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• Paracetamol during pregnancy may disrupt the developing sexual brain. • It inhibits the enzyme cyclooxygenase 2 and 3 (COX-2,3) to reduce pain and inflammation. • COX-2 is required for prenatal masculinization of the mPOA in the brain. • Paracetamol during brain development may cause iatrogenic sexual diversity and accompanying affective states. Although paracetamol is considered a safe medication during pregnancy at therapeutic doses, and despite animal studies showing no negative effects or reporting no adverse effects during pregnancy, there are no well-controlled clinical studies demonstrating the safety for both the mother and fetus. Therefore, its use in this situation depends on the physician's discretion. Its mechanism involves the inhibition of the enzymes cyclooxygenase 2 and 3 (COX-2,3), which modulate pain and inflammation by reducing prostaglandins. However, COX-2 and prostaglandins are also part of the molecular cascade organizing the brain towards a male direction during the prenatal period, and potentially during other periods as well. Herein, we hypothesize that paracetamol, a common medication used to treat fever and pain, may have long-term effects as a neuroendocrine disruptor of the sexual brain when administered during critical periods of development such as pregnancy, and to a lesser extent, during the neonatal, infancy, and puberty periods. Throughout this manuscript, we discuss the effects of COX-2,3 inhibition on the organization of the brain and sexual partner preference, particularly affecting masculinization of the medial preoptic area. We argue how administration of paracetamol in critical periods of plasticity might have an enduring effect on sexual behavior and other motivated behaviors. Impairments in each of these processes can directly impact individuals' sexual and mental health. We also discuss how sexual experience in adulthood ameliorated the impaired sexual preference of paracetamol-treated males, indicating an interaction between biological and environmental mechanisms. This manuscript is meant to warn professionals that although paracetamol is a very effective and safe drug, it is likely to disorganize the developing sexual brain, probably contributing to what we call iatrogenic sexual diversity. [ABSTRACT FROM AUTHOR]

Gastrointestinal (GI) involvement was described to be a poor prognostic factor in systemic necrotizing vasculitis. Its prognostic significance may vary according to clinical presentation and vasculitis subtype. This study investigated risk-factors associated to poor outcome in GI-involvement of vasculitis. Patients with systemic vasculitis as defined by the 2012 Chapel Hill Consensus Conference and presenting with GI involvement were retrospectively included. Baseline characteristics, treatments and outcome were recorded. Primary endpoint was a composite of admission to intensive care unit (ICU), emergency surgical procedure, or death. Two hundred and thirteen patients were included. Vasculitis were distributed as follows: 41% IgA vasculitis, 27% ANCA-associated vasculitis, 17% polyarteritis nodosa (PAN), and 15% other vasculitis. Eighty-three (39%) patients fulfilled the composite primary endpoint within 6 months. Predictive factors associated with the primary endpoint included PAN subtype (OR 3.08, 95% CI 1.29–7.34), performance status (OR 1.40, 1.05–1.87), use of morphine (OR 2.51, 0.87–7.24), abdominal guarding (OR 3.08, 1.01–9.37), ileus (OR 2.29, 0.98–5.32), melena (OR 2.74, 1.17–6.42), increased leukocytes (per G/L, OR 1.05, 1.00–1.10), low hemoglobin (per g/dL, OR 0.80, 0.71–0.91) and increased CRP (log mg/L, OR 1.21, 0.94–1.56). A risk prediction model for the achievement of primary endpoint had a very good performance [C-statistics 0.853 (0.810 to 0.895], and for overall survival as well. Vasculitis presenting with GI involvement have a poor outcome in more than one third of cases. An easy-to-use risk prediction model had a very good performance to predict the admission to ICU, emergency surgical procedure, or death. [Display omitted] • Gastrointestinal involvement in systemic vasculitis is a poor prognostic factor. • Risk factors associated to poor outcome were included in a scoring system. • These features quantified the predicted risk for clinical deterioration. [ABSTRACT FROM AUTHOR]

• C2 nerve and ganglion compression. • Occipital neuralgia. • C2 neurectomy or ganglionectomy. Occipital neuralgia typically arises in the setting of nerve compression by fibrosis, surrounding anatomic structures, or osseous pathology, such as bone spurs or hypertrophic atlanto-epistropic ligament. It generally presents as paroxysmal bouts of sharp pain in the sensory distribution of the first three occipital nerves. Due to the long course of the greater occipital nerve (GON), and its peculiar anatomy, and location in a mobile region of the neck, it is unsurprising that the GON is at high risk for compression. Little is known how to diagnose or treat this neuropathic pain syndrome. The objective of this paper is to isolate the etiology involved, and treat this condition promptly. After all nonoperative efforts are exhausted, surgical transection of the nerve is the treatment of choice in these cases. An isolated C2 neurectomy or ganglionectomy is performed for an optimal pain relief. C1-2 instrumented fusion can be considered if, extensive facet arthropathy with instability is identified. Authors review the spectrum of treatment options for this debilitating condition, and discuss the case example of a patient who required conversion to a C1-C2 instrumented fusion following C2 ganglionectomy due to an underlying extensive degenerative disease and intraoperative findings suggestive of atlantoaxial instability. [ABSTRACT FROM AUTHOR]

Highlights • Transarticular stepwise technique for SI joint arthrodesis. • SI joint fusion offers good outcome. Abstract Sacroiliac (SI) joint can produce debilitating lower back pain with radiation to groin, buttocks, and lower extremities. SI joint dysfunction poses a clinical challenge to the spine surgeons. Studies entailing surgical arthrodesis utilizing Titanium implants have been reported with reputedly high level of patient satisfaction. Authors have described technical aspects of surgical technique with use of titanium screw implants. The transarticular technique is used to places SI joint screw implants across the articular portion of SI joint. Cadaveric SI joint instrumentation is performed under fluoroscopic guidance. Moreover, Medline literature search is conducted to study surgical outcome, and patient satisfaction. 4 cadavers are prepped prone for the percutaneous approach. Bilaterally 6 screws are placed using transarticular placement technique under fluoroscopic guidance. The posterior technique utilizes alignment guide to place the screws inline on the inlet view, parallel in the outlet view, and parallel to the dorsal aspect of the sacral body in the lateral view. One C-arm is used in the entire technique. The technical aspects of surgical technique have been described in a stepwise fashion for easy reproducibility in the operating room. Each screw track is checked with tactile feel of a blunt K-wire before final deployment. All bilateral screws were checked on a set of fluoroscopic views. A detail clinical examination, diagnostic joint injection, with the radiological imaging must be considered before surgical consideration. SI Joint fusion utilizing 3 transarticular sacral screws is equally effective and safe procedure to treat chronic lower back pain ensuing from SI joint dysfunction. [ABSTRACT FROM AUTHOR]

