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Although extremely rare, acquired haemophilia A (AHA) can cause severe bleeding, which may be fatal. The underlying causes of autoantibody development are not fully understood. Treatment goals are bleeding control and autoantibody eradication. At the time of our study, there was no consensus on a standard treatment strategy for AHA. Previous data were mainly retrospective or from single-centre cohorts. We conducted a prospective, controlled, registry-based study of patients with AHA in France. The prospective French registry (Surveillance des Auto antiCorps au cours de l'Hémophilie Acquise [SACHA]) collected data on prevalence, clinical course, disease associations and outcomes for haemostatic treatment and autoantibody eradication in 82 patients with a 1-year follow-up. Similar to earlier studies, the prevalence of AHA was higher in the elderly, with two thirds of patients aged >70 years. Around half of AHA cases were associated with underlying disease, most commonly autoimmune disease and cancer in younger and older patients respectively. Haemostatic treatment was initially administered to 46% of patients. Complete resolution or improvement of initial bleeding occurred in 22/27 (81%) rFVIIa-treated patients and in all six cases receiving pd- aPCC. The majority of patients (94%) received immunosuppressive therapy, with complete remission at 3 months in 61% (36/59) and in 98% (50/51) at 1 year. Overall mortality was 33%: secondary to bleeding in only three patients but to sepsis in 10. Bypassing agents were effective at controlling bleeding in patients with AHA. Immunosuppressive therapy should be used early but with caution, particularly in elderly patients. [ABSTRACT FROM AUTHOR]

Acquired hemophilia (AH) is a rare, serious bleeding disorder most often associated with older age and life-threatening complications. The patient care pathway for AH is complex because of the different types of bleeding, the presence of comorbidities, and the heterogeneity of medical specialists who care for these patients. This observational study used the French national PMSI (Programme de médicalisation des systèmes d'information) database to characterize patients with AH in real-life practice and analyze their hospital pathway. In total, 180 patients with AH were identified over a 5-year study period (January 2010 to December 2014), based on three criteria: bypassing agent use, International Classification of Diseases, 10th revision code allocation, and aged over 65 years. Comparison of the incidence rate of AH versus registry data validated the PMSI as an epidemiological database. Rituximab was prescribed more often (60/180; 33.3%) than expected following guidelines and was associated in half of cases to early infections (32/60; 53.3%), surgery procedures were frequently performed during the year before AH onset (29/159; 18.2%), which may suggest a triggering effect, extended hospital stays (median: 20 days) and mortality remaining high (66/180; 36.7%) that occurred mainly during the first month after AH diagnosis. Median costs and number of injections were comparable between recombinant activated factor VII and plasma-derived activated prothrombin complex concentrate. These findings could inform future medico-economic approaches in this AH population (duration of stays, bypassing agents, rituximab use, comorbidities, hospitalizations with infections). [ABSTRACT FROM AUTHOR]

