Showing papers by "University of Rouen published in 2010"

Plant-specific glycosylation patterns in the context of therapeutic protein production.

Abstract: While N-glycan synthesis in the endoplasmic reticulum (ER) is relatively well conserved in eukaryotes, N-glycan processing and O-glycan biosynthesis in the Golgi apparatus are kingdom specific and result in different oligosaccharide structures attached to glycoproteins in plants and mammals. With the prospect of using plants as alternative hosts to mammalian cell lines for the production of therapeutic glycoproteins, significant progress has been made towards the humanization of protein N-glycosylation in plant cells. To date, successful efforts in this direction have mainly focused on the targeted expression of therapeutic proteins, the knockout of plant-specific N-glycan-processing genes, and/or the introduction of the enzymatic machinery catalyzing the synthesis, transport and addition of human sugars. By contrast, very little attention has been paid until now to the O-glycosylation status of plant-made therapeutic proteins, which is surprising considering that hundreds of human proteins represent good candidates for Hyp-O glycosylation when produced in a plant expression system. This review describes protein N- and O-linked glycosylation in plants and highlights the limitations and advantages of plant-specific glycosylation on plant-made biopharmaceuticals.

Macroecological patterns in soil communities

Abstract: Aim To review published evidence regarding the factors that influence the geographic variation in diversity of soil organisms at different spatial scales. Location Global. Methods A search of the relevant literature was conducted using the Web of Science and the author's personal scientific database as the major sources. Special attention was paid to include seminal studies, highly cited papers and/or studies highlighting novel results. Results Despite their significant contribution to global biodiversity, our taxonomic knowledge of soil biota is still poor compared with that of most above-ground organisms. This is particularly evident for small-bodied taxa. Global patterns of soil biodiversity distribution have been poorly documented and are thought to differ significantly from what is reported above-ground. Based on existing data, it appears that microorganisms do not respond to large-scale environmental gradients in the same way as metazoans. Whereas soil microflora seem to be mainly represented by cosmopolitan species, soil animals respond to altitudinal, latitudinal or area gradients in the same way as described for above-ground organisms. At local scales, there is less evidence that local factors regulate above- and below-ground communities in the same way. Except for a few taxa, the humpbacked response to stress and disturbance gradients doesn't seem to apply underground. Soil communities thus appear weakly structured by competition, although competitive constraints may account for assembly rules within specific taxa. The main factor constraining local soil biodiversity is the compact and heterogeneous nature of soils, which provides unrivalled potential for niche partitioning, thus allowing high levels of local biodiversity. This heterogeneity is increased by the impact of ecosystem engineers that generate resource patchiness at a range of spatio-temporal scales.

What are the best outcome measures for assessing plaque psoriasis severity? A systematic review of the literature.

Abstract: Background A wide variety of scoring systems have been proposed to assess severity of psoriasis. Given its importance as a health issue both for patients and health care systems, it is critically important to evaluate the validity and reliability of existing outcome measures. Objective The objective of this systematic review was to assess the extent of validation including the validity, reliability, sensitivity to change and ease of use of available outcome measures for psoriasis. Materials and Methods We conducted a systematic review of all clinical studies (prospective and retrospective) investigating the severity of psoriasis patients and published between January 1980 and June 2009. The following methodological validation and quality criteria were recorded systematically: construct validity, content validity and internal consistency, intra-observer variation and inter-observer variation, sensitivity to change and acceptability/ease of use assessed as time required to perform measurement. Results Based on methodological validation and quality criteria, six clinical severity scores were selected and analysed (PASI, BSA, PGA, LS-PGA, SPI and SAPASI scores). We did not find substantial evidence of construct validity for any of the psoriasis clinical severity scores. Content validity was studied by considering the PASI score as gold standard. The relative content validity was good for the LS-PGA, PGA, and SPI scores, which correlated strongly with the PASI score. The SAPASI and PASI scores showed moderate correlation. Internal consistency was good for the PASI and LS-PGA scores. The PASI, BSA, PGA and LS-PGA scores displayed limited intra-observer variation. The inter-observer variation was low for LS-PGA (ICC 0.8). The PASI score and the SAPASI displayed moderate sensitivity to change. Discussion Based on this systematic review, it appears that none of the severity scores used for psoriasis meets all of the validation criteria required for an ideal score. However, we can conclude that the PASI score is the most extensively studied psoriasis clinical severity score and the most thoroughly validated according to methodological validation criteria. Despite certain limitations, use of the PASI score can be recommended for scientific evaluation of the clinical severity of psoriasis.