Abstract Objectives Biofilm production in extended spectrum β-lactamase (ESBL)-producing Enterobacteriaceae provides a favourable environment for the exchange of antibiotic-resistance genes and could facilitate widespread dissemination. We aimed to assess biofilm development in ESBL-producing E. coli and K. pneumoniae isolates and determine how development relates to microbiological characteristics and clinical outcomes. Methods 147 ESBL-producing E. coli and 82 K. pneumoniae were genetically characterized. Biofilm formation was measured at 1.5, 4, 6, and 24 h during culture in blood heart infusion using a microbead immobilization assay (BioFilm Ring test®). Results were given as biofilm formation index (BFI) with lower values indicating increased presence of biofilm (range = 0–21). Results In total, 57.1% of strains were strong producers of biofilm (BFI < 2), whereas 13.4% lacked biofilm production (BFI > 18). Standard biofilm production (BFI < 7) was common in E. coli isolates (61.9%). For E. coli , biofilm production was less frequently observed in ST131 clones (p = 0.03) but more frequently in strains harbouring toxin (p = 0.008) or adhesin (p = 0.008) virulence factor genes. Despite almost all K. pneumoniae having standard biofilm production (90.2%), there was a 2.4-times higher odds of observing biofilm in ST29/147/323 versus other ST-types (p = 0.13). Patients with standard biofilm producing isolates were not at increased risk of transfer to intensive-care (odds-ratio=2.80, 95%CI=0.59–13.21) or death within 12-months (odds-ratio=1.61, 95%CI=0.75–3.43). Conclusion In these ESBL-producing strains, biofilm production is linked to certain virulence factors in E. coli and is common in K. pneumoniae. Further exploration of whether biofilm production increases dissemination and risk of severe clinical outcomes is needed in larger collections of isolates. [ABSTRACT FROM AUTHOR]

Purpose Nonoperative treatment of acute appendicitis appears to be feasible in adults. It is unclear whether the same is true for children. Methods Children 5–18 years with < 48 h symptoms of acute appendicitis were offered nonoperative treatment: 2 doses of piperacillin IV, then ampicillin/clavulanate × 1 week. Treatment failure (worsening on therapy) and recurrence (after completion of therapy) were noted. Patients who declined enrollment were asked to participate as controls. Cost–utility analysis was performed using Pediatric Quality of Life Scale (PedsQL®) to calculate quality-adjusted life month (QALM) for study and control patients. Results Twenty-four patients agreed to undergo nonoperative management, and 50 acted as controls. At a mean follow-up of 14 months, three of the 24 failed on therapy, and 2/21 returned with recurrent appendicitis at 43 and 52 days, respectively. Two patients elected to undergo an interval appendectomy despite absence of symptoms. Appendectomy-free rate at one year was therefore 71% (C.I. 50–87%). No patient developed perforation or other complications. Cost–utility analysis shows a 0.007–0.03 QALM increase and a $1359 savings from $4130 to $2771 per nonoperatively treated patient. Conclusion Despite occasional late recurrences, antibiotic-only treatment of early appendicitis in children is feasible, safe, cost-effective and is experienced more favorably by patients and parents. [ABSTRACT FROM AUTHOR]

Purpose Patient-centered outcomes research (PCOR) aims to give patients a better understanding of the treatment options to enable optimal decision-making. As nonoperative alternatives are now being evaluated in children for acute appendicitis, we surveyed patients and their families regarding their knowledge of appendicitis and evaluated whether providing basic medical information would affect their perception of the disease and allow them to more rationally consider the treatment alternatives. Methods Families of children aged 5–18 presenting to the Emergency Department with suspected appendicitis were recruited for a tablet-based interactive educational survey. One hundred subjects (caregivers and patients ≥ 15 years) were questioned before and after an education session about their understanding of appendicitis, including questions on three hypothetical treatment options: urgent appendectomy, antibiotics alone, or initial antibiotics followed by elective appendectomy. Subjects were clearly informed that urgent appendectomy is currently the standard of care. Results Only 14% of respondents correctly identified the mortality rate of appendicitis (17 deaths/year according to the 2010 US census) when compared with other extremely rare causes of death. Fifty-four and 31% thought it was more common than death from lightning (40/year) and hunting-associated deaths (44/year), respectively. Eighty-two percent of respondents believed it “likely” or “very likely” that the appendix would rupture if operation was at all delayed, and 81% believed that rupture of the appendix would rapidly lead to severe complications and death. In univariate analysis, this perception was significantly more prevalent for mothers (odds ratio, (OR) 5.19, confidence interval (CI) 1.33–21.15), and subjects who knew at least one friend or relative who had a negative experience with appendicitis (OR 5.53, CI 1.40–25.47). Following education, these perceptions changed significantly (53% still believed that immediate operation was necessary, and 47% believed perforation led to great morbidity and potential mortality, P < 0.001). In a survey of potential appendicitis treatment options, urgent appendectomy was considered a “good” or “very good” option by 74% of subjects, compared with 68% for antibiotics only without appendectomy and 49% for initial antibiotic therapy followed by elective outpatient appendectomy. Conclusion There was a striking knowledge gap in the participant perception of appendicitis. Appropriate education can correct anecdotally supported misconceptions. Adequate education may empower patients to make better-informed decisions about their medical care and may be important for future studies in alternative treatments for appendicitis in children. [ABSTRACT FROM AUTHOR]

Late eating has been linked to obesity risk. It is unclear whether this is caused by changes in hunger and appetite, energy expenditure, or both, and whether molecular pathways in adipose tissues are involved. Therefore, we conducted a randomized, controlled, crossover trial (ClinicalTrials.gov NCT02298790) to determine the effects of late versus early eating while rigorously controlling for nutrient intake, physical activity, sleep, and light exposure. Late eating increased hunger (p < 0.0001) and altered appetite-regulating hormones, increasing waketime and 24-h ghrelin:leptin ratio (p < 0.0001 and p = 0.006, respectively). Furthermore, late eating decreased waketime energy expenditure (p = 0.002) and 24-h core body temperature (p = 0.019). Adipose tissue gene expression analyses showed that late eating altered pathways involved in lipid metabolism, e.g., p38 MAPK signaling, TGF-β signaling, modulation of receptor tyrosine kinases, and autophagy, in a direction consistent with decreased lipolysis/increased adipogenesis. These findings show converging mechanisms by which late eating may result in positive energy balance and increased obesity risk. [Display omitted] • Late eating increases waketime hunger and decreases 24-h serum leptin • Late eating decreases waketime energy expenditure and 24-h core body temperature • Late eating alters adipose tissue gene expression favoring increased lipid storage • Combined, these changes upon late eating may increase obesity risk in humans In this randomized crossover trial that rigorously controlled for nutrient intake, physical activity, and sleep, Vujović et al. found that late eating increased hunger, modified appetite-regulating hormones, decreased daytime energy expenditure, and altered adipose gene expression consistent with increased adipogenesis/decreased lipolysis. Together, these findings may explain the increased obesity risk in late eaters. [ABSTRACT FROM AUTHOR]

NF-κB transcription factors regulate the expression of hundreds of genes primarily involved in immune responses. Signaling events leading to NF-κB activation constitute a major antiviral immune pathway. To replicate and persist within their hosts, viruses have evolved diverse strategies to evade and exploit cellular NF-κB immune signaling cascades for their benefit. We summarize recent studies concerning viral manipulation of the NF-κB signaling pathway downstream of pattern recognition receptors. Signal transduction mediated by pattern recognition receptors is a research frontier for both infectious disease and innate immunology. [ABSTRACT FROM AUTHOR]

