Effectiveness of written guidelines on the appropriateness of thromboprophylaxis prescriptions for medical patients: a prospective randomized study.

OBJECTIVE: To assess the effectiveness of providing doctors with written thromboprophylaxis prescription aids based on current recommendations. DESIGN: A prospective trial of specific anticoagulant prescription forms: a 1-day survey before and after the intervention in each centre. SETTING: 30 Internal Medicine departments of Assistance Publique-Hôpitaux de Paris. SUBJECTS: All inpatients were included, except those who were either admitted or discharged on the day of the survey, and those receiving curative anticoagulant treatment. INTERVENTIONS: The study included three parts: (i) a 1-day baseline survey; (ii) over the following 3-month period, departments were randomized into two groups: all practitioners in wards allocated to the intervention group were required to systematically use specific anticoagulant prescription forms, whilst doctors in the control group continued prescribing according to their usual practices and (iii) a 1-day postintervention survey. MAIN OUTCOME MEASURE: The proportion of prescriptions in accordance with the recommendations. RESULTS: 1,469 patients were included. The intervention produced a significant reduction in the frequency of over-prescriptions, from 25% to 14% of the patients who did not meet the guideline criteria (adjusted OR: 0.3; 95% CI: 0.1-0.8). Using specific forms did not improve under-prescription of anticoagulants. A little over 60% of the patients who met guideline criteria for thromboprophylaxis were prescribed anticoagulants in both intervention and control wards, either at baseline or after intervention. CONCLUSIONS: Multitargeted interventions using a variety of means and strategies should still be considered to improve prescriptions that may have a significant impact on health expenses and, most importantly, on patients outcomes.

[Pseudo-autoinflammatory attacks of temporal arteritis]. / Manifestations d'allure auto-inflammatoire d'une maladie de Horton.

INTRODUCTION: Usually, temporal arteritis progresses as a chronic disease. CASE REPORT: The authors report the observation of a 74-year-old woman who presented with two acute flares of temporal arteritis with headache, fever and inflammatory syndrome, which have spontaneously resolved. DISCUSSION: The observations of auto-inflammatory attacks of arteritis disease are rare, but maybe underestimated. The pathophysiology remains unclear.

[Familial Mediterranean fever, clinical and laboratory findings]. / Maladie périodique, symptomatologie clinique et biologique.

Familial Mediterranean fever is the best known of the recurrent hereditary autoinflammatory diseases. It predominantly affects subjects of Mediterranean origin, Sephardic Jews in particular. Its gene, MEFV, is located on chromosome 16 and has autosomal recessive transmission, with incomplete penetration. It codes for a protein called pyrin or marenostrin, which is probably involved in the inflammatory process. In most cases, the first episodes appear before the age of 20 years and very rarely after the age of 40. Episodes usually last a few days, although they may extent over several weeks when localized in joints. Fever, occasionally pseudo-malaria, may accompany various symptoms, the most frequent of which are abdominal, articular, pleural or cutaneous. The abdomen is the classic site of this disease, and acute abdominal flares masquerade as abdominal emergencies. Musculoskeletal involvement is revealed by episodes of inflammation of the joints (more often mono- than oligoarthritis) and muscle pain. The flares are usually brief and totally reversible. Flares of thoracic pain corresponding to pleural inflammation and erysipelas-like skin eruptions have been observed. Acute symptoms disappear between flares, but hepatic splenomegaly, swollen lymph nodes or abnormal fundus of the eye may persist. Laboratory findings are typical of nonspecific inflammation, accompanied by moderate hyperleukocytosis during the flares.

[Familial Mediterranean fever among the autoimmune diseases]. / La maladie périodique au sein des maladies auto-inflammatoires.

During the first attacks of familial Mediterranean fever, each of the disease symptoms can suggest a series of disorders. When the disease is older, the recurrence of symptoms may simulate some systemic diseases, but mainly suggests familial Mediterranean fever, one of a group of hereditary autoinflammatory diseases. Before the gene for familial Mediterranean fever was identified, various sets of criteria were used for diagnosis. The presence of MEFV mutations confirms the diagnosis, but the clinical criteria still determine who should undergo this genetic testing. The genotype-phenotype correlations add a prognostic dimension to the mutations identified. Genotyping can also lead to the diagnosis of the other autoinflammatory diseases, which constitute the basis of the differential diagnosis of familial Mediterranean fever. The hyperimmunoglobulinemia D syndrome (HIDS) is very similar to familial Mediterranean fever in its recessive transmission and abdominal and articular symptoms. It can be distinguished by the European origin of the patients, the presence of cervical lymph nodes and the increased IgD levels. Of the diseases with dominant transmission, the TNF receptor-associated periodic syndromes (TRAPS) are suggested by periorbital edema and migrating inflammatory cellulitis. Muckle and Wells syndrome is revealed by episodes of fever with urticaria and arthralgia, complicated by deafness and amyloidosis. Mutations in the same gene are responsible for two disorders, both appearing in childhood: familial cold urticaria syndrome (FCUS) and chronic infantile neurocutaneous articular syndrome (CINC). The pathogenesis of familial Mediterranean fever is still unclear. Pyrin/marenostrin, the protein produced by the MEFV gene, appears to hae a physiological antiinflammatory effect that inhibits proinflammatory cytokines. Mutation of the gene may eliminate this feedback mechanism and expose the patient to flares from any inflammatory stimulus, even minimal. Amyloid is produced by the serum amyloid A protein (SAA), and its occurrence is influenced by the type of MEFV mutation, but also the genotype of the gene producing SAA.

