Fatal Renal Abscess Caused by Porphyromonas gingivalis and Subcapsular Hemorrhage, Japan.

A 61-year-old man in Japan with abdominal pain was suspected of having a renal tumor. Despite initial treatment, his condition rapidly deteriorated, leading to death. Postmortem examination revealed a renal abscess and sepsis caused by Porphyromonas gingivalis. This case underscores the need to consider atypical pathogens in renal masses.

Gene Therapy with Fidanacogene Elaparvovec in Adults with Hemophilia B.

BACKGROUND: Fidanacogene elaparvovec, an adeno-associated virus (AAV) gene-therapy vector for hemophilia B containing a high-activity human factor IX variant (FIX-R338L/FIX-Padua), was associated with sustained factor IX activity in a phase 1-2a study. METHODS: We conducted a phase 3 open-label study of fidanacogene elaparvovec at a dose of 5×1011 vector genome copies per kilogram of body weight. Men 18 to 65 years of age with hemophilia B and a factor IX level of 2% or less were eligible for screening if they had received at least 6 months of therapy with prophylactic factor IX concentrate. The primary end point, tested for noninferiority, was the annualized bleeding rate (treated and untreated bleeding episodes) from week 12 to month 15 after treatment with fidanacogene elaparvovec as compared with the prophylaxis lead-in period. Superiority, additional efficacy end points, and safety were also assessed. RESULTS: Of 316 men who underwent screening for the lead-in study, 204 (64.6%) were not eligible; 188 (59.5%) of those were ineligible owing to the presence of anti-AAV neutralizing antibodies. Of the 45 participants who received fidanacogene elaparvovec, 44 completed at least 15 months of follow-up. The annualized rate of bleeding for all bleeding episodes decreased by 71%, from 4.42 (95% confidence interval [CI], 1.80 to 7.05) at baseline to 1.28 (95% CI, 0.57 to 1.98) after gene therapy, a treatment difference of -3.15 episodes (95% CI, -5.46 to -0.83; P = 0.008). This result shows the noninferiority and superiority of fidanacogene elaparvovec to prophylaxis. At 15 months, the mean factor IX activity was 26.9% (median, 22.9%; range, 1.9 to 119.0) by one-stage SynthASil assay. A total of 28 participants (62%) received glucocorticoids for increased aminotransferase levels or decreased factor IX levels (or both) starting between 11 and 123 days. No infusion-related serious adverse events, thrombotic events, development of factor IX inhibitors, or malignant conditions were observed. CONCLUSIONS: Fidanacogene elaparvovec was superior to prophylaxis for the treatment of participants with hemophilia B, leading to reduced bleeding and stable factor IX expression. (Funded by Pfizer; BENEGENE-2 ClinicalTrials.gov number, NCT03861273.).

Successful Conservative Treatment of Maternal Spontaneous Unilateral Adrenal Hemorrhage Causing Severe Anemia in the Third Trimester of Pregnancy-A Case Report.

We present the case of a 32-year-old pregnant woman in the 32 + 3 weeks of pregnancy who presented to the hospital with an exacerbation of pain in the right flank. The diagnostic evaluation revealed the presence of severe anemia and a spontaneous adrenal hemorrhage (SAH) in the right adrenal gland. The patient was transferred to the Perinatology, Obstetrics and Gynecology Clinic with the intention of undergoing preterm childbirth. However, the doctors made a risky decision to wait until week 37 and to terminate the pregnancy at that point. The decision was right, as a cesarean section was performed without complications, and the patient gave birth to a healthy child. Spontaneous adrenal hemorrhage (SAH) is a rare condition, defined as spontaneous hemorrhage without trauma or anticoagulant therapy. Due to bleeding and damage to the adrenal cortex, SAH can lead to adrenal insufficiency. Because of its non-specific symptoms and potentially fatal outcomes for the patient and fetus, it should be considered during diagnostics.

Primary robot-assisted laparoscopic partial nephrectomy for hemorrhage secondary to angiomyolipoma: a retrospective study from a large tertiary hospital in China.

