[Fighting against unexplained sudden death]. / Mort subite, en quête d'une explication.

Sudden cardiac death, mostly related to ventricular arrhythmia, is a major public health issue, with still very poor survival at hospital discharge. Although coronary artery disease remains the leading cause, other etiologies should be systematically investigated. Exhaustive and standardized exploration is required to eventually offer specific therapeutics and management to the patient as well as his/her family members in case of inherited cardiac disease. Identification and establishing direct causality of the detected cardiac anomaly may remain challenging, underlying the need for a multidisciplinary and experimented team.

[Malignant or benign hyperleukocytosis ?] / Hyperleucocytose maligne ou bénigne ?

Leukocytosis or an increase of white blood cells in the peripheral blood is a frequent anomaly. It is important to immediately distinguish if it is a benign or malignant leukocytosis and the analysis of the blood formula is the first step. The presence of abnormal cells such as blast cells is an alarm which must immediately consider the diagnosis of leukemia, and medullogram has to be performed. The presence of lymphoma cells will lead to the conclusion of lymphoproliferative disorder and the presence of myeloid precursors to a myeloproliferative disorder. However, a benign leukemoid reaction should be excluded. If there are no abnormal cells in the differential count, it will be necessary to distinguish hyperleucocytosis from myeloid origin (neutrophilies, eosinophilies, basophilies) from lymphoid origin. In the latter case, it is the lymphocytic phenotype that will confirm the malignancy by the presence of a clone of T or B lymphocytes but also by the monomorphic appearance of lymphocytes. In cytologically pleomorphic lymphocytosis, lymphoid reactions related to viral infections, autoimmune diseases or drug-related lymphoid reactions will be sought. The different causes are discussed in detail.

L'hyperleucocytose ou l'augmentation des globules blancs dans le sang périphérique est une anomalie fréquemment rencontrée. Il est important de distinguer immédiatement une hyperleucocytose bénigne d'une hyperleucocytose maligne par l'analyse de la formule sanguine. La présence de blastes est une alarme qui doit immédiatement faire envisager le diagnostic de leucémie aiguë. La présence de cellules lymphomateuses orientera vers un désordre lymphoprolifératif et la présence de précurseurs myéloïdes vers un syndrome myéloprolifératif. Toutefois, une réaction leucémoïde bénigne peut mimer un syndrome myéloprolifératif. S'il n'y a pas de cellules anormales dans l'examen de la formule sanguine, il faudra distinguer les hyperleucocytoses d'origine myéloïde (neutrophilies, éosinophilies, basophilies) des pathologies lymphoïdes. Dans ce dernier cas, c'est le phénotype lymphocytaire qui permettra de confirmer la malignité par la présence d'un clone de lymphocytes T ou B, mais également par l'aspect monomorphe des lymphocytes. Dans les lymphocytoses cytologiquement pléomorphe, on recherchera plutôt les réactions lymphoïdes liées à des infections virales, des maladies autoimmunes ou des réactions lymphoïdes liées à des drogues. Les différentes causes sont discutées en détail.

Pregnancy and newborn outcomes in arrhythmogenic right ventricular cardiomyopathy/dysplasia.

INTRODUCTION: The prognosis of pregnancy in patients with Arrhythmogenic Right Ventricular Cardiomyopathy/dysplasia (ARVC/D) is poorly documented. The aim of this study is to assess the cardiac risks during pregnancy and the impact of ARVC/D on fetuses/neonates/children. METHODS: We included all ARVC/D women with a history of pregnancy from the ARVC/D Pitié-Salpêtrière registry. Cardiac and obstetrical events having occurred during pregnancy/delivery/post-partum periods and neonatal data/follow-up were collected. RESULTS: Sixty pregnancies in twenty-three patients were identified between 1968 and 2016. Only two major non-fatal cardiac events (one sustained non-documented tachycardia and one ventricular tachycardia) were recorded during pregnancy in two different mothers (3% of pregnancies, 9% of mothers). None occurred during delivery or in the postpartum period. No mother developed heart failure. Beta-blocker therapy during pregnancy (n=15) was associated with lower birthweight (2730 vs 3400g, p=0.004). Only two preterm deliveries occurred, unrelated to cardiac condition. Caesarean section was performed in 13% of cases. Premature sudden-death occurred in 10% (n=5) of children before 25years-old including two in the first year of life. CONCLUSION: ARVC/D is associated with a low rate of major cardiac events during pregnancy and vaginal delivery appears safe. The risk of sustained ventricular arrhythmia seems poorly predictable and supports the continuation of beta-blockers during pregnancy. Major cardiac events were frequent in childhood, justifying close cardiac monitoring.

[Hyperthyroidism in family medecine]. / L'hyperthyroïdie en médecine générale.