[From familial Mediterranean fever to amyloidosis]. / De la maladie périodique à l'amylose.

The progression of familial Mediterranean fever is marked by the recurrence, at varying intervals, of acute flares that regress spontaneously. Prognosis, which depends on the occurrence of amyloidosis, has been transformed by colchicine treatment. Incidence of amyloidosis is higher in certain ethnic groups (Jews from North Africa, Turks) and depends on by the specific MEFV mutation. Amyloid is composed of clusters of protein strands identical to the AA protein of secondary amyloidosis and infiltrates the walls of all arterioles except those of the central nervous system. The earliest and most consistent localization is in the kidney, where it develops over several years and in 4 stages--preclinical (latency), proteinuric, nephrotic and uremic--before concluding in end-state renal failure. Before the advent of colchicine, dialysis and transplantation, only renal amyloidosis caused clinical manifestations and lethal complications; any amyloidosis at any other sites remained latent. Prolonged survival with hemodialysis and kidney transplantation now leaves time for manifestation of these other localizations, such as infiltration into the intestines causing malabsorption, or potentially lethal cardiac lesions. Treatment of familial Mediterranean fever is based on the continuous administration of colchicine, which at the average dose of 1 to 2 mg per day can prevent flares or at least reduce their frequency or intensity. Systematic use of colchicine also prevents the onset of amyloidosis, even in the rare cases where it cannot prevent flares. These data fully justify the systematic use of colchicine for continuous prophylactic treatment from diagnosis and even after kidney transplantation, to prevent recurrence of the grafted kidney or extension to other organs. The curative efficacy of colchicine on flares is debatable, although several studies report positive results against progression of early amyloidosis.

Brain abscess caused by Nocardia cyriacigeorgica in a patient with human immunodeficiency virus infection.

Nocardia cyriacigeorgica is a recently characterized species within the genus of Nocardia. We report a brain abscess, following a primary pulmonary colonization, due to this species in a human immunodeficiency virus-infected patient. This case confirms that isolation of Nocardia in sputum is associated with a high risk of disseminated infection in immunocompromised patients.

[Consequences of replacing abacavir for indinavir in successful antiretroviral treatment of a patient with HIV infection]. / Conséquences de la substitution de l'indinavir par l'abacavir chez un patient infecté par le VIH en succès thérapeutique.

INTRODUCTION: Simplification of combined antiretroviral therapy in HIV-infected patients is possible, but virological success can be compromised by the development or emergence of resistant viruses. CASE: Worsening renal functioning in a patient under successful combination antiretroviral therapy resulted led to the replacement of indinavir by abacavir. Eight weeks later, his viral load rose and he developed a mutant virus resistant to all the nucleoside analogs. DISCUSSION: Our case report illustrates the danger of streamlining combined antiretroviral therapy composed only of nucleoside analogs in patients already successfully treated with nucleoside analogs, by exposing them to the risk of the emergence of a mutant virus.

Regression of cutis calcinosis with diltiazem in adult dermatomyositis.

Calcinosis cutis is common in several connective tissue diseases such as dermatomyositis, scleroderma or lupus erythematous. In dermatomyositis, it is more likely to concern children than adults but it is not exceptional in adults. Several treatments have been used empirically with inconsistent success. We report a case of adult cutis calcinosis associated with dermatomyositis which responded dramatically to treatment with diltiazem.

Typical and atypical Cogan's syndrome: 32 cases and review of the literature.

