Prevalence of arterial hypertension in the Krasnoyarsk Krai (Siberia, Russia).

BACKGROUND: To estimate the prevalence, awareness, treatment and control of arterial hypertension among adult inhabitants of Krasnoyarsk Krai using the data from Russian multicenter epidemiological study ESSE-RF (Epidemiological Survey of cardiovascular diSEases in different regions of the Russian Federation). METHODS: The study included 1603 subjects 25 to 64 years old selected by means of systemic multistage stratified randomization among urban and rural inhabitants of Krasnoyarsk Krai recruited between February 2014 and June 2014. Office blood pressure (BP) was measured twice with "Omron" automated BP device on the right arm in the sitting position in presence of medical personnel. Arterial hypertension (HTN) was defined as systolic BP of at least 140 mmHg and/or diastolic BP of at least 90 mmHg or self-reported previous elevated BP registration or use of antihypertensive treatment. Treatment efficacy was defined as percentage of patients achieved the target BP level among those who received antihypertensive medications, and HTN control as percentage of people achieved the target BP level among all hypertensives. RESULTS: The gender distribution was 652 males (39.4%) and 951 females (60.6%). The average level of systolic BP was 133.4 ± 0.5 mmHg, diastolic BP - 82.9 ± 0.3 mmHg. The average prevalence of HTN was estimated at 49.4% and appeared to be higher than similar parameter based on the data of 10 regions from the ESSE-RF study (44%). The average prevalence of HTN was estimated at 56.3% in males and 43.7% in females. The HTN prevalence in rural community was significantly higher in comparison with urban community (63.4 ± 2.4 vs. 44.2 ± 1.5%, p < 0.01). The average rate of HTN awareness in Krasnoyarsk Krai was 77.9% (average Russian value from ESSE-RF study was 73.1%). The average use of antihypertensive treatment, its efficacy and HTN control in Krasnoyarsk Krai were estimated at 59.5%, 31.6% and 18.8%, respectively. CONCLUSIONS: Estimated prevalence of HTN in Krasnoyarsk Krai is higher than the average Russian parameter. The average HTN prevalence among men is higher than in women. The rural inhabitants are more likely to have hypertension compared with urban inhabitants. Despite high levels of HTN awareness and antihypertensive medication intake, the antihypertensive treatment efficacy in Krasnoyarsk Krai appeared to be lower in comparison with average Russian ESSE-RF values.

C2-C8 hydrocarbon measurement and quality control procedures at the Global Atmosphere Watch Observatory Hohenpeissenberg.

A new automated on-line GC-flame ionization detection system for long-term stationary measurements of atmospheric C2-C8 hydrocarbons in the lower ppt range is described. The system is operated at the Global Atmosphere Watch Observatory Hohenpeissenberg (47 degrees 48'N, 11 degrees 01'E) in rural south Germany. Atmospheric mixing ratios of more than 40 different hydrocarbons can be continuously measured in 80 min time intervals. Corresponding detection limits are below 3 ppt, except for propene, butenes and benzene (about 10 ppt). Detailed quality assurance and quality control protocols are described which are applied to routine operation and data analysis. The various error contributions, overall precision, and accuracy for all measured compounds are discussed in detail. Typical ambient air mixing ratios are in the range of a few ppt to a few ppb, and corresponding measurement accuracies are below 10% or 10 ppt. For less than 20% of the analyzed compounds measurement accuracies are worse, mainly because of insufficient peak separation, blank values or reduced reproducibilities. The present system was tested in international intercomparison experiments (NOMHICE, AMOHA). For most of the C2-C8 hydrocarbons analyzed, our results agreed better than +/- 10% (20% NOMHICE phase 5) or +/- 10 ppt with the corresponding reference values.

Molecular cloning of the critical region for glomerulopathy with fibronectin deposits (GFND) and evaluation of candidate genes.

