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Background Carotid endarterectomy (CEA) represents a standard procedure in case of symptomatic carotid stenosis of 50–99% within 2 weeks from onset of stroke or transient ischemic attack (TIA) symptoms. The optimal time to perform CEA after intravenous thrombolysis (IVT) is still unclear. The aim of this study was to analyze the safety of CEA performed within 2 weeks from IVT. Materials and methods A consecutive series of 70 patients affected by symptomatic carotid stenosis have been treated as per the international guidelines during 3 years. Eleven (15.7%) patients have been treated with IVT before CEA for ischemic stroke; remaining 59 (84.3%) patients received only CEA. CEA was performed in median 8 days (range: 2–13) after IVT. We examined the grade of disability before and after surgery as well as at 3 months follow-up, using the modified Ranking Scale (mRS). Results Among the patients who underwent CEA + IVT, CEA was performed in median 8 days (range: 2–13) after IVT. One patient received CEA within 48 hours from IVT, 3 patients within 72 hours, and 7 patients within 2 weeks. The complications within 90 days from surgery, in CEA + IVT group, were 3 cases of intracerebral hemorrhage (ICH) without symptoms. In patients who received only CEA, the complications were 1 case of stroke and 2 cases of ICH. The mortality registered was 0% in both groups. Among CEA + IVT group at 90 days after surgery, 9 patients had a mRS grade of 0–2, 2 patients had mRS of 3–5. Conclusions In our series, IVT before CEA did not seem to increase the rate of complications. However, the study has several limitations, and further studies must be performed before solid evidence is available for recommendations regarding the timing of CEA after IVT.

Understanding infections related to handling healthcare waste products is of critical importance and the application of simple and low-cost strategies remain a priority in low-income and middle-income countries to protect healthcare workers. We examined the potential effect of relative humidity (RH), air temperature and ultraviolet irradiation (UI) to establish an efficient and effective way to facilitate disposal of medical waste. Literature is emerging on the effect of high RH and high temperature, which would increase airborne mass deposition and decrease the viability of viruses in both airborne particles and on surfaces. On the other hand, severe acute respiratory syndrome coronavirus-2 has been proven to be susceptible to UI when suspended in air like other coronaviruses. An innovative approach utilizing environmental conditions might represent an effective and efficient way to ensure better and sustainable protection of the healthcare workers in low-resourced settings.

To compare unassisted and CAD-assisted detection and time efficiency of radiologists in reporting lung nodules on CT scans taken from patients with extra-thoracic malignancies using a Cloud-based system. Three radiologists searched for pulmonary nodules in patients with extra-thoracic malignancy who underwent CT (slice thickness/spacing 2 mm/1.7 mm) between September 2015 and March 2016. All nodules detected by unassisted reading were measured and coordinates were uploaded on a cloud-based system. CAD marks were then reviewed by the same readers using the cloud-based interface. To establish the reference standard all nodules ≥ 3 mm detected by at least one radiologist were validated by two additional experienced radiologists in consensus. Reader detection rate and reporting time with and without CAD were compared. The study was approved by the local ethics committee. All patients signed written informed consent. The series included 225 patients (age range 21–90 years, mean 62 years), including 75 patients having at least one nodule, for a total of 215 nodules. Stand-alone CAD sensitivity for lesions ≥ 3 mm was 85% (183/215, 95% CI: 82–91); mean false-positive rate per scan was 3.8. Sensitivity across readers in detecting lesions ≥ 3 mm was statistically higher using CAD: 65% (95% CI: 61–69) versus 88% (95% CI: 86–91, p