Dielectric properties (relative permittivity ɛ ′ and loss factor ɛ ″) are key parameters for microwave heating of food products. In view of continuous microwave processing of green coconut water (GCW), dielectric properties and electric conductivity were determined by open-ended coaxial probe technique at temperatures between 0 and 90 °C and frequencies between 500 and 3000 MHz. Simulated solutions of salts and sugars from GCW were also tested to evaluate component contributions and interactions. At 915 MHz, ionic conduction plays an important role in microwave heating, while a balance between ionic and dipolar mechanisms was observed at 2450 MHz, depending on temperature. Sugars had a weak effect on polarization or loss. Component interaction reduced ɛ ″ (approximately 12% at 915 MHz and 8% at 2450 MHz). It was possible to follow the change of the dielectric properties and the power penetration depth as the temperature raised and correlations were adjusted to model temperature dependence. [ABSTRACT FROM AUTHOR]

Infections caused by extended-spectrum beta-lactamase (ESBL)-producing Escherichia coli are an important cause of morbidity and mortality, especially in children. We compared 58 epidemiologically unrelated ESBL-producing E. coli strains that caused infections. They were isolated between 2008 and 2012 in two Parisian pediatric hospitals and grouped according to their origin into either community-acquired (CA) ( n = 37) or nosocomially acquired (NA) ( n = 21) strains. Molecular characteristics of the ESBLs, phylogenetic traits of the strains including their belonging to clone O25b-ST131, prevalence of associated virulence genes, growth capacities in different media, metabolic phenotype and biofilm formation abilities were studied. ESBL type, associated resistance and distribution of phylogenetic groups were similar in the CA and NA groups. More than 60% of the B2 phylogroup strains in both groups belonged to the ST131 clone. Interestingly, CA strains possessed more genes encoding virulence factors and the distribution of these genes differed significantly between the two groups: fyuA , hlyC , papC and papGII were more frequent in the CA group, whereas iroN was more frequent in the NA group. CA strains also showed enhanced growth capacities in Luria Bertani rich medium. They tended to produce more biofilm but the difference was not significant. This study confirms the wide spread of clone ST131 among infected children, regardless of whether their infections were community- or nosocomially acquired. It highlights genotypic and phenotypic differences according to the origin of the strains that could indicate adaptability of these multi-resistant bacteria to specific environmental and host factors. [ABSTRACT FROM AUTHOR]

Résumé: En France, la télémédecine qui consiste à utiliser les technologies de la télécommunication pour le diagnostic médical et les soins de santé est peu développée en médecine générale. Pourtant, les pouvoirs publics et les différents acteurs de cette nouvelle pratique ont conscience que tout se joue maintenant. Nous avons mené une étude rétrospective descriptive de téléconsultations de population âgée à mobilité réduite vivant en EHPAD et nous avons recensé les différents motifs de consultations de médecine générale traités en télémédecine entre le 20 mai 2011 et le 20 décembre 2012. Ces consultations associaient l’image et le son et se déroulaient en présence d’une partie du personnel de l’EHPAD. Dans la quasi-totalité des cas, le patient et/ou la personne de confiance étaient présents. Notre étude a mis en évidence de nombreux motifs de consultation de médecine générale, qu’il s’agisse de dermatologie, d’angiologie, de gastroentérologie, de cardiologie, de néphrologie, de rhumatologie, de diabétologie, de neurologie, de gériatrie, de psychiatrie ou concernant la prescription médicamenteuse. La télémédecine paraît être une pratique adaptée aux pathologies prises en charge en médecine générale. Elle favorise la prise en charge pluridisciplinaire du patient sur son lieu de vie. Cette étude basée sur un petit nombre de cas mérite d’être étendue à d’autres EHPAD et en médecine ambulatoire. [Copyright &y& Elsevier]

Abstract: Plasmids are the main vectors of antimicrobial drug resistance and virulence genes, especially in Enterobacteriaceae. Identification and classification of plasmids is essential for analysis of their distribution. The most widely used typing method is PCR-based replicon typing (PBRT). A new classification scheme based on relaxase gene typing has been described recently. We propose a practical application of this method, with the development of a multiplex PCR set targeting relaxase genes found on plasmids most frequently encountered in Enterobacteriaceae. This method, here called “plasmid relaxase gene typing” (PRaseT), was validated with 60 transconjugants and transformants harboring various replicon types. The method was tested with 39 multidrug-resistant clinical isolates including Escherichia coli, Klebsiella pneumoniae and Salmonella enterica subsp. enterica carrying 1–7 replicons as well as with 17 plasmids non-typeable using PBRT; all replicons were tested in parallel with PBRT for comparison. Six multiplex PCRs and one simplex PCR, including 24 pairs of primers, recognized plasmids of groups A/C, B/O, colE, FIA, FIB, FIC, FV, FIIk, HI1, HI2, I1, K, L/M, N, P1α, Q1, U, W, X1, X2, X3 and X4. There was perfect correlation between PRaseT and PBRT results in 31/39 (79.5%) clinical isolates. Moreover, 11/17 (64.7%) plasmids non-typeable by PBRT could be typed by PRaseT. Our set of multiplex PCRs showed high sensitivity and specificity for the classification of resistance plasmids. It has proved complementary to the widely used PBRT and will improve the monitoring of plasmid distribution in every-day practice. [Copyright &y& Elsevier]

Résumé: Propos: Il y a en France 4600 médicaments disponibles sous près de 15 000 formes galéniques. Alors que l’Organisation Mondiale de la Santé incite tous les pays développés à définir leur propre liste de médicaments essentiels, et que celle établie en 2008 par l’Agence française de sécurité sanitaire des produits de santé (Afssaps) est restée totalement confidentielle, un groupe d’internistes a décidé d’établir la ű liste sage Ƈ (wise list) des 100 médicaments leur paraissant essentiels pour traiter 95 % de la pathologie observée en France. Méthodes: Dix internistes ont accepté en juin 2011 d’établir leur liste de 100 molécules indispensables. Une réunion des participants en décembre 2011 a dégagé un premier consensus et chargé cinq d’entre eux de faire des propositions dans les domaines où une dispersion avait été constatée et dans ceux qu’une majorité de participants avait négligé. Après échanges par courrier électronique, une deuxième liste consensuelle complémentaire a ainsi été validée mi-janvier 2012. Résultats: Le premier consensus a retenu 54 molécules (dont neuf molécules retenues par les dix participants) et le second 46, constituant cette ű liste sage Ƈ de 100 médicaments. Cinquante-six de ces molécules sont génériquées en juin 2012. Une comparaison de cette liste avec celles établies dans cinq pays de l’Union Européenne est effectuée. Conclusion: Une telle liste est réalisable. Encore incomplète, elle bénéficiera de l’apport de 14 généralistes qui au travers la France ont établi une liste semblable, en cours de finalisation. La liste finale sera validée à plus large échelle au sein des sociétés françaises des généralistes enseignants et des internistes. [ABSTRACT FROM AUTHOR]

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Series of cases from our own practice as a means of reviewing the basic principles for the treatment of thoracolumbar fractures are presented in this article. Although there are no widely accepted evidence-based standards to guide surgeons in choosing treatment approaches, several basic and logical principles may be applied to each case to first decide whether a patient is best managed nonoperatively or with surgical treatment. If operative management is indicated, the surgeon must decide whether decompression and/or stabilization is indicated, whether surgery should be done from an anterior and/or posterior approach, the timing of surgery, the surgical technique, and how extensive the instrumentation should be. Through a series of case example, the authors emphasize the principles of short fixation for most cases to preserve spinal motion. Successful surgery for thoracolumbar fracture can be achieved for most cases with short anterior or posterior fixation. [ABSTRACT FROM AUTHOR]

