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Nephronophthisis (NPH) is an autosomal-recessive ciliopathy representing one of the most frequent causes of kidney failure in childhood characterized by a broad clinical and genetic heterogeneity. Applied to one of the worldwide largest cohorts of patients with NPH, genetic analysis encompassing targeted and whole exome sequencing identified disease-causing variants in 600 patients from 496 families with a detection rate of 71%. Of 788 pathogenic variants, 40 known ciliopathy genes were identified. However, the majority of patients (53%) bore biallelic pathogenic variants in NPHP1. NPH-causing gene alterations affected all ciliary modules defined by structural and/or functional subdomains. Seventy six percent of these patients had progressed to kidney failure, of which 18% had an infantile form (under five years) and harbored variants affecting the Inversin compartment or intraflagellar transport complex A. Forty eight percent of patients showed a juvenile (5-15 years) and 34% a late-onset disease (over 15 years), the latter mostly carrying variants belonging to the Transition Zone module. Furthermore, while more than 85% of patients with an infantile form presented with extra-kidney manifestations, it only concerned half of juvenile and late onset cases. Eye involvement represented a predominant feature, followed by cerebellar hypoplasia and other brain abnormalities, liver and skeletal defects. The phenotypic variability was in a large part associated with mutation types, genes and corresponding ciliary modules with hypomorphic variants in ciliary genes playing a role in early steps of ciliogenesis associated with juvenile-to-late onset NPH forms. Thus, our data confirm a considerable proportion of late-onset NPH suggesting an underdiagnosis in adult chronic kidney disease.

Modafinil, a wake-promoting compound now used worldwide in sleep medicine, was initially regarded as a sedative compound because mice were so quiet with respect to locomotion after receiving it that this behavioral state was qualified as sedation. In the early 1980's when modafinil was first assessed by polysomnography in a cat in our laboratory, surprisingly, the cat spent the whole night awake without even one minute of sleep! This initial observation resulted subsequently in a series of basic and clinical studies in order to define the pharmacological profile of modafinil and its mode of action and, notably, to identify the brain targets by which modafinil acts to promote wakefulness. These studies were undertaken using pharmacologic approach coupled with the Cerveau isole (brain transection) preparation, c-fos labelling and knockout mouse models. It was also in this context that we have developed a purified polyclonal antibody against modafinil. We expected that using immunohistochemistry with this antibody would allow us to localize the brain distribution of modafinil dosing. Surprisingly, we found discrete modafinil immunoreactive neuronal populations in several brain areas of modafinil-naive cats, rodents and humans. The most numerous and intensely labeled modafinil-immunoreactive neurons characterized by granular staining were found in the basal forebrain. They shared the regional location with cholinergic and aspartate-containing neurons but did not colocalize with them. In summary, we here present a newly identified neuronal population located in the basal forebrain that has never previously been published and suggests that these modafinil-immunoreactive neurons might be involved in forebrain functions such as sleep-wake control and cognition. This paper briefly reviews our journey with modafinil research and presents new unpublished experimental data.

Traditional agricultural landscapes support substantial levels of biological and cultural diversity. Tropical homegardens, which represent sustainable agro-ecosystems, are important components of such landscapes. In this study, homegardens of Basketo Special Woreda of Southwestern Ethiopia have been investigated. The study aims at understanding organization of homegardens, their role in maintenance of biological diversity and also the impact of ongoing changes on the composition and function of the gardens. A total of 60 homegardens (households) were sampled from 12 k’ebele (the smallest administrative unit) selected by employing a combination of purposive and stratified sampling methods. In the study, issues that pertain to local resource perception, management, and use norms as well as plant diversity of the different land-use systems in the landscape are addressed. A total of 207 species, of which 149 are maintained in the homegardens, were recorded from the managed landscape. Biodiversity has been cultivated in these farming units as a result of the farmers’ innate perception of biodiversity value and also due to the characteristic organization of the gardens which promote concentration of plant species. Currently, Basketo homegardens are undergoing unusual dynamics mainly due to market-driven factors. Some crops such as coffee/ buna ( Coffea arabica L.), which bring better economic return, are expanding and displacing enset / uuts ( Ensete ventricosum (Welw.) Cheeseman) (which traditionally forms the basic element of the garden) and other long-existed crops. Drastic alteration of these crop production units could lead to unwanted impacts including a serious deterioration of biological diversity and loss of the sustainability feature of the agro-ecosystems.

