[The radical minimal access thoracoplasty by funnel chest in adults].

A new technique of radical thoracoplasty, performed with a small size incision, using the sternocostal complex fixation with nitinol plates with shape memory and the surgical toolkit required for the purpose, has been successfully introduced. 23 patients underwent surgery using the technique. Tree patients had early postoperative complications: 2 cases of exudative pleurisy and 1 case of bronchitis aggravation. Good cosmetic results was achieved in all operated patients. The mechanical ground of the technique is based on the use of the effective force of the sternum traction and the pressure of the plate on the tissues below. The main advantage of the method is avoidance of the transverse sternotomy.

[Radical thoracoplasty by the funneled thorax malformation in adults].

Early and long-term results of radical thoracoplasty in 114 adult patients with funnel chest are presented. The Sulamaa-Paltia operation was performed in 24 patients, thoracoplasty with sternocostal complex fixing by arcuated nitinol plates was made in 30 patients. Thoracoplasty with sternocostal complex fixing by M-shaped nitinol plates was performed in 60 cases. Two patients after Sulamaa-Paltia operation demonstrated the plate displacement, which caused the relapse of the disease. Those patients, who had other techniques applied, showed no signs of relapse. Thus, Sulamaa-Paltia operation allowed good results in 83,3% of the treated patients; the use of arcuated and M-shaped plates showed good results in 93,3 and 98,3%, respectively.

[Indications for surgery and methods of surgical correction of infundibuliform abnormality of the chest in children]. / Pokazaniia k operatsii i metody khirurgicheskoi korrektsii voronkoobraznoi deformatsii grudnoi kletki u detei.

Based on the survey of 104 patients, a method has been developed for early diagnosis of progression of infundibuliform chest deformity (ICD), which defines indications for thoracoplasty in children over 2 years. A differential approach has been applied to the stabilization of the sternocostal complex, taking into account various ICD types. Experience in surgical management of 247 patients with simple and complex ICD types has been generalized. A procedure has been improved to stabilize the sternocostal complex with a metallic plate in critical ICD types. The sparing thoracoplasty variants have been developed for simple ICD types and Degree I progressive ICD ones, stabilizing the sternocostal complex with a niticolic brace and a CPK-22 apparatus in the modified resistant case.

[Surgical correction of early forms of funnel chest in children with Marfan's and Ehlers-Danlos syndromes]. / Khirurgicheskaia korrektsiia rannikh form voronkoobraznoi deformatsii grudnoi kletki u detei s sindromami Marfana i Elersa-Danlo.

A prophylactic trend was developed in surgical treatment of funnel chest on basis of a syndromal approach to solution of applied problems of clinical medicine. Patients with an established diagnosis of progressive funnel deformity underwent early prophylactic sparing one-stage thoracoplasty according to the authors' own method, which made it possible to prevent progress of the deformity in the early stage; such treatment had a positive effect on further physical and mental development of the child. Besides, the method of operation had a lesser injurious effect and reduced the number of complications; it also considerably reduced the expenditures and shortened the period of treatment.

[Excretion of pyridinoline with urine in children with Ehlers-Danlos syndrome and Marfan's syndrome and the effect of beta blocker and complex vitamin therapy on it]. / Ekskretsiia piridinolina s mochoi u detei s sindromami Elersa-Danlo i Marfana i vliianie na nee terapii beta-adrenoblokatorom i kompleksom vitaminov.

Excretion of pyridinoline and polypeptide-bound hydroxyproline with urine was studied in 27 children with hereditary impairment of connective tissue. At the same time, effects of beta-adrenoblocking agents and vitamin complex, prescribed during preoperation period before thoracoplasty in hereditary chest deformation, were investigated. Clinical efficiency of the treatment depended distinctly on the initial value of ratios pyridinoline/creatinine and polypeptide-bound hydroxyproline/creatinine. Total positive effect of the therapeutic course, considering also echocardiographic examination and postoperational complications, was observed in 44% of patients with Ehlers-Dunlop syndrome and in 75% of patients with Marfan syndrome.

