Control Propositus Mother Coagulation test Prothrombin time (sec) 12 - 14  16.4 16.1 Thrombin time (sec) 16 - 19 29.9 29.1 aPTT (sec) 27 - 35 29.1 29.5 Fibrinogen (mg/dL): Clauss 200 - 400 80 - 100 95 - 111 Polimerization       Plasma: Fibrinogen (mg/mL)1 Lag time (sec) Slope (mOD/sec) MaxAbs (mOD) Purified Fibrinogen: Lag time (sec) Slope (mOD/sec) MaxAbs (mOD) 300 15 3.3 ± 0.7 708 ± 30   9.1 ± 7.5 0.386 ± 0.129 90 ± 23 ND 15 1.6 ± 0.7 743 ± 11   ND 280 15 1.4 ± 0.4 785 ± 17   15* 0.183 ± 0.038* 74 ± 22* Permeation Fibrinogen (mg/mL)1 280   125   300 Ks (×10-9cm2) 8.2 ± 0.3 (18) 9.0 ± 1.4 (7) 5.0 ± 0.5* (17) LSCM FWHM (μm)   Density (peaks/μm) 0.58  ±  0.32 (1220) 0.457  ±  0.1 0.56  ±  0.31 (1055) 0.367  ±  0.08**   0.58  ±  0.32 (1573) 0.436  ±  0.08 1Fibrinogen quantified according reference 20. aPTT: partial activated thromboplastin time; MaxAbs: maximum absorbance. Ks: permeation coefficient or Darcy constant. LSCM: laser scanning microscopy. ND: not done. FWHM: full width at half maximum *p<0.05 compared to control; + p<0.05 compared to the propositus´ mother

Table 1: Summary of the coagulation screening tests, fibrin polymerization, permeation and laser scanning confocal microscopy studies. Results are reported as mean (± SD), and the number of measurements are in brackets.