Background/Aims: Dent’s disease is caused by mutations in the chloride/proton antiporter, CLC-5, or oculo-cerebro-renal-syndrome-of-Lowe (OCRL1) genes. Methods: Eighteen probands with Dent’s disease were investigated for mutations in CLC-5 and two of its interacting proteins, CLC-4 and cofilin. Wild-type and mutant CLC-5s were assessed in kidney cells. Urinary calcium excretion following an oral calcium challenge was studied in one family. Results: Seven different CLC-5 mutations consisting of two nonsense mutations (Arg347Stop and Arg718Stop), two missense mutations (Ser244Leu and Arg516Trp), one intron 3 donor splice site mutation, one deletion-insertion (nt930delTCinsA) and an in-frame deletion (523delVal) were identified in 8 patients. In the remaining 10 patients, DNA sequence abnormalities were not detected in the coding regions of CLC-4 or cofilin, and were independently excluded for OCRL1. Patients with CLC-5 mutations were phenotypically similar to those without. The donor splice site CLC-5 mutation resulted in exon 3 skipping. Electrophysiology demonstrated that the 523delVal CLC-5 mutation abolished CLC-5-mediated chloride conductance. Sixty percent of women with the CLC-5 deletion-insertion had nephrolithiasis, although calcium excretion before and after oral calcium challenge was similar to that in unaffected females. Conclusions: Three novel CLC-5 mutations were identified, and mutations in OCRL1, CLC-4 and cofilin excluded in causing Dent’s disease in this patient cohort.

Dans les pays du Sud, promouvoir et valoriser les productions et specialites locales est une tendance lourde : en temoignent les contributions, aussi riches que diversifiees reunies dans cet ouvrage et les nombreuses communications scientifiques proposees au Colloque international consacre a la question (Unesco, Paris, juin 2009). On peut voir dans cette dynamique partout constatee le reflet d'une volonte politique mondialisee (Jasanoff, 2004). Sous l'influence plus ou moins diffuse de grandes instances internationales comme la Banque mondiale, le FMI ou la Fao, les Etats se reforment, s'engagent dans la voie de la decentralisation et en privilegiant des modes de gouvernance plutot qu'un gouvernement, tentent de coordonner "un tissu mouvant d'acteurs varies qui interviennent aux diverses echelles" (Tubiana, 2005). Dans ce cadre, le local et le collectif sont reconnus comme des niveaux pertinents (Ostrom, 1990) et la societe civile est incitee a s'impliquer dans les politiques de developpement. Les communautes rurales, autochtones ou simplement locales, pour reprendre la terminologie chere a la Convention sur la Diversite Biologique, sont presentees comme les partenaires ideaux, et d'ailleurs souvent idealises, des politiques de developpement durable. Plutot que de recourir a une reglementation etatique globale, les gouvernements font de plus en plus appel a des instruments bases sur la contractualisation, point commun a toutes les demarches, en cours ou projetees, decrites et analysees dans les articles reunis dans ce numero de la revue Autrepart.

Twenty-seven patients (14 girls, 13 boys) affected by familial hypercholesterolemia who had begun low-density lipoprotein (LDL) apheresis treatment before the age of 15 were studied. The median age at diagnosis was 4 years and the blood LDL cholesterol level was 704 +/- 163 mg/dL. Screening was performed for homozygous or double heterozygous mutations of the LDL cholesterol receptor gene and mutations were found in 24 of the patients. The mean age at the beginning of treatment was 8.5 years and the mean length of follow up was 12.6 years. The two main procedures used were direct adsorption of lipoproteins and dextran sulfate cellulose adsorption. Nine patients experienced anaphylactic reactions due to bradykinin and six had to have their treatment changed. The LDL cholesterol level before the session was lowered by 45 +/- 11% of the value at diagnosis. The LDL cholesterol reduction in a session was 72 +/- 10%. Tendinous xanthomas disappeared or diminished dramatically in 62% of the children. In 22 patients no cardiovascular event occurred during LDL apheresis treatment. Three had angina pectoris; two others had surgical management of aortic stenosis, but no clinical manifestations. Seven children had normal cardiovascular pictures while on treatment. Eleven had abnormalities of the aortic root or coronary arteries, which in six cases had appeared before treatment; the other five children did not undergo prior cardiac evaluation. In five children the abnormalities appeared during treatment. Based on these data, LDL-apheresis can be recommended for the treatment of homozygous familial hypercholesterolemia, even in young children, with good efficiency on biological parameters, cutaneous lesions and cardiovascular events.

