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Background Besides peripheral cytopenias, bone marrow abnormalities, such as fibrosis, pure red cell aplasia and aplastic anemia have been reported in patients with systemic lupus erythematosus (SLE), suggesting that bone marrow may be a 25 target organ in SLE. Aim Our objective was to describe this bone marrow involvement. Methods This registry is a nationwide retrospective study. Centers provided data concerning medical history, SLE manifestations, type of hematologic disorder, treatments and outcome. Bone marrow aspirations and/or biopsies were transferred for centralized review. Results Thirty patients from 19 centers were included. Central hematologic manifestations comprised bone marrow fibrosis (n = 17; 57%), pure red cell aplasia (n = 8; 27%), myelodysplastic syndrome (n = 3; 10%), aplastic anemia and agranulocytosis (n = 1; 3% each). Bone marrow involvement was diagnosed concomitantly with SLE in 12 patients. Bone marrow biopsies showed fibrosis in 19 cases, including one case of pure red cell aplasia and one case of agranulocytosis and variable global marrow cellularity. Treatments included corticosteroids (90%), hydroxychloroquine (87%), rituximab (33%), intravenous immunoglobulins (30%), mycophenolate mofetil (20%) and ciclosporine (20%). After a median follow-up of 27 months (range: 1-142), 24 patients manifested complete improvement. No patient died. Conclusions This registry comprises the largest series of SLE patients with bone marrow involvement. It demonstrates the strong link between SLE and bone marrow fibrosis. Patients with atypical or refractory cytopenia associated with SLE should undergo bone marrow examination to enable appropriate, and often effective, treatment. Long-term prognosis is good.

Congenital aortic insufficiency is a challenging condition; without appropriate management from birth, it can lead to left ventricle dilation and heart failure. Aortic insufficiency due to leaflet agenesia is rarely described in literature. We report here a case of severe congenital aortic regurgitation that required surgical treatment until the boy reached five-year-old thanks to prenatal diagnosis and adequate medical care since birth. The patient underwent a mechanical Bentall procedure for associated dystrophic pulmonary valve with mild stenosis, ascending aortic aneurysm of 34 mm and anomalous position of the left coronary ostium. We safely performed a rare paediatric procedure with encouraging short-term results.

Objectives Systemic sclerosis (SSc) is a heterogeneous autoimmune disease associated with several antinuclear autoantibodies useful to diagnosis and prognosis. The aim of the present multicentric study was to determine the clinical relevance of anti-fibrillarin autoantibodies (AFA) in patients with SSc. Methods The clinical features of 37 SSc patients positive for AFA (AFA+) and 139 SSc patients without AFA (AFA-) were collected retrospectively from medical records to enable a comparison between AFA- and AFA+ patients. Anti-fibrillarin autoantibodies were screened by an indirect immunofluorescence technique using HEp2 cells and identified by an in-house Western blot technique and/or an EliA test. Results Comparing AFA+ and AFA– patients, AFA+ patients were significantly younger at disease onset (36.9 vs. 42.9; p=0.02), more frequently male (p=0.02) and of Afro-Caribbean descent (65% vs. 7.7%; p

Pressurized water reactor steam generators (PWR SGs) are large components whose main function is to cool the fission reactor by extracting the thermal power conveyed by the primary coolant, and thus to produce steam for the turbine generator. Tube support plate (TSP) clogging phenomena may occur in the SG when iron oxide particles carried in the secondary feedwater get unavoidably deposited inside the SG structure, and specifically in two main regions: on the U-tube outer walls and on the quatrefoil sections of the plates that support the U-tubes. They may reduce its cooling efficiency and impact its dynamic behaviour, thus leading to possible safety issues. Methods based on local inspections of the SG have been designed and are already in use. They produce estimators of the local TSP clogging ratio, during the yearly outage of the plant for refuelling and maintenance. A new method to assess a global estimator of the TSP clogging ratio is presented. This method is based on a 1D physical model of the SG th...

Resume Introduction Les sources actuelles inhabituelles d’exposition au plomb d’origine environnementale comprennent principalement les medecines traditionnelles, ayurvediques ou autres, les cosmetiques traditionnels (khol, surma), et l’utilisation culinaire de vaisselle artisanale, pour le stockage ou la cuisson. Observations Nous rapportons deux observations de saturnisme chez l’adulte, decouverts soit a l’occasion du bilan etiologique de douleurs abdominales paroxystiques, soit a l’occasion du bilan etiologique d’une anemie. Dans les deux cas, l’enquete environnementale conduira a l’identification d’une source principale d’exposition au plomb : une cruche artisanale vernissee dans laquelle macerait une boisson consommee par le patient, le « kefir de fruit » dans un cas, le the « kombucha » dans l’autre cas. Conclusion Il faut savoir rechercher une intoxication par le plomb chez un patient presentant une anemie inexpliquee. Les sources environnementales de plomb peuvent etre multiples ; elles doivent etre hierarchisees au sein de l’enquete environnementale, et parmi elles, les poteries artisanales doivent etre retenues comme une source d’exposition significative lorsque les boissons y sont stockees et peuvent ainsi macerer.

