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We analyzed the main modalities and clinical outcomes of the early discharge outpatient model in autologous stem cell transplantation (EDOM-ASCT) for multiple myeloma in Italy. EDOM-ASCT was employed in 382 patients, for a total of 522 procedures, between 1998 and 2012. Our study showed high homogeneity among centers in terms of inclusion criteria, supportive care, and in hospital readmission criteria. Overall, readmissions during the aplastic phase occurred in 98 of 522 transplantations (18.8%). The major extrahematological complication was neutropenic fever in 161 cases (30.8%), which required readmission in 76 cases. The incidence of severe World Health Organization grade 3 to 4 mucositis was 9.6%. By univariate analysis, fever, mucositis, altered renal function at diagnosis, second transplantation, and transplantation performed late in the course of the disease were significantly correlated with readmission, whereas fever, mucositis, altered renal function, and timing of transplantation remained the only independent predictors by multivariate analysis. Overall, transplantation-related mortality was 1.0%. No center effect was observed in this study (P = .36). The safety and low rate of readmission of the EDOM-ASCT in myeloma trial suggest that this strategy could be extended to other transplantation centers if a stringent patient selection and appropriate management are applied. (C) 2014 American Society for Blood and Marrow Transplantation.

INQoL, myotonic dystrophy, nondystrophic myotonias, quality of life, SF-36, skeletal muscle channelopathies Background and purpose: Fatigue and pain have been previously shown to be important determinants for decreasing quality of life (QoL) in one report in patients with non-dystrophic myotonia. The aims of our study were to assess QoL in skeletal muscle channelopathies (SMC) using INQoL (individualized QoL) and SF-36 questionnaires. Methods: We administered INQoL and SF-36 to 66 Italian patients with SMC (26: periodic paralysis, 36: myotonia congenita and 4: Andersen-Tawil) and compared the results in 422 patients with myotonic dystrophies (DM1: 382; and DM2: 40). Results: (i) INQoL index in SMC is similar to that in DMs (P = 0.79). (ii) Patients with myotonia congenita have the worst perception of QoL. (iii) Myotonia has the most detrimental effect on patients with myotonia congenita, followed by patients with DM2 and then by patients with DM1 and hyperkalemic periodic paralysis. (iv) Pain is a significant complaint in patients with myotonia congenita, hypokalemic periodic paralysis and DM2 but not in DM1. (v) Fatigue has a similar detrimental effect on all patient groups except for patients with hyperkalemic periodic paralysis in whom muscle weakness and myotonia more than fatigue affect QoL perception. (vi) Muscle symptoms considered in INQoL correlate with physical symptoms assessed by SF-36 (R from 0.34 to 0.76). Conclusions: QoL perception in patients with SMC is similar to that of patients with DMs, chronic multisystem disabling conditions. Our results provide information to target treatment and health care of these patients. The sensitivity of INQoL to changes in QoL in the SMC needs to be further explored in longitudinal studies.

Objectives: To compare the condylar, ramal, condylar-plus-ramal mandibular asymmetry of patients with ectodermal dysplasia and healthy control subjects with using cone beam computerized tomography (CBCT). Materials and methods: CBCT of 9 patients (4 girls, 5 boys) with ectodermal dysplasia and 10 healthy control subjects (5 girls and 5 boys) with normal occlusion were included to our study. Mandibular asymmetry index measurements (condylar, ramal, and condylar-plus-ramal) were made on the CBCT. The paired samples t-test was used to establish statistically significant differences between the sides for condylar, ramal and condylar-plus-ramal height measurements. Independent t test was used to determine possible statistically significant differences between the groups. Results: There was no statistically significant difference between the right and left sides in condylar, ramal and condylar-plus-ramal height measurements of the patients with ectodermal dysplasia and normal occlusion sample. There was no statistically significant difference between the groups in asymmetry index measurements. Conclusion: Patients with ectodermal dysplasia show similar condylar, ramal, condylar-plus-ramal measurements with healthy control subjects.

