[Tracheobronchial involvement in relapsing polychondritis and differential diagnoses]. / Atteinte trachéo-bronchique de la polychondrite atrophiante et diagnostics différentiels.

Relapsing polychondritis is a systemic auto-immune disease that mainly affects cartilage structures, progressing through inflammatory flare-ups between phases of remission and ultimately leading to deformation of the cartilages involved. In addition to characteristic damage of auricular or nasal cartilage, tracheobronchial and cardiac involvement are particularly severe, and can seriously alter the prognosis. Tracheobronchial lesions are assessed by means of a multimodal approach, including dynamic thoracic imaging, measurement of pulmonary function (with recent emphasis on pulse oscillometry), and mapping of tracheal lesions through flexible bronchoscopy. Diagnosis can be difficult in the absence of specific diagnostic tools, especially as there may exist a large number of differential diagnoses, particularly as regards inflammatory diseases. The prognosis has improved, due largely to upgraded interventional bronchoscopy techniques and the development of immunosuppressant drugs and targeted therapies, offering patients a number of treatment options.

Low-cost air quality portable sensors and their potential use in respiratory health.

Air pollution is an environmental risk for the general population and for patients with various diseases, particularly respiratory diseases. Little data are available on personal exposure, but the recent emergence of low-cost air quality sensors (LCSs) should enable a better understanding of the health impacts of air pollution at the individual level. However, the reliability and accuracy of most sensors in the market have not been established, and a thorough understanding of their strengths and limitations is needed. We therefore conducted a review to address the following questions: 1) What is an LCS and what is the extent of its possible application? 2) Is the data obtained a reliable indicator of exposure? 3) What are the advantages and disadvantages of LCSs? 4) Could LCSs be useful in investigating the impact of air pollution on respiratory health? Further studies are needed to promote the use of LCS in research settings and among respiratory patients. This will allow us to monitor exposure levels, provide alerts and study the respiratory effects of individual-level air pollution.

Does training respiratory physicians in clinical respiratory physiology and interpretation of pulmonary function tests improve core knowledge?

Lung function tests have a major role in respiratory medicine. Training in lung function tests is variable within the European Union. In this study, we have shown that an internship in a lung function tests laboratory significantly improved the technical and diagnostic skills of French respiratory trainees.

Functional regulation of alternatively spliced Na+/Ca2+ exchanger (NCX1) isoforms.

Alternative splicing of RNA transcripts is a general characteristic for NCX genes in mammals, mollusks, and arthropods. Among the family of three NCX genes in mammals, the NCX1 gene contains six exons, namely, A, B, C, D, E, and F, that make up the alternatively spliced region. Studies of the NCX1 gene transcripts suggested that 16 distinct gene products can be produced from the NCX1 gene. The exons A and B are mutually exclusive when expressed. Generally, exon A-containing transcripts are predominantly found in excitable cells like cardiomyoctes and neurons, whereas exon B-containing transcripts are mostly found in nonexcitable cells like astrocytes and kidney cells. Other alternatively spliced exons (C-F) appear to be cassette-type exons and are found in various combinations. Interestingly, exon D is present in all characterized transcripts. The alternatively spliced isoforms of NCX1 show tissue-specific expression patterns, suggesting functional adaptation to tissues. To investigate functional differences among alternatively spliced isoforms of NCX1, we expressed an exon A-containing transcript present in cardiac tissue (NCX1.1) and an exon B-containing transcript found in the kidney (NCX1.3) in Xenopus oocytes. We demonstrated that the Na(+)/Ca(2+) exchangers expressed by exon A- and exon B-containing transcripts display differences in activation by PKA and by [Ca(2+)](i). We also observed that these two isoforms show differences in voltage dependence. Surprisingly, the alternatively spliced isoforms of NCX1 display greater functional differences among themselves than the products of different gene loci, NCX1, NCX2, and NCX3.

[Transarterial chemoembolization in hepatocellular carcinomas: technique, indications, results]. / Transarterielle Chemoembolisation bei hepatozellulären Karzinomen: Technik, Indikationsstellung, Ergebnisse.

BACKGROUND: At time of diagnosis 80% of the patients with hepatocellular carcinoma (HCC) could not be treated with surgical treatments, so that transarterial chemoembolization (TACE) was used as an neoadjuvant or palliative treatment modality. MATERIAL AND METHODS: 60 patients were treated with 217 TACE courses, in the mean 3.6 TACE treatments per patient with an 4 week interval. TACE was performed with a dispersion of lipiodol, mitomycin C and spherex. 11 patients (18.3%) were treated in a neoadjuvant protocol with successful ablation. Lipiodol retention and size of the tumors were evaluated by CT and MRI. RESULTS: 60 patients were successful treated with TACE. After treatment a primary high lipiodol retention was displayed and in 68 (63.3%) patients a reduction of the tumor size and in 11 (20%) patients a reduction of tumor growth rate was noted. The 1 year survival rate was 59%. After response to TACE and reducing the tumor size 11 patients could be treated with MR-guided LITT 4 to 6 weeks post embolization. CONCLUSION: Chemoembolization is a minimal invasive and outpatient treatment protocol for HCC. TACE might be indicated as a palliative treatment to control the diseased liver. If repeated TACE alters the size and structure of primary unresectable HCC TACE expands the indication for MR-guided LITT.

Shiga toxin binds human platelets via globotriaosylceramide (Pk antigen) and a novel platelet glycosphingolipid.

Hemolytic-uremic syndrome is a clinical syndrome characterized by acute renal failure, microangiopathic hemolytic anemia, and thrombocytopenia that often follows infection by Shiga toxin- or verotoxin-producing strains of Escherichia coli. Because thrombocytopenia and platelet activation are hallmark features of hemolytic-uremic syndrome, we examined the ability of Shiga toxin to bind platelets by flow cytometry and high-performance thin-layer chromatography (HPTLC) of isolated platelet glycosphingolipids. By HPTLC, Shiga toxin was shown to bind globotriaosylceramide (Gb3) and a minor platelet glycolipid with an Rf of 0.03, band 0.03. In a survey of 20 human tissues, band 0.03 was identified only in platelets. In individuals, band 0.03 was expressed by 20% of donors and was specifically associated with increased platelet Gb3 expression. Based on glycosidase digestion and epitope mapping, band 0.03 was hypothesized to represent a novel glycosphingolipid, IV3-beta-Galalpha1-4galactosylglobotetraosylceramide. Based on incidence, structure, and association with increased Gb3 expression, band 0.03 may represent the antithetical Luke blood group antigen. By flow cytometry, Shiga toxin bound human platelets, although the amount of Shiga toxin bound varied in donors. Differences in Shiga toxin binding to platelet membranes did not reflect differences in platelet Gb3 expression. In contrast, there was a loose association between Shiga toxin binding and decreasing forward scatter, suggesting that Shiga toxin and verotoxins bind more efficiently to smaller, older platelets. In summary, Shiga and Shiga-like toxins may bind platelets via specific glycosphingolipid receptors. Such binding may contribute to the thrombocytopenia, platelet activation, and microthrombus formation observed in hemolytic-uremic syndrome.