INVESTIGATING PREFERENCES FOR ENVIRONMENT FRIENDLY PRODUCTION

This paper reports some preliminary results on a mixed logit random utility analysis of conjoint data from costumers' preferences over agricultural products. The data are collected via a telematic sample representative of Italian households. The survey instrument was implemented via a computer supported system. A multivariate normal full correlation structure is imposed in the mixed logit estimation and the implications of such a taste structure are examined.

Epilepsy in mucopolysaccharidosis disorders

Abstract The mucopolysaccharidosis (MPS) disorders are caused by deficiencies of specific lysosomal enzymes involved in the catabolism of glycosaminoglycans (GAGs). The resulting GAG accumulation in cells and tissues throughout the body leads to progressive multi-organ dysfunction. MPS patients present with several somatic manifestations, including short stature, musculoskeletal abnormalities, and cardiorespiratory dysfunction, and several primary and secondary neurological signs and symptoms. Epileptic seizures are neurological signs of MPS thought to develop due to accumulation of GAGs in the brain, triggering alterations in neuronal connectivity and signaling, and release of inflammatory mediators. The amount of literature on the prevalence, pathophysiology, clinical features, and management of epileptic seizures in patients with MPS is limited. This review discusses current knowledge on this topic, as well as two case examples, presented and discussed during a closed meeting on MPS and the brain among an international group of experts with extensive experience in managing and treating MPS

Recommendations for the management of MPS IVA : systematic evidence- and consensus-based guidance

Introduction: Mucopolysaccharidosis (MPS) IVA or Morquio A syndrome is an autosomal recessive lysosomal storage disorder (LSD) caused by deficiency of the N-acetylgalactosamine-6-sulfatase (GALNS) enzyme, which impairs lysosomal degradation of keratan sulphate and chondroitin-6-sulphate. The multiple clinical manifestations of MPS IVA present numerous challenges for management and necessitate the need for individualised treatment. Although treatment guidelines are available, the methodology used to develop this guidance has come under increased scrutiny. This programme was conducted to provide evidence-based, expert-agreed recommendations to optimise management of MPS IVA. Methods: Twenty six international healthcare professionals across multiple disciplines, with expertise in managing MPS IVA, and three patient advocates formed the Steering Committee (SC) and contributed to the development of this guidance. Representatives from six Patient Advocacy Groups (PAGs) were interviewed to gain insights on patient perspectives. A modified-Delphi methodology was used to demonstrate consensus among a wider group of healthcare professionals with experience managing patients with MPS IVA and the manuscript was evaluated against the validated Appraisal of Guidelines for Research and Evaluation (AGREE II) instrument by three independent reviewers. Results: A total of 87 guidance statements were developed covering five domains: (1) general management principles; (2) recommended routine monitoring and assessments; (3) disease-modifying interventions (enzyme replacement therapy [ERT] and haematopoietic stem cell transplantation [HSCT]); (4) interventions to support respiratory and sleep disorders; (5) anaesthetics and surgical interventions (including spinal, limb, ophthalmic, cardio-thoracic and ear-nosethroat [ENT] surgeries). Consensus was reached on all statements after two rounds of voting. The overall guideline AGREE II assessment score obtained for the development of the guidance was 5.3/7 (where 1 represents the lowest quality and 7 represents the highest quality of guidance). Conclusion: This manuscript provides evidence- and consensus-based recommendations for the management of patients with MPS IVA and is for use by healthcare professionals that manage the holistic care of patients with the intention to improve clinical- and patient-reported outcomes and enhance patient quality of life. It is recognised that the guidance provided represents a point in time and further research is required to address current knowledge and evidence gap

Recommendations for the management of MPS VI : systematic evidence- and consensus-based guidance

Introduction: Mucopolysaccharidosis (MPS) VI or Maroteaux-Lamy syndrome (253200) is an autosomal recessive lysosomal storage disorder caused by deficiency in N-acetylgalactosamine-4-sulfatase (arylsulfatase B). The heterogeneity and progressive nature of MPS VI necessitates a multidisciplinary team approach and there is a need for robust guidance to achieve optimal management. This programme was convened to develop evidence-based, expert-agreed recommendations for the general principles of management, routine monitoring requirements and the use of medical and surgical interventions in patients with MPS VI. Methods: 26 international healthcare professionals from various disciplines, all with expertise in managing MPS VI, and three patient advocates formed the Steering Committee group (SC) and contributed to the development of this guidance. Members from six Patient Advocacy Groups (PAGs) acted as advisors and attended interviews to ensure representation of the patient perspective. A modified-Delphi methodology was used to demonstrate consensus among a wider group of healthcare professionals with expertise and experience managing patients with MPS VI and the manuscript has been evaluated against the validated Appraisal of Guidelines for Research and Evaluation (AGREE II) instrument by three independent reviewers. Results: A total of 93 guidance statements were developed covering five domains: (1) general management principles; (2) recommended routine monitoring and assessments; (3) enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT); (4) interventions to support respiratory and sleep disorders; (5) anaesthetics and surgical interventions. Consensus was reached on all statements after two rounds of voting. The greatest challenges faced by patients as relayed by consultation with PAGs were deficits in endurance, dexterity, hearing, vision and respiratory function. The overall guideline AGREE II assessment score obtained for the development of the guidance was 5.3/7 (where 1 represents the lowest quality and 7 represents the highest quality of guidance). Conclusion: This manuscript provides evidence- and consensus-based recommendations for the management of patients with MPS VI and is for use by healthcare professionals that manage the holistic care of patients with the intention to improve clinical- and patient-reported outcomes and enhance patient quality of life. It is recognised that the guidance provided represents a point in time and further research is required to address current knowledge and evidence gaps

