Two cases of Factor XI deficiency: Use of Thrombin Generation Assays (TGA) to detect a non-bleeding phenotype.

Factor XI deficiency is a rare disorder of hemostasis. Previously also known as "hemophilia C", this defect has been regarded as a risk factor for bleeding. However, it has been known for long that bleeding tendency and severity of bleeding are not related to the residual factor XI activity in symptomatic patients. Moreover, a large proportion of patients with even severe factor XI deficiency are clinically unremarkable and do not show any signs of abnormal bleeding. Here, we present two cases of factor XI deficiency with a non-bleeding phenotype. Adequate diagnostic work-up and evaluation of the bleeding risk are reported and discussed with focus on thrombin generation assays (TGA) for the prediction of bleeding in affected patients. This is of high relevance in affected patients, particularly in the context of surgery.

Concomitant hypofibrinogenemia and factor XI deficiency as rare cause of bleeding during urgent dentistry: case report and short review of the literature.

Hypofibrinogenemia and Factor XI deficiency are rare defects of hemostasis, potentially leading to spontaneous bleeding manifestations and increased bleeding risk during surgery, dentistry, and interventions. Due to the different mode of inheritance, the concomitance of both defects is extremely rare and the clinical management of combined hypofibrinogenemia and factor XI deficiency is not standardized. Here, we report a rare case of concomitant genetically determined hypofibrinogenemia and factor XI deficiency as a cause of increased spontaneous bleeding and bleeding complications during dentistry. The diagnostic procedure including screening assays, single clotting factor determinations, genetic analyses, and also use of thrombin generation assays (TGA) are described. Also, we present our considerations regarding the development of an adequate prophylaxis of bleeding with fibrinogen concentrate in this case. The literature regarding the issue is briefly discussed.

[Anticoagulation after heart valve replacement]. / Antikoagulation nach Herzklappenersatz.

In patients after mechanical heart valve replacement anticoagulation is required for the prevention of thrombotic and thromboembolic events. In this setting oral anticoagulation can only be performed with vitamin K antagonists (VKA), while currently all available non-vitamin K dependent oral anticoagulants (NOAC) are contraindicated in patients with mechanical heart valve replacement. This review deals with anticoagulation in patients with mechanical heart valve replacement as well as coagulation inhibition after bioprosthetic or percutaneous valve replacement. In addition, recommendations are given for antithrombotic medication in patients with mechanical heart valve replacement in various clinical scenarios.

[Acquired inhibitory body hemophilia : A life-threatening reason for the tendency of hematoma-genesis.] / Erworbene Hemmkörperhämophilie : Eine lebensbedrohliche Ursache der Hämatomneigung.

Here, we report a rare case of acquired hemophilia which was diagnosed months after development of spontaneous hematoma. Clinical symptoms, diagnosis, and treatment are briefly summarized. Acquired hemophilia is a rare potentially life-threatening acquired bleeding disorders which should be considered in patients with acquired spontaneous hematoma.

Acquired hemophilia as a rare cause of excessive bleeding during dentistry: report of two cases and short review.

Acquired hemophilia is a rare coagulation disorder that is not diagnosed by routine clinical laboratory tests. Thus, many perioperative or acute emergent bleeding complications remain unclear until the underlying cause is specified. We report two cases of postoperative bleeding in the context of dental surgery in which subsequent acquired hemophilia could be confirmed and present a short review from the literature.

[Hematoma in lipedema: from cutaneous origin or defect of coagulation?] / Hämatomneigung bei Lipödem: Kutane Genese oder Gerinnungsdefekt?

Lipedema is a widespread in concern of etiology partially unknown disease especially in women. In many cases it is accompanied by bleeding complications. Our current work focuses on possible coagulation disorders as potential sources of such bleeding complications. Since only a minority of our patients showed a coagulation defect it is suggestive that the main underlying reason for bleeding in lipedema is of cutaneous origin what may only be forwarded by simultaneously existing coagulation disorders.

Cardiac stress after electroconvulsive therapy and spontaneous generalized convulsive seizures: A prospective echocardiographic and blood biomarker study.

