Erythropoietic protoporphyria without skin symptoms-you do not always see what they feel

Erythropoietic protoporphyria (EPP) is an inherited disorder of the porphyrin metabolism that often remains undiagnosed in children. We report on a 4-year-old girl who had been suffering for 1 year from recurrent painful crises affecting her hands, feet, and nose following sun exposure. Objective skin lesions were absent until the age of 6. Porphyrin analysis revealed elevated free erythrocyte protoporphyrin (FEP) levels confirming the diagnosis of EPP. This illustrates that skin lesions might be completely absent in children affected with EPP, a fact that has only been reported once previously. Because EPP can manifest with few and unspecific cutaneous symptoms or no skin lesions at all, like in this patient, the diagnosis of EPP might be delayed or missed. EPP should be excluded in all photosensitive children, especially when discomfort is disproportionate to the extent of the cutaneous lesions. The clinic, pathophysiology, diagnosis, complications, and therapy of EPP are discussed

Patient education after amputation: Systematic review and experts’ opinions

AbstractObjectiveTo perform a systematic review of the literature regarding amputee self-care, and analyze current experts’ opinions.MethodThe research in Medline and Cochrane Library databases was performed using the keywords “amputee self-care”, “amputee health care”, “amputee education”, and “amputee health management”. The methodological quality of the articles was assessed using four levels of evidence and three guideline grades (A: strong; B: moderate; C: poor).ResultOne prospective randomized controlled study confirm the level of evidence of self-care amputee persons with grade B, which is similar others chronic diseases self-care. Self-care of amputee persons contributes to improve functional status, depressive syndrome, and also health-related quality of life. A review of the patients’ needs and expectations in self-care amputee persons has been established thanks to the presence of qualitative focus group study.ConclusionA multidisciplinary self-care of amputee persons can be recommended. Regarding literature date, the level of evidence of self-care amputee persons is moderate (grade B). Experts groups are currently working on a self-care amputee persons guideline book in order to standardize practicing and programs in the physical medicine and rehabilitation departments

Isolation of a Repeated DNA Probe Showing Polymorphism among Meloidogyne incognita Populations

Several Meloidogyne incognita geographic populations were characterized by analysis of the restriction fragment length polymorphisms (RFLP) obtained after digestion of their total DNA and hybridization with a [³²P]-labeled probe. The probe consisted of a 1.7-kb-repeated DNA sequence, isolated from a M. incognita genomic library, that hybridized to multiple BamH I fragments in the genome of each isolate. The patterns showed sufficient polymorphism to enable the accurate differentiation of all the populations tested

Vers une production en temps réel d'intervalles prédictifs associés aux prévisions de crue dans Vigicrues en France

International audienceTowards the real-time production of predictive intervals around streamflow forecasts in Vigicrues in France. Although quantifying uncertainties in modelling is an old topic,many developments have been made over the past fifteen years in the context of flood forecasting, mainly with the development of probabilistic approaches. In France, within the national network for floodforecasting (Vigicrues), developments have been made to propose a post-processing methodology to quantify predictive uncertainty (OTAMIN), which automatically generates confidence intervals around stream flow forecasts, and a tool that is used by forecasters to expertise these confidence intervals (EAO/EXPRESSO). This paper presents the main principles of this general approach, gives an example of its recent application in operational conditions and discusses its usefulness for the users and the general public. Some perspectives of improvement are discussed.La quantification des incertitudes en modélisation est un sujet ancien, mais de nombreux développements ont été réalisés au cours des quinze dernières années dans un contexte de prévision des crues, avec l'essor notamment des approches probabilistes. En France, au sein du réseau national de prévision des crues (Vigicrues), cela a conduit à la mise au point d'une méthode automatisée de quantification des incertitudes prédictives (OTAMIN) rentrant dans la catégorie des approches de \post- traitement, qui génère des intervalles de confiance sur les prévisions de débit, et d'un outil d'expertise par les prévisionnistes de ces intervalles (EAO/EXPRESSO). Cet article présente les principes méthodologiques de cette démarche, en donne une illustration d'application opérationnelle récente et discute de son utilité pour des utilisateurs institutionnels et pour le grand public. Quelques perspectives d'amélioration sont avancées