Combined effects of long-living chemical species during microbial inactivation using atmospheric plasma-treated water.

Abstract: Electrical discharges in humid air at atmospheric pressure (nonthermal quenched plasma) generate long-lived chemical species in water that are efficient for microbial decontamination. The major role of nitrites was evidenced together with a synergistic effect of nitrates and H 2 O 2 and matching acidification. Other possible active compounds are considered, e.g., peroxynitrous acid.

IL-10 produced by activated human B cells regulates CD4(+) T-cell activation in vitro.

Abstract: IL-10-producing regulatory B cells have been identified in mice and shown to downregulate inflammation, making them potentially important for maintenance of tolerance. In this study, we isolated B cells from human blood and spleen, and showed that after a short period of ex vivo stimulation a number of these cells produced IL-10. The IL-10-producing B cells did not fall within a single clearly defined subpopulation, but were enriched in both the memory (CD27(+)) and the transitional (CD38(high)) B-cell compartments. Combined CpG-B+anti-Ig stimulation was the most potent IL-10 stimulus tested. B cells stimulated in this way inhibited CD4(+)CD25(-) T-cell proliferation in vitro by a partially IL-10-dependent mechanism. These findings imply that manipulating IL-10 production by human B cells could be a useful therapeutic strategy for modulating immune responses in humans.

The World Federation of Societies of Biological Psychiatry (WFSBP) guidelines for the biological treatment of paraphilias.

Abstract: Objectives. The primary aim of these guidelines was to evaluate the role of pharmacological agents in the treatment and management of paraphilia, with a focus on the treatment of adults males. Because such treatments are not delivered in isolation, the role of specific psychosocial and psychotherapeutic interventions was also briefly covered. These guidelines are intended for use in clinical practice by clinicians who diagnose and treat patients with paraphilia. The aim of these guidelines is to improve the quality of care and to aid physicians in clinical decisions. Methods. The aim of these guidelines was to bring together different views on the appropriate treatment of paraphilias from experts representing different continents. To achieve this aim, an extensive literature search was conducted using the English language literature indexed on MEDLINE/PubMed (1990–2009 for SSRIs) (1969–2009 for antiandrogen treatments), supplemented by other sources, including published reviews. Results. Each trea...

Constitutive β-catenin activation induces adrenal hyperplasia and promotes adrenal cancer development

Abstract: Adrenocortical carcinoma is a rare but aggressive cancer with unknown aetiology. Constitutive activation of beta-catenin is the most frequent alteration in benign and malignant adrenocortical tumours in patients. Here, we show that constitutive activation of beta-catenin in the adrenal cortex of transgenic mice resulted in progressive steroidogenic and undifferentiated spindle-shaped cells hyperplasia as well as dysplasia of the cortex and medulla. Over a 17 months time course, transgenic adrenals developed malignant characteristics such as uncontrolled neovascularization and loco-regional metastatic invasion. These oncogenic events were accompanied by ectopic differentiation of glomerulosa at the expense of fasciculata cells, which caused primary hyperaldosteronism. Altogether these observations demonstrate that constitutively active beta-catenin is an adrenal oncogene which triggers benign aldosterone-secreting tumour development and promotes malignancy.

Discovery and evolutionary history of gonadotrophin-inhibitory hormone and kisspeptin: new key neuropeptides controlling reproduction.

