Hereditary neuralgic amyotrophy and hereditary neuropathy with liability to pressure palsies: two distinct clinical, electrophysiologic, and genetic entities

Fistulas secondary to Crohn's disease occur in about 33% of patients. A colobronchial fistula complicating Crohn's disease is very rare, having been reported only twice previously. We present an unusual fistula secondary to Crohn's colitis that originated from the splenic flexure and crossed the diaphragm to involve the bronchial tree.     

A Deterministic Logical Semantics for Esterel

Esterel is a synchronous design language for the specification of reactive systems. There exist two main semantics for Esterel. On the one hand, the logical behavioral semantics provides a simple and compact formalization of the behavior of programs using SOS rules. But it does not ensure deterministic executions for all programs and all inputs. As non-deterministic programs have to be rejected as incorrect, this means it defines behaviors for incorrect programs, which is not convenient. On the other hand, the constructive semantics is deterministic (amongst other properties) but at the expense of a much more complex formalism. In this work, we construct and thoroughly analyze a new deterministic semantics for Esterel that retains the simplicity of the logical behavioral semantics, from which it derives. In our view, it provides a much better framework for formal reasoning about Esterel programs.     

To what extent is the tibia-calcaneum angle a reliable measurement of the triceps surae length? Radiological correction of the torque-angle curve (III)

Previous papers gave some methods for the reliable measurement of the tibia-calcaneum angle. It is of common use to evaluate the physical properties of triceps surae on the basis of torque-angle curves. However this method is reliable only if each tibia-calcaneum angle corresponds to a defined distance between the insertions of the muscle in subjects of the same height. Evidence is given by radiological measurements that this correspondance is correct in normal children. However, this is no longer true in certain cerebral palsied children because of abnormal translation of the calcaneum and/or abnormal ratio of bone sizes. In this case the torque-angle curves do not define properly the torque-length curves. A method of correction is given. This correction may be as high as 15 degrees.     

Comparison of Mono- and Di-Saccharides Release in Aqueous Solutions by Raw or Fried Dice of Onion (Allium Cepa L.) Bulbs using Quantitative Nuclear Magnetic Resonance (qNMR)

ABSTRACT:  Although onion bulb tissues, either raw or thermally processed, are widely used as culinary ingredients in homes, in restaurants, and in the food industry, especially for sauces, little is known about the chemical constituents released from such systems. To get a straightforward and fast analysis of sugars released from onion dice soaked in model aqueous solutions, quantitative nuclear magnetic resonance (qNMR) spectroscopy was applied, and the effect of a preliminary thermal processing in oil was investigated. Soaking of raw or fried onion bulb dice at room temperature was followed for 11 d as a model of long-term storage. For the Armstrong cultivar, the extracted dry matter (in milligrams per gram of fresh weight) as well as the content in 3 sugars (glucose, fructose, and sucrose) increased up to a maximum after about 48 h of soaking. Frying induces no measurable new water-soluble compounds. However, extraction kinetics are different (about 3 times faster with frying). Using additional microscopic studies, a possible extraction mechanism is proposed: compounds from sap—including sugars—would diffuse through conductive tissue channels.     

SHIM: a deterministic model for heterogeneous embedded systems

Typical embedded hardware/software systems are implemented using a combination of C and an HDL such as Verilog. While each is well-behaved in isolation, combining the two gives a nondeterministic model of computation whose ultimate behavior must be validated through expensive (cycle-accurate) simulation. We propose an alternative for describing such systems. Our software/hardware integration medium (shim) model, effectively Kahn networks with rendezvous communication, provides deterministic concurrency. We present the Tiny-shim language for such systems and its semantics, demonstrate how to implement it in hardware and software, and discuss how it can be used to model a real-world system. By providing a powerful, deterministic formalism for expressing systems, designing systems, and verifying their correctness will become easier.     

Characterisation of crosslinked polyethylene materials by luminescence techniques

Luminescence features of cross-linked polyethylene samples peeled from high voltage cables have been investigated. Special emphasis is given to the effect of crosslinking by-products on the emission. Photoluminescence and luminescence following excitation by a silent discharge have been examined in order to identify which of the luminescent centres are optically active following electrical charge recombination, and could therefore act as charge traps. Both fluorescence and phosphorescence contain signatures of volatile and bound species. However, the identification of the origin of these emissions is far from complete. The signature of acetophenone has been clearly identified in the phosphorescence spectra. Radiative charge recombination involves the long lived components of the phosphorescence spectrum, i.e. the lowest lying triplet states. Some routes are given for identifying more precisely the origin of the emissions.     

Identification and molecular characterization of a small 11q23.3 de novo duplication in a patient with Rett syndrome manifestations

BACKGROUND AND STUDY AIMS: Modern fine-caliber endoscopes enable clinicians to directly visualize the pancreatic duct. They allow intraductal manipulation under optical control. We tried to evaluate the additional diagnostic potential of pancreatoscopy in assessing inconclusive intraductal pancreatic changes. PATIENTS AND METHODS: We prospectively performed 20 pancreatoscopies in 18 patients with inconclusive ductal abnormalities that had been previously investigated by computed tomography (CT) scan, abdominal ultrasound and endoscopic retrograde cholangiopancreatography (ERCP). The CHF-BP 30 (Olympus Optical Co., Japan) endoscope with an outer diameter of 3.1 mm and an instrumentation channel of 1.2 mm was used. Biopsies, cytological brushing and fluid collection were carried out, and the site of ductal abnormality was visualized. Endoscopic sphincterotomy (EST) was carried out in every patient prior to insertion of the pancreatoscope. RESULTS: Seven intraductal tumors were histologically confirmed, i.e. five intraductal papillary mucinous tumors and two adenocarcinomas. Benign appearance of the intraductal lesion plus negative histopathological examinations were confirmed by a follow-up of two years in eight patients. Five had chronic pancreatitis, and a further three had pancreatitis with strictures, blood clot obstruction, and idiopathic benign stricture, respectively. There were no complications with the exception of one bleeding episode after EST; no pancreatitis occurred. CONCLUSIONS: Pancreatoscopy is of diagnostic value in addition to CT, transabdominal ultrasound and ERCP in the differential diagnosis of poorly defined pancreatic lesions, particularly when assessing alterations of the ductal caliber without parenchymatous lesions.     

Insulin-dependent diabetes associated with celisc disease in two families

Two families in which insulin-dependent diabetes was associated with coeliac disease were studied. All patients had the HLA B 8 antigens. In one case there was an association of HLA B 8 with BW 15. The HLA B 8 determinant was found in that of the parents with a familial diabetic trait. These findings contribute to replace the association diabetes-coeliac disease in the larger frame of auto-immune diseases.     

Excess Sludge Production in Membrane Bioreactors: A Theoretical Investigation

This paper presents a theoretical investigation on excess sludge production in membrane bioreactors for municipal wastewater treatment. Based on mass balances of sludge and substrate, a formula to predict the excess sludge production in membrane bioreactors is introduced and verified by experimental data. The effects of kinetic parameters and operating conditions on excess sludge production are discussed for strong-, medium-, and low-strength municipal wastewaters, respectively. The strategy for reducing excess sludge production is recommended in order of priority, as sludge retention time→kd→Y→hydraulic retention time. Furthermore, the differences between membrane bioreactors and activated sludge processes are analyzed from the viewpoint of excess sludge production.