Characteristics of Factors of Protozoa Blastocystis hominis Persistence.

Persistence activity manifested in the expression of anti-lysozyme, anti-lactoferrin, and antihistone factors promoting inactivation of natural anti-infection resistance factors in the body was revealed in Blastocystis hominis protozoa. Activities of these factors were ranged. The frequency of these factors in clinical isolates of blastocyst decreased in the following order: anti-lactoferrin activity (84.5±3.7%)→anti-lysozyme activity (64.8±5.7%)→anti-histone activity (48.1±2.3%). In healthy humans, the corresponding parameters were 7.3±1.3, 5.3±0.9, and 3.3±0.4%, respectively (p<0.05). It was shown that the studied activities in highly virulent blastocysts were higher than in groups of medium-, low-, and avirulent protozoa.

[Diagnostics and treatment of congenital malformations of the lung and mediastinum in newborn children and infants].

The article presents the experience of treatment of newborn children and infants with congenital malformations of the lung and mediastinum, which required a surgery. Children (138 cases) were treated during recent 18 years. There was a prevalence of full-term infants (73%). Fetal malformations were diagnosed in prenatal period in majority of cases. Computed tomography was the main method of diagnostics after delivery. Children (110 cases) were operated out of 138. Children with extrapulmonary sequestration didn't undergo surgery in case of absence of clinical manifestations. The authors made a conclusion that malformations of the lung and mediastinum should be included in number of differentiated diseases in case of respiratory distress syndrome in newborn children. The indications to early surgery should be the danger of contamination and malignant transformation, presence of intrathoracic tension syndrome in neonatal period.

[Differential diagnosis of persistent interstitial emphysema and other cystic lesions of the lung].

Neonatal interstitial emphysema (IE) may develop as a complication of the respiratory distress syndrome. IE is classified as acute and persistent and as diffuse and local forms. The differential diagnosis of persistent IE and congenital cystic malformations from clinical and imaging data may present problems. The histological pattern of persistent IE has specific features that permit the morphological diagnosis of this condition.

[Tuberculosis of the sternum in children].

Operations for tuberculosis and BCG lesion of the sternum were performed on 70 children of preschool age. Clinico-epidemiological features, results of complex X-ray investigation and complex treatment were analyzed. It was noted that in the recent years the number of children with the pathology in question became greater, the possibilities of standard roentgenography for the diagnosis are limited, computed tomography is of high diagnostic value as well as the efficiency of radical operations fulfilled against the background of combined antituberculous chemotherapy.

Experience of the use of velaxin (venlafaxine) in anxious depression.

Thirty patients (seven men and 23 women, mean age 35.3 +/- 7.8 years) with anxious and anxious-apathic depression were studied. Of these, 24 patients were treated in hospital and six in out-patient clinics. Patients were treated for eight weeks with venlafaxine at doses of 225-375 mg/day. Mental state was assessed using a series of scales (CGI, HDRS, BDI, HADS-21). A total of 27 patients (90%) completed treatment. There were 25 (83.3%) responders on the CGI scale: "improvement" in mental state occurred in 16 (59.3%) of patients and "marked improvement" occurred in nine (33.3%). "Insignificant improvement" was seen in two cases (7.4%). Complete elimination of symptoms of depression occurred in 33.3% of cases. Velaxin was found to be safe at intermediate therapeutic doses and there were improvements in laboratory results characterizing patients' somatic status.

[Defects in the cyclic adenosine monophosphate system--new hypothesis for the etiology and development of the myodystrophic process]. / Defekty sistemy tsiklicheskogo adenozinmonofosfata--novaia gipoteza vozniknoveniia i razvitiia miodistroficheskogo protsessa.

The role of the cyclic nucleotides in the development of myodystrophic process is demonstrated. It has been shown that in patients with X-chromosome-linked myopathies the metabolic defect is localized at the level of adenylcyclase (AC): this leads to a drop of the content of cyclic adenosine monophosphate (cAMP) in the muscular tissue below the threshold magnitudes of activation of protein kinases (PKs). Indications of the primary character of those changes are: absence of relationship between the degree of the enzyme activity disturbance and the disease stage; discovery of this defect in heterozygotic carriers and systemic character of the AC complex affection. In cases of autosomal myodystrophies the primary defect of the mutant gene manifests itself in an imitation of a rise of the cAMP level because of the structural peculiarities of PKs. The data obtained were used as the basis for developing an essentially new method of treating autosomal forms of neuromuscular diseases.

