[Cowden syndrome]. / Le syndrome de Cowden.

INTRODUCTION: Cowden syndrome is rare; oral symptoms are not always diagnosed. This case of Cowden syndrome was revealed by gingival hypertrophy. OBSERVATION: A 21-year-old female patient consulted for gingival hypertrophy and multiple papules in the mouth. She had a history of thyroid lobectomy due to a goiter. The gingival papillomatosis and the goiter suggested a Cowden syndrome. The diagnosis was confirmed clinically by facial skin papules. DISCUSSION: Cutaneous and oral lesions are usually the first symptoms of the syndrome. Diffuse gingival papillomatosis may suggest a Cowden syndrome and should lead to screen for associated symptoms. A high-risk diagnosis of breast and thyroid cancer is associated to Cowden syndrome and the patient should have a yearly follow-up.

Use of sedation for routine diagnostic upper gastrointestinal endoscopy: a European Society of Gastrointestinal Endoscopy Survey of National Endoscopy Society Members.

BACKGROUND/AIMS: Sedation rates may vary among countries, depending on patients' and endoscopists' preferences. The aim of this survey was to investigate the rate of using premedication for routine diagnostic upper gastrointestinal (UGI) endoscopy in endoscopy societies, members of the European Society of Gastrointestinal Endoscopy (ESGE). METHODS: We evaluated a multiple-choice questionnaire which was e-mailed to representatives of national endoscopy societies, which are members of the ESGE. The questionnaire had 14 items referring to endoscopy practices in each country and the representatives' endoscopy units. RESULTS: The response rate was 76% (34/45). In 47% of the countries, less than 25% of patients undergo routine diagnostic UGI endoscopy with conscious sedation. In 62% of the responders' endoscopy units, patients are not asked their preference for sedation and do not sign a consent form (59%). Common sedatives in use are midazolam (82%), diazepam (38%) or propofol (47%). Monitoring equipment is not available 'in most of the endoscopy units' in 46% (13/28) of the countries. Though they were available in 91% of the national representatives' endoscopy units, they are rarely (21%) used to monitor unsedated routine diagnostic UGI endoscopy. CONCLUSIONS: In about 50% of ESGE-related countries, less than 25% of patients are sedated for routine diagnostic UGI endoscopy. Major issues to improve include availability of monitoring equipment and the use of a consent form.

[Primary angiosarcoma of the breast. Apropos of 2 cases]. / Les angiosarcomes primitifs du sein. A propos de 2 cas.

We report here 2 cases of breast angiosarcoma observed at Centre Val-d' Aurelle of Montpellier over a period of 14 years (1980 to 1994). Based on a review of literature, we analyze the epidemiological, pathological, clinical, diagnostic and treatment aspects of this rare type of breast cancer. The difficulties of histological diagnosis are underlined. Mastectomy is the treatment of reference. Breast angiosarcoma has the worst prognosis of all mammary malignancies, with a mean survival of 24 months. The low histologic grade and an early diagnosis are the most important factors of good prognosis. The benefit of irradiation and chemotherapy as adjuvant therapy remains to be demonstrated.

[Radiation-induced sarcoma]. / Les sarcomes radio-induits.

Based upon the literature, we discuss epidemiological, histopathological, clinical and treatment aspects of postirradiation sarcomas. The risk of postirradiation sarcoma is very low and negligible in comparison with the beneficial effect of radiotherapy, and it should not be a major factor influencing treatment decision in patients with cancer. The radical surgical approach of postirradiation sarcoma is the treatment which offers the best chance for cure.

Mutations in STS1 suppress the defect in 3' mRNA processing caused by the rna15-2 mutation in Saccharomyces cerevisiae.

