718 research outputs found

    Improving energy efficiency of commercial buildings by Combined Heat Cooling and Power plants

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    Commercial buildings play a key-role in the energy consumption of the building sectors. Recent statistics have shown that as the number of commercial buildings is continuously increasing, their effects on energy consumption are expected to grow. These buildings are characterized by high energy demand mainly due to lighting and HVAC requirements. Rooms of energy saving exist by considering that: (i) electricity demands and HVAC requirements occur simultaneously during the day and (ii) both demands are currently satisfied by using separate energy systems. It is apparent that the adoption of polygeneration systems could represent a valid solution to achieve energy savings. To this aim, the paper investigated the profitability of a trigeneration system for commercial buildings, considering a big Do It Yourself shop located in the northern part of Italy, as case study. The analysis was based on (i) energy consumption data collected by energy-audits and (ii) a profit-oriented management strategy for the trigeneration systems proposed in literature. Results showed that trigeneration represents a profitable energy conversion system thanks to revenues achieved by selling surplus electricity and the support of financial mechanism for “High-Efficient” eligibility. In comparison with the currently adopted energy conversion systems, important reductions in energy consumption and CO2 emissions are observed

    PLEUROPULMONARY BLASTOMA: A DIFFERENTIAL DIAGNOSIS OF CHRONIC COUGH. LONG TERM SURVIVALAFTER MULTIMODAL AGGRESSIVE THERAPY

