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Cesena, Italy

Piazza O.,University of Naples Federico II | Venditto A.,Bufalini Hospital | Tufano R.,University of Naples Federico II
Panminerva Medica | Year: 2011

Aneurysmal subarachnoid hemorrhage (SAH), in addition to the direct effects of the initial hemorrhage and secondary neurological complications, predisposes to medical complications. The proportion of deaths caused by non-neurological medical complications (cardiac, pulmonary, gastrointestinal, renal, hematological) equals that from neurological complications. In particular, pulmonary complications are responsible for 50% of all deaths from medical complications. Neurogenic pulmonary edema (NPE) is an increase of interstitial and alveolar fluid occurring as direct consequence of any acute central nervous system injury. Two different pathogenetic mechanisms of NPE have been hypothesized: i) hemodynamic (an increase of pulmonary vascular pressure due to an oc-adrenergic response produces hydrostatic edema) and ii) inflammatory mechanism (brain cytokines and chemokines determinates an increase in the permeability of pulmonary capillaries causing exudative edema). Recent studies postulate that both mechanisms may be implicated in the pathogenesis of NPE. Brain injury is known to determine increased levels of S100B, a Ca- binding protein, in cerebrospinal fluid and in blood. Moreover, amine precursor uptake and decarboxylation (APUD) cells located in the respiratory tract produce and release S100B. This protein may contribute to the pathogenesis of NPE binding RAGE receptors in alveolar epithelial type I pneumocytes and amplifying the immune and inflammatory response causing lung injury. S100B can be the link between the brain and the lung and may be among the multiple pathological pathways that determine the development of pulmonary edema after bleeding.

Castaman G.,Hemophilia and Thrombosis Center | Giacomelli S.H.,Hemophilia and Thrombosis Center | Caccia S.,University of Milan | Riccardi F.,University of Parma | And 5 more authors.
Haemophilia | Year: 2013

Factor XI (FXI) deficiency is a rare inherited bleeding disorder invariably caused by mutations in the FXI gene. The disorder is rather frequent in Ashkenazi Jews, in whom around 98% of the abnormal alleles is represented by Glu117X and Phe283Leu mutations. A wide heterogeneity of causative mutations has been previously reported in a few FXI deficient patients from Italy. In this article, we enlarge the knowledge on the genetic background of FXI deficiency in Italy. Over 4 years, 22 index cases, eight with severe deficiency and 14 with partial deficiency, have been evaluated. A total of 21 different mutations in 30 disease-associated alleles were identified, 10 of which were novel. Among them, a novel Asp556Gly dysfunctional mutation was also identified. Glu117X was also detected, as previously reported from other patients in Italy, while again Phe283Leu was not identified. A total of 34 heterozygous relatives were also identified. Bleeding tendency was present in very few cases, being inconsistently related to the severity of FXI deficiency in plasma. In conclusion, at variance with other populations, no single major founder effect is present in Italian patients with FXI deficiency. © 2013 John Wiley & Sons Ltd.

Ricci-Maccarini A.,Bufalini Hospital | De Maio V.,Santobono Hospital | Murry T.,New York Medical College | Schindler A.,University of Milan
Journal of Voice | Year: 2013

Objectives/Hypothesis: To develop and validate the self-administered Voice Handicap Index-10 for children (CVHI-10) in Italian and evaluate its internal consistency and reliability in normal and disordered children's voices. Study Design: Cross-sectional survey study. Methods: CVHI-10 was developed after a series of individual interviews with 20 children, aged 8-14 years to discuss the phrasing and wording of the original VHI-10. Subsequently, 66 dysphonic children (group 1) provided input to test internal consistency, external validity, and clinical validity. The voices of group 1 children were rated using the Grade, Roughness, and Breathiness parameters of the Grade, Roughness, Breathiness, Asthenia, Strain (GRBAS) scale. The test-retest results of 30 children (group 2) who successfully underwent voice treatment were also analyzed for test-retest reliability and responsiveness to treatment. Children of group 2 completed CVHI-10 twice, with an interval of 2 weeks. Additionally, 40 children without voice disorders (group 3) were included as a control group to obtain clinical validity. Each child included in the study completed CVHI-10 autonomously. Results: Internal consistency measured with the Cronbach α coefficient was.85; test-retest reliability was 0.84. CVHI-10 positively correlated with G (r = 0.62) and B (r = 0.34) parameters of the GRBAS scale on Spearman rho test. The mean CVHI-10 score for group 2 was 12.4 ± 2.8 before treatment and 3.6 ± 1.6 after treatment; the difference was significant using the Wilcoxon sign test (P = 0.0001). The difference between CVHI-10 scores in groups 1 and 3 was significant using the Mann-Whitney U test (P = 0.0001). Conclusions: CVHI-10 is easily administered, highly reproducible, exhibits good clinical validity, and responsiveness to treatment. © 2013 The Voice Foundation.