With the continuous development of robot-assisted technology, Robot-assisted Laparoscopic Partial Nephrectomy (RALPN) has gradually become an optional method for the treatment of Hemorrhage secondary to angiomyolipoma (HSA). However, there are rare clinical reports of the primary RALPN for HSA. Therefore, this research aims to evaluate the efficacy and safety of primary RALPN for HSA. Fourteen patients(six males and eight females), aged 14-56 years, underwent primary RALPN for HSA and were retrospectively analyzed from 2015 to 2023. The initial blood routine examination revealed decreased hemoglobin in all patients, and Contrast-enhanced computed tomography (CT) indicated retroperitoneal hematoma. After correcting shock and electrolyte imbalance through fluid therapy and medical treatment, all primary RALPN procedures were performed with transabdominal access on the side of the Hemorrhage. After tumor resection and hematoma removal with a monopolar Curved Scissor, the absorbable barbed suture was performed for inner and outer running stitches, respectively. Patient demographic information, perioperative characteristics, and functional outcomes were collected and analyzed. The initial tumor size of fourteen patients ranged from 57 to 145 mm, and the RENAL ranged from 7 to 11. All of the HSA was controlled, and primary RALPN was successful. The operating time it was ranged from 105 to 265 min. Postoperatively, one patient exhibited chylous drainage (Clavien-Dindo II), and another patient developed pleural effusion (Clavien-Dindo III). No postoperative transfusion and Digital Subtraction Angiography (DSA) highly selective embolization of the bleeding vessel was needed. No patients developed urinoma or urinary fistula. Within the follow-up period, the overall complications were manageable. Primary RALPN is a safe and effective procedure for HSA, which may be considered an alternative to selective renal artery embolization.

How Protein Depletion Balances Thrombosis and Bleeding Risk in the Context of Platelet's Activatory and Negative Signaling.

Platelets are small cell fragments that play a crucial role in hemostasis, requiring fast response times and fine signaling pathway regulation. For this regulation, platelets require a balance between two pathway types: the activatory and negative signaling pathways. Activatory signaling mediators are positive responses that enhance stimuli initiated by a receptor in the platelet membrane. Negative signaling regulates and controls the responses downstream of the same receptors to roll back or even avoid spontaneous thrombotic events. Several blood-related pathologies can be observed when these processes are unregulated, such as massive bleeding in activatory signaling inhibition or thrombotic events for negative signaling inhibition. The study of each protein and metabolite in isolation does not help to understand the role of the protein or how it can be contrasted; however, understanding the balance between active and negative signaling could help develop effective therapies to prevent thrombotic events and bleeding disorders.

Development of a bleeding arteriovenous fistula task trainer.

BACKGROUND: As end-stage renal disease becomes more prevalent in the United States, the number of Americans with arteriovenous (AV) fistulas continues to increase. One of the most feared complications of AV fistulas is life-threatening hemorrhage, as patients can exsanguinate within minutes. OBJECTIVES: As frontline healthcare workers, emergency medicine (EM) providers need to be able to provide rapid and effective treatment for this rare presentation. We developed a task trainer model to simulate AV fistula hemorrhage to prepare and train EM residents. METHODS: This task trainer model was constructed with readily available materials and takes about 30 minutes to make. Twenty-one EM residents participated in the training session. The session consisted of a brief didactic on AV fistula hemorrhage control followed by hands on usage of the task-trainer model. The participants filled out an anonymous survey afterwards rating the model. RESULTS: Residents completed anonymous postcourse surveys rating the session on a five-point Likert scale. Both the overall teaching session and the task trainer were rated very highly. Compared to precourse ratings, residents reported statistically significant postcourse improvements in their level of confidence in managing AV fistula hemorrhage. CONCLUSIONS: To our knowledge, this is the first published task trainer model to simulate a bleeding AV fistula for EM residents. The model was well received by our trainees, is relatively inexpensive, and made from easily sourced materials. We believe this model can be used for trainees of all disciplines to prepare them for this potentially catastrophic patient presentation.

Neonatal cardiorespiratory failure caused by splenic hemorrhages: a case report on a fatal and a rescued case.

Two cases of neonatal splenic hemorrhage with acute cardiorespiratory failure are described in this report. The first case involves a full-term neonate who was found unresponsive without any witnesses and could not be successfully resuscitated. A postmortem diagnosis revealed a splenic hemorrhage. Second case is an extremely premature neonate who experienced a witnessed cardiovascular collapse on the 14th day of life. Rapid cardiovascular support was administered, resulting in a positive outcome. While splenic hemorrhage is commonly associated with traumatic events, these cases highlight the need of considering spontaneous splenic hemorrhages as a potential cause of acute neonatal compromise, even in the absence of birth-related trauma (e.g., asphyxia, prolonged labor, clavicle fractures, brachial plexus injuries). This report emphasizes the importance of including splenic hemorrhage timely in the differential diagnosis of neonatal cardiorespiratory instability, especially in the absence of more common diagnoses, and discusses the challenges associated with its recognition and treatment.

