[Periungueal capillaroscopy: an easy and reliable method to evaluate all microcirculation diseases]. / Capillaroscopie périunguéale : une évaluation simple et fiable de toute pathologie de la microcirculation.

Periungueal capillaroscopy is a simple and reliable non-invasive technique allowing evaluation of cutaneous microcirculation. It was promoted for decades in patients with Raynaud's phenomenon in order to differentiate between the benign primary Raynaud's phenomenon and the secondary form in connective tissue diseases, especially systemic sclerosis. Nevertheless, the value of this procedure has also been shown in numerous pathologies such as diabetes or cardiovascular diseases. This literature review points to the versatility of this useful exam and its results in a large spectrum of diseases with microvascular involvement.

[100 essential drugs. An internal medicine approach]. / Les 100 médicaments essentiels. Une approche de médecine interne.

PURPOSE: Up to 4600 drugs in about 15,000 pharmaceutical forms are available in France which may be a source of misuse with increased occurrence of side effects and costs. While the World Health Organization is encouraging each developed country to work out its own list of essential drugs. The list provided in 2008 by the French Office for the safety of health products has had so far limited impact on practice, so it became obvious to a group of internists to work out a "wise list" of 100 essential medicines covering 95% of the disorders observed in France. METHODS: In June 2011, 10 internists agreed to each provide a list of 100 essential medicines, according to individual experience. In December 2011, a meeting of the participants provided a list as initial consensus and mandated five among them to make proposals for those areas neglected by too many participants or in which needless dispersion of medicines was stated. After internet-facilitated exchanges, an additional list was validated in mild-January 2012. RESULTS: Fifty-four drugs were included in the list of initial consensus (including nine selected by all 10 participants), and 46 in the additional list. So the final "wise list" included 100 drugs. In June 2012, 56 of these drugs were available as generics. This list was compared to those lists set out by five countries in the European Union. CONCLUSION: Generating such a list is feasible. Undoubtedly still non-comprehensive, this list will benefit from the expertise of 14 general practitioners who are currently working out a similar list across France. The final list will be submitted for validation by the French associations of generalist teachers and Internists.

[High plasmatic concentration of vitamin B12: an indicator of hepatic diseases or tumors]. / Concentration plasmatique élevée de la vitamine B12 : un indicateur des maladies hépatiques ou tumorales.

PURPOSE: To identify the diseases that are associated with a high plasma concentration of vitamin B12 and to measure the strength of this association. PATIENTS AND METHODS: Retrospective study including all admissions between 1st May, 2005 and 30th April, 2008 in the UMAG pole departments (emergency, internal medicine, acute geriatrics and medical intensive care) with a test for plasma vitamin B12. The association between each of medical information system codes (solid tumors, malignant hematologic process, and renal disease) and a high or low vitamin B12 concentration was measured by odds ratios (OR) from logistic models taking into account repeated admissions, with adjustment for age and the weighted Charlson index. RESULTS: Among 3702 admissions, 12% had a B12 more than 820pg/ml, 10.4% a B12 less than 180pg/ml and 77.6% a normal B12 concentration. After adjustment for age and the weighted Charlson index, high concentration of vitamin B12 was associated with interstitial renal diseases (OR 2.7; 95% CI: [1.7-4.2]), and cirrhosis or hepatitis (OR 4.3; [2.9-6.4]). After additional adjustment for these parameters, it was still associated with tumors (OR 1.8; [1.2-2.6]), malignant hematologic diseases (OR 2.1; [1.3-3.5]), metastasis (OR 2.9; [1.5-5.9]), liver metastasis (OR 6.2; [2.7-14.5]), liver carcinoma (LC) (OR 3.3; [1.1-10.4]), liver tumors other than LC (OR 4.7; [1.2-17.9]) and lymphoma (OR 3.2; [1.6-6.4]) but not with myeloma (OR 1.9; [0.6-1.4]). Low concentration of B12 was associated with myeloma (OR 2.9; [1.3-6.6]). CONCLUSION: Finding a high plasma concentration of vitamin B12 should lead to a systematic search for a hepatic disease or a tumor, and particularly for a hepatic localization of a tumor.

[Pulmonary embolism revealed by a seizure: a case report and literature review]. / Embolie pulmonaire révélée par une crise convulsive : à propos d'un cas et revue de la littérature.

INTRODUCTION: Pulmonary embolism is a frequent disorder with a diagnostic approach based on probability estimation. Nevertheless, in some cases, prognosis may be impaired by delayed diagnosis resulting from atypical presenting manifestations. CASE REPORT: We report a 37-year-old woman, admitted for a seizure as the presenting manifestation of pulmonary embolism, and review nine additional similar cases reported in the literature since 1945. Seizures were always generalized tonico-clonic in nature without a past medical history of epilepsy in any case. Tachycardia was noted in nine patients over ten. Prognosis was usually severe leading to death by cardiovascular deficiency in 70% of cases. CONCLUSION: These observations suggest a systematic suspicion of pulmonary embolism in the presence of generalized convulsion with persistent tachycardia after resolution of the episode and no past medical history of seizures. More research is necessary to assess the role of d-dimer testing in these situations.

[Carotid-cavernous sinus fistula: an unusual cause of exophthalmia. Report of three cases]. / Une cause inhabituelle d'exophtalmie uni- ou bilatérale : les fistules artérioveineuses carotidocaverneuses. Trois observations.