Resume Introduction Nous avons evalue l’interet de la tomographie par emission de positons (TEP) au 18F-fluoro-deoxyglucose (18FDG) pour la recherche d’une neoplasie occulte devant une suspicion de syndrome paraneoplasique (SP). Methode Une TEP au 18FDG a ete realisee chez 31 patients, cliniquement suspects d’avoir un SP. Les SP etaient au nombre de 34 dont 12 maladie neurologique, huit endocriniennes, sept rhumatologiques, une dermatologique et six vasculaires. Nous avons compare les resultats d’une tomodensitometrie (TDM) et de la TEP aux conclusions finales des cliniciens portees au terme du bilan et d’une periode de suivi d’au moins deux mois. Resultats Nous avons obtenu une preuve histologique de cancer pour dix patients, mais n’avons pu identifier le site primitif pour seulement neuf d’entre eux. La TEP a mis en evidence six sites primitifs dont trois qui n’avaient pas ete visualises en TDM. La TDM a mis en evidence quatre sites primitifs dont un qui n’a pas ete visualise en TEP. Dans un cas, la TEP a montre des metastases ganglionnaires alors que celles-ci n’avaient pas ete identifiees par la TDM. Onze causes benignes ont ete conclues parmi lesquelles la TEP a joue un role majeur dans trois cas. Dix causes restent indeterminees a la fin de l’etude. Conclusion La TEP joue un role important dans l’identification d’une neoplasie occulte, devant une suspicion de syndrome paraneoplasique soit en identifiant le site primitif, soit en guidant la biopsie d’eventuelles metastases. De plus, elle peut permettre de reveler des causes non neoplasiques.

Resume La majorite des operations en chirurgie cardiaque du nouveau-ne et de l’enfant necessite une periode d’arret cardiaque afin de realiser le geste operatoire dans de bonnes conditions. Beaucoup d’efforts ont ete faits, afin de preserver au mieux le myocarde durant cette periode d’ischemie. Cependant, la plupart des etudes porte sur le cœur adulte et s’interesse peu a l’enfant et au nouveau-ne. Or, la reponse myocardique a l’ischemie semble differente entre le cœur « immature » en pediatrie et le cœur adulte. De plus, la frontiere entre ces deux etats physiologiques est loin d’etre precise. Par ailleurs, chez l’enfant, la physiologie myocardique des cardiopathies cyanogenes differe de celle des cardiopathies non cyanogenes. Le but de notre etude est d’abord de decrire cette specificite pediatrique et congenitale puis d’effectuer une synthese de la litterature sur les differents types de protection myocardique en chirurgie cardiaque pediatrique.

Resume Introduction L’hypertrichose lanugineuse acquise est une dermatose paraneoplasique obligatoire caracterisee par la reapparition a l’âge adulte d’un lanugo. Nous rapportons un cas ou cette hypertrichose a permis de faire le diagnostic de cancer gastrique. Observation Une femme de 51 ans etait vue en consultation pour une hypertrichose evoluant depuis 3 mois. L’examen clinique a permis le diagnostic d’hypertrichose lanugineuse acquise, ce qui a conduit a la decouverte d’un adenocarcinome gastrique. L’exerese chirurgicale de la tumeur a ete suivie de la disparition de l’hypertrichose, sans reapparition pendant les 13 mois qui ont suivi. Discussion L’hypertrichose lanugineuse acquise est rare. Une cinquantaine de cas a ete rapportee dans la litterature, depuis la premiere description en 1865 par Turner. Ces cas confirment le caractere paraneoplasique obligatoire de cette dermatose. Notre observation est originale car il s’agit de la premiere association avec un adenocarcinome gastrique. Elle se singularise egalement par une evolution strictement parallele de l’hypertrichose lanugineuse acquise et de la tumeur, avec disparition complete de l’hypertrichose dans les semaines suivant le traitement chirurgical, et par une survie prolongee (remission complete 17 mois apres le debut des symptomes). Le mecanisme de l’hypertrichose lanugineuse acquise est inconnu. Deux hypotheses ont neanmoins ete soulevees. L’hypertrichose lanugineuse acquise pourrait etre liee a la secretion par la tumeur d’un facteur serique pour l’instant non identifie, ou a une carence nutritionnelle qui pourrait accompagner ce cancer.