AIM: Knowledge about cardiac stress related to seizures in electroconvulsive therapy (ECT) and spontaneously occurring generalized convulsive seizures (GCS) is limited. The aim of the present study was to analyze cardiac function and circulating markers of cardiac stress in the early postictal period after ECT and GCS. METHODS: Patients undergoing ECT in the Department of Psychiatry, Psychotherapy and Psychosomatics and patients undergoing diagnostic video-EEG monitoring (VEM) in the Department of Neurology were prospectively enrolled between November 2017 and November 2018. Cardiac function was examined twice using transthoracic echocardiography within 60 min and >4 h after ECT or GCS. Established blood markers (troponin T high-sensitive, N-terminal pro brain natriuretic peptide) of cardiac stress or injury were collected within 30 min, 4 to 6 h, and 24 h after ECT or GCS. In the ECT group, the troponin T values were also correlated with periprocedural heart rate and blood pressure values. Because of organizational or technical reasons, the measurement was not performed in all patients. RESULTS: Twenty patients undergoing ECT and 6 patients with epilepsy with a GCS during VEM were included. Postictal echocardiography showed no wall motion disorders and no change in left ventricular and right ventricular functions. Four of 17 patients displayed a transient increase in high-sensitive cardiac troponin T 4-6 h after the seizure (3 patients with ECT-induced seizure). None of these 4 patients had signs of an acute cardiac event, and periprocedural blood pressure or heart rate peaks during ECT did not significantly differ in patients with and without troponin T elevation. CONCLUSIONS: Signs of mild cardiac stress can occur in some patients following ECT or GCS without clinical complications, probably related to excessive catecholamine release during the seizure.

[Oral anticoagulation in atrial fibrillation: differential therapy with non vitamin K antagonist oral anticoagulants (NOAC) and vitamin K antagonists (VKA)]. / Orale Antikoagulation bei Vorhofflimmern : Differentialtherapie mit Nicht-Vitamin-K-abhängigen oralen Antikoagulanzien (NOAK) und Vitamin-K-Antagonisten (VKA).

BACKGROUND: Non-vitamin K-dependent oral anticoagulants (NOAC) have changed the management of patients with oral anticoagulation. This raises the question of which patients should preferably be anticoagulated with NOAC and which preferably with vitamin K antagonists (VKA). This discussion has so far been insufficiently conducted and often decided on a flat-rate basis in favor of the NOAC. METHOD: To clarify the question owhich form of anticoagulation - NOAC or VKA - is the best choice for patients with atrial fibrillation, an interdisciplinary team of experts met. RESULTS AND CONCLUSIONS: The experts discussed essential practical aspects of NOAC and VKA therapy. Based on typical clinical scenarios, they developed assistance, comments and tips on the differentiated use of oral anticoagulants in patients with atrial fibrillation. A criteria served amongst others practicability in daily medical practice, contraindications, side effects and interactions, but also the patient's desire. The advantages and disadvantages of therapy with VKA and NOAC were summarized in a table.

[Risk factors for hypertensive and cerebral amyloid angiopathy associated intracerebral hemorrhage: a retrospective comparison]. / Risikofaktoren für hypertensive und zerebrale Amyloidangiopathie-assoziierte intracerebrale Blutungen: ein retrospektiver Vergleich.

INTRODUCTION: The aim of this study was to compare possible risk factors for the most common forms of spontaneous intracerebral hemorrhage (ICH), namely hypertensive and cerebral amyloid angiopathy (CAA) associated ICH. METHODS: Retrospectively, different parameters and factors were compared in patients with hypertensive ICH (n = 141) and patients with a CAAassociated ICH (n = 95). These included age, INR value and blood pressure at admission, cardiovascular risk factors as well as pre-medication. The Chi-square test with the Yates' continuity correction and the t-test were used as test methods. RESULTS: Patients of the group with CAA-associated ICH were significantly older than patients with a hypertensive ICH (p = 0.001). In addition, there was a significantly higher incidence of acetylsalicylic acid prior treatment (p = 0.042) and a previous stroke (p = 0.048) in the CAA patients. Patients of both groups had a high proportion of arterial hypertension as pre-diagnosis, which was significantly more common in patients with hypertensive ICH (p < 0,001). Patients with a hypertensive ICH also had significantly higher systolic and diastolic blood pressure values (p < 0.001) and higher INR values (p = 0.005) at admission. A subgroup analysis of all patients without anticoagulation (ZAA group: n = 78, hypertensive ICB group: n = 99) showed similar results. However, there was no significant difference (p = 0.037) for a previous stroke, but there was a significant difference in premedication with a statin (p = 0.032). DISCUSSION: Arterial hypertension is a relevant risk factor in both forms of intracerebral hemorrhage and should therefore receive adequate prophylaxis. For a more detailed classification of the other risk factors, further studies with larger cases are necessary.