Castaman G.,San Bortolo Hospital | Coppola A.,University of Naples Federico II | Zanon E.,University of Padua | Boeri E.,Gaslini Hospital | And 16 more authors.
Haemophilia | Year: 2013

Von Willebrand disease (VWD) is an inherited bleeding disorder caused by the quantitative or qualitative deficiency of von Willebrand factor (VWF). Replacement therapy with plasma-derived VWF/factor VIII (FVIII) concentrates is required in patients unresponsive to desmopressin. To assess the efficacy, safety and ease of use of a new, volume-reduced (VR) formulation of VWF/FVIII concentrate Haemate® P in patients requiring treatment for bleeding or prophylaxis for recurrent bleeding or for invasive procedures. Pharmacoeconomic variables were also recorded. Data were analysed using descriptive statistics. This was a multicentre, prospective, observational study. Consecutively enrolled patients received Haemate® P VR according to their needs, and were followed for 24 months. Of the 121 patients enrolled, 25.6% had type 3 VWD and more than 40% had severe disease. All patients were followed for 2 years, for a total of 521 visits. On-demand treatment was given to 61.9% of patients, secondary long-term prophylaxis to 25.6% and prophylaxis for surgery, dental or invasive procedures to 45.5%. The response to treatment was rated as good to excellent in >93-99% of interventions. The new formulation was well tolerated by all patients with no report of drug-related adverse events. The switch to volume-reduced Haemate® P was easy to perform and infusion duration was decreased twofold compared with the previous formulation. Volume-reduced Haemate® P was at least as effective and well-tolerated as the previous formulation. © 2012 Blackwell Publishing Ltd.

Ricci-Maccarini A.,Bufalini Hospital | De Maio V.,Santo Bono Hospital | Murry T.,Loma Linda University | Murry T.,Cornell College | Schindler A.,University of Milan
Journal of Voice | Year: 2016

Objectives/Hypothesis The Children's Voice Handicap Index-10 (CVHI-10) was introduced as a tool for self-assessment of children's dysphonia. However, in the management of children with voice disorders, both parents' and children's perspectives play an important role. Because a self-tool including both a children's and a parents' version does not exist yet, the aim of the study was to develop and validate an assessment tool which parallels the CVHI-10 for parents to assess the level of voice handicap in their child's voice. Study Design Observational, prospective, cross-sectional study. Methods To develop a CVHI-10 for parents, called "CVHI-10-P", the CVHI-10 items were adapted to reflect parents' responses about their child. Fifty-five children aged 7-12 years completed the CVHI-10, whereas their parents completed the CVHI-10-P. Each child's voice was also perceptually assessed by an otolaryngologist using the Grade Breathness Roughness (GRB) scale. Fifty-one of the 55 children underwent voice therapy (VT) and were assessed afterward using the GRB, CVHI-10, and CVHI-10-P. Results CVHI-10-P internal consistency was satisfactory (Cronbach alpha =.78). Correlation between CVHI-10-P and CVHI-10 was moderate (r = 0.37). CVHI-10-P total scores were lower than CVHI-10 scores in most of the cases. Single-item mean scores were always lower in CVHI-10-P compared with CVHI-10, with the exception of the only one item of the CVHI-10-P that directly involves the parent's experience (item 10). Data gained from one tool are not directly related to the other, suggesting that these two tools appraise the child's voice handicap from different perspectives. The overall perceptual assessment scores of the 51 children after VT significantly improved. There was a statistically significant reduction of the total scores and for each item in CVHI-10 and CVHI-10-P after VT. These data support the adoption of the CVHI-10-P as an assessment tool and an outcome measure for management of children's voice disorders. Conclusions CVHI-10-P is a valid tool to appraise parents' perspective of their child's voice disorder. The use of the CVHI-10 and the CVHI-10-P is recommended for objectively determining the level of voice handicap in children by parents and child. © 2016 The Voice Foundation.

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