Introduction: The adult emergency department at Rouen University hospital (CHU) welcomes over 100.000 patients per year. In order to streamline unscheduled hospital admissions from the emergency room (ER), a 20-bed pre-hospitalization unit and a centralized bed management system (bed manager, bed manager software, dedicated beds) have been put into place., Patients and Methods: Emergency admissions have increased by (+3.5% between 2017 and 2018) with 20% direct hospitalization from the ER to other conventional units (2/3 in medicine, 1/3 in surgery). In 2018, 3450 patients, of which 54% aged over 75 years have been admitted in the pre-hospitalization unit with an average length of stay of 1.3±1.4 days: 35.4% stayed less than 24hours and 34.8% more than 48hours of which 5.2% stated more than 4 days, 132 patients (3.8%) died, 805 patients (23.3%) were discharged at home, 220 (6.4%) transferred to another facility, and 2287 (66.3%) were secondarily hospitalized in another hospital unit: more than 9 times out of 10 in a medicine unit (internal medicine 30%, geriatrics 27.9%, respiratory medicine 12.2%). This unscheduled emergency hospitalization allowed a daily hospitalization of 50 short stay inpatients beds. It has to be noted that the number of available inpatient beds clearly decreases during the week-ends. The main pathologies were respiratory infections (14.2%), heart diseases (9.7%), metabolic disorders (3.9%), and urinary tract infections (13.6%)., Conclusion: This pre-hospitalization unit associated with a centralized bed management system has clearly improved the unscheduled hospital admissions, in particular concerning the emergency medical sector. The lack of inpatient beds at the week-end and the management of epidemic periods still remain a challenge that has to be taken up., (Copyright © 2019 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Objective: To assess prevalence of aortic involvement in relapsing polychondritis (RP) patients; to evaluate clinical features and long-term outcome of RP patients exhibiting aortitis, aortic ectasia and/or aneurysm., Methods: One hundred and seventy-two RP patients underwent aortic computed tomography (CT)-scan; they were seen in 3 medical centers., Results: Eleven patients (6.4%) had aortic involvement, occurring within a median time of 2 years after RP diagnosis. CT-scan showed isolated aortitis (n=2); the 9 other patients exhibited: aortitis and aortic aneurysm (n=2) or ectasia (n=1), isolated aortic aneurysm (n=4) or ectasia (n=2); aortic localizations were as follows: thoracic (n=6), abdominal (n=2), thoracic and abdominal (n=4) aorta. Patients exhibited: resolution (n=3) improvement (n=3), stabilization (n=4) or deterioration (n=1) of aortic localization. Five patients experienced recurrence of aortic localization; one patient died of aortic abdominal aneurysm rupture. Predictive factors of death related to aortic complications were: aortitis on CT-scan, higher median levels of erythrocyte sedimentation rate. Predictive parameters of aortic relapses were: aortitis on CT-scan and involvement of the abdominal aorta., Conclusions: This study underlines that aortic involvement is severe in RP. Furthermore, we suggest that RP patients exhibiting poor prognostic factors, including panaortitis and higher values of ESR, may require more aggressive therapy., (Copyright © 2017 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.)

Introduction: Multi-component compression, inelastic, short stretched or coated strips bandages, and class 4 stockings have been recommended since 2010 by the French Superior Health Authority as first- and second-line treatment for venous leg ulcers., Objectives: Assess the prescribing habits and knowledge of general practitioners about compression therapy and determine factors predictive of prescriptions consistent with recommendations., Methods: Evaluation study of professional practices conducted during a six-week period. A questionnaire was sent to 210 general practitioners asking them to report their prescribing practices and the last prescription written for a patient with venous ulcers., Results: The response rate was 36.2% (76 responses). For the last patient seen, long stretched bands were prescribed by 50.8% of responders, stockings by 40% and multi-component compression by 7.7%. Stockings were class 2 for 87.7% of the prescriptions. Knowledge of short stretched bands and multi-component compression was reported by 45.8% and 38.9% of general practitioners respectively. Guidelines were followed by 10.8% of the physicians. No factor related to the general practitioner (age, sex, modalities of exercise, experience and education), to the patients (age, sex, under-nutrition, autonomy) or to the leg ulcer (size, exudates, course, complexity of care), was associated with compliance or not with the guidelines. General practitioner training enabled a non-significant improvement of prescriptions (14.3% vs 4%, P=0.24)., Conclusion: Compliance with the guidelines for compression therapy is insufficient in general medicine. Better training and knowledge of modalities for compression therapy could be useful to improve prescription practices and encourage use of multi-component compression and short stretched bandages, known to be more effective and better tolerated., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Numerous autoimmune-inflammatory rheumatic diseases have been associated with accelerated atherosclerosis or other types of vasculopathy leading to an increase in cardiovascular disease incidence. In addition to traditional cardiovascular risk factors, endothelial dysfunction is an important early event in the pathogenesis of atherosclerosis, contributing to plaque initiation and progression. Endothelial dysfunction is characterized by a shift of the actions of the endothelium toward reduced vasodilation, a proinflammatory and a proadhesive state, and prothrombic properties. Therefore, assessment of endothelial dysfunction targets this vascular phenotype using several biological markers as indicators of endothelial dysfunction. Measurements of soluble adhesion molecules (ICAM-1, VCAM-1, E-selectin), pro-thrombotic factors (thrombomodulin, von Willebrand factor, plasminogen activator inhibitor-1) and inflammatory cytokines are most often performed. Regarding the functional assessment of the endothelium, the flow-mediated dilatation of conduit arteries is a non-invasive method widely used in pathophysiological and interventional studies. In this review, we will briefly review the most relevant information upon endothelial dysfunction mechanisms and explorations. We will summarize the similarities and differences in the biological and functional assessments of the endothelium in different autoimmune diseases., (Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

