Congenital Plasminogen Deficiency

01/01/2018 - "An open-label phase 2/3 study of human Glu-plasminogen administered IV at 6.6 mg/kg every 2 to 4 days in 15 patients with congenital plasminogen deficiency is ongoing. "
09/01/2011 - "Isoelectric focusing pattern of plasminogen mutants of patients with hypoplasminogenemia: correlation of in-vitro data with computer-predicted isoelectric points (pI)."
04/01/2009 - "Recent investigations have linked the etiology of this condition to plasminogen deficiency (hypoplasminogenemia). "
03/01/2008 - "Circulating plasminogen activity was repeatedly low, and congenital plasminogen deficiency was identified as the underlying cause of the ocular, oral, and respiratory lesions. "
03/01/2001 - "Oral lesions indicative of plasminogen deficiency (hypoplasminogenemia)."

07/01/2003 - "Contraceptive pills induce an improvement in congenital hypoplasminogenemia in two unrelated patients with ligneous conjunctivitis."

08/01/1986 - "Congenital plasminogen deficiency associated with venous thromboembolism: therapeutic trial with stanozolol."

09/01/2012 - "Type 1 congenital plasminogen deficiency (CPD) is a rare autosomal recessive disease which causes formation of fibrin pseudomembranes that affect multiple systems/organs: the eyes, respiratory system, urinary and genital systems, gastrointestinal system, and the central nervous system. "
12/01/2007 - "Severe hypoplasminogenemia is associated with compromised extracellular fibrin clearance during wound healing, leading to pseudomembraneous (ligneous) lesions on affected mucous membranes (eye, middle ear, mouth, pharynx, duodenum, upper and lower respiratory tract and female genital tract). "
06/01/2007 - "Hypoplasminogenemia is a rare condition that is associated with ligneous conjunctivitis, a form of chronic conjunctivitis characterized by firm, fibrin-rich, pseudomembranous lesions on the tarsal conjunctivae and oral lesions. "
07/01/1990 - "This may allow for more plasminogen to be available for fibrin binding even though newborns have hypoplasminogenemia."
01/01/2018 - "Congenital plasminogen deficiency is caused by mutations in PLG, the gene coding for production of the zymogen plasminogen, and is an ultrarare disorder associated with abnormal accumulation or growth of fibrin-rich pseudomembranous lesions on mucous membranes. "

07/01/1990 - "Hypoplasminogenemia with normal (adult) levels of alpha 2-plasmin inhibitor and plasminogen activator inhibitor are characteristic of the newborn's lytic system. "
09/01/1990 - "These include hypoplasminogenemia, decreases in plasminogen activator, increases in plasminogen activator inhibitor, and Factor XII deficiency."
08/01/1995 - "Because the newborn has hypoplasminogenemia and a possible existence of a dysfunctional plasminogen with normal adult levels of plasminogen activator inhibitor type 1 and alpha 2-antiplasmin and elevated levels of plasminogen activator inhibitor type 2, it could be expected that the response to standard concentrations and doses of plasminogen activators would be reduced. "

09/01/1990 - "These include hypoplasminogenemia, decreases in plasminogen activator, increases in plasminogen activator inhibitor, and Factor XII deficiency."
08/01/1995 - "Because the newborn has hypoplasminogenemia and a possible existence of a dysfunctional plasminogen with normal adult levels of plasminogen activator inhibitor type 1 and alpha 2-antiplasmin and elevated levels of plasminogen activator inhibitor type 2, it could be expected that the response to standard concentrations and doses of plasminogen activators would be reduced. "

09/01/2011 - "One healthy individual with homozygous plg gene polymorphism 504W studied so far exhibited low normal plg antigen and slightly decreased plg activity, suggesting that this polymorphism is associated with (mild) hypoplasminogenemia. "
01/01/1989 - "We report a 45-year-old female patient with recurrent spontaneous deep vein thrombosis associated with an isolated hypoplasminogenemia (plasminogen activity and antigen level of 42% and 37%, respectively). "

01/01/1994 - "In conclusion, although the incidence of thromboembolic events in type I plasminogen deficiency is certainly lower than that described in other congenital clotting disorders such as AT III, protein C and protein S, patients with hypoplasminogenemia should be considered at risk for thrombosis, particularly when triggering factors are present."

01/01/1994 - "In conclusion, although the incidence of thromboembolic events in type I plasminogen deficiency is certainly lower than that described in other congenital clotting disorders such as AT III, protein C and protein S, patients with hypoplasminogenemia should be considered at risk for thrombosis, particularly when triggering factors are present."

08/16/2013 - "Postmenopausal women treated with estrogen hormone replacement therapy and female patients with hypoplasminogenemia receiving oral contraceptives show increasing plasminogen (PLG) concentrations. "

01/01/2021 - "Currently, there is no specific replacement therapy approved for the treatment of congenital plasminogen deficiency. "
01/01/2018 - "Plasminogen replacement therapy should provide an effective treatment of the manifestations of congenital plasminogen deficiency. "
01/01/2018 - "This study provides critical first evidence of the clinical utility of ongoing replacement therapy with human Glu-plasminogen for the treatment of children and adults with congenital plasminogen deficiency. "
01/01/2019 - "Replacement Therapy with Glu-Plasminogen for the Treatment of Severe Respiratory and Auditory Complications of Congenital Plasminogen Deficiency."
01/01/2018 - "Plasminogen replacement therapy for the treatment of children and adults with congenital plasminogen deficiency."

08/16/2013 - "Postmenopausal women treated with estrogen hormone replacement therapy and female patients with hypoplasminogenemia receiving oral contraceptives show increasing plasminogen (PLG) concentrations. "