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Background East and South East Asian subjects as well as Amerindians and Hispanic subjects are predominantly affected by Vogt-Koyanagi-Harada disease. In Europe, only few studies have described the clinical features and treatment of this disease, especially in France. Methods This retrospective case series was based on data collected from patients with a VKH disease diagnosed from January 2000 to March 2017, provided by three French Tertiary Centers. Results Forty-one patients (16 men and 25 women) were diagnosed: average age at diagnosis was 38.7 years. Patients were mainly from Maghreb (58%), but ethnic origins were multiple. Pleiocytosis was observed in 19 cases (63%) and 17 out of 41 patients showed audio vestibular signs (41%), and 11 showed skin signs (27%). Thirty-four were treated with corticosteroids (83%), 11 with an immunosuppressant treatment (27%) and 5 with biological therapy drugs (13%). Relapse was observed in 41% patients, even though final average visual acuity had improved. We did not find any significant clinical difference in the population from Maghreb compared to other populations, but for age and sex trends, since there was a majority of younger women. Conclusion We report here the second largest French cohort reported to date to our knowledge. The multiethnicity in our study suggests that VKH disease should be evoked whatever patients’ ethnicity.

International audience; Primary antibody deficiency (PAD) is characterized by a defective immunoglobulin production and recurrent infections, mostly involving respiratory and gastrointestinal tracts. Chronic or recurrent diarrhea is reported in up to 23%. Campylobacter infection is a common cause of infectious diarrhea, reported in 1.2% to 7.5% of patients with common variable immunodefi-ciency (CVID), the most frequent PAD. The aim of this study was to describe Campylobacter infection in patients with PAD included in a large nationwide study and analyze factors associ-ated with susceptibility to this pathogen. The DEFI (DEFicit Immunitaire) study is an ongoing large cross-sectional French multicentric study of adults with PAD, with retrospective collection of clinical data. All patients with a history of bacteriologically documented Campylobacter infection were identified, and clinical data were collected for each episode. Factors associated with recurrent infection were assessed as oddsratio (OR) and 95% confidence interval (CI), calculated by means of simple regression analysis. In patients with available material, strains of each episode were characterized using molecular analysis and compared (Table E1, available in this article’s Online Repository at www.jaci-inpractice.org). A com-parison of immunodeficiency-related characteristics of patients with and without Campylobacter infection was performed in the homogeneous group of patients with CVID. The control group included patients with CVID from DEFI centers who confirmed that patients did not develop Campylobacter infection after enrollment (Figure E1, available in this article’s Online Repository at www.jaci-inpractice.org). After correction for multiple comparisons, P

Resume Introduction La maladie de Gaucher (MG) est une maladie genetique lysosomale, due a un deficit d’activite de la beta-glucocerebrosidase, caracterisee par des atteintes hematologiques, viscerales et osseuses. L’objectif principal de notre etude etait de decrire le parcours diagnostique de patients atteints de MG de type 1 et le role du medecin interniste. Methodes Etude retrospective multicentrique incluant des patients atteints de MG de type 1, menee au sein de 16 centres, entre 2009 et 2011. Resultats Cinquante-cinq patients atteints d’une MG de type 1 ont ete inclus, suivis par un interniste ou un hematologue. Ils etaient initialement hospitalises dans 8 services de specialite differents. Le diagnostic a ete etabli par un myelogramme chez 22 patients (40 %), par un dosage enzymatique de la glucocerebrosidase chez 15 patients (27 %), par une biopsie medullaire chez 9 patients (16 %). Le recours au dosage enzymatique etait plus frequent apres 1990. Le delai entre la date de premiere hospitalisation pour symptomes en lien avec la MG et le diagnostic definitif etait inferieur a 1 an pour 38 patients (81 %). Les suspicions de MG etaient majoritairement adressees a un medecin interniste. Conclusion La maladie semble mieux reconnue et diagnostiquee plus rapidement depuis 1990 malgre la multiplicite des parcours, dans lesquels le role du medecin interniste parait important.