OBJECTIVE: To report our experience on a multicentre series of 32 patients with either typical or atypical Cogan's syndrome, to combine our results with a detailed review of the literature, and to compare the clinical manifestations of typical and atypical Cogan's syndrome. METHODS: Patients were identified from a survey conducted with physicians affiliated to the French National Society for Internal Medicine, and were classified into typical or atypical Cogan's syndrome according to the Haynes criteria. Clinical data were collected in a standardized manner. A comprehensive literature review using the Medline database and the reference lists of identified articles was performed. RESULTS: Seventeen patients had typical Cogan's syndrome and 15 had atypical Cogan's syndrome. Apart from non-syphilitic interstitial keratitis, the ocular manifestations of patients with atypical Cogan's syndrome were mainly uveitis and episcleritis. All but one patient presented with Ménière-like syndrome, and at the end of follow-up 11 were deaf and 19 additional patients had developed a significant decrease in auditory acuity. Twenty-five patients (78%) developed systemic manifestations, including aortitis in four. Comparison of typical and atypical Cogan's syndrome showed that some systemic manifestations were more common in atypical Cogan's syndrome, but these differences may be explained by reporting bias in the literature. CONCLUSION: Differences regarding the associated systemic manifestations of typical and atypical Cogan's syndrome may reflect reporting bias in the literature. However, the diversity of the ocular and audiovestibular manifestations and the acceptable lengthy delay between the two types of involvement in atypical Cogan's syndrome should make one cautious before accepting this diagnosis as the diagnosis may mimic various other systemic diseases.

[Extra-meningeal meningococcal infection: report of 14 cases]. / Localisations extraméningées des infections à méningocoque : à propos de 14 cas.

INTRODUCTION AND METHOD: Fifty-five patients (17 adults, 38 children) with meningococcal infection were admitted between 1986 and 2002 in a university hospital (500 beds). Fourteen of them (nine adults, five children) presented with an extra-meningeal infection. We compared adults and children presentations. RESULTS: All adults had immunodeficiency. Septic locations were various (three bacteriemia, four pneumoniae, one infected ascitis, one cutaneous abscess). All patients received amoxicillin or third generation cephalosporin. Hospitalisation was prolonged (mean: 47 days). Seven patients required intensive care unit admission, and two of them died. All children (all were less than 36-month-old) presented with fever. Only one was immunodeficient (infected by human immunodeficiency virus). Neisseria meningitidis grew from blood in four, and in the throat for the remaining one. Hospitalisation was of short duration (mean: 4 days) and none of the children required intensive care unit. All the children recovered rapidly with antibiotics. CONCLUSION: Outcome of extra-meningeal infection with N. meningitidis is different in adults and children. Adults present with immunodeficiency, infection is severe and patients present with various clinical features; children have a more homogeneous clinical presentation (fever) and outcome is excellent.

Rheumatoid arthritis and schizophrenia: a negative association at a dimensional level.

There is wide evidence for a decreased risk of rheumatoid arthritis in patients with schizophrenia. Nevertheless, very few studies have looked at the risk of schizophrenia in a group of patients with rheumatoid arthritis. We prospectively investigated, with the SCL-90R, 220 consecutive outpatients with rheumatoid arthritis and 196 consecutive outpatients with various medical conditions, half of them suffering from psoriatic arthritis (a medical condition close to rheumatoid arthritis). The SCL-90R appears to be a valuable tool to distinguish patients with schizophrenia from the outpatients of our sample, the former having more "paranoid ideation" (p = 0.004) and more "psychoticism" (p < 0.001) than the latter. The "paranoid ideation" dimension was significantly lower (25% decrease) in the sample of patients with rheumatoid arthritis compared to the combined control group (p = 0.005), ratings under the median value being more frequent in the former group (p = 0.025). Confounding factors might not explain this difference according to the regression logistic analysis performed. As patients with rheumatoid arthritis have a lower score of paranoid ideation than controls in our sample, even after controlling for age, gender and severity of the disease, these data represent further evidence for a decreased risk of schizophrenia in individuals with rheumatoid arthritis.

[Bone marrow reconversion and magnetic resonance imaging: case report]. / Le phénomène de reconversion médullaire et son aspect en imagerie par résonance magnétique : à propos d'un cas.

INTRODUCTION: Bone marrow is divided into red marrow mainly constituted of hemopoietic cells and fatty yellow marrow. In some situations, yellow marrow may be converted into red marrow and this process is called marrow reconversion. Magnetic resonance imaging may be misleading with an invading bone marrow neoplastic process. EXEGESIS: We report a patient with non-Hodgkin's lymphoma with vertebral invasion. Clinical features at presentation were misleading with lower limbs migratory pain suggestive of inflammatory myositis. An MRI study of thigh muscles revealed femoral nodular lesions suggestive of bone marrow reconversion. CONCLUSION: Bone marrow reconversion is a physiologic and reversible process. Awareness of its radiographic features may help to avoid a diagnostic biopsy procedure.