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Anti-neutrophil cytoplasmic antibodies (ANCA) are typically found in patients with necrotizing vasculitis, especially Wegener’s granulomatosis for cytoplasmic ANCA (cANCA), which recognizes protein...

Clinical reasoning and treatment challenges within the scope of general practice led to the development of an internal medicine assistance line provided by Nantes University Hospital. The primary outcome of this study was to describe callers' profile, their requests and answers provided.A prospective, cross-sectional, observational, descriptive study was undertaken. For each call were identified the calling physician, her/his specialty and work setting, the call's object and adequacy, the answer provided, the time needed to connect with the assistance line, the time devoted by the internal medicine physician to provide an answer to the request, and whether the assistance line prevented a visit to the emergency room. Each calling physician was then called back to obtain demographic and professional characteristics, and data relating to the call and to the assistance line.Sixty-three days were analyzed and 276 calls identified. The 237 identified calling physicians were mainly females (54%, n=93), with a mean age of 46 years, graduated from Nantes University (65%, n=86), practicing ambulatory general medicine (69%, n=164) in Loire-Atlantique department area (82%, n=176) for a mean duration of 15 years. Calls were mostly associated with diagnostic challenges (61%, n=166) concerning clinical issues (57%, n=155). A sole telephone advice was the main type of answer provided (56%, n=147) and a visit to the emergency room was prevented for 17% of calls.The assistance line activity is adequate with its missions and seems to facilitate patients' healthcare delivery advocating for the development of similar structures in other units. Improvements relating to the information, availability and physicians' training should be considered.

Pulmonary hypertension (PH) may occur in patients with antisynthetase syndrome (ASS) but this association is poorly studied. In this article, we report 4 new cases of PH associated with ASS, and we discuss PH mechanisms in this specific disease.Four patients (3 females, 1 male) with confirmed ASS associated with anti-Jo1 (n=3), anti-PL7 (n=1), and anti-Ro52 (n=3) antibodies were analyzed. They presented with subacute dyspnea in average ten years after they were first diagnosed as ASS. Diagnosis of pre-capillary PH was made (mean of mPAP: 34mmHg): PAH (n=1), group 3 PH (n=2) and PH associated to hyperthyroidism (n=1). Among three patients who received specific PAH therapy, two had significant improvement in both clinical and hemodynamic parameters.During ASS, PH may occur in 5 to 10 % of cases, caused by various mechanisms. Unexplained dyspnea may be due to PH among ASS patients.

Introduction Le syndrome de Kleine-Levin (SKL) est une pathologie rare (1 a 5 cas par million d’habitants) atteignant preferentiellement l’homme (2/3 des cas) a l’adolescence. Ce syndrome est caracterise par des episodes recurrents d’hypersomnie (14 a 20 h de sommeil par jour), d’apathie, de derealisation associees a des troubles des fonctions cognitives ou du comportement (hyperphagie, hypersexualite…) et des troubles psychiatriques. Observation Un homme de 29 ans sans antecedent consultait en medecine interne pour des episodes recurrents inexpliques d’asthenie intense avec hypersomnie. Il decrivait 4 episodes d’hypersomnies d’une dizaine de jours avec un intervalle libre de 2 a 5 ans entre les episodes. La premiere crise etait survenue a l’âge de 20 ans. Il ne prenait aucun traitement ni drogue. Il existait un facteur declenchant a chaque episode (prise d’alcool, privation de sommeil, surmenage professionnel). Chaque episode associait hypersomnie (20 a 23 h/j), derealisation (impression de mort, trouble de la perception thermique), sensation d’ivresse et apathie. Il existait des troubles cognitifs a type de ralentissement psychomoteur, de difficulte de comprehension, de concentration, et une amnesie partielle des faits recents. Il presentait egalement des troubles psychiatriques : anxiete, impressions de « deja-vus », hallucinations visuelles et auditives et une perception de presence associes a des troubles du comportement (irritabilite, agressivite). Il a presente une hyperphagie lors de trois des quatre episodes, avec a l’inverse une anorexie et un amaigrissement au premier episode. Chaque episode etait suivi d’une asthenie et de sensation d’ivresse pouvant durer plusieurs semaines. Il ne persistait aucun trouble en dehors des episodes. Un TDM cerebral et un electroencephalogramme (EEG) realise au cours de l’une des crises etaient sans particularite. L’IRM encephalique, l’EEG, la ponction lombaire et la biologie realises en dehors des episodes etaient normaux. L’histoire clinique et la normalite du bilan permettaient d’evoquer le diagnostic de SKL, 9 ans apres la crise initiale. Discussion Le SKL est une pathologie dont les mecanismes physiopathologiques sont mal connus. L’entree dans la maladie se fait classiquement a l’adolescence, mais il existe des formes de debut infantile ou a l’âge adulte. Les crises sont frequemment declenchees par une infection, une privation de sommeil ou une alcoolisation aigue. Les criteres diagnostic de la classification internationale des troubles du sommeil (American Academy of Sleep Medicine) associent une hypersomnie recurrente avec des troubles comportementaux, cognitifs, une hyperphagie et une hypersexualite. Les examens complementaires (biologie, imagerie cerebrale, EEG) sont normaux pendant et hors des crises, en dehors de frequentes anomalies aspecifiques de l’EEG realise en crise. L’imagerie fonctionnelle objective une hypoperfusion thalamo-hypothalamique, frontale et cingulaire. Les diagnostics differentiels d’ordre neurologique (epilepsie, encephalites…) ou psychiatriques sont exclus devant la normalite des explorations. La prise en charge repose sur l’eviction des facteurs declenchant, les thymoregulateurs (lithium) en cas de recurrence frequente et les psychostimulants (amantadine) en cas de crise, avec une efficacite modeste. L’evolution naturelle tend vers des episodes moins frequents et de duree moins longue, avec une guerison spontanee apres la troisieme decade. L’abstention therapeutique est souvent de mise. Conclusion Le syndrome de Kleine-Levin est une cause rare d’asthenie et d’hypersomnie repetees susceptibles d’etre vu en consultation de medecine interne. L’interrogatoire precis, la presence de troubles neuropsychiatriques lors des episodes et la normalite du bilan doivent faire evoquer ce diagnostic.

