[Mosaic forms of ataxia-telangiectasia].

Ataxia-telangiectasia (AT) is a severe hereditary autosomal recessive neurodegenerative disease associated with accelerated aging and caused by mutation in both alleles of atm gene. This gene encodes a key protein of cell response to DNA damage--an ATM protein kinase. Normally, upon formation of DNA double strand breaks ATM is autophosphorylated and its active form phospho-ATM (P-ATM) appears.

[Diagnostics of ataxia-telangiectasia by the express-test found on the method of indirect immunofluorescence].

Ataxia-telangiectasia (AT) is a hereditary severe neurodegenerative disease developing, when mutations take place in both alleles of the atm gene, which encodes the key protein of the cellular response to DNA damage (DDR)--ATM proteinkinase. In response to the occurrence of double-strand DNA breaks, the ATM proteinkinase pass the autophosphorylation, and its active form--the phospho-ATM (P-ATM) appears in cells. In the nuclei of cells having the atm gene, P-ATM is revealed, being absent in cells with mutated forms of this gene, by means of the application of the modified method of indirect immunofluorescence.

[The influence of age of donors on the ability of cells to homologous recombination DNA repair].

Old and young donors cells show different ability to homologous recombination (shown on the first stage--the chromosome transference) in vitro, that we suppose could be the reasons of the genome instability in aging. Homologous recombination, induced by X-radiation, is limited in cells taken from donors older than 70 years. Alpha-amanitin, the RNA-polymerize II repressor, in toxic doze, could induce the chromosome transference in the cells from all studied groups: from old and young donors and donors with repair process defect (with BRCA 1, 2 mutations).

[Telomere length in the population of long-lived persons of North-West region of Russia].

Interdisciplinary study of telomere length, polymorphism of genes of renin-angiotensin (ACE) and serotonin (5HTR2A and 5HTTPR) systems in population of aged and old inhabitants of the North-West of Russia was conducted, in their relations to data from clinical and geriatric anamnesis, and psychological functioning. Regular link between telomere length and respondent's age was demonstrated in subgroups of old respondents and long-livers, by method of factor analysis.

[Oxygenation state of brain in children upon reamberin infusion during post-anesthesia recovery].

The effect of reamberin (1.5% solution of succinic acid and ion complex) on the oxygenation status of the brain was studied in children aged 1 - 14 years during recovery from anesthesia. It is established that reamberin in a dose of 2 ml/kg administered twice at the end of an operation increases the fraction of oxidized hemoglobin and cytochrome oxidase and enhances the regional saturation of brain tissue with oxygen, which is indicative of an increase in the potential of metabolism activation in neurons.

[Reamberin administration for early postnarcosis recovery in children].

The possibility of using 1.5% succinic acid solution (reamberin) in order to activate recovery from anesthesia was studied in a group of 91 patients aged 1-14 years. Based on clinical data and BIS-index, it is established that the administration of reamberin during exit from anesthesia stage shortens the wake-up period, decreases the time of restoration of the motor activity and adequate respiration, and accelerates brain function recovery.

[Metformin slows down ageing processes at the cellular level in SHR mice].

It has been shown recently that metformin, the indirect mTOR-kinase inhibitor, significantly increases medium (by 37.8%) and maximum (by 10.3%) life span of SHR mice (Anisimov et al.

[Telomere shortening is the main mechanism of natural and radiation aging].

Adduced proofs of the telomere shortening are the main or even the sole mechanism of the natural and radiation aging. All apparent contradictions, primary, the absence of exact inverse correlation between residual telomere length and the donor age are explained within the frames of the telomere theory. We try to explain in what way telomere shortening might be the cause of aging and longevity restriction.

[Immune reaction to anesthesia and surgical injury in neonatal infants].

The impact of surgical injury and anesthesia on cellular and humoral immunity parameters was studied in neonatal infants with surgical pathology. These were found to have immunosuppressive activity, which appeared as evolving absolute lymphopenia and a reduction in the serum concentration of IgG. The magnitude of these changes depends on the functional state of the immune system in the preoperative period and is higher in infants with signs of an infectious-inflammatory process in the preoperative period, which increases the risk of septic complications.

