718 research outputs found
Improving energy efficiency of commercial buildings by Combined Heat Cooling and Power plants
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
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].
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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-
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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?
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
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
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
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
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
Corrigendum: The silent epidemic of diabetic ketoacidosis at diagnosis of type 1 diabetes in children and adolescents in italy during the covid-19 pandemic in 2020(Front. Endocrinol., (2022), 13, (878634), 10.3389/fendo.2022.878634)
[This corrects the article .]
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