[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.

[Abnormal structure of type II collagen in a patients with funnel chest]. / Anomaliia struktury kollagena II tipa u bol'nogo s voronkoobraznoi deformatsiei grudnoi kletki.

The electrophoretical analysis and CNBr-peptide mapping of the collagens, isolated from the costal cartilage of 30 patients with non-classified and syndromal forms of pex excavatum (funnel chest) (27 patients) and pex carinatum (3 patients) was carried out. In case of one patient with the nonclassified form of funnel chest the electrophoretical mobility of CB 9.7-peptide was found to be decreased. The electrophoretical mobilities of other peptides are not markedly changed. The data obtained allow one to suggest the mutation causing the defect in the region about 160 amino acid residues distant from the C-end of alpha 1 (II) chain of type II collagen of the patient.

[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.

[Pathohistology of costal cartilage and immunomorphologic characteristics of collagen in funnel chest]. / Patogistologiia rebernogo khriashcha i immunomorfologicheskaia kharakteristika kollagena pri voronkoobraznoi grudi.

Biopsy samples from the costal cartilage tissue were studied for 68 children with funnel deformity and from 20 children with normally formed chest. The authors present general morphologic features characteristic of the costal cartilage structure in norm and in case of funnel chest. These features include vast acellular sites, map-like areas, unmasked chondrin fibers and "marrow" cavities. In funnel chest they develop, however, 6-7 years earlier, than normally, and are consequent stages of the accelerated costal cartilage involution. Costal cartilage matrix was found to have an increased content of fibronectin and V collagen type in case of funnel chest. Besides III and IV procollagen types were noted in the chondrocytes. The accelerated growth of costal cartilages is involved in the formation of funnel chest.

[Urinary excretion of hydroxyproline in funnel chest deformity]. / Ekskretsiia oksiprolina s mochoi pri voronkoobraznoi deformatsii grudnoi kletki.

Excretion of hydroxyproline with urine was studied in 16 children with localized form of funnel chest deformation simultaneously with Marfan and Ehlers-Danlos syndromes, in 9 children with the localized form of deformation within 6-8 months after thorax surgical plastic operation as well as in 3 children with Ehlers-Danlos syndrome but without funnel chest deformation. Funnel chest deformation of the II-III degree, independently of its form, was accompanied by a decrease of total hydroxyproline in urine as compared with healthy children of the similar age. The hydroxyproline excretion was normalized after thoracoplastic operation in the children with localized form of the chest deformation. In Ehlers-Danlos syndrome, independently on presence or absence of the chest deformation, relative content of free hydroxyproline was increased in urine, while the peptide-bound amino acid was decreased (peptides with molecular mass above 700 daltons); this phenomenon appears to be a characteristic property of the syndrome.

[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.

[Extractability of collagen from the rib cartilage and skin in funnel chest in children]. / Ekstragiruemost' kollagena rebernogo khriashcha i kozhi pri voronkoobraznoi deformatsii grudnoi kletki u detei.

Content of collagen and relative content of unextractable collagen were increased by 35% and 50%, respectively, while the content of immobilized water was decreased in costal cartilage of children with the isolated form of funnel chest or with the deformation accompanied by Ehlers-Danlos syndrome. These anomalies may be considered as indicators of the cartilage premature ageing. In skin of the children with isolated form of funnel chest the ratio of collagen extracted with acetic acid was increased 2-fold.

[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.

[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.

[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.

[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.

[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.