Introduction Les vascularites gynecologiques sont rares. Le plus souvent, il s’agit d’une decouverte histologique fortuite a l’occasion d’une hysterectomie effectuee pour une lesion associee (myome, kyste annexiel, neoplasie). Il s’agit le plus souvent d’une vascularite gynecologique isolee. Cependant, des lesions de vascularite gynecologique peuvent parfois s’inscrire dans le cadre d’une vascularite systemique, en particulier une maladie de Horton. Nous rapportons le cas d’une patiente exploree pour une alteration de l’etat general avec syndrome inflammatoire persistant et masse annexielle chez qui la mise en evidence de lesions de vascularite gynecologique conduira au diagnostic de maladie de Horton. Observation Une femme de 67 ans consultait pour une alteration de l’etat general avec un amaigrissement de 12 kg en environ 6 mois. Elle decrivait egalement quelques douleurs scapulaires predominant nettement a gauche, d’horaire mixte, avec des radiographies retrouvant une ascension de la tete femorale gauche en lien avec une rupture partielle de la coiffe des rotateurs. Elle ne presentait aucun symptome usuel d’arterite temporale et l’examen vasculaire etait normal. La biologie retrouvait un syndrome inflammatoire avec une CRP a 61 mg/L, une anemie inflammatoire a 10,8 g/dL. La tomodensitometrie thoraco-abdomino-pelvienne mettait en evidence une volumineuse masse kystique ovarienne droite. Il n’y avait pas de stigmate d’aortite, y compris apres relecture orientee. Une ovariectomie bilaterale est realisee sous cœlioscopie. L’analyse anatomopathologique montrait d’une part un cystadenome sereux benin mais surtout la presence de lesions d’angeite granulomateuse epithelioide et gigantocellulaire non necrosante des vaisseaux de moyen calibre des mesosalpynx. Le syndrome inflammatoire persistait apres la chirurgie. La patiente decrivait de minimes douleurs de la racine des cuisses, sans retentissement fonctionnel. La biopsie d’artere temporale retrouvait des lesions d’arteriosclerose, et une fibrose parietale segmentaire d’allure cicatricielle, de signification incertaine. Le PET-TDM mis en evidence un hypermetabolisme abarticulaire rhizomelique bilateral et une atteinte vasculaire aorto-arterielle etendue, signant le diagnostic de maladie de Horton. Discussion La decouverte d’une vascularite gynecologique est un phenomene rare et quasiment constamment fortuit (0,15 % des hysterectomies/annexectomies toutes causes), les hysterectomies ou annexectomies etant realisees dans la majorite des cas pour d’autres raisons. Dans l’immense majorite des cas il s’agit d’une vascularite localisee, sans evolution vers une forme systemique. Il s’agit alors le plus souvent de lesions de vascularite non granulomateuse, monofocale, en particulier du col uterin. Plus rarement l’atteinte des arteres genitales s’inscrit dans le cadre d’une vascularite systemique. Les patientes sont alors plus âgees, et presentent generalement des signes generaux et/ou un syndrome inflammatoire biologique, comme notre patiente. Sur le plan histologique les lesions sont plus souvent diffuses, touchant l’uterus et/ou les autres organes (trompes, ovaires, parametres). La maladie de Horton est de loin la vascularite systemique la plus souvent en cause. Dans la majorite des cas les patients presentent des signes d’arterite et/ou une pseudopolyarthrite rhizomelique. Conclusion Notre observation illustre un mode de decouverte peu commun de la maladie de Horton, qui merite d’etre connu. Le recours au PET-TDM a permis d’affirmer formellement le diagnostic, malgre la pauvrete du tableau clinique et la negativite de la biopsie d’artere temporale. La decouverte de lesions de vascularite gynecologique est le plus souvent en rapport avec des lesions limitees mais chaque cas doit faire l’objet d’une confrontation anatomo-clinique et biologique et d’un suivi simple de quelques mois afin d’eliminer une vascularite systemique.

