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the Clinical characteristic of patients with SMD

Clinical implications SMD included various signs of a connective tissue dysplasia, and at the majority of patients the combination of 2 and more signs of a mesenchymal dysplasia was revealed. At overwhelming number surveyed (68,7 %), and among all groups, prevailed signs of dysfunction of cover collagens (I and III types) - a skin hyperelastosis, retardation of healing of wounds, hypermobility and presence in the anamnesis of primary subluxations of hinged joints of average calibre, backbone deformation in a kind kifoskolioticheskoj postures. At patients with syndrome Marfana and "owners" of the phenotype with the same name prevailed, against the asthenic constitution, osteal anomalies - an arachnodactyly, a spider finger, lens subluxations, the Gothic sky. At syndrome Asharda dysplastic changes of endocardiac communications dominated (prolapsy valves, anomalies of the chordal apparatus), were often observed angioektazii. At a syndrome of an imperfect bone formation besides disturbances of a pigmental exchange (a symptom of "blue scleras”), various dysplastic changes in organs (a liver, kidneys, easy) prevailed.

Distribution of patients with SMD taking into account the leading clinical syndrome characterising the basic character of available shifts in system of a hemostasis, is presented to tab. 3.

Table 3

Clinical implications of disturbances of coagulation of blood in the basic group

Syndrome Number of patients
Abs. %
Microcirculatory type, including:

- Dermal implications

- Slizisto-skin implications

120

51

69

51,1

21,7

29,4

Gematomnyj type 10 4,3
The admixed type 78 33,2
Absence of hemorrhages and clottages 3 1,3
Trombotichesky implications 24 10,2
In total 235 100,0

Apparently from tab. 3, at patients with SMD the staxis on microcirculatory type (51,1 %) prevails, on 2 place on frequency there is a staxis on the admixed type (33,2 %), on 3 – trombotichesky a syndrome.

It is important to underline, that earlier terms of a debut of disease became perceptible at patients with hereditary coagulopathies (a hemophilia – 6,52,8 years) and disturbances of a final stage of coagulation (7,82,5 years), proceeding in the conditions of SMD. Whereas in group of comparison terms of occurrence of the first symptoms of disease fell to teenage age (10,60,8 and 13,20,9 years accordingly). At patients with thrombogenic variants SMD the disease debut fell to age 17,24,5 years whereas tromboticheskie implications at patients of group of comparison demonstrated aged 24,12,8 years. Thus the hemorrhagic syndrome at patients with diferrentsirovannymi trombotsitovazopatijami demonstrated at early age and had relapsing character. To thicket it has been bound to seasonal exacerbations and provoked by medicamental influences or presence of active infectious-inflammatory process (an exacerbation of an adenoid disease, a helminthic invasion), or reception dezagregantov (medicinal and alimentary).

Thereby it is defined, that presence SMD is an adverse background as for an early manifesting hemorrhagic and tromboticheskih syndromes, and for features of clinical presentation: prevalence of the admixed staxis at patients with MD and microcirculatory hemorrhages at patients without dysplastic implications.


Hemorrhagic implications at patients MD were different localisation and expression degree (fig. 2).

Fig. 2. Localisation of hemorrhagic implications at patients of the basic group (n=211)

At the overwhelming majority of them the contact staxis of 1-2 and more localisations was observed.

Trombotichesky equivalents at patients with trombofilicheskimi dysplasia variants, are presented to tab. 4.

Table 4

Frequency and structure tromboembolicheskih implications at patients with SMD (n=24)

Clinical implications of clottages Abs. %
Thrombophlebites

Deep vein thrombosis of the bottom and top extremities

postkateterizatsionnye clottages

Clottage of ileal veins and the bottom vena cava

Clottage of uteroplacental vessels:

- horianalnogo plexuses

- Uteroplacental platform

Microclottage of vessels endometrija

Sine-thrombosis

7

3

2

3

5

3

2

2

1

29,1

12,3

8,3

12,3

20,8

12,3

8,3

8,3

4,2

IN TOTAL 24 100,0

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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

Other medical related information the Clinical characteristic of patients with SMD:

  1. THE WORK GENERAL CHARACTERISTIC
  2. MATERIALS AND RESEARCH METHODS
  3. the Clinical characteristic of patients with SMD
  4. the Characteristic of disturbances of a hemostasis at the surveyed patients