Abstract: Gonadotrophin-releasing hormone (GnRH) is the primary hypothalamic factor responsible for the control of gonadotrophin secretion in vertebrates. However, within the last decade, two other hypothalamic neuropeptides have been found to play key roles in the control of reproductive functions: gonadotrophin-inhibitory hormone (GnIH) and kisspeptin. In 2000, we discovered GnIH in the quail hypothalamus. GnIH inhibits gonadotrophin synthesis and release in birds through actions on GnRH neurones and gonadotrophs, mediated via GPR147. Subsequently, GnIH orthologues were identified in other vertebrate species from fish to humans. As in birds, mammalian and fish GnIH orthologues inhibit gonadotrophin release, indicating a conserved role for this neuropeptide in the control of the hypothalamic-pituitary-gonadal axis across species. Subsequent to the discovery of GnIH, kisspeptin, encoded by the KiSS-1 gene, was discovered in mammals. By contrast to GnIH, kisspeptin has a direct stimulatory effect on GnRH neurones via GPR54. GPR54 is also expressed in pituitary cells, but whether gonadotrophs are targets for kisspeptin remains unresolved. The KiSS-1 gene is also highly conserved and has been identified in mammals, amphibians and fish. We have recently found a second isoform of KiSS-1, designated KiSS-2, in several vertebrates, but not birds, rodents or primates. In this review, we highlight the discovery, mechanisms of action, and functional significance of these two chief regulators of the reproductive axis.

Metallic composites processed via extreme deformation: Toward the limits of strength in bulk materials

Abstract: We review microstructures and properties of metal matrix composites produced by severe plastic deformation of multiphase alloys. Typical processings are wire drawing, ball milling, roll bonding, equal-channel angular extrusion, and high-pressure torsion of multiphase materials. Similar phenomena occur between solids in frictional contact such as in tribology, friction stir welding, and explosive joining. The resulting compounds are characterized by very high interface and dislocation density, chemical mixing, and atomic-scale structural transitions at heterointerfaces. Upon straining, the phases form into nanoscaled filaments. This leads to enormous strengthening combined with good ductility, as in damascene steels or pearlitic wires, which are among the strongest nanostructured bulk materials available today (tensile strength above 6 GPa). Similar materials are Cu-Nb and Cu-Ag composites, which also have good electrical conductivity that qualifies them for use in high-field magnets. Beyond the engineering opportunities, there are also exciting fundamental questions. They relate to the nature of the complex dislocation, amorphization, and mechanical alloying mechanisms upon straining and their relationship to the enormous strength. Studying these mechanisms is enabled by mature atomic-scale characterization and simulation methods. A better understanding of the extreme strength in these materials also provides insight into modern alloy design based on complex solid solution phenomena.

Guidelines in the management of obstructing cancer of the left colon: consensus conference of the world society of emergency surgery (WSES) and peritoneum and surgery (PnS) society

Abstract: Obstructive left colon carcinoma (OLCC) is a challenging matter in terms of obstruction release as well of oncological issues. Several options are available and no guidelines are established. The paper aims to generate evidenced based recommendations on management of OLCC. The PubMed and Cochrane Library databases were queried for publications focusing on OLCC published prior to April 2010. A extensive retrieval, analyses, and grading of the literature was undertaken. The findings of the research were presented and largely discussed among panellist and audience at the Consensus Conference of the World Society of Emergency Surgery (WSES) and Peritoneum and Surgery (PnS) Society held in Bologna July 2010. Comparisons of techniques are presented and final committee recommendation are enounced. Hartmann's procedure should be preferred to loop colostomy (Grade 2B). Hartmann's procedure offers no survival benefit compared to segmental colonic resection with primary anastomosis (Grade 2C+); Hartmann's procedure should be considered in patients with high surgical risk (Grade 2C). Total colectomy and segmental colectomy with intraoperative colonic irrigation are associated with same mortality/morbidity, however total colectomy is associated with higher rates impaired bowel function (Grade 1A). Segmental resection and primary anastomosis either with manual decompression or intraoperative colonic irrigation are associated with same mortality/morbidity rate (Grade 1A). In palliation stent placement is associated with similar mortality/morbidity rates and shorter hospital stay (Grade 2B). Stents as a bridge to surgery seems associated with lower mortality rate, shorter hospital stay, and a lower colostomy formation rate (Grade 1B). Loop colostomy and staged procedure should be adopted in case of dramatic scenario, when neoadjuvant therapy could be expected. Hartmann's procedure should be performed in case of high risk of anastomotic dehiscence. Subtotal and total colectomy should be attempted when cecal perforation or in case of synchronous colonic neoplasm. Primary resection and anastomosis with manual decompression seems the procedure of choice. Colonic stents represent the best option when skills are available. The literature power is relatively poor and the existing RCT are often not sufficiently robust in design thus, among 6 possible treatment modalities, only 2 reached the Grade A.

