Ileocecal appendix involvement in fabry disease mimicking an acute abdomen

Anderson-Fabry disease (AFD) is a rare, X-linked, lysosomal storage disorder due to a deficiency of alphagalactosidase A. The direct consequence is a lipid storage with the accumulation of glycosphingolipids throughout the body. The clinical picture is highly variable and depends on cellular storage deposition ranging from neurological, cutaneous and renal symptoms to cardiac and gastrointestinal ones. We are reporting about the case of a young female carrier of alpha-galactosidase A (agalA) gene mutation who was treated at our out-clinic practice for minimal neurological involvement (achroparaestesia). She was subsequently admitted in order to undergo appendectomy because of an acute severe abdominal pain. The histological examination of her appendix revealed only a deposition of globotriaosylceramide (Gb3) without any sign of acute inflammation. This case confirms the extreme clinical variability of Fabry disease and how the gastrointestinal involvement diagnosis can be misse

Kinematics of the Southern Rhodope Core Complex (North Greece)

The Southern Rhodope Core Complex is a wide metamorphic dome exhumed in the northern Aegean as a result of large-scale extension from mid-Eocene to mid-Miocene times. Its roughly triangular shape is bordered on the SW by the Jurassic and Cretaceous metamorphic units of the Serbo-Macedonian in the Chalkidiki peninsula and on the N by the eclogite bearing gneisses of the Sideroneron massif. The main foliation of metamorphic rocks is flat lying up to 100 km core complex width. Most rocks display a stretching lineation trending NEâ SW. The Kerdylion detachment zone located at the SW controlled the exhumation of the core complex from middle Eocene to mid-Oligocene. From late Oligocene to mid-Miocene exhumation is located inside the dome and is accompanied by the emplacement of the synkinematic plutons of Vrondou and Symvolon. Since late Miocene times, extensional basin sediments are deposited on top of the exhumed metamorphic and plutonic rocks and controlled by steep normal faults and flat-ramp-type structures. Evidence from Thassos Island is used to illustrate the sequence of deformation from stacking by thrusting of the metamorphic pile to ductile extension and finally to development of extensional Plio-Pleistocene sedimentary basin. Paleomagnetic data indicate that the core complex exhumation is controlled by a 30� dextral rotation of the Chalkidiki block. Extensional displacements are restored using a pole of rotation deduced from the curvature of stretching lineation trends at core complex scale. It is argued that the Rhodope Core Complex has recorded at least 120 km of extension in the North Aegean, since the last 40 My

Arc–continent collision : the making of an orogen

There is no one model, no paradigm, that uniquely defines arc–continent collision. Natural examples and modelling of arc–continent collision show that there is a large degree of, and variation in, complexity that depend on a number of key first-order parameters and the nature of the main players; the continental margin and the arc–trench complex (the arc–trench complex includes the arc and the subduction zone). Although modelling techniques can be used to gain insights into these, they cannot and do not aim at reproducing the messiness of nature. In natural examples, identifying the nature of the main players involved, such as the age, physical properties, and pre-existing structure of the margin and the arc is just a beginning. Once this is done, parameters such as time, convergence velocity and vector need to be taken into account when determining the tectonic processes that were operative in any one arc–continent collision. In active examples, such as those in the southwest Pacific, some of these first-order parameters can be readily determined, and the nature of the main players easily assessed. Fossil arc–continent collisions, however, have commonly undergone post-collision deformation, erosion, and possibly partial dispersion to be left outcropping in the middle of a forest, with many of the key ingredients missing or hidden. This leaves the geologist to resort to comparison with other natural examples and with models that are mechanically constrained and simplified reproductions of the process to reconstruct and explain what may have been there and, importantly, what processes may have been operating and when. We attempt to show that this is not an easy task that can be put into one simple model. In this chapter we do not present a model for arc–continent collision. Instead, we begin with the main players involved, highlighting the characteristics of each that likely have a major influence on an arc–continent collision. Then, we investigate a range of possible processes that could take place once an intra-oceanic volcanic arc collides with a continental margin.17 page(s

Understanding Factors Associated With Psychomotor Subtypes of Delirium in Older Inpatients With Dementia

Objectives: Few studies have analyzed factors associated with delirium subtypes. In this study, we investigate factors associated with subtypes of delirium only in patients with dementia to provide insights on the possible prevention and treatments. Design: This is a cross-sectional study nested in the “Delirium Day” study, a nationwide Italian point-prevalence study. Setting and Participants: Older patients admitted to 205 acute and 92 rehabilitation hospital wards. Measures: Delirium was evaluated with the 4-AT and the motor subtypes with the Delirium Motor Subtype Scale. Dementia was defined by the presence of a documented diagnosis in the medical records and/or prescription of acetylcholinesterase inhibitors or memantine prior to admission. Results: Of the 1057 patients with dementia, 35% had delirium, with 25.6% hyperactive, 33.1% hypoactive, 34.5% mixed, and 6.7% nonmotor subtype. There were higher odds of having venous catheters in the hypoactive (OR 1.82, 95% CI 1.18-2.81) and mixed type of delirium (OR 2.23, CI 1.43-3.46), whereas higher odds of urinary catheters in the hypoactive (OR 2.91, CI 1.92-4.39), hyperactive (OR 1.99, CI 1.23-3.21), and mixed types of delirium (OR 2.05, CI 1.36-3.07). We found higher odds of antipsychotics both in the hyperactive (OR 2.87, CI 1.81-4.54) and mixed subtype (OR 1.84, CI 1.24-2.75), whereas higher odds of antibiotics was present only in the mixed subtype (OR 1.91, CI 1.26-2.87). Conclusions and Implications: In patients with dementia, the mixed delirium subtype is the most prevalent followed by the hypoactive, hyperactive, and nonmotor subtype. Motor subtypes of delirium may be triggered by clinical factors, including the use of venous and urinary catheters, and the use of antipsychotics. Future studies are necessary to provide further insights on the possible pathophysiology of delirium in patients with dementia and to address the optimization of the management of potential risk factors