Mycotic aneurysms are exceptional complications after intravesical instillations of bacillus Calmette-Guérin (BCG) for bladder carcinoma. We report a patient who underwent an emergency operation for a ruptured carotid aneurysm 16 months after BCG therapy. Postoperative investigations discovered multiple other synchronous aneurysms. Culture of an abscess surrounding the right carotid artery identified Mycobacterium bovis var BCG. The patient improved clinically with antituberculous agents prescribed for 9 months but died from recurrence of bladder carcinoma 16 months later. A mycotic origin should be evoked when an aneurysm is discovered after BCG therapy. Microbiologic investigation of the artery wall is diagnostic. [Copyright &y& Elsevier]

OBJECTIVE: Arterial aneurysms are characteristic of medium-size vessel vasculitis but are a very unusual feature of Wegener's granulomatosis (WG). We describe a typical WG case, complicated by arterial aneurysms and review previously reported cases. METHODS: Medline database search of cases published between January 1978 and July 2006, in English, reporting arterial aneurysms complicating WG. RESULTS: Five years after diagnosis, a 29-year-old man with typical WG developed macro- and microaneurysms located on branches of the hepatic and renal arteries during a disease relapse. The main symptoms were abdominal pain, vomiting, and altered general status. He was successfully treated by coil embolization in combination with prednisone, intravenous mycophenolate mofetil, and high-dose immunoglobulins. Twelve additional cases of WG complicated by arterial aneurysms are reported in the English literature. This represents a life-threatening complication since rupture occurred in half of the patients. CONCLUSIONS: Although small-vessel injury predominates in WG, inflammation of medium-size arteries may occur and lead to aneurysm formation. Abdominal angiography should be recommended when unexplained abdominal pain occurs during a WG flare. Copyright © 2008 by Elsevier Inc. [ABSTRACT FROM AUTHOR]

Abstract: Background: Postoperative bowel obstruction (PBO) plagues patients of all ages after intraabdominal surgery. We examined the incidence, risk factors, and the need for operative intervention of PBO. Methods: We reviewed all children who underwent a laparotomy or laparoscopy. Parameters included age, diagnosis, type and number of procedures, complications, time interval to PBO, treatment of PBO, morbidity, and mortality. Results: From 2001 to 2005, 2187 abdominal operations were performed. Overall, 61 patients (2.8%) developed a PBO; 43 (70.5%) required reoperation. Postoperative bowel obstruction was more common in patients younger than 1 year (28/601, 4.7%) compared with older children (33/1586, 2.1%; P = .01, β = .80). In infants, PBO was not influenced by initial diagnosis (P = .26) or whether the initial operation was clean/clean-contaminated or contaminated/dirty (P = .12). In children older than 1 year, nonoperative treatment was more likely to be successful if PBO occurred within 12 weeks of initial operation (12/16 vs 3/14; P = .01). In contrast, all but one infant (16/17) with early PBO required reoperation. Conclusion: The incidence of PBO is significantly higher in newborns and infants than in older children (who have rates comparable to those reported in adults). Infants are significantly more likely to require operative intervention, particularly if PBO occurs early after the initial operation. [Copyright &y& Elsevier]

Abstract: Background: Most congenital cystic lung lesions (CCLLs) do not require in utero or perinatal intervention. The management of asymptomatic lesions is controversial: the theoretical risk of infection and malignancy is offset by whether thoracotomy in asymptomatic children is justified. We examined our recent experience and the role of minimally invasive surgery. Methods: We analyzed the pre-, peri-, and postnatal findings of all consecutive CCLLs diagnosed between 1997 and 2005. We reviewed records for pre-, and postnatal imaging, management, and outcome. Results: Thirty-five CCLL were diagnosed prenatally. Since 2000, all asymptomatic lesions were removed endoscopically at 6 to 18 months (thoracoscopy for 6 extralobar sequestrations, 3 intralobar sequestrations/congenital cystic adenomatoid malformations, 5 bronchogenic cysts, and retroperitoneal laparoscopy for 2 intraabdominal sequestrations). Congenital cystic adenomatoid malformation elements were present in more than 70%. Two abdominal lesions have regressed, and 2 patients are awaiting intervention. One symptomatic infant underwent thoracotomy for congenital lobar emphysema. Conclusions: It has been argued that the risks associated with congenital lung lesions (infection and malignancy) justify intervention in the asymptomatic patient. In our experience, all these lesions could be safely removed using endosurgical techniques. Counseling of (future) parents should be updated to include minimally invasive surgery in the management algorithm. [Copyright &y& Elsevier]

Abstract: Background: Increased utilization of computed tomography (CT) has led to a rise in the diagnosis of pulmonary contusion. Its clinical significance, in the absence of findings on chest radiograph (CXR), has not been defined. This study examines the clinical course of patients with CT-only diagnosis of pulmonary contusion and compares it with that of patients with CXR-proven pulmonary contusion. Methods: The trauma database identified all children undergoing chest CT for blunt thoracic trauma during a 3-year period. Records were reviewed for age, mechanism of injury, Injury Severity Score (ISS), length of hospital stay (LOS), need for intensive care unit admission, and need for endotracheal intubation. A pediatric radiologist reviewed all films in a blinded fashion. Statistical analysis was performed using analysis of variance and Fisher''s Exact test for 2 × 3 tables. Results: Eighty-two patients were identified. There were no CXR-positive, CT-negative cases. A CT diagnosis of pulmonary contusion was made in 46 patients. Of these, 31 had a contusion on CXR as well (CXR+ group) and 15 had a normal CXR (CT+ only group). Mean ISS score did not differ significantly between the two groups (27 ± 12.3 and 22 ± 10.3, respectively). Thirty-six patients had a normal CT (control). Mean LOS was significantly longer in the CXR+ group (13 ± 12.0 days) than in the CT+ only and control groups (5 ± 3.6 and 9 ± 9.5 days, respectively; P < .01). The percentages of children requiring intensive care unit admission and intubation were also significantly higher in the CXR+ group. Conclusion: The finding of pulmonary contusion by CT alone does not increase patient morbidity and appears to be of limited clinical significance. [Copyright &y& Elsevier]

The key to uncomplicated spinal surgery relies on the perfect knowledge of spinal anatomy. The following chapter will review applied lumbar, sacral, and pelvic anatomy pertinent to all aspect of lumbo-sacral spinal surgery. Our objective is to provide to the spinal surgeon the relevant spinal anatomy required to undertake spinal surgery. An example of such applied anatomy is the ability of identifying the intersection of the mid-transverse process and the mammillary processes, coupled with the knowledge that the orientation of the pedicles converge in an incremental fashion by 5° per level from T12 (0° neutral) to L5 (25°), thus allowing one to safely proceed to instrument the lumbar spine with pedicle screws. [ABSTRACT FROM AUTHOR]