A non myeloablative conditioning with total lymphoid irradiation (TLI) and antithymocyte globulin (ATG) was shown to protect against graft-versus-host disease (GVHD). To evaluate the effects of TLI-ATG in a multicenter study, 45 heavily pretreated patients, median age 51, with lymphoid (n = 38) and myeloid (n = 7) malignancies were enrolled at 9 centers. Twenty-eight patients (62%) received at least 3 lines of treatment before allografting, and 13 (29%) had refractory/relapsed disease at the time of transplantation. Peripheral blood hematopoietic cells were from HLA identical sibling (n = 30), HLA-matched (n = 9), or 1 antigen HLA-mismatched (n = 6) unrelated donors. A cumulative TLI dose of 8 Gy was administered from day −11 through −1 with ATG at the dose of 1.5 mg/kg/day (from day −11 through −7). GVHD prophylaxis consisted of cyclosporine and mycophenolate mofetil. Donor engraftment was reached in 95% of patients. Grade II to IV acute GVHD (aGVHD) developed in 6 patients (13.3%), and in 2 of these patients, it developed beyond day 100. Incidence of chronic GVHD (cGVHD) was 35.8%. One-year nonrelapse mortality was 9.1%. After a median follow-up of 28 months (range, 3-57 months) from transplantation, median overall survival was not reached, whereas median event-free survival was 20 months. This multicenter experience confirms that TLI-ATG protects against GVHD and maintains graft-vs-tumor effects.

Increased mobilization of circulating endothelial progenitor cells may represent a new biological hallmark of myeloproliferative neoplasms. We measured circulating endothelial colony forming cells (ECFCs) in 106 patients with primary myelofibrosis, fibrotic stage, 49 with prefibrotic myelofibrosis, 59 with essential thrombocythemia or polycythemia vera, and 43 normal controls. Levels of ECFC frequency for patient's characteristics were estimated by using logistic regression in univariate and multivariate setting. The sensitivity, specificity, likelihood ratios, and positive predictive value of increased ECFC frequency were calculated for the significantly associated characteristics. Increased frequency of ECFCs resulted independently associated with history of splanchnic vein thrombosis (adjusted odds ratio = 6.61, 95% CI = 2.54-17.16), and a summary measure of non-active disease, i.e. hemoglobin of 13.8 g/dL or lower, white blood cells count of 7.8×10(9)/L or lower, and platelet count of 400×10(9)/L or lower (adjusted odds ratio = 4.43, 95% CI = 1.45-13.49) Thirteen patients with splanchnic vein thrombosis non associated with myeloproliferative neoplasms were recruited as controls. We excluded a causal role of splanchnic vein thrombosis in ECFCs increase, since no control had elevated ECFCs. We concluded that increased frequency of ECFCs represents the biological hallmark of a non-active myeloproliferative neoplasm with high risk of splanchnic vein thrombosis. The recognition of this disease category copes with the phenotypic mimicry of myeloproliferative neoplasms. Due to inherent performance limitations of ECFCs assay, there is an urgent need to arrive to an acceptable standardization of ECFC assessment.

Pain still afflicts most cancer patients, mainly in the metastatic phases, and under-treatment is well documented. Transdermal delivery systems (TDS) containing fentanyl or buprenorphine could potentiality have advantages over oral and parenteral routes, but evidence from comparative trials are scanty. In the framework of a wider initiative, an Outcome Research Study was carried out in Italy in 2006 to evaluate the effects of various analgesic options, particularly buprenorphine TDS.This is a multicenter, open-label, prospective, nonrandomized study. Data were collected using a web-based standardized system, with a follow-up up of to 3 months. Pain intensity, the primary outcomes of the study, was measured using 11-point numerical rating scales from the Brief Pain Inventory.One-hundred ten centers recruited 1801 cases, most of which (60%) were receiving a strong opioid at the time of inclusion. Of these, 257 had TDS buprenorphine as first choice. Of the remaining 709 patients who at the time of inclusion were not on a strong opioid, 325 changed to a strong opioid and in 43% it was TDS buprenorphine. During the follow-up, physicians had to increase the dosage to control pain (average increase between 16% and 17%). About 34% of patients had an improvement of at least 2 points in worst pain, 15% had a 20% improvement in pain relief, and 40% in satisfaction. Results were in line with those of patients receiving other World Health Organization-level III opioids.Despite the limitations owing to the observational design, these findings may be useful to clinicians to judge the value of the drug under evaluation better and to help researchers design further comparative studies.

