Are there indications for reconstructive surgery in severe mitral regurgitation after acute myocardial infarction?

From 1972 to 1988, early surgery were performed in 26 patients with acute mitral regurgitation (MR) unresponsive to medical management complicating a recent acute myocardial infarction (AMI). The indication was acute pulmonary oedema (11), major left ventricular insufficiency (5), cardiogenic shock (10). Surgery was performed within 3 weeks following AMI. Mitral lesions were as follows: rupture of chordae tendinae (9) of papillary muscle (6), haemorrhagic necrosis of one (17) or two (9) papillary muscles. The mitral annulus was never found to be enlarged. The LV posterior wall was necrotic in 23, with a septal rupture in 3 and a giant aneurysm in 5. Valve replacement was performed in all but one patient. The 30-day mortality included 8 patients (31%). The cause of death was myocardial insufficiency in 5, early thrombosis of a disk valve in 1 and unrelated complications in 2. One patient deteriorated rapidly and had a Jarvik device implanted. Late results (1 month-15 years) showed 4 cardiac related deaths within the first year. One patient had to be transplanted after 1 year. Two patients died of non-cardiac problems at 3 months and 5 years. The probability of survival at 5 years is 43% and at 10 years 22%. In conclusion, there are still indications for early surgery in MR post AMI. Anatomical lesions of both papillary muscles and ventricular wall do not allow conservative surgery and new non-invasive technics provide a more promising strategy in such desperately ill patients.     

Diagnostics, prognostic and therapeutic exploitation of telomeres and telomerase in leukemias

Telomeres are specialized structures at the end of human chromosomes. Telomere length decreases with each cell division, thus, reflecting the mitotic history of somatic cells. Telomerase, the ribonucleoprotein enzyme which maintains telomeres of eukaryotic chromosomes, is up-regulated in the vast majority of human neoplasia but not in normal somatic tissues. In contrast to other somatic cells, normal primitive human hematopoietic cells and some peripheral blood cells expressed low levels of telomerase activity. This activity is thought to play an important role in self-renewal of hematopoietic stem cells. In malignant disorders, telomere lengths are generally shortened and telomerase expression and activity enhanced with high differences in the levels. Although it is necessary to be cautious in interpreting these data, there are indications that telomere length and telomerase expression and activity can serve as a molecular marker of the clinical progression and prognosis of most leukemias. Approaches that directly target telomerase, telomeres or telomerase regulatory mechanisms have been developed. Some of these anti-telomerase strategies in combination with conventional drugs proved to be promising in some types of leukemias.     

The role of amniocentesis in the management of chorioamnionitis with Listeria monocytogene

Listeriosis is prevalent in pregnant women. Associated morbidity includes miscarriage, chorioamnionitis, intrauterine and neonatal death. Maternal symptoms are not specific and the diagnosis is difficult with a high rate of false-negative microbiology results. We report here the case a patient who developed a chorioamnionitis at 31 weeks gestation. Diagnosis was established by examination of the amniotic fluid. We report a case of Literiosis in pregnancy diagnosed by direct examination of amniotic fluid obtained by amniocentesis.     

Retinoid/arsenic combination therapy of promyelocytic leukemia: induction of telomerase-dependent cell death.

Acute promyelocytic leukemia (APL) is efficiently treated with a cell differentiation inducer, all-trans retinoic acid (ATRA). However, a significant percentage of patients still develop resistance to this treatment. Recently, arsenic trioxide (As2O3), alone or in combination with ATRA, has been identified as an alternative therapy in patients with both ATRA-sensitive and ATRA-resistant APL. Previous investigations restricted the mechanism of this synergism to the modulation and/or degradation of PML-RARalpha oncoprotein through distinct pathways. In this study, using several ATRA maturation-resistant APL cell lines, we demonstrate in vitro that the success of ATRA/As2O3 treatment in APL pathology can be explained, at least in part, by a synergistic effect of these two drugs in triggering downregulation of telomerase efficient enough to cause telomere shortening and subsequent cell death. Such long-term low-dose combinatorial therapy strategies, developed also to avoid acute side effects, reinforce the notion that the antitelomerase strategy, based on a combination of active agents, should now be considered and evaluated not only in APL but also in other malignancies.     

Prenatal diagnosis of hydrocephalus-stenosis of the aqueduct of Sylvius by ultrasound in the first trimester of pregnancy. Report of two cases.

Hydrocephalus-stenosis of the acqueduct of Sylvius sequence (HSAS) is characterized by hydrocephalus, macrocephaly, adducted thumbs, spasticity, agenesis of the corpus callosum and mental retardation. X-linked hydrocephalus is known to be due to mutations in the gene coding for the neural cell adhesion molecule L1 (L1-CAM) and diagnosis is made by identification of a mutation in the L1-CAM gene. Prenatal diagnosis of HSAS is usually suggested on ultrasound examination showing hydrocephalus in a male fetus associated with bilateral adducted thumbs. Mutation screening of the L1-CAM gene is indicated when neuropathological examination shows hypoplasia of the corticospinal tract associated with aqueductal stenosis. We report here two cases of HSAS diagnosed within the same family by ultrasound examination in the first trimester of pregnancy when bilateral adducted thumbs were the only early ultrasound marker.