[Modern problems of hereditary neuromuscular diseases]. / Sovremennye problemy nasledstvennykh nervno-myshechnykh zabolevanii.

The authors outline some disputable questions of the nozological independence of different nervous-muscular diseases and their classification. Special attention is being drawn to the pathogenesis of progressive muscular dystrophy and to the importance of studying cyclic nucleotide metabolism.

[Treatment of paroxysmal myoplegia]. / Lechenie paroksizmal'noi mioplegii

The paper contains a concise review of the existing methods in the treatment of paroxysmal myoplegia. It is tressed that Kalii preparations, aldosterone antagonists and diets are not sufficient. The authors report of a stable preventive effect of acetozolamide in hypokaliemic forms of myoplegia seen in 5 patients with frequent attacks and "myopathic" symptoms in some of these cases. The possible mechanisms of the action of this preparation is discussed. Recommendations are given in respect to the treatment of hyperkaliemic variants of the disorder. A typical case history with a good improvement is given as an illustration.

[Use of an adenyl preparation for treating neuralgic amyotrophy]. / Primenenie preparata adenila dlia lecheniia bol'nykh s nevral'noi amiotrofiei.

The authors suggest that adenyl should be included into the therapeutic combination used for treating patients suffering from neural amyotrophy. The drug is to be injected intramuscularly twice a day for a month. Observations of 24 patients showed that in 23 of them positive changes could be noted. These changes manifested not only in a subjective, but also in a distinct objective increase of the muscular force and motor potentialities, and even in appearance of weak tendon reflexes in some cases. No side effects were observed.

[Pathogenesis of creatinuria and aminoaciduria in neuromuscular diseases]. / K patogenezu kreatinurii i aminoatsidurii pri nervno-myshechnykh zabolevaniiakh.

By introducing inhibitors of phosphodiestherase and adenylacyclase the authors studied the role of cyclic AMP in the pathogenesis of creatinuria and aminoaciduria in neuro-muscular diseases. It was demonstrated that in patients with Duchenne myopathy creatinuria and aminoaciduria were possibly due to a deop of nucleotide level in the muscles. And on the contrary in cases of Erb's and Landouzy-Dejerine myopathy the changes of nitrogen metabolism are due to an increased content of cyclic AMP. These data may be used for differential diagnosis of myopathy.

[Change in the ultrastructure of the muscles in thyrotoxic periodic paralysis]. / Izmenenie ul'trastruktury myshts pri tireotoksicheskom periodi cheskom paraliche

The article presents data on the clinico-morphological study of three patients suffering from thyrotoxicosis with attacks of periodic paralysis. The muscles obtained by biopsy in two patients at the moment of attack and in one patient--in the absence of attack were investigated by the electron microscopy method. The data obtained make it possible to assume that in the thyrotoxic periodic paralysis the action of the thyroid hormones causes not only impairment of the mineral metabolism, but brings about changes in the structure of the membranes of the sarcolemma and T-system, which leads to disturbances of conductance of action potential into the fibre. These changes affect the function of the end cisterns and lead to distorsion of the processes of conjugation of excitation-contraction with resulting development of paresis and paralysis of muscles. Moreover, during the attack there take place secondary disturbances of carbohydrate metabolism, which aggravates the pathological process.

[Use of lithium carbonate to treat Kugelberg--Welander spinal amyotrophy]. / Primenenie uglekislogo litiia dlia lecheniia spinal'noi amiotrofii Kugel'berga--Velandera.

On the basis of data on disturbances of the metabolism of cyclic 3',5'-adenosine monophosphate in patients with Kugelberg-Welander's amyotrophy it is suggested that lithium carbonate which is an inhibitor of adenylcyclase should be used as a medicine. It was shown that the drug exerted a stabilizing influence on the pathological process. Positive clinical and biochemical changes due to the application of lithium carbonate are noted.

[Cyclic 3--5-adenosine monophosphate metabolism in patients with Duchenne myodystrophy]. / Obmen tsiklicheskogo 3--5-adenozinmonofosfata u bol'nykh s miodistrofiei Diushenna.