The treatment of cystic fibrosis (CF) patients homozygous for the F508del mutation with Orkambi&reg, a combination of a corrector (lumacaftor) and a potentiator (ivacaftor) of the mutated CFTR protein, resulted in some amelioration of the respiratory function. However, a great variability in the clinical response was also observed. The aim of this study was to evaluate the response to Orkambi&reg, in a small cohort of F508del/F508del patients (n = 14) in terms of clinical and laboratory parameters, including ex vivo CFTR activity in mononuclear cells (MNCs), during a 12-month treatment. Patients responded with an increase in percent predicted forced expiratory volume in 1 s (FEV1%) and body mass index (BMI) as well as with a decrease in white blood cell (WBC) total counts and serum C-reactive protein (CRP) levels, although not significantly. Sweat chloride and CFTR-dependent chloride efflux were found to decrease and increase, respectively, as compared with pre-therapy values. CFTR and BMI showed a statistically significant correlation during Orkambi&reg, treatment. Clustering analysis showed that CFTR, BMI, sweat chloride, FEV1%, and WBC were strongly associated. These data support the notion that CFTR-dependent chloride efflux in MNCs should be investigated as a sensitive outcome measure of Orkambi&reg, treatment in CF patients.

Supplemental Digital Content is available in the text., Background and Purpose: We aimed to investigate the rate of hospital admissions for cerebrovascular events and of revascularization treatments for acute ischemic stroke in Italy during the coronavirus disease 2019 (COVID-19) outbreak. Methods: The Italian Stroke Organization performed a multicenter study involving 93 Italian Stroke Units. We collected information on hospital admissions for cerebrovascular events from March 1 to March 31, 2020 (study period), and from March 1 to March 31, 2019 (control period). Results: Ischemic strokes decreased from 2399 in 2019 to 1810 in 2020, with a corresponding hospitalization rate ratio (RR) of 0.75 ([95% CI, 0.71–0.80] P

The Chapman psychosis-proneness scales—also known as Wisconsin schizotypy scales (WSS)—are among the most used tools to measure schizotypy. The factor structure of the short-form WSS was investigated in a mixed sample of patients with chronic mental disorders and of healthy subjects from the general population. One hundred patients with a chronic mental disorder were enrolled over a 6-month period. For each patient, two controls of same sex and similar age (±5 years) were enrolled; 131 accepted to take part in the study. The unidimensional, the correlated four-factor, the second-order two-factor models, and the bifactor model with two or four orthogonally independent factors of the short-form WSS were tested with confirmatory factor analysis. Good reliability of the short-form WSS was confirmed, as its capacity of differentiating people with and without schizotypy. The bifactor models were superior to other models. However, in both bifactor models the explained common variance (ECV) attributable to the general factor and the percentage of uncontaminated correlations (PUC) were too low to use a general summary score as a measure of a single latent schizotypy variable. Symptoms scores derived from the short-form WSS can be better appreciated within a multidimensional model of schizotypy.

Giant cell tumor (GCT) of the bone is a benign but locally aggressive tumor arising in the bones. The sacrum represents the most common involved site of the spine. At this level GCT might cause relevant neurological deficits and/or impair activities of daily living. The mainstay of treatment is still represented by the adequate removal of the tumor limiting at the same time the impact of surgery. However, GCT may relapse or present in “difficult” locations or with very large destructive lesions that may not be operated preserving the function of the involved bone. In this scenario, subcutaneous administration of denosumab, a fully-humanized monoclonal antibody specifically directed against RANK-L, can reduce surgical risks and invasiveness improving outcomes. Here we present the case of a young lady affected by a large GCT of the sacrum that emphasizes the importance of the multidisciplinary management of this disease taking into account patient’s preferences.

Dabigatran is one of the nonvitamin K antagonist oral anticoagulants. Thrombolytic treatment with intravenous recombinant tissue plasminogen activator is contraindicated in patients taking a DOAC. Idarucizumab was recently approved for dabigatran-activity reversing in severe bleeding, emergency surgery, or urgent procedures, but many attempts have been made to use idarucizumab in patients presenting with acute ischemic stroke in order to be eligible for thrombolysis.Our patient was an 89-year-old woman with severe aphasia who was treated with dabigatran for nonvalvular atrial fibrillation. She received an infusion of idarucizumab followed by thrombolytic therapy, with complete remission of symptoms after 24 hours.Idarucizumab is a safe option for patients with acute ischemic stroke treated with dabigatran; otherwise eligibles for thrombolysis, even in very old people like our patient.