Introduction Neuroendocrine tumors are rare, with poor prognosis when not or poorly differentiated. Exegesis The authors report the case of a patient who presented with a metastatic poorly differentiated neuroendocrine tumor that was resistant to chemotherapy. The different sites of the carcinoma were lung, pancreas, kidney and lymph nodes. The patient received no treatment and complete spontaneous remission appeared after 21 months. This remission has been lasting for five years. Conclusion It is the first case of spontaneous remission of a metastatic poorly differentiated neuroendocrine tumor. The evolution and classical sites of neuroendocrine tumors are reviewed. Renal metastasis is also unusual. This report illustrates the fact that neuroendocrine tumors are heterogeneous.

Ethnopharmacological relevance Different parts of Mimosa pigra (MPG) are used in traditional medicine in Madagascar, tropical Africa, South America and Indonesia for various troubles including cardiovascular disorders. Aim of the study To investigate the mechanisms underlying the vascular effects of MPG by assessing in vitro its antioxidant and anti-inflammatory properties, and its vascular relaxing effects, and in vivo, its action on hypoxic pulmonary hypertension (PAH) in rats. Material and methods The antioxidant activity of MPG leaf hydromethanolic extract was determined by using both the 1,1-diphenyl-2-picrylhydrazyl radical scavenging and the oxygen radical absorbance capacity in vitro assays. Anti-inflammatory properties were assayed on TNFα-induced VCAM-1 expression in endothelial cells. The vasorelaxant effect of MPG extract was studied on rat arterial rings pre-contracted with phenylephrine (1 μM) in the presence or absence of the endothelium. In vivo MPG extract effects were analyzed in chronic hypoxic PAH, obtained by housing male Wistar rats, orally treated or not with MPG extract (400 mg/kg/d), in a hypobaric chamber for 21 days. Results MPG leaf extract had antioxidant and anti-inflammatory properties. It induced endothelium-dependent, NO-mediated relaxation of rat aorta and pulmonary artery. In vivo, chronic MPG treatment reduced hypoxic PAH in rat by decreasing by 22.3% the pulmonary arterial pressure and by 20.0% and 23.9% the pulmonary artery and cardiac remodelling, respectively. This effect was associated with a restoration of endothelium function and a 2.3-fold increase in endothelial NO synthase phosphorylation. MPG leaf hydromethanolic extract contained tryptophan and flavonoids, including quercetin glycosides. Both compounds also efficiently limit hypoxia-induced PAH. Conclusions Our results show endothelial protective action of MPG leaf hydromethanolic extract which is likely to be due to its antioxidant action. MPG successfully attenuated the development of PAH, thus demonstrating the protective effect of MPG on cardiovascular diseases.

Aortitis is a serious complication of giant cell arteritis (GCA), because of the risk of aortic aneurism, rupture, or dissection. Aortitis is present either at presentation or, more frequently, occurs as a delayed complication, typically as an aortic aneurism of the ascending part of the aorta. An aortic aneurism may occur in up to 10% of patients. Aortitis is sometimes associated to arteritis of the supra-aortic vessels. Risk factors for aortitis remain unknown. Recent clinical studies indicate that prevalence of aortitis was initially under-estimated. Imaging studies show signs of infra-clinical aortitis in 20 to 65% of cases at diagnosis. Using ultrasonography, thickening of the vascular wall with an hypoechoic halo around the abdominal aorta is suggestive of abdominal aortitis. Positron emission tomography shows a metabolic hypersignal of the aorta in about 50% of patients with giant cell arteritis. Aortic computed tomographic (CT) scan visualizes aneurysmal dilatations, ectasia or focal or concentric parietal thickenings. When present at the time of diagnosis of GCA, these findings seem to be associated with frequent relapses and perhaps with a higher long-term vascular mortality rate. Therefore, we recommend the screening of aortitis lesions at GCA diagnosis by an aortic CT-scan and follow-up. Therapeutic trials should be conducted to try to improve the treatment of aortitis in GCA.