Gaucher disease (GD) is a rare genetic lysosomal storage disorder caused by a beta-glucocerebrosidase deficiency and responsible for a lysosomal storage disorder. GD is characterized by haematological, visceral and bone involvements. The aim of this study was to describe the diagnostic journey of type 1 GD patients as well as the role of the internist.A retrospective multicentric study involving type 1 GD patients has been conducted in 16 centers, between 2009 and 2011.Fifty-five type 1 GD patients were included, under the care of an internist or an haematologist. They were originally hospitalized in 8 different specialized units. Diagnosis was established by bone-marrow aspiration in 22 patients (40%), by enzymatic assay of glucocerebrosidase activity in 15 patients (27%), and by bone-marrow biopsy in 9 patients (16%). The use of enzymatic assay became more frequent after 1990. The delay between first hospitalization due to GD symptoms and definitive diagnosis was less than one year for 38 patients. Patients with suspected GD were mainly referred to an internist physician.GD seems to be better recognized and quickly diagnosed since 1990 in spite of the multiplicity of journeys. The role of the internist seems important.

Background Although anaphylaxis has been considered a priority public health issue in the world allergy community, epidemiological data on morbidity and mortality remain suboptimal. We performed the first multicenter epidemiological study in French emergency departments (EDs). The study covered 7 EDs over a period of 1 year. The objectives were to identify areas that are amenable to change and to support ongoing national and international efforts for better diagnosis, management, and prevention of anaphylaxis. Methods Ours was a descriptive study based on data routinely reported to French institutional administrative databases from 7 French public health institutions in the Lorraine region between January and December 2015. Data were collected based on the anaphylaxisrelated codes of the International Classification of Diseases (ICD)-10, and cases were clinically validated as anaphylaxis. Results Of the 202 079 admissions to the EDs, 4817 had anaphylaxis-related codes; of these, 323 were clinically validated as anaphylaxis. Although 45.8% were severe, adrenaline was prescribed in only 32.4% of cases. Of the 323 cases, 57.9% were subsequently referred for an allergy work-up or evaluation (after or during hospitalization), and 17.3% were prescribed autoinjectable epinephrine. Conclusion Our results highlight an urgent need for improved public health initiatives with respect to recognition and treatment of anaphylaxis. We flag key problems that should be managed in the coming years through implementation of national and international actions.

Belimumab (an anti-BLyS monoclonal antibody) was recently approved for the treatment of systemic lupus erythematosus (SLE). The aim of the study was to describe efficacy and safety of the drug as well as its impact on serologic parameters and the role of long-term systemic sparing of treatment in clinical practice in LE.We conducted a retrospective study at Reims University Hospital between 2012 and 2016 including consecutive patients with LE treated with belimumab. Efficacy was evaluated in terms of clinical progression, and normalisation of laboratory factors (anti-DNA antibody and C3 serum levels) and sparing of associated long-term systemic therapies for LE.Among the 15 patients included, a therapeutic response was obtained in 9 patients (60%), with partial remission in 8 of 9 cases. The median titre of anti-DNA antibody was 50IU/mL (range: 4-50) and the median C3 level was 0.82g/L (range: 0.36-1.23) before initiation of belimumab, vs. 25.5IU/mL (range: 2-50) and 0.89g/L (range: 0.34-1.22) at the last evaluation, respectively, without significant modification (P=0.12 and P=0.45). The median dose of prednisone at the time of the first belimumab infusion was reduced from 9.5mg/day (range: 0-18) to 6mg/day (range: 0-20) at the last clinical evaluation. Eight patients (53%) experienced adverse events, and these were very slight or moderate in all cases.Belimumab appears to be an effective and well-tolerated treatment for moderately severe systemic LE, allowing sparing of maintenance corticosteroid therapy in order to decrease its frequent adverse events.

