Type 1 plasminogen deficiency is a rare genetic disorder. Type 1 plasminogen deficiency is characterized by fibrin-rich pseudomembrane formation on mucosal surfaces, particularly the conjunctiva. Tracheobronchial tree involvement is a less common reported manifestation of type 1 plasminogen deficiency. Pseudomembranes in the tracheobronchial tree may result in respiratory compromise and ultimately fail if not recognized and treated. Currently, there is no specific replacement therapy approved for the treatment of congenital plasminogen deficiency. In the present paper, we report that type 1 plasminogen deficiency with novel frameshift mutation and pulmonary involvement was treated initially with systemic fresh frozen plasma followed by pulmonary lavage with fresh frozen plasma and tissue plasminogen activator.
Background: In the Republic of Croatia cancer is the second cause of mortality after cardiovascular disease. Patients with cancer have a 4 to 7-fold higher risk of developing venous thromboembolism (VTE) and those who developed VTE have poorer survival than those without VTE. The greatest risk of developing VTE is present in pancreatic, brain, stomach and ovarian cancer, while the lowest risk is with breast and prostate cancer.Aims: Analyze characteristics of patients with cancer who were treated for VTE in General Hospital Dr. Josip Bencˇevic´ for a period of five years. Methods: In the General Hospital Dr Josip Bencˇevic´ -location of Slavonski Brod longitudinal observational analyzes of patients with cancer and newly diagnosed VTE treated in hospital during 2011-2015. Results: A total of 546 cases of VTE were diagnosed in the period of 5 years, out of which 134 (25%) patients with cancer. In patients with cancer and VTE (134) 46% were female while the median age was 67 years, ranging from 38 to 89 years. 87 (65%) patients had deep vein thrombosis (DVT), pulmonary embolism (PE) had a 34 (25%) while 13 (10%) patients had PE and DVT simultaneously. Cancer was only risk factor for development of VTE in 44 (33%) patients, while others had one or more additional risk factors; 51 patients (38%) were treated with possible thrombogenic chemotherapy, 36 (27%) had surgery within 6 months of diagnosis, 15 (11%) patients had sepsis and 18 (13%) were immobilized. A total of 27 (20%) patients died, 16 out of pulmonary embolism, 10 from cancer and 1 from sepsis as a main cause of death. The most common primary cancer sites were: lung 17%, colon and rectum 16%, haematological malignancies 11%, pancreas 9%, ovary 8%, prostate 7%, breast 6%, liver and gallbladder 6%, urinary bladder 5 % and stomach 4%. Summary/Conclusion: The results of our analysis are consistent with data from the literature on VTE incidence and the percentage of VTE caused by cancer. Distrubution of patients with cancer and VTE according to primary cancer site is also like data from literature -most cases were with lung and colon cancer who are the most common cases of cancer in our population while the most thrombogenic cancer in our study was pancreas. VTE prevention in patients with high risk of developing VTE is a priority in today's treatment. Strong adherence of the appropriate thromboprophylaxis is required to reduce the incidence of VTE in the cancer patients.
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