[Cellular repair potential in families of ataxia-telangiectasia patients].

The key protein of the global cellular response to DNA damage is proteinkinase ATM. Ataxia-telangiectasia (AT), a genetic disorder due to mutations in both alleles of ATM gene, is characterized by numerous neurological abnormalities, increased frequency of malignant tumors, premature ageing and increased radio sensitivity. AT is the most frequently found disease displaying inherited radio sensitivity.

[Telomere length of cord blood cells chromosomes as additional quality characteristic of sample for transplantation].

Telomeres are the ends of the chromosomes and represent repeated DNA sequence and protein complex. Telomeres shorten with each cell division, which limits proliferative potential of cells. There is a great progress in clinical application of telomere length now.

[Syndrome of premature ageing in ataxia-telangiectasia patients].

Ataxia-telangiectasia (AT), a genetic disorder due to mutation of gene atm characterized by progressive neurological abnormalities in combination with oculocutaneous telangiectasias, immunodeficiency, and increased frequency of malignant formations, is inherited according to autosome recessive mechanism. Cells of the patients with AT show increased radio sensitivity and some markers of premature ageing. The telomere lengths are sharply shortened in these cells already from the birth.

[An atypical case of Werner syndrome: epigenetic control and DNA damage response alterations].

A case of adult progeria has been described. It has been suggested that this case is an atypical form of Werner syndrome with laminopathy--not WRN helicase-nuclease defect. During detailed studies of the patient's cells, epigenetic control and DNA damage response alterations were detected.

[The atypical of Werner syndrome: effect of laminopathy].

A case of adult progeria has been described. During detailed studies of the cells from this patient the nuclear lamina and cytoskeleton aberrations were detected. It has been suggested that this case is an atypical form of Werner syndrome with laminopathy--not the WRN helicase-nuclease defect.

[High-frequency oscillatory lung ventilation in neonatal infants with surgical diseases].

The paper presents data on the varieties and modes of the present artificial ventilation and high-frequency artificial ventilation (AV). It describes the basic impact of various ventilation modes on gas exchange, hemodynamics, and mechanical lung properties. The data on the use of high-frequency AV in neonatal infants with various surgical diseases are given.

[Non-drug prevention of pain in neonatal infants].

When treated at a hospital unit, full-term and preterm neonatal infants undergo a large number of manipulations and procedures, most of them are rather painful; anesthesia is used inadequately frequently. Pain prevention during procedures is able to reduce a baby's discomfort and to improve his/her tolerance of medical manipulations and care procedures.

[Characteristics of anesthesia in newborn infants].

The paper describes the specific features of anesthetic maintenance in preterm and full-term neonates, intraoperative monitoring, and the effects of inhaled anesthetics, narcotic analgesics, sedatives, and myorelaxants with emphasis on the anatomic and physiological features of a developing organism.

[Myoplegia with rocuronium bromide in children].

This study has evaluated the efficacy and safety of different doses of Esmerone in children under fluorothane anesthesia. It enrolled 85 children from a senior age group (7-14 years). According to the myorelaxant used, all the patients were divided into 2 groups: S and R.

[Prevention of pain during finger prick in neonatal infants].

Finger pricking for blood sampling is the most common manipulation in hospital units. Pain control using the rating scales for neonatal acute pain and its analgesia are necessary as the consequences of experienced pain are well known. The oral use of glucose solutions substantially relieves pain during this procedure as compared with neonates receiving no analgesics.

[Prevention of pain during manipulations in neonatal infants: analysis of physicians' questionnaire survey].

The paper presents the results of an anonymous questionnaire survey among the physicians of 7 children's health care facilities, who should assess and prevent pain in neonatal infants from 5 Russia's cities (Moscow, Cheboksary, Kursk, Novosibirsk, and Kyzyl). The questionnaire survey has indicated that all responding physicians agree with the statement that a neonate senses pain, but the severity of pain being uncontrolled and the pain syndrome unevaluated, pain analgesia and prevention are inadequately applied by comfort measures, particularly the use of glucose through a nipple, a pacifier, diapering, when procedures are performed. All the physicians are unanimous that analgesics or comfort measures should be more frequently used during any manipulations and procedures irrespective of whether they are performed for a long or short period.