Hyperthyroidism and sub-hyperthyroidism are common illnesses. Their diagnosis and their treatment are accessible to the general practitioner in the any great majority of the cases. A careful clinical examination already allows to direct the diagnosis. The development is simple and consists of a blood dosage, an ultrasound and sometimes a scintigraphy. Rare cases and severe forms are to be recognized and to refer to specialized centre. The treatment of first intention are betablockers. The comorbidities are cardiovascular (atrial fibrillation mostly) and skeletal (osteoporosis). Considering the increase of cardiovascular risks and the fracture risk in this pathology, the screening is especially indicated for women above 65 years. This screening is simple and little invasive, it consists of the annual dosage of the TSH.

L'hyperthyroïdie et l'hyperthyroïdie subclinique sont des pathologies fréquentes. Leur diagnostic et leur traitement sont accessibles au médecin généraliste dans la toute grande majorité des cas. Une anamnèse et un examen clinique attentif permettent déjà d'orienter le diagnostic. La mise au point est simple et consiste en un dosage sanguin, une échographie et parfois une scintigraphie. Des cas rares et des formes sévères sont à reconnaître et à référer en centre spécialisé. Les risques associés à ces pathologies sont principalement d'ordre cardiovasculaire (fibrillation auriculaire) et fracturaire (augmentation de l'ostéoporose) Les traitements de première intention sont les bêtabloquants et les antithyroïdiens de synthèses. Compte tenu de la majoration des risques cardiovasculaire et fracturaire liés à cette pathologie, le dépistage est indiqué surtout chez la femme de plus de 65 ans. Ce dépistage est simple et peu invasif, il consiste en un dosage annuel de la TSH.

[Vaccination before and during pregnancy]. / La vaccination de la femme avant la grossesse et pendant la grossesse.

Vaccination of pregnant women is designed to protect the mother and the fetus from preventable diseases through vaccination. Authorized vaccines contain inactivated viruses, otherwise (l ive virus), they cannot be made during pregnancy. Vaccines offered in all circumstances are those against influenza, at any point in pregnancy, and diphtheria-tetanus-pertussis, in the 3rd trimester. Hepatitis A and/or B, meningitis, pneumococcal, rabies, anthrax vaccines must be considered in post exposure. There is not enough data on vaccines against typhoid fever, Japanese encephalitis, cholera during the pregnancy. Vaccines to be done at distance of pregnancy are those against chickenpox, rubella, measles and mumps for unprotected women, as well as the vaccine against HPV, BCG, yellow fever and smallpox. In conclusion, only influenza vaccines and diphtheria-tetanus-pertussis showed a safe profile during pregnancy. Other vaccines should be considered in the event of exposure to risk of illness with the help of an infectious disease specialist.

La vaccination de la femme enceinte vise à protéger la mère et le foetus de maladies évitables par cette démarche. Les vaccins autorisés contiennent des virus inactivés, dans le cas contraire (virus vivants), ils sont contre indiqués pendant la grossesse. Les vaccins à proposer en toutes circonstances sont ceux de la grippe à n'importe quel moment de la grossesse et de la diphtérie-tétanos-coqueluche, au 3e trimestre. En cas d'exposition à risque, on peut envisager la vaccination contre l'hépatite A et/ou B, la méningite, le pneumocoque, la rage, l'anthrax. Les données sont insuffisantes concernant les vaccins de la fièvre typhoïde, de l'encéphalite japonaise et du choléra. Les vaccins proposés en dehors de la grossesse sont ceux de la varicelle, la rubéole, la rougeole et les oreillons pour les femmes non protégées, ainsi que le vaccin de l'HPV, la fièvre jaune et le BCG. En conclusion, seuls les vaccins antigrippaux et anti diphtérie-tétanos-coqueluche ont montré un profil d'innocuité sûr pendant la grossesse. Les autres vaccins doivent s'envisager en cas d'exposition à risque après avis d'un infectiologue.

Does high-resolution computed tomography image analysis of the distal radius provide information independent of bone mass?

This study aimed to investigate the usefulness of computed tomography (CT) image analysis of the distal radius for comparing two groups of postmenopausal women matched for age and bone mineral density at both the lumbar spine and femoral neck. The first one consisted of 16 women with at least one vertebral fracture and the second consisted also of 16 women without disease affecting bone mass or bone metabolism. Eight slices were selected in each patient: four consecutive coronal slices and four consecutive axial slices. Bone texture analysis was performed using structural methods leading to the measurement of 24 features. Most of the structural variables derived from histomorphometric parameters and were measured after segmentation from a binary or a skeletonized image. Nine variables were significantly different between the two groups on axial slices: valley number, valley surface area, apparent bone volume/tissue volume (BV/TV), apparent trabecular separation, apparent trabecular number, trabecular bone pattern factor, trabecular skeletal length, node count, and node-to-node strut count. Also four variables were significantly different between osteoporotic women and controls on coronal slices: apparent BV/TV, trabecular partition, node-to-node strut count, and terminus-to-terminus strut count. In conclusion this study suggests that bone texture analysis could yield additional data on bone mass for explaining bone strength and therefore could be used for improving the prediction of fracture risk.