The sarcoidosis-lymphoma syndrome is a recognised entity. However, the presence of granulomas in patients with a haematological disease should not lead too easily to a diagnosis of sarcoidosis. The presence of granulomatous lesions during the follow-up of these patients raises diagnostic and therapeutic issues.We included 25 patients followed by the department of haematology in a French hospital (Centre Léon-Bérard). These patients presented with granulomatous lesions. Patients with a history of sarcoidosis were excluded. We report the type of haematological disease, the time of onset of the granulomatous disease compared to that of lymphoma, associated symptoms, aetiology and outcome. Patients were divided into three groups according to the time of onset of the granulomatous lesions.Granulomatous lesions appeared before the haematological disease in 4 cases, was concomitant in 8 cases and appeared later in 13 remaining cases. The two main subtypes of lymphoma encountered were: diffuse large cell lymphoma (36%) and Hodgkin's lymphoma (28%). Granulomatous lesions were related to the progression of the hematological disease in 11 cases, to sarcoidosis in 4 cases, to infection in 3 cases, to drug allergy in one case, to inflammatory bowel disease in one case, to granuloma annulare in one case and was isolated in 4 cases (no identified etiology). In the group where granulomas appeared after the haematological disease, mean SUV was 11 for the haematological disease versus 6.4 for granulomas.Granulomatous diseases in lymphomas can be due to various aetiologies: infection, reaction to the haematological disease, or systemic sarcoidosis. It is an important challenge for clinicians, who can miss the diagnosis of lymphoma and or conclude to a treatment failure or a relapse. Computed tomography scan (CT-scan) or (18)F-deoxyglucose-positron emission tomography scan can help establish a diagnosis but do not replace biopsy.

To develop French recommendations about the management of vaccinations, the screening of cervical cancer and the prevention of pneumocystis pneumonia in systemic lupus erythematosus (SLE).Thirty-seven experts qualified in internal medicine, rheumatology, dermatology, nephrology and pediatrics have selected recommendations from a list of proposition based on available data from the literature. For each recommendation, the level of evidence and the level of agreement among the experts were specified.Inactivated vaccines do not cause significant harm in SLE patients. Experts recommend that lupus patient should receive vaccinations accordingly to the recommendations and the schedules for the general public. Pneumococcal vaccination is recommended for all SLE patients. Influenza vaccination is recommended for immunosuppressed SLE patients. Live attenuated vaccines should be avoided in immunosuppressed patients. Yet, recent works suggest that they can be considered in mildly immunosuppressed patients. Experts have recommended a cervical cytology every year for immunosuppressed patients. No consensus was obtained for the prevention of pneumocystis pneumonia.These recommendations can be expected to improve clinical practice uniformity and, in the longer term, to optimize the management of SLE patients.

The Fas/Fas ligand and mitochondria pathways have been involved in cell death in several cell types. We combined the genetic inactivation of the Fas receptor ( lpr mice), on the one hand, to the pharmacological inhibition of the mitochondrial permeability transition pore (mPTP), on the other hand, to investigate which of these pathways is predominantly activated during prolonged ischemia-reperfusion. Anesthetized C57BL/6JICO (control) and C57BL/6- lpr mice were pretreated with either saline or cyclosporin A (CsA; 40 mg/kg, 3 times a day), an inhibitor of the mPTP, and underwent 25 min of ischemia and 24 h of reperfusion. After 24 h of reperfusion, hearts were harvested: infarct size was assessed by 2,3,5-triphenyltetrazolium chloride staining, myocardial apoptosis by caspase 3 activity, and mitochondrial permeability transition by Ca2+-induced mPTP opening using a potentiometric approach. Infarct size was comparable in untreated control and lpr mice, ranging from 77 ± 5% to 83 ± 3% of the area at risk. CsA significantly reduced infarct size in control and lpr hearts. Control and lpr hearts exhibited comparable increase in caspase 3 activity that averaged 57 ± 18 and 49 ± 5 pmol·min−1·mg−1, respectively. CsA treatment significantly reduced caspase 3 activity in control and lpr hearts. The Ca2+overload required to open the mPTP was decreased to a similar extent in lpr and controls. CsA significantly attenuated Ca2+-induced mPTP opening in both groups. Our results suggest that the Fas pathway likely plays a minor role, whereas mitochondria are preferentially involved in mice cardiomyocyte death after a lethal ischemia-reperfusion injury.

Resume Introduction. – Le syndrome des antiphospholipides s’associe volontiers a des maladies systemiques. Exegese. – Nous rapportons l’observation d’une patiente de 59 ans presentant un syndrome des antiphospholipides associe a un lupus erythemateux systemique et/ou une polychondrite atrophiante complique d’un syndrome catastrophique des antiphospholipides. Conclusion. – Cette observation a l’issue fatale par sa prise en charge trop tardive montre l’interet des demarches diagnostiques precoces et invasives dans le diagnostic et le traitement des maladies systemiques.

Resume Objectif : Colliger les pratiques d’antibioprophylaxie en chirurgie cardiaque en regard des recommandations de la Sfar. Patients : Adultes operes sous circulation extracorporelle a l’exception des endocardites infectieuses evolutives. Materiel et methode : Enquete adressee en 1999 a 64 centres francais et 70 centres d’Europe Occidentale ; comparaison des pratiques francaises et etrangeres par test de χ 2 . Resultats : Taux de reponses : 87 % pour la France et 67 % pour les centres etrangers. Les centres ayant repondu pratiquent 51 405 interventions annuelles. L’injection a lieu lors de l’induction anesthesique dans tous les cas en France, mais dans 11 % des cas au moment de la premedication a l’etranger ( p p p p p Conclusion : Les recommandations de la Sfar semblent influencer les pratiques francaises dans le choix des antibiotiques et leur duree d’administration mais cette derniere devrait etre raccourcie dans la majorite des centres europeens non francais.

