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Involvement of the renal artery is common in Takayasu arteritis. We, herein, present on a patient with Takayasu arteritis causing severe renal failure and a successful auto-transplantation. This case shows that early diagnosis and immediate appropriate interventions are life-saving in patients with Takayasu arteritis. Renal auto-transplantation performed in selected cases increases dialysis-free survival.

For proton exchange membrane fuel cell systems (PEMFC) integrated with fuel processors, the calorific value of reformate gases produced during the start-up phase must be recovered. An appropriate exhaust after treatment system has crucial importance for PEMFC systems. Catalytic combustion is a promising alternative regarding its total oxidation capability of low calorific value gases at low temperatures, thereby reducing environmentally hazardous emissions. The aim of the study is to develop an after treatment system using a catalytic burner with a nominal capacity of 5 kW(t), which is also adaptive to partial loads of PEM fuel cell capacity. Fuel type, fuel composition and fuel loads are important parameters determining the operating window of the catalytic burner. Precious metal based catalysts, as proved to be the most active catalysts for the oxidation of hydrocarbons, can withstand temperatures of about 1073 K without exhibiting a rapid deactivation. This is the main barrier dictating the operating window and thereby determining the capacity of the burner. In this work, 1.5% natural gas (NG) alone was found to be the upper limit to control the catalyst bed temperature below 1073 K. In the case of catalytic combustion of hydrogen-NG mixture, 7% of hydrogen with NG up to 0.6% could be totally oxidized below 1073 K. Within the experimented ranges of fuel loads, between 2.5 kW, and 5.5 kW(t), the temperature of the catalyst bed was seen to increase with increasing the fuel load at constant fuel percentages. It has been observed that fuel type was another parameter affecting the exhaust gas temperature. (C) 2010 Professor T. Nejat Veziroglu. Published by Elsevier Ltd. All rights reserved.

Objective: The aim Of this Study was to determine the laboratory diagnosis and genetic origins of the hemoglobin (Hb) variants, Hb D-Los Angeles and Hb Beograd observed frequently in our region. Material and Methods: Hb variants were investigated in one Hb D-Los Angeles and two Hb Beograd families. These families were unrelated with each other. For the determination of Hb variants, alkaline/acid electrophoresis, HPLC, DE-52 micro-colum chromatography procedures were applied. Mutations were determined by non-radioactive fluorescence automated DNA sequencing. Beta globin gene cluster haplotypes were identified by RFLP analysis at seven loci known as epsilon-Hinc II, G gamma-Hind III, A gamma-Hind III, 5'psi beta-Hinc II, 3'psi beta-Hinc II, beta-Ava II ve 3'beta-Hinf I. Results: Three novel beta globin gene cluster haplotypes were identified as in relation with Hb D-Los Angeles [--+-+++], Hb Beograd [+----++ and -+-(+/- )(+/-)+(+/-)]. These haplotypes were reported for the first time in the world population Conclusion: In this study we emphasize the importance of DNA sequencing and other laboratory procedures for the identification of Hb variants in premarital diagnosis. On the other hand we discuss also the genetic origins of these Hb variants. (Turk J Hematol 2009; 26: 17-20)

Objective: The aim of this study was to determine the laboratory diagnosis and genetic origins of the hemoglobin (Hb) variants, Hb D-Los Angeles and Hb Beograd observed frequently in our region. Material and Methods: Hb variants were investigated in one Hb D-Los Angeles and two Hb Beograd families. These families were unrelated with each other. For the determination of Hb variants, alkaline/acid electrophoresis, HPLC, DE-52 micro-column chromatography procedures were applied. Mutations were determined by non-radioactive fluorescence automated DNA sequencing. Beta globin gene cluster haplotypes were identified by RFLP analysis at seven loci known as ?-Hinc II, G?-Hind III, A?-Hind III, 5'?ß-Hinc II, 3'?ß-Hinc II, ß-Ava II ve 3'ß-Hinf I. Results: Three novel beta globin gene cluster haplotypes were identified as in relation with Hb D-Los Angeles [--+-+++], Hb Beograd [+----++ and -+-(+/-)(+/-)+(+/-)]. These haplotypes were reported for the first time in the world population Conclusion: In this study we emphasize the importance of DNA seqeuncing and other laboratory procedures for the identification of Hb variants in premarital diagnosis. On the other hand we discuss also the genetic origins of these Hb variants.

