ABSTRACT
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.
Subject(s)
Ehlers-Danlos Syndrome/complications , Funnel Chest/surgery , Marfan Syndrome/complications , Age Factors , Child , Child, Preschool , Female , Humans , Male , Orthopedic EquipmentABSTRACT
Parallel measurements of pyridinoline content, the ratio of soluble:insoluble collagen, and the percentage of endogenous collagenolysis were made in samples of costal cartilage from forty-five children with funnel chest (FC) and from twenty-two control children. From an analysis of the influence of different factors, such as FC type (isolated or syndromic), a diagnosed syndrome, extent of FC depression (second or third), and age, two biochemical variants of FC-variants a and b, which were not related to the presence or absence of a known concurrent syndrome, were distinguished. These variants differed from each other in all the parameters under study. Variant a occurred about five times less frequently than b, and was characterized by a pyridinoline content of about 50% of that of b, an elevated soluble:insoluble collagen ratio, and an increased percentage of endogenous collagenolysis compared to controls. For variant b, the pyridinoline content fell within normal limits, but the soluble:insoluble collagen ratio, and the percentage of endogenous collagenolysis were below normal. The data suggest that the formation of variant a may be related to defect(s) in collagen crosslinking, whereas the formation of variant b may result from other unknown factor(s) involved in the formation and maturation of costal cartilage.
Subject(s)
Cartilage/chemistry , Collagen/chemistry , Funnel Chest/metabolism , Adolescent , Amino Acids , Child , Child, Preschool , Collagen/metabolism , Humans , SolubilityABSTRACT
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.