Sarcoidosis is a systemic granulomatous disorder of unknown aetiology. It may rarely affect the gastrointestinal tract.We reported a 54-year-old woman with a delayed diagnosis of duodenal sarcoidosis. She presented with gastric and right upper abdominal pain associated with vomiting and marked weight loss. Abdominal computed tomographic scan showed non-compressive retroperitoneal lymph nodes and histological examination revealed non-caseating epithelioid granulomas typical of sarcoidosis. Diagnosis of duodenal sarcoidosis was obtained at the third gastroscopy. The patient's condition improved quickly with corticosteroid therapy.Gastrointestinal sarcoidosis should be looked for in patients with digestive symptoms and another sarcoid localisation. Furthermore, it is important to repeat gastroscopy to confirm diagnosis because treatment improved most patients.

We hypothezised that patients (cases) who are hospitalized for a major ischemic event--myocardial infarction, stroke, decompensation of peripheral arterial disease--acquire better knowledge than a control population--atheromatous patients without a major ischemic event, patients consulting for a vein disease or a diabetes evaluation, and accompanists--about cardiovascular risk factors (smoking, hypertension, diabetes, dyslipidemia, obesity) and have a better understanding of the usefulness of making changes in their lifestyle (quit smoking, regular exercise, Mediterranean diet, low salt diet, weight control, diabetes care).A questionnaire was proposed at vascular surgery consultations and vascular and cardiac functional explorations, at the M Pavillon of the Édouard-Herriot hospital, Lyon, France. In five months, 395 questionnaires (135 cases and 260 controls) were analyzed.The global knowledge score was statistically higher for cases than for controls (cases 3.23±1.81; controls 2.77±2.03; P=0.037). Cases did not abide by monitoring and dietary rules better, except as regards the management of diabetes. Regular physical activity was statistically more prevalent among controls than among cases. Cases mainly received their information from their doctors (general practitioner for 59% of controls and 78% of cases, cardiologist for 25% of controls and 57% of cases) while controls got their information more through magazines or advertising.Our results show that after a major ischemic event, cases' knowledge of risk factors is better than the rest of the population without improved rules lifestyle changes. This suggests the usefulness of evaluating a therapeutic education program for atheromatous disease.

Infectious aortic aneurysms are rare conditions, being responsible of 2% of aortic aneurysms. Most published results are surgical case series concerning infected abdominal aorta. In this retrospective study, we assessed clinical features and outcome of patients presenting infectious thoracic aortic aneurysms.Diagnosis was based upon a combination of imaging evidence for thoracic aorta aneurysm and evidence for an infective aetiology including a culture of a causative pathogen, or a favourable outcome with anti-infective therapy. Retrospective case series.Six men and one woman were included, with a mean age of 66 years. All the patient presented at least one cardiovascular risk factor or atherosclerosis localisation. Fever (71%) and chest pain (42%) were the most common clinical presenting manifestations. The causative pathogens were: Staphylococcus aureus (N=1), Salmonella enteritidis (N=3) and Candida albicans (N=1). The contrast-enhanced computed-tomography disclosed an aneurysm whose diameter reached more than 50 mm (N=5), that increased rapidly in size (N=5), or presented an inflammatory aspect of the aortic wall (N=4). Management was both medical and interventional: surgery (N=3) or endoluminal repair (N=4). Outcome was favourable in six patients; one patient died from aneurysm-related complications.Clinical manifestations revealing an infectious thoracic aneurysm are variable. Diagnosis should be considered in patients presenting a rapidly-growing aneurysm, especially in the presence of elevated acute phase reactants. Endoluminal repair constitutes a treatment option. The role of FDG-PET for diagnosis and follow-up remains to be defined.

The link between systemic disease and cancer is not fortuitous. An autoimmune disease can represent the starter for developing a non-Hodgkin lymphoma. This is particularly true for Sjögren's syndrome that is associated with the highest risk of lymphoma (odds ratio up to 44). Other systemic autoimmune diseases concerned are systemic lupus with an odds ratio of 4.5 and rheumatoid arthritis with an odds ratio of 2 to 3. It is now well established that high inflammatory activity, rather than immunosuppressive treatment, is the major risk determinant. The association between solid cancer and autoimmune systemic disease is uncommon and concerns in particular scleroderma and lung cancer. Concerning biotherapy-induced cancers, there is no demonstrated increased risk with anti-TNFα (except for cutaneous carcinoma and maybe melanoma) or with tocilizumab and abatacept even if studies with longer follow-up are needed at least for these two last drugs.

