Table 1.

Hemostasis Parameters and Molecular Genetic Findings in Two Families With Ligneous Conjunctivitis

SubjectsFibrinogen (mg/dL)*t-PA (ng/mL)PAI-1 (U/mL)Plasminogen Activity (%)ρPlasminogen AntigenMutations in the Plasminogen Gene
(mg/dL)1-154First Allele/Second Allele
Family 1 
Patient 1 (II-1) 346 5.0 10.6  6.0 <0.4 Arg216 → His/Arg216 → His 
Mother (I-1) 249 3.0 3.5  66   2  Arg216 → His/Wild type 
Father (I-2) 306 6.2 3.0  60   7  Arg216 → His/Wild type 
Sister (II-2) 289 3.6 8.0  76   7.5 Arg216 → His/Wild type 
Family 2 
Patient 2 (II-1) 400 2.0 3.1 <5  <0.4 Trp597 → Stop/Trp597 → Stop 
Mother (I-1) 250 3.7 3.1  60   5.2 Trp597 → Stop/Wild type 
Father (I-2) 240 1.1 3.1  15   1.2 Trp597 → Stop/Trp597 → Cys 
Sister (II-2) 330 2.0 0.89  66   6.9 Wild type/Trp597 → Cys 
SubjectsFibrinogen (mg/dL)*t-PA (ng/mL)PAI-1 (U/mL)Plasminogen Activity (%)ρPlasminogen AntigenMutations in the Plasminogen Gene
(mg/dL)1-154First Allele/Second Allele
Family 1 
Patient 1 (II-1) 346 5.0 10.6  6.0 <0.4 Arg216 → His/Arg216 → His 
Mother (I-1) 249 3.0 3.5  66   2  Arg216 → His/Wild type 
Father (I-2) 306 6.2 3.0  60   7  Arg216 → His/Wild type 
Sister (II-2) 289 3.6 8.0  76   7.5 Arg216 → His/Wild type 
Family 2 
Patient 2 (II-1) 400 2.0 3.1 <5  <0.4 Trp597 → Stop/Trp597 → Stop 
Mother (I-1) 250 3.7 3.1  60   5.2 Trp597 → Stop/Wild type 
Father (I-2) 240 1.1 3.1  15   1.2 Trp597 → Stop/Trp597 → Cys 
Sister (II-2) 330 2.0 0.89  66   6.9 Wild type/Trp597 → Cys 
*

Fibrinogen (normal range, 180-450 mg/dL).

Tissue-type plasminogen activator (normal range, 1-12 ng/mL).

Plasminogen activator inhibitor type 1 (normal, <10 U/mL).

ρ Plasminogen activity (normal range, 80%-120%).

F1-154

Plasminogen antigen (normal range, 6-25 mg/dL).

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