BACKGROUND Ivacaftor is a significant innovation in the treatment of cystic fibrosis (CF) with gating mutations. A substantial percentage of patients with CF have severe lung involvement, but these patients are usually excluded from phase III clinical trials. Thus, the effectiveness of ivacaftor in this population has not been fully determined. METHODS Data were collected from Italian CF centers with patients enrolled in an ivacaftor compassionate use programme (percent predicted [pp] forced expiratory volume in 1 second [FEV1 ]

Bone metastases occur in up to 70% of cancer patients, and frequently involve the spine. Spine metastases are often associated with pain, disability and progressive deformity, and may also have neurological complications, all of which can dramatically impair quality of life. There are a number of different approaches to managing vertebral metastases, including surgery, vertebroplasty and radiotherapy. The variety of treatment modalities involved, the presence of underlying cancer and frequent severe pain means that patients with vertebral metastases need to be managed by a multidisciplinary team, ideally including a medical oncologist, radiation oncologist, interventional radiologist, pain therapist and spine surgeon. Although a number of different multidisciplinary therapeutic algorithms have been proposed, there is no clear consensus on the best way to manage vertebral metastases. After reviewing current literature, this article proposes a new visual algorithm created by merging some existing guidelines and introducing additional interventional radiology techniques.

Few mutations in cis have been annotated for F508del homozygous patients. Southern Italy patients who at a first analysis appeared homozygous for the F508del mutation (n=63) or compound heterozygous for the F508del and another mutation in the cystic fibrosis transmembrane conductance regulator gene (n=155) were searched for the A238V mutation in exon 6. The allelic frequency of the complex allele [A238V;F508del] was 0.04. When the whole data set was used (comprised also of 56 F508del/F508del and 34 F508del/other mutation controls), no differences reached the statistical significance in the clinical parameters, except chloride concentrations which were lower in [A238V;F508del]/other mutation compared with F508del/other mutation (P=0.03). The two study groups presented less complications than the control groups. Within the minimal data set (34 F508del/F508del, 27 F508del/other mutation, 4 [A238V;F508del]/F508del cases and 5 [A238V;F508del]/other mutation cases); that is, presenting all the variables in each patient, forced expiratory volume in 1 s and forced vital capacity presented a trend to lower levels in the study groups in comparison with the F508del/F508del group, and C-reactive protein approximated statistically significant higher levels in the [A238V;F508del]/other mutation as compared with F508del/F508del patients (P=0.09). The analysis of statistical dependence among the variables showed a significant anticorrelation between chloride and body mass index in the [A238V;F508del]/other mutation group. In conclusion, the complex allele [A238V;F508del] seems to be associated with less general complications than in the control groups, on the other hand possibly giving a worse pulmonary phenotype and higher systemic/local inflammatory response. These findings have implications for the correct recruitment and clinical response of F508del patients in the clinical trials testing the new etiological drugs for cystic fibrosis.

Very few cases of gastric paragangliomas have been reported in the literature to date. We report a rare case of parietal gastric paraganglioma fortuitously detected during intraoperative exploration. A 82-years-old woman presented to our emergency room for abdominal pain. On physical examination abdomen was painful on palpation and Blumberg's sign was present. The laboratory exams showed a neutrophilia in absence of leukocytosis. Acute appendicitis was suspected and a laparoscopy was performed. At exploration, the vermiform appendix was normal while a lumpy, hard-fibrous and white-pinkish extraluminal lesion of the anterior wall of the gastric body near the greater curvature of about 2 cm in diameter was present. Laparoscopic resection of the gastric lesion was performed. The patient was discharged in good condition in the fourth postoperative day. Pathologic examination revealed a gangliocitic paraganglioma. The patient is alive and well without evidence of relapse 6 months after surgery. Gastric paraganglioma is a very rare tumor and its diagnosis is very difficult. Surgical excision is the treatment of choice which can be performed successfully with laparoscopy.