Glomerulopathy with fibronectin deposits (GFND, MIM 601894) is an autosomal dominant kidney disease that leads to terminal renal failure at a median age of 47 years. It represents a distinct entity of membranoproliferative glomerulonephritis (MPGN) type III and is characterized by the unique feature of massive glomerular deposits of fibronectin. We have recently localized a gene locus for GFND to human chromosome 1q32 by total genome linkage analysis in a large kindred, within a 4.1-cM critical interval between markers D1S2872 and D1S2891. This interval contains a cluster of genes for "regulators of complement activation" (RCA), which represent strong candidates for GFND. To identify positional candidate genes for GFND within the critical genetic interval, we here report the cloning of the entire critical GFND region in a complete YAC and partial PAC contig. We constructed a high-resolution transcriptional map, thereby defining positional and functional candidate genes for the disease. To evaluate their role in GFND, we performed functional studies on RCA proteins in GFND patients from the large kindred, as well as mutational analysis of the genes for complement receptor-2 (CR2), membrane cofactor protein (MCP), and decay accelerating factor (DAF). Although no loss-of-function mutation has been identified as yet, these data provide a basis for the examination of candidate genes for GFND and other genes for MPGN, which localize to the vicinity of the GFND region.

A Bartter's syndrome mutation of ROMK1 exerts dominant negative effects on K(+) conductance.

Mutations in the gene encoding the renal epithelial K(+) channel ROMK1 (Kir 1.1) is one of the causes for Bartter's syndrome, an autosomal recessive disease. It results in defective renal tubular transport in the thick ascending limb of the loop of Henle that leads to hypokalemic metabolic alkalosis and loss of salt. Two novel ROMK1 mutations, L220F/A156V, have been described recently in a compound heterozygote patient demonstrating typical manifestations of Bartter's syndrome. Functional properties of these ROMK1 mutants were studied by coexpressing in Xenopus oocytes and by means of double electrode voltage clamp experiments. When both ROMK1 mutants were coexpressed no K(+) conductance could be detected. The same was found in oocytes expressing A156V-ROMK1 only or coexpressing wild type (wt) ROMK1 together with A156V-ROMK1. In contrast, K(+) conductances were indistinguishable from that of wt-ROMK1 when L220F-ROMK1 was expressed alone. Activation of protein kinase C signaling inhibited the conductance in both L220F-ROMK1 and wt-ROMK1 expressing oocytes. These effects were not seen in A156V-ROMK1 expressing oocytes. Because no further abnormalities in the properties or regulation of L220F-ROMK1 were detected, we conclude that A156V-ROMK1 has a dominant negative effect on L220F-ROMK1 thereby causing Bartter's syndrome type two in this patient.

The gene for human fibronectin glomerulopathy maps to 1q32, in the region of the regulation of complement activation gene cluster.

Fibronectin glomerulopathy (GFND) is a newly recognized autosomal dominant disease of the kidney that results in albuminuria, microscopic hematuria, hypertension, renal tubular acidosis type IV, and end-stage renal disease in the 2d to 6th decade of life. The disease is characterized histologically by massive deposits of fibronectin (Fn) present in the subendothelial spaces of renal glomerular capillaries. The cause of human GFND is unknown. In order to localize a candidate gene for GFND, we performed linkage analysis of a large, 193-member pedigree containing 13 affected individuals. Since we had previously excluded the genes for Fn and uteroglobin as candidate genes for GFND, a total-genome search for linkage was performed. Examination of 306 microsatellite markers resulted in a maximum two-point LOD score of 4.17 at a recombination fraction of. 00 for marker D1S249, and a maximum multipoint LOD score of 4.41 for neighboring marker D1S2782. By detection of recombination events, a critical genetic interval of 4.1 cM was identified, which was flanked by markers D1S2872 and D1S2891. These findings confirm that GFND is a distinct disease entity among the fibrillary glomerulopathies. Gene identification will provide insights into the molecular interactions of Fn in GFND, as well as in genetically unaltered conditions.

[Strategy and results of advanced tumors in emergency situations]. / Strategie und Ergebnisse bei fortgeschrittenem Tumorleiden in der Notfallsituation.