OBJECTIVE: The aim of this study was to determine the laboratory diagnosis and genetic origins of the hemoglobin (Hb) variants, Hb D-Los Angeles and Hb Beograd observed frequently in our region. MATERIAL AND METHODS: Hb variants were investigated in one Hb D-Los Angeles and two Hb Beograd families. These families were unrelated with each other. For the determination of Hb variants, alkaline/acid electrophoresis, HPLC, DE-52 micro-column chromatography procedures were applied. Mutations were determined by non-radioactive fluorescence automated DNA sequencing. Beta globin gene cluster haplotypes were identified by RFLP analysis at seven loci known as ε-Hinc II, Gγ-Hind III, AΨβ-Hind III, 5'Ψβ-Hinc II, 3'Ψβ-Hinc II, β-Ava II ve 3'β-Hinf I. RESULTS: Three novel beta globin gene cluster haplotypes were identified as in relation with Hb D-Los Angeles [--+-+++], Hb Beograd [+----++ and -+-(+/-)(+/-)+(+/-)]. These haplotypes were reported for the first time in the world population Conclusion: In this study we emphasize the importance of DNA seqeuncing and other laboratory procedures for the identification of Hb variants in premarital diagnosis. On the other hand we discuss also the genetic origins of these Hb variants.

This study explores issues of consistency and inspectability in usability test data analysis processes and reports. Problem reports resulting from usability tests performed by three professional usability labs in three different countries are compared. Each of the labs conducted a usability test on the same product, applying a test protocol that was collaboratively developed by the labs. Each lab first analyzed their own findings as they always do in their regular professional practice. A few weeks later, they again analyzed their findings but then everyone applied the same method (SlimDEVAN: a simplified version of DEVAN, a method developed for facilitating comparison of findings from usability tests in an academic setting). It was found that levels of agreement between labs did not improve when they all used SlimDEVAN, suggesting that there was inherent subjectivity in their analyses. It was found that consistency of single analyst teams varied considerably and that a method like SlimDEVAN can help in making the analysis process and findings more inspectable. Inspectability is helpful in comparative studies based on identified usability problems because it allows for tracing back findings to original observations, as well as for laying bare the subjective parts of the data analysis.

PubMedID: 15643295 Familial Mediterranean fever (FMF) is an autosomal recessive disease that is prevalent among eastern Mediterranean populations, mainly non-Ashkenazi Jews, Armenians, Turks, and Arabs. Since a large proportion of all the FMF patients in the world live in Turkey, the Turkish FMF Study Group (FMF-TR) was founded to develop a patient registry database and analyze demographic, clinical, and genetic features. The cohort was composed of 2838 patients (mean age, 23.0 ± 13.33 yr; range, 2-87 yr), with a male:female ratio of 1.2:1. There was a mean period of 6.9 ± 7.65 years from disease onset to diagnosis; the period was about 2 years shorter for each decade since 1981. Ninety-four percent of patients were living in the central-western parts of the country; however, their familial origins (70% from the central-eastern and Black Sea regions) reflected not only the ongoing east to west migration, but also the historical roots of FMF in Turkey. Patients' clinical features included peritonitis (93.7%), fever (92.5%), arthritis (47.4%), pleuritis (31.2%), myalgia (39.6%), and erysipelas-like erythema (20.9%). Arthritis, arthralgia, myalgia, and erysipelas-like erythema were significantly more frequent (p < 0.001) among patients with disease onset before the age of 18 years. Genetic analysis of 1090 patients revealed that M694V was the most frequent mutation (51.4%), followed by M680I (14.4%) and V726A (8.6%). Patients with the M694V/M694V genotype were found to have an earlier age of onset and higher frequencies of arthritis and arthralgia compared with the other groups (both p < 0.001). In contrast to other reported studies, there was no correlation between amyloidosis and M694V homozygosity in this cohort. However, amyloidosis was still remarkably frequent in our patients (12.9%), and it was prevalent (27.8%) even among the 18 patients with a disease onset after age 40 years. Twenty-two patients (0.8%) had nonamyloid glomerular diseases. The high prevalence of vasculitides (0.9% for polyarteritis nodosa and 2.7% for Henoch-Schönlein purpura) and high frequency of pericarditis (1.4%) were striking findings in the cohort. Phenotype II cases (those patients with amyloidosis as the presenting or only manifestation of disease) were rare (0.3% or less). There was a high rate of a past diagnosis of acute rheumatic fever, which suggested a possible misdiagnosis in children with FMF presenting with recurrent arthritis. To our knowledge, this is the largest series of patients with FMF reported from 1 country. We describe the features of the disease in the Turkish population and show that amyloidosis is still a substantial problem.