Sleep in genetically confirmed pantothenate kinase-associated neurodegeneration: a video-polysomnographic study.

Pantothenate kinase-associated neurodegeneration (PKAN) is a familial or sporadic disease characterized by extrapyramidal and corticospinal signs with dementia. Patients show iron accumulation in the basal ganglia, with neuronal loss and gliosis. A mutation of pantothenate kinase (PANK2) gene localized on chromosome 20p13 has been described in familiar forms, as well as in sporadic patients.

Immunological parameters related to the adjuvant effect of the ordered mesoporous silica SBA-15.

In 2006, the first report of a nanostructured material as adjuvant was described establishing the effectiveness of the ordered mesoporous SBA-15 silica as an immune adjuvant. The present study evaluated the SBA-15 capacity to modulate the immune responsiveness of High and Low responder mice immunized with BSA encapsulated/adsorbed in SBA-15 by the intramuscular or oral route and the adjuvant effect was compared with the responsiveness induced by BSA in aluminum hydroxide salts or emulsified in Incomplete Freund adjuvant. These results demonstrate the ability of the non-toxic SBA-15 nanoparticles to increase the immunogenicity and repair the responsiveness of the constitutively low responder individuals inducing both the IgG2a and the IgG1 isotypes, independently of the immune cell committed and conditioning the low phenotype.

Toxic encephalopathy in elderly patients during treatment with capecitabine: literature review and a case report.

Capecitabine is now-a-days rapidly replacing 5-Fluorouracil in daily clinical practice. Neurologic toxicity during a treatment with fluoropyrimidines, as 5-fluorouracil, represents a well-known side-effect, largely described in literature. Central nervous system (CNS) toxicity, mainly encephalopathy with or without seizures, occurs occasionally even when conventional doses are used.

Polysomnographic diagnosis of idiopathic REM sleep behavior disorder.

The presence of either excessive tonic chin EMG activity during REM sleep, or excessive phasic submental or limb EMG twitching is required to diagnose REM sleep behavior disorder (RBD). The aim was to identify cut-off values and to assess the sensitivity and specificity of these values taken separately or combined to diagnose idiopathic RBD patients. Eighty patients presenting with a clinical diagnosis of idiopathic RBD and 80 age- and gender-matched normal controls were studied in the sleep laboratory.

Rehabilitation of the nose using CAD/CAM and rapid prototyping technology after ablative surgery of squamous cell carcinoma: a pilot clinical report.

Restoration of a nasal defect after ablative surgery for squamous cell carcinoma necessitates replacing the missing volume and anchoring a prosthesis to the patient's face. This report describes the failure of plastic reconstructive surgery after ablation of a squamous cell cancer of the nose and the esthetic and functional restoration of the patient with a nasal prosthesis. The process of making an implant-supported prosthesis using digital technology, including digitized anatomic models from a "nose library," and the rapid prototyping of the mesiostructure for bar anchorage and of the mold for silicone processing are presented.

Small cell neuroendocrine tumor of the breast in a 40 year-old woman: a case report.

Introduction: Small cell neuroendocrine cancer of the breast is a rare tumor with less than 30 cases reported in the literature. The morphological and immunohistochemical patterns of this tumor are similar to small cell neuroendocrine cancer of the lung. For this reason, it is often difficult to distinguish a primary small cell neuroendocrine cancer of the breast from a metastatic lesion from other sites.

NMR analysis of a series of imidazobenzoxazines.

The complete (1)H and (13)C NMR assignment of a series of imidazobenzoxazines by a combination of one- and two-dimensional experiments (COSY, HSQC and HMBC) is studied. Moreover, 2D NOESY and 1D selective NOESY are reported. This procedure allows the identification of the regioisomers obtained.

Crystallite size-dependent phases in nanocrystalline ZrO(2)-Sc(2)O(3).