Digital ischemia associated with cancer (DIAC) is increasing in frequency and recent reports have suggested the concept of paraneoplastic manifestation. The aims of this study were to characterize the clinical presentation of DIAC and identify clinical features that could lead physicians to diagnose underlying cancer.From January 2004 to December 2011, 100 patients were hospitalized in the Department of Internal Medicine at Rouen University Hospital, France for a first episode of DI. Fifteen (15%) exhibited symptomatic or asymptomatic cancer during the year preceding or following vascular episode and constituted the DIAC group. Other patients without cancer made up the digital ischemia (DI) group.Median time between diagnosis of cancer and episode of digital necrosis was 2 months [0.25-9]. Diagnosis of DI and concomitant cancer was made in 7 of the 15 patients, while DI preceded the malignant disorder in 2 cases and followed it in 6 cases. Histological types were adenocarcinoma for 7 (46.7%), squamous cell carcinoma for 4 (26.7%), and lymphoid neoplasia for 3 patients (20%). Six patients (40%) had extensive cancer. Three patients were lost to follow-up and 5 patients died <1 year after diagnosis of cancer. Cancer treatment improved vascular symptoms in 6 patients (40%). Patients with DIAC, compared to patients with DI, were significantly older (56 years [33-79] vs 46 [17-83] P =0.005), and had significantly lower hemoglobin and hematocrit levels (12.7 g/dl vs 13.9 g/dl; P =0.003 and 38% vs 42%; P =0.003, respectively). Patients with DIAC had a higher platelet rate (420 vs 300 G/L P =0.01), and 6 patients with DIAC (40%) had thrombocytosis. There was no difference between groups either in C-reactive protein level (12 mg/L vs 5 mg/L; P =0.08) or regarding cardiovascular risk factors, presence of autoimmunity, or monoclonal protein.This retrospective study suggests that DIAC may be more prevalent than previously reported. Outcomes of the 2 diseases were not strictly chronologically parallel. However, in the majority of cases, treatment of the tumor resolved vascular involvement. Our findings suggest that age >50 years and thrombocytosis should alert physicians to consider a possible occult malignancy when digital necrosis occurs.

Introduction: Anticoagulation clinics and computerized management of chronic oral anticoagulation increase the time spent in the therapeutic range with both mortality and morbidity reduction. Usually, anticoagulation clinics are hospital-based medical care centers. We report the five-year results from a general medicine center (CSCTA) using a computer-assisted management., Methods: A prospective cohort observational study of 530 primary care patients that were receiving long term oral anticoagulation., Results: Cardiac arrhythmia (55%), heart valve disease and venous thrombo-embolic disease (30%) represented the most common indications of oral anticoagulation. Patients received fluindione, warfarin and acenocoumarol in 80%, 13% and 7%, respectively. The duration of treatment was at least one year in 54% of the cases, and was at least three years in 25% of the cases. The rate of patients that were in average within the therapeutic range (INR 2-3) was 72%, while 12% were under and 16% over the therapeutic range. Corresponding rates were 82, 17 and 1% respectively for all anticoagulation targets (INR 1.5-4.5). Twenty-six bleeding events (4.9 per 100 patient-years) and four thrombotic complications (0.75 per 100 patient-years) occurred. Life-threatening hemorrhage occurred in 1.3 per 100 patient-years. After the equilibration of the anticoagulation, the average delay of control between two consecutive INR was 19 days., Conclusion: The results obtained with CSCTA were similar to those reported by other anticoagulation clinics regarding hemorrhagic complications and time spent in the therapeutic range. In contrast, thrombotic events were less frequent. Because of the absence of a control group, a medico-economic analysis could not be performed.

The "Standards, Options: Recommendations" (SOR) project has been undertaken by the French National Federation of Cancer Centers (FNCLCC) and is now part of the French National Cancer Institute. The project involves the development and updating of evidence-based Clinical Practice Guidelines (CPG) in oncology. In order to answer questions related to venous thromboembolic events (VTE) treatment and to central venous catheter thrombosis (CVCT) management in cancer patients, the SOR elaborated national guidelines, here presented in a short report. It results of a collaborative work with members from three learned societies (<< Société nationale française de médecine interne >> : SNFMI, << Société française de médecine vasculaire >> : SFMV and << Société française d'anesthésie-réanimation >> : SFAR).