Acquired haemophilia A (AHA) is a rare bleeding disorder, due to the presence of an inhibitor directed against factor VIII (FVIII). About 50% of the AHA are idiopathic, while the remaining 50% are related to an underlying disorder or condition (autoimmune diseases, malignancies, postpartum, etc.).We report on a monocentric retrospective cohort of 39 patients with AHA. Data were collected and compared to recent published data.Thirty-nine patients were admitted for AHA between 1993 et 2011. Mean age at diagnosis was 71.3 years, and we noted a marked male predominance. Although the majority of patients presented a bleeding event at diagnosis (94.9%), the hemorrhagic mortality was low (2.6%). On the contrary, immunosuppressive morbidity and mortality were high in this elderly population. There was a clear correlation between initial FVIII inhibitor titer and complete remission delay. We did not identify prognostic factor for global survival.AHA is a rare but potentially fatal disorder. Rapidity of diagnosis and treatment initiation is crucial. Morbidity and mortality, particularly of infectious cause, due to immunosuppressive treatment, should lead to consider other available therapeutical options.

Idiopathic granulomatous mastitis (IGM) is a rare localized granulomatosis of unknown aetiology that usually affects women of childbearing age. It often mimics breast carcinoma or abscess. Histopathologic evaluation and elimination of the others aetiologies of granuloma play a crucial role in the diagnosis. Its etiopathogeny remains poorly understood, but Corynebacteria might be involved. The disease course is usually protracted, with a significant impact on quality of life. The management of IGM remains controversial, but corticosteroids are usually the first-line treatment.

In this article we focus on two interrelated aspects of the process of learning to teach mathematics for understanding: (a) ideas and practices for teaching procedural knowledge and (b) ideas and practices for teaching conceptual knowledge. We explore one student teacher's ideas and practices, together with the messages about teaching for procedural and conceptual knowledge that were presented by the teacher education program in which the student teacher was enrolled and the placement schools in which she student taught. We reveal a pattern in which the student teacher, her mathematics methods course instructor, her cooperating teachers, and the administrators of her placement schools expressed a variety of strong commitments to teaching for both procedural and conceptual knowledge; but with these commitments, the student teacher taught, learned to teach, and had opportunities to learn to teach for procedural knowledge more often and more consistently than she did for conceptual knowledge. We find that the actual teaching pattern (what was done) was the product of unresolved tensions within the student teacher, the other key actors in her environment, and the learning-to-teach environment itself. We hypothesize that situational supports constructed to emphasize more consistently teaching for conceptual knowledge might help resolve at least some of the tensions, and we suggest that such supports should be developed if the national goal to increase the teaching of mathematics for understanding is to be achieved.

Pulmonary hypertension (PH) is a serious complication of connective tissue diseases. The prevalence of PH in systemic lupus erythematosus (SLE) ranges from 0.5 to 17.5%, depending on whether echocardiography or right heart catheterization is used as the gold standard for diagnosis. The recent guidelines for the diagnosis and treatment of pulmonary hypertension include several potential causes of PH in SLE, including: a primary vasculopathy similar to idiopathic pulmonary arterial hypertension (PAH); left heart diseases; post-thromboembolic disease; hypoxia and fibrosis resulting from interstitial lung disease; and the infrequent SLE-associated pulmonary veno-occlusive disease. The pathogenesis of PAH associated with lupus is yet unclear, but likely includes a role for the genetic background, the presence of antiphospholipid antibodies, and some level of endothelial dysfunction. The evolution of SLE-associated PH is highly variable and difficult to elicit because the published series have used heterogeneous inclusion criteria. Optimal therapeutic management of PAH associated with lupus is unclear because no dedicated randomized controlled trial is yet available. Treatment usually includes arterial pulmonary vasodilators and immunosuppressive agents when the patients have NYHA functional class II, III or IV dyspnea.

Myelitis occurs in less than 5% of the patients during the disease course of systemic lupus erythematosus (SLE). Longitudinal myelitis, characterized by inflammatory involvement of at least four medullar segments, is a particular form of myelitis.We report a 31-year-old woman with SLE, admitted for paraparesia and delirium. Lumbar puncture and MRI led to the diagnosis of longitudinal myelitis. The patient rapidly improved after corticosteroid therapy.Transverse myelitis in SLE patients has been already commonly reported, but longitudinal myelitis is uncommon. Longitudinal myelitis has to be suspected in case of paraplegia or tetraplegia, with sensory defects and bladder dysfunction. MRI shows typically T2 medullar hypersignals. This may result in neurologic sequela. Cyclophosphamide has been used in patients where corticosteroids were inefficient.