Darbepoetin alpha (DA) is a unique long-acting treatment for anaemia in patients with chronic renal failure (CRF). This study assessed the mean dose of DA to achieve and maintain haemoglobin (Hb) levels between 11 g/dl and 13 g/dl in CRF children aged 11 years to 18 years. This observational, prospective study was conducted in 39 patients treated with DA. Twenty-nine patients were switched from recombinant human erythropoietin (r-HuEPO), and ten patients were naive to r-HuEPO. Naive patients received initial doses of 0.45 microg/kg of DA. Switched patients received a dose adjusted to the prior dose of r-HuEPO (200 IU r-HuEPO:1 microg DA). Among the switched patients, 79.3% received dialysis. No naive patients underwent dialysis. Overall, 74% of patients showed increased Hb level, with a mean value of 11.6 +/- 1.6 g/dl, using a mean DA dose of 0.63 +/- 0.48 microg/kg per week, and 66.7% patients reached the target Hb level. Hb increased in naive patients from 9.5 (95% CI: 7.7, 11.4) to 11.7 (95% CI: 10.9, 12.6) g/dl and in switched patients from 11.1 (95% CI: 10.6, 11.5) to 11.5 (95% CI: 10.8, 12.2) g/dl). Higher doses of DA were needed in the "switched" than in the "naive" patients to maintain Hb levels over 11 g/dl, respectively 0.73 (95% CI: 0.54, 0.92) and 0.34 (95% CI: 0.16, 0.52) microg/kg per week. Our results indicate the doses of DA necessary to treat CRF patients aged 11 years to 18 years. DA was an effective treatment to stabilise CRF patients at extended dosing intervals.

The emergence of biodiversity standards in the nature conservation literature requires that we consider the interactions between conservation and local practices from a new angle. The coastal forest of Monogaga, a protected area inhabited by a local population, is an ideal terrain for comparing the impact of local agricultural practices and the activities of Sodefor, the government agency charged with the management of this conservation area. The discourses and uses of forest resources of these two actors allow us to compare the biodiversity of forest cover categories recognized by peasant farmers and Sodefor, using the standard statistical methods for measuring biodiversity (the Shannon and Weaver index, species richness, number of special status species).For Sodefor, it is the most dense forest ecosystems (the ‘black forests’ ) and the lands that they occupy that constitute the area's natural heritage. The agency believes that these forests must be protected from all human uses, especially farming, if the forest is to be transmitted to future generations. In contrast, Wanne farmers view the old forests (kporo) as long-term fallows (teteklwoa) or reserves of fertile land that will be cleared when there is a need for more farmland in the future. For them, patrimony is constituted by the intergenerational transmission of a bundle of resource access and farming rights within lineages.With regard to biodiversity, a comparison of the two types of resource management practices (Sodefor and farmer) gives nuanced results. The farmers' areas are more diverse than those of Sodefor when considering the Aké Assi threatened species list. For the Sassandrian species list, both management types maintained the same quantity of species. For endemics and the IUCN red list species, the spatial units controlled by Sodefor show more diversity.

Roussel Bernard. Duplessis-Mornay et l’exégèse des écrits bibliques : les lieux bibliques controversés dans De l’institution, usage et doctrine du saint Sacrement. In: Albineana, Cahiers d'Aubigné, 18, 2006. Philippe Duplessis-Mornay, sous la direction de Hugues Daussy et Véronique Ferrer. pp. 81-99.