Autoimmune diseases may reveal or occur during the course of a neoplasia or its treatment. Autoimmune cytopenia, especially haemolytic anaemia, is common in lymphoproliferative disorders such as chronic lymphoid leukemia. The link between cancer and myositis is well established. Dermatomyositis is associated with an increased relative risk of cancer of 3.4 to 4.4. A combination of detection of antibodies against p155 and TEP-computed tomography may be the best approach to ascertain the presence of occult malignancy in patients with dermatomyositis. A cutaneous or a systemic vascularitis may reveal a cancer, most often a haematological malignancy such as hairy cell leukemia. Paraneoplastic polyarthritis have been described in particular with adenocardinoma of the lungs. Underlying neoplasia should be considered in male smokers patients with new onset polyarthritis and poor health status. The prevalence of autoimmune conditions in myelodysplastic syndromes is 10 to 30%. Vasculitis and relapsing polychondritis are the most commonly reported manifestations. Immune manifestations can also be related to treatment. The most common treatment complications are autoimmune haemolytic anaemia with fludarabine and thyroiditis related to interferon and cervical radiotherapy.

A 4 year old girl is referred to our institution for resection of a nephroblastoma with an extension of the tumor into the lumen of the inferior vena cava. To perform a correct resection of the tumor, the operation was conducted under cardiopulmonary bypass. At the end of the procedure, a bilateral mydriasis was noticed. A CT-scan concluded to a massive venous air emboli. As the procedure was unventful, and no other cause of air emboli was found, the etiology of this emboli is problably retrograde because of the large opening of the inferior vena cava that was required to remove the tumor. To avoid similar case the use of transcranial doppler monitoring may be of interest.

A 62-year-old patient was scheduled for coronary artery bypass surgery because of tritroncular coronary artery disease. The early postoperative period was uncomplicated until the 10th postoperative day when purulent fluid appeared from the sternal wound. Cultures of blood, wound and mediastinal tissues yielded Streptococcus pneumoniae with decreased susceptibility to penicillin G. Despite prompt surgical debridement and appropriate antibiotics, a septic shock with multiorgan failure occurred and the patient died on the 19th postoperative day. Although Streptococcus pneumoniae is uncommonly implicated in postoperative mediatinitis, it frequently leads to multiple organ failure and death.

In vitro fertilisation with embryo transfer (IVFET) is associated with an increased risk of venous thrombosis. In order to determine to which extent the hormonal preparation required for IVFET could be involved in this complication, we have measured major plasma fibrinolytic components in 13 women before and during the ovarian stimulation. After pituitary desensitization with buserelin, plasma levels of fibrin degradation products (FbDP) and fibrinogen were significantly decreased. Tissue-type plasminogen activator antigen (t-PA), plasminogen activator inhibitor 1 antigen (PAI-1), FbDP and fibrinopeptide A remained unchanged. After a subsequent stimulation by human menopausal gonadotropins, FbDP plasma levels remained low, while fibrinogen returned to normal levels. t-PA antigen and PAI-1 antigen plasma levels decreased and were correlated with increased serum estradiol levels. Therefore, estradiol could be involved in the regulation of the fibrinolytic balance to some extent; during IVFET hormonal preparation, a decreased plasma fibrinolytic capacity can be questioned, thus favouring the appearance of a thrombotic event.

Esophageal involvement occurs in about 80% of patients with systemic sclerosis, with a marked diminution of peristaltic pressures in the distal two-thirds of the esophagus. Our aims were to more fully characterize esophageal motility disorders in systemic sclerosis using high-resolution manometry (HRM) and to determine predictive factors of esophageal involvement. Fifty-one patients (46 females) with systemic sclerosis were included in this retrospective study. Esophageal motility was characterized with HRM. The demographic data, esophageal symptoms, presence of other organ involvement, and autoantibody profile (anti-Scl70 antibodies [Scl70], anticentromere antibodies [ACA]) were recorded for all patients. Esophageal body dysmotility was present in 33 patients (67.3%) and was associated with hypotensive esophagogastric junction in 27 patients (55.1%). The velocity of proximal contractions was higher in patients with esophageal body dysmotility compared to patients with normal peristalsis (median 10.8 cm/s vs. 5.5, P = 0.04). The amplitude of middle esophageal contraction but not of distal esophageal contraction was reduced in patients with hypoperistalsis. Diffuse esophageal skin involvement, presence of Scl70 and absence of ACA were associated with esophageal involvement. Esophageal symptoms encountered in 87.5% of patients were not predictive of esophageal dysmotility. This HRM series confirms the high prevalence of esophageal body dysmotility in systemic sclerosis. Diffuse skin involvement, positive Scl70 and negative ACA, but not esophageal symptoms, may predict esophageal body dysmotility.

Thrombotic events may occur in celiac disease. In this study, we analyzed clinical features and risk factors for thrombosis in seven patients who had celiac disease and thrombosis.We retrospectively studied 87 patients with adult celiac disease and identified seven cases of thrombosis. We searched if risk factors for thrombosis were identified and tested retrospectively antiphospholipid antibodies on the serum.In our study, the global prevalence of thrombosis was 8 %, and 5.7 % for spontaneous thrombosis, with venous thrombosis (n=5) or arterial thrombosis (n=1) or both (n=2). The seven patients consisted in six women and one man with a mean age of 44.8 years at time of thrombosis. Thrombotic events occurred before the diagnosis of celiac disease in four cases. In three cases, venous thrombosis was in unusual sites: portal (n=2), splenic vein thrombosis (n=1). In six cases, we identified risk factors for thrombosis, which could be linked to celiac disease: hyperhomocysteinemia (n=1), protein C and S deficiency due to vitamin K deficiency (n=3) and antiphospholipid antibodies (n=2).Such risk factors for thrombosis should be identified in patients in adult celiac disease in order to correct them and add a thromboembolic prophylaxis.

