Basilar Artery Changes in Fabry Disease

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Nurses' perceptions of aids and obstacles to the provision of optimal end of life care in ICU

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Morphometric correlates of dysarthric deficit in amyotrophic lateral sclerosis.

Our objective was to investigate the volumetric correlates of speech in amyotrophic lateral sclerosis (ALS). Twenty-three ALS patients had a structural 3D MRI scan, neuropsychological, linguistic and speech assessments. Twenty-three healthy adults of comparable age, education, white-matter hyperintensity load and intracranial volumes were also recruited. Between-group differences in grey matter and white matter (WM) were examined to characterize ALS patients accurately. The association between residual speech and volumetric maps was studied in these patients. Results demonstrated that ALS patients showed a pattern of WM reduction, which was located in peri-cortical motor/premotor fibres bilaterally, and in a large volume extending from the pons/midbrain to the cerebellum. A speech composite score was computed, and this was positively associated with premotor/supplementary-motor WM bilaterally, and right cerebellar WM. Since premotor associations were found in volumes where ALS patients showed WM reduction, this region is believed to be directly involved in speech execution in this group. Since cerebellar associations were instead found in volumes free from shrinkage, this region is interpreted as playing a modulatory role, compensating for the impact of ALS pathology

Systematic review of surgical treatment of subclinical Cushing's syndrome

none6BackgroundSubclinical Cushing's syndrome (SCS) is a condition of biochemical cortisol excess without the classical clinical features of overt hypercortisolism; it may be associated with some consequences of metabolic syndrome. The most appropriate treatment remains controversial. This study aimed to assess the outcomes of adrenalectomy for SCS. MethodsA systematic review was performed. MEDLINE, Embase and Cochrane Databases (1980-2013) were searched for studies reporting the outcomes of unilateral adrenalectomy with respect to hypertension, diabetes, dyslipidaemia, obesity and osteoporosis in patients with SCS. Studies with a questionable diagnosis of SCS, bilateral adrenal involvement and insufficient data were excluded. ResultsOf the 105 papers screened, seven were selected; there were six retrospective studies and one randomized clinical trial, including 230 patients. Data analysis was limited by heterogeneity in definition of SCS and endpoints. Hypercortisolism was cured in all operated patients. Laparoscopy was the preferred approach, with a morbidity rate of 08 per cent. A beneficial effect of surgery on blood pressure, glucometabolic control and obesity was evident in all studies, with cure or improvement in 72, 46 and 39 per cent of patients respectively, compared with conservative management. The results for lipid metabolism were equivocal, because of a decrease in triglyceridaemia but discordant effects on cholesterol metabolism among the different studies. No beneficial effects on osteoporosis were found. ConclusionLaparoscopic adrenalectomy seems to be beneficial in reversing several metabolic effects of hypercortisolism, with a low morbidity rate. However, the heterogeneity and low quality of the available studies preclude definitive recommendations. Surgery may have a beneficial effectmixedM. Iacobone;M. Citton;M. Scarpa;G. Viel;M. Boscaro;D. NittiIacobone, Maurizio; Citton, Marilisa; M., Scarpa; Viel, Giovanni; Boscaro, Marco; Nitti, Donat

Lamniform vertebrae from the Aptian-Albian Marne a Fucoidi of Umbria-Marche domain (central Italy)

The elasmobranch fossil record is essentially represented by isolated teeth due to high fossilization potential of hard dental components. Conversely, the cartilaginous skeletal elements, even though mineralized, are less prone to survive to the biostratinomic and diagenetic processes with negative implications in our understanding of the evolution of the elasmobranch skeletal anatomy. Even if fossil shark taxonomy is almost based on tooth characters, fossil vertebrae can provide useful information concerning the age, ontogenetic stage, and total length of the individual, along with crucial taphonomical and paleoecological information. In the present contribution we report and describe the first occurrence of lamniform vertebrae from the Cretaceous of the Umbria-Marche Domain (Central Italy), from the Aptian–Albian Marne a Fucoidi cropping out at Mt Nerone (Umbria-Marche Ridge, Northern Apennines, Italy). Although the material is solely represented by ten vertebrae (four found semi-articulated), the presence of well-calcified, amphicoelous, and slightly dorso-ventrally compressed centra having notochordal spaces constricted and reduced to intervertebral lenses confidently support their attribution to the group of lamniform sharks. In addition to taphonomic considerations, we have tried to provide a possible age for the individual and to estimate the minimum total length for the entire body. Based on the general morphology of the centra, size and annual growth bands, the preserved material can be referred to a small-medium sized fusiform and pelagic lamniform shark, with a body length reaching not less than 1.59 m, and being characterized by an approximately sub-circular trunk area, and a moderately fast swimming and consistent maneuverability. Considering the relative scarcity of shark vertebral remains in the fossil record, the material described herein represents an important source of information for the paleobiology of the Umbria-Marche Domain of Central Italy during the Cretaceous period

The result of surgery for mediastinal parathyroid tumors: a comparative study of 63 patients.

Purpose: Parathyroidectomy for ectopic mediastinal hyperfunctioning glands could be performed by transcervical approach, sternotomy, thoracotomy, and recently by thoracoscopic and mediastinoscopic approaches. This study was aimed to analyze the results of traditional and video-assisted parathyroidectomy for mediastinal benign hyperfunctioning glands. Methods: Fifty-one upper mediastinal exploration by a conventional cervicotomy, 12 by video-assisted approaches (two thoracoscopy and 10 transcervical mediastinoscopy) and six by sternotomy were performed in 63 patients with primary hyperparathyroidism. Results: Video-assisted and sternotomic parathyroid explorations achieved biochemical cure in all cases; following conventional transcervical mediastinal exploration, a persistent hyperparathyroidism occurred in 11.8% of patients, who were subsequently cured by sternotomic approach. No complications occurred after video-assisted parathyroidectomy, while an overall morbidity rate of 50% and 10% was found after sternotomic and conventional cervicotomic approaches. Postoperative pain and hospital stay were significantly increased following sternotomy; patient's subjective cosmetic satisfaction was significantly higher after video-assisted and conventional cervicotomic approaches. Conclusions: Conventional cervicotomic parathyroidectomy may achieve satisfactory results, especially for upper mediastinal glands. Sternotomic approaches are effective, but should be limited because of invasiveness and increased morbidity. In case of deep and lower hyperfunctioning mediastinal parathyroids, video-assisted approaches represent a less invasive, effective, and safe alternative and might be the technique of choice