Parmi les facteurs irrationnels intervenant pour modifier la prescription médicale, la notion de « pression de prescription » s’avère importante bien que peu étudiée. Dans ce travail, nous avons voulu quantifier l’importance de cette « pression de prescription » et rechercher un certain nombre de facteurs favorisants. Durant 599 consultations de médecine générale, nous avons relevé systématiquement une « pression de prescription » définie comme toute demande explicitement formulée par le patient, venant interférer avec la stratégie diagnostique et/ou thérapeutique décidée par le médecin. Nous avons recueilli les informations concernant 246 hommes et 353 femmes (âge moyen 45,4 ans). Cent quarante-deux patients sur 599 ont exercé une « pression de prescription », soit 23,7 % (intervalle de confiance [IC] 95 % : 20,7-27,4 %). Si on considère les patients uniquement âgés de 18 ans, cette valeur augmente : elle concerne 122 patients sur 452 (27,0 % [IC 95 % : 23,0-31,4 %]). La « pression de prescription » concerne en premier lieu les médicaments (17,3 %), puis les actes de biologie (2,5 %), les arrêts de travail (2,0 %), les demandes d’imagerie (1,1 %), les demandes de certificat médical (1,0 %), les actes de kinésithérapie (0,8 %), les consultations spécialisées (0,6 %). Les médicaments faisant le plus souvent l’objet d’une « pression de prescription » sont représentés par les antalgiques (27,6 %), les anti-inflammatoires (non stéroïdiens et stéroïdiens) [17,1 %], les médicaments de la sphère gastroentérologique (16,2 %), les anti-infectieux (14,3 %), les vitamines et « antiasthéniques » (11,4 %), les antiseptiques (10,5 %), les médicaments de la sphère ORL (9,5 %), les médicaments « vasodilatateurs » et « veinotoniques » (8,6 %). Les pressions concernant les médicaments neuropsychiatriques ne concernent que 7,5 % des demandes de médicaments. Les facteurs favorisants de cette « pression de prescription » correspondent à l’âge (plus la population est âgée, plus la « pression de prescription » s’élève) et, dans une moindre mesure, au genre féminin (les femmes ont tendance à exercer plus de « pression de prescription » que les hommes). La notion de « pression de prescription » représente un axe futur de travail en Pharmacologie Sociale. Mots clés : pression de prescription, médecine générale, médicaments, antalgiques, anti-inflammatoires, anti-infectieux, Pharmacologie Sociale Abstract Among the irrational factors involved in medical prescribing, few studies have investigated the problem of the ‘pressure to prescribe’. The aim of the present work was to quantify this ‘pressure to prescribe’ and to investigate some potential predisposing factors. Each instance of a patient’s behaviour suggesting ‘pressure to prescribe’ was registered for 599 outpatient general-practice clinics. ‘Pressure to prescribe’ was defined as each clear request by a patient that counteracted the diagnostic and/or therapeutic strategy of physicians. The study included 246 men and 356 women (mean age: 45.4 years). ‘Pressure to prescribe’ was found in 142 patients, i.e. 23.7% (95% confidence interval [CI]: 20.7-27.4%). Among patients aged >18 years, the percentage increased: 27.0% (CI 95%: 23.0-31.4%), i.e. 122 of 452 patients. ‘Pressure to prescribe’ was first observed with requests for drugs (17.3%), biological tests (2.5%), sick-leave from work (2.09%), radiological procedures (1.1%), medical certificates (1.0%), physiotherapy (0.8%), and referral to a medical specialist (0.3%). The drugs most often associated with ‘pressure to prescribe’ were analgesic (27.6%), nonsteroidal plus steroidal anti-inflammatory (17.1%), gastroenterological (16.2%), anti-infectious (14.3%) drugs followed by vitamins plus antiasthenic (11.4%), antiseptic (10.5%), nasal decongestant (9.5%), ‘vasodilatory’ plus ‘veinotonic’ (8.6%) drugs. Pressure to prescribe neuropsychiatric drugs involved only 7.5% of requests. Two predisposing factors were identified: age (with an increased ‘pressure to prescribe’ for older people) and, less importantly, female gender (women tended to exert more ‘pressure to prescribe’ than men). Analysis of ‘pressure to prescribe’ is an important topic in the area of Social Pharmacology. Keywords: pressure to prescribe, general practitioners, drugs, analgesics, anti-inflammatory drugs, anti-infectious drugs, Social Pharmacology Texte reçu le 17 juin 2003 ; accepté le 7 octobre 2003 [ABSTRACT FROM AUTHOR]

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The study of the dynamic sorption isotherms of parchment coffee revealed that the maturity stage has an effect on the water availability of the bean, and that such dependence might affect the bean drying. The cultivar had an impact on the dispersion at high water activity values (a w > 0.8); hence, depicting dissimilar conditions before the drying is actually performed. The Guggenheim-Anderson-de Boer (GAB) sorption model revealed that stages 2, 3 and 7 present higher monolayer moisture content (X m), K values close to one for all stages indicated a multilayer behaviour close to free water mobility and low C values for stages 2 and 7 represented a stronger water binding to the monolayer for these stages. Under static conditions and the drying conditions defined according to the moisture content of the beans, the unripe stages depicted an accelerated water activity decay that might promote stalling after roasting due to low values of the attribute (a w < 0.4). In a second phase of the study, the dielectric spectroscopy technique was used to evaluate the effect of the maturity and the drying category, and the response depended on the drying category rather than on the maturity stage except for the drying category with the higher moisture content range. The dielectric constant was utilized to predict both the moisture content and the water activity of the beans. The kernel-based ridge method with radial basis function for the moisture content and sigmoid function for the water activity model had good performance (R2: 0.82–0.88). A feature selection model based on a pipelined framework to include the whole spectra selected the 0.3 GHz to 0.9 GHz range as the optimal estimator, which was consistent with the performance of the single frequency models at 0.3 GHz. Although the errors were lower for the feature selection model, the single frequency is preferable as it has minimal complexity. As a non-linear dependence was evidenced, the machine learning strategies were necessary to respond to this condition. Consequently, the technology could be considered for the assessment of the water features of the bean during coffee drying or the storage of green coffee. • The dynamic sorption isotherms revealed that maturity might have an effect on coffee drying. • Stalling and bean spoilage for unripe stages is possible due to accelerated water activity decay. • Maturity affects the dielectric response of parchment coffee at high water activity values. • Radial basis and sigmoid functions were employed to model the water features of parchment coffee. • Single frequency models are preferred over feature selection due to similar accuracy and reduced complexity. [ABSTRACT FROM AUTHOR]

Texte reçu le 6 mars 2003 ; accepté le 24 juin 2003 Cas notifié au CRPV de Toulouse, le 13 janvier 2003 Mots clés : syndrome de fuite capillaire, piroxicam, angiœdème médicamenteux Keywords: capillar escape, piroxicam, drug-induced angioedema [ABSTRACT FROM AUTHOR]

Eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss) is a multisystem syndrome associating asthma, hypereosinophilia, and signs of peripheral vasculitis. We report the case of a 21-year-old man admitted for cardiogenic shock revealing a severe left and right ventricular dysfunction. Hypereosinophilia, history of asthma, and peripheral neuropathy strongly suggested the diagnosis of EGPA. Cardiac magnetic resonance imaging confirmed heart involvement with a diffuse subendocardial late gadolinium enhancement. The patient was successfully treated with systemic corticosteroids, intravenous cyclophosphamide, and inotropes. At 6-month follow-up, the patient is doing well. Cardiac involvement is rare in EGPA and often carries poor prognosis. Specific early steroid therapy may improve hemodynamic at short term, allowing postponing the need for circulatory mechanical support or heart transplantation. [ABSTRACT FROM AUTHOR]