[Anesthesiologic and postoperative complications in children with isolated forms of funnel chest]. / Anesteziologicheskie i posleoperatsionnye oslozhneniia u detei s izolirovannymi formami voronkoobraznoi deformatsii grudnoi kletki.

In thoracoplasty for isolated funnel-shaped deformity of the chest in children, the main anesthesiologic complications which develop are the following: delayed restoration of muscular tonicity and adequate respiration and disorders in cardiac rhythm. At the postoperative period, the complications, are mainly caused by injury to parietal pleura at intervention. The pulmonary, cardiac, gastroenterologic and hemorrhagic complications were observed less often.

[Adrenocortical function in children with connective tissue syndromes and funnel chest deformation]. / Funktsional'noe sostoianie kory nadpochechnikov u detei s soedinitel'notkannymi sindromami i voronkoobraznoi deformatsiei grudnoi kletki.

Measurement of the content of C-21-corticosteroids in the diurnal urine by thin-layer chromatography has demonstrated that in children with isolated and syndromal forms of funnel-like chest deformation, grades II and III, adrenocortical function is decreased. In preschool children, such a decrease is accompanied by proneness to the tense biosynthesis of glucocorticoids. Children with congenital hereditary connective syndromes manifest adrenocortical dysfunction, most characteristic of Marfan's syndrome, and, to a less degree, of Ehlers-Danlos syndrome and unclassified complex of developmental defects with the Marfan-like phenotype. In view of this fact, the children with Marfan's, Ehlers-Danlos syndromes and with the unclassified complex of developmental defects with the Marfan-like phenotype and children with funnel-like chest deformation, grades II and III, should be given glucocorticoid hormones in stressful conditions (severe diseases, traumas, operations).

[Early diagnosis of progression of funnel chest in children]. / Ranniaia diagnostika progressirovaniia voronkoobraznoi deformatsii grudnoi kletki u detei.

104 patients with progressive forms of funnel chest deformity of the II-III degree with Ehlers-Danlos-Marfan syndrome, Marfan-like phenotype, isolated funnel chest deformity and unclassified funnel chest deformity of the first degree were subjected to synromologic examination. There were detected 10 dysplastic signs, indicating funnel chest deformity progression: anti-Mongol shape of the eyes, arachnodactyly, high palate, Ehlers-Danlos syndrome, floor of the auricle's dysplasia, dolichostenomely, posture disturbance, mitral valve prolapse, umbilical hernia, wide filter. The results of biochemical examination of collagen metabolite--hydroxyproline++ (the first and the second hydroxyproline++ fraction ratio disturbance, decrease of the first hydroxyproline++ fraction percentage, disturbance of direct correlation between total hydroxyproline++ and percentage of the first hydroxyproline++ fraction)--corroborated the clinical data.

[Immunologic disorders in children with developmental thoracic defects]. / Immunologicheskie narusheniia u detei s defektami razvitiia grudnoi kletki.

As the result of immunological examination of 21 children with developmental defects of the chest and analysis of the course of the postoperative period in 136 children, among which 36 had hereditary syndromes of systemic connective-tissue dyshistogenesis, it was found that suppurative complications of thoracoplasty, which are encountered in 15% of children with isolated developmental chest defects and in 33.3% of those with the above mentioned syndromes, were caused to a great measure by disorders of the immune status. The most serious immunological deviations were encountered in the Marfan syndrome due to impaired phagocytic activity of neutrophils and monocytes, decreased number of T, T active, and B lymphocytes, and diminished function of T helpers. In unclassified complexes of developmental defects with Marfaneic ++ phenotypes, the immunological disorders were similar, but less deep. In the Ehlers-Danlos syndrome, a decrease of the number of immunocompetent cells, function of T helpers, and neutrophils was mainly revealed. In isolated forms of funnel chest the function of monocytes and the number of immunoglobulins are mainly decreased.

[The effect of collagen-normalizing therapy on the incidence and severity of anesthetic and postoperative complications in children with connective tissue syndromes]. / Vliianie kollagenonormalizuiushchei terapii na chastotu i tiazhest' anestesiologicheskikh i posleoperatsionnykh oslozhenii u detei s soedinitel'notkannymi sindromami.