In this study, an innovative injectable and bioresorbable composite cement (Spine-Ghost) has been developed by combining a radiopaque glass-ceramic powder (SCNZgc) and spray-dried mesoporous bioactive particles (W-SC) into type III alpha calcium sulphate hemihydrate (α-CSH) (composition α-CSH/SCNZgc/W-SC, 70/20/10 wt%). The Spine-Ghost cement and pure α-CSH (as a reference) were characterised in terms of physical and mechanical properties and compared to a commercial reference (Cerament® – Bonesupport AB, Sweden). The Spine-Ghost cement had a setting time comparable with Cerament® showing a good injectability in the range of 8–20 minutes after the end of mixing. In addition, the Spine-Ghost cement showed a good radiopacity when compared with standard PMMA (BonOs Inject, aap Biomaterials GmbH Germany) and higher compressive strength when compared to healthy cancellous bone. The bioactivity of both Spine-Ghost and Cerament® was evaluated through in vitro soaking in simulated body fluid (SBF). Spine-Ghost samples were highly bioactive, inducing the precipitation of hydroxyapatite crystals in the first week of soaking in vitro. It was also found that the degradation kinetics of the Spine-Ghost cement were faster than those of pure α-CSH and comparable to those of Cerament® after approximately 1 month of soaking in SBF. Moreover, the Spine-Ghost cement was cytocompatible in indirect-contact culture in vitro. Overall results indicate that the Spine-Ghost cement might be a very good candidate for vertebroplasty application and could enhance new bone formation in vivo.

Follow-up aims to precociously identify recurrences, metastases or treatment-related adverse events so as to undertake the appropriate therapy. Guidelines admit lack of knowledge on optimal surveillance schedule, but suggest follow-up based on experts' opinion and risk stratification. To identify the impact, if any, of regular follow-up, we interrogated our prospectively collected database whether early detection of recurrences affected both clinical management and, likely, the outcome.We required information to be available on primary surgery and ≥3°years of follow-up for non-recurring patients. We analysed recurrence characteristics (asymptomatic versus symptomatic, low- versus high tumour burden) and computed tomography (CT) scan counts to detect one recurrence. Kaplan-Meier method estimated recurrence-free survival (RFS), post-recurrence progression-free survival (PR-PFS), and disease-specific overall survival (OS). Comparisons used Hazard ratios (HR) with 95% confidence intervals (CIs). Multivariate analyses employed the Cox proportional hazards model. All tests were two-sided.Between 01/2001 and 12/2012 we found 233 study-eligible patients. Estimated 5- and 10-year RFS were 61.8% and 50.4%, respectively. After a 68-month median follow-up, we observed 94 (40.3%) recurrences [73/94 (77.7%) asymptomatic versus 21/94 (22.3%) symptomatic and 45/94 (47.9%) low- versus 49/94 (52.1%) high tumour burden]. Multivariate analysis revealed that symptomatic and high tumour burden recurrences were highly predictive of both worse PR-PFS (HR:3.19, P 0.001; HR:2.80, P = 0.003, respectively) and OS (HR:3.65, P 0.001; HR:2.38, P = 0.026, respectively). Finally, 29 second (primary) cancers were detected during follow-up.Regular follow-up detects recurrences at an earlier stage and may be associated with a better PR-PFS and OS for these patients. In the absence of randomised trials, these evidences support follow-up effort and cost.