Introduction Les autorités de santé incitent actuellement au soutien et à l’évaluation des expériences d’élaboration et d’utilisation des listes de médicaments essentiels, inspirée de l’expérience conduite en Suède, comme outil d’aide à la prescription médicale. En juin 2011 un groupe de dix internistes a décidé de réaliser une telle liste de médicaments, limitée à 100 molécules, capable de traiter la plupart des maladies observées en France chez l’adulte. Cette liste a été publiée en août 2013. En juin 2013 le groupe « médicaments essentiels » de la SNFMI, constituant le groupe d’initiative, a décidé de solliciter les membres de celle-ci pour réaliser une validation nationale de cette liste et éventuellement l’implémenter. Matériels et méthodes Une enquête Delphi a été menée auprès d’un groupe d’experts formé par appel à participation aux 980 membres titulaires de la SNFMI en France métropolitaine en 2013. Il leur a été demandé de valider grâce à un questionnaire anonymisé en ligne une liste de médicaments leur « paraissant essentiels pour traiter (en ville et/ou à l’hôpital) les pathologies observées chez l’adulte en France, en excluant les vaccins, les traitements destinés à l’hématocancérologie et les traitements spécifiques éventuels des maladies rares (atteignant moins de 5/10 000 habitants) ». Cette liste, obtenue après soustraction des molécules à visée onco-hématologique de la liste des 100 médicaments essentiels sélectionnés par le groupe d’initiative, comportait 87 molécules. Leur validation par le groupe d’experts a été effectuée selon le consensus formalisé par l’HAS en 2010. Le degré de consensus a été également analysé selon les méthodes Delphi publiées dans la littérature (très bon et bon consensus). Au premier tour de l’enquête chaque participant pouvait proposer 3 nouvelles molécules et faire les commentaires de son choix. Les deux premiers tours de l’enquête ont été effectués par voie électronique, le troisième tour par voie postale. Au décours de l’enquête, le groupe d’initiative était chargé d’analyser la liste avec possibilité de rajouter des médicaments manifestement manquants (omission prévisionnelle liée au fait que certaines maladies pourtant fréquentes sont rarement prises en charge par les internistes). Résultats Sur les 980 membres titulaires de la SNFMI sollicités, 101(10,3 %) ont accepté de participer. Parmi eux, 76,9 % ont répondu au premier tour, validant 26 molécules et en proposant 15 de plus (suggérées par au moins trois participants). Au deuxième tour, 90 % ont répondu, validant 9 molécules de plus. Pour le dernier tour, 89 % des 101 participants ont répondu, permettant de valider 28 molécules. A la fin des trois tours, 63 molécules ont été validées selon la méthode HAS. Le groupe d’initiative a analysé les résultats de l’enquête. Parmi les molécules non validées, 12 ont fait l’objet d’un très bon consensus et 10 d’un bon consensus. Après discussion (molécules devenues obsolètes, ajout de molécules manifestement manquantes) une liste complémentaire de 24 molécules a été constituée. Conclusion Il s’agit d’une première démarche de validation d’une liste de médicame [ABSTRACT FROM AUTHOR]

Abstract: Long-term treatment with glucocorticoids results in many adverse effects. Prevention of osteoporosis is well codified, but prevention of other adverse effects is not. If there is some consensus on the prevention of glucocorticoid-induced adverse events, there are also many habits since interventional studies are lacking. A low caloric and low carbohydrate diet as well as a regular physical training are certainly necessary to avoid lipodystrophy, weight gain and diabetes mellitus. Some patients benefit from the repeated intervention of a dietetic or nutrition specialist. Physical training is often neglected though it is efficacious to limit severity of glucocorticoid-induced myopathy and probably to reduce vascular risk. Low sodium intake has no effect on lipodystrophy and its efficacy to prevent hypertension is doubtful. Benzodiazepines may be useful against anxiety, insomnia and nervousness when these symptoms are cumbersome. Anti-ulcer drugs are generally not indicated because glucocorticoids are not ulcerogenic. Hypokaliemia rarely occurs, so we prefer controlling serum potassium level 1 and 3months after glucocorticoid initiation rather than systematically prescribe potassium supplementation. Patients on glucocorticoids are at increased risk for cardiovascular events. Due to the lack of studies specific to patients on long-term glucocorticoid therapy, the rules for the prescription of statins are the same as in the general population. There is no known prevention for cutaneous atrophy. However, use of adhesive tape should be strictly avoided when skin atrophy is severe. Prevention of infections is developed elsewhere. [Copyright &y& Elsevier]

Introduction Depuis la publication du Livre Blanc de la médecine interne en France en 2004, aucune étude nationale n’a évalué l’état de la médecine interne hospitalière française. Les objectifs de cette étude étaient de préciser l’environnement local des services de médecine interne en 2015, quantifier l’activité d’accueil post-urgences, évaluer l’organisation structurelle, les moyens médicaux à disposition, leur évolution récente et future. Matériels et méthodes Étude réalisée en juillet 2015 auprès des services de médecine interne métropolitains. Un questionnaire adressé aux médecins internistes exerçant en secteur hospitalier a été transmis par l’intermédiaire de la SNFMI et de son fichier. Ce questionnaire standardisé visait à préciser la localisation géographique du service et évaluer son environnement local, ses orientations thématiques secondaires, son organisation fonctionnelle, le nombre de médecins et leur statut, les évolutions récentes et futures. Après élimination des doublons, les données ont été saisies et analysées grâce au logiciel Epi Info 7. Résultats Quatre-vingt-quinze questionnaires ont été recueillis, représentant 88 services de médecine interne répartis sur l’ensemble du territoire métropolitain. Dans 63 % des cas, le service était situé dans un CHU ou un CHR et, dans 26 % des cas à Paris ou en Île-de-France. Au sein du centre hospitalier, l’existence d’un autre service de médecine interne (45,4 %), d’un court séjour gériatrique (85,2 %), d’un aval des urgences géré par un urgentiste (53,4 %) ou d’un service d’immunologie clinique (35,2 %) était rapportée. Le nombre moyen de lits affectés à l’activité post-urgences, dans le cadre d’une unité dédiée ou non, était de 18 ± 23,4 (0–87), avec un nombre moyen de lits d’hospitalisation complète par service de 33,7 ± 18,9 (5–107). La répartition du nombre de lits en fonction de la situation géographique du service et de son appartenance ou non à un CHU, ainsi que celle des médecins séniors et des internes sont présentées dans le Tableau 1 . En termes d’évolution, 31 % des services déclaraient avoir perdu un ou plusieurs postes médicaux depuis 2012 et 44,3 % une création de poste prévue. La création d’une unité concurrentielle de gériatrie (17,0 %), de post-urgences (13,6 %), de médecine polyvalente (10,2 %) et d’immunologie clinique (3,4 %) était rapportée. Par rapport aux autres services médicaux de leur établissement, 51,1 % des services de médecine interne déclaraient prévoir des difficultés futures spécifiques. Conclusion En 2015, l’activité des services de médecine interne est souvent placée dans un environnement concurrentiel qui semble devoir s’accentuer dans le futur. L’activité d’aval des urgences est quantitativement importante. Alors que des moyens médicaux ont été perdus dans un tiers des services depuis 2012, moins d’un service sur deux attend ou espère une création de poste. [ABSTRACT FROM AUTHOR]

