[Comparative evaluation of the methods of surgical treatment of congenital clubfoot in children]. / Sravnitel'naia otsenka metodov operativnogo lecheniia vrozhdennoi kosolaposti u detei.

The observation and treatment of 142 children have shown that most favorable results were obtained in operations on soft tissues of the foot not involving the integrity of tendons of the peroneal group of muscles. The method developed by the authors is the most physiological one and contributes to the quickest recovery of functions of the foot and talocrural articulation.

Cystinosis phenotypes have identical defective cystine clearance pattern.

Exposure of cultured skin fibroblast of normal, infantile nephropathic, juvenile-late-onset and adult type cystinotic patients and their corresponding obligate heterozygotes to 0.5 mmol/l of 35S cystine dimethyl ester for 30 minutes, resulted in an accumulation of cystine within the cells, and was used to look for differences in cystine clearance between the different cell types. The results suggested that all cystinotic variants are defective in their capacity to eliminate cystine to the same extent. The presented data imply that, separate clinical phenotypic variants of cystinosis, cannot be differentiated biochemically by the assay of cystine egress.

[Surgical treatment of benign bone tumors in children]. / Khirurgicheskoe lechenie dobrokachestvennykh opukholei kostei u detei.

Based upon the data obtained in analyzing results of the surgical treatment of 248 children with different benign tumors of the skeleton the authors consider that the operation volume depends on the kind, localization and spread of the pathological process in the bone. The method of autoplasty gives the best results in substitution of bone defects after removal of the tumor in the children age.

Cystine accumulation and clearance in normal and cystinotic fibroblasts exposed to cystine dimethyl ester.

Exposure of cultured skin fibroblasts of normals and cystinotic patients to 0.5 mmol/l[35S]cystine dimethyl ester for 30 min resulted in an accumulation of cystine in excess to that naturally occurring in cystinotic skin fibroblasts. These equal levels of cystine accumulation achieved in both cystinotic and normal cells, permitted comparative experiments to look for differences in cystine disposal between normal and cystinotic cells. Cystinotic fibroblasts demonstrated very low cystine clearance with a lower ratio of cysteine-N-ethylmaleimide to cystine than normal. The results on cystinotic fibroblasts are consistent with those observed in leucocytes, suggesting that fibroblasts can be useful in further studies to elucidate the clearance defect of cystine in cystinosis as well as its potential in antenatal diagnosis.

Comparative study of cystine clearance in cystinotic and I-cell fibroblasts upon exposure to cystine dimethyl ester.

I-cell fibroblasts can accumulate cystine at levels comparable to those seen in homozygous cystinotic fibroblasts. Cystine accumulation in cystinosis is accounted for cystine clearance defect in situ. To unravel the question whether the same clearance defect or two different mechanisms cause cystine accumulation in I-cell disease, we used the cystine loading technique upon exposure of skin fibroblasts to radioactive cystine dimethyl ester. Normal, cystinotic and I-cell fibroblasts were exposed to radioactive cystine dimethyl ester, and the clearance of the generated radioactive cystine was measured. Cystinotic cells showed a marked defect in cystine clearance in situ, as compared to normal fibroblasts. In I-cell fibroblasts, we observed slow hydrolysis of cystine dimethyl ester to cystine, indicating low esterase activity, but no defect in clearance of the generated cystine. Cysteine production from the exogenous cystine dimethyl ester, presumably by cytoplasmic hydrolysis of the generated cystine, is normal in I-cell fibroblasts. Thus, our results indicate that, unlike cystinosis, there is no cystine clearance defect in situ for cystine in I-cell disease, and probably unrelated mechanisms cause cystine storage in cystinosis and I-cell disease.

Isolation and partial characterization of proteins from platelet pseudopods.

Isolated pseudopods of human platelets activated by ADP, thrombin, or low temperature contain, as a major constituent, a 70,000-dalton membrane protein. This protein, which is labeled in intact platelets by [125I]iodonaphthylazide, is either pushed out of the membrane plane during activation or undergoes a conformational change or, as a result of activation, is aggregated in the pseudopod region. The relative amount of actin present in the pseudopod fraction is higher in the case of thrombin activation than that of ADP- or temperature-induced activation. The relative amounts of glycoproteins IIb and III in these pseudopod fractions were found to be similar with each of the preparative methods used and also similar to their relative concentrations in whole platelets.

[Results of treatment of postoperative bronchopleural complications in lung cancer]. / Rezul'taty lecheniia posleoperatsionnykh bronkhoplevral'nykh oslozhnenii pri rake legkogo

An analysis of the results of treatment in 138 patients with bronchopleural complications after 1400 operations on the lung for cancer is given. Conservative methods of therapy (punctures and drainage of the pleural cavity, instillation and of the fisula with 30% silver nitrite and intratracheal injections of antibiotics) were utilized in 105 patients with bronchial fistulas and pleural empyemas. Thirty there patients were treated surgically (tamponade, thoracoplasty, rethoracotomy with suturing of the bronchial fistula, transsternal suturing of the bronchial fistulas). The rate of mortality from bronchopleural complications was 4.8%, i.e. one third of all causes of death. However, recently it reduced more than twice in comparison with the initial period. The direct causes of lethal issues are complications on the part of other organs: pneumonia of the remained lung (24), septicopyemia (12), hemorrhage (10), and others (19).