Nécroses digitales du membre supérieur (à propos de 278 observations)

LILLE2-BU Santé-Recherche (593502101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Maladies orphelines : évaluation et enregistrement des nouvelles thérapeutiques

Les nouveaux traitements des maladies orphelines se doivent d’être évalués en respectant les principes de base de la méthodologie des essais thérapeutiques : essais contrôlés et randomisés, permettant, sur un critère d’évaluation unique, de mettre en évidence une différence statistiquement significative et cliniquement signifiante. Les contraintes liées aux maladies orphelines peuvent entraîner des entorses aux règles habituelles de la méthodologie mais ne doivent pas remettre en question le niveau de preuve de l’efficacité thérapeutique

Pregnancy may be followed by an inflexion of the immune reconstitution in HIV-infected women who receive antiretroviral drugs before conception

Background Whether pregnancy has an impact on the evolution of CD4 cell counts in women treated with highly potent antiretrovirals before conception remains largely unknown. Methods Among patients enrolled in the ANRS CO8 (APROCO/COPILOTE) cohort, we selected all women aged between 18 and 50 years at initiation of combination antiretroviral therapy (cART). Slopes of CD4 cell counts during follow-up were estimated using mixed longitudinal models with time-dependent indicators for pregnancy and delivery. Results Of the 260 selected HIV-infected women, a pregnancy occurred in 39 women in a median follow-up time of 66 months. Women who became pregnant had higher CD4 cell count at baseline but this difference progressively lessened during follow-up because they had a slower increase than women who did not become pregnant. The estimated slope of CD4 cell count decreased significantly from +2.3 cells/mu L/month before pregnancy and in women who did not become pregnant to -0.04 cells/mu L/month after delivery (P=0.0003). Conclusion A significant increase in CD4 cell count may be preferable before pregnancy in women treated with cART, in order to overcome the evolution observed after pregnancy

New Short Tandem Repeat-Based Molecular Typing Method for Pneumocystis jirovecii Reveals Intrahospital Transmission between Patients from Different Wards.

Pneumocystis pneumonia is a severe opportunistic infection in immunocompromised patients caused by the unusual fungus Pneumocystis jirovecii. Transmission is airborne, with both immunocompromised and immunocompetent individuals acting as a reservoir for the fungus. Numerous reports of outbreaks in renal transplant units demonstrate the need for valid genotyping methods to detect transmission of a given genotype. Here, we developed a short tandem repeat (STR)-based molecular typing method for P. jirovecii. We analyzed the P. jirovecii genome and selected six genomic STR markers located on different contigs of the genome. We then tested these markers in 106 P. jirovecii PCR-positive respiratory samples collected between October 2010 and November 2013 from 91 patients with various underlying medical conditions. Unique (one allele per marker) and multiple (more than one allele per marker) genotypes were observed in 34 (32%) and 72 (68%) samples, respectively. A genotype could be assigned to 55 samples (54 patients) and 61 different genotypes were identified in total with a discriminatory power of 0.992. Analysis of the allelic distribution of the six markers and minimum spanning tree analysis of the 61 genotypes identified a specific genotype (Gt21) in our hospital, which may have been transmitted between 10 patients including six renal transplant recipients. Our STR-based molecular typing method is a quick, cheap and reliable approach to genotype Pneumocystis jirovecii in hospital settings and is sensitive enough to detect minor genotypes, thus enabling the study of the transmission and pathophysiology of Pneumocystis pneumonia

Association of hidradenitis suppurativa and familial Mediterranean fever: A case series of 6 patients

International audienceObjectives: Familial mediterranean fever (FMF) is the most common monogenic autoinflammatory disease. Hidradenitis suppurativa (HS) is an inflammatory cutaneous disease. Those diseases can occur simultaneously among the same individual. Our objective was to describe the features of patients displaying both FMF and HS.Methods: We screened the French adult FMF reference center for FMF patients with HS.Results: Six patients out of 151 (4%) with a median age of 36 years old were concerned. Among them, FMF was symptomatic at a median age of 11.5years old and colchicine was introduced at a median age of 20.5years old. HS was diagnosed at a median age of 31.5years old. An elderly patient displayed AA amyloidosis in the outcome of FMF, with a late diagnosis of HS, with response to anakinra. There was no temporal relation between FMF and HS attacks. Some patients had a persistent inflammatory syndrome under treatment.Conclusion: FMF and HS are both inflammatory diseases involving young patients, with HS possibly being an autoinflammatory disease. Although their association seems to be fortuitous, both can induce an important inflammation state that could lead to AA amyloidosis and require a close monitoring of clinical signs and acute-phase reactants. Anakinra was successful in treating the only patient with both HS, FMF and amyloidosis

Transmission map of the 10 patients in whom the <i>P</i>. <i>jirovecii</i> genotype 21 was detected.

<p>The date corresponds to the time at which the patient was present in the hospital and is delineated as a circle. Colored circles show <i>P</i>. <i>jirovecii</i> exposure of a given patient on a given day. Color coding distinguishes the different groups of patients (purple for patients 01–03, blue for patients 04–06 and turquoise for patients 07–10). The red bar corresponds to transmission between two groups of patients. Colored bars show the transmission routes.</p

Summary of genotype categories for <i>P</i>. <i>jirovecii</i> samples.

<p>Summary of genotype categories for <i>P</i>. <i>jirovecii</i> samples.</p

Distribution of the different alleles in the 106 samples tested for the intronic (A), exonic (B) and intergenic (C) markers.

<p>Distribution of the different alleles in the 106 samples tested for the intronic (A), exonic (B) and intergenic (C) markers.</p

Characteristics of the STR markers and primers used in this study.

<p>Characteristics of the STR markers and primers used in this study.</p

Minimum spanning tree analysis of 61 genotypes from 55 samples harboring a unique genotype (one allele per marker) or multiple genotypes (multiple alleles in one marker).

<p>The number of allelic mismatches among STR profiles was used as distance. Each circle corresponds to one genotype (Gt), with its arbitrary number indicated next to it. The size of the circle is correlated with the number of isolates possessing the corresponding Gt, from one (smallest circle) to nine (Gt21). Dark, dashed and thin connecting bars corresponds to one, 2 or >2 different markers observed between linked genotypes. Gray zones surrounding some groups of circles indicate that these profiles belong to the same genetic cluster, meaning that they have a single allelic mismatch with at least one other member of the group. Cluster 2, which was significantly associated with renal transplant recipients, is shown by a dashed line. The color of the circles indicates the underlying disease of the patient in whom this specific genotype was recovered (Green, HIV patient; Red, hematology patient; Purple, renal transplant recipient; Yellow, other cause of immunosuppression).</p