bleeding disorder caused by quantitative or qualitative defects in the von Willebrand factor (VWF) protein. Type 3 VWD has a severe bleeding phenotype caused by the absence of VWF, in which treatment usually involves replacement therapy with VWF-containing products. The immune system can react to the VWF product and form anti-VWF antibodies to neutralize or clear the VWF, which can compromise efficacy of treatment or lead to anaphylaxis. Current diagnostic testing is limited to the detection of anti-VWF antibodies that neutralize VWF binding to platelets by using a ristocetin cofactor assay. We set out to develop assays to identify both neutralizing and nonneutralizing antibodies to screen, quantify, and characterize anti-VWF antibodies in samples from the Zimmerman Program, a large multicenter study of patients with VWD. We detected anti-VWF immunoglobulin G (IgG) or IgM antibodies in 18% of 49 unrelated individuals with type 3 VWD. The antibodies ranged in concentration and consisted of 33% nonneutralizing and 67% neutralizing to factor VIII, collagen III, platelet glycoprotein Ib alpha (GPIbα), and/or collagen IV binding. Of the positive type 3 VWD samples, 8 of 9 were IgG, which were further subclassified into mostly IgG1 and IgG4 antibodies. Through a series of testing methods, we identified VWF-specific antibodies in 9 unrelated individuals with type 3 VWD with varying demographics, bleeding phenotypes, and genetic variants. This anti-VWF antibody testing strategy provides a useful tool to assess risk and better navigate treatment options for patients with type 3 VWD.

A comparative study of ultrasonography versus peripheral nerve stimulator-guided obturator nerve block for transurethral resection of bladder tumor under spinal anesthesia

Background: Transurethral resection of bladder tumor (TURBT) is usually done spinal anesthesia but it does not prevent the obturator reflex leading to bladder perforation, bleeding, or incompletely resection of resection of the tumor which could be prevented by obturator nerve block (ONB) in TURBT. Aims and Objectives: The aim of our study is to compare the effectiveness of ONB by peripheral nerve stimulator (PNS) and ultrasonography (USG) in TURBT and also compare morbidity between USG- and PNS-guided ONB in TURBT under spinal anesthesia. Materials and Methods: Sixty patients were randomly divided into two groups. Group (1) patients had a PNS-guided ONB and Group (2) patients had ONB-guided ONB. The presence or absence of adductor muscles reflexes was recorded. Bladder perforation and bleeding during surgery were also recorded. Results: A success rate of 90% was achieved in the Group (2) compared to 66% in Group (1) which was clinically significant with P=0.049. Complete bladder perforation was detected in four patients in Group (1) whereas no perforation was observed in Group (2). Bleeding was observed in both groups but severe bleeding was present in five patients in Group (1) and required a blood transfusion. Conclusion: We concluded that USG-guided ONB is more efficient and safer as compared to PNS-guided ONB.