A new method of collagen-normalizing therapy has been worked out to prevent specific anesthesia-induced, operative and postoperative complications and to treat some symptoms in children with Ehlers-Danlos and Marfan's syndromes and with non-classified Marfan-like malformations. The technique involves a combined use of beta-adrenoblocker in the age-matched doses and vitamins C/0.03 g/(kg.day)/, B2/0.0004 g/(kg.day)/and B6/0.002 g/(kg.day)/ for 2.5 months before surgery and during the first 2 weeks of the postoperative period. The above therapy reduces the incidence of delayed recovery of the muscular tone and adequate respiration, spontaneous and recurrent pneumothorax, hemorrhagic and gastroenterologic complications, as well as the incidence and severity of intestinal paresis. The indexes of effective collagen-normalizing therapy are as follows: body weight increase, echocardiographic pattern of reduced diameter of the aorta and mitral valve prolapse, normalization of the urinary excretion of total and polypeptide-bound oxyproline. The efficacy of therapy depends on the baseline level of oxyproline excretion.

[Structural characteristics of collagens from the skin and rib cartilage of patients with Ehlers-Danlos syndrome type II]. / Strukturnye kharakteristiki kollagenov kozhi i rebernogo khriashcha bol'nykh s sindromom Elersa-Danlo II tipa]

Collagens were analyzed in skin and rib cartilage of 9 patients with Ehlers-Danlos syndrome of the II type. Electrophoresis and CNBr-peptide mapping showed that extended inserts and deletions as well as rough impairments of post-translation processing were not detected in collagens of the I, II and III types from these patients. In the patients with Ehlers-Danlos syndrome of the II type distinct increase was observed both in the total ratio of collagens III/I (P = 0.95) and in the ratio of intact collagens III/I free of cross-links. A decrease in content of dimers beta 11 and beta 12 was found in two patients. The data obtained suggest that the Ehlers-Danlos syndrome of the II type involved deteriorations in the structure of collagens I responsible for decrease in stability and sometimes for impairments in cross-link formation. Increase in content of collagen II fraction, predisposed to proteolytic hydrolysis of terminal sites, as well as elevated sensitivity of collagen II to pepsin hydrolysis were found in collagens of rib cartilage from patients with the syndrome and with funnel chest deformation. This suggests the lowered stability of collagen II from rib cartilage in funnel chest deformation.

[Changes in the structure of type I collagen and cross-links between type I and type III collagen chains in a patient with funnel chest]. / Narushenie struktury kollagena I tipa i nalichie sshivok mezhdu tsepiami kollagenov I i III tipa u bol'nogo s izolirovannoi formoi kilevidnoi deformatsii grudnoi kletki.

Skin and rib cartilage collagens were studied in patient 1.I.K. with isolated form of pigeon chest as well as in a group of children without any impairments of connective tissue. Distinct decrease in stability of collagen I, an increase in the ration of alpha 1 (I)/alpha 2(I) chains and impairment in formation of beta 12 dimers were detected in the patient with pigeon chest. In the patient skin total ratio between collagens I and III, calculated from a content of BrCN-peptides, was similar to normal level, whereas the proportion was markedly increased between intact molecules of collagens III and I free of cross-links, which was calculated from the ratio of alpha 1(III)/alpha 2(I) chains. Presence of cross-links between alpha 1 (III) and alpha 2 (I) chains as well as between alpha 1 (III) and alpha 2 (I) chains was detected after peptide mapping of polypeptides arranged in the region of beta 11 and beta 12 dimers. All the collagen I preparation, extracted from skin of the patient 1.I.K., contained molecules with unstabilized N-terminal sites. These results suggest that mutation occurred in the N-terminal region of alpha 1(I) chain. Analysis of collagen from the patient 1.I.K. rib cartilage demonstrated a slight decrease in total stability of collagen II as well as elevated concentration of collagen II molecules containing unstabilized N-terminals. Mechanisms responsible for formation of cross-links between polypeptide chains of collagens I and III detected in human skin are discussed.