Background IgG replacement therapy (IgRT) in primary immunodeficiencies (PID) is a lifelong treatment which may be administered intravenously (IVIg) or subcutaneously (SCIg), at hospital or at home. The objective of the VISAGE study was to investigate if route and/or place for IgRT impact patients’ satisfaction regarding IgRT and quality of life (QoL) in real-life conditions. Methods The study enrolled PID patients at least 15 years old receiving IgRT for at least 3 months. Satisfaction and QoL were evaluated at enrollment and over a 12-month follow-up period by Life Quality Index (LQI) which measures 3 dimensions of satisfaction: treatment interference, therapy related problems and therapy settings (factors I, II and III) and SF-36 v2 questionnaire. Results The study included 116 PID patients (mean age 42 ± 18 years, 44 % males, 58 % with scholar or professional occupation) receiving IgRT for a mean of 8.5 ± 8.4 years. At enrollment they were receiving either home-based SCIg (51 %), hospital-based IVIg (40 %) or home-based IVIg (9 %). Patients exhibited a high degree of satisfaction regarding IgRT whatever the route and place for administration. LQI factor I was higher for home-based SCIg (86 ± 2) than for hospital-based IVIg (81 ± 3) and home-based IVIg (73 ± 5; p = 0.02 versus home-based SCIg); no difference was found for LQI factor II; LQI factor III was higher for home-based SCIg (92 ± 2) than for hospital-based IVIg (87 ± 5) and hospital-based IVIg (82 ± 3; p = 0.005 versus home-based SCIg). By contrast, every dimension of QoL was impaired. Over the follow-up period, 10 patients switched from hospital-based IVIg to home-based SCIg and improved LQI factor I (p = 0.004) and factor III (p = 0.02), while no change was noticed in LQI factors II and QoL. Meanwhile, no change in satisfaction or QoL was found in patients with stable route of IgRT. When asked on their preferred place of treatment all but one patient with home-based treatment would choose to be treated at home and 29 % of patients treated at hospital would prefer home-based IgRT. Conclusion PID patients expressed a high degree of satisfaction regarding IgRT, contrasting with impaired QoL. In real-life conditions awareness of patient’s expectations regarding the route or place of IgRT may be associated with further improvement of satisfaction.

International audience; BACKGROUND:In 2014, the European Society for Immune Deficiencies (ESID) revised the common variable immunodeficiency (CVID) diagnosis criteria by incorporating new clinical and biological markers. The new definition appeared more restrictive but had not yet been evaluated in a large cohort of patients.OBJECTIVE:The objective of this study was to evaluate the impact of this new definition in a large cohort of patients with primary hypogammaglobulinemia.METHODS:Evaluation of 3 different CVID definitions (ESID/Pan-American Group for Immunodeficiency [PAGID] 1999, ESID 2014, DEFI 2015) in 521 patients included in the French DEFI study with a diagnosis of primary hypogammaglobulinemia.RESULTS:Using the ESID/PAGID 1999 definition, 351 patients were classified as CVID. The new ESID 2014 definition excluded 62 (18%) patients. Most of them (n = 56; 90%) had a less severe disease, whereas 6 (10%) presented with a severe disease with major T-cell defect. We propose different criteria (occurrence of opportunistic infection or very low naive CD4+ T-cell count) to define this population with severe T-cell defect. Sixty-two patients fulfilled these criteria, represented 20% of the initial CVID population but accounted for 77% of the deaths, with a 5-year overall survival of 67.6% (95% confidence interval, 51.0-79.6), and were considered as late onset combined immunodeficiency (LOCID).CONCLUSIONS:The new ESID definition for CVID still fails to exclude a large number of patients with severe T-cell defect. We propose a new definition (DEFI 2015) that excluded more patients with a T-cell defect and consider these patients as LOCID. This population has a poor outcome and should be considered as a distinct group requiring specific care.

Fabry disease is caused by a deficiency of a-galactosidase A which leads to the progressive intra-lysosomal accumulation of ceramide trihexoside (CTH), also known as globotriaosylceramide (Gb3), in different cell types and body fluids. The clinical manifestations are multisystemic and predominantly affect the heart, kidney and central nervous system. The role of CTH in the pathophysiological process of Fabry disease is not established, and the link between the degree of accumulation and disease manifestations is not systematic. The use of CTH as a diagnostic tool has been proposed for several decades. The recent introduction of a specific treatment for Fabry disease in the form of enzyme replacement therapy (ERT) has led to the need for a biological marker, in place of a clinical sign, for evaluating the efficacy of treatment and also as a tool for following the long term effects of treatment. The ideal biomarker must adhere to strict criteria, and there should be a correlation between the degree of clinical efficacy of treatment and a change in its concentration. This review of the literature assesses the utility of CTH as a diagnostic tool and as a marker of the efficacy of ERT in patients with Fabry disease. Several techniques have been developed for measuring CTH; the principles and the sensitivity thresholds of these methods and the units used to express the results should be taken into consideration when interpreting data. The use of CTH measurement in Fabry disease should be re-evaluated in light of recent published data.