Aim The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) potentiator ivacaftor (Kalydeco®) improves clinical outcome in G551D cystic fibrosis (CF) patients. Here, we have investigated whether ivacaftor has a clinical impact on non-G551D gating mutations and function of circulating leukocytes as well. Methods Seven patients were treated with ivacaftor and evaluated at baseline, and at 1-3 and 6 months. Besides clinical and systemic inflammatory parameters, circulating mononuclear cells (MNC) were evaluated for CFTR-dependent chloride efflux by spectrofluorimetry, neutrophils for oxidative burst by cytofluorimetry and HVCN1 mRNA expression by real time PCR. Results Ivacaftor determined a significant decrease in sweat chloride concentrations at all time points during treatment. Body mass index (BMI), FEV1, and FVC showed an increasing trend. While C-reactive protein decreased significantly at 2 months, the opposite behavior was noticed for circulating monocytes. CFTR activity in MNC was found to increase significantly at 3 and 6 months. Neutrophil oxidative burst peaked at 2 months and then decreased to baseline. HVCN1 mRNA expression was significantly higher than baseline at 1-3 months and decreased after 6 months of treatment. The chloride efflux in MNC correlated positively with both FEV1 and FVC. On the other hand, sweat chloride correlated positively with CRP and WBC, and negatively with both respiratory function tests. A cluster analysis confirmed that sweat chloride, FEV1, FVC, BMI, and MNC chloride efflux behaved as a single entity over time. Discussion In patients with non-G551D mutations, ivacaftor improved both chloride transport in sweat ducts and chloride efflux in MNC, that is, functions directly imputed to CFTR.

Angiosarcoma is a rare and highly malignant mesenchymal tumor. Similar to other soft tissue sarcomas, it may arise in any organ, although it occurs more frequently within skin structures like the scalp. Angiosarcoma has a characteristic pattern of local and distant relapse involving primary site, regional lymph nodes, and lung. Patients affected by unresectable relapses or metastases have a dismal prognosis with a median overall survival of less than 9 months. We present the case of a 74-year-old woman who previously underwent total mastectomy for a radiotherapy-induced angiosarcoma of the breast. She subsequently developed a rapidly growing gingival tumor lesion that was in fact a unique distant metastasis of her angiosarcoma. In general, surgery is the mainstay of angiosarcoma treatment, and even metastases are aggressively resected whenever feasible. We describe the successful multidisciplinary treatment that avoided a likely mutilating surgery and review the literature regarding primary and metastatic gingival angiosarcoma.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) may involve many target organs with relevant variability among affected individuals. We performed a multi-organ assessment tapping nervous system, skeletal muscle and cardiovascular system in thirty-nine individuals belonging to 16 families from Central Italy sharing the same R1006C CADASIL mutation. Stroke prevalence was larger in female patients (66.7%) than in males (23.8%); high levels of CKemia were quite frequent (21.6%) and were related to a myopathy without mitochondrial alterations; several individuals had atrial septal aneurysm (10.3%). No specific relationships between common cardiovascular risk factors and clinical manifestations were found. The present systematic study thus identified several gender-related, myopathic and cardiovascular peculiarities of R1006C mutation. This kind of comprehensive approach is necessary to define clinical course, prognosis and treatment options for a multi-organ disease such as CADASIL.

Cystic fibrosis (CF) is caused by CFTR (cystic fibrosis transmembrane conductance regulator) gene mutations. We ascertained five patients with a novel complex CFTR allele, with two mutations, H939R and H949L, inherited in cis in the same exon of CFTR gene, and one different mutation per patient inherited in trans in a wide population of 289 Caucasian CF subjects from South Italy. The genotype-phenotype relationship in patients bearing this complex allele was investigated. The two associated mutations were related to classical severe CF phenotypes.

Abnormally viscous bronchial secretions, a characteristic feature of cystic fibrosis (CF), may trap bacteria and fungi, allowing transient or chronic lung colonization. We report here a case of persistent Scedosporium apiospermum colonization in a patient with CF, who subsequently developed a lung mycetoma, and died with neurological symptoms suggestive of cerebral fungal involvement. Six isolates from consecutive sputum samples were molecularly typed by random amplification of polymorphic DNA (RAPD) using primers UBC701, UBC703, and GC70. Moreover, in vitro susceptibility of these isolates to current antifungals (amphotericin B, itraconazole, voriconazole, posaconazole, caspofungin and anidulafungin) was investigated by means of both E-test and CLSI methods. Antifungal susceptibility testing showed low minimum inhibitory concentration values only for triazole drugs. However, a unique genotype was isolated over a 12-month period, despite antifungal treatment with voriconazole for three months. This case report illustrates the therapy-refractory feature of this fungus, and provides new evidence that, as already reported, once a genotype of S. apiospermum has established colonization, it seems not to be replaced by others.