Intrathecal delivery of protein therapeutics to the brain: A critical reassessment

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In vitro and in vivo gene transfer to pulmonary cells mediated by cationic liposomes

Cationic liposomes have been proposed as alternative to adenovirus in the treatment of cystic fibrosis lung disease. Therefore, we have investigated the efficiency of two lipid mixtures in mediating gene transfer in in vitro and in vivo models. The cationic lipid DOTMA (N-(1-(2,3(dioleyloxy)propyl)-n,n,n-trimethylammoniumchloride) and DOGS (dioctadecylamidoglycylspermine) were used in combination with the neutral lipid DOPE (dioleoylphosphatidylethanolamine). The relative transfection efficiencies of the two cationic liposomes were tested using the bacterial β-galactosidase (lacZ) and the firefly luciferase genes. Gene expression was detected in both cell lines and primary culture of rhesus monkey airway epithelium after transfection with plasmid DNA complexed with DOGS/DOPE or DOTMA/DOPE. Transfection efficiency of both types of lipids was higher in the mouse fibroblast 3T3 cell line as compared to human carcinoma A549 cells and primary epithelial cultures. Administration of DNA-liposome complexes via intratracheal instillation resulted in expression of the lacZ and luciferase marker gene in the mouse airways. In vivo transfection mediated by both types of liposomes were proven to be far less efficient than adenovirus treatment

rehabilitation protocol for unilateral laryngeal and lingual paralysis tapia syndrome comment about a challenging case of tapia syndrome after total thyroidectomy by ildem deveci mehmet surmeli and reyhan surmeli

Tapia syndrome is a rare complication after surgery, with ipsilateral paralysis of vocal cord and tongue due to extracranial involvement of recurrent laryngeal and hypoglossal nerves. Tapia's case report is extremely interesting for both the rarity of the reported cases and for the importance of an early rehabilitation. In a previous work, we reported a case of Tapia syndrome after cardiac surgery for aortic aneurysm, and the protocol of logopedic rehabilitation adopted. In the postoperative period, he developed severe dyspnea and dysphagia that required a tracheostomy and a logopedic rehabilitation therapy that led to a fast and efficient swallowing without aspiration after 47 sessions (less than 4 months). The progressive recovery of the function suggests aprassic nerve damage. However, the logopedic therapy is recommended to limit the possibility of permanent functional deficits and quickly recover swallowing and phonation

An Energy-aware Brokering Algorithm to Improve Sustainability in Community Cloud

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Accuracy of magnetic resonance imaging to identify pseudocapsule invasion in renal tumors

Purpose: To evaluate accuracy of MRI in detecting renal tumor pseudocapsule (PC) invasion and to propose a classification based on imaging of PC status in patients with renal cell carcinoma. Methods: From January 2017 to June 2018, 58 consecutive patients with localized renal cell carcinoma were prospectively enrolled. MRI was performed preoperatively and PC was classified, according to its features, as follows: MRI-Cap 0 (absence of PC), MRI-Cap 1 (presence of a clearly identifiable PC), MRI-Cap 2 (focally interrupted PC), and MRI-Cap 3 (clearly interrupted and infiltrated PC). A 3D image reconstruction showing MRI-Cap score was provided to both surgeon and pathologist to obtain complete preoperative evaluation and to compare imaging and pathology reports. All patients underwent laparoscopic partial nephrectomy. In surgical specimens, PC was classified according to the renal tumor capsule invasion scoring system (i-Cap). Results: A concordance between MRI-Cap and i-Cap was found in 50/58 (86%) cases. ρ coefficient for each MRI-cap and iCap categories was: MRI-Cap 0: 0.89 (p < 0.0001), MRI-Cap1: 0.75 (p < 0.0001), MRI-Cap 2: 0.76 (p < 0.0001), and MRI-Cap3: 0.87 (p < 0.0001). Sensitivity, specificity, positive predictive value, negative predictive value, and AUC were: MRI-Cap 0: Se 97.87% Spec 83.3%, PPV 95.8%, NPV 90.9%, and AUC 90.9; MRI-Cap 1: Se 77% Spec 95.5%, PPV 83.3%, NPV 93.5%, and AUC 0.86; MRI-Cap 2- iCap 2: Se 88% Spec 90%, PPV 79%, NPV 95%, and AUC 0.89; MRI-Cap 3: Se 94% Spec 95%, PPV 88%, NPV 97%, and AUC 0.94. Conclusions: MRI-Cap classification is accurate in evaluating renal tumor PC features. PC features can provide an imaging-guided landmark to figure out where a minimal margin could be preferable during nephron-sparing surgery