Syndrome des antiphospholipides « séronégatif » : mythe ou réalité ?

The antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thrombosis and/or obstetrical manifestations and the persistent presence, at least 12 weeks apart, of antiphospholipid antibodies (aPL) such as lupus anticoagulant (LA) and/or anticardiolipin antibodies (ACL) and/or anti-β2 glycoprotein I antibodies (aβ2GPI). The finding of patients with clinical profile highly suggestive of APS but who are negative for conventional biological criteria has led to the concept of seronegative APS. In the last few years, new antigen targets and methodological approaches have been employed to more clearly identify this syndrome in patients with thrombosis or obstetrical complications without conventional aPL. Although seronegative APS is still controversial, there is increasing recognition of the existence of this subgroup. However, clinical relevance of non conventional aPL need to be confirmed by efforts toward standardizing new biological tools and longitudinal studies involving large cohort of patients.     

Les kystes hydatiques cardiaques à propos de 17 cas opérés

Cardiac hydatid cyst is a rare parasitic disease. The purpose of this study was to describe the clinical, pathological features and the outcome of the surgical treatment of cardiac hydatid disease in our unit over a twenty-year period.MethodsBetween May 1994 and May 2014, seventeen cases of cardiac hydatid cysts were operated at our unit. Overall, twelve patients were male (mean age 25 ± 13 years). All patients were complaining of dyspnea and 71% presented with chest pain. The diagnosis, based on histological examination, was suspected on echocardiography and computed tomography of chest.ResultsOur study revealed five possible locations, which were in decreasing order of frequency: left ventricle, interventricular septum, right ventricle, left atrium and pulmonary artery. The surgical procedure was a controlled puncture and aspiration of the cyst content, with cystectomy (69%), or pericystectomy (31%). The resulting cavity left open in 6 cases (37.5%) or carefully closed in 10 (62.5%). Hospital mortality was 11.8% (n = 2). Morbidity was marked by conduction abnormalities (n = 2), bleeding and hematoma of the residual cavity that required surgical treatment (n = 3). Eleven patients were followed with a mean period of 40.5 ± 19.4 months. At follow-up, neither late deaths nor recurrence have occurred.ConclusionCardiac hydatid cyst is a serious disease whose treatment is surgical. Cystectomy and pericystectomy remain the two surgical techniques able to offer good chance of cure with acceptable morbidity and mortality.     

Allergies alimentaires précoces du nourrisson de 6 à 18 mois : étude de 69 cas

We report our findings on a group of 69 children without cow’s milk allergy who had one or several other food allergies between 6 and 18 months of age. Their initial symptoms were of moderate to severe intensity: 61 (88.5%) of them had had systemic symptoms, including angioedema (52%), generalized urticaria (36%), laryngeal edema (13%) and asthma (10%). In 11.5% of the patients, the first sign was severe acute eczema that did not respond to the usual treatment. The most important allergens, those identified with skin tests, serological assay for specific IgE antibody and labial or oral challenge tests, were egg (60.2%), peanut (50%), fish (10.3%) and cashew nut (5.8%). An atopic background was present in 90% of these infants. Of those allergic to eggs, 53% subsequently had no reaction to this food, whereas only one child allergic to peanuts and none of those allergic to cashew nuts or fish became symptom-free. Forty-three percent of the infants suffered from multiple food allergies, and asthma had developed in 33 (48%) of them. In conclusion, the early onset of food allergy and the presence of multiple sensitivities in this group of patients pointed to the seriousness and the unfavorable evolution of their allergic condition.     

Évaluation de la fréquence et des facteurs associés à l’hypotension orthostatique chez les diabétiques de type 2

Orthostatic hypotension is a major criterion for diabetic autonomic cardiac neuropathy. It increases overall and cardiovascular mortality.PurposeThe aim of our study was to evaluate the orthostatic hypotension frequency in type 2 diabetics and to investigate the associated factors.MethodsThis prospective cross-sectional study included adult patients with type 2 diabetes. Orthostatic hypotension was defined as a decrease in the systolic arterial pressure > 20 mm-Hg or a decrease in the diastolic arterial pressure > 10 mm-Hg, after 3 minutes of standing position. The arterial pressure was measured with a validated electronic device. Vascular risk factors and micro and macro-vascular complications were systematically investigated by the appropriate diagnostic means.ResultsWe evaluated 300 diabetic patients. Of these women represented 57%. The mean age was 58 years old. The average duration of diabetes was 8 years. The antidiabetic drugs were metformin (82%), sulfonylureas (36%) and insulin (40%). High blood pressure was present in 49% among whom 50% of patients received angiotensin II receptor blockers, 47% diuretics, 24% angiotensin converting enzyme inhibitors, 17% beta blockers, and 17% calcium channel blockers. Orthostatic hypotension has been identified in 26 patients (8.6%). In multivariate analysis, the associated factors with orthostatic hypotension were elevated systolic blood pressure and insulin treatment.ConclusionThe frequency of orthostatic hypotension is relatively low in our patients with type 2 diabetes and it was significantly associated with systolic blood pressure and insulin treatment.     

Gestion des toxicités induites par les inhibiteurs de checkpoint immunologique : données de la réunion de concertation pluridisciplinaire « ToxImmun » en Occitanie Est

Immune checkpoint inhibitors (ICIs) can cause numerous and complex immune-related adverse events whose management need a multidisciplinary approach. Herein, we investigated 114 requests, mostly concerning patients suffering from lung cancer, that were submitted to the « ToxImmun » multidisciplinary meeting in Eastern Occitania between December the 17th 2018 and January the 20th 2020. The leading reasons for the request concerned the putative causal link between immunotherapy and immune-toxicity and its management, followed by possible retreatment after temporary withdrawn because of adverse event, and finally the possibility to initiate ICIs in patients with pre-existing autoimmunity. Colitis, hepatitis and myocarditis were the most frequent immune-related adverse events (IRAEs), both all grade and grade 3–4. Sicca syndrome (with or without Sjogren criteria) was also frequent (26% of cases) and seems to be associated with severe toxicity and multi-toxicity. The mean time to first IRAE was 3.8 months, a time shortened with the use of anti-PD-L1 agents or ICI combination. A majority of requests came from initial evaluation by the internist confirming the early and main role of this specialty in the management of immunotoxicity. Expansion of this regional multidisciplinary meeting, coordinated by internists and medical oncologists, could improve management of immune-related adverse events for the patients’ benefits.