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    ITAL J PEDIATR 2006;32:122-125 CASE REPORT CASO CLINICO Pleuropulmonary blastoma: a differential diagnosis of chronic cough. Long-term survival after multimodal aggressive therapy Blastoma pleuro-polmonare: rara diagnosi differenziale da tosse cronica. Remissione a lungo termine dopo aggressiva terapia multimodale P. D''ANGELO, V. MANZO*, S. VENEZIA*, R. ALAGGIO", E SIRACUSA*, M. LO CURTO* Unit\ue0 Operativa di Oncoematologia Pediatrica, Ospedale dei Bambini " G . Di Cristina", Palermo; * Dipartimento Materno Infantile, Universit\ue0 di Palermo; ** Istituto di Anatomia Patologica, Universit\ue0 di Padova Summary Pleuropulmonary blastoma (PPB) in childhood is a rare clinicopathologic entity distinct from adult pneumoblastoma. This tumour may originate from the lung, the pleura, or the mediastinum; it can metastasize and is usually associated with a poor outcome. We report the case of a 5-year-old boy who developed PPB manifesting with respiratory distress. At the standard x-ray and magnetic resonance imaging of the chest there was opacity covering the entire right lung. The histological and immunohistological tests led to the diagnosis of blastematous, malignant mesenchymatous PPB with pluridirectional differentiation. Treatment consisted of preoperative chemotherapy to reduce tumour volume, complete surgical resection of the residuai tumour mass, and post-surgical chemotherapy. Following this approach, the child is alive in continuous complete remission 9 years after diagnosis. Riassunto II Blastoma Pleuro-Polmonare (BPP) infantile \ue8 un ''entit\ue0 clinico patologica ben distinta dal Pneumoblastoma dell''adulto. Questo tumore pu\uf2 prendere origine dal polmone, dalla pleura o dal mediastino; pu\uf2 metastatizzare e ha spesso una prognosi infausta. Riportiamo il caso di un bambino di 5 anni, in cui il BPP si manifest\uf2 con un distress respiratorio; la radiografia del torace e la Risonanza Magnetica hanno evidenziato una grossa massa che occupava l''emitorace destro. L''esame istopatologico ha permesso di porre diagnosi di BPP. Il paziente \ue8 stato trattato con chemioterapia, che ha ridotto il volume della massa, con asportazione del tumore e chemioterapia post-operatoria; tale trattamento ha consentito i''eradicazione della malattia; il paziente \ue8 in remissione completa continua a 9 anni dalla diagnosi. Introduction Pleuropulmonary blastoma (PPB) is an extremely rare and aggressive malignancy of childhood. It was originally described as a distinct entity by Manivel et al. ''. Prior to its identification it was reported in the early literature by Spencer as pulmonary blastoma or embryonic sarcoma 2. It is characterized by primitive mesenchymal tissue and epithelial tubular structures resembling the foetal lung. The eponymous PPB defines the paediatric variety of pulmonary blastoma. In PPB, the dysembrionic neoplasm shows blastematous and sarcomatous components and a lack of carcinomatous components (which are instead present in adult pulmonary blastoma), sometimes on previous dysplastic pulmonary conditions 3 . PPB is classified in 3 subtypes: type I (cystic), type II (mixed solid and cystic) and type III (solid)4. Key words Pleuropulmonary blastoma \u2022 Childhood lung cancer \u2022 Adjuvant chemotherapy Parole chiave Blastoma pleuropolmonare \u2022 Tumore polmonare infantile \u2022 Chemioterapia post-operatoria Submitted: March 16, 2005 Accepted: July 19, 2005 Correspondence: Prof. Margherita Lo Curto Dipartimento di Pediatria Universit\ue0 di Palermo Istituto Materno Infantile via Cardinale Rampolla 1 Palermo, Italy Tel. +39 091 6555476 E-mail: [email protected]. 122 PLEUROPULMONARY BLASTOMA IN A CHILD The predominant clinical features are cough, tachypnea, fever, respiratory distress; secondary pneumothorax 5 and chest pain have also been reported 6. Since these features are not specific, an infectious disease is often erroneously diagnosed; hence, when eventually detected, the neoplasm is often very large, may even involve an entire hemithorax, and present metastases. Despite the different therapeutic procedures - surgery, chemotherapy and radiotherapy - prognosis is often poor: Indolfi et al. 7 report 42% and Priest et al. 6 45% of event free survival (EFS) at 2 years. Poor prognostic factors are histological subtype II or III6 , a maximum diameter greater than 5 cm 7 , failure to completely remove the mass, extrapulmonary effusion such as pleura or pericardium, metastases 8. We report the case of a five-year-old child who, despite the large tumour size at diagnosis and histological subtype II, after a treatment with chemotherapy before and after surgery, is in continuous complete remission (CCR) nine years after the diagnosis. Case report A 5-year-old boy was admitted to our ward for hyporexia, cough, shortness of breath, progressive thinning and pallor in the previous 2 months. The physical examination showed poor clinical conditions, tachydyspnea (R.F. 45/min.), hypophonesis and reduction of the physiologic vescicular murmure of the middle and lower regions of the right lung, meteoric abdomen with the liver margin 5 cm below the right costai margin. The results of the laboratory investigations were Hb 8.5 g/dl, white blood cells 18.800/ul (N 68%, L 22%, M 6%, E 4%), platelets 611.000/u.l, VES (K.I.) 65, CRP 2.4 mg/dl; serum levels of copper 168 ixg/dl, ferritin 292 ng/dl, LDH 1.261 u/1, oc-FP 6.3 u/1. Chest radiographs showed a bulky mass in the right hemithorax displacing the mediastinum leftward and the liver downward (Fig. 1). The thoracic-abdominal ultrasound scan showed a poorly confined voluminous mass, having diameters of 120 x 86 mm, with echogenic-hyperechogenic structure and some hypo-anechogenic areas, arising in right hemithorax and displacing the liver and right kidney downwards. A magnetic resonance imaging (MRI) of the thorax showed a mass involving entirely the right hemitorax, with a centrai hemorrhagic component that displaced the mediastinum and the heart to the left. The patient underwent surgical thoracotomy, which revealed an unencapsulated mass with smooth surface and tense-elastic consistency, entirely covered by pleura, not adherent to the thoracic wall; since the conspicuous extension of the mass did not allow resection, only a biopsy was performed. Microscopically, the biopsy specimen showed a predominantly solid neoplasm with focal cysts. The tumour contained mesenchymal elongated cells arranged in sheets, and more primitive blastematous foci. There Fig. 1. Posterior-anterior chest radiograph at presentation, showing a bulky mass, displacing the mediastinum. was no evidence of typical rhabdomyoblasts or cartilage. Cysts exhibited an epithelial lining, with flattened to columnar cells and an underlying layer of primitive mesenchymal