One hundred six early features were retrospectively collected on 44 patients with acute pancreatitis and stored in a database programmed on a microcomputer. Intraoperative/autopsy findings or clinical course categorized patients as having severe (n = 16) or mild (n = 28) pancreatitis. The frequency of 88 early available variables was analyzed. Twenty significant differences were found and used to program a Bayesian prediction of severity, assuming their independence. The computer's performance was assessed by retrospective testing of the patients, and a further 47 (14 severe, 33 mild) were studied prospectively, with a comparison carried out involving the unaided prediction of the clinicians. Finally, all 91 patients were retrospectively tested with the early predictive Ranson's signs and the results were compared with the computer's output. In the prospective evaluation, the computer's performance was 89.4% accuracy, 100% sensitivity, 84.8% specificity, while that of the physicians was 65.9%, 71.4%, 63.6%, respectively, like the performances of Ranson's criteria. Methodologic problems were: selecting patients to study, based on wide inclusion and exclusion in hindsight of the false-positives; dividing them into groups, based on both pathologic and clinical criteria; and the assumption of independence within the variables. The use of the procedure in other populations requires a specific analysis of the discriminant variables, but this study suggests a method to compare several samples of patients which allows prospective and controlled clinical trials.

We are reporting the suppurative abscess of an endometriosic ovarian cyst due toSalmonella brandenburg in a 30-year-old woman. We have stressed the extra-intestinal localization, e. g. an endometriosic ovarian cyst, and other well-known focal manifestations of salmonellosis.

In this paper we investigate the structural and optical properties of few strain-unbalanced multiple Ge/GeSi quantum wells pseudomorphically grown on GeSi reverse-graded substrates. The obtained high epitaxial quality demonstrates that strain symmetrization is not a mandatory requirement for few quantum-well repetitions. Photoluminescence data, supported by a thorough theoretical modeling, allow us to unambiguously disentangle the spectral features of the quantum wells from those originating in the virtual substrate and to evaluate the impact on the optical properties of key parameters, such as quantum confinement, layer compositions, excess carrier density, and lattice strain. This detailed understanding of the radiative recombination processes is of paramount importance for the development of Ge/GeSi-based optical devices.

A first screening by ultrasound can be relevant to set a specific diagnostic and therapeutic route for a patient with a COVID-19 infection. The finding of bilateral B-lines and white lung areas with patchy peripheral distribution and sparing areas is the most suggestive ultrasound picture of COVID-19 pneumonia. Failure to detect bilateral interstitial syndrome (A pattern) on ultrasound excludes COVID-19 pneumonia with good diagnostic accuracy, but does not exclude current infection. The use of shared semiotic and reporting schemes allows the comparison and monitoring of the COVID-19 pulmonary involvement over time. This review aims to summarise the main data on pulmonary ultrasound and COVID-19 to provide accurate and relevant information for clinical practice.

We present THz quantum cascade emitters realized on a Si substrate. The emission centered at 3.4 and 4.9 THz originates from L-valley transitions in strain-compensated n-Type Ge/SiGe heterostructures. This is an important step towards the realization of Si-based THz quantum cascade lasers.

