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Ethylmalonic encephalopathy
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
Dilated tortuous retinal vessels (Figures 102.11 and 102.12) may be seen as early as three to four months of life. Hematuria may be observed and erythrocytes were reported in the cerebrospinal fluid (CSF) [5, 7, 11]. An association with nephrotic syndrome has been previously reported [12], and we have encountered a case with an episode of nephrosis which was responsive to conventional steroid treatment. One patient had a terminal hemoperitoneum [7]. Biopsies of the skin lesions showed nothing but hemorrhage [7]. There was no evidence for an immunologic abnormality, nor were there abnormalities of bleeding, clotting, or platelets. A markedly elevated level of plasminogen activator inhibitor-1 has been encountered [9]. Terminal events in two patients appeared to be pulmonary edema and one had cerebral edema.
Plasminogen Activator Enzymes and Inhibitors in Epithelial Biology and Pathology
Published in Pia Glas-Greenwalt, Fibrinolysis in Disease Molecular and Hemovascular Aspects of Fibrinolysis, 2019
B. Lyons-Giordano, E.M. Spiers, P.J. Jensen, D.J. Loskutoff, G.S. Lazarus
Plasminogen activators are serine proteases that catalyze the conversion of plasminogen to plasmin.1-4 Two types of plasminogen activator enzymes have been identified: urokinase-type (u-PA) and tissue-type (t-PA) plasminogen activators. These enzymes are encoded by separate genes and exhibit distinct immunologic and functional properties. Plasmin, the product of plasminogen activation, is a broadly active serine protease that degrades a diversity of proteins and converts latent metalloproteases to their active forms. Thus, the process of plasminogen activation initiates a cascade of proteolytic activity.
Acquired Bleeding Disorders Associated with the Character of the Surgery
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
William A. Rock, Robert F. Baugh
Excessive bleeding in urological surgery is frequently confined to prostate surgery. Occasionally, renal surgery or renal trauma can result in life-threatening hemorrhage. In both cases the operative site which is actively bleeding forms clots which are in turn lysed by the urokinase in urine. Urokinase is a potent plasminogen activator, which converts plasminogen to plasmin, the active enzyme. Bleeding continues as all clots are lysed. The use of epsilon-aminocaproic acid or tranexamic acid blocks the activation of plasminogen. This use is not without controversy (160). Nonetheless, the antifibrinolytic agents have been useful in stopping or slowing the blood loss in prostate surgery (161–164).
Temporal course of peripheral inflammation markers and indexes following acute ischemic stroke: prediction of mortality, functional outcome, and stroke-associated pneumonia
Published in Neurological Research, 2022
Ahmet Adiguzel, Ethem Murat Arsava, Mehmet Akif Topcuoglu
A total of 205 patients with acute ischemic stroke, admitted to Hacettepe University Neurology Intensive Care and Stroke Unit for more than 3 days in the last 2 years, were included in the study. The data were collected prospectively, recorded in the departmental database, and processed in a retrospective fashion for this study. Details of our stroke database are described elsewhere [13]. In short, the data is filled directly by a stroke neurologist on a daily basis. All patients undergo transthoracic echocardiography, 24-hour Holter monitoring or continuous bedside cardiac monitoring, cerebral computed tomography (CT), cerebral magnetic resonance (MR) imaging, CT or MR angiography and follow-up neuroimaging. Intravenous tissue plasminogen activator and/or neurointerventional treatments are used per established guidelines. The quality metrics for acute treatment methods and post-acute care were largely followed and included in the database. The Causative Classification of Stroke algorithm was used for etiological classification [14]. The local ethics committee approved the database and protocols.
FlowTriever Retrieval System for the treatment of pulmonary embolism: overview of its safety and efficacy
Published in Expert Review of Medical Devices, 2021
Vivian L. Bishay, Omosalewa Adenikinju, Rachel Todd
Current guidelines recommend ST as first line therapy for high risk PE patients and intermediate high risk patients who decompensate unless otherwise contraindicated. ST can be administered rapidly with regimens of 100 mg over 15 min and 50 mg over 1 min in the most critically ill patients and does not require specialized equipment and set-up. Yet, there are clearly opportunities for alternative therapies that do not utilize lytics. Tissue plasminogen activator is an expensive drug with many patients contraindicated or relatively contraindicated including the elderly, those who have undergone recent surgery and patients with malignancy depending on type and location [8]. These are often the very patients at greatest risk for PE. Administration requires intensive care unit (ICU) monitoring, the most expensive bed in a hospital system, and the morbidity associated with bleeding complications can be costly and increase hospital lengths of stay. Furthermore, the long- term sequelae of residual pulmonary vascular obstruction potentially tied to an undertreatment of PE must also be considered given the treatment and disability costs incurred with long-term adverse PE outcomes.
Impact of Premorbid Malnutrition and Dysphagia on Ischemic Stroke Outcome in Elderly Patients: A Community-Based Study
Published in Journal of the American College of Nutrition, 2019
Fereshteh Aliasghari, Azimeh Izadi, Mohammad Khalili, Mehdi Farhoudi, Shahram Ahmadiyan, Reza Deljavan
The diagnosis of ischemic stroke was confirmed by neurologists. Patients with a prestroke history of oropharyngeal dysphagia, head/neck surgery, or trauma that could affect swallowing ability were excluded from the study. Similarly, patients under treatment by tissue plasminogen activator were excluded from the study. For each patient, a brief questionnaire containing information on demographic variables; admission origin; comorbidities such as diabetes, arthritis, and renal, cardiovascular, gastrointestinal, thyroid, and liver diseases; as well as complications at the time of admission such as presence of dysphagia, infections, tachycardia, and pressure ulcers was filled out according to the patients’ medical records and through interviews with the patients, their relatives, and/or nurses. The protocol of the study was approved by the ethics committee of Tabriz University of Medical Sciences (No. TBZMED.REC.1394.507).