scholarly journals A Case Report of Congenital Afibrinogenemia

2014 ◽  
Vol 1 (1) ◽  
pp. 27-30
Author(s):  
Aysen Turedi Yildirim ◽  
Gokmen Bilgili ◽  
Ozlem Buga ◽  
Ozen Tekin ◽  
Huseyin Gulen

Congenital afibrinogenemia is a rare bleeding disorder. It may be manifested as umblical, mucosal, intramuscular, intraarticular, or life-threatening intracranial bleeding. A third-day-old infant was admitted for umblical cord bleeding, and was found to have a prolonged prothrobin time [PT], and activated partial thromboplastin time [aPTT], and a very low fibrinogen level. He was diagnosed as congenital afibrinogenemia, and reported for the rarity of disease, and discussion of novel therapeutic approaches

1984 ◽  
Vol 12 (01n04) ◽  
pp. 116-123 ◽  
Author(s):  
Jih-Pyang Wang ◽  
Mei-Feng Hsu ◽  
Che-Ming Teng

Bleeding time in rats was markedly prolonged after the adminstration of the water extract of Hsien-Ho-T'sao. This antihemostatic effect was more marked in the group of i.p. injection of the drug than in the group of p.o. administration for 2 to 7 consecutive days. Blood coagulation studies showed that plasma prothrombin time, activated partial thromboplastin time and stypven time were prolonged, while thrombin time adnd fibrinogen level were not changed. The thromboelastographic recording showed that reation time was prolonged and maximal elasticity of clot was decreased. In addition, ADP- and collagen- induced aggregations of platelet-rich plasma was suppressed. In conclusion, the prolongation of the bleeding time might be due to both anticoagulant and antiplatelet action of the drug.


2016 ◽  
Vol 136 (4) ◽  
pp. 229-232 ◽  
Author(s):  
Murat Kose ◽  
Oguz Kagan Bakkaloglu ◽  
Shirkhan Amikishiyev ◽  
Timur Selcuk Akpınar ◽  
Basak Saracoglu ◽  
...  

Acquired hemophilia is a relatively rare clinical presentation, and most cases present with acquired FVIII inhibitor. The co-occurrence of inhibitors to multiple coagulation factors is uncommon. These autoantibodies may induce spontaneous life-threatening bleeding in patients who have had no previous bleeding disorder. Herein, we present a patient with postpartum acquired FVIII and FIX inhibitors who developed intramuscular hematoma and hemothorax during follow-up. She was then treated with activated prothrombin complex concentrate and methylprednisolone.


2019 ◽  
Vol 6 (8) ◽  
Author(s):  
Nancy Louisa Merridew ◽  
Ravinder Singh Phagura ◽  
Edward Anderson ◽  
Louise Anne Cooley ◽  
Graeme Alfred Pollock ◽  
...  

Abstract Mycobacterium chelonae keratitis is rare and difficult to treat. This is the first known case worldwide of effective treatment using intrastromal amikacin injections in a corneal transplant recipient who had metastatic breast cancer. The challenges and principles of management, applicable to other causes of infective keratitis, are reviewed.


Dose-Response ◽  
2020 ◽  
Vol 18 (4) ◽  
pp. 155932582098218
Author(s):  
Li Jiang ◽  
Yuning Li ◽  
Fang Wang ◽  
Xindao Zhang ◽  
Ruiping Zhao

S-Allyl cysteine (SAC), an organic compound and a natural constituent of Allium sativum, commonly known as garlic have been consumed in routine foods are known to possess various biological activities. Nevertheless, scientific evidence on the protective effect of SAC against neonatal asthmatic rats is not available. Hence, the present study aimed at investigating the anti-asthmatic activity of SAC in neonatal asthmatic rats using Wistar rats. The study conducted in 4 groups consists of normal control rats, asthma-induced, asthma animals administered with SAC (25 mg/kg), and SAC control. At the end of the experimental period, inflammatory cells in bronchoalveolar lavage fluid (BALF), inflammatory markers, fibrinogen level, activated partial thromboplastin time, coagulation factor activity, and histopathology were elucidated. The current investigation exhibits that SAC significantly reduced the total leukocytes, with restored fibrinogen level, and activated partial thromboplastin time. In addition, the levels of inflammatory cytokines such as TNF-α (tumor necrosis factor- α), IL-6 (Interleukin 6), and IL-1β have also attenuated in SAC treated animals. Furthermore, the mRNA expression levels of COX2 (cyclooxygenase-2), MCP-1 (monocyte chemoattractant protein-1), RANTES (regulated upon activation, normal T cell expressed and secreted), and eotaxin were reduced in SAC treated animals. Treatment of rats with SAC significantly reduced inflammation and eosinophil infiltration in the lungs. These results suggest that SAC exert protection in neonatal asthmatic rats suffering from acute or chronic inflammation by inducing anti-inflammatory and cell-protective responses.