cells. The morphologic appearance was consistent with a diagnosis of PPB type II. Immunostains emphasized the doubl\ue9 component with a positive staining for cytokeratin (MNF116, pancytokeratin) in the epithelial component and a positive vimentin staining in mesenchymal component. Occasionai spindle cells were positive for desmin; oc-fetoprotein, S-100 protein, CD99, NB84A were negative in both the epithelial and the stremai component. In order to stage the disease the patient underwent total body bone scan with "Tc-MDP, brain and abdominal CT scan, and bone marrow aspirate; no metastatic spread was detected, and a stage III was defined. The child underwent chemotherapy with carboplatinum (CBP) 400 mg/m2 + etoposide (VP16) 150 mg/m2 days 1, 2; vincristine (VCR) 1.5 mg/m2 + actinomycin-D (ACT-D) 1.5 mg/m2 day 21 + ifosfamide (IFO) 1500 mg/m2 days 21-23, for overall 3 cycles; thereafter, 2 cycles were scheduled, including VCR 1.5 mg/m2 + ACTD 1.5 mg/m2 day 1, doxorubicin 40 mg/m2 days 1-2 and IFO 1500 mg/m2 days 1-3. The number of cycles were established according to the features of imaging studies. A chest x-ray survey showed a very good response (Fig. 2) to chemotherapy. Six months after the diagnosis complete resection of the tumour was performed through a right posterior-lateral thoracotomy by the fifth intercostal space. The tumour was capsulated and located between the upper and middle lobe of the right lung, displacing caudally the middle and lower pulmonary lobes. The centrai zone of the mass was composed of hyalinized fibrous stroma nodules and very small fragments of blastomatosous tumoral tissue, at about 2 cm from the resection borders. The neoplasm was almost entirely necrotic. 123 P. D''ANGELO ET AL. Fig. 2. Posterior-anterior chest radiograph, after chemotherapy, before surgical excision. After surgery the patient underwent 2 more cycles of chemotherapy with CBP 400 mg/m2 + VP 16 150 mg/m2/day x 2 days. There was clinical and imaging evidence of a progressive normalisation of lung morphology and function. The patient was monitored with clinical and radiological investigations according to the following schedule: chest radiograms every 3 months the first year, every 6 months the second and third year, every 12 months for the 4t h, 5l h and 6* year; MRI at 1 and 3 years after withdrawal of therapy. Nine years after the diagnosis, the child is in continuous complete remission. Discussion PPB in childhood is very rare. Our patient, as most of those reported in the literature 5 8 , presented unspecific respiratory symptoms; the x-ray revealed a large intrathoracic mass, suggesting the need for further imaging studies. It is important to emphasize the role of an early imaging examination (x-ray, ultrasound scan, CT or MRI) to detect as soon as possible the mass, in order to proceed to more specific investigations to elucidate the nature and staging of this malignant tumour. Radiographic findings of pleuropulmonary blastoma are not specific, especially when most of the neoplasm is cystic, resembling the radiographic features of teratoma. In this respect we note that PPB may initially manifest with clinical and radiologie signs and symptoms of pneumothorax 5 and may arise from other dysplastic conditions; as a matter of fact, cystic pulmonary adenomatoid malformation (CPAM) can be associated with PPB, which is also described in association with some congenital dysembriogenic abnormalities as cystic nephroma 3 . The clinical and radiological presentation in our patient showed mediastinal involvement; the mass was not excisable at the first surgical look because the neoplasm involved the pleura and was very large. The histopathologic diagnosis was consistent with type II PPB. The features described usually correlate with a poor prognosis 6 8 . The patient was submitted to intensive multiagent neoadiuvant chemotherapy, which reduced the tumour mass, making the complete surgical resection feasible, and allowing eradication of the malignancy. Such intensive multiagent chemotherapy is in most cases necessary for the reduction and complete excision of the tumor, which represents the most favourable factor for long term survival. In a recent report describing 11 patients 7 , two underwent total excision of the tumour at diagnosis, and were both alive without disease at 23 and 132 months respectively, with no adjuvant chemotherapy administered in the latter; another 3 patients remained disease free, two after macroscopic total resection and polychemotherapy and one after polychemotherapy and delayed complete surgery. The effectiveness of chemotherapy has also been reported by other Authors 8 1\ub0. The choice of the antiblastic agents used in our patients was due to their known effectiveness on mesenchymal and epithelial tumors n. Our patient was not treated with radiotherapy, which has proven to be effective in few patients 1. In conclusion, this case suggests that PPB may be taken in consideration for the differential diagnosis in respiratory distress. According to our experience and to other literature reports, total remission of this condition may be achieved with complete surgical excision (primary or delayed) and intensive chemotherapy. References 1 Manivel JC, Priest JR, Watterson J, Steiner M, Woods WG, Wick MR. Pleuropulmonary blastoma: the so called pulmonary blastoma of childhood. Cancer 1988;62:1516-26. 2 Spencer H. Pulmonary blastoma. J Pathol Bacteriol 1961 ;82:161-5. 3 Priest JR, Watterson J, Woods WG, Brid RI. Pleuropulmonary blastoma: a marker forfamilial disease. J Pediatr 1996;128:220-4. 4 Dehner LP. Watterson J, Priest J. Pleuropulmonaiy blastoma. A unique intrathorac\uecc-pulmonary neoplasm of childhood. In: Askin FB, Langston C, Rosemberg HS, eds. Pulmonary disease: perspectives in pediatrie pathology. Basel: Karger 1995, p. 214-26. 5 Guler E, Kutluk MT, Yalcin B, Cila A, Kale G, Buyukpamukcu M. Pleuropulmonary blastoma in a child presenting with pneumothorax. Tumori 2001;87:340-2. 6 Priest JR, McDermott MB, Bathia S, Watterson J, Manivel JC, Dehner LP. Pleuropulmonary blastoma. A clinic-pathologic study ofSOcases. Cancer 1997;80:146-61. 7 Indolfi P, Casale F, Carli M, Bisogno G, Ninfo V, Cecchetto G, et al. Pleuropulmonary blastoma: management and prognosis of 11 cases. Cancer 2000;89:1396-401. 8 Romeo C, Impellizzeri P, Grosso M, Vitarelli E, Gentile C. Pleu- 124 PLEUROPULMONARY BLASTOMA IN A CHILD ropulmonary blastoma: long-term survival and literature review. Med Pediatr Oncol 1999;33:372-6. Parsons SK, Fishman SJ, Hoorntje LE, Jaramillo D, Marcus KC, Perez-Atayde AR, et al. Aggressive multimodal treatment of pleuropulmonary blastoma. Ann Thorac Surg 2001;72:939-42. Ozkajnak MF, Ortega JA, Laug W, Gilsanz V, Isaacs H Jr. Role of chemotherapy in pediatrie pulmonary blastoma. Med Pediatr Oncol 1990;18:53-6. 12