Predictors of cerebral palsy in very preterm infants: the EPIPAGE prospective population-based cohort study.

Abstract: Aim The aim of this study was to assess the independent role of cerebral lesions on ultrasound scan, and several other neonatal and obstetric factors, as potential predictors of cerebral palsy (CP) in a large population-based cohort of very preterm infants. Method As part of EPIPAGE, a population-based prospective cohort study, perinatal data and outcome at 5 years of age were recorded for 1812 infants born before 33 weeks of gestation in nine regions of France in 1997. Results The study group comprised 942 males (52%) and 870 females with a mean gestational age of 30 weeks (SD 2wks; range 24–32wks) and a mean birthweight of 1367g (SD 393g; range 450–2645g). CP was diagnosed at 5 years of age in 159 infants (prevalence 9%; 95% confidence interval [CI] 7–10%), 97 males and 62 females, with a mean gestational age of 29 weeks (SD 2wks; range 24–32wks) and a mean birthweight of 1305g (SD 386g; range 500–2480g). Among this group, 67% walked without aid, 14% walked with aid, and 19% were unable to walk. Spastic, ataxic, and dyskinetic CP accounted for 89%, 7%, and 4% of cases respectively. The prevalence of CP was 61% among infants with cystic periventricular leukomalacia, 50% in infants with intraparenchymal haemorrhage, 8% in infants with grade I intraventricular haemorrhage, and 4% in infants without a detectable cerebral lesion. After controlling for cerebral lesions and obstetric and neonatal factors, only male sex (odds ratio [OR] 1.52; 95% CI 1.03–2.25) and preterm premature rupture of membranes or preterm labour (OR 1.72; 95% CI 0.95–3.14) were predictors of the development of CP in very preterm infants. Interpretation Cerebral lesions were the most important predictor of CP in very preterm infants. In addition, infant sex and preterm premature rupture of membranes or preterm labour were also independent predictors of CP.

DNA Barcoding Reveals Cryptic Diversity in Lumbricus terrestris L., 1758 (Clitellata): Resurrection of L. herculeus (Savigny, 1826)

Abstract: The widely studied and invasive earthworm, Lumbricus terrestris L., 1758 has been the subject of nomenclatural debate for many years. However these disputes were not based on suspicions of heterogeneity, but rather on the descriptions and nomenclatural acts associated with the species name. Large numbers of DNA barcode sequences of the cytochrome oxidase I obtained for nominal L. terrestris and six congeneric species reveal that there are two distinct lineages within nominal L. terrestris. One of those lineages contains the Swedish population from which the name-bearing specimen of L. terrestris was obtained. The other contains the population from which the syntype series of Enterion herculeum Savigny, 1826 was collected. In both cases modern and old representatives yielded barcode sequences allowing us to clearly establish that these are two distinct species, as different from one another as any other pair of congeners in our data set. The two are morphologically indistinguishable, except by overlapping size-related characters. We have designated a new neotype for L. terrestris. The newly designated neotype and a syntype of L. herculeus yielded DNA adequate for sequencing part of the cytochrome oxidase I gene (COI). The sequence data make possible the objective determination of the identities of earthworms morphologically identical to L. terrestris and L. herculeus, regardless of body size and segment number. Past work on nominal L. terrestris could have been on either or both species, although L. herculeus has yet to be found outside of Europe.