The clinical value of immunoenzymatic (enzyme-linked immunosorbent assay) detection of anti- Toxoplasma immunoglobulin E (IgE) was assessed by studying 2,036 sera from 792 subjects, comprising seronegative controls and subjects with acute, active, reactivated, or congenital toxoplasmosis. Included were nonimmunized adults; pregnant women with recently acquired infection (acute toxoplasmosis); immunocompetent subjects with recently acquired severe infection (active toxoplasmosis) expressed as fever, adenopathies, splenomegaly, pneumonia, meningitis, or disseminated infection; subjects—some of them immunocompromised—whose previously moderate IgG antibody levels rose, suggesting a reactivation of quiescent toxoplasmosis; and infants born to seroconverted mothers and evaluated for diagnosis of congenital infection and therapeutic management. Specific IgE antibodies were never detected in seronegative subjects. They were present in 85.7% of asymptomatic seroconverters and in 100% of seroconverters with overt toxoplasmosis, following two different kinetics: in the former, the specific IgE titer generally presented a brief peak 2 to 3 months postinfection and then fell rapidly, whereas specific IgE persisted at a very high titer for several months in the latter. IgE emerged concomitantly with the increase in IgG during toxoplasmic reactivation. For neonatal diagnosis of congenital toxoplasmosis, IgE was less informative than IgM and IgA (sensitivities, 59.5, 64.3, and 76.2%, respectively) and had a specificity of 91.9%. Nevertheless, simultaneous measurement of the three isotypes at birth improved the diagnostic yield to 81% relative to the combination of IgA and IgM. Emergence of specific IgE during postnatal treatment for congenital toxoplasmosis is a sign of poor adherence or inadequate dosing.

Fabry's disease is an X-linked inherited lysosomal storage disorder caused by the deficient activity of alpha-galactosidase A. The interrelationships between clinical symptoms in Fabry patients have not yet been fully established. Using cluster and multivariate analysis, the aim of the study was to determine the relationships among clinical symptoms and organ involvement, and predictive clinical symptoms for disease severity.Clinical data obtained from 108 French Fabry patients were retrospectively collected and analysed using multiple correspondence analysis and hierachical ascendant classification. Multivariate analysis was also performed to determine among clinical symptoms predictors for cardiac disease (HRT), renal involvement (KDN) and brain complication (STR).The cohort comprised 41 male patients (aged 28.9 ± 11.6 years) and 67 female patients (aged 40.4 ± 15.5 years). Three main clusters of clinical symptoms could be delineated, characterising disease progression: the first cluster grouped digestive disorders (found in 30% of the patients) and exercise intolerance (32%), the second, cluster dyshidrosis (47%), acroparesthesia (67%), angiokeratoma (44%) and cornea verticillata (54%), the third, cluster grouped KDN (30%), HRT (39%) and STR (25%) and hearing loss (44%). In univariate analysis, the patient age predicted HRT and KDN, dyshidrosis predicted HRT and STR, angiokeratoma predicted KDN and cornea verticilla and hearing loss predicted KDN, HRT and STR. In multivariate analysis, hearing loss and age were independent predictors of organ complication.Among the various interrelated clinical symptoms occurring in Fabry disease, patients with dyshidrosis and particularly hearing disorders appear to be at higher risk of organ complications.

