The most frequent chromosomal violations at the person are anomalies of sexual chromosomes. Among all anomalies the monosomiya X, or the Turner's syndrome has essential clinical value. Frequency of this syndrome in different populations of the world according to different authors fluctuates from 1 on 2 000 to 1 on 10 000 newborn girls. In 1925 professor N.A.Shereshevsky at meeting of the Russian endocrinological society in the report "About interrelation of congenital developmental anomalies and endokrinopatiya" gave the description of the 25-year-old woman.

The classical description of a syndrome was abroad made by the American doctor of Henry H. Turner in 1930 and the German doctor of O.Ulrich the same year. Therefore in literature this disease is named as "Shereshevsky-Terner-Ulrich's Syndrome". Some fenotipic manifestations at Turner's syndrome: small growth, the high sky, lymphatic hypostases of extremities, developmental anomalies of somatic bodies, can be connected with features of functioning of genetic, endocrine and immune system.In this regard by us were defined the purpose and problems of this research.

RESEARCH OBJECTIVE

To study clinic - laboratory, anthropometric data, and also structurally - metabolic parameters of lymphocytes of peripheral blood at patients with Turner's syndrome depending on degree of a variegation of a monosomy X and at girls with an amenorrhea, without karyotype violation for specification endokrinno - immune interactions at these conditions.

RESEARCH PROBLEMS

To give a complex comparative assessment to fenotipic manifestations and anthopometrical parameters at patients with Turner's syndrome.

OBJECT OF RESEARCH

Teenage girls with Turner's syndrome (I Group) and the girls having a normal karyotype, but suffering a time delay of sexual development are inspected (II Group). Age of the inspected - 14-16 years.

All of them were at the time of research in a satisfactory status, except for the complaints connected to a time delay of sexual development. All patient the map of a family tree was formed and methods of determination of anthopometrical data, ultrasonic investigation of internal genitalias, X-ray analysis of bones of hands in the purpose of definition of bone age; research of concentration of gonadotrophic hormones of a forward share of a hypophysis in blood - follitropin and lutropin. On each person the genetic map was filled. The state of health was defined by doctors of interfacing specialties according to the anamnesis and laboratory analyses.

RESEARCH TECHNIQUES:

1. METHOD OF COLLECTING ANAMNESIS.
2. METHOD OF MORPHOLOGICAL AND FUNCTIONAL INDICATORS OF THE BODY.
3. METHOD OF CULTIVATION OF CAGES AND RECEIVING PREPA RATIONS OF METAPHASE CHROMOSOMES FROM LYMPHOCYTES.
4. STATISTICAL RESEARCH TECHNIQUES.

CLINICAL CHARACTERISTIC OF ANTROPOMETRICAL PARAMETERS OF PATIENTS

The main and reference indicators in medicine are the weight, length of a body and a thorax circle. We compared indicators of weight of a body of patients to Turner's syndrome and indicators of average weight of a body of girls of 2 groups. Thus, the average mass of a body of girls with Turner's syndrome was 38,64 kg, the mass of a body of girls of 2 groups - 46,83 kg. The mass of a body of patients of 1 and 2 groups lagged behind the average weight of a body of healthy girls.

Indicators of mass of a body of girls with Turner's syndrome and with a normal karyotype were distributed as follows (table 1). Apparently from the table, in group with Turner's syndrome total absence of part of girls with the increased mass of a body becomes perceptible, in the second group the similar share of girls made 8,65%.

At the same time among girls with a Turner's syndrome a large number of persons with an oligotrophy is observed 48,35 %, in the II group them were less 28,85 %.In group without disturbance in a karyotype 17,31 % had a depression of mass of a body, and with Turner's syndrome - 2,20 %.

DISTRIBUTION OF INDICATORS OF MASS OF THE BODY OF PATIENTS (%) M±m.

table 1

Body height of patients of 1 group with Turner's syndrome lagged behind indicators of body height of sick 2 groups. This indicator is peer 144,30 cm - 1 group and 156,45 cm - the 2nd group. These parameters also there is less than parameters of average height of girls in the general population.

Distribution of indicators of body height of patients in groups is shown in table 3.2. Apparently from the table, in 1 group of tall girls wasn't, in the 2nd group them there were 3,85%. The main share of representatives of 2 groups was made by girls with normal body height - 62,5%, in 1 group of the girl with normal body height made a share in 28,58%. The main share of persons with Turner's syndrome is presented by girls with a nanizm - 57,14%, in the 2nd group without karyotype disturbances this share made 19,23%. Reliable differences between shares at representatives with the reduced and low body height in 1 and the 2nd group us it wasn't taped. In the analysis of correlation communications inverse relationship of parameters of mass of a body of patients of 1 group from percentage of a pathological clone of cells 45,X (r = - 0,276; P<0,05; r = -0,365 P<0,001).