Résumé Propos La cryofibrinogénémie est méconnue et les études peu nombreuses. L’objectif de notre étude était de décrire la fréquence, les manifestations cliniques et les pathologies associées chez les patients ayant une cryofibrinogénémie isolée. Méthodes Il s’agit d’une étude monocentrique rétrospective. Ont été inclus, à partir de la base de données du laboratoire d’immunologie, les patients ayant eu une cryofibrinogénémie isolée entre le 1 er janvier 2011 et le 31 décembre 2012. Résultats Deux cent quatre-vingt-une recherches consécutives de cryofibrinogénémie ont été répertoriées. Soixante-treize patients avaient une recherche de cryofibrinogénémie positive : 12 patients avaient une cryofibrinogénémie isolée et 61 (soit 84 %) avaient simultanément une cryofibrinogénémie et une cryoglobulinémie. L’âge moyen était de 59 ± 19 ans, et 7 patients étaient des femmes (58 %). Les manifestations cutanées étaient présentes chez la moitié des patients. Une atteinte rénale était présente chez 7 patients (58 %), une neuropathie périphérique dans 5 cas (42 %) et une manifestation rhumatologique chez 4 patients (33 %). La survenue d’une manifestation thrombotique était signalée chez 7 patients (58 %). La concentration médiane de cryofibrinogène était de 254 ± 304 mg/L. Cinq patients avaient une cryofibrinogénémie secondaire. Le traitement le plus souvent utilisé était la corticothérapie. Conclusion La cryofibrinogénémie est une entité méconnue des cliniciens. La recherche de cryofibrinogénémie est moins fréquente que la recherche de cryoglobulinémie alors que les manifestations cliniques sont proches. La recherche est souvent positive, avec une forte prévalence des événements thrombotiques dans cette population. Ceci souligne l’importance de réaliser des études prospectives concernant les cryofibrinogénémies. Purpose Cryofibrinogenemia is an unknown disorder and studies dedicated to it are limited. The aim of our study was to report on the incidence, clinical manifestations and associated diseases in patients with isolated cryofibrinogenemia. Methods This is a retrospective single-center study. Patients included in this study had a positive and isolated detection of cryofibrinogen between January 1st, 2011 and December 31st, 2012. Identification was possible through the database of the laboratory of immunology. Results Two hundred and eighty-one consecutive orders of cryofibrinogenemia were identified. Seventy-three patients had a positive detection of cryofibrinogenemia. Among them, 12 had an isolated cryofibrinogenemia and sixty-one patients (84%) had concomitant cryofibrinogenemia and cryoglobulinemia. The mean age was 59 ± 19 years. Seven patients were female (58%). Cutaneous manifestations were present in half case. Peripheral nerve involvement was present in 5 cases (42%) and rheumatic manifestations in 4 patients (33%). A thrombotic event was reported in 7 patients (58%). Renal impairment was present in 7 patients. The median cryofibrinogen concentration was 254 ± 304 mg/L. Five patients had a secondary cryofibrinogenemia. The most often prescribed treatment was corticosteroids. Conclusion Cryofibrinogenemia is an unknown disorder. Testing for cryoglobulinemia is more frequent than for cryofibrinogenemia whereas clinical manifestations are similar. Detection of cryofibrinogen is positive in most of the cases, with an important prevalence of thrombotic events in this population. This study confirms the importance of conducting prospective studies on cryofibrinogenemia. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: The frequency of adrenal insufficiency after a prolonged, continuous course of oral high-dose corticosteroids is poorly documented. We evaluated it retrospectively in our internal medicine department. Methods: The patients were included between February 2000 and June 2007 and were administered a Synacthene® 250μg test (ST250) before tapering prednisone dose below 5mg per day. A non-responsive test was defined by a cortisol increase below 18μg/dL, 60min after stimulation. We also studied the risk factors associated with biological adrenal insufficiency by a multivariate logistic regression analysis. Results: Hundred patients were included (mean age: 61.5±16.3 years). Mean initial dose of corticosteroids was 65.5±112mg/d. Forty-five patients failed to respond to the ST250. A normal ST250 was negatively associated with a duration of corticosteroids therapy longer than 19.5 months (OR=0.38 [0.15–0.94]; p =0.04) and positively with an age over 63.5 years (OR=2.5 [1.1–6.4]; p =0.05). Two patients experienced a clinical adrenal insufficiency crisis. Conclusion: Biological adrenal insufficiency is very common after a prolonged course of oral high-dose corticosteroids. The risk does not seem to increase with age. The clinical benefit of a systematic ST250 at the time of corticosteroids withdrawal followed by hydrocortisone substitution if the test is non responsive remains unknown, and this practice is still a matter of debate. [Copyright &y& Elsevier]

Abstract: Introduction: There are insufficient data regarding the efficacy and safety of vaccination in patients with auto-immune disease (AID) and/or drug-related immune deficiency (DRID). The objective of this study was to obtain professional agreement on vaccine practices in these patients. Methods: A Delphi survey was carried out with physicians recognised for their expertise in vaccinology and/or the caring for adult patients with AID and/or DRID. For each proposed vaccination practice, the experts’ opinion and level of agreement were evaluated. Results: The proposals relating to patients with AID specified: the absence of risk of AID relapse following vaccination; the possibility of administering live virus vaccines (LVV) to patients not receiving immunosuppressants; the pertinence of determining protective antibody titre before vaccination; the absence of need for specific monitoring following the vaccination. The proposals relating to patients with DRID specified that a 3–6 month delay is needed between the end of these treatments and the vaccination with LVV. There is no contraindication to administering LVV in patients receiving systemic corticosteroids prescribed for less than two weeks, regardless of their dose, or at a daily dose not exceeding 10mg of prednisone, if this involves prolonged treatment. Out of 14 proposals, the level of agreement between the experts was “very good” for eleven, and “good” for the remaining three. Conclusion: Proposals for vaccine practices in patients with AID and/or DRID should aid with decision-making in daily medical practice and provide better vaccine coverage for these patients. [Copyright &y& Elsevier]

Abstract: Introduction: Giant cell arteritis (GCA) is a granulomatous vasculitis of the large and medium size vessels with a remarkable sensitivity to corticosteroids, although it may be dependent to therapy. In rare cases, a vasculitis of the medium or small-size vessels may mimic, be associated to, or follow GCA. We report a case of GCA dependent to corticosteroids that was followed five years after diagnosis by an alveolar hemorrhage leading to the diagnosis of a possible Wegener''s granulomatosis. Exegesis: A 70-year-old man had a diagnosis of GCA fulfilling the ACR criteria in 1999. Temporal artery biopsy revealed a typical histological pattern. The initial response to corticosteroids was excellent, but the patient became dependent to corticosteroids, so he was given methotrexate from 2002. Severe alveolar haemorrhage occurred in December 2004, leading to the diagnosis of possible ANCA positive, anti-proteinase 3 positive Wegener''s granulomatosis. Conclusion: ANCA-positive vasculitis may complicate the course of GCA. This evolution should be rapidly recognized, because its treatment differs to that of GCA. [Copyright &y& Elsevier]