A multivariate analysis was used to identify factors influencing the immunogenicity of rabies vaccine and to assess the efficacy of booster injections in a cohort of 407 people monitored prospectively for 10 years after primary vaccination. Rabies vaccine (HDCV or PVRV) was injected by intramuscular route either on days 0 and 28 or on days 0, 7 and 28. All the participants received a booster injection on day 365. At the end of follow-up (year 10), 163 subjects had titers >0.5IU/ml (group A) and 59 subjects had titers

In the present retrospective study, we described a series of 45 non-icteric leptospirosis and 44 nephropathia epidemica (NE) patients diagnosed in the northeast of France from 1995 to 2005 and compared their clinical picture and laboratory parameters, as well as some epidemiological data. Loin pain (P < 0.001), abdominal pains (P = 0.007), rise of blood pressure (P < 0.001) and pharyngitis (P = 0.01) were more frequently found in NE patients. Aspartate aminotransferase (ASAT) (P = 0,006), creatine phosphokinase (CPK) (P < 0.0001) and C-reactive protein (CRP) (P < 0.0001) were higher in leptospirosis, whereas creatinine (P = 0.009) was higher in NE. Leptospirosis mainly concerns occupational hazards, e.g. farmers, and leisure activities like swimming, and NE concerns professional foresters or leisure activities in the forest and the cleaning of attics. During hospitalisation, patients receiving antibiotics were more frequent among leptospirosis than among NE patients (80% versus 59%, P = 0.06). Among the various common clinical signs, only acute myopia appeared to be a pathognomonic but inconsistently observed clinical sign, which was only observed in 47% of NE cases.

Nocardia species is an aerobic soil-saprophyte bacterium, responsible for rare opportunistic infections, mainly reported in immunocompromised patients. Nocardia brain abscess accounts for 1 to 2% of cerebral abscesses. Prognosis is poor.We describe clinical, radiological and bacteriological findings along with therapeutic aspects for five patients and review the literature on Nocardia cerebral abscess.The clinical features of Nocardia brain abscess are insidious and nonspecific, occurring frequently with a medical background of obvious or latent immunodeficiency; fever, if any, is observed subordinate to extracerebral nocardiosis. Computerized tomography scan and conventional magnetic resonance (MR) scan show lesions with a necrotic core and multilobed thick walls enhancing after injection of gadolinium or iodine. Abscesses are mainly located in the brain stem, basal ganglia and cerebral cortex of the frontal, parietal and occipital lobes; cerebellar and spinal locations are uncommon. MR diffusion-weighted imaging with calculation of apparent diffusion coefficient and proton MR spectroscopy can provide additional data for accurate differential diagnosis between abscess and other necrotic lesions, such as tumor and cyst formations. Bacteriological identification has progressed with advances in molecular microbiology: 16S rRNA sequencing, allowing a more rapid routine identification of Nocardia strains from clinical samples. Clinical management of patients with a Nocardia brain abscess relies upon early use of intravenous antibiotics adapted to the strains identified and their susceptibility. Most Nocardia strains display susceptibility to cotrimoxazol, amikacin and linezolid, but develop beta-lactamase activity.Early pus samples, obtained by biopsy or surgical resection, are needed to establish a certain bacteriological diagnosis and initiate appropriate intravenous antibiotics.

Summary Background Fabry disease (also known as Anderson–Fabry disease) is a rare, X-linked lysosomal storage disorder that is characterized by accumulation of globotriaosylceramide throughout a range of tissues in the body. Objectives To ascertain the prevalence and nature of cutaneous manifestations in patients with Fabry disease and to relate these to the severity of systemic manifestations of the disease. Methods We have documented the dermatological features of this disease with reference to data from 714 patients (345 males, 369 females) registered on the Fabry Outcome Survey (FOS), a multicentre European database. Results We confirm that the commonest disease manifestation is angiokeratoma. Overall, 78% of males and 50% of females had one or more dermatological abnormality, the commonest being angiokeratoma (66% males, 36% females), hypohidrosis (53% males, 28% females), telangiectasia (23% males, 9% females) and lymphoedema (16% males, 6% females). We demonstrate for the first time that the presence of cutaneous vascular lesions correlates with the severity of the systemic manifestations of the disease (pain, renal failure, cardiac disease, premature cerebrovascular disease) as assessed by a severity scoring system. Although the condition is X linked, there is a surprisingly high prevalence of abnormalities in females. Conclusions The FOS database is a useful epidemiological tool in establishing the variety and relevance of cutaneous manifestations in Fabry disease. The present study confirms that the presence of dermatological manifestations appears to be a marker of greater severity of systemic disease, which emphasizes the importance of the dermatological assessment of these patients.