ANTHROPOMETRIC INDICATORS OF PATIENTS 1; 2 GROUPS (M±m)

table 2

P1; P2; Pk - reliability of differences between the relevant group.

Indexes of a circle of a thorax designate a tendency to formation of an asthenic body build both at representatives of 2 groups, and in 1 group. Indexes of a circle of a thorax are lowered in comparison with the standard parameters, and girls of 2 groups more astenik and have narrower thorax. This fact is visually shown by consideration of an index of a thorax at patients with Turner's syndrome and patients without karyotype violations. So, the thorax index at patients of 1 group was on the average equal 48,09 while, in the 2nd group it equaled 44,86. Thus, patients with Turner's syndrome had a wide shape of a thorax, "barrel".

At survey of a thorax the hypertelorism of mammas became perceptible. In our researches the distance between papillas are slightly more at girls of 1 bunch and 19,32 ± 0,33 sm was peer; in 2nd bunch - 18,42 ± 0,26 see. At the expense of decrease of a circle of a thorax we had not augmentation of an intermammillary index. This index were peer 1 bunch - 27,07, and in 2nd bunch - 25,98. Normal indexes of an intermammillary index at able-bodied girls was peer 23. At the analysis of a syndrome of Turner with a variant of a monosomy and a tesselated variant separately, us it had not been notic essential augmentation of an intermammillary index at patients with a monosomy. Probably, absence of the express differences in size of a medicalpapillary index at patients 1 and 2nd bunches spoke about the general tendency to delection of a clinical pattern of a syndrome of Turner and a prevailation in a phenotype of sick asthenic fig.

At the analysis somas graphically construct morfogrammes the prevailation of a part them towards an intersexual body build became perceptible. Such it had been tap 52,5 ± 5,32 % in 1 bunch of patients with Turner syndrome and very few patients (5,0 ± 2,38 %) were in this bunch were with an isosex body build. The body build, characteristic for a hypo-ovaria, in 1 bunch of patients had been not by us at 34 girls (in 38,2±5,14 %).

Consider a difference of anthropometric indexes in a body build of girls with a syndrome of Turner and without disturbances in a karyotype, we had decid to apply a method of an identification of a soma to representatives of the yield bunches. Among all girls subject to an identification of a soma, have been evolv the following categories of phylums of a body in 1 bunch: 70,59 ± 4,81 % - stenoplastik; 11,76 ± 3,44 % - mesoplastic; the asthenic; euriplastik and subathletic - on 5,9 ± 2,55 %. In 2nd bunch the following phylums of a soma have been evolv: 33,33 ± 4,81 % - stenoplastik; 33,33 ± 4,65 % - subathletic; mesoplastic, asthenic, pyknic and euriplastik had compound on 8,33 ± 2,73 %.

The futty component of a body had been more express at representatives of 2nd bunch. From lump of a body the quantity of fat at teenagers of 1 bunch had compound 24,877 ± 8,85 % while persons have 2nd bunches - 27,523 ± 1,54 % (table 3). The absolute mass of a muscle tissue at persons of 2nd bunch slightly exceeding mass of a muscle file at girls of 1 bunch. Percentage of an osteal tissue slightly differed from each other in 1 and 2nd bunch.

Thus, the data of anthropometric researches taped a difference in a componental body composition between bunches. The first bunch had the least development of all three components of a body. The second bunch had more high maintenance of fat, muscles and bones. However the difference did not reach the statistical significance for an one index.

COMPONENTAL COMPOSITION OF THE SOMA OF PATIENTS OF 1 AND 2 BUNCHES (M ± m)

table 3

CONCLUSIONS

1. Girls with Turner's syndrome have a decrease of weight of a body, body lengths, decrease in level of sexual development meets more often than in group of girls an amenorrhea not a chromosomal genesis.
2. The identification of a structure of a body revealed at patients with Turner's syndrome a dominance the stenoplastic type (77,59 %). At girls with a delay of sexual development without karyotype violations most often along with stenoplastic type of a body (33,33 %) the subathletic type (33,33 %) met also.

2. Turner H.H. A syndrome of infantilism, congenital webbed neck, and cubitus valgus // Endo-crinology. -1930. –Vol. 23. -P 566.

3. Ullrich O. Űber typische Kombinationsbilder multipler Abartungen // Zschr. Kinderheilk. -1930. –Bd. 49. -S. 271-276.