Contrasted effect of biochar and earthworms on rice growth and resource allocation in different soils

Abstract: Adding biochar to soils and maintaining high earthworm biomasses are potential ways to increase the fertility of tropical soils and the sustainability of crop production in the spirit of agroecology and ecological engineering. However, a thorough functional assessment of biochar effect on plant growth and resource allocations is so far missing. Moreover, earthworms and biochar increase mineral nutrient availability through an increase in mineralization and nutrient retention respectively and are likely to interact through various other mechanisms. They could thus increase plant growth synergistically. This hypothesis was tested for rice in a greenhouse experiment. Besides, the relative effects of biochar and earthworms were compared in three different soil treatments (a nutrient rich soil, a nutrient poor soil, a nutrient poor soil supplemented with fertilization). Biochar and earthworm effects on rice growth and resource allocation highly depended on soil type and were generally additive (no synergy). In the rich soil, there were both clear positive biochar and earthworm effects, while there were generally only positive earthworm effects in the poor soil, and neither earthworm nor biochar effect in the poor soil with fertilization. The analysis of earthworm and biochar effects on different plant traits and soil mineral nitrogen content, confirmed that they act through an increase in nutrient availability. However it also suggested that another mechanism, such as the release in the soil of molecules recognized as phytohormones by plants, is also involved in earthworm action. This mechanism could for example help explaining how earthworms increase rice resource allocation to roots and influence the allocation to grains.

Blastic plasmacytoid dendritic cell neoplasm: is transplantation the treatment of choice?

Abstract: Background Blastic plasmacytoid dendritic cell neoplasm (BPDCN) represents the malignant counterpart derived from plasmacytoid dendritic cells. This rare entity is usually revealed and diagnosed on cutaneous lesions associated or not with a leukaemic component. The prognosis associated with BPDCN is very poor. Objectives To perform a retrospective review of BPDCN cases registered in the French Study Group on Cutaneous Lymphoma database from June 1995 to May 2008. Methods Forty-seven patients were included. Demographic data, initial staging, therapeutic management and outcome were recorded. Results The mean survival was 16.7 months (95% confidence interval 12.6-20.8). Only eight (17%) and one (2%) patients reached respectively 2 and 5 years of survival. Initial spreading of the disease did not represent, in this cohort, a reliable prognosis factor. The outcome was overall influenced by treatment provided. While radiation therapy, monochemotherapy or even polychemotherapy regimens did not significantly affect the course of the disease, the survival of bone marrow transplanted patients was significantly higher. Conclusions Despite the fact that BPDCN is often initially limited to the skin, only an aggressive initial therapy may improve the patients' prognosis. Local treatments, such radiation therapy, are definitively useless. Regardless of the initial extension of the disease, in our experience only bone marrow transplantation significantly improved the outcome.

Regularizing Effect and Local Existence for the Non-Cutoff Boltzmann Equation

Abstract: The Boltzmann equation without Grad’s angular cutoff assumption is believed to have a regularizing effect on the solutions because of the non-integrable angular singularity of the cross-section. However, even though this has been justified satisfactorily for the spatially homogeneous Boltzmann equation, it is still basically unsolved for the spatially inhomogeneous Boltzmann equation. In this paper, by sharpening the coercivity and upper bound estimates for the collision operator, establishing the hypo-ellipticity of the Boltzmann operator based on a generalized version of the uncertainty principle, and analyzing the commutators between the collision operator and some weighted pseudo-differential operators, we prove the regularizing effect in all (time, space and velocity) variables on the solutions when some mild regularity is imposed on these solutions. For completeness, we also show that when the initial data has this mild regularity and a Maxwellian type decay in the velocity variable, there exists a unique local solution with the same regularity, so that this solution acquires the C∞ regularity for any positive time.

Temperature-responsive polymer brushes switching from bactericidal to cell-repellent.

Abstract: Materials exhibiting antibacterial properties at room temperature and turning biocompatible and non-adhesive for in vivo conditions, are extremely attractive for devices that have to be ultimately introduced in living beings. Indeed, infections related to the use of invasive biomedical and medical items are still one of the main medical complications that cause high rates of mortality. [ 1 ] Despite sanitation protocols, a well-identifi ed route for patient bacterial infection is transmission through contaminated instruments such as intubation tubes, catheters, surgical drains or endoscopes that bypass the natural protective barriers of the body. [ 1 ]

Multilocus sequence typing for Clostridium difficile.