    The HARPS search for southern extra-solar planets XXXV. The interesting case of HD41248: stellar activity, no planets?

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    The search for planets orbiting metal-poor stars is of uttermost importance for our understanding of the planet formation models. However, no dedicated searches have been conducted so far for very low mass planets orbiting such objects. Only a few cases of low mass planets orbiting metal-poor stars are thus known. Amongst these, HD41248 is a metal-poor, solar-type star on which a resonant pair of super-Earth like planets has In the present paper we present a new planet search program that is using the HARPS spectrograph to search for Neptunes and Super-Earths orbiting a sample of metal-poor FGK dwarfs. We then present a detailed analysis of an additional 162 radial velocity measurements of HD41248, obtained within this program, with the goal of confirming the existence of the proposed planetary system. We analyzed the precise radial velocities, obtained with the HARPS spectrograph, together with several stellar activity diagnostics and line profile indicators. A careful analysis shows no evidence for the planetary system previously announced. One of the signals, with a period of about 25 days, is shown to be related to the rotational period of the star, and is clearly seen in some of the activity proxies. The remaining signal (P~18 days) could not be convincingly retrieved in the new data set. We discuss possible causes for the complex (evolving) signals observed in the data of HD41248, proposing that they may be explained by the appearance and disappearance of active regions on the surface of a star with strong differential rotation, or by a combination of the sparse data sampling and active region evolution.Comment: Accepted for publication in A&