The present study describes a new culture protocol allowing the activation and proliferation of autologous tumor infiltrating T lymphocytes (TIL), and the generation of antitumor specific CTL in non-Hodgkin's lymphoma (NHL). Cells from eight patients with indolent NHL were used. We performed 3-week co-cultures of TIL with irradiated autologous malignant B cells in the presence of low doses of IL-1beta, IL-2 and IL-12. The proliferation, phenotype and cytotoxicity, and antitumor specificity of T cells recovered were studied. T-cell clonality was analyzed using TCRgamma gene rearrangement amplification by a multiplex PCR. Under these culture conditions, TIL proliferated, and the CD8+ T lymphocytes that were in a minority at the beginning of the culture increased dramatically in 6 out of 8 cases. In two cases, CD4+ T lymphocytes expanded. We showed that an oligoclonal selection of reactive T cells occurred in culture. Specific cytotoxicity developed against autologous malignant B cells in the 6 cases where there was an expansion of CD8+ T lymphocytes. Inhibition experiments performed with mAb directed against HLA class I and II molecules, CD4, CD8 and TCRgammadelta showed that the cytotoxic effector cells were CD8+ T lymphocytes probably expressing TCRalphabeta+. Cytokine secretion was analyzed in culture medium, and we detected significant levels of IFN-gamma, TNF-alpha, and IL-10 and no IL-4 (except in one case). Our results demonstrate that memory T cells from lymphoma patients can be amplified and differentiated into antitumor cytotoxic cells using a combination of the cytokines IL-1beta, IL-2, and IL-12 in association with non modified tumor cells.

Antiviral activity was characterized from the culture broth of the Basidiomycete Macrocystidia cucumis (Pers. ex Fr.) Heim. When the stationary phase was reached (21 d), the culture broth was shown through an ELISA assay to contain antiviral activity against herpes simplex virus type 1 (HSV-1), as assessed in baby hamster kidney cells (BHK-21). Once the presence of the anti-HSV-1 activity in the culture broth was demonstrated, we proceeded with the purification and isolation of the active principle using a semi-preparative HPLC technique. The activity was associated with a purine nucleoside designated McA. This compound displayed no cytotoxicity at antivirally effective concentrations and proved to be a novel nucleoside analogue.

Dans les pays du Nord comme dans ceux du Sud, la patrimonialisation de la nature connaît actuellement un engouement de plus en plus vif. Des espèces aux espaces, des pratiques aux savoirs, les champs du patrimoine ne cessent de s'étendre, de se diversifier, et les processus de patrimonialisation de se multiplier : les populations locales interviennent de plus en plus fréquemment dans les démarches d'identification, de reconnaissance, de réhabilitation et de valorisation de leur patrimoine. Cette mise en patrimoine s'accompagne cependant souvent de conflits de représentations ou d ́intérêts. Les acteurs locaux questionnent notamment les objectifs des instruments de réglementation ou de qualification et s'inquiètent de la multiplication des normes et obligations qui accompagnent la culture imposée d'une plante, la mise en place d ́une indication géographique ou la création d ́un parc national. Cet ouvrage pluridisciplinaire met l'accent sur les nouveaux types de patrimoines (cultivars, pratiques gastronomiques, savoirs et savoir-faire locaux, etc.) et les nouveaux instruments de mise en valeur du patrimoine, naturel et culturel. Sont en particulier analysés les jeux d'acteurs, du local au global, les recompositions sociales, les réorganisations spatiales et institutionnelles, dans des contextes écologiques, politiques, économiques et sociaux en pleine mutation. Il offre un état des lieux actualisé et original sur les dangers de la patrimonialisation, ses limites et ses dérives.

Two alternative methods of Raman imaging, via global (wide-field) illumination and via point illumination in combination with confocal light collection, have been applied to the study of heterogeneous polymer systems. From the results obtained it becomes apparent that the fluorescence inherent to most polymer systems severely limits the use of global illumination. Furthermore, the lack in depth resolution in Raman imaging by global illumination ruled out this method for the study of bulk polymer samples. Also as a consequence of the absence of depth resolution, the global illumination technique appeared more vulnerable to artifacts arising from scattering effects due to the sample geometry and fluorescence. Hence, for a general application of Raman imaging to the study of polymer samples, Raman imaging by point illumination in conjunction with confocal light collection is the method of choice