Spontaneous hepatic hemorrhage as presentation of metastasized papillary thyroid carcinoma: a case report.

BACKGROUND: Spontaneous hepatic hemorrhage is a rare condition, most commonly diagnosed in patients with hepatocellular carcinoma or hepatic adenomas, and is seldom caused by metastatic disease. In this case report, we present a patient with spontaneous hepatic hemorrhage due to hepatic metastasis of papillary thyroid carcinoma, an exceptionally rare occurrence. CASE PRESENTATION: The patient was a 77-year-old white male with a history of atrial fibrillation treated with apixaban. He presented at a local hospital with abdominal pain and nausea. A CT scan revealed a hepatic lesion in segment 3 with an adjacent hematoma. He was referred to our tertiary center and treated conservatively. Further evaluation revealed an intrathoracic goiter containing a tumorous process diagnosed as a papillary thyroid carcinoma (PTC), and the patient subsequently underwent thyroidectomy. A biopsy of the hepatic lesion confirmed it as a PTC metastasis. Due to worsening abdominal pain and anorexia, the patient underwent subacute hepatic segmental resection. Postoperatively, he developed iodine-refractory disease with disseminated metastasis and passed away 22 months after the initial admission. CONCLUSIONS: To our knowledge, this is the first recorded case of metastasized papillary thyroid carcinoma presenting with spontaneous hepatic hemorrhage-adding to the list of rare causes for this condition.

An Unusual Presentation of Fatal Acute Haemorrhagic Pancreatitis in a Young Adult.

Acute haemorrhagic pancreatitis is a medical emergency and the most severe form of Acute Pancreatitis. It is characterized by severe epigastric pain that radiates to the back and is associated with vomiting. If not diagnosed and managed promptly, it may result in sudden, unexpected, unexplained deaths which fall within the medicolegal domain. In such cases, the role of an autopsy is of paramount importance to determine the cause of death. Here we report a young adult, who presented to the local hospital with vague abdominal discomfort and vomiting following alcohol intake and referred to our tertiary care center for further management. But he was received dead on arrival at our hospital. The diagnosis of acute haemorrhagic pancreatitis was made only after the post mortem examination. Awareness of the physicians about the unusual symptoms in acute haemorrhagic pancreatitis and the need for pancreas examination at autopsy of all sudden deaths is emphasised.

The (reverse) paradox of lupus anticoagulant: A case report.

INTRODUCTION: Systemic Lupus Erythematosus (SLE) is often associated with antiphospholipid syndrome (APS), which manifests as recurrent thrombotic events or obstetric complications in presence of antiphospholipid antibodies. Hereby we present a case of a child who presented with low grade fever, superficial thrombophlebitis with mucosal bleeding and was diagnosed as Lupus Anticoagulant Hypoprothrombonemia Syndrome (LAHS). CASE: A 7-year-old girl was hositalized with complaints of fever and spontaneous bleeding from gums and epistaxis. On examination, she had multiple small tender nodular lesions with greenish hue of overlying skin suggesting superficial thrombophlebitis and mild non-tender hepatosplenomegaly. Her coagulogram revealed normal platelet counts and deranged PT and APTT. ESR and CRP were raised. Serology for viral infections, blood and urine cultures were negative. Patient had persistent coagulopathy, mucosal bleeding and low-grade fever despite supportive treatment. She was tested for anti-nuclear antibodies (ANA) in view of suspicion of autoimmune process. ANA was positive in high titer with speckled pattern on indirect immunofluorescence. Mixing studies showed correction of PT and non-correction of APTT. PT based factors were normal except for prothrombin (FII) which was low and remained low despite dilution. APTT based factors (FVIII and FIX) were low but corrected on dilution. This was suggestive of prothrombin deficiency and a presence of a nonspecific inhibitor of APTT pathway (likely lupus anticoagulant). Presence of antiprothrombin antibodies established the diagnosis of LAHS. ENA profile was positive for SmD1, Ro60 and Ku. Complement levels were low. Direct Coomb's test was positive but there was no evidence of hemolysis. Lupus anticoagulant by DRVVT and anti-cardiolipin antibodies by ELISA were positive. Patient was diagnosed as Systemic Lupus Erythematosus with Lupus Anticoagulant Hypoprothrombinemia Syndrome. She was treated with IV methylprednisolone. Patient showed significant improvement in form of resolution of fever, mucosal bleeding, correction of deranged INR and reversal of hypocomplementemia. She was discharged on hydroxychloroquine, mycophenolate mofetil and tapering doses of prednisolone. On follow up, child was doing well and her prothrombin time and complement levels had normalized. Low dose aspirin was aspirin was added for thromboprophylaxis.