Thirty-two patients with acute, proximal-vein thrombosis were treated with heparin and alteplase (0.25 versus 0.5 mg/kg/24 h during 3-7 days) in a randomized, double-blind, multicenter, European (ETTT) trial. The treatment resulted in a decrease of the venographic Marder's score from 18 (6-25) to 13 (2-24) units (median, range) in Group I (0.25 mg/kg/24 h, n = 15, median decrease 3.0, p = 0.32) and from 17.5 (3-33) to 15.5 (0-27) in Group II (0.5 mg/kg/24 h, n = 16, median decrease 4.0, p = 0.23). Comparison of the sequential venograms could be performed in 14 cases of Group I and in 15 cases in Group II. A minority of patients showed substantial partial recanalization of the initially obstructed veins on the control venogram (one in each treatment group) and most of the control venograms showed either thrombus size reductions (5 in Group I, 7 in Group II) or no change or even deterioration (8 in Group I, 7 in Group II). Major bleedings were observed in 7 patients (7/32, 22%), 5 of them occurring in Group II (5/17, 29%). Thus, the results of the ETTT trial show that the used low dosages of alteplase administered intravenously over 3-7 days in heparinized patients cannot be recommended as a treatment for patients with deep venous thrombosis of lower limbs and/or pelvis. Further studies are needed to define a more suitable dosage regimen of alteplase in this indication.

We report a 39-year-old woman with systemic lupus who presented with recurrent aseptic meningitis secondary to treatment with nonsteroidal anti-inflammatory drugs (NSAIDs). She presented two episodes following ibuprofen administration that were characterized by aseptic meningitis with high protein level in cerebrospinal fluid, and increased serum acute phase reactants. No evidence of an infection or vasculitis was documented. Clinical manifestation resolved rapidly with ibuprofen discontinuation, and corticosteroids therapy was unnecessary. Aseptic meningitis related to NSAIDs reported in lupus patients should be considered because of their specific modality of care and their favourable outcome.

Current unusual environmental sources of lead exposure mainly include traditional medicines, either ayurvedic remedies or others, traditional cosmetics (kohl, surma), and the use of traditional earthenware, for storage or cooking.We report two cases of lead poisoning in adults initially identified by paroxysmal abdominal pain or anemia. In both cases, the environmental investigation evidenced one main source of lead exposure, namely a lead-glazed earthenware jug in which a drink was stored, "kefir" in the first case, and "kombucha" tea in the second one.It is recommended to search for lead intoxication in patients with unexplained anemia. Environmental sources of lead can be multiple. Their relative importance has to be ranked during the environmental investigation and among these, lead-glazed earthenware must be considered as a source of high lead exposure when drinks are stored inside and thus can soak.

Alveolar echinococcosis (AE) is a rare but potentially serious zoonosis for which an early diagnosis is of primary importance. We report the first observation of AE in a cardiac transplant patient infected by hepatitis C virus. He first presented with a single asymptomatic hepatic nodule. The liver biopsy showed an epithelioid granuloma with necrosis. We review the clinical features, diagnosis and outcome of this disease in immunocompromised hosts. In immunocompromised patients living in areas endemic for Echinococcus multilocularis, AE should be included in the differential diagnosis of tumor like lesions of the liver.

To study the initial clinical features and describe the outcome of systemic sclerosis in a cohort of French men.Patients with systemic sclerosis based on Leroy's criteria were included. In this retrospective study we compared a cohort of men to a cohort of women, diagnosed between 1997 and 2005 in departments of internal medicine and rheumatology.One hundred and twenty-one patients were included amongst which thirty-six men. The mean follow-up duration was 6.5 years. The time to diagnosis was significantly shorter in men than in women. Diffuse cutaneous systemic sclerosis, cutaneous ulcers and interstitial syndrome on chest radiograph were more frequent at diagnosis in men than in women. An environmental factor (silica) was observed in only nine men. During the follow-up, incidence of restrictive lung disease was significantly higher in men than in women (37% versus 14% p=0.01) with higher rates of oxygen dependency (22% versus 5% p0.01). Cumulated survival rates in men were 92% at 5 years, 72% at ten years and 43% at 15 years, respectively. The mean survival was 13 years in men (IC 95%: 10-16) versus 23 years in women (IC 95%: 10-36) with no statistical difference (p=0.27).If interstitial and restrictive lung disease, oxygen dependency and diffuse systemic sclerosis were more frequent in men than in women, this data did not provide any evidence of survival difference between men and women with systemic sclerosis.

