[Correction of funnel chest by using a magnetic system of permanent samarium-cobalt magnets].
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Adolescent↗
Biomedical subjects
S S Rudakov
Publications and source records attributed to S S Rudakov.
A new method of surgical treatment of funnel chest with help of permanent magnets.
35 patients have benefited from the following procedure: After a double chondrotomy accompanied if need be by resection of the costal cartilages and a sternotomy, a magnet is placed in the retrosternal space. The wound is closed. A second magnet is placed externally on special corset. The magnetic chest expansion is achieved in between one and one and a half months. The internal magnet is removed six months after the operation. Its authors claim a series of 33 good results, without any relapses, after a follow-up of 2 1/2 years.
Funnel Chest↗
[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].
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.
Adolescent↗
[Structural characteristics of collagens from the skin and rib cartilage of patients with Ehlers-Danlos syndrome type II].
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.
Cartilage↗
[The effect of collagen-normalizing therapy on the incidence and severity of anesthetic and postoperative complications in children with connective tissue syndromes].
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.
Adolescent↗
[Molecular heterogeneity of proteoglycan aggregates of human hyalin cartilage in normal conditions and in systemic bone dysplasia].
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.
Cartilage↗
[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].
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.
Cartilage↗
[Effect of therapy with beta-adrenoblockers and vitamin complexes on indices of oxyproline excretion in various hereditary connective tissue diseases].
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.
Adolescent↗
[Relation between infections during the pregnancy and the rate of hydroxyproline excretion in children with congenital chest deformities].
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.
Adolescent↗
[Extractability of collagen from the rib cartilage and skin in funnel chest in children].
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.
Cartilage↗
[Urinary excretion of hydroxyproline in funnel chest deformity].
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.
Adolescent↗