Relationship between body mass index and clinical events in patients with atrial fibrillation undergoing percutaneous coronary intervention.

BACKGROUND: It is still unclear whether body mass index (BMI) affects bleeding and cardiovascular events in patients requiring oral anticoagulants (OAC) for atrial fibrillation (AF) and antiplatelet agents after percutaneous coronary intervention (PCI) for coronary artery disease (CAD). The aim of this study was to evaluate the relationship between BMI and clinical events in patients who underwent PCI under OAC therapy for AF. METHOD: This was a multicenter, observational cohort study conducted at 15 institutions in Japan. AF patients who underwent PCI with drug-eluting stents for CAD were retrospectively and prospectively included. Patients were divided into the Group 1 (BMI <21.3 kg/m2) and the Group 2 (BMI ≥21.3 kg/m2) according to the first-quartile value of BMI. The primary endpoint was net adverse clinical events (NACE), a composite of major adverse cardiovascular events (MACE) and major bleeding events within one year after index PCI procedure. RESULTS: In the 720 patients, 180 patients (25.0%) had BMI value <21.3 kg/m2. While the rates of NACE and MACE were significantly higher in the Group 1 than the counterpart (21.1% vs. 11.9%, p = 0.003 and 17.2% vs. 8.9%, p = 0.004), that of major bleeding did not differ significantly between the 2 groups (5.6% vs. 4.3%, p = 0.54). The cumulative rate of NACE and MACE was significantly higher in the Group 1 than the Group 2 (both log-rank p = 0.002), although that of major bleeding events was equivalent between the 2 groups (log-rank p = 0.41). In multivariable Cox regression analyses, while BMI value <21.3 kg/m2 was not associated with major bleeding events, that cut-off value was an independent predictor for increased NACE and MACE. CONCLUSIONS: Among the patients undergoing PCI for CAD and requiring OAC for AF, BMI value was a useful indicator to predict major adverse clinical events.

Acute respiratory distress syndrome as the initial symptom of hepatic angiosarcoma with Kasabach-Merritt syndrome: A case report.

RATIONALE: Hepatic angiosarcoma (HSA) has a poor prognosis. Our understanding of its clinical features, diagnosis, treatment, and prognosis remains limited. In certain cases, vascular tumors such as HSA can induce bleeding complications due to thrombocytopenia, known as Kasabach-Merritt syndrome (KMS). When KMS symptoms occur in the lungs, its clinical manifestations closely resemble those of ARDS, leading to misdiagnosis and poor outcomes. Unfortunately, this condition is extremely rare and there is a lack of relevant case reports, which further adds to the difficulty of its diagnosis and treatment. PATIENT CONCERNS: This case report describes a patient who initially presented with symptoms of ARDS. Due to the unique nature of these symptoms, the patient underwent a complex diagnostic and treatment process before finally being diagnosed with HSA complicated by KMS through pathological examination. DIAGNOSES: The patient was eventually diagnosed with HSA by pathology and KMS with multiorgan hemorrhage. INTERVENTIONS: Highly misleading clinical manifestations were recorded during the diagnosis and treatment, which, to our knowledge, have not been previously reported. OUTCOMES: The patient died from a massive pulmonary hemorrhage. LESSONS: Dysfunction of a single organ or system may be the external manifestation of a multi-system clinical disease. Therefore, in the clinical diagnosis and treatment process, especially during early diagnosis, while it is important to focus on the primary or typical clinical symptoms, it is equally crucial not to underestimate or ignore accompanying symptoms that lack specificity. When diagnosis and treatment reach an impasse, these "atypical" symptoms often prove to be key in solving the puzzle.

Association of bullous pemphigoid with acquired hemophilia A: a case report.

