the Characteristic of disturbances of a hemostasis at the surveyed patients

Taped in system of a hemostasis of disturbance at patients with hereditary kollagenopatijami consisted in essential depression agregatsionnoj functions of thrombocytes (Tr) practically on all physiological inductors of aggregation used in standard delutions (ADF, adrenaline, collagen, Thrombinum).

Thus hypoaggregation was more often found out on ADF - adrenaline - a fibrinogen and collagen-aggregations Tr, was much less often oppressed Thrombinum-aggregation of plates, reaction of liberation of contents of granules of storage (basically ADF) was slowed down also. Similar found out changes trombotsitarnoj are presented activation and in similar foreign researches [Mayne K. еt al., 2001]. Similar shift is found out and at application of not physiological agonist of aggregation – ristomitsina/ristotsetina. However at patients with serious dysplastic syndromes hypoaggregation Tr was observed mainly on contact and pristenochnye inductors (fibrin, collagen), is less expressed on membranous inductors of aggregation Tr (ADF, adrenaline, Thrombinum), that patognomonichno for trombotsitopaty at syndrome SMD.

At patients with syndrome Marfana it was observed subpartial dizagregatsionnaja trombotsitopatija with disturbance of membranous activation of thrombocytes (ADF, adrenaline, collagen-aggregation). At patients with syndrome Elersa-Danlosa it is taped partial dizagregatsionnaja trombotsitopatija, caused by depression ADF - and in some cases and-or collagen-aggregations Tr that will be compounded with data of some similar researches [Anstey A. et al., 1991]. At patients with syndrome Vrolika-Lobshtejna disturbances Thrombinum - and adrenaline-aggregations of thrombocytes were observed. Besides, at a syndrome of the imperfect bone formation bound to anomalies of collagen Ia and IV types adhesive activity of thrombocytes to collagen and fiber glass was essentially broken. In some cases (25,6 % of observations) have been noted depression at the given category of patients of activity of plasma factor Villebranda, as explained character dizadgezivnyh disturbances [Mannichi M., 1999].

At patients with syndrome Marfana and Elersa-Danlosa depression mezhtrombotsitarnogo interactions at the expense of qualitative anomaly of endogenous collagen, a smoothness of II wave and, as consequence, offensive of the early disaggregation testifying to absence effective vnutritrombotsitarnoj of generation endogenous proagregantov (TR-А2) was taped also. Clinically at these patients the microcirculatory staxis prevailed and hemorrhages appeared after traumas or microinvasive operations (an ex-traction of a teeth, an adenotomy, cosmetic manipulations and so forth) [Cуханова A, Barkagan Z.S., 2004; Winter M. еt al., 2002].

It is revealed, that in group of patients with undifferentiated SMD in the form of Marfanopodobnogo, Elersopodobnogo and MASS-like phenotypes average indexes of the induced aggregation on all kinds of agonists and degree of adhesion of thrombocytes to collagen it is authentic below control parametres. Thus hypoaggregation was more often found out on ADF - adrenaline - a fibrinogen and collagen, is much more rare on Thrombinum; reaction of liberation of contents of granules of storage (basically ADF) was slowed down also.

At all patients with Marfanopodobnym a phenotype it was observed subpartial dizagregatsionnaja trombotsitopatija with disturbance of membranous activation of thrombocytes (ADF - adrenaline - collagen-aggregation).

At patients with Elersopodobnym a phenotype it is taped partial dizagregatsionnaja trombotsitopatija, more often caused by depression of ADF-AGGREGATION and only at a number of patients - collagen-aggregations of thrombocytes. In a quarter of cases (25,6 % of observations) depression of activity of plasma factor Villebranda is noted, as explained character dizadgezivnyh disturbances. Practically it was not broken Thrombinum-aggregation Tr, indirectly haraktrizujushchaja intracellular negeratsiju progaregantov, that Farm laborer T.A will be compounded with data of other authors [., 1999, Sukhanov G. A, 2002, 2004; Anstey A., Mayne K., Winter M. et al., 1991].

