structure of congenital defects at a syndrome of Down

Characteristic for SD the congenital anomalies influencing their life expectancy, include anomalies of heart, zheludochnoyokishechnogo a tract and genitourinary system.

One of principal causes of an early mortality of children with SD are the congenital heart diseases, which frequency, according to the literature, reaches 50 %, and this category of children makes 7 % from all babies with VPS [135, 130, 126].

Among heart diseases, most often there is perimembranoznyj defect of an interventricular septum, a persistent arterial duct, defect of an interatrial septum, the general open atrioventricular channel (AVK), tetrada Fallo, and other defects, in the sum of 1 % making less [50]. From the middle of last century the set of researches on revealing of frequency and specificity of heart diseases at these children was spent. So, during the period of 1970-1980th rising of frequency of congenital anomalies of cardiovascular system at patients with SD became perceptible, that has been bound to improvement of diagnostics of an open arterial duct and defect of an interatrial septum.

It is known, that at newborns with SD quite often it becomes perceptible morfofunktsionalnaja immaturity. At children with morfofunktsionalnoj immaturity the sizes of defect of the interatrial are often underestimated
Septums which survey as an open oval window. Circulatory unefficiency first signs, such as feeding disturbances, a low increase of mass can be regarded as signs of the syndrome of Down, and absence of treatment will lead to development of a congestive pulmonary hypertensia and pneumonia occurrence. Fixing and refractory to therapy the current of a pneumonia at children with a trisomy of 21st chromosome is bound with characteristic at these sick of immunologic disturbances.

Ultrasonic research of anatomy of heart on the first month of a life at all newborns with SD [30, 147, 29, 145] allows to avoid diagnostic mistakes, and also is timely (before development of a serious circulatory unefficiency) to prescribe conservative treatment and to optimise terms of surgical correction of a congenital heart disease.

Necessity of carrying out of early EHOKG-INSPECTION at all babies with SD it has been shown by research group of scientists under the guidance of McElhinney [87]. They have established, that informativnost physical inspection for revealing of warm anomalies at children with a trisomy of 21st chromosome does not exceed 80 %. It has appeared, that 15 from 114 investigated children at survey had no signs of congenital heart diseases, but at ultrasonic research warm anomalies have been diagnosed for them. Moreover, devjaterym from these patients operative treatment further was required.

The urgency of early diagnostics VPS is dictated also by appreciable successes for last decades in area kardiohirurgii which have allowed to raise survival rate of babies with SD having a congenital pathology of cardiovascular system from 78 % in 1985 to 90 % to 2004 [61], and also it is essential to improve the remote forecast of surgical correction VPS.

At comparison of a current and outcomes of operative treatment of the full form atriventrikuljarnogo the channel at babies with SD and without a syndrome, in the work spent on the basis of NTSSSH by it. Bakuleva, T.I.Zadko notices, that at children with SD the pulmonary hypertensia, the important pathogenetic mechanism in which development, obviously faster develops, is the oxidising stress [2].

Genetically caused features of antioxidatic system, including initially low level glutationa, and higher antioxidatic activity of Serum at children with SD testify to higher oxidising stress at development of a pulmonary hypertensia at heart diseases with a pulmonary hypervolemia [4].

From early complications of surgical correction of the atrioventricular channel at children with SD meet infektsionnoyosepticheskie complications, whereas at children without a syndrome - an acute heart failure is more often. This circumstance speaks anatomic features of defect and available immunologic disturbances at babies with a trisomy of 21st chromosome [2].

At the analysis of postoperative complications and a mortality after surgical correction VPS at patients with and without SD, it has appeared, that essential differences in these groups are not present [51].

Developmental anomalies of a gastroenteric tract meet at 4-10 % of [49] children with SD and can clinically not be shown throughout several months. The carried out researches have shown high prevalence of anomalies of development of twelve perstnoj intestines and about 25-30 % of all these anomalies it is necessary on children with a trisomy of 21st chromosome. The structure of congenital defects of a gastroenteric tract at children with SD includes an esophagus atresia/traheopishchevodnyj a fistula (0,3-0,8 %), a stomach pyloric stenosis (0,3 %), a stenosis \atreziju

Well-known constipations about prevalence at children with a trisomy of 21st chromosome [116]. It speaks two principal causes: a hypomyotonia and a low motility of an intestine. Both these of the factor, promote a long delay of a chair in an intestine and to its inspissation to means of loss of a liquid because of reabsorbtsii waters in a colon. The chair becomes firm, the big diameter. Constipation treatment is referred on a ramollissement of fecal masses. The diet with expansion of a drinking regimen and introduction of the lactulose promoting attraction of a liquid in a cavity of an intestine and a ramollissement of a chair [7] is for this purpose applied. At obstipations and an inefficiency of standard therapy the exception of illness Girshprunga and a hypothyrosis is necessary.

At children with SD risk of congenital defects of genitourinary system, such as the hydronephrosis, a hydroureter, pielektazija and gipoplazija/aplazija kidneys, etc. is considerably raised and makes according to the spent researches of 3,2 % [73]. Clinical symptoms in the form of an incontience of urine and difficulties of schooling of the child to a pot can be accepted as consequence of a delay of mental development. Careful urological inspection allows to avoid diagnostic mistakes and in due time to prescribe necessary treatment.


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Scientific source Semenova Natalia Aleksandrovna. STATE OF HEALTH of CHILDREN With the SYNDROME of Down. The dissertation on competition of a scientific degree of the candidate of medical sciences. Moscow - 2013. 2013

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