Acquired hemophilia A, a rare condition resulting in spontaneous bleeding without prior bleeding disorders, arises due to autoantibody-mediated inhibition of coagulation factor VIII and is typically associated with autoimmune, neoplastic, drug, or obstetric factors. We present the case of a 31-year-old woman with bullous pemphigoid, managed with corticosteroids since 2013, who presented spontaneous hemorrhagic manifestations. Upon admission, laboratory tests revealed hypochromic microcytic anemia, prolonged activated partial thromboplastin time, and a factor VIII level < 1%, indicative of acquired hemophilia A. Further assessments showed elevated Ristocetin cofactor activity, von Willebrand factor antigen, and a factor VIII inhibitor level of 665 BU. This underscores the importance of considering acquired hemophilia A in autoimmune dermatological conditions like bullous pemphigoid, highlighting the association between autoimmune disorders and coagulation abnormalities, particularly in cases of spontaneous hemorrhagic events.

Association of office blood pressure with ischemic and bleeding events in patients undergoing percutaneous coronary intervention.

Optimal blood pressure (BP) for patients with coronary artery disease (CAD) undergoing percutaneous coronary intervention (PCI) remains unclear. This study aims to identify the optimal BP by investigating the association between average office BP and future clinical events in patients undergoing PCI. Consecutive patients undergoing PCI from 2012 to 2016 were included. They were divided into five groups according to the average follow-up BP after discharge. The co-primary outcomes were net adverse clinical events (NACE) and major adverse cardiac and cerebrovascular events (MACCE) up to 5 years. NACE was defined as a composite of MACCE (all-cause death, non-fatal myocardial infarction (MI), non-fatal stroke, or any revascularization) or major bleeding. A total of 2845 patients were included, and among them, 787 (27.7%) experienced the NACE during the follow-up period. Patients in the highest SBP group (adjusted hazard ratio [HR] 1.495, confidence interval [CI] 1.189-1.880) and lowest SBP group (adjusted HR 1.625, CI 1.214-2.176) had a significantly higher risk of 5-year NACE. Similar associations were observed between SBP and the risk of MACCE, and similar results based on DBP categories were also observed. There was a J-curve relationship between SBP and DBP with respect to 5-year NACE and MACCE. The nadir point of risk for NACE and MACCE was found at 121.4/74.8 and 120.4/73.7 mmHg. In patients underwent PCI, there is a significant correlation between office BP level and clinical events, indicates the importance of efforts for optimal BP control to reduce ischemic and bleeding events.Trial registration: HanYang University Medical Center (HYUMC) Registry, NCT05935397.

External validation of the MiGUTS nomogram for the prediction of bleeding control intervention after renal trauma.

INTRODUCTION: The American Association for the Surgery of Trauma (AAST) renal trauma grading leads to a variable management of patients with high-grade renal injuries. For a better prediction of the risk for bleeding interventions, Keihani et al. introduced the multi-institutional genito-urinary trauma study (MiGUTS) renal trauma nomogram in 2019. The aim of this study was to conduct an external validation and generalization for all kidney trauma cases of the nomogram with a European cohort of a Swiss level 1 trauma center. METHODS: We collected data from the clinical information system of the Kantonsspital Graubünden, Chur, Switzerland. All patients ≥ 18 years of age from 01.01.2008 to 01.12.2020 with a renal trauma who underwent computed tomography imaging of the abdomen were included. The descriptive analysis was performed by a t-test/Wilcoxon signed-rank test and a Chi-square test. The predictions of the nomogram were analysed by the Pearson correlation coefficient. The threshold of prediction of a bleeding intervention was optimized by a ROC analysis. RESULTS: Overall, 166 patients were included. Most patients were male (80.7%) with a median age of 44 years. Using the prediction from the MiGUTS nomogram developed by Keihani et al. we were able to identify a threshold with a sensitivity of 1.00, specificity of 0.87, positive predictive value of 0.44, negative predictive value of 1.00 and accuracy of 0.88. CONCLUSION: The MiGUTS nomogram by Keihani et al. demonstrated to be reliable in the prediction of an intervention for bleeding control in our validation study in a European cohort.

The influence of the interventionalist's sex on the outcome and complications of transbronchial lung cryobiopsy.