Enzyme replacement therapy (ERT) has been used to treat Fabry disease - a progressive lysosomal storage disorder - since 2001. Two preparations of the enzyme alpha-galactosidase A are available in Europe: agalsidase alpha, produced in a human cell line, and agalsidase beta, produced in Chinese hamster ovary cells. To review critically the published evidence for the clinical efficacy of these two enzyme preparations. A systematic literature search was undertaken to identify open or randomised controlled trials published on Fabry disease since 2001. Eleven trials fulfilled the criteria for inclusion in this review, of a total of 586 references on Fabry disease. To date, no direct comparisons exists between the two available enzyme preparations. Significant clinical benefits compared with placebo, however, have been demonstrated with ERT, with positive effects on the heart, kidneys, nervous system and quality of life. The quality of most of these publications was less than optimal. Further prospective studies are required to confirm the long-term clinical benefits of ERT. More studies are also needed on the effects of ERT in women and on the use of ERT early in the course of Fabry disease, to prevent organ damage. Large national and international outcomes databases will also be invaluable in evaluating treatment effects and safety.

Hypohidrosis is a classic feature of Fabry disease; in contrast, hyperhidrosis has only been rarely described. The aim of the study is to characterise the baseline descriptive data on hyperhidrosis (frequency, age at onset, sex ratio and outcome with and without enzyme replacement therapy) in hemizygous male and heterozygous female patients with Fabry disease. We describe case histories of five patients with Fabry disease and hyperhidrosis seen at three different centres. We have also analysed a cohort of 21 paediatric patients in the UK and a large European cohort of patients enrolled in the Fabry Outcome Survey (FOS). Five patients (three female, two male) with hyperhidrosis were originally identified, although each had additional symptoms related to Fabry disease. The age at onset of hyperhidrosis was less than 18 years in four cases. In the cohort of 21 paediatric patients (12 female, nine male), one female had hyperhidrosis; the age at onset of this symptom was 11 years. In the FOS cohort, 66 of 714 patients with Fabry disease had hyperhidrosis (44 of 369 females, 11.9%; 22 of 345 males, 6.4%). The female predominance was observed in seven of nine countries from which data were analysed. Hyperhidrosis is an increasingly recognised feature of the Fabry disease phenotype. It is more prevalent in females than in males and often appears in childhood or adolescence. The efficacy of enzyme replacement therapy on this recently recognised symptom should be assessed.

Aims To develop a population pharmacokinetic model for pyrimethamine (PYR) and sulfadoxine (SDX) in children with congenital toxoplasmosis. Methods Children were treated with PYR (1.25 mg kg−1) and SDX (25 mg kg−1) (Fansidar®) plus folinic acid (Lederfoline® 5 mg). Plasma concentrations, available from a therapeutic drug monitoring database, were determined by high-performance liquid chromatogrphy. Population pharmacokinetic analysis was performed using a nonlinear mixed effects model. Results Eighty-nine children, aged 1 week to 14 years and weighing 2.9–59 kg, were available for evaluation. Both PYR and SDX concentration-time profiles were best described by a one-compartment open model. Volume of plasma distribution (V) and clearance (CL) were significantly related to body weight (BW) using an allometric function. Typical CL and V estimates (95% confidence interval), for a child weighing 11 kg were 5.50 (5.28, 5.73) l day−1 and 36 (33, 39) l for PYR and 0.26 (0.25, 0.27) l day−1 and 2.1 (1.9, 2.3) l for SDX. For BW between 3.5 and 60 kg, plasma half-lives were predicted to vary from 4.0 to 5.2 days for PYR, and from 5.0 to 7.5 days for SDX. Conclusion This study indicated that body weight influences PYR and SDX pharmacokinetics in children. To optimize PYR/SDX combination treatment in congenital toxoplasmosis, short dosing intervals in very young low-wight children are probably appropriate.