Abstract: Multilocus sequence typing (MLST), a nucleotide sequence-based characterization of allelic polymorphism of housekeeping genes, has been proposed as a new approach for population and evolutionary genetics and global epidemiology of bacterial pathogens. MLST provides unambiguous sequence data that can be generated from various laboratories and should be shared in a common web database. Here are presented most of materials, methods, and programs or software necessary to perform MLST on Clostridium difficile.We also describe an example of an MLST scheme for C. difficile based on sequence analysis of six housekeeping gene loci and use a set of 74 C. difficile isolates from various hosts, geographic sources, and PCR-toxigenic types (A+B+, A-B+, and A-B-). Thirty-two "sequence types" (ST) are defined from the combination of allelic data, which correlate well with toxigenic types. The estimation of linkage disequilibrium between loci reveals a clonal population structure. Mutational evolution of C. difficile is characterized, with point mutation generating new alleles at a frequency eightfold higher than recombinational exchange. Phylogenetic analysis shows that human and animal isolates do not cluster in distinct lineages, and that no hypervirulent lineage can be characterized within the population of toxigenic human isolates studied (strains from pseudomembranous colitis and antibiotic-associated diarrhea do not cluster in distinct lineages). However, all A-B+ variant isolates belong to a divergent but very homogeneous lineage in the population studied.An MLST database specific for this species is now hosted at the web site of the Institut Pasteur Paris. Since MLST data reflect evolutionary genetics of the species, they could be used as typing markers, possibly in combination with virulence genes data, for long-term global epidemiology of C. difficile.

Bridging multiferroic phase transitions by epitaxial strain in BiFeO3.

Abstract: We report the influence of epitaxial strain on the multiferroic phase transitions of BiFeO3 films. Using advanced characterization techniques and calculations we show that while the magnetic Ne'el temperature hardly varies, the ferroelectric Curie temperature TC decreases dramatically with strain. This is in contrast with the behavior of standard ferroelectrics where strain enhances the polar cation shifts and thus TC. We argue that this is caused by an interplay of polar and oxygen tilting instabilities and that strain can drive both transitions close together to yield increased magnetoelectric responses.

Assessment of risk of psoriatic arthritis in patients with plaque psoriasis: a systematic review of the literature.

Abstract: Objective The aim of this study was to determine the prevalence of psoriatic arthritis (PsA) in the patients with plaque psoriasis and to give recommendation for the diagnosis of PsA for dermatologists. Methods A systematic search was performed in Pubmed, Cochrane and Embase databases. The key-words used from the Medical Searching Heading (MeSH) were: ‘Psoriasis’, ‘Arthritis, Psoriatic’, ‘Uveitis’ and ‘Dactylitis’. We selected cross-sectional epidemiological studies written in English or French between January 1980 and October 2009. Results The initial literature search identified 2171 references. Based on abstract reading and exclusion of studies not written in English or French, and without control group, the final selection included 20 epidemiological studies. With the eight studies using rheumatologically validated criteria, the prevalence of PsA among psoriasis patient spanned a wide range from 7% to 26%. Conclusion Psoriasis arthritis may affect up to 24% of psoriasis patients. Dermatologist should be aware of main clinical sign of PsA to promote earlier recognition and treatment of PsA.

Cardiovascular risk factors in patients with plaque psoriasis: a systematic review of epidemiological studies.

Abstract: Introduction Many epidemiological studies have associated psoriasis with an increased risk of coronary artery disease, resulting from a higher prevalence of cardiovascular risk factors in psoriasis patients compared with unmatched controls. However, the results of epidemiological studies vary depending upon the populations studied. The aim of this systemic review was to evaluate the risk of diabetes, hypertension, dyslipidaemia and obesity in adults with plaque psoriasis. In addition, we assessed the relationship between the risk of cardiovascular risk factors and psoriasis severity. Methods A systematic search was performed on Pubmed, Cochrane and Embase databases, using the key-words ‘psoriasis’, ‘diabetes’, ‘hypertension’, ‘high blood pressure’, ‘dyslipidaemia’, ‘metabolic syndrome’ and ‘obesity’, during the period from 1980 to June 2009. Results The initial literature search identified 353 articles. The final selection included 18 cross-sectional case-control studies. An increased risk of metabolic syndrome was observed in psoriatic patients (OR = 1.3–5.92), and a trend for a higher risk of obesity (OR = 1.18–5.49), especially in patients with severe psoriasis. For hypertension, hypertriglyceridaemia, and diabetes, the association was not significant in all studies. Discussion There was important heterogeneity in study design preventing from pooling results. Most often lifestyle factors such as smoking, alcohol consumption, physical activities were not taken into account. Conclusion There is an increased risk of obesity and metabolic syndrome in psoriasis. For hypertension, diabetes and dyslipidaemia no consistency was found across studies. Prospective epidemiological studies with thorough recording of cardiovascular risk factors are required in psoriasis patients.