ZrO(2)-10, 12 and 14 mol% Sc(2)O(3) nanopowders were prepared by using a nitrate-lysine gel-combustion synthesis. These materials were studied by synchrotron X-ray powder diffraction (SXPD) and Raman spectroscopy after calcination at different temperatures from 650 to 1200 degrees C, which led to samples with different average crystallite sizes, up to about 100 nm. The results from SXPD and Raman analyses indicate that, depending on Sc(2)O(3) content, the metastable t''-form of the tetragonal phase or the cubic phase are fully retained at room temperature in nanocrystalline powders, provided an average crystallite sizes lower than approximately 30 nm.

Sodium channel blockers for neuropathic pain.

Importance Of The Field: The voltage-gated sodium channels (VGSCs) play a fundamental role in controlling cellular excitability and their abnormal activity is related to several pathological processes, including cardiac arrhythmias, epilepsy, neurodegenerative diseases, spasticity, chronic and neuropathic pain. In particular, neuropathic pain (e.g.

Radiation protection measurements around a 12 MeV mobile dedicated IORT accelerator.

Purpose: The aim of this study is to investigate radioprotection issues that must be addressed when dedicated accelerators for intraoperative radiotherapy (IORT) are used in operating rooms. Recently, a new version of a mobile IORT accelerator (LIAC Sordina SpA, Italy) with 12 MeV electron beam has been implemented. This energy is necessary in some specific pathology treatments to allow a better coverage of thick lesions.

Hip fracture: effectiveness of early surgery to prevent 30-day mortality.

To estimate the effect of delay to surgery for hip fracture on 30-day mortality using a risk adjustment strategy to control for the effect of demographic and clinical confounders. This observational study was carried out on all patients admitted with a hip fracture and discharged between January 2004 and December 2007 from a teaching hospital. Gender, age, time to surgery, mortality and medical comorbidities were derived from hospital discharge records (SDO), while International Normalised Ratio (INR) and American Society of Anaesthesiologists (ASA) score were retrieved from clinical records.

The Morfeo Study: A 1-year follow-up of complications of vegetative state in a dedicated facility.

Objectives: Vegetative State (VS) implies significant issues. The aim of the MORFEO study is to identify the most relevant complications in VS patients and to supply clinicians and policy-makers with data derived from the analysis of a cohort of patients treated in a dedicated long-term facility setting.

Methods: A cohort of 22 VS patients treated between 2003 and 2007 were enrolled and followed up for 1 year.

Interferon-gamma-expressing cells are a major source of interleukin-21 in inflammatory bowel diseases.

Background: We previously demonstrated that in inflammatory bowel disease (IBD) there is enhanced production of interleukin (IL)-21, a cytokine that activates multiple pathways that sustain mucosal inflammation. However, the phenotype of IL-21-producing cells in IBD, and the cytokine(s) they coproduce, is not known. We here characterized the cell source of IL-21 and determined which factors regulate IL-21 in the human gut.

Peripartum hysterectomy and cesarean delivery: a population-based study.

Objective: To estimate the incidence of peripartum hysterectomy in an Italian Region (Emilia-Romagna) and investigate its association with cesarean delivery.

Design: Population-based retrospective study using hospital discharge records.

Setting: All public and private hospitals in Emilia-Romagna region, Italy.

Microwave assisted efficient synthesis of imidazole-based privileged structures.

A simple and efficient microwave assisted synthesis of imidazobenzoxazines, imidazobenzoxazin-5-ones, and imidazobenzoxazin-5-thiones with broad chemistry scope is described. The molecules were prepared both under conventional as well as microwave heating conditions, to provide in high yields with clean and scalable reactions a small library of imidazole-based privileged structures for drug discovery.

The lymphatic system controls intestinal inflammation and inflammation-associated Colon Cancer through the chemokine decoy receptor D6.