Acquired hypertrichosis lanuginosa is a form of obligatorily paraneoplastic disease characterised by the recurrence of lanuga hair during adulthood. We report a case in which this hypertrichosis allowed diagnosis of gastric cancer.A 51 year-old woman was seen for hypertrichosis present for 3 months. Clinical examination led to diagnosis of acquired hypertrichosis lanuginosa, which subsequently resulted in the discovery of gastric adenocarcinoma. Surgical excision of the tumour resulted in the disappearance of hypertrichosis with no recurrence during the ensuing 13 months.Acquired hypertrichosis lanuginosa is rare, with only 50 or so cases reported in the literature since the condition was first described in 1865 by Turner. These cases confirm the obligatorily paraneoplastic nature of this particular dermatosis. Our finding is original since it is the first recorded case of association with gastric adenocarcinoma. It is also unique in terms of the strictly parallel development of acquired hypertrichosis lanuginosa and the tumour, with complete disappearance of the hypertrichosis in the weeks following surgical removal of the tumour, and in terms of prolonged survival (complete remission 17 months after the onset of symptoms). The mechanism responsible for acquired hypertrichosis lanuginosa is unknown. Two hypotheses have nevertheless been suggested: acquired hypertrichosis lanuginosa could be associated with secretion by the tumour of an as yet unidentified serum factor, or with a nutritional deficiency that may accompany this form of cancer.

Equivalence trials are actually frequently used to prove non-inferiority in anticoagulant therapy. Equivalence trials consist to demonstrate that two treatments are not too much different. This difference has to be under a margin previously determined. The margin corresponds to an efficacy loss that is defined to be acceptable, in accordance to the advantages due to the new treatment. The aim of this work is to explore the equivalence trial published in the thromboembolic disease by focus on the non-inferiority margin used.We identified published equivalence trials in the venous thromboembolic disease, by a systematic search in Medline. We calculated the efficacy loss by reference with the value of the smallest effect size of the standard treatment compared to placebo.We found 9 equivalence trials used in venous thromboembolic disease. The mean value of the efficacy loss was 434%, and the median value was 357%. Eighty-five percent of the values of the efficacy loss were above 100%.Eighty-five percent of the equivalence trials conclude to equivalence despite a complete efficacy loss of the effect of the standard treatment compared to placebo. The results of equivalence trials should be interpreted warily. The corresponding non-inferiority margin should be chosen more rigorously and by reference with the value of the smallest effect size of the standard treatment compared to placebo.

To examine the results of right heart derivations and clinical outcomes according to preoperative characteristics and operative strategy implemented.Fontan operations were performed in 65 patients (mean age = 10.3 years, 41 males). The majority of cardiopathies were single ventricles (SV) with (49% of patients) or without (26%) tricuspid atresia. A palliative bidirectional cavo-pulmonary (BCP) anastomosis was performed prior to Fontan in 15 patients. Intra-atrial Fontan tunnelling was performed in 43 patients, Kreutzer-type operations in 10, and extracardiac tubes were used in 8 patients. The mean duration of follow-up was 6.1 +/- 0.3 years.The 30-day mortality was 13.8%. Early mortality was higher among patients with SV with than without tricuspid atresia (P0.01), and among patients4 years old. Early reoperations were required in 5 patients, including dismounting in 1, BCP anastomosis after Kreutzer procedure in 1, and tube thrombosis in 1 patient. A single death occurred past 30 days, and late adverse events included protein-losing enteropathy in 1 patient, complete atrioventricular block in 1, and tube thrombosis treated with heparin in 2 patients. At the end of follow-up, 75% were in New York Heart Association functional class I.Our intermediate-term results of Fontan-type operations were satisfactory, and steadily improving. The prognosis was better in patients operated at age 4 or older. A prior BCP anastomosis improved the results. A higher morbidity was observed with intra- than with extra-atrial Fontan procedures. The merit of fenestration procedures with respect to morbidity remains the be evaluated.

Aortic valve sparing operations are now widely accepted for ascending aortic aneurysm surgery. We herein report our experience of the Tirone David procedure in larger indications. From January 1997 to August 2003, 50 Tirone David procedure have been performed on 36 male and 14 female (mean age: 60 +/- 15). Five patients presented a Marfan disease and 4 acute dissections. Grade III or IV aortic insufficiency was frequent (40%). Aortic diameter was not particularly dilated, ranging from 44 to 78 mm (mean: 57 +/- 10 mm). Mean ejection fraction: 57 +/- 10%. Mean left ventricular end diastolic diameter =63 +/- 7 mm. An associated mitral valve repair and 1 coronary bypass were necessary. Mean cross clamp and bypass times =94 min and 122 +/- 28 min respectively. There was one in-hospital mortality. Secondary mortality affected 2 patients (non-cardiac deaths), for a cumulative follow-up of 946 months. During follow-up continence control was always excellent, only 1 bicuspid valve had an aortic insufficiencygrade II. Tirone David procedure gave satisfactory results as regards both aortic ectasia and aortic regurgitation control. We consider it feasible even in case of aortic dissection but caution is required when facing bicuspid aortic valves.