[Molecular heterogeneity of proteoglycan aggregates of human hyalin cartilage in normal conditions and in systemic bone dysplasia]. / Molekuliarnaia geterogennost' komponentov proteoglikanovykh agregatov gialinovogo khriashcha cheloveka v norme i pri sistemnykh kostnykh displaziiakh.

Components of proteoglycan aggregates of human hyalin cartilage were studied under conditions of normal state and in some forms of osteochondrodysplasia. Extraction of uronic acids and protein from the tissue, amount of fractions and electrophoretic mobility of proteoglycan monomers, rations protein/glycosaminoglycans, keratan sulfate/chondroitin sulfate, a level and type of sulfatation as well as molecular mass of chondroitin sulfate, amino acid composition of rod protein, heterogeneity of binding proteins (concerning their isoelectric points and molecular masses) and immunoreactivity of protein moiety in proteoglycan aggregates were studied in rib cartilage, knee joint and ala ossis ilii. Structural parameters of proteoglycan aggregates proved to be dissimilar and depended on cartilage localization and age of the donors. Impairments in the rate of chondroitin sulfate sulfatation were detected in achondrogenesis of the II type and in diastrophic dysplasia; an extraction ability and amount of proteoglycan fractions, relative content of glycosaminoglycans and binding proteins were altered in some other forms of osteochondrodysplasias. Numerous biochemical markers of extracellular matrix deterioration were detected, which are typical for various morphofunctional alterations in hyalin cartilage--hyperproliferative reactions, tissue prematuration, persistence of the embryonal type of metabolism.

[Effect of therapy with beta-adrenoblockers and vitamin complexes on indices of oxyproline excretion in various hereditary connective tissue diseases]. / Vliianie terapii beta-adrenoblokatorom i kompleksom vitaminov na pokazateli ékskretsii oksiprolina pri nekotorykh nasledstvennykh bolezniakh soedinitel'noi tkani.

Excretion of hydroxyproline with urine was studied in 16 children (5-14 years old) with Marphan-Like syndrome and Marphan, Ehlers-Dunlos and Larson syndromes after therapy involving propranolol and a complex of vitamins (ascorbic acid, riboflavin and pyridoxine) and recommended on the basis of echocardiographic analyses. The therapeutic course appears to cause quantitative and qualitative correction of collagen and apparently of elastin fibrilles development. Depending on initial patterns of hydroxyproline excretion and the syndrome form the correction could be complete or partial, while positive effect of the treatment was stable or provisional. The data obtained suggest that the complex treatment developed might be applied as a preoperative therapy of the patients with Marphan-like syndrome as well as with syndromes of Marphan and Ehlers-Dunlos before thoracoplastics caused by hereditary chest deformation and by impairments of cardiovascular system.

[Relation between infections during the pregnancy and the rate of hydroxyproline excretion in children with congenital chest deformities]. / Issledovanie sviazi mezhdu infektsiiami v period beremennosti i urovnem ékskretsii oksiprolina u detei s vrozhdennymi deformatsiiami grudnoi kletki.

In a group of women, which had children with hereditary isolated and syndromal chest deformations, acute respiratory diseases, tonsillitis or influenza occurred during pregnancy in 42.9 +/- 6.6% and 53.8 +/- 13.8% of cases, respectively, whereas in the control group only 17.8 +/- +/- 7.2% of the women were impaired with identical infectious diseases during pregnancy (P less than 0.02 and P less than 0.05, respectively). Excretion of hydroxyproline was distinctly dissimilar in 22 children with isolated and in 13 children with syndromal chest deformations depending on presence or absence of the above-mentioned infections during pregnancy. Under conditions of these infections the higher level of total hydroxyproline excretion was noted as well as relatively lower content of bound hydroxyproline was detected in those peptides, which appear to be responsible for the content of newly synthesized collagen. The data obtained suggest that acute respiratory diseases, influenza, chronic and acute tonsillitis may impair collagen metabolism in children with hereditary chest deformations as well as that these infectious diseases occurred during pregnancy may increase the risk of the pathology development in the children.