[Mitochondrial dysfunction in children with hepatic forms of glycogen storage disease].

AIM: The purpose of the study was to assess mitochondrial dysfunction severity in patients with hepatic forms of glycogen storage disease (GSD). PATIENTS AND METHODS: We examined 53 children with GSD in the dynamics. Distribution of children by disease types was: 1st group--children with GSD type I, 2nd group--children with GSD type III, 3rd group--children with GSD type VI and IX; comparison group consisted of 34 healthy children. Intracellular dehydrogenases activity: succinate dehydrogenase (SDH), glycerol-3-phosphate-dehydrogenase (GPDH). nicotinamideadenin-H-dehydrogenase (NADH-D) and lactatdehydrogenase (LDH) was measured using the quantitative cytochemical method in the peripheral lymphocytes. RESULTS: It was revealed decrease of SDH- (p < 0.001) and GPDH-activities (p < 0.001), along with increase of the NADH-D activity (p < 0.05) in all patients with GSD, (SDH/ NADH-D) index was decreased (p < 0.001). LDH activity was increased in groups 1 (p < 0.05) and 3 (p < 0.01), compared with comparison group. The most pronounced intracellular enzymes activity deviations were observed in children with GSD type I, that correspond to more severe clinical form of GSD. It was found strong correlation between intracellular enzymes activity and both hepatomegaly level (R = 0.867) and metabolic acidosis severity (R = 0.987). CONCLUSION: Our investigation revealed features of mitochondrial dysfunction in children with GSD, depending on the GSD type. Activities of lymphocytes enzymes correlates with the main disease severity parameters and can be used as an additional diagnostic criteria in children with hepatic form of GSD.

[Zinc and copper blood plasma and erythrocyte value in adolescent with the different types of pathology].

Comparative analysis the value of the cation of zinc and copper in the blood plasma and erythrocytes were performed in the different diseases in children and adolescent. The patients with bronchopulmonary diseases, liver diseases, low cardiovascular malformation and growth inhibition were examined. Increase the value of the intracellular zinc and copper was detected in patients with the definite bronchopulmonary diseases, which can on the one hand, reflected the activation of the antioxidant protection system and by another hand, reflected destructive metalloproteinase. Decrease the value of the intracellular copper in bronchopulmonary patients with the lung emphysema and Kartagnera syndrome indicate about the falure of the compensatory reactions and needs the additional investigation. It was detected that copper content in the blood plasma and the copper accumulation in erythrocyte were decrease in patients with liver diseases in comparison with the health children. The increase of copper value in erythrocyte in children with low cardiovascular malformation was detected and need the additional investigation. In children with the growth inhibition was detected appreciable decrease the value of the free zinc and copper ions in erythrocytes and copper in the blood plasma, which can explained the physical developmental lagging.

[Differential diagnosis of neoplasms of the lacrimal gland using ultrasonic scanning]. / Differentsial'naia diagnostika novoobrazovanii v oblasti sleznoi zhelezy metodom ul'trazvukovogo skanirovaniia.

The paper describes results of ultrasound scanning in 32 patients with new formations in the lacrimal gland area, using a device "Ophthalmoocuscan, Model 200 A" (USA), with ultrasound fluctuation frequency--10 mHz, exposition time of probing impulse--0.2 microseconds. It was found that in case of lacrimal gland pathology the highest information value is received when the probe is placed at the superior external margin of the orbit and its scanning surface is directed on the site of the lacrimal gland projection. Characteristics of scanograms of different histologic types of new formations in the lacrimal gland area described: those of pleomorphic adenoma, adenocarcinoma, lymphoma, inflammatory pseudotumor, retention cysts. Typical scanograms were obtained in 78.1% of patients. The obtained characteristics of scanograms are recommended to be used in a complex with other methods for differential diagnostics of lacrimal gland new formations of different genesis.

[The degree of malignancy and late treatment results in epithelial tumors of the lacrimal gland]. / Stepen' zlokachestvennosti i otdalennye rezul'taty lecheniia épitelial'nykh opukholei sleznoi zhelezy.

The paper gives information on the degree of malignancy and effectiveness of treatment in 120 patients with epithelial tumors of the lacrimal gland. According to WHO histologic classification (1984), the following histologic types of tumors were verified: pleomorphic adenoma--in 47 patients; carcinoma in pleomorphic adenoma--in 10; myxoma--in 2; mucoepidermoid tumor--in 2; adenocarcinoma--in 44; adenocystic carcinoma--in 15 patients. The treatment resorted to was surgical (46 patients) and combined (74 patients). It is established that malignancy degree of epithelial tumors of the lacrimal gland is revealed by its ability to recur (39.2 +/- 4.5%), locally destructive growth leading to the tumor spreading into the cranial cavity and the patient's death as a result of a damage of vital brain centers (60 +/- 9.1%) as well as by its hematogenic metastasizing (33.3 +/- 8.8%). The incidence of recurrences of a tumor and survival of patients depend on the degree of anaplasia of the tumor and its stage of development determined by the clinicoanatomic classification by TNM-system. The treatment of malignant forms of epithelial tumors of the lacrimal gland should be combined, as it twice prolongs the average lifetime of the patients.