2017 ◽  
Vol 126 (2) ◽  
pp. 338-344 ◽  
Author(s):  
Ton Lisman ◽  
Robert J. Porte

Preoperative hemostasis testing may have limited use in patients with liver disease, and an abnormal platelet count, prothrombin time, activated partial thromboplastin time, and fibrinogen level should not trigger prophylactic transfusion of blood product components.


2020 ◽  
Vol 3 (2) ◽  
pp. 79
Author(s):  
Ibnu Purwanto

<p>Hemofilia A yang didapat adalah penyakit yang jarang terdiagnosis dan seringkali salah terdiagnosis namun berpotensi menyebabkan perdarahan yang mengancam nyawa. Penyakit autoimun akibat pembentukan autoantibodi (inhibitor) terhadap FVIII ini hampir setengahnya memiliki gangguan lain yang mendasari. Pemanjangan activated partial thromboplastin time, mixing test yang tidak terkoreksi, rendahnya aktivitas FVIII, dan bukti inhibitor FVIII mendukung penegakan diagnosis Hemofilia A yang didapat. Rintangan dalam manajemen pasien dimulai dari penegakan diagnosis hingga penentuan terapi, baik terapi hemostatik, imunosupresi, serta pengobatan penyakit penyerta. Pemilihan terapi serta pengendalian terhadap efek samping dari pengobatan memerlukan perhatian khusus agar tercapai hemostasis dan remisi yang bertahan lama.</p><p>Acquired Hemophilia A can potentially cause life-threatening conditions due to profuse bleeding, but this autoimmune disease is mostly underdiagnosed. Hemophilia A occurs due to the development of an antibody against FVIII, moreover up to half of these cases have underlying conditions. Prolonged activated partial thromboplastin time, uncorrected mixing test, low FVIII activity, and detection of FVIII inhibitors support the diagnosis of acquired Hemophilia A. However, several challenges lay within patients’ management strategy, such as diagnosis workup and therapeutical choices. Treatment for acquired hemophilia A encompasses hemostatic therapy, immunosuppression, and treatment of underlying disease. Moreover, therapeutical choice and side effects control require special consideration to achieve hemostasis and durable remission.</p>


1994 ◽  
Vol 111 (6) ◽  
pp. 733-738 ◽  
Author(s):  
Heidi L. Close ◽  
Thomas C. Kryzer ◽  
John H. Nowlin ◽  
Barbara M. Alving

The purpose of this prospective study in patients undergoing tonsillectomy was to determine whether perioperatve bleeding could be predicted by use of a standardized questionnaire concerning bleeding risk combined with measurement of the activated partial thromboplastin time and prothrombin time. Of the 96 patients enrolled in the study, none had a history of a severe bleeding disorder, but 6 (6%) had histories suggestive of a mild bleeding disorder. Further evaluation showed possible von Willebrand's disease in one of these patients. Of the 90 patients with negative questionnaires, 16% had prolongation of the activated partial thromboplastin time. One of these patients had possible von Willebrand's disease. However, none of the patients with positive questionnaires or a prolonged activated partial thromboplastin time bled after surgery. Bleeding that resulted in additional hospital use occurred in 2% of patients, and blood-tinged sputum was described by 4% after discharge; all of these patients had negative questionnaires and normal screening studies. The data provide further evidence that routine measurement of the activated partial thromboplastin time and prothrombin time in asymptomatic patients is not useful for predicting postoperative bleeding. In addition, histories suggestive of a mild bleeding disorder are also not accurate predictors of postoperative bleeding. Excessive bleeding associated with tonsillectomy is usually not a result of an identifiable coagulation disorder.


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