Abstract: Purpose. – To describe the clinical and radiographic features of patients with primary localized amyloidosis of the urinary tract. Methods. – We report a case of localized amyloidosis of the ureters and bladder. The medical records of four other cases from the French Register of localized amyloidosis were reviewed. Results. – The mean age of three men and two women was 53 years. All patients presented with gross hematuria, four patients presented with renal colic, only one patient had irritative lower urinary tract symptoms. Ureter and bladder were involved in three patients, both ureters in two patients and the bladder only, in one patient. Clinical and radiographic presentations mimicked a neoplasia excluded by histologic analysis. Immunohistochemical study was performed in only two cases and revealed lambda light chain amyloidosis. The median follow-up was eight years. Various treatments were performed, and recurrences occurred in two cases. None of the five patients developped monoclonal gammapathy or systemic amyloidosis. Conclusion. – Primary localized amyloidosis of the urinary tract is a rare disorder and can easily be confused with a neoplasm. The physiopathology is unknown, the prognosis is usually good. There is no specific treatment, and repeated work-up for systemic amyloidosis is unnecessary as local recurrences appear to be the main complication. [Copyright &y& Elsevier]

Purpose. – (1) To describe the causes of muscular symptoms in patients undergoing a muscle biopsy in an internal medicine department; (2) to evaluate the diagnostic value of electromyography (EMG), CPK level and muscle biopsy.Methods. – A retrospective study including 90 patients from June 1995 to March 2001.Results. – The diagnosis were: inflammatory diseases (n = 35), non-organic (n = 24), peripheral neuropathy (n = 8), undetermined organic diseases (n = 7), metabolic diseases (n = 5), toxic diseases (n = 4), infectious diseases (n = 4), amyloidosis (n = 3). Diagnosis value of EMG, CPK and biopsy for organicity were: sensibility: 82%, 47% and 29%; specificity: 46%, 91%, 100%; positive predictive value: 78%, 94% and 100%; negative predictive value: 50%, 40% and 36%. Muscle biopsy is always normal when CPK and EMG are normal. It allows a diagnosis in one out of three cases if EMG and CPK are differing. It is also indicated when CPK are normal and EMG is myogenic.Conclusion. – Numerous diseases account for muscular symptoms. The low rate of diagnostic muscle biopsy imposes a comprehensive clinical approach of the patient and justify the implication of internal medicine physicians in his care. Early intervention of a psychosomatic medicine practitioner in the diagnostic procedure should be evaluated to diminish the number of non-contributory biopsies. [Copyright &y& Elsevier]

Purpose. – To describe the clinical features, biological datas and outcome of patients with systemic sarcoidosis and splenomegaly.Methods. – A retrospective analysis of 17 patients presenting splenomegaly and sarcoidosis with histological proof.Results. – Splenomegaly was clinicaly perceptible in 13 patients, with a spleen size that extended 4 cm or more below the costal margin in 11 patients. It was painful in five cases. The more frequent clinical features are constitutional symptom (fever in 9 cases) and hepatomegaly (N =7). Chest X-ray showed bilateral hilar lympadenopathy in nine patients and no abnormality in five cases. Serum angiotensin converting enzyme levels were elevated in 81% of cases. Thrombopenia (N =5) and hypersplenism (N =5) were also observed. Corticosteroid were given to 88% with a good clinical and biological response including a decrease in the spleen volume. Corticotherapy and splenectomy (performed in two patients to rule out lymphoma) didn’t change outcome of disease. Sarcoidosis is often chronical (82%) and extensive.Conclusion. – Splenomegaly may be present in sarcoidosis. Management is not standardized. Corticosteroid is indicated for symptomatic or massive splenomegaly. Splenomegaly is frequently in chronic and extensive sarcoidosis. [Copyright &y& Elsevier]

Abstract: Introduction: Pericarditis is rarely the presenting manifestation of giant cell arteritis. We report two additional patients. Case reports: Two patients aged over 70 years presented with acute chest pain. Echocardiography evidenced a pericarditis. Laboratory features showed increased level of acute phase reactants. On questioning, both patients had cephalic symptoms related to giant cell arteritis. Temporal artery biopsy histopathology was characteristic of giant cell arteritis in both. Oral prednisone therapy (20mg/D) led to a complete remission with no clinical relapse of pericarditis after a follow-up of one year and three years, respectively. Conclusion: Giant cell arteritis must be evoked in elderly patients with pericarditis because corticosteroids are necessary to avoid ischemic complications of the disease. However, fortuitous association can also be considered. No severe complication of pericarditis has been reported in the literature on corticosteroid therapy. [Copyright &y& Elsevier]

Abstract: Introduction: Pemphigus vulgaris frequently requires corticoids and immuno-suppressive drugs. The disease and the side effects of the drugs severely affect the quality of life, and sometime the vital prognosis of the patients. Other treatments than corticosteroids and immunosuppressive drugs are needed. Exegesis: We report 2 additional cases of pemphigus vulgaris uncontrolled by corticoids and immuno-suppressive drugs that responded spectacularly to rituximab. One patient had a recently onset disease, that was active despite 1,5 mg/kg/day prednisone and 1,5 g/day mycophenolate. She had a complete remission during 15 months after rituximab treatment. At relapse, another rituximab cycle led to a prompt remission. The other patient had longstanding pemphigus vulgaris complicated by cutaneous infections on prednisone (20 mg/d), immunosuppressive drugs and intravenous immune globulins. She had a prompt and complete remission after rituximab. Conclusion: Rituximab seems to be a promising drug for refractory pemphigus vulgaris. The benefit to risk ratio of this drug in this new indication must be precisely documented. [Copyright &y& Elsevier]

Abstract: Introduction: Thoracic neuropathy is rare, and is usually associated with diabetes mellitus. We report a first case of isolated multi-metameric thoracic neuropathy revealing Sjögren''s disease. Exegesis: A 64-year old man consulted for symptoms suggesting a progressive and extensive (from T7 to T10) bilateral thoracic neuropathy. Diabetes mellitus and other causes of neuropathy were excluded. Spinal MRI, electromyography and CSF analysis were normal. Though the patient had no sicca syndrome symptoms, the diagnosis of Sjögren''s syndrome was made on hypergammaglobulinemia, elevated ACAN (1/5000) with anti-SSA specificity and a grade III minor salivary gland biopsy. He improved spectacularly on corticosteroids (prednisone, 1 mg/kg/d). Sicca syndrome became clinically evident in April 2006. Conclusion: Pure sensitive thoracic neuropathy can reveal Sjögren''s syndrome. [Copyright &y& Elsevier]

Introduction. – Histoplasmosis is a tropical fungal infection sharing many similarities with tuberculosis: the transmission by air dropplets, the usually asymptomatic primary-infection, the disseminated infection encountered among immunosuppressed patients and the granulomatous pathological lesions. In France, histoplasmosis is uncommon and may be misdiagnosed as tuberculosis.Observation. – A 78 years old male patient presents with a raspberry-like lesion of the mouth causing difficulties to eat and weight loss of 14 kg. The diagnosis of tuberculosis is evoked because of the presence of a giant-cell granuloma in one of the biopsies. The histoplasmosis serology, requested because the patient stayed in Africa, is positive. Revisions of the pathology put into evidence the presence of spores in histiocytes confirming diagnosis of histoplasmosis. The treatment with itraconazole is effective.Conclusion. – Histoplasmosis is a differential diagnosis of tuberculosis, especially in endemic regions. The histoplasmosis serology can be useful. The reference in diagnosis examinations keeps being the microscopic observation of spores and their mycological growth. [Copyright &y& Elsevier]