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MeSH Review:

Blood Coagulation Disorders, Inherited

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Disease relevance of Blood Coagulation Disorders, Inherited

The purpose of this study was to compare the clinical thrombosis outcome of patients with (GpI) or without (GpII), inherited coagulation disorders, who suffered from an acute MI with NCA [1].
Hemophilia A and B are hereditary coagulation disorders that result from functional deficiencies of factor VIII (FVIII) or factor IX (FIX), respectively [2].
BACKGROUND AND OBJECTIVES: This open-label multicenter trial of solvent/detergent (SD) plasma involving 17 patients with recessively inherited coagulation disorders (one afibrinogenemia, four FV, six combined FV and FVIII, one FX and five FXI deficiencies) evaluated the pharmacokinetics of the deficient factors and hemostatic efficacy [3].

High impact information on Blood Coagulation Disorders, Inherited

Severe factor X (FX) deficiency is one of the severest inherited coagulation disorders [4].
Desmopressin: therapeutic limitations in children and adults with inherited coagulation disorders [5].
Additional inherited coagulation disorders were found in 1/10 (V:10%) and 2/7 (A:28%) APC-resistant patients [6].
However, since an acquired risk of thrombo-embolic complications frequently masks the inherited deficiency in affected children, children with thrombo-embolism should have adequate laboratory evaluation for inherited coagulation disorders, especially the protein C pathway [7].
BACKGROUND: Factor X deficiency is one of the most rare hereditary coagulation disorders [8].

References

Long term prognosis of patients with myocardial infarction and normal coronary angiography: impact of inherited coagulation disorders. Da Costa, A., Tardy, B., Haouchette, K., Mismetti, P., Cerisier, A., Lamaud, M., Guyotat, D., Isaaz, K. Thromb. Haemost. (2004) [Pubmed]
Viral vector-mediated gene therapy for hemophilia. VandenDriessche, T., Collen, D., Chuah, M.K. Current gene therapy. (2001) [Pubmed]
Solvent/detergent plasma for prevention of bleeding in recessively inherited coagulation disorders: dosing, pharmacokinetics and clinical efficacy. Santagostino, E., Mancuso, M.E., Morfini, M., Schiavoni, M., Tagliaferri, A., Barillari, G., Mannucci, P.M. Haematologica (2006) [Pubmed]
The role of primary prophylactic factor replacement therapy in children with severe factor X deficiency. McMahon, C., Smith, J., Goonan, C., Byrne, M., Smith, O.P. Br. J. Haematol. (2002) [Pubmed]
Desmopressin: therapeutic limitations in children and adults with inherited coagulation disorders. Nolan, B., White, B., Smith, J., O'Reily, C., Fitzpatrick, B., Smith, O.P. Br. J. Haematol. (2000) [Pubmed]
Resistance to activated protein C (APCR) in children with venous or arterial thromboembolism. Nowak-Göttl, U., Koch, H.G., Aschka, I., Kohlhase, B., Vielhaber, H., Kurlemann, G., Oleszcuk-Raschke, K., Kehl, H.G., Jürgens, H., Schneppenheim, R. Br. J. Haematol. (1996) [Pubmed]
Inherited defects of the protein C anticoagulant system in childhood thrombo-embolism. Nowak-Göttl, U., Auberger, K., Göbel, U., Kreuz, W., Schneppenheim, R., Vielhaber, H., Zenz, W., Zieger, B. Eur. J. Pediatr. (1996) [Pubmed]
Factor X deficiency in North Pakistan. Anwar, M., Hamdani, S.N., Ayyub, M., Ali, W. Journal of Ayub Medical College, Abbottabad : JAMC. (2004) [Pubmed]

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