[Gardner syndrome. Maxillofacial management of an extremely advanced form]. / Syndrome de Gardner. Prise en charge maxillo-faciale d'une forme évolutive extrême.

Gardner's syndrome is a very rare hereditary disease resulting from an embryo genesis disorder involving all three ectodermal, mesodermal and endodermal layers. Generally non-digestive tract signs disclose the syndrome. Underlying silent digestive anomalies should be investigated. Endodermal anomalies may have a fundamental effect on prognosis. Manifestations in our case were severe, with ectodermal and mesodermal anomalies occurring late. Maxillofacial management was required because of the critical esthetic and functional implications.

Survival response of a human glioma cell line to hyperthermia associated with rhein.

The effect of association of hyperthermia with the anti-inflammatory drug rhein (RH), 4,5-dihydroxyanthraquinone-2-carboxylic acid, on the clonogenic activity of human glioma cells has been examined. RH inhibits neoplastic growth mainly through an ATP depletion, but thermal cell killing is not mediated by the drug-induced changes in the energy status of the cell. The analysis of the interaction between RH and hyperthermia, performed with the isobolar method, demonstrates an additivity of the response so that the effectiveness of the combined treatment is the result of two independent effects. Although the effect of this combination is purely additive, RH allows us to achieve a pre-established cell killing with exposure times at 42 degrees C, which is generally accepted to be clinically achievable. RH might, therefore, be employed to reduce the side effects of hyperthermia without impairing its therapeutic effectiveness.

Protein synthetic activity and adenylate energy charge in Rhein-treated cultured human glioma cells.

The effect of Rhein (RH) on the protein synthetic activity and adenylate energy charge in human glioma cells cultured in vitro has been investigated. The results demonstrate that in RH-treated cells, the protein synthesis is strongly decreased, but no modifications in the qualitative pattern occur. The extent of inhibition is a function of the drug concentration as well as of the time of exposure. Such an inhibition must be ascribed mainly to a reduction of adenylate energy charge brought about by RH because of its effect on respiration and glycolysis. The correlation between the adenylate energy charge and cell viability, as well as the possibility of using rhein as a biochemical modulator to reduce or to reverse multidrug resistance, are also discussed.

Cytotoxic effect of the association of BCNU with rhein or lonidamine on a human glioma cell line.

The effect of association of 1,3-bis-(2-chloroethyl)-l-nitrosourea (BCNU)- Rhein (RH) and BCNU-Lonidamine (LND) on the clonogenic activity of human glioma cells was evaluated. Both RH and LND modulate the lethal effect of BCNU regardless of the schedule of treatment. The analysis of the interactions, performed with the isbolar method according to Berembaum, demonstrates an additivity of the effects. The possible mechanisms as well as the implications for the design of brain tumor schedule treatment are discussed.