In this retrospective trial we examined 142 patients with advanced unresectable cancer, who had bowel obstruction, bleeding, bowel lesion and abscesses and who were treated with different surgical procedures (resection, bypass, enterostomy). Mean survival rate was 8.6 months (range: 0-57 months). The mortality rate reached 21.7%. Surgical reintervention was necessary in 15.8% because of bowel obstruction and did not influence the survival rate. Despite advanced tumor disease and intestinal obstruction most patients had a good quality of life after surgical intervention.

[Cervico-facial suppuration of oro-dental origin due to Eikenella corrodens]. / Suppurations cervico-faciales d'origine bucco-dentaire à Eikenella corrodens.

Several cases are reported of cervico-facial suppuration of buccodental origin, the infection being due to Eikenella corrodens, a facultative anaerobic germ that is difficult to culture. Conditions appropriate to the growth of these germs are discussed with respect to etiological, bacteriological and clinical factors. Therapy is outlined and a selective bibliography proposed.

[Mandibular bone tumors. Diagnostic difficulties]. / Tumeurs osseuses mandibulaires. Les difficultés du diagnostic.

A session of the "Société de Stomatologie et de Chirurgie maxillo-faciale de France" was devoted to a discussion of diagnostic difficulties in cases of mandibular bone tumors. Several case-reports were presented as a basis for comments on clinical and radiologic criteria of the benign or malignant nature of bone tumors, these being the foundation on which to establish the most effective surgical approach to adopt. Pre-operative biopsy is not considered essential when confirmation of diagnostic has been obtained.

[Malignant non-Hodgkin's lymphoma]. / Les lymphomes malins non hodgkiniens.

Rapid regression of all symptoms was obtained after moderate chemotherapy in two women aged 69 and 77 years respectively with malignant non-Hodgkin's lymphomas. Cervico-facial locations of these tumors are discussed in relation to definition, etiology, geographic factors, genetic markers, and associated immunologic disorders. Diagnosis requires a series of explorations including, obviously as a last resort, exploratory cervicotomy. Other regions may be involved and must be investigated, but lesions not affecting lymph nodes occur in only approximately 2 p. cent of patients with cervico-facial malignant non-Hodgkin's lymphoma (approximately 10 p. cent of all malignant non-Hodgkin's lymphomas). Other localizations include the hard palate, gums, sinuses, and salivary glands. Burkitt's lymphoma represents, on the contrary, 30 p. cent of malignant non-Hodgkin's lymphoma seen in European children. The different therapeutic modalities available are discussed.

[Thrombopenia as the presenting symptom of acute disseminated lupus erythematosus: Moschcowitz's disease]. / Thrombopénie, symptôme inaugural d'un lupus érythémateux aigu disséminé: maladie de Moschcowitz.

Possible relationships between Moschcowitz's disease and acute systemic lupus erythematosus are analyzed based on findings in one case. Initial exclusively buccal symptoms suggested a diagnosis of lupus, and then of Moschcowitz's disease leading to a fatal outcome. The main points discussed relate to diagnostic difficulties to enable distinction between the two diseases. A thrombotic thrombopenic purpura during the second phase of the disease showed a characteristic microangiopathy associated with a non-autoimmune hemolytic anemia and a schizocytosis: two forms of the affection are recognized, one with a chronic and one with an acute course. The latter, which has a fulminating progression leading to death, was that described in the case reported.

[Anomalies of mineralization of the temporal styloid process (author's transl)]. / Les anomalies de minéralisation de l'appareil stylien.

Three cases treated by medical means are reported, and reports in the published literature briefly reviewed. --Anatomically the temporal styloid process is related to four main structures : the carotid artery and vein, and the facial and glossopharyngeal nerves. --Hypotheses as to the pathogenesis of these lesions are based on clinical findings. The anomalies may be discovered : 1) On radiological examination. 2) Present as pain in the tonsillar region radiating to the neck. 3) Fronto-orbital or temporo-occipital headaches. 4) Sudden repetitive bouts of syncope. --Therapy includes psychotherapy, local infiltration of an anesthetic, or removal of a fragment of the calcified styloid process by a cervical or pharyngeal approach.