At patients with implications SMD, among the other taped shifts in hemostasis system, dominate sochetannye disturbances in hemostasis system (35,0 %). Among them there are various, sometimes difficult for verification, combinations hereditary trombotsitopaty with other disturbances in hemostasis system. Thus combined defects trombotsitarnyh dysfunctions dominated in all groups surveyed. Nevertheless, in groups of patients with partial and subpartial dizagregatsionnymi NTP disturbances contact and pristenochnoj activation trombotsitarnogo a pool, registered on more or less expressed hypoaggregation Tr on corresponding inductors – native fibrin (fibrinogen) and collagen, and in some cases and on Ristomycinum (ristotsitin) prevailed, especially at sochetannom deficiency of activity of factor Villebranda. The given patterns logically speak physiology contact and preendotelialnoj activation of thrombocytes. So, interaction of the last with a fibrinogen by linkage with receptors GP IIb/IIIa (integrinami αIIbβ3). The membranous activation of thrombocytes induced ADF, adrenaline - and Thrombinum-agonistami, was broken in much smaller degree.

A little conceded them on frequency of occurrence of the form with primary disturbance agregatsionnoj functions of thrombocytes (at 40,3 % of patients). With the greatest constancy subpartial hypoaggregation (aggregation weakening on 2-3 inductors) was observed. The partial (isolated) hypoaggregation taped at use only of 1 inductor of aggregation, and, more often on collagen and native fibrin that contacted disturbance pristenochnoj activation as a result of defect of a collagenic matrix endothelial vystilki Less often took place.

Among the last disorders of processes of polymerisation of a fibrin-MONOMERS (55,2 % of patients) that was shown by augmentation antsistrodonovogo coagulation time were most often found out. Thus in a number of observations moderate deficiency of factor Villebranda (vWF) in a blood plasma on the average in 1,34 times below the control specification was simultaneously taped. At these patients syndrome Villebranda of plasma type (in 52,1 % of cases), characterised by moderate depression of activity vWF (within 25-40 % from norm), trombotsitopatiej with aggregation disturbance mainly on Ristomycinum has been documented. Less often (33,3 children) had syndrome Villebranda trombotsitarnogo type at which trombotsitopatija with the expressed hypoaggregation on Ristomycinum proceeded against normal or subnormal activity vWF (not less than 60 % from norm).

On the basis of results of paraclinic researches the conclusion has been drawn on basic differences in indicators sosudisto-trombotsitarnogo a link of a hemostasis at patients with implications MD and healthy children. So, at the thrombocytes first quantity in the blood analysis had the distinct tendency to depression (p 0,5).

Practically changes in basic coagulative tests, such as APTV, TV, PV, plasma level of a fibrinogen that will be compounded with observations of other authors [Cуханова A, 1994 were not taped; Suvorova A.V., 2000]. At the same time the reactions characterising efficiency of a final stage of coagulation, such as auto - and heteropolymerization of a fibrin-monomers (Auto - and Getero-PfM), has appeared prolonged. Taking into account diagnostic possibilities of the given techniques these facts allow to document at the surveyed patients of disturbance at a final stage of coagulation of blood in the form of a dysfibrinogenemia.

Special interest represent особенноcти disturbances of a final stage at patients with kollagenopatijami in the conditions of SMD. At 80 % of patients of the basic group were registered sochetannye the defects of a hemostasis consisting in prolongation of rate Auto - and Getero-PfM (r

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Scientific source Sturov Victor Gennadevich. Disturbances of the FINAL STAGE of Coagulation of blood at children and teenagers with a syndrome of a system mesenchymal dysplasia. The dissertation AUTHOR'S ABSTRACT on competition of a scientific degree of the doctor of medical sciences. Novosibirsk - 2007. 2007

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