This article analyzes from several vantage points a classroom lesson in which a student teacher was unsuccessful in providing a conceptually based justification for the standard division-of-fractions algorithm. We attempt to understand why the lesson failed, what it reveals about learning to teach, and what the implications are for mathematics teacher education. We focus on (a) the student teacher's beliefs about good mathematics teaching, her knowledge related to division of fractions, and her beliefs about learning to teach; and (b) the treatment of division of fractions in the mathematics methods course she took. The student teacher's conception of good mathematics teaching included components compatible with current views of effective mathematics teaching. However, these beliefs are difficult to achieve without a stronger conceptual knowledge base and a greater commitment to use available resources and to engage in hard thinking than she possessed. Further, the mathematics methods course did not require the student teacher to reconsider her knowledge base, to confront the contradictions between her knowledge base and at least some of her beliefs, or to reassess her beliefs about how she would learn to teach. These findings suggest that mathematics teacher education programs should reconsider how they provide subject matter knowledge and opportunities to teach it, and whether and how they challenge student teachers' existing beliefs.

The Gleich's syndrome is a rare disease that causes recurrent angioedema associated with major eosinophilia with good response to corticosteroids. We describe a 53-year-old man who presented with a Gleich's syndrome with a 6-year follow-up and propose a literature review. This case emphazises the favourable prognosis of this disease. In case of poor tolerance of corticosteroids, mepolizumab could be tested.

Sensorineural hearing loss may be due to an autoimmune mechanism. The mechanisms that could induce autoimmune inner ear damage are now better understood, but are not exclusive. Moreover, there is no specific immunologic test available for the diagnosis of autoimmune sensorineural hearing loss, which could also complicate the disease course of other autoimmune systemic diseases. Thus, the incidence of sensorineural autoimmune hearing loss is probably underestimated. The aim of this study was to review the experimental immunologic data in favour of an autoimmune mechanism in this subgroup of sensorineural hearing loss: humoral specific response against inner ear (autoantibodies against a transmembrane transporter) and also cellular response (against cochlin: one of the major proteins expressed in the inner ear). The aim of this review was also to focus on clinical and epidemiological human data that provide evidence for an autoimmune etiopathogeny of some sensorineural hearing loss. Therapeutic options such as immunosuppressive treatments (oral corticosteroids and other immunosuppressive drugs, such as methotrexate and anti-TNFalpha) are also discussed.

Systemic sclerosis-related pulmonary arterial hypertension (PAH) is a severe disease affecting about 1000 patients in France. In 2008, all scleroderma patients are screened for PAH by a yearly cardiac Doppler ultrasonography. The pathogenesis of systemic sclerosis-related PAH is poorly known but it seems that besides common arteriolar remodeling (media hypertrophy, intimal thickening, endothelial proliferation), venular lesions suggesting obstructive venous disease and inflammatory lesions may be also be involved. Prostacyclin and analogues, phosphodiesterase-5 inhibitors (sildenafil) and endothelin-1 receptor antagonists are proposed as specific treatments for systemic sclerosis-related PAH. Unlike bosentan, which is non-selective, inhibiting both ETA and ETB receptors, sodium sitaxentan is highly selective for ETA receptors; this could favor pulmonary vasodilation.

Temporal arteritis is the most common systemic vasculitis of the elderly. A definitive diagnosis is obtained on temporal artery biopsy examination (TAB). However, 30% of TAB yields false negative results. In such cases, diagnosis relies on clinical presentation and exclusion of alternative diagnosis. Conversely, false positive TAB are uncommon.We report two patients who presented with headache, scalp tenderness and increased acute phase reactants, suggesting temporal arteritis, but which turned out to reveal a skull metastase. Temporal artery biopsy performed in one case demonstrated arterial wall inflammation.Clinicians must be aware that a skull lesion can mimick temporal arteritis.

The face is frequently involved in systemic sclerosis. The main stomatologic manifestations include limited mouth opening, xerostomia, skin atrophy, trigeminal neuralgia. The objective of this study was to describe oral and facial manifestations observed in scleroderma patients from our cohort.Between March and October 2006, a stomatologic consultation was included in the follow-up of scleroderma patients seen during consultation or daily hospital in internal medicine or dermatology units. Demographic, clinical and biological data were collected. Stomatologic examination comprised measure of the mouth opening, sugar's and Schirmer's tests, orthopantomogram analysis, and evaluation of the repercussion of symptoms on quality of life using a visual analogical scale (VAS between 0 and 10).This study included 30 patients (women 87 %, mean age 58.6 + or - 13.6 years). Mean duration of systemic sclerosis (n=20 limited cutaneous form, n=10 diffuse form) was eight years. Stomatologic manifestations were: skin atrophy (n=28), peribuccal rhagades (n=25), telangiectasia (n=21), decreased mouth opening (n=20), xerostomia (n=20), xerophtalmia (n=16), periodontal ligament space widening (n=10), bone resorptions (n=2), trigeminal neuralgia (n=1). Xerostomia was considered more discomforting (mean VAS=3.8) than decreased mouth opening (mean VAS=2.6). Xerostomia was the second more discomforting sign of scleroderma and was significantly associated to the limited cutaneous form (p=0.045) and to anticentromeres antibodies expression (p=0.002). Decreased mouth opening was correlated to oesophageal involvement (p=0.025).Oral and facial manifestations are frequently observed in scleroderma patients. These manifestations lead to major functional discomfort, mainly due to decreased mouth opening that seems to be frequently associated to oesophageal involvement. Xerostomia is also frequent and is commonly observed in anticentromere antibodies positive cutaneous limited forms of systemic sclerosis. Evolution of radiographic abnormalities like periodontal ligament space widening (33 % of cases), or osteolytic lesions (7 %) is poorly known.

