Cardiac transplantation in an extreme emergency

Urgent cardiac transplantation in very unstable patients, kept alive in intensive care unit, is a significant (34 percent) part of the Henri-Mondor's transplantation program. Nineteen patients received a donor graft from january 1986 to october 1988. Sixteen were bridged pharmacologically as 5 received a mechanical bridge. Time on waiting list ranged from 12 to 21 days. Six patients died in the first post-operative month. Death was due to uncontrolled preoperative problems in 5, donor graft failure in one. The survival rate at one year was 52 percent, to be opposed to 76 percent in stable patients. These figures must be considered when selecting the priority of the transplantation candidates.     

Nephrotic syndrome with minimal glomerular lesions, sensitive to corticoids, disclosing a chordoid sarcoma

A 65 year old woman had a minimal changes nephrotic syndrome (MCNS) with steroid-induced remission. An underlying malignancy was discovered at the time of relapse of proteinuria: it was a retroperitoneal chordoid sarcoma. Even though the tumor could not be excised, complete remission was again observed with corticosteroids. There was no second relapse when prednisone was discontinued and during the seven months before the patient died. This is a new unusual case of MCNS associated with carcinoma. The response of nephrotic syndrome steroid therapy is further suggestive evidence that deficiency in T-cell function may be involved.     

Benign mucus-secreting tumors of the appendix. Six cases]

The authors report six cases of mucus-secreting tumor of the vermicular appendix, in four women and two men (mean age 59 years). All were benign. Pain in the right lower quadrant was the initial sign in 4 cases, while the mucus-secreting tumor was an incidental finding in the other two cases. A mass was palpated in the right lower quadrant in one case. A barium enema was performed in four cases and showed an extrinsic compression of the caecal base in one case, and of the right colon in another case. Four appendectomies, one associated with removal of the adjacent caecal tissues and two right colectomies were performed. Analysis of these six cases and a review of the literature allow us to recall the histologic classification of these tumors, which determines the prognosis and to emphasize the sonographic and CT scan findings which might lead to earlier preoperative diagnosis in the future. Management depends upon the circumstances under which the tumor is discovered, the local anatomy, and the type of tumor. Appendectomy is the treatment most often performed. Faced with a tumor of the appendix, without histologic proof or when the local anatomic conditions increase the risk of opening an abscess, right colectomy is recommended.     

A variant translocation t(2;18) in follicular lymphoma involves the 5' end of bcl-2 and Ig kappa light chain gene

We have examined three cases of human lymphoma bearing a t(2;18)(p11;q21) chromosome translocation. The bcl-2 gene appeared to be rearranged in all three cases and breakpoints were clustered in the 5' flanking region of the gene. In all three cases, bcl-2 was juxtaposed to J segments of the Ig kappa gene. This juxtaposition of the bcl-2 and Ig kappa genes is very similar to the variant chromosome translocations of Burkitt lymphoma that juxtapose the c-myc locus to IgL genes.     

Factors predictive of perinatal outcome in pregnancies complicated by hypertension

Perinatal outcome and various indicators of perinatal risk were analyzed in a prospective study of 268 pregnant women with hypertension. Poor perinatal outcome was defined by stillbirth (n = 13), neonatal death (n = 2), and in surviving babies, by birth before 32 weeks or a birthweight below 1500 g (n = 13). In multivariate analysis, proteinuria and onset of hypertension between the 27th and 36th weeks of amenorrhea were the only two independent indicators of poor outcome (relative risks of 4.0 and 3.7, p less than 0.001 and p less than 0.01 respectively). Both these indicators were more frequent in mothers with no history of pre-pregnancy hypertension.     

hTERT promotes imatinib resistance in chronic myeloid leukemia cells: therapeutic implications

Imatinib mesylate has shown remarkable efficacy in the treatment of patients in the chronic phase of chronic myeloid leukemia. However, despite an overall significant hematological and cytogenetic response, imatinib therapy may favor the emergence of drug-resistant clones, ultimately leading to relapse. Some imatinib resistance mechanisms had not been fully elucidated yet. In this study we used sensitive and resistant sublines from a Bcr-Abl positive cell line to investigate the putative involvement of telomerase in the promotion of imatinib resistance. We showed that sensitivity to imatinib can be partly restored in imatinib-resistant cells by targeting telomerase expression, either by the introduction of a dominant-negative form of the catalytic protein subunit of the telomerase (hTERT) or by the treatment with all-trans-retinoic acid, a clinically used drug. Furthermore, we showed that hTERT overexpression favors the development of imatinib resistance through both its antiapoptotic and telomere maintenance functions. Therefore, combining antitelomerase strategies to imatinib treatment at the beginning of the treatment should be promoted to reduce the risk of imatinib resistance development and increase the probability of eradicating the disease.     

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.