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C Vilain

Publications and source records attributed to C Vilain.

At least 19 recordsLinked to original sources
PubMed1999

Sporadic case of trichorhinophalangeal syndrome type III in a European patient.

Trichorhinophalangeal syndrome type III (TRP III) shares common traits with TRP I and II, including sparse hair, a "pear-shaped" nose, osteodysplasia with cone-shaped epiphyses, and autosomal dominant inheritance, but is distinguished by the presence of severe brachydactyly. TRP III was first described in 1984 in Japanese patients, one sporadic case [Sugio and Kajii, 1984: Am. J. Med. Genet. 19:741-753,1984] and two families [Niikawa and Kamei, 1986: Am. J. Med. Genet. 24:759-760; Nagaï et al., 1994: Am. J. Med. Genet. 49:278-280], and more recently in a Turkish family [Itin et al., 1996: Dermatology 193:349-352]. We report an additional observation in a patient of European descent, who presented with short stature, cone-shaped epiphyses, sparse hair, a pear-shaped nose, normal intelligence and severe brachydactyly. Neither parent had manifestations of TRP and there was no other reported case in the family, indicating a presumably fresh mutation. Our observation refines the clinical spectrum of TRP III in another ethnic background and may be of help in identifying the gene or genes for TRP syndromes.

Abnormalities, Multiple↗
PubMed1998

Decrease of telomere length in thyroid adenomas without telomerase activity.

In somatic cells, telomeres shorten with population doubling, thus limiting their capacity to divide. Telomerase, which synthesizes telomeric repeats, can compensate for such shortening. Telomerase activity is known to be absent from most somatic differentiated cells but is present in germline cells, immortal cell lines, or a large majority of malignant tumors. Autonomous thyroid adenomas are benign tumors composed of highly differentiated cells characterized by TSH-independent function and growth. Telomere length and telomerase activity were measured in autonomous and hypofunctioning adenomas and their surrounding tissues. A significant decrease of 3.8+/-1.0 kilobases (kb) was observed in the length of the terminal restriction fragments (TRF) in 12 autonomous adenomas (8.6+/-1.1 kb), compared with the TRF length of their surrounding tissues (12.4+/-1.6 kb). The same kind of decrease, 3.5+/-1.2 kb, was also observed in 16 hypofunctioning adenomas (12.3+/-1.7 kb in surrounding tissue and 8.8+/-1.6 kb in the adenomas). No telomerase activity was detected either in the 12 autonomous adenomas studied or in most of the quiescent tissues (10 of 12). Most of the hypofunctioning adenomas tested (15 of 16) did not display telomerase activity. These results suggest that the cells have undergone a higher number of cell divisions in the adenomas than in the surrounding tissue. Moreover, there is a larger spread of the TRF length distribution in autonomous adenomas than in the collateral tissue. This could reflect the heterogeneity in proliferation status of the cells in the nodule, some of which have reached the end of their life span, whereas others are still proliferating (but with no malignant potential for the autonomous adenomas). In conclusion, benign adenomas exhibit a shorter and more variable telomere length than the normal collateral quiescent tissue, with no telomerase activity to compensate this loss in telomere length.

Adenoma↗
PubMed1997

Characteristics of chronic venous insufficiency in 895 patients followed in general practice.

BACKGROUND: This study was designed to describe the main semiological and etiological characteristics of chronic venous insufficiency (CVI) and to determine if there was a relationship between the extent of objective signs, severity of symptoms and aetiology. MATERIALS AND METHODS: 895 outpatients presenting CVI of the lower limbs over a period of at least one year, irrespective of grade of severity or aetiology, were included in this retrospective study. They were treated with 2 different pharmaceutical forms of the same venoactive medication (1000 mg of micronised flavonoid fraction) for 2 months. Organic CVI (OCVI) was classified, in stages of increasing severity, according to the Widmer and Porter classification. In the absence of anatomical lesions of the main veins or their valvular system, CVI was termed functional (FCVI). RESULTS: Analysis indicated that CVI was more frequent in women than in men (sex ratio 10:1). 26% were FCVI and 91% of OCVI were of varicose origin. The mean progression time of the disease was 13+/-11 years. Disease began earlier in women than in men (34+/-14 vs 41+/-14 years). Oedema was the first objective sign in 68% of patients and the only one in 20% of FCVI. Heaviness was more frequent in FCVI and its intensity was not related to the severity of CVI. Trophic complications were more frequent in the advanced stages. CONCLUSIONS: In order to avoid progression to more severe forms which are disabling or expensive to treat, a rational approach to the management of early CVI is essential.