The humid tropical zone of west Africa is perceived as having a rich but threatened flora and vegetation. Historical and scientific sources show that there is nothing new about this perception. An image of biological richness was developed with the first voyages to the region. It was not until the end of the nineteenth century that scientific inventories were made and there was a growing awareness of the impoverishment of flora and degradation of plant cover. This analysis also shows how European political and economic interests have always played a major role in the development of representations., La flore et la végétation de la zone tropicale humide de l'Afrique de l'Ouest sont actuellement perçues comme riches et menacées à la fois. Cette perception n'est pas nouvelle comme le montre l'analyse des sources historiques et scientifiques anciennes. Dès les premiers voyages, s'est élaborée une image mettant en valeur la richesse biologique de ces régions. Ce n'est qu'à partir de la fin du 19e siècle que les premiers inventaires scientifiques ont été réalisés et qu'apparurent les premières prises de conscience concernant l'appauvrissement de la flore et la dégradation des couverts végétaux. Cette analyse montre également que les intérêts économiques et politiques des nations européennes ont de tout temps joué un rôle important dans l'élaboration des représentations., Juhé-Beaulaton Dominique, Roussel Bernard. Tropiques d'abondance ou Tropiques menacées. Regards d'Européens sur la flore et la végétation de l'Afrique tropicale humide (XVIIe-XXe siècles). In: Journal d'agriculture traditionnelle et de botanique appliquée, 36ᵉ année, bulletin n°2,1994. Phytogéographie tropicale : réalités et perspectives. Propos d'ethnobiologie, sous la direction de Jacques Barrau et Bernadette Lizet. pp. 25-38.

Between 1555 and 1575, the reformed Churches of France organize themselves. Their rituals take shape, and amongst them, the Lord's Supper holds a preeminent place. It consists of an intimately linked sequence of words and of actions. Breaking of bread, pouring of wine into a cup accompanied by the words of institution, communion in both kinds: these are the principal elements of the rite. At the same time, local circumstances, differences in the comportment of the consistories who are responsible for fencing the table, shifting social and historical contexts, mean that the different elements of the common ritual - the anamnesis, the eschatological and anagogical references - are accentuated differently according to the particular circumstances. The rite evidently has a variety of functions. The reformed Supper seems above all to be a rite which has symbolic value, which gives to each communicant an element of salvation, the union of each with their heavenly saviour, an element whose full value is only experienced at the moment of death, a death which is not accompanied by any funerary rites. These description and analysis are incomplete, since no comparison is made with the equivalent contemporary practices of catholics, lutherans, anabaptists, nor is the historical evolution of the rite studied., Des églises réformées s'organisent dans le Royaume de France entre 1555 et 1575. Un système rituel s'esquisse, dans lequel la cène tient une place déterminante. Elle consiste en une séquence de gestes et de paroles qui ne sauraient être dissociés : la fraction du pain et le versement du vin dans une coupe, avec les paroles bibliques qui sont alors prononcées, la réception par tous des deux espèces, la consommation du pain et du vin au terme d'une procession en sont les éléments majeurs. La diversité des circonstances, les orientations des divers consistoires qui en contrôlent l'accès, des conditions socio-historiques changeantes, font que les traits d'anamnèse, d'eschatologie, d'anagogie, recueillis dans un unique formulaire rituel se trouvent diversement mis en valeur selon les situations. Diverses fonctions du rite sont indiquées. La cène réformée apparaît avant tout comme le rite qui produit symboliquement et communique à chaque croyant un bien de salut, son union à un sauveur céleste, un bien dont l'obtention plénière est différée jusqu'au décès du fidèle, qui n'est entouré d'aucun rite funéraire. Cette description et cette analyse sont partielles, car sont exclues ici les comparaisons avec des pratiques contemporaines équivalentes, catholiques, luthériennes, anabaptistes ou réformées, ainsi que l'étude des transformations du rite dans la longue durée., Entre 1555 y 1575 fueron organizadas en el Reino de Francia Iglesias Reformadas. Surge un análisis ritual en el que la cena ocupa un espacio determinante. Se trata de una secuencia de gestos y de palabras que no pueden ser disociados : la fración del pan y el derrame del vino en una copa, con las palabras bíblicas asociadas, la recepción por todos de las dos especies, el consumo del pan y del vino previa una procesión, son los elementos centrales. La diversidad de circunstancias, las orientaciones de los diversos consis- torios que controlan el acceso el cambio de las circunstancias históricas hacen que las características de la amnésis de la escatología de la analogía reunidos en un mismo formulario ritual, se encuentran diversamente valorizadas según las situaciones. La cena reformada aparece como el rito por exce lencia que produce simbólicamente comunica cada creyente un bien de salvación su unión un saver celeste un bien cuya obtención plena es di ferida hasta el fallecimiento de los fieles momento que no será rodeado de ningún rito funerario. Esta descripción este análisis son parciales ya que excluímos las comparaciones con las prácticas contemporáneas equivalentes católicas luteranas anabatistas reformadas el estudio de las transforma ciones del rito sobre el largo plazo., Roussel Bernard. «Faire la Cène» dans les églises réformées du royaume de France au XVIe siècle / « Taking Communion » in the Reformed Churches of the French Kingdom in the XVIth Century.. In: Archives de sciences sociales des religions, n°85, 1994. pp. 99-119.