The state of metabolism of cyclic adenosinmonophosphate was studied. It was established that the concentration of nucleotides was distinctly lower in patients with this disease. The activity of adenilacyclose did not differ from normal rates. The enzyme to a large degree lost its capability of reacting to hormones, which made the metabolism in tissues to a certain extent autonomous. These data permit to rise the question of conducting a correcting treatment. In other forms of progressive muscular dystrophy the level of cyclic adenosinmonophosphate did not differ significantly from normal indices.

[Mitochondrial pathology]. / Mitokhondrial'naia patologiia.

Three adult patients with mitochondrial disease underwent clinical-morphological studies which demonstrated a diffuse and marked defect in mitochondria. The characteristic clinical picture of "ophthalmoplegia plus", a slow progression of the disease, the quantitative and structural changes in the mitochondria of muscle fibers justified the diagnosis of mitochondrial disease.

[Spinal form of Ullrich's disease in monozygotic twins]. / Spinal'naia forma bolezni Ul'rikha u monozigotnykh bliznetsov.

The authors describe congenital Ulrich's arthrogryposis in two five-year-old boys who were monozygotic twins. Examinations of biopsy specimens from muscles revealed in both of them fascicular atrophy of the muscular fibres, the fact, that pointed to the neurogenic character of the disease. The EMG findings pointed to the spinal level of the affection. The results of the examination have given the authors grounds to regard the patients' disease as a spinal form of Ulrich's disease. This form is described in literature for the first time. A brief review of literature dealing with Ulrich's arthrogryposis is presented.

[Clinical and genetic heterogeneity of the Ehlers-Danlos syndrome]. / Klinicheskaia i geneticheskaia geterogennost' sindroma Elersa--Danlosa.

A clinical description of Type I and II Ehlers-Danlos syndrome in a 3-year-old girl and a 19-year-old boy is presented. The syndrome is characterized by skin hyperelasticity, articular overextensibility, hemorrhagic manifestations and also by excessive vulnerability of tissues. Clinical descriptions are supplemented with roentgenologic, morphologic and electron microscopic findings as well as with the results of amino-acid analysis and of dermatoglyphic examination of the patient and her parents.

[Ophthalmoplegic myopathy]. / Oftal'moplegicheskaia miopatiia.

Data of a clinico-morphological examination of 4 patients with the ophthalmoplegic form of myopathy are presented. An electronmicroscopic examination has made it possible to disclose the primary morphological defect in the mitochondria of the patients' muscles. The mitochondrial pathology manifested itself in an increase of the number of these organelles, changes of their shape and size, structural abnormalities and presence of inclusions. The pathology of the mitochondria in muscular fibres with normal structure of the latter's contractile apparatus, gradual destruction of the mitochondria, and deepening of the destructive changes in the mitochondria in parallel to the lysis of the myofibrillary apparatus give one grounds to speak about the primary character of the mitochondrial pathology in those patients, and to regard this disease as a variant of mitochondrial myopathies.

[Normokalemic variant of paroxysmal myoplegia]. / Normokaliemicheskii variant paroksizmal'noi mioplegii

For the first time in Soviet literature the authors describe a family where patients from 2 generations suffered from normokalemic periodical paralysis. The patients had undergone several examinations which confirmed this diagnosis. This report confirms the existence of a normopotassemic variant of periodical paralysis. The authors demonstrate the absence of a direct relation between the development of myoplegic attacks in these patients and disorders of the electrolyte balance. The histological studies of the muscular biopsy during the attacks detected a vacuolization of muscular fiberes. Histochemical studies of some metabolities of the carbohydrate metabolism did not detect any significant changes. The achieved results point only to an increase of the glyconeogenesis process and aerobie glycolisis.¿

[Sporadic distal myopathy]. / Sporadicheskaia distal'naia miopatiia.

The presented description of two cases of sporadic distal myopathy represents the first report on this disease published in the national literature. The clinical picture of the disease was consistent with that of five patients with this disease entity described in the world literature and was characterized by the disease onset in young age expressed in the form of distal muscular atrophies, a dramatic increase in the activity of serum creatine kinase, with electromyographic, histologic and electron microscopic findings indicating the presence of the myopathic process. A differential diagnosis from other diseases manifesting themselves in distal muscular atrophies is also outlined.