[Cardiac manifestations of sickle cell anemia].

HEMOGLOBINS S AND C: Drepanocytosis, the occurrence of sickle cells (drepanocytes) in the blood, is an inherited condition. Electrophoresis demonstrates hemoglobin SS in homozygous subjects who present the typical clinical features of severe hemolytic sickle-cell anemia. Heterozygous subjects have sickle-cell anemia trait, an asymptomatic condition associated with a 50% hemoglobin S and 50% hemoglobin C at electrophoresis.

Double heterozygosity for mutations in the beta-myosin heavy chain and in the cardiac myosin binding protein C genes in a family with hypertrophic cardiomyopathy.

Familial hypertrophic cardiomyopathy is a genetically heterogeneous autosomal dominant disease, caused by mutations in several sarcomeric protein genes. So far, seven genes have been shown to be associated with the disease with the beta-myosin heavy chain (MYH7) and the cardiac myosin binding protein C (MYBPC3) genes being the most frequently involved. We performed electrocardiography (ECG) and echocardiography in 15 subjects with hypertrophic cardiomyopathy from a French Caribbean family.

[Venous thromboembolic complications following air travel. Retrospective study of 40 cases recorded in Martinique].

Thromboembolic events following air travel do occur, and have been reported several times in the literature. The authors report a high frequency of these incidence in their geographical region. A retrospective study of 40 cases of phlebitis or pulmonary embolism associated with air travel was conducted over the last 6 years.

[Congenital coronary-cardiac fistula. Diagnosis by color Doppler echocardiography in an infant].

The authors report the case of a 2 month old child in whom a coronary artery fistulae was diagnosed by colour Doppler echocardiography. Routine two-dimensional echocardiography showed a very dilated right coronary artery. Turbulent flow was detected in this vessel by pulsed Doppler echocardiography.

[Left ventricule function and sickle-cell anemia. Echocardiographic study].

The dimensions of the cardiac chambers and left ventricular function were studied by echocardiography in 40 patients with homozygous sickle cell disease (SS) and 25 patients with heterozygous sickle cell trait (12 AS, 10 SC, 3 BS) and compared with the same parameters in 30 normal subjects. Patients with homozygous SS disease had significantly larger left atrial (32.2 +/- 6.

[Pulmonary embolism and disseminated intravascular coagulation after being bitten by a Bothrops lanceolatus snake. Apropos of a case].

The authors report the case of a Bothrops lanceolatus snake bite complicated by severe pulmonary embolism a few hours after admission. This thromboembolic complication developed despite heparin therapy and was followed by disseminated intravascular coagulation (DIC). Vascular thrombosis and pulmonary embolism are rare after Bothrops lanceolatus snake bite as patients are usually hypocoagulable due to DIC.

[Congenital diverticulum of the right ventricle apropos of a case complicated by pulmonary endocarditis].

Congenital diverticulum of the right ventricle is an extremely rare abnormality, usually discovered by chance during evaluation of a more complex cardiac malformation. We report a case of isolated diverticulum of the right ventricle in a 14-year old boy who also had pulmonary valve endocarditis secondary to acute osteomyelitis. Owing to the evolutive risks inherent in the diverticulum and to the persistence of highly mobile pulmonary valve vegetations 5 years after the initial infectious episode, surgical treatment of the lesions had to be performed.

[Bouillaud's disease in Martinique. Epidemiological and nosological features].

From the epidemiological point of view there does not appear to be any particular geographical pattern of this disease. Indeed, it is poor living conditions, low family income, large family size and poor oro-dental hygiene which are responsable for the outbreak of small familial epidemics of acute rheumatic disease of the joints; from the clinical point of view, acute rheumatic joint disease presents no particular features in the Antilles. All the characteristics described in the classical works are found, including the malignant form, whose rarity is emphasised.