Algorithms to optimise water supply networks are developed and compared when a given set of transfer arcs is constrained to carry the same (or proportional) amounts of flow. Initially, a dynamic multi-period mathematical model is designed as a network flow model. A network simplex-based algorithm, Equalflow, and a subgradient-based algorithm, SUBeqf, are then presented. Both algorithms are characterised by polynomial complexity and can solve large-scale instances that arise when considering water system requirements. A specialised interface for water supply networks provides a data input processor to generate the overall multi-period network model in the implementation of the procedures. The implementation of the algorithms allows the consideration of different types of equal flow instances that take place in water resource system optimisation. The performances of the two algorithms and analysis of their computational efficiency with respect to state-of-the-art mathematical programming software are reported, considering equal-flow instances from a synthetically generated network and two water supply systems in the Sardinia region of Italy.

The aim of this study was to assess the effectiveness and safety of percutaneous vertebroplasty, a new technique for the treatment of vertebral pain deriving from fracture or gross osteolytic lesion due to multiple myeloma spinal involvement.Spinal osteolytic lesions are frequently associated with hematologic malignancies due to primary localization of disease (multiple myeloma and rarely lymphoma) or secondary effect of intensive corticosteroid therapy.We treated 64 patients (34 males, 30 females; mean age 71.4+/-9.6 y) with pain refractory to conventional medical therapy (analgesics, bed-rest, bracing with orthopedic devices for more than 3 wk) localized in spine, in the absence of neurologic signs.This treatment generated swift pain relief associated with an evident augmentation in vertebral resistance. Average preprocedural pain level for all patients was reported to be 8.04+/-1.4 whereas average pain level at 1 and 6 months follow-up period was 1.82+/-1.84 and 1.92+/-1.68, respectively. Although preprocedure and postprocedure demonstrated a statistically significant reduction in numeric pain scores (P0.01), the pain level at 1 and 6 months was not considered statistically significant. No procedure-related complications were observed in either leakages of polymethylmethacrylate in the epidural or foraminal area or in complications of pulmonary embolism for venous plexus involvement.Vertebroplasty is widely considered as an alternative, effective, simple, and safe technique in the treatment of neoplastic vertebral localizations consequent to hematologic malignancies. The same injection of polymethylmethacrylate can be executed before radiation therapy treatment, synergizing its delayed analgesic action to pain, after failure or in the case of local recurrences.

We genotyped three polymorphisms of the promoter region of the interleukin-10 (IL-10) gene in 220 CF patients from the CF Center of Bari, and tested for an association between genetic variants of the cytokine and chronic airway colonization with Pseudomonas aeruginosa. We found that carriers of the high-IL-10-producing-GCC haplotype had significantly higher risk of chronic pulmonary infection with the pathogen.

The aim of this study was to assess if percutaneous vertebroplasty (PVP) could relieve back pain, reduce drug consumption, and improve the mobility of patients with metastases and vertebral compression fractures. From August 2002 to July 2004, 283 patients (216 females; mean age: 73.8 +/- 9.9 years) underwent PVP on 749 vertebrae. Pain was evaluated with the pain intensity numeric rating scale (PI-NRS) (0 = no pain; 10 = worst pain) before the procedure and at the end point in September 2004 (follow-up:1-24 months; median: 7 months). A reduction of at least two points of the PI-NRS score was considered clinically relevant. Two hundred four patients were available for evaluation at the end point. Overall results showed a reduction of the median pain score from 8 at baseline to 1 at the end point (p < 0.0001); a clinically relevant pain reduction was observed in 176/205 patients (86%); 89/147 patients (61%) gave up a brace support (p < 0.0001); and 117/190 patients (62%) gave up drug therapy. Results were similar in different subgroups stratified according to age, underlying pathology, number of fractured or treated vertebrae, and length of follow-up. This study adds evidence that PVP is effective in treating painful vertebral fractures. A significant reduction in drug assumption and significant mobility improvement can also be achieved.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), the most common hereditary cerebral small vessel disease, is caused by mutations in the NOTCH3 gene on chromosome 19. Clinical manifestations of CADASIL include recurrent transient ischemic attacks, strokes, cognitive defects, epilepsy, migraine and psychiatric symptoms. Parkinsonian features have variably been reported in CADASIL patients, but only a few patients showed a clear parkinsonian syndrome. We studied two patients, a pair of monozygotic twins, carrying the R1006C mutation of the NOTCH3 gene and affected by a parkinsonian syndrome. For the first time in CADASIL patients, we used transcranial sonography (TCS) to assess basal ganglia abnormalities. TCS showed a bilateral hyperechogenic pattern of substantia nigra in one twin, and a right hyperechogenic pattern in the other. In both patients, lenticular nuclei showed a bilateral hyperechogenic pattern, and the width of the third ventricle was slightly increased. The TCS pattern found in our CADASIL patients is characteristic neither for Parkinson's disease, nor for vascular parkinsonism and seems to be specific and related to the disease-specific pathological features.

