[Thermodynamic functions of lysozyme, myoglobin, beta-lactoglobulin, albumin, and poly-L-tryptophane in the temperature range 0-330 K]. / Termodinamicheskie funktsii lizotsima, mioglobina, beta-laktoglobulina, al'bumina i poli-L-triptofana v diapazone temperatury 0-330 K.

The temperature dependence of heat capacity for lysozyme, myoglobin, beta-lactoglobulin, albumin, and poly-L-tryptophan was studied in the temperature range 5-330 K using an adiabatic vacuum calorimeter. The accuracy of measurements was approximately 0.2%. From the data obtained, the thermodynamic functions of these proteins and the polyamino acid Cp degree (T), H degree (T)-H degree (0), S degree (T)-S degree (0), G degree (T)-H degree (0) in the temperature range 0-330 K, and the values of fractal dimension D and intrinsic temperature theta max were calculated.

[Disorders of the cerebrospinal fluid dynamics in young children with pathology of the nervous system]. / Narusheniia likvorodinamiki u detei rannego vozrasta s patologiei nervnoi sistemy.

Two groups of patients with abnormalities of the cerebrospinal fluid dynamics were examined. There were 40 children with the hypertensive hydrocephalic syndrome and 67 with active hydrocephalus that had occurred before two years of age. A study was made of the anamnestic, clinical, ultrasonic and computer-aided tomographic parallels of those conditions. In the first group, computer-aided tomography of the brain revealed a moderate enlargement of the cerebrospinal fluid space. Two-dimensional ultrasonic examination of the brain via the open large fontanel also demonstrated the widening of the frontal cornua of the lateral ventricles and of the III ventricle. The second group children manifested an appreciable enlargement of the cerebrospinal fluid system, while the occlusive character of cerebrospinal fluid abnormalities required surgical treatment (drainage via the shunting system). A table of the differential diagnosis of the clinical and preclinical symptoms recorded in the two patients' groups is presented.

[Diagnosis and treatment of the convulsive syndrome in children with a history of closed craniocerebral injury]. / O diagnostike i lechenii sudorozhnogo sindroma u detei, perenesshikh zakrytuiu cherepno-mozgovuiu travmu.

In 170 children aged 3 to 14 the convulsive syndrome was studied at the acute, rehabilitation and remote stages of the nonpenetrating head trauma (NHT). The patients were divided into groups having permanent seizures of these appearing after a certain seizure-free stage. Characteristic shifts in hemodynamic, cerebrospinal-fluid dynamic indices, sympathoadrenal system activity and electrolyte balance were shown in each group. With these leading pathogenic mechanisms of NHT-induced convulsive syndrome elucidated, and effective method of their treatment was introduced.

[Clinical, biochemical and electroencephalographic findings in children with a post-traumatic convulsive syndrome]. / Klinicheskie, biokhimicheskie i élektroéntsefalograficheskie dannye pri posttravmaticheskom sudorozhnom sindrome u detei.

Clinical, biochemical and electroencephalographic examinations were carried out in 96 children with the post-traumatic convulsive syndrome. The time of the development of convulsive syndrome and its clinical manifestations are reviewed. In 75 children, convulsive attacks took the course of traumatic arachnoiditis; in 21 children, the clinical picture of the disease with consideration for the characteristics and frequency of convulsive seizures and changes in the character of the children was suggestive of epilepsy development. Possible pathogenetic mechanisms of the development of convulsive attacks in children who have had a closed craniocerebral injury are analyzed.

[Status of the sympathetic-adrenal system in the initial and early periods of closed craniocerebral injuries in children]. / Sostoianie simpatiko-adrenalovoi sistemy v nachal'nom i rannem periodakh zakrytoi cherepno-mozgovoi travmy u detei.