A patient with advanced gastrointestinal stromal tumor (GIST) receiving second-line treatment with sunitinib developed edema, increase of the serum creatinine, weight gain, nephrotic syndrome with proteinuria of 12 g/24 h, dyslipidemia, hypoalbuminemia and also presented with hypertension. A kidney biopsy showed an immunocomplex glomerulonephritis. Steroid treatment was started, but the clinical conditions and laboratory values did not improve. So in the hypothesis that the nephrotic syndrome was induced by sunitinib, sunitinib was temporarily discontinued with a subsequent reduction of proteinuria and improvement in blood pressure control. In the last years, the introduction of sunitinib has modified the natural history of advanced GIST. However, due to chronic and prolonged intake of this drug, there is increasingly frequent detection of late and unknown toxicities in clinical practice. In particular, the late renal toxicity from sunitinib may be the primary clinical problem with this drug in the case of prolonged treatment. Monitoring of kidney function and blood pressure should be performed for early detection of side effects such as hypertension and kidney dysfunction in advanced GIST patients receiving long-term treatment with sunitinib. A clinical collaboration between oncologists and nephrologists could be useful with the objective to optimize the management of sunitinib.

This study defined the clinical features and assessed the prognosis of 47 patients (17 males, 30 females, median age 63 years) with primary nodal marginal zone B-cell lymphoma. Forty-five per cent had stage IV disease. Hepatitis C virus serology was positive in 24%. According to the Follicular Lymphoma International Prognostic Index (FLIPI), 33% were classified as low-risk, 34% as intermediate-risk, and 33% as high-risk. The 5-year overall survival (OS) was 69%. In univariate analysis worse OS was associated with: FLIPI (P = 0.02), age > 60 years (P = 0.05) and raised lactate dehydrogenase (P = 0.05). In multivariate analysis, only FLIPI predicted a worse OS (P = 0.02).

Objective:We report updated results at a median follow-up of 12 years of a phase II trial assessing first-line MATILDE chemotherapy and response-tailored radiotherapy in patients with primary CNS lymphomas (PCNSL).Methods:Forty-one HIV-negative patients (18-70 years; Eastern Cooperative Oncology Group performance status 3) with histologically confirmed PCNSL received 3 courses of MATILDE chemotherapy followed by whole-brain radiotherapy (WBRT). Chemotherapy activity was the primary endpoint.Results:Overall response rate was 76% (95% confidence interval [CI] 63%-89%) after chemotherapy and 83% (95% CI 71%-95%) after chemotherapy radiotherapy. At a median follow-up of 144 months (range 47-153), 31 patients experienced an event: relapse in 24, progressive disease in 3, and toxic death in 4, with a 5-year progression-free survival of 24% 8%. Two patients experienced a late relapse (100 and 101 months). Nine patients are alive and disease-free, 8 of whom are alive at >10 years, with a 5-year overall survival of 30% +/- 7%. At 10 years from diagnosis, no patient showed chronic hematologic and nonhematologic toxicities, with a Mini-Mental State Examination score of 29 in all cases but one.Conclusions:At a median follow-up of 12 years, MATILDE regimen followed by WBRT confirmed the previously reported survival plateau, which further proves its long-lasting efficacy with acceptable neurologic deficits.Classification of evidence:This study provides Class IV evidence that in patients with PCNSL, MATILDE chemotherapy followed by response-tailored radiotherapy increases the probability of disease remission at 12 years. OI Baldini, Luca/0000-0003-2964-9981