INTRODUCTION: Exophthalmia can be associated with several disorders. Although unusual, a carotid-cavernous fistula should be ruled out systematically as it may be associated with severe complications. CASE REPORTS: We report three cases associated with both types of carotid-cavernous fistula that highlight the clinical presentation and diagnostic process. CONCLUSION: A carotid-cavernous fistula should be systematically ruled out in patients with a uni- or bilateral exophthalmia. Careful examination with auscultation of the ocular globe contributes greatly to the diagnosis that may be confirmed by MRI or arteriography.

Serum CC chemokine ligand-18 predicts lung disease worsening in systemic sclerosis.

Elevated serum CC chemokine ligand (CCL)18 reflects lung fibrosis activity in systemic sclerosis (SSc) and could be an early marker of lung function worsening. Therefore, we sought to evaluate whether serum CCL18 levels at baseline could predict worsening of lung disease in SSc. In this prospective study, 83 SSc patients were analysed longitudinally over a 4-yr observation period for the risk of occurrence of combined deleterious events, defined as a 10% decrease from baseline of total lung capacity or forced vital capacity % predicted, or death, according to serum CCL18 at inclusion. Receiver operating characteristic (ROC) curve analysis was performed for prediction of events during the first year after inclusion. The best cut-off level of serum CCL18 for prediction of a combined event within the follow-up period was 187 ng · mL(-1), with 53% sensitivity and 96% specificity (area under the ROC curve 0.86; p < 0.001). After a mean ± SD follow-up of 33.7 ± 10.8 months, a higher rate of disease progression occurred in the group with serum CCL18 levels >187 ng · mL(-1). The adjusted hazard ratio was 5.36 (95% CI 2.44-11.75; p < 0.001). In summary, serum CCL18 is an accurate predictive biomarker for the identification of patients with a higher risk of subsequent scleroderma lung disease worsening.

[Salmonella bredney: a rare cause of mycotic aneurysm]. / Salmonella bredney: une cause rare d'anévrisme mycotique.

Mycotic aneurysms are rare, remain asymptomatic for a long time, and may be life threatening by their rupture if therapy is delayed. Historically associated with Streptococcus pyogenes and Staphylococcus aureus, they now frequently involve Salmonella species in elderly or immunodeficient patients, and complicate vascular investigation or surgical procedures. Frequently located in the abdominal aorta, they can also be found rarely in other location. Therapy associates antibiotics and surgical debridement with reestablishment of vascular continuity. We report a case of ruptured popliteal aneurysm with Salmonella bredney bacteraemia.

[Late onset Becker muscular dystrophy. A case report and literature review]. / Dystrophie musculaire de Becker à révélation tardive. À propos d'un nouveau patient et de 12 observations de la littérature.

INTRODUCTION: The first manifestations of Becker's muscular dystrophy usually occur during childhood, as Duchenne's muscular dystrophy, a related disease. However, clinical presentation is usually more heterogeneous and less severe. CASE REPORT: We report a 54-year-old man who presented with a late onset of Becker's muscular dystrophy, and review 12 additional similar cases previously reported in the literature. The disease onset ranged from 30 to 65 years old. Genetic deletion involved mainly exons 45 to 55 with variable phenotype: distal muscular hypertrophy in four cases, cardiac involvement (electrocardiography, radiography or echocardiography) in six out of the 12 studied cases. Compared to the paediatric form, functional impairment was milder, as only three patients after seven to 20 years of follow-up were wheelchair bound. CONCLUSION: The first manifestations of Becker's muscular dystrophy may occur after the age of 30 with heterogeneous phenotypes. Severity and limb disability is milder in these patients.

[Peripheral neuropathy with autonomous nervous system involvement in the course of dermatomyositis]. / Neuropathie périphérique avec atteinte du système nerveux autonome au cours d'une dermatomyosite.

A peripheral neuropathy occurs rarely during the course of an inflammatory myopathy. Once the classical aetiologies of peripheral neuropathies are ruled out, the diagnosis of neuromyositis can be accepted. We report a patient with dermatomyositis who presented a peripheral neuropathy revealed by dysautonomia. The presence of associated vasculitis led us to consider that this constituted the mechanism of the neurological involvement.

[Recurrent fever and skin eruption revealing chronic meningococcemia]. / Fièvre prolongée associée à une éruption cutanée diffuse révélant une méningococcémie chronique.

INTRODUCTION: Chronic meningococcemia is an unusual clinical presentation within the spectrum of infections due to Neisseria meningitidis. CASE REPORT: We report a 32-year-old man who presented with a 15-day history of fever and maculopapular skin rash, in the absence of meningeal irritation or severe sepsis manifestation. Blood culture identified N. meningitidis. Clinical course was uneventful after antibiotic treatment was initiated. CONCLUSION: Early diagnosis of chronic meningococcemia is crucial for optimal management of the patient and his/her contacts. Such a diagnosis should be suspected in the presence of the characteristic clinical triad (recurrent fever, skin rash and arthralgia), and this clinical presentation should be distinguished from systemic vasculitis as inadequate prescription of corticosteroids may be deleterious.

[Dyspnea secondary to pulmonary hypertension in a patient with splenic myeloid metaplasia]. / Dyspnée révélant une hypertension artérielle pulmonaire chez une malade ayant une métaplasie myéloïde de la rate.

There are several possible pathophysiological links between the development of pulmonary hypertension and myelofibrosis with myeloid metaplasia. We report a woman with myelofibrosis and myeloid metaplasia who presented with dyspnea and massive, painful splenomegaly. Right heart catheterization evidenced pulmonary hypertension. Her management consisted of splenic irradiation associated to sildenafil. Dyspnea in patients with myelofibrosis and myeloid metaplasia can be secondary to pulmonary hypertension and conversely the differential diagnosis of pulmonary hypertension should include a myeloproliferative syndrome.