The authors studied the interaction between the sympathoadrenal system, hemodynamics and respiration in the initial and early periods of closed craniocerebral injury in children, taking into account specific features of the clinical course. As regards the ratio between irritation and inhibition symptoms, three variants of the course of the initial period were elicited, namely; 1) a "smooth course", 2) one with psychomotor excitation and 3) one with "dissociation" of emotional and motor activity. Statistically significant changes in catecholamine metabolism characteristic of the initial and early periods as well as those of every variant of the course were established. The role of vascular insufficiency and of a decreased respiration rate contributing to a drop in the saturation of the arterial blood with oxygen was specified. Corrective impact of some drugs on different links of the changes observed were studied and their optimal combinations were tried.

[Autonomic reactions in patients with infantile cerebral palsy]. / Osobennosti vegetativnykh reaktsii u bol'nykh detskim tserebral'nym paralichom.

The authors examined the neurovegetative reactions in 20 children aged 2 to 5 years suffering from infantile cerebral paralysis. The children examined had suffered perinatal hypoxia and natal intracranial injuries. Combined examinations of the cardiovascular, respiratory, and thermoregulatory systems were carried out in standard conditions in a microclimate chamber. Various types of the response of the above systems to the orthostatic, solar, and oculocardiac loads were revealed. The disturbances of the homeostatic functions of the central and peripheral genesis on the one hand, and mode-rate hypertensive-hydrocephalic syndrome on the other were found to be in parallel. On the basis of the data of their experimental study the authors conclude that in the children examined the higher vegetative centres responsible for the body homeostasis have suffered a hypoxic damage.

[Neurovegetative and hypothalamic syndromes in children with infantile cerebral palsy]. / Neirovegetativnyi i gipotalamicheskii sindromy u bol'nykh detskim tserebral'nym paralichom.

An analysis of the neuropsychic and vegetative status of 108 children aged 3 months to 7 years suffering from infantile cerebral paralysis has shown that in a great part of the patients a neurovegetative or hypothalamic syndrome can be additionally specified. An analysis of the totality of the background vegetative characteristics shows that the effection of this division of the nervous system is of a mixed character. Different direction of the vegetative reactions, i.e. sympathetic or parasympathetic, can be noted in different forms of the paralysis. The neurovegetative syndrome can be discerned in children with a noticeable psychic defect, while the hypothalamic one in children with a good psychic development.

[Relationship between the severity of brain damage in phenylketonuria and the degree of hyperphenylalaninemia]. / Zavisimost' tiazhesti porazheniia mozga pri fenilketonurii ot stepeni giperfenilalaninemii.

The frequency of the various degrees of the intellectual defect and convulsive syndrome was studied in 473 patients with phenylketonuria divided into four groups as regards the phenylalanine blood level, that was 150 to 199 mg/l in group I, 200 to 299 mg/l in group II, 300 to 399 mg/l in group III, and 400 mg/l and higher in group IV. A substantial increase of the number of patients with grave mental derangement and epileptic seizures was noted in groups II and IV: this shows that the risk of the grave mental affection is the greater the higher the phenylalanine serum level. An increased number of patients with a slight intellectual derangement in group I (43.2%) was noted, while in all other groups, the frequency of the mild course of the disease remained stable (about 7.5%). Possibility of existence of several pathogenic thresholds of hyperphenylalaninemia, as well as possible mechanisms of brain protection are discussed.

[Clinico-genealogic analysis of 2 families with an unusual syndrome combining dwarfism with dysostosis of the facial cranium and pyramido-extrapyramidal pathology]. / Kliniko-genealogicheskoi analiz dvukh semei so svoeobraznym sindromom sochetaniia karlikovosti s dizostozom litsevogo cherapa i piramidno-ékstrapiramidnoi patologiei.

The presentation is concerned with a clinico-genealogical analysis of 7 patients from 2 relative famalies with inbreeding marriages. In all patients the authors observed a peculiar syndrome of combination of proportional dwarfism with dysostosis of the facial cranium and pyramidal-extrapyramidal pathology of a different degree of expressiveness. The onset of the disease was at the end of the 1st year of life with a following steady progression. The given syndrome is of great interest as a rare autosomno-recessive form of hereditary diseases which has not been described in literature.