Background And Aims: Inflammatory CC chemokines have long been associated with cancer, but unequivocal evidence of a role in clinically relevant models of carcinogenesis is lacking. D6, a promiscuous decoy receptor that scavenges inflammatory CC chemokines, plays a non-redundant role in reducing the inflammatory response in various organs. As inflammation is a key player in the development of inflammatory bowel disease (IBD) and IBD-associated colorectal cancer, we investigated D6 expression in human colitis and colon cancer, and its role in experimental colitis and inflammation-associated colon cancer.

CAD/CAM bilateral ear prostheses construction for Treacher Collins syndrome patients using laser scanning and rapid prototyping.

Ear defects in patients affected by Treacher Collins syndrome necessitate the replacement of the existing anatomic residuals of the ears with custom-made prostheses. This paper describes a multidisciplinary protocol involving both medicine and computer-aided design/computer-aided manufacturing for manufacturing ear prostheses. Using innovative prototyping technologies together with conventional silicone processing procedures, a step-by-step procedure is presented.

Inhibition of colon carcinogenesis by 2-methoxy-5-amino-N-hydroxybenzamide, a novel derivative of mesalamine.

Background & Aims: Mesalamine has been reported to protect against inflammatory bowel disease-related colorectal cancer (CRC), but several drug-related issues have limited its use in chemopreventive programs. We evaluated the antineoplastic properties of mesalamine derivatives using in vitro and in vivo models of CRC.

Methods: CRC cell proliferation and cell-cycle progression were evaluated by flow cytometry after exposure to mesalamine or mesalamine derivatives.

Immediate facial rehabilitation in cancer patients using CAD-CAM and rapid prototyping technology: a pilot study.

Purpose: This study describes the workflow in a procedure to create a provisional facial prosthesis for cancer patients using digital and rapid prototyping technologies without the need for supporting craniofacial implants.

Materials And Methods: An integrated workflow procedure aimed at the construction of provisional silicone prosthesis was used to rehabilitate a facial disfigurement in a patient who had undergone ablative surgery of the midface. A laser scan of the defect was obtained, and a digital model of the patient's face was constructed using virtual mirroring of the healthy side and referencing the "Nose Digital Library.

Interleukin-25 inhibits interleukin-12 production and Th1 cell-driven inflammation in the gut.

Background & Aims: During the pathogenesis of Crohn's disease (CD), interleukin (IL)-12, a cytokine produced by mucosal CD14+ monocyte-like cells, promotes tissue-damaging T helper cell (Th) 1-mediated inflammation through mechanisms that are not fully understood. IL-25 promotes Th2 cell responses by activating major histocompatibility complex class II-positive non-T and non-B cells. Because Th1 and Th2 cells, and the cytokines they release, are often mutually antagonistic, we examined whether IL-25 affects IL-12 production or Th1 cell-mediated inflammation in the gut.

Common immunologic mechanisms in inflammatory bowel disease and spondylarthropathies.

Spondyloarthropathies (SpA) are commonly observed extra-intestinal manifestations of both Crohn's disease (CD) and ulcerative colitis (UC), the two major forms of inflammatory bowel diseases (IBD). However, the immunological link between these two clinical entities is still poorly understood. Several lines of evidence indicate that SpA may originate from the relocation to the joints of the immune process primarily induced in the gut.

Recent advances in the medicinal chemistry of sodium channel blockers and their therapeutic potential.

The voltage-gated sodium channels (VGSCs) play a fundamental role in controlling cellular excitability. Abnormal activity of sodium channels is related to several pathological processes, including cardiac arrhythmias, epilepsy, chronic pain, neurodegenerative diseases and spasticity. In view of this, VGSCs are considered important therapeutic targets for the treatment of these disorders.

2,4(5)-diarylimidazoles as inhibitors of hNaV1.2 sodium channels: pharmacological evaluation and structure-property relationships.

Sodium (Na) channels continue to represent an important target for the development of novel anticonvulsants. We have synthesized and evaluated a series of 2,4(5)-diarylimidazoles for inhibition of the human neuronal Na(V)1.2 Na channel isoform.