We introduce and demonstrate two innovative macro-Raman mapping modes for advanced microstructural and mechanical characterization of photovoltaic (PV) materials. The macro- and micro- Raman results presented in this work are obtained on multicrystalline silicon (mc-Si) thin films on glass for solar cells. We show that detailed information can be extracted from the same first-order Raman spectra of solar silicon. This enables us to understand the interaction between stresses, defects, doping/impurities, and microstructure at identical positions as well as at different length scales. The fast and large-scale imaging Raman instrument allows us to evaluate statistically the materials properties and to see clearly the differences originating from different preparation conditions/processing. Based on these results, we believe that the Raman technique will become increasingly important in the PV community not only for fundamental studies but also for optimization and in-line quality check in a PV factory.

Confocal Raman microscopy provides a high spatial resolution because it operates in short wavelength region and utilizes confocal optics. However, the spatial resolution of a confocal Raman microscopy is not well understood, and often confused with the smallest measurable sample size. When performing Raman hyperspectral imaging with a confocal Raman microscope, it is also confused with the smallest distance a mapping stage can step. While all these parameters are pertinent to record a good Raman spectrum or a good Raman map, they are not spatial resolution, and thus have different impacts to the data and results. In this and subsequent papers, we will begin with the theoretical definition, examine the instrumental implementations and present the empirical applications of these parameters with examples.

Multichannel measurement of Raman bands with frequencies very close to the excitation line has traditionally been possible with the use of triple monochromator systems. These systems are very flexible, but they can be complex to use, large, low in throughput, and expensive. We have recently demonstrated the use of a single monochromator Raman system where the Rayleigh filtering elements are made of Volume Bragg Gratings (VBGs); such a system enabled recording of Stokes and antiStokes modes with frequencies less than 10 cm.

OBJECTIVE. We report a prospective, randomized, multicenter trial that compared the effect of 3 vs 8 days of intravenous ceftriaxone treatment on the incidence of renal scarring at 6 to 9 months of follow-up in 383 children with a first episode of acute pyelonephritis. METHODS. After initial treatment with intravenous netilmicin and ceftriaxone, patients were randomly assigned to either 5 days of oral antibiotics (short intravenous treatment) or 5 days of intravenous ceftriaxone (long intravenous treatment). Inclusion criteria were age 3 months to 16 years and first acute pyelonephritis episode, defined by fever of >38.5°C, C-reactive protein level of >20 mg/L, and bacteriuria at >105/mL. All patients underwent 99m technetium-dimercaptosuccinic acid scintigraphy 6 to 9 months after inclusion. A total of 548 children were included, 48 of whom were secondarily excluded and 117 of whom were lost to follow-up or had incomplete data; therefore, 383 children were eligible, 205 of them in the short intravenous treatment group and 178 in the long intravenous treatment group. RESULTS. At inclusion, median age was 15 months, median duration of fever was 43 hours, and median C-reactive protein level was 122 mg/L. A total of 37% (143 of 383) of patients had a vesicoureteral reflux grades 1 to 3. Patient characteristics at inclusion were similar in both groups, except for a significantly higher proportion of girls in the short intravenous treatment group. The frequency of renal scars at scintigraphy was similar in both groups. Multivariate analysis demonstrated that renal scars were significantly associated with increased renal height at initial ultrasound and with the presence of grade 3 vesicoureteric reflux. CONCLUSIONS. The incidence of renal scars was similar in patients who received 3 days compared 8 days of intravenous ceftriaxone. Increased renal height at initial ultrasound examination and grade 3 vesicoureteric reflux were significant risk factors for renal scars.