The <> (SOR) project has been undertaken by the French National Federation of Cancer Centers (FNCLCC) is now part of the French National Cancer Institute. The project involves the development and updating of evidence-based Clinical Practice Guidelines (CPG) in oncology. In order to answer questions related to venous thromboembolic events (VTE) treatment and to central venous catheter thrombosis (CVCT) management in cancer patients, the SOR elaborated national guidelines, here presented in a short report. It results of a collaborative work with members from three learned societies ("société nationale française de médicine interne": SNFMI, "société française de médicine vasculaire": SFMV and "société française d'anesthésie-réanimation": SFAR).

Objectives: The present study assessed the outcome of several cases of cryofibrinogenaemia detected in our hospitals during a 10-yr period (December 1996-April 2007), and also attempted to evaluate the clinical manifestations and associated diseases., Methods: We performed a retrospective study in a series of 61 consecutive cryofibrinogenemia patients detected in our hospitals., Results: In the 61 cryofibrinogenaemia patients, 18 had essential cryofibrinogenaemia and 43 secondary cryofibrinogaemia. Five out of the 18 patients with primary cryofibrinogaemia (27%) developed lymphoma after a 5-yr follow-up period. The main manifestations were cutaneous, and there were no differences in clinical presentation and disease severity in both types of cryofibrinogenaemia. A small number of patients (six) had cryofibrinogenaemia associated with cryoglobulinaemia, and in two cases, hepatitis C virus infection was detected; but no differences were observed between these two groups of patients., Conclusion: Cryofibrinogenaemia was found in our study with a high prevalence, suggesting that this pathology is rather underestimated. Our data further suggests that these patients should have a regular follow-up because of the high risk of symptom recurrence. We also hypothesize that in some cases essential cryofibrinogenaemia might be a prerequisite for a secondary disease.

Introduction: Proton pump inhibitors (PPIs) are an efficient therapy, being widely used by physicians. In 2004, cost of PPIs' therapy was as high as 748 millions of euros (for The Caisse primaire d'Assurance Maladie) in France. Although validated indications of PPIs are well known, numerous un-necessary prescriptions of PPIs are common. The aim of this prospective study was to evaluate PPIs' prescriptions of patients in a department of internal medicine., Patients and Methods: This is a 12-week assessment of medical charts of patients, receiving PPI therapy in patients in our department of internal medicine. Data were collected by a standardized questionnaire, with regards to: PPIs' nature and regimen, PPIs' indications as well as duration of therapy., Results: The medical charts of 729 consecutive patients, with a mean age of 67 years, were collected. Two hundred (and) twenty-four patients (30.7%) received PPI therapy; 157 of these patients were given PPI before admission in our department. Omeprazole was used in 71% of patients. Duration of PPI therapy was over one year in 45% of cases. Thirty-five per cent of family physicians' PPI prescription were validated and 23.8% of those of physicians working in the department of internal medicine. The main non-conform PPI's indications, by family physicians and internists were as follows: prevention of hemorrhagic risk of anti-platelet agent (21 vs 16.4%), anticoagulant (17.8 vs 16.4%), steroids (8.3 vs 13.4%) or non-steroid anti-inflammatory therapy without risk factor (1.9 vs 9%). Finally, in patients receiving PPI therapy before admission, this therapy was maintained in 76% of cases., Conclusion: This prospective study confirms the frequent prescription of PPI therapy in a department of internal medicine (31% of patients). It also underscores the importance of PPIs' use by family physicians and physicians working in a department of internal medicine; this series further highlights the difficulties to interrupt this well tolerated therapy. To date, PPI therapy should be prescribed with a cautious consideration of cost and benefit.