Background Kidney transplant recipients (KTRs) manifest hypercoagulable state that contributes to an increased incidence of deep vein thrombosis (DVT), not only early but also late in their course. KTRs display an imbalance of hemostatic mechanisms with a multifactorial rise in procoagulant factors, partly related to traditional risk factors and partly to transplantation. The aim of this study was to evaluate the incidence of first episodes of DVT among KTRs, focusing on risk factors. Methods From 2008 to 2011, we evaluated 30 kidney transplant patients who ≥4 months there after transplantation developed DVT in the lower limbs only, lower limbs complicated by pulmonary embolism or retinal thrombosis. We analyzed causes of primary nephropathy, immunosuppressive regimen, post-transplantation infections, and erythrocytosis. DVT was diagnosed by color Doppler ultrasound or eye examination. Results A significantly increased incidence of DVT was observed among patients receiving cyclosporine or cyclosporine + mammalian target of rapamycin inhibitors, affected by polycystic kidney diseases, systemic lupus erythematosus or nephrotic syndrome, or displaying rapid and/or excessive correction of hematocrit values. DVT was not significantly related to an acute infection (cytomegalovirus) or to the prior dialysis modality. Conclusions Hypercoagulability is a multifactorial condition in KTRs, representing a severe complication in stable patients. Prevention may consist of either accurate pretransplantation screening for thrombophilia or identification of patients at higher DVT risk.

Introduction Ectodermal dysplasia syndromes are a heterogeneous group of inherited diseases characterised by abnormal development of tissues of ectodermal origin. The most common form of ectodermal dysplasia syndromesis X-linked hypohidrotic ectodermal dysplasia characterised by abnormalities of the skin, teeth, hair and sweat glands. The intraoral abnormalities include hypodontia, malformed teeth (conically shaped) and reduced alveolar ridge height. It causes severe impairment of chewing, swallowing, speech, aesthetics and affects social relation. Early dental treatment at 2-3 years is essential to improve oral function and reduce the social impairment. This may include resin bonded restorations to conventional prosthetic treatment. In some cases suffering from severe hypodontia, however, conventional prostheses are inadequate due to lack of retention and instability. The replacement of teeth by implants is usually restricted to patients with completed craniofacial growth; however, implants can be used as abutments for overdentures.This study reports a 9-year follow-up case of a child affected with X-linked hypohidrotic ectodermal dysplasiaaccompanied by anodontia. Case report At the age of 2 years, conventional upper and lower removable prostheses were fabricated. Subsequently, at the age of 11 years and 11 months; the patient was treated with a lower implant supported overdenture placed on two tapered implants (3.8 × 10 mm) in the anterior mandible. Cone beam computer tomography of the mandible was done and Dicom data used to obtain a rapid stereolithographic model. Conclusion Implants can be successfully placed, restored and loaded in growing patients with ectodermal dysplasia

Real-time elastography (RTE) is a novel technique for measuring tissue elasticity. The aims of this study were to prospectively measure liver stiffness with RTE in patients with chronic viral hepatitis and to evaluate the possible correlation between RTE data and the extent of fibrosis based on liver biopsy findings (Ishak score).Between February and October 2011, 26 patients (18M, 8F, mean age 41 ± 13 [standard deviation], range 22-62) with chronic viral hepatitis were prospectively evaluated with ultrasonography (US) that included RTE. All patients then underwent US-guided percutaneous liver biopsy (right lobe) for evaluation of fibrosis. Examinations were performed with a iU22 scanner (Philips, Bothell, WA, USA); a convex transducer (C5-1) was used for the US examination, and a linear transducer (L12-5) for RTE. In the RTE images, relative tissue stiffness is expressed according to a color scale with soft areas represented in green/red and hard areas in blue. Patients were examined in the supine position in suspended normal respiration; three loops of 20 RTE frames were recorded for each case. For each patient, we calculated the mean strain ratio (MSR) for the 3 loops. The Spearman correlation coefficient was used to assess correlation between the ASR and fibrosis stage (F) reflected by the Ishak score.The Spearman coefficient showed significant correlation between the MSR and F (Rho = 0.470, p = 0.015).RTE appears to be a useful tool for noninvasive evaluation of fibrosis in patients with chronic viral hepatitis although these findings need to be confirmed in larger case series.L'elastosonografia real-time (ERT) è una nuova tecnologia integrata all’ultrasonografia convenzionale in grado di rappresentare la rigidità dei tessuti. Scopo dello studio è quantificare la rigidità epatica tramite ERT in pazienti con epatopatia cronica virale e ricercare una correlazione con lo staging istologico di malattia.Da Febbraio a Ottobre 2011, 26 pazienti (18M, 8F, età media 41 ± 13 [deviazione standard], range 22–62) sono stati valutati prospetticamente con ecografia addominale (US) ed ERT e sono stati sottoposti a biopsia epatica (lobo destro) secondo indicazione clinica. Gli esami sono stati eseguiti con l'ecografo iU22 (Philips, Bothell, WA, USA) usando per l'US una sonda convex (C5-1) e per l'ERT una lineare (L12-5). L'apparecchiatura rappresenta la rigidità relativa dei tessuti esplorati secondo una scala cromatica, mostrando le porzioni più rigide in blu e quelle più morbide in verde/rosso. Per tutti i pazienti, sono stati registrati 3 loop di 20 frame di ERT; è stata ottenuta la media delle medie degli strain ratio dei 3 loop (MSR) ed è stato calcolato l’indice di correlazione di Spearman (IS) tra la MSR e lo stadio di fibrosi (F) secondo Ishak.L’IS è risultato significativo (rho = 0.470,l'ERT potrebbe essere utile per la quantificazione non invasiva della fibrosi nei pazienti con epatopatia cronica su base virale, ma per confermare questa ipotesi è necessaria una casistica più ampia.