Cardiac thrombosis is a rare complication of Behçet's disease (BD), which may present as a cardiac tumor. Its discovery precedes, in half of the cases, the diagnosis of BD. The high mortality may be associated to postsurgical complications and/or an associated involvement of pulmonary arteries.We present the case of a 31 years old Caucasian French woman, with a history of venous thromboembolic disease, who had surgery after the discovery of a right ventricle tumor. That was an organised thrombus with endomyocardial fibrosis and a diagnosis of Behçet's disease was made after the surgery. The outcome was favourable under medical treatment associating corticosteroids, colchicine and antivitamin K (AVK), without relapse four years later.The discovery of an intracardiac mass in a young patient must evoke the diagnosis of cardiac thrombus and Behçet's disease, even in the absence of predisposing ethnic or geographic factor.

The aim of this retrospective study was to analyse cases of infectious endocarditis (IE) of native or repaired ventricular septal defects (VSD) to determine its incidence, the circumstances of its occurrences, the outcome and prognosis of this complication. From 1966 to 2002, 36 IE occurred in 19 boys and 17 girls: the age at diagnosis was 13.4 +/- 11.8 years; 26 had an isolated VSD and 10 had VSD associated with a minor lesion. Eleven of the 36 cases (30.5%) had been previously operated: repair of an isolated VSD with a patch in 5 cases, associated with a Crafoord procedure for coarctation of the aorta in 2 cases, three times with conservative treatment of associated aortic regurgitation (AR) and with ligature of patent ductus arteriosus (PDA) in 1 case. Twenty-five of the 36 cases (69.5%) had not been operated before: 21 isolated type 1 VSD; 2 VSD + AR, 1 VSD with PDA (undiagnosed), 1 VSD with valvular pulmonary stenosis (PS). The portal of entry was post-surgical in 7 out of 36 cases (19.4%): 4 VSD patches, 2 VSD patches + Crafoord and 1 VSD patch with ligature of PDA. The source of infection was dental in 14 out of the 36 cases (38.9%): one isolated VSD repair with residual shunt, 11 native VSDs, and 2 cases of unoperated VSD + AR. The other infectious causes (15 = 41.7%) were ENT (2 cases), skin (2 cases), gastrointestinal (2 cases), pulmonary (1 case) or unknown (8 cases), on operated lesions (3 VSD patches + AR) or native lesions (12 cases: 10 isolated VSDs, 1 VSD with PSD and 1 VSD with PS). Twelve episodes occurred (33.3%) despite antibiotic prophylaxis, 7 out of 7 post-surgical and 5 out of 14 dental cases. The commonest localisation was the tricuspid valve (10 cases, always in isolated VSD). Embolism was observed in 60% of right heart endocarditis (always multiple) and in 55% of IE of the left heart (single embolism). Early surgery was required in 6 patients (16.7%). The risk of early surgery was higher in patients with VSDs associated with other lesions (4 out of 10 = 40%) than in isolated VSD (2 out of 26, 7.7%, p = 0.027). Thirteen patients underwent secondary surgery after an average interval of 2.96 years, median 0.86 years (from 4 months to 22.8 years) for VSD repair (10 cases), aortic valve replacement (2 cases) and aorto-aortic conduit (1 case). The global follow-up period was 7.4 +/- 8.3 years, from 28 days to 27.9 years (median 3.3 years). Five deaths were observed on average 3.7 +/- 6.2 years after the episode of IE (median 6 months): 2 were early, occurring less than 6 months after IE and directly related to the infective episode. The survival was 97.1% at 1 month, 94.3% at 6 months, 91.4% at 1 year and 86.6% at 5 and 10 years after IE. VSD is a benign cardiac lesion, the prognosis of which can be severely compromised by infectious endocarditis: surgical repair reduces the risk but does not totally exclude it because of minor associated abnormalities. Prophylactic antibiotic therapy and the diagnosis of latent infectious problems, particularly dental, remains essential before and after cardiac surgery.

Antiphospholipid syndrome is frequently associated with auto-immune disorders.We report the case of a 59-years-old woman with an antiphospholipid syndrome associated with systemic lupus erythematosus and/or relapsing polychondritis.This fatal evolution as a consequence of a delayed management shows that fast and invasive steps are required for diagnosis and treatment of systemic diseases.

A 4 year old girl is referred to our institution for resection of a nephroblastoma with an extension of the tumor into the lumen of the inferior vena cava. To perform a correct resection of the tumor, the operation was conducted under cardiopulmonary bypass. At the end of the procedure, a bilateral mydriasis was noticed. A CT-scan concluded to a massive venous air emboli. As the procedure was unventful, and no other cause of air emboli was found, the etiology of this emboli is problably retrograde because of the large opening of the inferior vena cava that was required to remove the tumor. To avoid similar case the use of transcranial doppler monitoring may be of interest.

This paper analyses the contribution of the harmonic flux density in the total iron losses of an induction machine. After presenting the modelling of these losses, simple experimental tests are used which show that the effects are significant in the stator core as well as in the rotor core. Finally, a procedure allows definition of the contribution of each harmonic.