Adolescent↗
PubMed1990

[Aortic-pulmonary chemodectoma (non-chromaffin paraganglioma). Apropos of a case which followed an adrenal pheochromocytoma].

The authors present the 61st published case of an aorticopulmonary chemodectoma diagnosed in patient of 59 years who had been operated on 7 years previously for a right sided adrenal pheochromocytoma. The diagnosis was provided by the histological examination of the operative specimen, since computerised tomography had predicted that this large hypervascular tumour of the anterior mediastinum would be totally resectable.

Adrenal Gland Neoplasms↗
PubMed1990

[A rare tumor of the mediastinum: benign hemangioma].

Mediastinal hemangiomas are rare tumours occurring more often in children and young adults. A new case is reported in a 21 years old male who had an anterior mediastinal mass detected on a routine chest roentgenogram. Pre-operative investigations including CT, venous digital angiography, MRI did not aid in the right diagnosis. The mass was totally removed surgically although involving extensively adjacent structures. Histologic examination of the tumour showed it to be a benign venous hemangioma. Clinical, radiological, pathologic features of mediastinal hemangiomas are reviewed and discussed.

Adult↗
PubMed1989

[Chronic relapsing polychondritis. Clinical characteristics].

Some imperfectly known clinical aspects of polychondritis chronica atrophicans (relapsing polychondritis), as extracted from a series of 12 cases, are presented. The osteoarticular lesions are sometimes unusual, involving the temporomandibular or cervical articulations, and the renal lesions may be severe. Pericarditis seems to be more frequent than usually mentioned in the literature. Pulmonary complications are not always those which are classically associated with lesions of the tracheo-bronchial cartilages. Haematological manifestations are not uncommon and must be taken into account when evaluating the prognosis of the disease.

Aged↗
PubMed1987

[Use of very low doses of intravenous immunoglobulins for the treatment of bullous pemphigoid].

High dose intravenous immunoglobulins have proved effective immunosuppressants in various autoimmune diseases, including bullous pemphigoid. However, their specific point of impact remains undetermined and no study supports the need for high doses. We treated elderly patients with proven and corticosteroid-resistant bullous pemphigoid, using a therapeutic regimen consisting of continuous oral corticosteroid therapy and continuous infusion of immunoglobulins in very low doses by means of an electric syringe. Clinical improvement was observed in every case without any characteristic change in immunological parameters (anti-basal membrane antibody, cutaneous immune deposits), which suggests that immunoglobulins administered in that way act on immune response regulatory mechanisms. The therapeutic effect observed potentiates that of corticosteroids which can thus be given in lower doses in bullous pemphigoid.

Aged↗
PubMed1987

Characterization of axolotl heavy and light immunoglobulin chains by monoclonal antibodies.

Axolotl specific antibodies to 2,4-dinitrophenyl (DNP) were purified by affinity chromatography from the sera of animals immunized with 2,4,6-trinitrophenylated sheep red blood cells (TNP-SRBC). The purified anti-TNP/DNP antibodies, when analyzed by SDS-PAGE, were constituted of high molecular weight molecules, which in reducing conditions, were separated into heavy 72-88 kD and light 27-30 kD polypeptides. The axolotl heavy antibody chains strongly bound Concanavalin-A and migrate faster in SDS-PAGE after endoglycosidase-F (Endo-F) treatment. Using the same techniques, no carbohydrate components were detected onto light chains. Monoclonal antibodies (MAbs) were obtained against these purified axolotl immunoglobulins (Ig) and their specificities were studied by immunoblotting. MAbs 33.45.1 and 33.101.2 respectively recognized heavy and light chains determinants of the Ig molecule. These determinants were resistant to Endo-F digestion, suggesting that the two MAbs were not directed to polypeptide-associated N-linked high mannose or complex oligosaccharides. MAbs 33.45.1 and 33.101.2 were compared to 11.5.2, an anti-axolotl thymocytes MAb which was reactive for both axolotl leucocytes and soluble Ig. MAb 11.5.2 reacted in immunoblotting against several high molecular weight axolotl serum proteins, including heavy Ig chains. Light chains were not recognized. However, 11.5.2 did not further recognize Endo-F treated Ig, suggesting its specificity for a carbohydrate determinant of the heavy chain, and link to a large diversity of soluble or membrane glycoproteins.

