Intensive therapy in syndromes of disorders of cerebral blood circulation in the bed of the anterior cerebral arteries following the removal of middle basal tumors

255 patients have been examined in the early postoperative period after the removal of tumors located in diencephalic and sella turcica regions. The early postoperative period was complicated by circulation disturbances in the vascular bed of the anterior cerebral or anterior communicative arteries. The clinical pattern, CT scans and morphological data were analysed. Three principal clinical syndromes of the postoperative period were lined out: 1) extrapyramidal-diencephalic syndrome; 2) extrapyramidal-capsular syndrome; 3) frontal-corpocallosal syndrome. The reason for these syndromes was the occlusion of the anterior cerebral and anterior communicative arteries or their branches. The diencephalic syndrome had poor prognosis. The postoperative therapy included hormones. It was necessary to prevent the onset of adrenal insufficiency and diabetes insipidus. The differential diagnosis of thrombosis and vasospasm was of great importance. In case of vasospasm, such medicines as sermion, redergine and nimodipine had a strong therapeutic effect. In vascular thrombosis these medicines were ineffective. Systemic approach to circulation disturbances seems of utmost importance for the analysis of postoperative complications.     

Incidence rates of articular pain in urban and rural population of Russia (preliminary data)

AIM: Screening of the population sample for articular pain and swelling. MATERIAL AND METHODS: A screening questionnaire for detection of arthritides and arthralgias was filled in for each adult rural and urban citizen aged over 18 years from 15 RF regions. Rural population was covered with a complete examination, urban one was examined by formation of a representative sample from general population. The screening covered a total of 31424 persons, mean age of whom was 47.6 +/- 17.6 years. Among them 14001 lived in the cities and 17423 in the villages. 61% respondents were women. RESULTS: Prevalence of arthralgias in the population reaches 46%. While the symptoms alleviated with time in 8%, they became chronic in the rest. The articular complaints rate in the population is sex-related and depends on the age. In persons under 20 years arthralgia occurs in 9%. Urban citizens have articular pains more frequently than rural population--43 and 33%, respectively. CONCLUSION: Pilot results of the screening program demonstrate prevalence of arthralgias in Russia and the importance of joint pathology problem in Russia.     

Novel oncogene amplifications in tumors from a family with Li–Fraumeni syndrome

Li–Fraumeni syndrome (LFS) represents an inherited tumor syndrome that is typically caused by germline mutations of the tumor suppressor gene TP53. TP53 dysfunction secondarily disturbs the genetic integrity of the cell. Here, we report a family with LFS harboring a germline TP53 mutation (R248W) located in the functional domain of the protein that binds to the minor groove of the DNA. In this family, tumors of the central nervous system were diagnosed as primary malignancies in all carriers of the mutation. The index patient developed an anaplastic medulloblastoma with unusual genomic profile exhibiting six distinct high‐level genomic amplifications, two of them targeting the MYCN and GLI2 genes, respectively. In an extrarenal rhabdoid tumor from the same patient, we found a novel high‐level amplification of the MYC oncogene. The father of this patient was diagnosed with myxopapillary ependymoma (WHO °I), whereas a brother died from an early relapse of a choroid plexus carcinoma. The analysis of this LFS familiy thus revealed novel oncogene amplifications as different second hits that are likely to also play a role in the pathogenesis of their sporadic counterparts. © 2009 Wiley‐Liss, Inc.     

Occupational hygiene of railway transport workers as a specific branch of occupational medicine]

Occupational hygiene of the railway transport is a well defined sphere of knowledge. It is closely connected with the general occupational medicine and yet occupies a significant place in the railway medicine. Occupational medicine of the railway transport is based on results of sciences connected with each other and at the same time it serves for the favourable research is included sciences, such as occupational physiology and hygiene pathology, sanitary technics, chemistry, acoustics et al.     

Gains at the 1p36 chromosomal region are associated with symptomatic leptomeningeal dissemination of supratentorial glioblastomas

Symptomatic craniospinal dissemination of glioblastomas is considered rare, and unambiguous pathologic or molecular predictors of tumor leptomeningeal spread remain to be defined. We performed molecular analysis of 8 glioblastomas with symptomatic leptomeningeal dissemination by using fluorescence in situ hybridization and array-based comparative genomic hybridization (array-CGH). The most frequently encountered alteration was gain of the 1p36 locus, which was found in all 8 samples examined. Array-CGH analysis of 3 available samples also disclosed numerous gains at the 1pter-p36.1 locus involving at least 3 DNA clones from this chromosomal region. Consequently, an analysis of 1p copy number status might be kept in mind as an additional tool for further predicting the clinical course of glioblastoma.     

Comparative integrated molecular analysis of intraocular medulloepitheliomas and central nervous system embryonal tumors with multilayered rosettes confirms that they are distinct nosologic entities

Intraocular medulloepithelioma (IO MEPL) is an uncommon embryonal neuroepithelial neoplasm of the eye. These ocular neoplasms have been compared with intracranial medulloepitheliomas or other histologic variants of CNS embryonal tumor with multilayered rosettes (CNS ETMR) due to their morphological mimicry. We performed comprehensive molecular analysis to explore the histogenetic and biologic relationships between 22 IO MEPL and 68 CNS ETMR. Routinely prepared paraffin‐embedded samples were assessed for genome‐wide methylation profiles using the Illumina Methylation 450k BeadChip array. We identified strong cytogenetic and epigenetic differences between ocular neoplasms and CNS ETMR. None of the IO MEPL cases displayed the ETMR‐specific amplification of the C19MC locus. Instead, cytogenetic analysis of the IO MEPL showed numerous copy number aberrations which involved either whole chromosomes or chromosomal arms; recurrent aberrations in these tumors affected chromosomes 1p, 4, 8 and 16p. DNA methylation patterns were also strikingly different between these two tumor entities, suggesting that they do not share common origins and biological behaviors. Comparative cluster analysis of 198 pediatric CNS tumors and 22 IO MEPL revealed a clear demarcation of the CNS ETMR and IO MEPL profiles from other CNS entities. In conclusion, although IO MEPL shares some histopathological features with CNS ETMR, they manifest striking molecular diversity at the cytogenetic and epigenetic levels. Consequently they deserve a separate nosologic designation in future tumor classifications, where CNS MEPL could be designated as a histological variant of CNS ETMR.