Plasminogen deficiency

Abstract

Summary. Plasminogen (plg) deficiency has been classified as (i) hypoplasminogenemia or ‘true’ type I plg deficiency, and (ii) dysplasminogenemia, also called type II plg deficiency. Both forms, severe hypoplasminogenemia and dysplasminogenemia, are not causally linked to venous thrombosis. Dysplasminogenemia does not lead to a specific clinical manifestation and probably represents only a polymorphic variation in the general population, mainly in Asian countries. 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). Ligneous conjunctivitis is by far the most common clinical manifestation. More than 12% of patients with severe hypoplasminogenemia exhibit congenital occlusive hydrocephalus. In milder cases of ligneous conjunctivitis, topical application of plg‐containing eye drops, fresh frozen plasma, heparin, corticosteroids or certain immunosuppressive agents (such as azathioprine) may be more or less effective. Oral treatment with sex hormones was successful in two female patients with ligneous conjunctivitis. In severe cases with possibly life‐threatening multi‐organ involvement, true therapeutic options are not available at present. The plg‐knockout mouse is a useful tool to study the many different properties of plg in a variety of settings, such as wound healing, tissue repair and tissue remodeling, virulence and invasiveness of certain bacteria in the human host, tumor growth and dissemination, as well as arteriosclerosis.

Epithelial Integrity Is Maintained by a Matriptase-Dependent Proteolytic Pathway

“…An illustrative example of such a defect is deficiency of the serine protease, plasminogen, which was long described as a disease affecting only the eyes and the conjunctiva but now is well established as a systemic disease affecting multiple epithelia. 44 In summary, this study has revealed an essential role of matriptase in the maintenance of global epithelial homeostasis in the mouse and has provided an important animal model for the further exploration of matriptase function in multiple physiological and pathological processes.…”

Marine Polysachharides Used in the Treatment of Beleeding Disorder-a Review

“…In spite of the fact that the hyperfibrinolytic state is related with expanded propensity to drain, lack of the same inclines to thromboembolism. [29] Excessive enactment of fibrinolysis might be seen amid cardiopulmonary sidestep, thus antifibrinolytics have a valuable part in the aversion of same.…”

Ligneous membranitis in Scottish Terriers is associated with a single nucleotide polymorphism in theplasminogen(PLG) gene

“…Similar splice‐site mutations have previously been noted in affected humans with LM (Schuster et al . 2007). The non‐carriage of c.1256+2T/A in 24 normal Scottish Terriers indicates that carriers of the mutation are generally not common in this breed.…”

“…2008), the literature for humans supports hypoplasminogenemia as the most likely cause of LM (Schuster et al . 2007). Therefore, it is difficult to explain the apparently normal plasminogen activity in the affected male.…”