''Milia en plaque'' in the supraclavicular area

'Milia en plaque' is an unusual skin disease. Up to date only 6 cases have been reported, and all of them were located in the retro-auricular area. We report herein the first case of milia en plaque developed in the supraclavicular area. The absence of a known aetiologic factor in contrast to the previously published cases suggests that the present case belongs to the group of primary milia.     

Pachydermoperiostosis. An ultrastructural study

BACKGROUND: Pachydermoperiostosis (PDP) is a rare genetically determined disease belonging to the group of hypertrophic osteoarthropathies. Its aetiopathogenesis remains unclear. Most hypotheses favour an exogenous stimulation of fibroblasts. METHODS: A clinically typical patient with PDP was studied by electron microscopy with particular reference to the dermis and its cellular constituents. Fibroblasts from involved skin were cultured and studied in comparison with control cells. RESULTS: Remarkable modifications of the structure of the dermis were observed, encompassing irregular caliber of collagen fibres, extracellular deposits of microfibrils and of amorphous granular substance corresponding to the Alcian blue positive deposits seen by conventional histochemistry. The in vitro growth of fibroblasts was normal. CONCLUSION: Authors reviewed aetiopathogenic hypotheses. Our data suggest a genetically determined alteration of extracellular matrix production by fibroblasts as a possible explanation for the development of PDP.     

Correct-by-construction model driven engineering composition operators

Model composition is a crucial activity in Model Driven Engineering both to reuse validated and verified model elements and to handle separately the various aspects in a complex system and then weave them while preserving their properties. Many research activities target this compositional validation and verification (V & V) strategy: allow the independent assessment of components and minimize the residual V & V activities at assembly time. However, there is a continuous and increasing need for the definition of new composition operators that allow the reconciliation of existing models to build new systems according to various requirements. These ones are usually built from scratch and must be systematically verified to assess that they preserve the properties of the assembled elements. This verification is usually tedious but is mandatory to avoid verifying the composite system for each use of the operators. Our work addresses these issues, we first target the use of proof assistants for specifying and verifying compositional verification frameworks relying on formal verification techniques instead of testing and proofreading. Then, using a divide and conquer approach, we focus on the development of elementary composition operators that are easy to verify and can be used to further define complex composition operators. In our approach, proofs for the complex operators are then obtained by assembling the proofs of the basic operators. To illustrate our proposal, we use the Coq proof assistant to formalize the language-independent elementary composition operators Union and Substitution and the proof that the conformance of models with respect to metamodels is preserved during composition. We show that more sophisticated composition operators that share parts of the implementation and have several properties in common (especially: aspect oriented modeling composition approach, invasive software composition, and package merge) can then be built from the basic ones, and that the proof of conformance preservation can also be built from the proofs of basic operators.     

Reasoning over time into models with DataTime

Software and Systems Modeling - Models at runtime have been initially investigated for adaptive systems. Models are used as a reflective layer of the current state of the system to support the...     

Modeling modeling modeling

Model-driven engineering and model-based approaches have permeated all branches of software engineering to the point that it seems that we are using models, as Molière??s Monsieur Jourdain was using prose, without knowing it. At the heart of modeling, there is a relation that we establish to represent something by something else. In this paper we review various definitions of models and relations between them. Then, we define a canonical set of relations that can be used to express various kinds of representation relations and we propose a graphical concrete syntax to represent these relations. We also define a structural definition for this language in the form of a metamodel and a formal interpretation using Prolog. Hence, this paper is a contribution towards a theory of modeling.     

High index microlenses for GaAlAs laser-fibre coupling

The best compromise between coupling efficiency, accuracy of positioning and reproducibility has been obtained for a high index hemispherical lens melted on the tip of an intermediate fibre between the GaAlAs laser and the transmission fibre. The letter describes the lens fabrication technique and gives various experimental results of coupling efficiencies and mechanical tolerances.     

Microwave synthesis of yttria stabilized zirconia

Yttria stabilised zirconia (YSZ) nanocrystals, with a mean size between 5 and 10 nm, were prepared by microwave flash synthesis. Flash synthesis was performed in alcoholic solutions of yttrium, zirconium chloride and sodium ethoxide (EtONa) using a microwave autoclave (RAMO system) specially designed by authors. Energy dispersive X-ray analysis (EDX), X-ray powder diffraction (XRD), BET adsorption technique, photon correlation spectroscopy (PCS) transmission and scanning electron microscopy (TEM and SEM) are used to characterized these nanoparticles. Compared with conventional synthesis, nanopowders can be produced in a short period (e.g. 10 s), both high purity and stoechiometric control are obtained. Nevertheless, this mean of production is more cheaper and much faster than the ones commonly used to produce yttria stabilized zirconia (YSZ) by conventional sol-gel techniques.     

Multiple Moll''s gland cysts (apocrine hidrocystomas) of the eyelids

A 90-year-old man with a longstanding bilateral essential tremor presented with right-sided weakness of sudden onset. The CT scan of the brain showed a hypodense area in the left side of the pons consistent with an infarction. The paralysis disappeared without recurrence of the tremor on the right, but the tremor persisted on the left. It is likely that the fronto-rubro-spinal pathway had been interrupted at the level of the pons abolishing the tremor on the right side.     

Case for diagnosis. Actinic granuloma

We present a 3-year-old boy with scleroatrophic syndrome of Huriez, a rare autosomal dominant condition with only seven affected families worldwide. Although assumed to occur from birth, an evolving case has not previously been documented. Infants do not possess the focal areas of keratoderma on the palms or soles; these develop in adult life. Of particular interest is the high incidence of squamous cell carcinomas that arise from the scleroatrophic skin.     

Questionability of the benefits of routine laparotomy as the surgical approach for pheochromocytomas and abdominal paragangliomas

BACKGROUND: Improvement of preoperative imaging of pheochromocytomas and abdominal paragangliomas may render routine laparotomy questionable as the surgical approach of choice for these lesions. METHODS: We studied the records of 100 patients with chromaffin tumors who underwent abdominal exploration. The disease was familial in 28 patients and was malignant in 19. Seventy-five patients had intraadrenal disease (bilateral in 13). Computed tomography (CT), metaiodobenzylguanidine (MIBG) scintigraphy, and magnetic resonance imaging (MRI) were performed since 1979, 1984, and 1987 in 97, 73, and 43 patients, respectively. False-positive and false-negative results were defined as any discrepancy between imaging results and surgical findings. RESULTS: Overall accuracy of preoperative localization was 85% with CT scan, 77% with MIBG scintigraphy, and 86% with MRI. In unilateral pheochromocytoma, accuracy was 94% with CT scan, 80% with MIBG scintigraphy, and 96% with MRI. When all three studies were performed (n = 38), overall accuracy was 97% and only one extraadrenal tumor in a patient with familial pheochromocytoma was overlooked. CONCLUSIONS: The outstanding accuracy of available imaging techniques questions the strategy of routine laparotomy for sporadic and seemingly benign pheochromocytomas, favoring more elective approaches such as the posterior approach or laparoscopy.