Early postictal serum lactate concentrations are superior to serum creatine kinase concentrations in distinguishing generalized tonic-clonic seizures from syncopes.

Concentrations of serum creatine kinase (CK) and serum lactate are frequently measured to help differentiate between generalized tonic-clonic seizures (GTCS) and syncope. The aim of this prospective cohort study was to systematically compare these two markers. The primary outcome is the measurement of serum lactate and CK in blood samples drawn within 2 h of the event in patients admitted with either a GTCS (n = 49) or a syncope (n = 36). Furthermore, the specificity and sensitivity of serum lactate and CK are determined as diagnostic markers in distinguishing between GTCS and syncope. GTCS patients have significantly higher serum lactate levels compared to syncope patients (p < 0.001). In contrast, CK does not differ between groups at admission. Regarding the first hour after the seizure, we identify a cut-off for serum lactate of 2.45 mmol/l for diagnosing GTCS as the cause of an impairment of consciousness with a sensitivity of 0.94 and a specificity of 0.93 (AUC: 0.97; 95% CI 0.94-1.0). In the second hour after the event, the ROC analysis yields similar results (AUC: 0.94; 95% CI 0.85-1.0). Serum lactate is a sensitive and specific diagnostic marker to discriminate GTCS from syncope and is superior to CK early after admission to the emergency department.

[Oral anticoagulation using coumarins - an update]. / Orale Antikoagulation mit Vitamin K-Antagonisten ­ ein Update.

Vitamin K antagonists (VKA) are used for the prophylaxis and treatment of thrombotic and thromboembolic events. Most important indications are venous thrombosis and pulmonary embolism, atrial fibrillation, and mechanical heart-valve replacement. Hence they are from high relevance for the neurologist and his daily praxis in cardio-neurological routine. Here, we give an overview about VKA with focus on mode of action, indications and efficacy, practical aspects of treatment, side effects, and monitoring.

An unusual agent for an unusual localization of infective endocarditis.

We report on a 32-year-old male patient with acute left-hemispheric stroke caused by embolism due to infective endocarditis affected from the HACEK group. Additionally, atypical findings from the transesophageal echocardiography (TEE) which showed fluttering structures belonging to the papillary muscle could be proven as infectious agents with the help of a glucose positron emission tomography (PET) scan. TEE controls showed increasing vegetation involving the mitral valve so that surgery became necessary. The current work reflects, in detail, the emergent clinical course of this young patient, suffering from both an unusual localization and an infrequent cause of endocarditis and focuses on an actual view to the literature.

[Health care economic guidance in Germany from the example Morbi-RSA]. / Gesundheitsökonomische Steuerung in Deutschland am Beispiel des morbiditätsorientierten Risikostrukturausgleichs (Morbi-RSA).

Increasing costs in health care represent still a major challenge in most industrial contries. A lot of attempts especially in Germany have been made to manage such problems and for a fair allocation oft he underlying resources. One of this ist the Morbi-RSA. The current review reflects all historical, medical and economical aspects of the Morbi-RSA and gives a perspective to possible future developments.

Cardiovascular causes of emergency neurology presenting to an ICU.

Stroke or transient ischemic attacks (TIA) represent an urgent clinical entity that is not limited only to elderly patients. The underlying causes of stroke and TIA are diverse, with those of cardiovascular origin being among the most prominent. This review seeks to elucidate some of the most important aspects of the disease in the context of emergency and critical care practice.

Severe ARDS induced by fusobacterial infections: a rare clinical presentation of Lemierre syndrome.

Acute respiratory distress syndrome (ARDS) poses a major challenge in intensive care settings. The main underlying causes of ARDS are trauma, pancreatitis, and pulmonary manifestation of systemic inflammatory response syndrome/sepsis.Lemierre syndrome represents a nearly forgotten entity arising from oropharyngeal infections with Fusobacterial species, and it is of renewed and increasing interest because of evolving antibiotic resistances.We report two cases of young female patients afflicted by Lemierre syndrome with additional severe ARDS and present an overview of the current literature.