[Obstetrical APS: Is there a place for additional treatment to aspirin-heparin combination?] / Le syndrome des antiphospholipides obstétrical : quelle est la place d'un traitement complémentaire à la combinaison aspirine et héparine ?

Obstetrical APS is defined by thrombosis and/or obstetrical morbidity associated with persistent antiphospholipid antibodies. The aspirin and low molecular weighted heparin combination dramatically improved obstetrical outcome in APS patients. Several factors could be associated with obstetrical prognosis, as previous history of thrombosis, associated SLE, the presence of lupus anticoagulant and triple positivity of antiphospholipid antibodies. Obstetrical APS with isolated recurrent miscarriages is mostly associated with isolated anticardiolipids antibodies and have better obstetrical outcome. The pregnancy loss despite aspirin and heparin combination define the refractory obstetrical APS, and the prevalence could be estimated to 20-39%. Several other treatments have been used in small and open labeled studies, as steroids, intravenous immunoglobulins, plasma exchanges and hydroxychloroquine to improve the obstetrical outcome. Some other drugs as eculizumab and statins could also have physiopathological rational, but studies are necessary to define the place of these various drugs.

[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.

[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.

[Systemic sclerosis and pregnancy]. / Sclérodermie et grossesse.

Pregnancy in women diagnosed with systemic sclerosis is an infrequent situation with a generally favourable outcome according to the most recent studies. Women with systemic sclerosis who wish to become pregnant should obtain preconception counselling in order to withdraw teratogenic drugs and to carefully assess the disease activity. Severe organ impairment, early diffuse systemic sclerosis and pulmonary hypertension ought to discourage patients from pregnancy, as these situations are at high risk of complications for both mother and fetus during pregnancy and puerperium. During pregnancy, the most frequent maternal complication is worsening of gastroesophageal reflux, and the most severe complications are renal crisis and flaring arterial pulmonary hypertension. Angiotensin converting enzyme inhibitors are indicated at any stage of the pregnancy if renal crisis is suspected. Adverse obstetric outcomes are dominated by prematurity which may be the consequence of intra-uterine growth restriction or preeclampsia. Those complications might account for systemic sclerosis-associated vasculopathy, or for the presence of antiphospholipid antibodies. Patients with a previous history of placental insufficiency might benefit from treatment with aspirin, low dose anticoagulants or even nitric oxide donors. During the pregnancy, abnormal bilateral uterine artery Doppler is a predictor of vascular insufficiency and might guide initiation of further preventive treatments. Multicentric prospective cohort studies are warranted to identify more precisely the predictors of pregnancy-related complications and to define the best management of these patients.

[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.

[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.