Eosinophilic gastroenteritis of unknown origin could be isolated or integrated in idiopathic hypereosinophilic syndrome. Clinical expression is variable since the lesion may affect any area of the gastrointestinal tract and any layer of the wall.A 25-year-old male patient had digestive symptoms such as peritoneal, obstructive and diarrheal signs, associated with blood eosinophilia, giving evidence for eosinophilic jejuno-ileitis. Computer tomography revealed an extensive obstruction of the jejuno-ileum and thickening of the intestinal wall. The diagnosis was obtained using laparoscopy and controlled wedge biopsy, which showed a predominantly external infiltration of the intestinal wall by eosinophils. The disease evolution was favorable with corticosteroid therapy.Worrying and persistent digestive symptomatology, associated with blood eosinophilia, particularly when intestinal wall infiltration is revealed by computer tomography, should lead one to perform a laparoscopy to guide a surgical biopsy of the intestinal wall.

Neurological involvement in Crohn's disease is unusual.We report two cases of Crohn's disease. Central nervous system deficit was observed in the first patient, while psychiatric disorders were present in the second patient. In both cases, MRI confirmed the presence of a T2 hypersignal in the white matter.Central neurological disorders could either be part of extra-intestinal symptoms in Crohn's disease or precede diagnosis. However, their frequency is not well known or documented. The association of Crohn's disease with multiple sclerosis may obscure the diagnosis. Cerebrovascular accident, seizures, headaches, focal neurological deficits, or sometimes severe psychiatric disorders are encountered. Numerous pathophysiological hypotheses have been advocated. The high frequency of abnormalities that have been observed during systematic MRI provide confirmation of the hypothesis of neurological disorders occurring during Crohn's disease.

Coagulase-negative Staphylococci (CNS), considered for many years to be commensal bacteria of the skin are now recognized as major agents of nosocomial infection. Bacterial factors (increased resistance), host factors (immune status) and multiplication of the portals of entry (presence of foreign material) have contributed to the increased incidence of nosocomial infections. The importance of the role of NCS in urology is due to their great capacity to colonize catheters and most prostheses. The particular organization of these bacteria into a conglomerate called biofilm is responsible for prosthetic infections, which can impair renal function and can sometimes be life-threatening. The authors review the current increase of the number of CNS isolated in urology departments and describe the various therapeutic strategies that can be proposed to eradicate these bacteria.

Abrikossoff's tumor, also called granular cell tumor, is an uncommon condition, generally benign, which can affect every organ and specially skin and tongue. The authors report an observation of a bronchial tumor and review the literature. Possible relapse after treatment, locally or everywhere in the body, may occur as local or regional complication that may necessit heavy surgery. It appears that a benign tumor can have macroscopic and even microscopic aspect of malignancy, when real malignant tumors are extremely rare. Wide excision still remain current treatment of Abrikossoff's tumor. Endoscopic methods are an interessant and less invasive alternative to treat some deep tumors.

Mycotic aneurysm of the posterior tibial artery and pseudophlebitis: role of color Doppler sonography A case of a 78-year-old male patient presenting with endocarditis caused by Streptococcus bovis and pseudophlebitis of the left lower limb is described. Color Doppler sonography ruled out thrombophlebitis and showed a large pulsatile mass of the posterior compartment of the leg due to a mycotic aneurysm of the posterior tibial artery. This aneurysm was confirmed by angiography and treated by surgery. The important role of color Doppler sonography for the diagnosis of this particular case is emphasized.

A cannulation technique for continuous sampling of cerebrospinal fluid in unanesthetized freely moving rabbits was established. A permanent Teflon catheter constructed easily and inexpensively from commercially available material was placed into the cisterna magna and fixed to the skull by anchoring screws and dental cement. The surgical procedure required a minimum of time and induced minimal trauma, allowing studies in which physiologic conditions are required. The Teflon catheter induced minimal foreign body reaction, the system had minimal dead volume, and its size was small enough to allow the simultaneous implantation of other devices.

BACKGROUND Common variable immunodeficiency (CVID) is a primary immune deficiency defined by defective antibody production. In most series, a small proportion of patients present with opportunistic infections (OIs). METHODS The French DEFI study has enrolled patients with primary hypogammaglobulinemia and allows a detailed clinical and immunologic description of patients with previous OIs and/or at risk for OIs. RESULTS Among 313 patients with CVID, 28 patients (8.9%) presented with late-onset combined immune deficiency (LOCID), defined by the occurrence of an OI and/or a CD4(+) T cell count