    Probe-dependent negative allosteric modulators of the long-chain free fatty acid receptor FFA4

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    High-affinity and selective antagonists that are able to block the actions of both endogenous and synthetic agonists of G protein–coupled receptors are integral to analysis of receptor function and to support suggestions of therapeutic potential. Although there is great interest in the potential of free fatty acid receptor 4 (FFA4) as a novel therapeutic target for the treatment of type II diabetes, the broad distribution pattern of this receptor suggests it may play a range of roles beyond glucose homeostasis in different cells and tissues. To date, a single molecule, 4-methyl-N-9H-xanthen-9-yl-benzenesulfonamide (AH-7614), has been described as an FFA4 antagonist; however, its mechanism of antagonism remains unknown. We synthesized AH-7614 and a chemical derivative and demonstrated these to be negative allosteric modulators (NAMs) of FFA4. Although these NAMs did inhibit FFA4 signaling induced by a range of endogenous and synthetic agonists, clear agonist probe dependence in the nature of allosteric modulation was apparent. Although AH-7614 did not antagonize the second long-chain free fatty acid receptor, free fatty acid receptor 1, the simple chemical structure of AH-7614 containing features found in many anticancer drugs suggests that a novel close chemical analog of AH-7614 devoid of FFA4 activity, 4-methyl-N-(9H-xanthen-9-yl)benzamide (TUG-1387), will also provide a useful control compound for future studies assessing FFA4 function. Using TUG-1387 alongside AH-7614, we show that endogenous activation of FFA4 expressed by murine C3H10T1/2 mesenchymal stem cells is required for induced differentiation of these cells toward a more mature, adipocyte-like phenotype

    Untargeted lipidomics uncovers lipid signatures distinguishing severe versus moderate forms of acutely decompensated cirrhosis

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    BACKGROUND AND AIM: Acutely decompensated of cirrhosis is a heterogeneous clinical entity associated with moderate mortality. In some patients, this condition develops quickly into a more often deadly acute-on-chronic liver failure (ACLF), in which other organs such as the kidneys or brain fail. The aim of this study was to characterize the blood lipidome in a large series of patients with cirrhosis and identify specific signatures associated with acute decompensation and ACLF development. METHODS: Serum untargeted lipidomics was performed in 561 patients with acutely decompensated (AD) cirrhosis (518 without and 43 with ACLF) (discovery cohort) and in 265 AD patients (128 without and 137 with ACLF) in whom serum samples were available to perform repeated measurements during the 28-day follow-up (validation cohort). Analyses were also performed in 78 AD patients included in a therapeutic albumin trial, 43 patients with compensated cirrhosis and 29 healthy subjects. RESULTS: The circulating lipid landscape associated with cirrhosis was characterized by a generalized suppression, which was more manifest during acute decompensation and in non-surviving patients. By computing discriminating accuracy and the variable importance projection score for each of the 223 annotated lipids, we identified a sphingomyelin fingerprint specific for AD cirrhosis and a distinct cholesteryl ester and lysophosphatidylcholine fingerprint for ACLF. Liver dysfunction, mainly, and infections were the principal net contributors to these fingerprints, which were dynamic and interchangeable between AD patients whose condition worsened to ACLF and those who improved. Notably, blood lysophosphatidylcholine levels increased in these patients after albumin therapy. CONCLUSIONS: Our findings provide insights into the lipid landscape associated with decompensation of cirrhosis and ACLF progression and identify unique noninvasive diagnostic biomarkers of advanced cirrhosis. LAY SUMMARY: Analysis of lipids in blood from patients with advanced cirrhosis reveals a general suppression of their levels in the circulation of these patients. A specific group of lipids known as sphingomyelins are useful to distinguish compensated from decompensated patients with cirrhosis. Another group of lipids designated cholesteryl esters further distinguish patients with decompensated patients who are at risk of developing organ failures