Abstract 2009 To evaluate the role of allografting from unrelated donors in the treatment of myeloma we conducted a retrospective study through the Italian Bone Marrow Transplantation Donor Registry. Overall, from 2000 through 2009, 196 myeloma patients, median age 51 years (32–67), for a total of 199 allografts, were transplanted from an unrelated donor at 34 Centers in Italy. Fifty-two (28.1%%), 69 (37.3%), and 64 (34.6%) patients were prepared for transplant with a myeloablative, a reduced-intensity and a non-myeloablative conditioning respectively. Patient characteristics of the 3 cohorts are reported in Table 1.ConditioningMyeloablativeReduced-intensityNon-myeloablativePatient number (%)52/185 (28)69/185 (37)64/185 (35)Median Age455355Previous therapy lines < 2 (%)23 (27)33 (38)30 (35)Previous therapy lines ≥ 2 (%)29 (29)36 (37)34 (34)Stem Cell Source BM (%)24 (57)18 (43)0 (0)Stem Cell Source PBSC (%)28 (19)51 (36)64 (45) Cumulative incidence of acute grade II-IV graft-versus-host-disease (GVHD) was 46.4% whereas chronic GVHD was 45.1%. There was no difference in GVHD incidence among the 3 cohorts defined by type of conditioning. Complete and partial remissions in patients who survived at least 3 months post-transplant were 27% and 28% for an overall response rate of 55%. At a median follow up of 32 (0–118) months post-transplant, in the entire study population, median OS from diagnosis was 70.6 months while OS and EFS from the allograft were 18.9 and 14.9 months. Overall, the cumulative incidence of transplant related mortality (TRM) was 29.6% at 1 year and 32.4% at 5 years post-transplant. OS from diagnosis and EFS from transplant were 70.6 and 28.2 months; 66.8 and 9.1 months; and 111.9 and 22.4 months in patients who respectively underwent a myeloablative, a reduced-intensity and a non-myeloablative transplant. One-year and 5-year TRM was 33.3% and 35.7%, 32.2% and 34.4%, and 22.1% and 26.5% respectively. Univariate and multivariate analyses, assessed by multivariate Cox proportional hazards models, were performed for the following variables: number of previous chemotherapy lines (1,2 vs. ≥3), disease status at transplant, HLA- matched antigens (10/10 vs. 9/10 vs. ≤8/10), recipient/donor gender combinations, hematopoietic cell source (peripheral blood vs. bone marrow), conditioning (myeloablative vs. reduced-intensity vs. non-myeloablative), use of anti-thymoglobulin in the conditioning, acute GVHD, chronic GVHD, best response post-transplant, year of transplant (2000–02 vs. 2003–05vs. 2006–09). By univariate analysis, lower number of chemotherapy lines before the allograft, disease status at transplant, a fully HLA-identical donor, the use of peripheral hematopoietic cells rather than bone marrow were statistically significant variables for better OS whereas disease status at transplant, a fully HLA-identical donor, chronic GVHD (either limited or extensive) were statistically significant for better EFS. However, by multivariate analysis, only the development of chronic GVHD (HR 0.50; p Disclosures: Patriarca: Roche: Honoraria; Celgene: Honoraria; Schering-Plough: Honoraria; Janssen: Honoraria. Boccadoro:Celgene: Consultancy, Membership on an entity's Board of Directors or advisory committees, Research Funding; Janssen-Cilag: Consultancy, Membership on an entity's Board of Directors or advisory committees, Research Funding.