Inflammatory involvement of extracranial large-sized arteries occurs in 10-20% of patients with giant cell (temporal) arteritis. Aortic involvement may reveal giant cell arteritis or occur as a late-onset complication, and represents one of the most serious manifestation of the disease with the risk of aortic dissection and/or aneurysm rupture. The thoracic aorta is more frequently involved but abdominal aortitis may also occur in giant cell arteritis. To date, few data are available about abdominal aorta changes at the initial stage of giant cell arteritis.This prospective monocentric study was conducted between May 1998 and May 2002, and included 30 consecutive patients with biopsy-proven giant cell arteritis. Standard clinical and biological data were collected. Each patient underwent an abdominal aortic Doppler-sonography that looked for aneurysm, ectasia, thickening of the vascular wall, and hypoechoic halo around the aorta.Among the 30 patients of this study (25 women, 5 men, mean age 68.5 years), 4 (13%) had an abdominal aortic aneurysm, with a low diameter (23 to 27 mm), measuring 2 to 5.5 cm in length. A vascular wall thickening superior or equal to 3 mm was noted in 17 patients (68%). A 4 to 8 mm periaortic hypoechoic halo was found in 10 patients (33%). This halo was present in 3 out of the 4 patients with aneurysm.Aortic involvement is a potentially serious complication of giant cell arteritis. The question of a systematic screening of this complication remains open to discussion. Our study shows that Doppler sonography may detect morphological abnormalities on the abdominal aorta at the initial stage of giant cell arteritis. These abnormalities comprise mild aneurysms, thickening of the vascular wall and periaortic halo, which could correspond to inflammatory locations of the disease. Complementary studies are needed to assess their specificity and their seriousness.

Few data are available on the prevalence and causes of hyponatremia in medical setting and to our knowledge, no recent descriptive study has been performed about hyponatremias in the French Departments of internal medicine.A "one day" descriptive multicentric study was performed in the medicine departments of the France West area. A questionnaire was mailed to physicians who had to take part in a annual regional meeting about "hyponatremias", one month later. Hyponatremia was defined by a blood sodium level under the normal value of the local laboratory. Each internist had to precise for all hyponatremias in course at the study day, the exact value, the discovery circumstances, the mechanisms and etiologies, the associated diseases, the course and treatments.Seventy-four hyponatremias were identified. The overall prevalence was 12,1%. The prevalence of severe hyponatremias (under 120 mmol/l) was 1,1%. These latter represented 9,4% of the whole hyponatremias (7/74). Associated symptoms and diseases, the mechanisms, the suspected etiologies, the course and treatments are described in detail.This multicentric study reports for the first time the prevalence, the clinical and etiological characteristics of hyponatremias coming from Internal Medecine Departments of the West area from France. The overall prevalence is lower in comparison with values usually reported in hospitalized patients, but the frequency of severe and moderate hyponatremias, the mechanisms and the suspected etiologies are identical to those reported in others countries.

Background Churg–Strauss syndrome (CSS) is a necrotizing systemic vasculitis with extravascular granulomas and eosinophilic infiltrates of small vessels. CSS is usually revealed by nonspecific signs of necrotizing vasculitis in a context of late-onset asthma and blood eosinophilia. It is considered a systemic vasculitis with the highest prevalence of cardiac involvement and can lead to rapid-onset heart failure due to specific cardiomyopathy. Pericardial effusion may also occur during CSS and is usually well tolerated. Objective The objective of these case reports was to indicate that CSS may present as tamponade, with or without other visceral involvement. Methods Among CSS patients treated during the past 10 years at 2 French university hospitals, we have identified and described 2 cases revealed by tamponade with pericardial biopsy-proven granulomatous vasculitis. We have also reviewed the international medical literature in PubMed on cardiac involvement in CSS. Results The first case report describes a 66-year-old man who had an isolated cardiac tamponade with both inflammatory syndrome and eosinophilia. Long-term remission was obtained with corticosteroids. The second case report describes a 46-year-old woman whose CSS presented with tamponade and associated central nervous system and myocardial involvement. Remission was obtained with corticosteroids and cyclophosphamide. In both cases, CSS was assessed by histological analysis of a pericardial sample. Conclusions CSS may present as isolated cardiac tamponade. Whereas pericarditis with myocardial injury warrants immunosuppressive therapy, isolated pericarditis without other visceral involvement of poor prognosis only requires corticosteroid therapy.