An innovative, resorbable and injectable composite cement (Spine-Ghost) to be used for augmentation and restoration of fractured vertebrae was developed. Type III α-calcium sulfate hemihydrate (CSH) was selected as the bioresorbable matrix, while spray-dried mesoporous bioactive particles (SD-MBP, composition 80/20% mol SiO2/CaO), were added to impart high bioactive properties to the cement; a glass-ceramic containing zirconia was chosen as a second dispersed phase, in order to increase the radiopacity of the material. After mixing with water, an injectable paste was obtained. The developed cement proved to be mechanically compatible with healthy cancellous bone, resorbable and bioactive by soaking in simulated body fluid (SBF), cytocompatible through in-vitro cell cultures and it could be injected in ex-vivo sheep vertebra. Comparisons with a commercial control were carried out.

This paper proposes a mathematical programming approach for empty container management. Since directional imbalances in trade activities result in a surplus or shortage of empty containers in ports and depots, their management can be thought of as a min cost flow problem whose arcs represent services routes, inventory links and decisions concerning the time and place to lease containers from external sources. We adopt an hourly time-step in a dynamic network and, although this time-period generates large-size instances, the two implemented algorithms show a good computational efficiency. A possible case study of the Mediterranean basin is proposed and results are presented with a graphical representation, providing a useful support to decision-makers in the field. Maritime Economics & Logistics (2005) 7, 199–222. doi:10.1057/palgrave.mel.9100136

Background and Purpose— To describe parkinsonism as a clinical manifestation of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. Methods— We report 5 patients carrying the R1006C mutation in the exon 19 of NOTCH3 gene. All cases presented late onset, slowly progressive parkinsonism, not responsive to l-dopa. We performed brain MRI and 123 I-FP-CIT SPECT in all and in 3 additional patients carrying the same mutation but without parkinsonism. Four patients with parkinsonism underwent myocardial 123 I-meta-iodobenzylguanidine scintigraphy. Results— In all patients, brain MRI showed widespread ischemic lesions in the periventricular white matter, the internal and external capsules, the basal ganglia, and thalami. 123 I-FP-CIT SPECT showed symmetrical or asymmetrical reduction of tracer uptake in the putamen, with inconstant caudate involvement. Myocardial 123 I-meta-iodobenzylguanidine scintigraphy resulted normal. Nigrostriatal denervation was also demonstrated in 2 patients without parkinsonism. Conclusions— In cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, parkinsonism may be a not rare, late onset manifestation. The clinical picture, the lack of response to dopaminergic treatment, and MRI findings suggest a vascular parkinsonism, which may be preceded by a protracted presymptomatic phase.