Background and purpose: A quality of life (QoL) questionnaire for neuromuscular diseases was recently constructed and validated in the United Kingdom in a sample of adult patients with a variety of muscle disorders. Preliminary results suggested it could be a more relevant and practical measure of QoL in muscle diseases than generic health measures of QoL. The purpose of our work was: (i) To validate INQoL in Italy on a larger sample of adult patients with muscle diseases (ii) to compare INQoL to SF-36. Methods: We have translated into Italian and applied language adaptations to the original UK INQoL version. We studied 1092 patients with different muscle disorders and performed (i) test–retest reliability (n = 80); (ii) psychometric (n = 345), knowngroup (n = 1092), external criterion (n = 70), and concurrent validity with SF-36 (n = 183). Results: We have translated and formally validated the Italian version of INQoL confirming and extending results obtained in the United Kingdom. In addition to good results in terms of reliability, known-group and criterion validity, a comparison with the SF-36 scales showed a stronger association between INQoL total index and SF-36 physical (r = )0.72) than mental (r = )0.38) summary health indexes. When considering comparable domains of INQoL and SF-36 with respect to an objective measure of muscle strength assessment (MMRC), regression analysis showed a stronger correlation using INQoL rather than SF-36 scores. Conclusions: INQoL is recommended to assess QoL in muscle diseases because of its ability to capture physical limitations that are specifically relevant to the muscle condition.

AML patients (total 129; median age =50 years; range 16-72) in first CR received BU and melphalan (BU/Mel) as conditioning regimen before auto-SCT. In all, 82 patients (63.6%) received PBSCs and 47 patients (36.4%) received BM cells. The distribution of cytogenetic categories was conventionally defined as favorable (15.5%), intermediate (60.1%) and unfavorable (24.3%). With a median follow-up of 31 months, the 8-year projected OS and disease-free survival (DFS) was 62 and 56% for the whole population, respectively. The relapse rate was 46% and the non-relapse mortality was 4.65%. Although PBSC transplantation led to a faster hematological recovery than BM transplantation, in univariate analysis the stem cell source, cytogenetics and different BU formulations did not significantly affect OS and DFS, whereas age and the number of post-remission chemotherapy cycles did have a significant effect on the clinical outcome. Multivariate analysis identified age

In this paper, on the basis of a simplified hybrid model of an Automated Manual Transmission (AMT) system with servo-actuated clutch and gearbox, the control strategy for the gearshift is formally addressed as a hybrid optimal control problem and solved by means of numerical optimization method. The gear upshift request is performed with locked clutch and achieving the synchronization of the requested gear by exploiting engine cut-off and properly controlling the engine and the gearbox. Performance achievable with the proposed strategy under various vehicle conditions and engine regimes are shown.