Ten to fifteen percent of granulomatous hepatitis are idiopathic. If symptoms like prolonged fever are present, empirical treatment is discussed. The goal of this study is to describe the empirical treatment proposed in this situation by French specialists of internal medicine.We conducted a practice investigation among the French national society of internal medicine (SNFMI), using an anonymous questionnaire that related a case of idiopathic granulomatous hepatitis. This questionnaire was proposed to all French internists present at the SNFMI congress in June and December 2004. French specialists of internal medicine had to answer if they would prescribe an empirical treatment and if so, to specify this treatment.Thirty-six French specialists of internal medicine answered to the questionnaire. In the proposed situation, 89% of them initiate an empirical treatment. In 18/36 cases (50%), a first-line anti-tuberculosis empirical treatment is proposed (quadritherapy in 11 cases). In 7 cases (19%), an empirical treatment with prednisone, 0.4 mg/kg/d (N=1) and 1 mg/kg/d (N=6), would be prescribed. Seven internists (19%) would prescribe an empirical treatment with cyclins at the dose of 100 to 400 mg/d. Median duration of the empirical treatment would be 28 days (range: 8-252d). The evaluation parameters mentionned are: fever (69%), weight (59%), seric level of C-reactive protein (59%), and liver biology (53%). In case of failure of first-line empirical treatments, 69% of all questionned internists prescribe a second-line treatments: prednisone at the dose of 0.4 to 2 mg/kg/d (72%), anti-tuberculosis treatments (16%), cyclins 200 mg/d (12%), with a median duration of 28 days. Seven internists (19%) propose to combine two empirical treatments.Faced with a problem of idiopathic granulomatous hepatitis, French internists questionned propose four therapeutics options: no treatment, anti-tuberculosis treatment, cyclins or steroids treatment. First-line anti-tuberculosis treatment is a coherent proposition regarding to the high prevalence of tuberculosis. There are only few data available concerning empirical treatment with steroids or cyclins. Specific proposition of such empirical treatments should be defined.The management of idiopathic granulomatous hepatitis is difficult. Our study shows that therapeutics practices of French internists are heterogenous. The main proposition consists in a first-line anti-tuberculosis empirical treatment, that has to be evaluated after four weeks, and switched with steroids (prednisone, 1 mg/Kg/d) in case of failure. This study is not an expert proposition but contributes to suggest clinical practice guidelines for a rare, complex, heterogenous, and typically internist situation.

Five years after introducing the Permanent Access to Healthcare activity (PASS), it became necessary to analyse how it works.A computerized data collection and assessment system intended to evaluate the PASS health activities has been set up in 11 University Hospitals and ten General Hospitals. From January 1st to June, 30th 2003 data was captured in a computer.The patients requiring medical advice are young (with an average age of 35 years) and present several signs of poverty in terms of accommodation, social relationships and financial means. Besides, almost all of them are uninsured. The PASS public corresponds completely to the created system. Poverty risk factors vary according to the geographic origin. Indeed, French people often suffer from isolation, whereas foreign patients present financial problems. The major part of patients are foreigners and more than a third of them do not speak French, which is an additional obstacle to care. Most of the time, the PASS patients present digestive disorders, nevertheless there are some differences between French and foreign patients. Indeed, foreigners very frequently have digestive and osteoarticluar problems, whereas French patients suffer from psychic disorders and present addictive behaviours. Some patients are sent to physicians downtown (9%) and to external medicosocial assistance centres (39.5%).This study (first one in France) provides us with homogenous data regarding the activities of PASS centres nationwide. The usefulness of computers and its acceptability facilitate data diffusion, with possibilities of adapting to each centre while preserving a common basis.

Considering the consequences of social instability within the health care system has become a public health priority. Each public hospital center in France is obliged to create a unit for welcoming impoverished and vulnerable people (Permanently Maintained Health Care Facilities). The university hospital in Nantes opened a socio-medical out-patient consultation service in November 1999 with the main objective being to bring these patients back into the usual ambulatory care system. The evaluation of the first few months of its operation reveals that one out of every two patients is a young person with a low-income and without any social protection. The possibilities of reorientation towards the regular care system are discussed.

Temporal artery biopsy is performed to confirm the diagnosis of giant cell arteritis. This proof is quite absolute and allows using corticosteroid treatment associated with considerable morbidity in elderly patients. Biopsy is necessary in patient supposed to suffer from giant cell arteritis. Treatment test, which is often difficult to interpret, is done only when temporal artery biopsy is impossible. The biopsy is easy, bilateralisation improves its efficiency. Sometimes the temporal artery biopsy allows to diagnose other vasculitides. Although this biopsy is easy, a strict clinical reflexion must precede its realisation.