We report the case of a patient with an apparent homozygosity for the D1152H mutation located in exon 18 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The parents had no personal history of cystic fibrosis (CF) and referred to our laboratory after the diagnosis of fetal bowel hyperechogenicity. The proband presented with meconium ileus and normal sweat chloride test. Sequencing of the CFTR exon 18 together with quantitative genomic assays, such as real-time PCR and the multiplex ligation probe amplification (MLPA) techniques, were performed and revealed that the father was heterozygous for the D1152H mutation and the mother carried a large deletion of the CFTR gene encompassing the genomic sequence including the same mutation. The child inherited D1152H from his father and the large deletion of the CFTR gene from his mother. We suggest that D1152H likely acts as a mild mutation with a dominant effect on the severe deletion of exon 18, considering that after 3 years of clinical examinations the child shows no classical signs and symptoms of CF. Not testing for large deletions in subjects with apparent homozygosity for a mutated CFTR allele could lead to the misidentification of CFTR mutation carrier status.

We describe the case of a 44-year-old female cystic fibrosis (CF) patient (R334W/852del22) who presented symptoms of prolonged acute respiratory infections and recurrent episodes of pneumonia. Computed tomography (CT) scan images of the chest showed that the patient presented airway and parenchymal changes throughout both lungs. She also had decreased lung function performances. In March 2004, she underwent live-related donor renal transplant and started an immunosuppressive therapy with cyclosporine. CT scan images taken respectively 2 and 6 years after transplantation documented a progressive significant size reduction of structural lung damages in both lungs and clinical signs and symptoms of improvements.

In seeking more specific biomarkers of the cystic fibrosis (CF) lung inflammatory disease that would be sensitive to antibiotic therapy, we sought to evaluate the gene expression profiles of neutrophils in CF patients before treatment in comparison with non-CF healthy individuals and after antibiotic treatment. Genes involved in neutrophil-mediated inflammation, i.e. chemotaxis, respiratory burst, apoptosis, and granule exocytosis, were the targets of this study. Microarray analysis was carried out in blood and airway neutrophils from CF patients and in control subjects. A fold change (log) threshold of 1.4 and a cut-off of p

Background Vanishing bile duct syndrome has been associated with different pathologic conditions (adverse drug reactions, autoimmune diseases, graft versus host disease, and cancer). Though its causes are unknown, an immune-related pathogenesis is the most likely one. Vanishing bile duct syndrome can evolve to hepatic failure and, eventually, to death. The treatment is uncertain, but it needs the resolution of the underlying pathologic condition. Case presentation We describe the association of Hodgkin’s lymphoma with a syndrome characterized by cholestasis, aminotransferase elevation and an histological picture of bile duct loss. All other causes of hepatic function impairment were excluded (in particular, drugs, viral and autoimmune related diseases) eventually leading to the diagnosis of vanishing bile duct syndrome. Despite the fact that the dysfunction is not caused by hepatic Hodgkin’s lymphoma involvement, liver impairment can limit the optimal therapy of Hodgkin’s lymphoma. A treatment consisting of ursodeoxycholic acid, prednisone, and full dose chemotherapy restored hepatic function and achieved complete and long-lasting remission of Hodgkin’s lymphoma. Conclusion We reviewed all case reports showing that vanishing bile duct syndrome is a dismal paraneoplastic syndrome being fatal in a high proportion of patients if not adequately treated. Indeed, this syndrome requires both an early recognition and an appropriate aggressive treatment consisting of full dose upfront chemotherapy which is the only way to achieve a resolution of the vanishing bile duct syndrome. Delayed or reduced intensity treatments unfavorably correlate with survival.

cited By 13; International audience; A radiopaque mesoporous bioactive glass (named MBGZ-7) was obtained through a combined sol-gel and evaporation induced self-assembling (EISA) route, adding zirconium propoxide to the synthesis batch as the zirconia precursor. The nitrogen sorption analysis confirmed the mesoporous nature of the glass. The assessment of in vitro bioactivity by soaking in acellular simulated body fluid (SBF) and SEM observation showed the deposition of hydroxyapatite crystals on its surface after one week. The good radiopacity level was demonstrated by comparing X-ray images of MBGZ-7 and a blank sample that did not contain radiopaque additives. It is envisaged the use of MBGZ-7 as a promising dispersed phase in composite materials for minimally invasive surgery procedures, such as injectable bone cements, in order to allow the visualization of the implant under fluoroscopic control, during both injection and follow-up. © 2014 Elsevier B.V.