The authors assessed MATILDE chemotherapy followed by response-tailored radiation therapy in 41 patients aged 70 years or younger with primary CNS lymphoma in a Phase II trial. With response rates of 76% after MATILDE and 83% after chemotherapy with or without radiation therapy, this was an active strategy, particularly in low- to intermediate-risk patients (International Extranodal Lymphoma Study Group [IELSG] score). Myelosuppression was the dose-limiting toxicity, with 9.5% of lethal complications. After a median follow-up of 49 months, a plateau in the survival curve (5-year overall survival: 41 +/- 7%) was obtained.

Control of an electrohydraulic servo-actuated clutch for an automatic gear-shifting system for commercial cars is considered. A simplified model, which takes into account the fundamental behavior of the system, is mandatory for the design of the clutch control. The clutch position tracking controller, designed in the framework of backstepping technique, is based on a cascade structure with nested position, speed and pressure control loops. Feed-forward terms obtained through model inversion increase the dynamic performances, while closed-loop regulators provide robustness with respect to model uncertainties. In the controller design, nonlinearities of the mechanical actuator and of the servovalve are taken into account in order to improve achievable performances. The controller behavior during tracking of smooth position references has been experimentally tested.

Camless internal combustion engines offer major improvements over traditional engines in terms of efficiency, pollutant emissions, maximum torque and power. Electromechanical valve actuators are very promising in this context, but still present significant control problems. Low valve seating velocity, small transition time for valve opening and closing are conflicting objectives that need to be jointly considered when designing the valve control system. The paper presents a hybrid control system architecture capable to deal with all these issues. The design of the valve position controller is addressed in detail, in the framework of backstepping approach. Simulation results show the effectiveness of the proposed solution.

The aim of this study was to describe the clinical treatment of young patients, affected by ectodermal dysplasia (ED), and to possibly establish clinical guidelines. The study design was case series. ED syndromes (EDs) are a heterogeneous group of inherited diseases characterised by abnormal development of tissues of ectodermal origin. The most common form of EDs is X linked hypohidrotic ED (HED). Characteristic triad of HED is oligo-anodontia, hypotricosis, hypo-anhydrosis. Oligo-anodontia is one of the most severe impairment, since it affects chewing, swallowing, speech, esthetics and social relation. Early prosthetic rehabilitation (at 2-3 years of age), with partial or complete dentures, is essential to improve oral function and reduce the social impairment.

Terahertz (THz) semiconductor based quantum cascade lasers (QCLs) represent powerful and compact integrated light sources. To date they have been realized with various III-V materials where LO phonon emission becomes very effective as the temperature is increased, limiting the maximum operating temperature to 200K. To overcome this limitation, non-polar material systems are attractive because of their weaker e-phonon interaction. Theoretical studies have indicated n-type Ge/SiGe heterostructures where transport is associated to L electrons, as the most promising architecture [1]. Experimentally, sharp intersubband transitions in n-type strain compensated Ge/SiGe QWs have been observed in the 20–50 meV region [2]. Recently, non-equilibrium Green's functions calculations proved to be efficient in optimising high temperature performance of GaAs/AlGaAs THZ QCLs [3]. In the effort of realizing a Ge/SiGe THz QCL, we present here a QCL structure designed by means of non-equilibrium Green's functions [4], whose the bandstructure is reported in Fig.1(a). Epitaxial growth of strain-compensated Ge/SiGe heterostructure (≈1.5 μm thick) has been carried out by means of UHV-CVD yielding very good sample quality as visible from Fig.1(b). Mesa devices for transport experiments have been fabricated by dry etching and metallization. In Fig.1(c) a series of I-V curves as a function of the heatsink temperature display a thermally activated behaviour with E act ≃ 10 meV. From low temperature (4 K) magnetotransport [5] in a magnetic field parallel to the current direction shows characteristics oscillations. we can deduce an energy distance between the lasing states of E 23 =19.5 meV, in good agreement with the theoretical calculation. Optical experiments are ongoing and experimental results will be presented.