Document Detail


Plasminogen deficiency.
MedLine Citation:
PMID:  17900274     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
V Schuster; B Hügle; K Tefs
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Publication Detail:
Type:  Journal Article; Review     Date:  2007-09-26
Journal Detail:
Title:  Journal of thrombosis and haemostasis : JTH     Volume:  5     ISSN:  1538-7933     ISO Abbreviation:  J. Thromb. Haemost.     Publication Date:  2007 Dec 
Date Detail:
Created Date:  2007-11-23     Completed Date:  2008-01-08     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  101170508     Medline TA:  J Thromb Haemost     Country:  England    
Other Details:
Languages:  eng     Pagination:  2315-22     Citation Subset:  IM    
Affiliation:
Hospital for Children and Adolescents, Medical Faculty of Leipzig University, Liebigstrasse 20a, Leipzig, Germany. volker.schuster@medizin.uni-leipzig.de
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MeSH Terms
Descriptor/Qualifier:
Amino Acid Sequence
Animals
Blood Coagulation Disorders* / blood,  classification,  complications,  drug therapy,  epidemiology,  genetics
Conjunctivitis / etiology*
Disease Models, Animal
Fibrinolysin / metabolism
Fibrinolysis*
Genetic Predisposition to Disease
Heterozygote
Homozygote
Humans
Mice
Mice, Knockout
Molecular Sequence Data
Mutation
Phenotype
Plasminogen / chemistry,  deficiency*,  genetics
Protein Conformation
Risk Assessment
Risk Factors
Venous Thrombosis / etiology*
Chemical
Reg. No./Substance:
9001-91-6/Plasminogen; EC 3.4.21.7/Fibrinolysin

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