Among various processes of recognition and development of local know-how related to biodiversity, the protection systems based on Geographical Indications seem to open up interesting perspectives for the countries of the South. Ethiopia is on the way to equipping itself with such a tool. In this article we argue that Ethiopia offers an exceptionally good terrain for setting up such a mechanism. It has many products derived from the exploitation of biodiversity by a variety of cultural groups. Many of these products already have reputations linked to their cultural geographical origin. The existence of competitive national and international markets requires labels and protection systems. Nevertheless adoption of a Geographical Indications system is not without its problems. The specific circumstances of the Ethiopian context--social and institutional as well as environmental--raise questions as to the limitations and possible risks of such a system, including the unequal development of certain components of biodiversity, standardization and loss of know-how, modification of current territorial subdivisions and the corresponding social and administrative organizational structures. RESUME Parmi les processus de reconnaissance, de valorisation et de protection des savoir-faire locaux sur la biodiversite, les systemes d'indications geographiques semblent ouvrir d'interessantes perspectives aux pays du Sud. L'Ethiopie est en passe de se doter d'un tel outil. Il sera d'abord montre en quoi ce pays offre un champ d'application tout a fait exceptionnel a ce type de dispositif : de nombreux produits issus de l'exploitation de la biodiversite par des groupes culturels tres varies, jouissent deja de reputations liees a leur provenance; l'existence de marches concurrentiels nationaux et internationaux rendent judicieux la mise en place de labels et de protection. Cependant l'adoption d'un systeme d'Indications Geographiques n'est pas sans presenter certaines difficultes. Les specificites du contexte ethiopien, aussi bien sociales, institutionnelles et juridiques qu'environnementales, amenent a s'interroger sur les limites, voire les eventuels risques, d'un tel systeme: valorisation inegale d'elements de la biodiversite, normalisation et appauvrissement des savoir-faire, remise en cause des decoupages territoriaux actuels et des organisations sociales et administratives correspondantes. ********** Discussions at international meetings on biodiversity protection over the past decade have repeatedly emphasized 'knowledge, innovations and practices of indigenous and local communities'. (1) It is of course difficult, perhaps illusory, to define such complex and controversial notions in a few simple terms. But once past this obstacle, the fundamental aim is to find new pathways for conservation by strengthening the ties between human societies and their natural environments. Another aim is to find new ways to enhance the value of biodiversity and to share the benefits that can be drawn from it. Protection of ecosystems, species, plant varieties and animal breeds goes hand in hand with recognition of the knowledge and know-how of the societies that use them. Initially tools of sustainable management, this knowledge and these practices become in turn a heritage that must be inventoried, maintained, protected and brought to fruition (Cormier-Salem and Roussel 2002). From this point of view, Ethiopia is an exceptionally rich example. This vast territory in the Horn of Africa offers remarkable biological diversity (2) alongside a wide range of human cultures, often at quite ancient sites. Well known for its diverse mountain ecosystems, Ethiopia is also a major world centre from which cultivated plants originated (Engels et al. 1991; Zemede Asfaw 1997). It is thus not surprising that Ethiopia has emerged as one of the major players in elaborating positions that are common to all of Africa in international bodies such as the Food and Agriculture Organization (FAO) or the Rio Convention. …

A l'usage, la notion de « peuples autochtones » apparait comme une categorie plus « globale » que locale : elle prend sens et acquiert une reconnaissance en reference aux instances et forums internationaux ou elle a ete introduite et est en permanence redefinie. Nee de preoccupations onusiennes de defense des droits de peuples minoritaires, la categorie connait un nouvel essor dans le cadre de la Convention sur la Diversite Biologique. La reference aux peuples autochtones et a leurs savoirs « traditionnels » en matiere de gestion de la biodiversite est desormais au' coeur de ses travaux. L'analyse porte tout d'abord sur le contexte d'emergence de la notion dans ce champ. Sont ensuite abordes les glissements de sens que provoque le passage d'une arene internationale a l'autre. Cette fluctuation terminologique renvoie aux contextes d'usage et d'instrumentalisation du concept qui participent a la construction d'une categorie dont l'operationnalite importe plus que la substance. On compare pour conclure l'usage de cette categorie avec celui d'ethnie qui, dans le contexte colonial africain, avait de facon significativement similaire servi a penser un « ordre » social et politique.

