La Cardiomyopathie de Tako-tsubo est-elle réellement réversible ?

Tako-tsubo cardiomyopathy is characterized by a total reversibility of wall motion abnormalities of the left ventricle (LV) as well as normalization of LV ejection fraction after the acute phase. However, recent studies have shown that some patients present functional, metabolic, and morphologic abnormalities away from the acute phase suggesting an incomplete recovery of the disease. In this revue we discuss about this topic through several tools used in those studies (echocardiography, exercise test, MRI, nuclear imaging, biology, as well).     

Hypercholestérolémie familiale : un risque cardiovasculaire largement sous-estimé

BackgroundFamilial hypercholesterolemia is a monogenic autosomal dominant dyslipidemia characterized by a permanent and isolated increase of cholesterol carried by low-density lipoproteins. The prevalence of its heterozygous form is estimated between 1/500 and 1/250, and in the absence of specific treatment, this form is responsible for an increase by a factor of 13 of the risk of premature coronary artery disease compared to patients non-affected by the disease.ObjectivesTo perform an inventory of the knowledge of heterozygous familial hypercholesterolemia in France for physicians involved in the management of the disease.MethodsA survey was conducted (by phone and internet) among a representative sample of 495 physicians (cardiologists, endocrinologists/diabetologists, gynecologists, general practitioners) who, in parallel, completed 579 patient records.ResultsThirty-two percent (95% CI [27.8; 36.2]) of physicians reported the difference between polygenic hypercholesterolemia and familial hypercholesterolemia. The presence of tendinous xanthomas, a key element of diagnosis, was spontaneously mentioned by 44% (95% CI [34; 54.2]) of cardiologists. Six percent (95% CI [2.2; 12.6]) of them gave a correct estimate of the prevalence of familial hypercholesterolemia. The likelihood of transmission of heterozygous familial hypercholesterolemia, when one parent is affected, was known for 59% (95% CI [48.7; 68.7]) of surveyed cardiologists. A cascade screening was performed systematically by 4% (95% CI [1.1; 9.9]) of them. Eighteen percent (95% CI [11; 26.9]) of cardiologists gave an accurate estimation of cardiovascular risk of heterozygous familial hypercholesterolemia. Fifty-seven percent (95% CI [46.7; 66.8]) of cardiologists admitted being misinformed about the heterozygous familial hypercholesterolemia and 83% (95% CI [74.1; 89.7]) expressed a need for information about this disease.ConclusionThe lack of knowledge of heterozygous familial hypercholesterolemia and its associated cardiovascular risk is probably the cause of a diagnostic default leading to inappropriate management of this disease.     

La PON1 est-elle un facteur du risque cardiovasculaire chez les diabétiques de type 2 ?

ObjectiveWe evaluate the association between the decrease of serum paraxonase 1 activity and the risk of cardiovascular disease in type 2 diabetes.MethodsOne hundred and fourteen patients with type 2 diabetes were included in the present study. Seventy-one of them have significant coronary disease. The control group consisted of 53 healthy adults.ResultsPON1 activity was significantly reduced in diabetic patients compared to controls (P = 0.021), especially in those with significant coronary disease (P = 0.013). No significant variation in PON1 activity according to age was observed both in controls and in patients. When HDLc ≥ 1.03 mmol/L, the PON1 activity was significantly higher in patients without significant coronary disease compared to those with significant coronary disease (0.030). In case of significant coronary disease, a decrease of 12.23% in PON1 activity was observed in smokers compared with non-smokers, but without statistical significance. The PON1 activity did not very significantly according to the presence or absence of hypertension in patients with significant coronary disease.ConclusionThe implication of diabetes in the decrease of PON1 activity seems highly probable but PON1 activity seems not to be in itself a marker of cardiovascular disease.     

Méningite récidivante révélant un syndrome de Bing-Neel

IntroductionBing-Neel syndrome is a rare complication of Waldenström macroglobulinemia, defined by monoclonal lymphoplasmocytic cells in the cerebrospinal fluid or in central nervous system biopsy.Case reportWe report a 47-year-old man, with no prior history, who presented a recurrent aseptic lymphocytic meningitis with central nervous manifestations. The presence of a monoclonal lymphoplasmacytic proliferation in cerebrospinal fluid, blood and bone marrow biopsy results was compatible with a diagnosis of Bing-Neel syndrome. Despite the absence of any specific treatment, there was no recurrence of symptoms at 4-month follow-up, and the MRI lesions remained stable.ConclusionWe report a case of Bing-Neel syndrome revealed by a recurrent meningitis. Outcome without treatment was favorable at 4-month follow-up.     

Prévalence et facteurs de risque de la rétinopathie drépanocytaire dans un centre de suivi drépanocytaire d’Afrique subsaharienne

Introduction

Retinopathy is a chronic complication with severe functional consequences in patients with sickle cell disease. Its prevalence is not well known in sub-Saharan Africa because of the absence of screening. We report here the results of a routine screening for sickle retinopathy in a Comprehensive Sickle Cell Center in Sub-Saharan Africa.

Ischémie aiguë de membre inférieur,révélant un thrombus aortique flottant paranéoplasique

The floating mural thrombus of abdominal aorta is a rare and serious pathology detected as cause of peripheral and visceral embolism. Isolated aortic mural thrombosis is an unusual pathology occurring in an apparently normal aorta. A thorough search of embolic source must be initiated. Therapeutic management is based on systemic anticoagulation with the use of surgical approach in some cases.