Clinical features of pain in amyotrophic lateral sclerosis: A clinical challenge.

Pain in amyotrophic lateral sclerosis (ALS) is paradoxical in this disease of the upper and lower motor neurons. As such, it remains an underestimated and neglected clinical problem because it is poorly identified by physicians, its mechanisms are numerous and its treatments are generally not effective. Pain may be primary in the form of cramps, spasticity and neuropathy, or secondary as nociceptive pain, and may arise before the first motor symptoms. It may also lead to depression and, in all cases, affect patients' daily activities and quality of life. Given the high frequency of pain in ALS, the use of analgesic or sedative drugs is necessary and should reduce the course of the disease. Nevertheless, it is important to understand the pathophysiological mechanisms of pain in ALS, and to train physicians how to detect ALS pain early on and provide dedicated treatments. In France, the implementation of ALS centers is a positive response to the public-health problem resulting from this disorder.

Development of an integrated methodology for the sustainable environmental and socio-economic management of river ecosystems.

The development of the Water Framework Directive aimed to establish an integrated framework of water management at European level. This framework revolves around inland surface waters, transitional waters, coastal waters and ground waters. In the process of achieving the environment and ecological objectives set from the Directive, the role of economics is put in the core of the water management. An important feature of the Directive is the recovery of total economic cost of water services by all users. The total cost of water services can be disaggregated into environmental, financial and resource costs. Another important aspect of the directive is the identification of major drivers and pressures in each River Basin District. We describe a methodology that is aiming to achieve sustainable and environmental and socioeconomic management of freshwater ecosystem services. The Ecosystem Services Approach is in the core of the suggested methodology for the implementation of a more sustainable and efficient water management. This approach consists of the following three steps: (i) socio-economic characterization of the River Basin area, (ii) assessment of the current recovery of water use cost, and (iii) identification and suggestion of appropriate programs of measures for sustainable water management over space and time. This methodology is consistent with a) the economic principles adopted explicitly by the Water Framework Directive (WFD), b) the three-step WFD implementation approach adopted in the WATECO document, c) the Ecosystem Services Approach to valuing freshwater goods and services to humans. Furthermore, we analyze how the effects of multiple stressors and socio-economic development can be quantified in the context of freshwater resources management. We also attempt to estimate the value of four ecosystem services using the benefit transfer approach for the Anglian River Basin, which showed the significance of such services.

Sex differentiation in an all-female (XX) rainbow trout population with a genetically governed masculinization phenotype.

Sex determination is known to be male heterogametic in the rainbow trout, Oncorhynchus mykiss; however, scattered observations that deviate from this rather strict genetic control have been reported. Here, we provide a detailed morphological and histological characterization of the gonadal differentiation and development (from 43 days postfertilization to 11 months of age) in an all-female (XX) population with a genetically governed masculinization phenotype. In comparison with control males and females, the gonadal differentiation in these animals was characterized by many perturbations, including significantly fewer germ cells. This decrease in germ cells was confirmed by the significantly decreased expression of 2 germ cell maker genes (vasa and sycp3) in the masculinized XX populations as compared with the control females and control males. Although only a proportion of the total adult population was partially or fully masculinized, this early differentiating phenotype affected nearly all the sampled animals. This suggests that the adult masculinization phenotype is the consequence of an early functional imbalance in ovarian differentiation in the entire population. We hypothesize that the lower number of germ cells that we observed in this population could be one cause of their masculinization.

Semen cryopreservation for ex situ management of genetic diversity in chicken: creation of the French avian cryobank.

The need for semen preservation in domestic birds is a result of the reduction in genetic variability of domestic bird livestock and of the increasing risk of line extinction for health and safety reasons. Cryopreservation of embryos and primordial germ cells (PGC) is not routinely feasible in birds. The project therefore involved semen frozen in optimal safety and traceable conditions. Whole blood samples were also frozen to provide samples of analyses of genomes and health status. The feasibility of using ex situ conservation, i.e., collecting biological material to be stored outside the usual production area of the species (ex situ genetic stock), to preserve and manage rare breeds was tested with 4 subfertile populations: 3 rare experimental lines used for research into energy metabolism (R+), growth (Y33), and immunity (B4/B4), reared under known health status and the oldest endangered patrimonial French breed, the Gauloise dorée with an unknown health status. A general infrastructure was set up for the health screening and remediation of diseases, collection and storage of frozen cells and 2 sites were created for the storage of frozen samples. The screening and remediation of diseases of the Gauloise dorée, which was contaminated with various Salmonella and Mycoplasma strains, was achieved by successive treatment of parents, incubated eggs and young chicks with Baytril followed by Tiamulin. For each line, 474 to 994 semen straws have been frozen, thawed, and the semen evaluated. Insemination of frozen-thawed semen into females of the same genetic origin or of an egg-type commercial breed produced chicks in every case. For the most subfertile lines, insemination with egg-type females significantly increased the reproductive success. In conclusion, we report on the benefits of a semen and blood cryobanking complex for the management of endangered lines and strains of domestic birds. Current stocks made possible the restoration of more than 96% of the initial genome. This project also provided technical solutions to resolve some of the health problems frequently encountered for gene preservation in poultry.

[Clinical, biological, histological, ultrastructural and therapeutic studies in one case (author's transl)]. / Une observation de glucagonome. 1--Etude clinique, biologique, histologique, ultrastructurale et thérapeutique.

The diagnosis of glucagonoma was made in a 51 year-old woman who suffered from a polymorphous dermatitis and an insulin-dependent diabetes mellitus. Denutrition was present and there was a previous history of thrombo-embolism. Immunoreactive plasma glucagon was constantly higher than 1 000 pg/ml (N less than 175). Plasma aminoacids were low. After angiographic confirmation, the tumour and part of its hepatic metastases were resected. The dermatitis disappeared soon after. Its recurrence required chemotherapy (successively mithramycin, streptozotocin, DTIC) and good clinical results were obtained. On histological examination, the cutaneous lesions consisted of an epidermal edema, and a bullous intra-epidermic detachment. The pancreatic tumour was of the trabecular type with a very important sclerosis. On electron microscopy, the tumoral cells, some with a syncitial aspect, contained granules of the D1 type. These granules are different from the typical glucagon granules. The clinical and biological features in this case are compared with those of the 41 cases of glucagonoma previously published.

[Pulmonary artery thrombosis during hormonal contraceptive therapy. Treatment with urokinase]. / Thrombose de l'artère pulmonaire sous traitement contraceptif hormonal. Traitement par l'urokinase

PIP: A 32-year-old mother of 3 who had taken .05 mg ethinyl estradiol and .5 mg norgestrel for 3 months had a severe right thoracic pain of 2 weeks' duration, which was diagnosed as pulmonary arterial thrombosis and treated with high doses of urokinase. She was first given 600 mg heparin/24 hours, then 300,000 U of urokinase over 12 hours. There was some improvement, so urokinase was repeated and heparin continued. The patient was then asymptomatic, but she was found to have hyperlipidemia, hyperglycemia, insulinemia, and uricemia. She was well 6 months later on a carbohydrate- and fat-controlled diet. High doses of urokinase are preferred by some who believe that urokinase is thrombolytic in proportion to dose, well tolerated, and not antigenic.^ieng