Variations in lipoprotein(a) [Lp(a)] levels were evaluated during the course of the nephrotic syndrome in 20 children (17 males, 3 females, aged 2-16 years), to evaluate the use of this parameter in the prognosis and monitoring of the disease. One patient was in relapse, 12 in remission, and 7 alternated between remission and relapse. Results were compared with those obtained in a control population of 100 age-matched children. Lp(a) was measured by a previously described immunonephelometric technique. Serum Lp(a) levels were increased in children with relapsing nephrotic syndrome compared with controls (median value of 419 mg/l vs. 86 mg/l). The median Lp(a) level in patients with nephrotic syndrome in remission was different from controls (270 mg/l under steroid therapy and 163 mg/l without steroid therapy), but remained within the reference range. Of the patients in relapse, 66% had Lp(a) levels above the generally accepted limit for cardiovascular risk of 300 mg/l, compared with 16% of controls, 44% of patients with nephrotic syndrome in remission under steroid therapy, and 18% of patients with nephrotic syndrome in remission without steroid therapy. In 2 patients, Lp(a) was measured repeatedly and was significantly higher during the acute phase of the disease (up to sixfold basal level). Changes in Lp(a) levels correlated with cholesterol levels, but the kinetics and the extent of variations were different. These data suggest that measurement of Lp(a) is useful for monitoring the nephrotic syndrome in children, particularly for detecting complications.

Roussel Bernard. Friches et jachères : questions d'actualité, problèmes de toujours. In: Journal d'agriculture traditionnelle et de botanique appliquée, 38ᵉ année, bulletin n°1,1996. "Biodiversité, friches et jachères" sous la direction de Bernard Roussel, Claude Sastre et Paul Arnould. pp. 7-18.

Murine monoclonal antibodies (mAbs) to human β 2-glycoprotein I (β 2GPI), a plasma protein required for the binding of some antiphospholipid antibodies, have been shown to possess lupus anticoagulant properties and to activate platelets via Fcγ receptor (FcγR) crosslinking. Here we investigated their ability to induce polymorphonuclear leukocyte (PMN) functional responses. The six mAbs (IgGl isotype) tested in combination with β 2GPI led to a concentration-dependent activation of human PMNs as appreciated by granule release, H2O2 production, and cytosolic Ca2+ increase. This activation process was accompanied by the enhancement of PMN-mediated heparan sulfate loss from the endothelial cell line EA.hy 926 without evidence for cell lysis or detachment. F(ab’)2 fragments of one of the mAbs bound to PMNs in a β 2GPI-dependent manner but were devoid of activating effects. Carbamylated β 2GPI was unable to mediate PMN-antibody binding and subsequent activation. In addition, cationization of β 2GPI or removal of its sialic acid groups led to higher efficiency in binding to the PMN surface and triggering activation in comparison with the untreated protein. Thus, the process of PMN activation depends on mAb binding to these cells through both Fab (via β 2GPI) and Fc domains, as confirmed by the suppression of all responses upon treatment with an anti-FcγRII, but not anti-FcγRIII, antibody. Our data suggest a model of cellular activation by β 2GPI-dependent antiphospholipid antibodies. J. Leukoc. Biol. 57: 387–394; 1995.

Diffuse oesophageal leiomyomatosis (DL), an inherited smooth muscle proliferation process, has been reported to be associated with Alport syndrome (AS), a familial nephropathy, mainly dominant X-linked inherited, and characterized by ultrastructural changes of the glomerular basement membrane. The COL4A5 gene, encoding the alpha 5 chain of type IV collagen, has been identified as the site of mutations in families with X-linked AS. Recently, a novel alpha 6(IV) collagen chain encoding gene has been mapped closely upstream of COL4A5, and disruption of the 5' end of both genes has been reported in four patients with DL and AS (DL-AS). Here, we report a long-range restriction map around the COL4A6 locus, and show that the COL4A5/COL4A6 deletion observed in seven patients with DL-AS encompasses only the two first exons of COL4A6, with a breakpoint located in the second intron of COL4A6, whose size exceeds 65 kb. Furthermore, we demonstrate that three patients with AS without DL, known to have a deletion of the 5' part of the COL4A5 gene, display a larger deletion in COL4A6. Moreover, a COL4A6 mRNA product was detected by reverse-transcription-polymerase chain reaction in an oesophageal tumour sample of a patient with DL-AS. These results suggest that DL-AS could be caused by an abnormal truncated alpha 6(IV) chain.

Fetal and neonatal toxic effects of angiotensin II receptor antagonists have been described in animals and humans. Five cases of fetal or neonatal deaths have been reported following maternal use of sartans for hypertension. We report a case of neonatal transient renal failure following telmisartan therapy during pregnancy. This class of antihypertensive drugs should be avoided during pregnancy and breastfeeding.