What are the best outcome measures for assessing quality of life in plaque type psoriasis? A systematic review of the literature

Abstract: Background The assessment of health-related quality of life (QOL) is important in psoriasis. Despite this, among the wide variety of QOL questionnaires used in psoriasis, there is no consensus as to which is the best. Objective The objective of this systematic review was to identify which of these measurements have acceptable evaluation criteria for both monitoring disease and research purposes. We looked at validity, reliability, sensitivity to change (responsiveness) and acceptability, which are common criteria for any score and specific criteria for QOL questionnaire. Materials and Methods We performed a systematic review of all clinical studies investigating the QOL of psoriasis patients, published between January 1988 and June 2009. Results Twenty-one QOL questionnaires were identified. Eight of these satisfied most of the validation criteria; they were multidimensional and had been used in clinical research: short form 36 (SF 36), Dermatology Life Quality Index (DLQI), Skindex 29, Skindex 17, Dermatology Quality of life Scale (DQOLS), Psoriasis Disability Index (PDI) Impact of Psoriasis Questionnaire (IPSO) and Psoriasis Index of Quality of Life (PSORIQOL). Construct validity, content validity and internal consistency were adequately assessed and good performance was achieved for all questionnaires. Reproducibility (undetermined for Skindex 17) and acceptability were good for all questionnaires. Sensitivity to change was determined good for the SF36, DLQI, Skindex 29 and PDI. Item bias, unidimensional structure and cross-cultural equivalence were poor for all but the Skindex 17 and SF36 questionnaires. Conclusion On the basis of this systematic review, we can conclude that the DLQI is easy to use in clinical practice because of its brevity and simplicity. SF36 is widely used in clinical trials. Skindex 29 and Skindex 17, although rarely used, are interesting because of their cross-cultural validation.

Intravenous immunoglobulins for steroid-refractory esophageal involvement related to polymyositis and dermatomyositis: a series of 73 patients.

Abstract: Objective To assess the long-term outcome of esophageal complications in the group of patients receiving intravenous immunoglobulins (IVIG) for the treatment of severe steroid-refractory esophageal involvement related to polymyositis/dermatomyositis (PM/DM). Methods We retrospectively reviewed the medical records of 73 patients (39 with PM, 34 with DM) with steroid-resistant esophageal involvement. Esophageal involvement was evaluated by clinical and manometric investigations. Results Seventy-three patients with steroid-refractory esophageal involvement related to PM/DM received IVIG therapy (2 gm/kg monthly). The median interval between PM/DM diagnosis and the onset of esophageal complications was 6 months. The most common clinical manifestations revealing esophageal dysfunction were dysphagia (69.9%), coughing while eating (61.6%), and gastroesophageal reflux into the pharynx and/or mouth (34.2%). Twenty-five patients exhibited life-threatening esophageal complications requiring exclusive enteral feeding; 33 patients (45.2%) with esophageal impairment developed aspiration pneumonia. Sixty patients (82.2%) exhibited resolution of esophageal clinical manifestations, leading to a return to normal oral feeding and ablation of feeding enteral tubes. Four other patients (5.5%) improved, although they still experienced mild dysphagia intermittently. Because of impaired cricopharyngeal muscle relaxation, another patient successfully underwent cricopharyngeal myotomy. Eight patients died from aspiration pneumonia (n = 6) and cancer (n = 2). Muscle weakness, thoracic myopathy, and aspiration pneumonia were independent predictive factors of IVIG-treated esophageal complications in PM/DM patients. Conclusion Our findings indicate that IVIG should be considered in life-threatening esophageal impairment complicating steroid-resistant PM/DM. We also suggest that combined therapy of IVIG and high-dose steroids may be the first-line therapy in PM/DM patients with life-threatening esophageal manifestations.