BACKGROUND: To date there are no data on sex aspects evaluating outcomes of interventional pneumology (IP). Our aim was to investigate sex differences in transbronchial lung cryobiopsy (TBLC) outcomes in the diagnosis of interstitial lung disease (ILD). METHODS: All consecutive (TBLC)s performed for ILD evaluation between Nov 17 and Dec 21 at a tertiary referral center for ILDs and IP were analyzed. The indication for the procedure was determined by a multidisciplinary discussion (MDD). Final results including bronchoalveolar lavage (BAL) and histology were discussed in a 2nd MDD and outcomes and procedure related complications were assessed. RESULTS: TBLC was performed in 406 patients (38.4 % female/67.8 years/FVC 76.8 %). Among 32 interventionalists, 16 females performed 53 % of interventions. Females had longer procedure times (29.9 vs. 26.6 min, p = 0.046), used fluoroscopy more often (76.7 vs. 50.3 %, p < 0.001) and obtained more samples (3.6 vs. 3.2, p = 0.021) than their male counterparts. No difference was found for major bleeding or pneumothorax. MDD was able to conclude on a diagnosis in 88.4 % of interventions performed by women and in 78.5 % performed by men (p = 0.010). In a multivariate analysis, female gender (OR 1.93) and lower FVC% values (OR 0.98) were significantly associated with diagnostic yield, whereas the number of biopsies, professional experience, use of fluoroscopy or antiplatelet drugs were not relevant. CONCLUSION: The results of this study strengthen the role of women in endoscopy and may help to motivate women to pursue a career in IP.

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.

Procedure-Related Complication Rates With the Use of Vascular Closure Devices; Does Size Only Matter? A Large Single Centre Retrospective Study.

INTRODUCTION: Our retrospective study aimed at assessing safety of vascular closure devices (VCDs) used in a large single-centre Interventional Radiology (IR) department. Complication and deployment failure rates using collagen-based (Angio-seal) and suture-based (ProGlide) devices for common femoral artery haemostasis were compared. MATERIALS AND METHODS: Data from VCDs deployed over a 6-year period were retrospectively analysed for patient age, procedure indication, puncture mode (antegrade/retrograde), sheath size, deployment failure and complications (haematoma, pseudoaneurysm formation, limb occlusion). Numerical and statistical analysis was undertaken. RESULTS: Overall, 1321 common femoral artery punctures in 1217 patients were closed using VCDs. Failure rate using ProGlide was significantly higher when compared with Angio-seal (P=<0.001) in sheath sizes ≤8 Fr. Heparin was not administered in embolisation procedures compared with angioplasty with or without stenting. Therefore, haematoma tended to occur more frequently following angioplasty without stenting (P = 0.003) and angioplasty with stenting (P = 0.001), when compared with embolisation. Deployment failure occurred more frequently when heparin was used during the procedure (P = 0.005). CONCLUSION: Although complications relating to sheath size are well established in the literature, there remains a paucity of data assessing the impact of procedure specific factors when comparing VCDs. Our study challenges that size is the sole determinant of VCD success and invites a more holistic view of VCD deployment strategies. This study advocates continued research into the nuances of other potential confounding variables to optimise patient outcomes.

Diffuse alveolar hemorrhage in patients with systemic lupus erythematosus: data from the Spanish society of rheumathology Lupus Register (RELESSER).

INTRODUCTION:  Diffuse alveolar hemorrhage (DAH) is a rare complication with high mortality in patients with systemic lupus erythematosus (SLE). Early diagnosis and treatment are essential to improve patient prognosis. To determine the characteristics of patients with DAH and their mortality in a Spanish cohort of patients with SLE. METHODS:  Patients from the RELESSER (Spanish Society of Rheumatology Lupus Register) who had had at least one confirmed episode of DAH were included. Epidemiological, clinical, and laboratory characteristics were analyzed. RESULTS:  4024 patients were included in the RELESSER register, 37 (0.9%), had at least one recorded episode of DAH. Only further data for 14 patients could be analyzed. In total, 92.9% were women, and for 4 (28.6%) DAH coincided with the debut of SLE. More than 80% of patients had renal involvement and thrombocytopenia. The most frequent manifestations were dyspnea (85.7%) and hypoxemia (100%), with the classic triad of hemoptysis, anemia and pulmonary infiltrates, appearing in 6 (46.2%) patients. The most frequently used treatments were glucocorticoids (85.7%) and cyclophosphamide (69.2%); plasmapheresis was utilized in 5 patients (35.7%) and 8, (57.1%) received intravenous immunoglobulins; 12 (85.7%) patients required admission to the ICU and 5 (35.7%) died. Tobacco use, history of lupus nephritis (LN), concomitant infection, and treatment with cyclophosphamide were more frequent in patients who died. CONCLUSIONS:  DAH is rare in patients with SLE; in up to one-third of patients, it may appear at the onset of the disease. Some factors, such as smoking, a history of LN, treatment with cyclophosphamide, or concomitant infection, are more prevalent in patients with an unfavorable outcome.