In a search for proteins associated with Rna15p in processing the 3' ends of messenger RNAs, we have looked for suppressors that correct, even partially, the thermosensitive growth defect of the rna15-2 mutant. Mutations in a single locus that we named SSM5, were able to suppress both the thermosensitivity of cell growth and the mRNA 3' processing defect associated with the rna15-2 mutation, but only slightly alleviated the thermosensitive growth defect of an rna14-1 mutant. The ssm5-1 mutant is sensitive to hydroxyurea at 37 degrees C, a drug that inhibits DNA synthesis. By screening for complementation of the hydroxyurea-sensitive phenotype we cloned the corresponding wild-type gene and found that it corresponds to the essential gene STS1 (also named DBF8). Sts1p has an apparent molecular weight of 30 kDa and was confirmed to be a cytosolic protein by immunofluorescence analysis. Western blot analysis indicates that the thermosensitive mutant strains rna15-2, rna14-1 and pap1-1 present a very low level of the Rna15p at 37 degrees C. The ssm5-1 mutation restores the level of Rna15p in the rna15-2 ssm5-1 double mutant. Use of the two-hybrid system suggests that Sts1p does not interact directly with Rna15p, but may be active as a homodimer. The present data suggest that Sts1p may play a role in the transport of Rna15p from the cytoplasm to the nucleus.

[Anorectal malignant melanomas. Apropos of 5 cases. Review of the literature]. / Les mélanomes malins anorectaux. A propos de 5 cas: revue de la littérature.

This study reports five cases of anorectal melanoma treated at Centre Val d'Aurelle-Paul Lamarque in Montpellier. There follows a discussion based upon the literature of epidemiological, clinical, histopathological and therapeutic aspects. Surgery is the treatment of choice but there is major controversy regarding the best method. The decisive prognostic factor appears to be the stage of the disease rather than the type of treatment. The role of radiotherapy, adjuvant chemotherapy and immunotherapy remains to be evaluated.

Hypotheses for a re-evaluation of peroxidase activity in the oral inflammation: NAD(P)H-dependent hypohalite reduction in human neutrophils.

The balance of NADH oxidation by neutrophil lysates in the presence and in the absence of peroxidase/H2O/halide systems, pointed out a NADH oxidation excess when these hypohalite generators were actually present. This unexplained NADH consumption could not be attributed to a direct oxidation by hypohalites nor by the lysate self. Using OCI- as substrate, the NADH oxidation excess ranged from 52 to 99 mU/ml at pH 7.40 and from 446 to 1346 mU/ml at pH 6.00 (N = 6). Similar results were also found while using hypoiodous and hypothiocyanous compounds at pH 7.40 but not at pH 5.50. The authors discuss different hypotheses to explain this phenomenon and accordingly elaborate on the possible consequences of neutrophils degranulation in the oral tissues during inflammation processes.

[Statistical evaluation of biochemical data by the method of discrimination analysis. Selection of the discriminant biochemical variables. Attempted biochemical discrimination of intrahepatic cholestasis, extrahepatic obstruction and liver cancer]. / Traitement statistique des données biochimiques par la méthode d'analyse discriminante. Sélection des variables biochimiques discriminantes. Essai de discrimination biochimique de la cholostase intrahéatique de l'obstacle extrahépatique et du cancer du foie

An original method of statistical treatment of biological data is proposed. It permits satisfactory biochemical classification of 322 patients divided up into 3 groups : intrahepatic cholestasis (235 patients), extrahepatic obstruction (44 patients) and carcinoma of the liver (43 patients). On the basis of 32 tests, it was possible to define discriminating areas permitting satisfactory diagnosis in 95 per cent of published cases. The reduction in the number of tests necessary for diagnosis was considered. The selection technic used was original to the extent that it dose not require, like most methods used today, the determination of better individual discriminators, but the establishment of a better discriminating subunit, obtained from the initial subunit composed of a group of variables. From the 32 parameters contained in the standard liver function tests, a search for a better discriminating subunit consisting of the best four tests, permitted the authors to select a group of 10 tests : bilirubin, alkaline phosphatase, 5-nucleotidase, Thymolturbidity, Cetavlon test, serum albumin, total LDH, TGP (ALAT), OCT, GLDH, of which the discriminating value remains very satisfactory.