ABSTRACT
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.
Subject(s)
Adrenergic beta-Antagonists/therapeutic use , Amino Acids/urine , Ehlers-Danlos Syndrome/urine , Marfan Syndrome/urine , Vitamins/therapeutic use , Adolescent , Child , Child, Preschool , Creatinine/urine , Ehlers-Danlos Syndrome/drug therapy , Humans , Hydroxyproline/urine , Infant , Marfan Syndrome/drug therapyABSTRACT
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.
Subject(s)
Anesthesia, Epidural/adverse effects , Funnel Chest/surgery , Postoperative Complications/etiology , Thoracoplasty/adverse effects , Adolescent , Child , Child, Preschool , HumansABSTRACT
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).
Subject(s)
Adrenal Cortex/physiopathology , Connective Tissue Diseases/physiopathology , Funnel Chest/physiopathology , Adolescent , Adrenal Cortex/drug effects , Adrenal Cortex Function Tests , Child , Child, Preschool , Connective Tissue Diseases/drug therapy , Funnel Chest/drug therapy , Glucocorticoids/biosynthesis , Glucocorticoids/therapeutic use , HumansABSTRACT
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.
Subject(s)
Ehlers-Danlos Syndrome/immunology , Funnel Chest/immunology , Immunologic Deficiency Syndromes/complications , Marfan Syndrome/immunology , Surgical Wound Infection/etiology , Thoracoplasty/adverse effects , Adolescent , Child , Child, Preschool , Ehlers-Danlos Syndrome/complications , Funnel Chest/etiology , Funnel Chest/surgery , Humans , Marfan Syndrome/complicationsABSTRACT
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.