Ambystoma↗
PubMed1986

Cyclic regulation of cytokinesis in amphibian eggs.

The effects of amphibian egg cytoplasm extracted at different times after activation and during the first four cleavages on cytokinesis were examined. Extracts of artificially activated or fertilized Xenopus or Pleurodeles eggs taken at the time of activation (T = 0) provoked precocious cleavage furrows in Pleurodeles eggs. Between T = 0.25 and T = 0.75 of the first cell cycle, the period corresponding to interphase, an inhibitory effect was found, and the division of injected eggs was delayed up to 30%. After T = 0.75, that is during mitosis, the cleavage induction effect was observed again. These enhancing and inhibitory effects were also found in the two fractions obtained following gel filtration of the cytoplasmic extracts. These experiments support the hypothesis that two antagonistic factors control cytokinesis. The inhibitory factor is active only during interphase, while the positive factor is present during mitosis and appears to regulate cytokinesis.

Animals↗
PubMed1985

[Raynaud's phenomenon after chemotherapy. Apropos of 3 cases].

Raynaud's phenomenon is studied in three patients after cancer chemotherapy. In these cases, Raynaud's phenomenon occurs as: a true coincidence, a secondary complication of which neoplastic biological signs are thrombocythemia, hyperfibrinemia, immune complexes, cryoglobulinemia..., a therapeutic implication. The rate of therapeutic implication is difficult to precise. Raynaud's phenomenon is a little symptom over a severe illness. Several drugs are responsible for this fact. A literature review mentions Vincristine, Bleomycin, Cisplatin... For our three patients as in the literature review the tumoral nature is less important than the drug type. For us Bleomycin alone as in association is able to induce Raynaud's phenomenon. The occurrence of single Raynaud's phenomenon may be a systemic scleroderma is first sign. However it is important to affirm the true single Raynaud's phenomenon. In this case there is a lack of signs: scleroderma may be paraneoplastic, drugs may be pulmonary toxic, anticentromere antibodies may be positive during antineoplastic treatment. The pathogenesis for Raynaud phenomenon drug induction is unknown. For Bleomycin action the role of skin concentration, fibroblastic lesion, and vascular disturbance is discussed.

Adenocarcinoma↗
PubMed1985

[National information campaign in France. "One drink is O.K., 3 drinks is asking for trouble"].

Despite a downward trend in the consumption of alcohol during the last 10 years, France remains the main consumer of alcohol in the world. One in every 15 deaths is related to alcohol. Some of the reasons for the decrease in alcohol consumption are: the privilege to distill one's own alcohol is now limited to few people, advertisements are controlled, taxes on liquors have been increased, drunken drivers are severely penalized. Moreover, there have been structural changes, one of them being the installation of an improved piped water system. For the first time, in 1984, the French Committee for Health Education launched an information campaign concerning alcoholism. Traditions and customs related to the consumption of alcohol still have a strong positive connotation in France. So this very important consideration had to be taken into account by the planners of the campaign. The keynote of the campaign was therefore that one should drink moderately. The slogan chosen contains a note of humour which the translation does not render: "One glass is OK, three glasses, and your troubles begin". Television spots were widely used in the campaign as well as pamphlets, and various public relations meetings took place. In a preliminary evaluation, a nation-wide survey showed that 70% of the viewers remembered the slogan three months after the end of the campaign. Out of these, 86% thought the campaign was necessary, and 69% considered the campaign had been well-conceived.

Adolescent↗