Purpose: Recent studies have suggested that the prevalence of Helicobacter pylori may be more frequent in patients with primary Raynaud's phenomenon (PRP) compared to healthy subjects. These data prompted us to conduct this prospective study, in order to assess the prevalence of H. pylori infection in a large series of patients with PRP., Methods: Forty consecutive patients with a definite diagnosis of PRP were included in the study. The findings in the PRP patients were compared with those of 80 age- and sex-matched healthy subjects. H. pylori infection was diagnosed using serology and urease breath test., Results: The prevalence of H. pylori infection was as high as 12.5% in PRP patients using both serology and urease breath test, whereas it was found to be 16.7% and 18%, respectively, in healthy controls., Conclusion: As prevalence of H. pylori infection was similar in PRP patients compared to controls (P=0.53 and 0.43, respectively), our data underscore that H. pylori infection may not play a role in the genesis of PRP-related vascular complication onset. Interestingly, PRP patients exhibited more commonly digestive symptoms consistent with H. pylori infection compared to controls (P<0.05).

Even at the beginning of the next millennium, aspirin will still offer surprises. Its relatively young pharmacological history compares with the early use of salicylate-containing plants since antiquity. The Assyrians and the Egyptians were aware of the analgesic effects of a decoction of myrtle or willow leaves for joint pains. Hippocrates recommended chewing willow leaves for analgesia in childbirth and the Reverend Edward Stones is acknowledged as the first person to scientifically define the beneficial antipyretic effects of willow bark. At the beginning of the 19th century salicin was extracted from willow bark and purified. Although a French chemist, Charles Gerhardt, was the first to synthesize aspirin in a crude form, the compound was ignored, and later studied by Felix Hoffmann. He reportedly tested the rediscovered agent on himself and on his father, who suffered from chronic arthritis--a legend was born and Bayer Laboratories rose to the heights of the pharmacological world. First used for its potent analgesic, antipyretic and anti-inflammatory properties, aspirin was successfully used as an antithrombotic agent. Sir John Vane elucidated aspirin's active mechanism as an inhibitor of prostaglandin synthetase and received the Nobel Price in Medicine for this work in 1982. Two isoform of cyclooxygenase (COX-1 and COX-2) have now been identified, each possessing similar activities, but differing in characteristic tissue expression. The cox enzyme is now a target of drug interventions against the inflammatory process. After two centuries of evaluation, aspirin remains topical, and new therapeutic indications are increasingly being studied.

Low-molecular-weight heparin are widely used for prophylaxis or curative treatment of thromboembolic disease. In France, low molecular weight heparin can only be used for the surgical prophylaxis (general and orthopaedic) of thromboembolic disease, for the initial treatment of deep vein thrombosis and in haemodialysis. In an economic approach we try to assess costing information which takes account of drug acquisition costs but also costs of drug administration and costs of drug therapy failure (failed prophylaxis, bleeding and recurrent thrombosis). At least, it has been argued that low molecular weight heparin may take it possible to discharge patients earlier and even to treat patients at home or as out-patients, thus creating further cost savings.

For curative treatment of deep vein thrombosis, all trials have shown that twice a day low molecular weight heparin were at least as efficacious as unfractionated heparin on clot reduction or stabilisation. Through the unit cost of low molecular weight heparin is higher, the real cost of treatment should take into account not only the cost of the drug, but also the cost of materials used and lab tests as well as the time necessary. We have therefore prospectively compared the operating and overall costs of nadroparin and intravenous heparin treatment of deep vein thrombosis in hospitalised patients (21 with unfractionated heparin and 19 with nadroparin). The results show that low molecular weight heparin is no more expensive than treatment with unfractionated heparin (336 +/- 74.9 F for unfractionated heparin and 344.9 +/- 44.05 F for nadroparin). In addition, using nadroparin rather than heparin saves approximately one hour per patient per week nursing time (42 +/- 7 minutes vs 104.0 +/- 10.7 minutes, p < 0.05). The methodology of this study should be repeated with other low molecular weight heparin and low molecular weight heparin compare not only to standard i.v. heparin, but also to standard SC heparin.

Serum cholesterol was measured in all patients admitted to the Department of Internal Medicine over one year. 6.2 percent had hypocholesterolaemia defined as total serum cholesterol level below 4 mmol/l. Comparison with non-hypocholesterolaemic patients showed male predominance and indirect arguments for increased morbidity: longer duration of hospital stay, greater number of rehospitalizations, greater number of associated diseases. In over a quarter of the cases, hypocholesterolaemia was associated with malignant disease. These results should entice physicians to caution when finding a low serum cholesterol level: this could be a marker of associated disease, especially malignant.