Sulfites are compounds which are mainly used in the agricultural, food and pharmaceutical industry as preservatives. The possibility of manifestations of acute hypersensitivity appearing after the ingestion or inhalation of sulfites has already been described several times, over the past twenty years. Amongst these manifestations and indeed the one that is most often present is bronchospasm, which can sometimes be severe. A few cases of occupational intolerance through contact eczema to sulfites have also been published. The idea of occupational asthma to metabisulphates is, on the other hand, extremely recent; its legal recognition only dates back to 1989. The authors present three new cases of occupational asthma to metabisulphites stressing the value of a specific provocation test and the different hypotheses for the pathogenesis of the disorder are reviewed. Preventative measures are always preferable to avoid the development of severe occupational asthma. In all cases, notification of occupational asthma is necessary.

Neuroendocrine tumors are rare, with poor prognosis when not or poorly differentiated.The authors report the case of a patient who presented with a metastatic poorly differentiated neuroendocrine tumor that was resistant to chemotherapy. The different sites of the carcinoma were lung, pancreas, kidney and lymph nodes. The patient received no treatment and complete spontaneous remission appeared after 21 months. This remission has been lasting for five years.It is the first case of spontaneous remission of a metastatic poorly differentiated neuroendocrine tumor. The evolution and classical sites of neuroendocrine tumors are reviewed. Renal metastasis is also unusual. This report illustrates the fact that neuroendocrine tumors are heterogeneous.

The postpartum period is characterized by a rebound in autoimmunity secondary to immune tolerance induced by pregnancy, creating favorable conditions for flare up of Graves' disease or autoimmune thyroiditis. Postpartum thyroiditis is a recognized clinical entity.Six years after onset of Graves' disease treated with antithyroid drugs, a 25-year-old woman had a high serum level of antithyroperoxidase antibodies a few months before she became pregnant. Six weeks after delivery, she developed signs of hyperthyroidism and goiter. The diagnosis of postpartum thyroiditis was retained. Her condition regressed spontaneously to euthyroidism then hypothyroidism.The therapeutic options involved underline the importance of distinguishing between Graves' disease and postpartum thyroiditis. The diagnosis of postpartum thyroiditis is based on history taking, clinical findings, and laboratory tests, especially isotope uptake.

Willebrand's syndrome is rarely acquired. We report four cases associated with lymphoproliferative syndromes.We observed four patients with lymphoid hemopathies who developed acquired Willebrand's syndrome. Two patients had Waldenström's disease (kappa), one had a monoclonal gammapathy of undetermined signification (kappa immunoglobulin M) and the fourth had chronic lymphoid leukemia with mast cell infiltration of the skin. Anti-vWFRCo antibodies were evidenced in only 1 case. Chemotherapy, used in 3 cases, improved hemostasis in one patient. Intravenous immunoglobulins (1 patient) and desmopressin (2 patients) were ineffective. The pathogenic mechanisms and possible therapeutic approaches to acquired Willebrand's syndrome are discussed.Acquired Willebrand's syndrome rarely occurs in association with lymphoproliferative disorders appears to be uncommon but the frequency is probably underestimated because appropriate tests are not always performed. The diagnostic search is important however since the hemostasis disorders due to acquired Willebrand's syndrome could be corrected if appropriate etiological treatment is given.

Thirteen ovarian tumours were the subject of a histochemical and immunohistochemical study using antibodies to collagen IV, III, I, and to laminin. Our study shows that it is not possible to assess the integrity of the basement membrane using simple histochemical methods (P.A.S., Gordon Sweets staining). In contrast, in benign epithelial tumours, a continuous basement membrane may be visualized beneath the epithelium of the cysts using antibodies to collagen IV and to laminin, collagen IV and laminin being two of the constituents of basement membranes. In malignant epithelial tumours, the immunoreactive material of collagen IV type or of laminin type, is arranged in a distinctly discontinuous manner, and is of unequal thickness around the edge of the cysts or of the neoplastic clusters. In three cases of borderline epithelial tumours, the discontinuity of the immunoreactive material (collagen IV or laminin) lying beneath the epithelium of the cysts, is not so pronounced. In comparison with studies carried out on the cervix uteri, this latter type of tumor, because of the constitution of its basement membranes, would appear to occur at a particular moment in their evolution, i.e. when they pass from the intra-epithelial stage and become infiltrative.

International audience; Objective. Renal involvement is a rare event during primary SS (pSS). We aimed to describe the clinico-biological and histopathological characteristics of pSS-related nephropathy and its response to treatment. Methods. We conducted a French nationwide, retrospective, multicentre study including pSS patients fulfilling American–European Consensus Group criteria or enlarged American–European Consensus Group criteria, and with biopsy-proven renal involvement. Results. A total of 95 patients were included (median age 49 years). An estimated glomerular filtration rate (eGFR) of