Cardiopulmonary assessment in primary ciliary dyskinesia.

Background Primary ciliary dyskinesia (PCD) is a rare, usually autosomal recessive disorder of ciliary dysfunction associated with lung involvement, which has a great impact on health. There is limited information concerning the aerobic fitness of children and adolescents with PCD. The aim of this study was to assess cardiopulmonary functional capacity and its relationship with pulmonary function and physical activity (PA) levels in patients with PCD. Design Ten patients with PCD (age 13·2±2·8years) underwent spirometry and cardiopulmonary exercise testing. PA was investigated through a questionnaire. Eight age- and body mass index-matched healthy children were enrolled as controls. Main variables were forced expiratory volume at 1s, peak oxygen uptake (VO 2peak) and time spent in PA. Results Forty per cent of patients with PCD had impaired lung function as expressed by FEV 1<85% predicted. Only patients with impaired lung function exhibited reduced VO 2peak (18·1±7·9mL/kg/min). Time spent in total daily PA was slightly lower in patients than controls, with no difference between patients with normal or reduced lung function. In multiple regression models, male gender (??=0·518, P=0·018), age (??=0·752, P=0·035) and time spent in vigorous PA (??=0·353, P=0·049) were independent predictors of aerobic fitness. Conclusions Assessment of resting pulmonary function and cardiopulmonary functional capacity could contribute to the evaluation of pulmonary impairment in PCD. Given the benefit of physical exercise on airway clearance and on general health and quality of life, patients with PCD should be encouraged to adopt an active lifestyle

Zebrafish, a novel model system to study uremic toxins: The case for the sulfur amino acid lanthionine

The non-proteinogenic amino acid lanthionine is a byproduct of hydrogen sulfide biosynthesis: the third endogenous vasodilator gas, after nitric oxide and carbon monoxide. While hydrogen sulfide is decreased in uremic patients on hemodialysis, lanthionine is increased and has been proposed as a new uremic toxin, since it is able to impair hydrogen sulfide production in hepatoma cells. To characterize lanthionine as a uremic toxin, we explored its effects during the early development of the zebrafish (Danio rerio), a widely used model to study the organ and tissue alterations induced by xenobiotics. Lanthionine was employed at concentrations reproducing those previously detected in uremia. Light-induced visual motor response was also studied by means of the DanioVision system. Treatment of zebrafish embryos with lanthionine determined acute phenotypical alterations, on heart organogenesis (disproportion in cardiac chambers), increased heart beating, and arrhythmia. Lanthionine also induced locomotor alterations in zebrafish embryos. Some of these effects could be counteracted by glutathione. Lanthionine exerted acute effects on transsulfuration enzymes and the expression of genes involved in inflammation and metabolic regulation, and modified microRNA expression in a way comparable with some alterations detected in uremia. Lanthionine meets the criteria for classification as a uremic toxin. Zebrafish can be successfully used to explore uremic toxin effects

Amifostine (wr-2721), A Cytoprotective Agent During High-dose Cyclophosphamide Treatment Of Non-hodgkin's Lymphomas: A Phase Ii Study.

Clinical trials indicate that amifostine may confer protection on various normal tissues without attenuating anti-tumor response. When administered prior to chemotherapy or radiotherapy, it may provide a broad spectrum of cytoprotection including against alkylating drugs. The mechanism of protection resides in the metabolism at normal tissue site by membrane-bound alkaline phosphatase. Toxicity of this drug is moderate with hypotension, nausea and vomiting, and hypocalcemia being observed. We report a phase II study using amifostine as a protective drug against high-dose cyclophosphamide (HDCY) (7 g/m2), used to mobilize peripheral blood progenitor cells (PBPC) and to reduce tumor burden. We enrolled 29 patients, 22 (75. 9%) affected by aggressive and 7 (24.1%) by indolent non-Hodgkin's lymphoma (NHL), who were submitted to 58 infusions of amifostine and compared them with a historical group (33 patients) affected by aggressive NHL and treated with VACOP-B followed by HDCY. The most important results in favor of amifostine were the reduction of intensity of cardiac, pulmonary and hepatic toxicity, and a significant reduction of frequency and severity of mucositis (P = 0. 04). None of the 29 patients died in the protected group, while in the historical group 2/33 patients died because of cardiac or pulmonary toxicity and 2 patients stopped therapy due to toxicity. Amifostine did not prevent the aplastic phase following HDCY. PBPC collection and hematological recovery were adequate in both groups. The number of CFU-GM (colony-forming units-granulocyte/macrophage) colonies and mononuclear cells in the apheresis products was significantly higher in the amifostine group (P = 0.02 and 0.01, respectively). Side effects were mild and easily controlled. We conclude that amifostine protection should be useful in HDCY to protect normal tissues, with acceptable side effects.33791-

Serological Monitoring Of A Toxoplasma Infection After Hematopoietic Stem Cell Transplantation.

We report a primary response to Toxoplasma gondii following a hematopoietic stem cell transplantation in a patient with multiple myeloma. The primary response to T. gondii was supported by IgM, IgG and IgA seroconversion. The patient was promptly treated and there were no complications related to toxoplasmosis in the subsequent months.52225-

Monitoramento sorológico de uma infecção toxoplásmica após transplante de células progenitoras hematopoiéticas

We report a primary response to Toxoplasma gondii following a hematopoietic stem cell transplantation in a patient with multiple myeloma. The primary response to T. gondii was supported by IgM, IgG and IgA seroconversion. The patient was promptly treated and there were no complications related to toxoplasmosis in the subsequent months.Esse relato de caso descreve uma resposta primária ao Toxoplasma gondii após transplante de células progenitoras hematopoiéticas em paciente com mieloma múltiplo. A resposta primária para o T. gondii foi evidenciada pela soroconversão observada na resposta de anticorpos IgM, IgG e IgA. O paciente foi prontamente tratado e complicações relacionadas à toxoplasmose não foram observadas nos meses subseqüentes

Measurements and tests on FBK silicon sensors with an optimized electronic design for a CTA camera

In October 2013, the Italian Ministry approved the funding of a Research & Development (R&D) study, within the "Progetto Premiale TElescopi CHErenkov made in Italy (TECHE)", devoted to the development of a demonstrator for a camera for the Cherenkov Telescope Array (CTA) consortium. The demonstrator consists of a sensor plane based on the Silicon Photomultiplier (SiPM) technology and on an electronics designed for signal sampling. Preliminary tests on a matrix of sensors produced by the Fondazione Bruno Kessler (FBK-Trento, Italy) and on electronic prototypes produced by SITAEL S.p.A. will be presented. In particular, we used different designs of the electronics in order to optimize the output signals in terms of tail cancellation. This is crucial for applications where a high background is expected, as for the CTA experiment.Comment: 5 pages, 6 figures; Proceedings of the 10th Workshop on Science with the New Generation of High-Energy Gamma-ray experiments (SciNeGHE) - PoS(Scineghe2014)00