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Inflammation
Published in George Feuer, Felix A. de la Iglesia, Molecular Biochemistry of Human Disease, 2020
George Feuer, Felix A. de la Iglesia
Hemorrhagic syndromes are connected with inherited defects of coagulation factors (Table 2). In these disorders the defect is mainly due to the synthesis of an abnormal protein rather than the lack of production.376 Deficiences of Factors VII, VIII, and IX are the most frequently occurring disease conditions. Both defects are related to extrinsic and intrinsic pathways of coagulation 89,157 Factor VIII and Factor IX deficiencies are X chromosomelinked disorders, and they are mainly found in males. The management of Factor VIII inhibitors in nonhemophilic patients seems to be essential.157 Hereditary antithrombin III deficiency is connected with venous thrombosis.309 In certain conditions, such as colon adenocarcinoma, the production of acquired factors has been observed.81
Miscellaneous: Dextran, Dermatan Sulfate, Low Molecular Weight Heparinoids (Org 10172), Pentosan Polysulfate (Sp54), Defibrinating Agents (Ancrod And Reptilase)
Published in Hau C. Kwaan, Meyer M. Samama, Clinical Thrombosis, 2019
M. M. Samama, P. C. Desnoyers, H. C. Kwaan
Pentosan polysulfate was as active a prophylactic drug as Dextran 70 in orthopedic surgery and better than Dextran 70 in general surgery. It is being used in other trials at 50 mg b.i.d., but larger series are needed. Finally, this drug may be useful in patients with antithrombin III deficiency since its action is independent of antithrombin III.36
Point-of-care ultrasound for diagnosis of pneumothorax in a pregnant COVID-19 patient in the emergency department
Published in Journal of Obstetrics and Gynaecology, 2022
Muge Gulen, Salim Satar, Nurdan Unlu, Cemre Ipek Esen, Mehmet Bozkurt, Sarper Sevdimbas, Selen Acehan
Except for the most common pneumothorax aetiologies, the connective tissue dysplasias (Rheumatoid arthritis, Ankylosing spondylitis, Polymyositis and dermatomyositis, Scleroderma, Marfan’s syndrome, Ehlers–Danlos syndrome, Pleuroparenchymal fibroelastosis and SLE) can also causes pneumothorax. SLE causes secondary pulmonary fibrosis, resulting in pneumothorax (Sahn and Heffner 2000). Because of the presence of pneumothorax with thrombophilia in our patient, tests were performed for antiphospholipid syndrome secondary to SLE. The patient’s Lupus Anticoagulant, Anticardiolipin Antibody and Anti β 2-glycoprotein I antibodies, anti-nuclear antibodies (ANA) and anti-double stranded DNA (anti-dsDNA) tests were also negative (Knight and Kanthi 2022). Since the patient had a history of recurrent abortion and deep vein thrombosis, tests were also performed for conditions that could cause thrombophilia (Grandone and Piazza 2021). Belonging to the most common causes of thrombophilia, Antithrombin III deficiency, Protein C deficiency, Protein S deficiency, Factor V Leiden mutation and Prothrombin G20210A mutation tests were negative.
Acute portal vein thrombosis in noncirrhotic patients – different prognoses based on presence of inflammatory markers: a long-term multicenter retrospective analysis
Published in Scandinavian Journal of Gastroenterology, 2019
Radan Keil, Jana Koželuhová, Jiří Dolina, Aleš Hep, Radek Kroupa, Vladimír Kojecký, Tomáš Krejčí, Jan Havlín, Ivana Hadačová, Jitka Segethová, Petra Koptová, Zdena Zádorová, Jan Matouš, Barbora Frýbová, Petr Chmátal, Martin Wasserbauer, Jan Šťovíček, Melvin Bae, Tolga Guven, Mahmood Zaeem, Štěpán Hlava
Portal vein thrombosis (PVT) is a partial or complete thrombotic occlusion of the portal vein. Occlusion can be successive, in chronic PVT, or sudden, in acute PVT. The prevalence of PVT in the general population is 1.1% [4]; while it occurs in 75% of patients with cirrhosis or hepatobiliary malignancies [4]. Acute noncirrhotic, nonmalignant PVT is much rarer [4,5]; however, precise data on the incidence do not exist. Patients with acute PVT typically have abdominal pain that develops suddenly or progresses over a few days. The first manifestation can be life-threatening, acute variceal bleeding. Asymptomatic PVT diagnosed as an accidental finding, using imaging methods indicated for another reason, is relatively rare except in cirrhotic patients [6]. Disorders associated with a higher risk of PVT development are well-described and, based on Virchow's triad, similar to peripheral thrombosis – stasis of blood flow, endothelial injury, and hypercoagulation. Procoagulant disorders are more important in acute noncirrhotic, nonmalignant PVT due to the limited possibility of venostasis and endothelial dysfunction. Hereditary procoagulant disorders associated with the risk of thromboembolism are factor V Leiden, prothrombin, and JAK2 gene mutations [7]; protein C, S, and antithrombin III deficiency; or increased levels of factor VIII [7–9]. Acquired disorders are an antiphospholipid syndrome, undiagnosed myeloproliferative disorders, malignancy, severe abdominal infection, or trauma [10,11].
Clinical profiles and risk assessment in patients with antiphospholipid antibodies
Published in Expert Review of Clinical Immunology, 2019
Masaru Kato, Ryo Hisada, Tatsuya Atsumi
Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by vascular thrombosis and pregnancy morbidity in the presence of persistent antiphospholipid antibodies (aPL). Thrombosis occurs in both arteries and veins, whereas other thrombophilia, such as factor V Leiden, protein S deficiency, protein C deficiency, and antithrombin III deficiency, predominantly affects veins. Pregnancy morbidity includes fetal loss after the 10th week of gestation, severe preeclampsia, placental insufficiency, and recurrent early miscarriages. Patients with APS may also have thrombocytopenia, renal microangiopathy, heart valve disease, livedo reticularis, or neurological manifestations, which are called as non-criteria manifestations of APS. Catastrophic APS is a rare, with its prevalence of less than 1% in patients with APS, but most severe form of APS which should be suspected in front of a patients with severe multiorgan involvement with thrombotic microangiopathy over a short period of time [1]. APS occurs primarily (so-called primary APS) in approximately half of the patients, whereas the other half is associated with other autoimmune diseases (so-called secondary APS), particularly with systemic lupus erythematosus (SLE), with similar clinical and immunological profiles in both groups [2].