    Metabolic Treatment of Wolfram Syndrome

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    Wolfram Syndrome (WS) is a very rare genetic disorder characterized by several symptoms that occur from childhood to adulthood. Usually, the first clinical sign is non-autoimmune diabetes even if other clinical features (optic subatrophy, neurosensorial deafness, diabetes insipidus) may be present in an early state and may be diagnosed after diabetes' onset. Prognosis is poor, and the death occurs at the median age of 39 years as a consequence of progressive respiratory impairment, secondary to brain atrophy and neurological failure. The aim of this paper is the description of the metabolic treatment of the WS. We reported the experience of long treatment in patients with this syndrome diagnosed in pediatric age and followed also in adult age. It is known that there is a correlation between metabolic control of diabetes, the onset of other associated symptoms, and the progression of the neurodegenerative alterations. Therefore, a multidisciplinary approach is necessary in order to prevent, treat and carefully monitor all the comorbidities that may occur. An extensive understanding of WS from pathophysiology to novel possible therapy is fundamental and further studies are needed to better manage this devastating disease and to guarantee to patients a better quality of life and a longer life expectancy

    Detection of barium in the atmospheres of the ultra-hot gas giants WASP-76b and WASP-121b

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    Context. High-resolution spectroscopy studies of ultra-hot Jupiters have been key in our understanding of exoplanet atmospheres. Observing into the atmospheres of these giant planets allows for direct constraints on their atmospheric compositions and dynamics while laying the groundwork for new research regarding their formation and evolution environments. Aims. Two of the most well-studied ultra-hot Jupiters are WASP-76b and WASP-121b, with multiple detected chemical species and strong signatures of their atmospheric dynamics. We take a new look at these two exceptional ultra-hot Jupiters by reanalyzing the transit observations taken with ESPRESSO at the Very Large Telescope and attempt to detect additional species. Methods. To extract the planetary spectra of the two targets, we corrected for the telluric absorption and removed the stellar spectrum contributions. We then exploited new synthetic templates that were specifically designed for ultra-hot Jupiters in combination with the cross-correlation technique to unveil species that remained undetected by previous analyses. Results. We add a novel detection of Ba+ to the known atmospheric compositions of WASP-76b and WASP-121b, the heaviest species detected to date in any exoplanetary atmosphere, with additional new detections of Co and Sr+ and a tentative detection of Ti+ for WASP-121b. We also confirm the presence of Ca+, Cr, Fe, H, Li, Mg, Mn, Na, and V on both WASP-76b and WASP-121b, with the addition of Ca, Fe+, and Ni for the latter. Finally, we also confirm the clear asymmetric absorption feature of Ca+ on WASP-121b, with an excess absorption at the bluer wavelengths and an effective planet radius beyond the Roche lobe. This indicates that the signal may arise from the escape of planetary atmosphere. Key words: planets and satellites: atmospheres / planets and satellites: composition / planets and satellites: gaseous planets / techniques: spectroscopic / planets and satellites: individual: WASP-76b / planets and satellites: individual: WASP-121
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