[Syndrome-based approach to the problem solving in pediatric surgery: theoretic rationale, achievements and perspectives].
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Biomedical subjects
Publications and source records attributed to S S Rudakov.
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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.
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).
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.
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.
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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.
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.
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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.
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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.
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