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Homozygous type I plasminogen deficiency
A M Mingers1, N Heimburger, P Zeitler
1Children's Hospital, University of Würzburg, Germany.
Seminars in Thrombosis and Hemostasis
|January 1, 1997
Summary
This study reports the first cases of homozygous type I plasminogen (Plg) deficiency in three female patients with ligneous conjunctivitis. This deficiency causes impaired fibrin clearance, leading to pseudomembranous lesions.
Area of Science:
- Hematology
- Ophthalmology
- Genetics
Background:
- Ligneous conjunctivitis is a rare condition with unknown causes.
- Homozygous type I plasminogen (Plg) deficiency has not been previously documented in humans.
- This condition involves extensive fibrin deposition in mucous membranes due to impaired plasmin-mediated clearance.
Purpose of the Study:
- To report the first cases of homozygous type I plasminogen deficiency.
- To investigate the etiological link between plasminogen deficiency and ligneous conjunctivitis.
- To characterize the clinical and laboratory findings in affected patients.
Main Methods:
- Clinical case reporting of three unrelated female patients.
- Biochemical analysis of plasminogen activation capacity via infusions.
- Assessment of fibrinolytic activity and related markers (e.g., D-dimer).
Main Results:
- Three patients with homozygous type I plasminogen deficiency and ligneous conjunctivitis were identified.
- Infusions confirmed absent fibrinolytic activity due to plasminogen deficiency.
- Lysine-plasminogen (Lys-Plg) infusions led to transient plasminogen recovery and increased plasmin-antiplasmin complexes and D-dimer.
- One patient had co-existing partial factor XII deficiency, which did not impede plasminogen activation.
Conclusions:
- Homozygous type I plasminogen deficiency is a newly identified cause of ligneous conjunctivitis and other pseudomembranous lesions.
- The condition results from the inability to clear fibrin effectively.
- Lys-Plg therapy shows potential for plasminogen level restoration.