A process for making certain spiroheterocyclic pyrrolidine dione derivatives.

Present invention relates to a process and an installation (100) for the preparation of hydrogen cyanide by the Andrussow process, and more precisely for improving the conditions of mixing the reactant gases before feeding the Andrussow type reactor (60), in order to improve safety, to avoid any risk of explosion and to produce HCN in safe and efficient manner. The installation is configured in such a manner that oxygen is pre-mixed with air with a ratio comprised between 20.95% and 32.5% by volume, preferably between 25% and 30.5% by volume; methane containing gas and ammonia are simultaneously added in the pre-mixture of oxygen-enriched air in such a manner that the volumic ratio of methane to ammonia is comprised between 1.35 and 1.02 depending on the content of oxygen into air; said obtained reactant gases mixture having a temperature comprised between 80 C. and 120 C., preferably between 95 C. and 115 C. for feeding the Andrussow type reactor (60).

Present invention relates to a process and an installation (100) for the preparation of hydrogen cyanide by the Andrussow process, and more precisely for improving the conditions of mixing the reactant gases before feeding the Andrussow type reactor (60), in order to improve safety, to avoid any risk of explosion and to produce HCN in safe and efficient manner. The installation is configured in such a manner that oxygen is pre-mixed with air with a ratio comprised between 20.95% and 32.5% by volume, preferably between 25% and 30.5% by volume; methane containing gas and ammonia are simultaneously added in the pre-mixture of oxygen-enriched air in such a manner that the volumic ratio of methane to ammonia is comprised between 1.35 and 1.02 depending on the content of oxygen into air; said obtained reactant gases mixture having a temperature comprised between 80 C. and 120 C., preferably between 95 C. and 115 C. for feeding the Andrussow type reactor (60).

The invention relates to a thermal conversion vessel (200) used during amidification step of acetone cyanohydrin (ACH), in the industrial process for production of a methyl methacrylate (MMA) or methacrylic acid (MAA). The thermal conversion vessel (200) is used for converting an hydrolysis mixture of -hydroxyisobutyramide (HIBAM), -sulfatoisobutyramide (SIBAM), 2-methacrylamide (MACRYDE) and methacrylique acid (MAA), into a mixture of 2-methacrylamide (MACRYDE). It comprises: at least one compartment (C1, C2, C3, . . . Ci) comprising an inner wall (206a, 206b, 206i) separating said compartment into two communicating parts (C1a, C1b) by a passage provided between the bottom of said vessel and said inner wall, said compartment having a space above said inner wall, for separating gas phase from liquid phase during thermal conversion, said compartment being connected to an outlet valve (204a, 204b, . . . 204i).

Such vessel allows obtaining a high yield thermal conversion in very safe conditions.

The invention relates to a thermal conversion vessel (200) used during amidification step of acetone cyanohydrin (ACH), in the industrial process for production of a methyl methacrylate (MMA) or methacrylic acid (MAA). The thermal conversion vessel (200) is used for converting an hydrolysis mixture of -hydroxyisobutyramide (HIBAM), -sulfatoisobutyramide (SIBAM), 2-methacrylamide (MACRYDE) and methacrylique acid (MAA), into a mixture of 2-methacrylamide (MACRYDE). It comprises: at least one compartment (C1, C2, C3, . . . Ci) comprising an inner wall (206a, 206b, 206i) separating said compartment into two communicating parts (C1a, C1b) by a passage provided between the bottom of said vessel and said inner wall, said compartment having a space above said inner wall, for separating gas phase from liquid phase during thermal conversion, said compartment being connected to an outlet valve (204a, 204b, . . . 204i).

Such vessel allows obtaining a high yield thermal conversion in very safe conditions.

The invention relates to a thermal conversion vessel (200) used during amidification step of acetone cyanohydrin (ACH), in the industrial process for production of a methyl methacrylate (MMA) or methacrylic acid (MAA). The thermal conversion vessel (200) is used for converting an hydrolysis mixture of -hydroxyisobutyramide (HIBAM), -sulfatoisobutyramide (SIBAM), 2-methacrylamide (MACRYDE) and methacrylique acid (MAA), into a mixture of 2-methacrylamide (MACRYDE). It comprises: at least one compartment (C1, C2, C3, . . . Ci) comprising an inner wall (206a, 206b, 206i) separating said compartment into two communicating parts (C1a, C1b) by a passage provided between the bottom of said vessel and said inner wall, said compartment having a space above said inner wall, for separating gas phase from liquid phase during thermal conversion, said compartment being connected to an outlet valve (204a, 204b, . . . 204i).

Such vessel allows obtaining a high yield thermal conversion in very safe conditions.

The present invention provides compounds and methods for the treatment of an individual having or at risk of having a condition associated with alpha-1-antitrypsin by using a pharmacological chaperone. In particular, such methods are useful for the treatment and/or prevention of lung disorders associated with alpha-1-antitrypsin deficiency as well as liver disorders associated with an excess of alpha-1-antitrypsin. Suitable pharmacological chaperones include peptides and low-molecular weight compounds. The present invention also provides an assay for determining whether a test compound modulates alpha-1-antitrypsin activity.

The present invention provides compounds and methods for the treatment of an individual having or at risk of having a condition associated with alpha-1-antitrypsin by using a pharmacological chaperone. In particular, such methods are useful for the treatment and/or prevention of lung disorders associated with alpha-1-antitrypsin deficiency as well as liver disorders associated with an excess of alpha-1-antitrypsin. Suitable pharmacological chaperones include peptides and low-molecular weight compounds. The present invention also provides an assay for determining whether a test compound modulates alpha-1-antitrypsin activity.

The present invention provides a method for producing -hydroxyisobutyric acid amide by hydration of acetone cyanohydrin under the presence of a catalyst composed mainly of manganese oxide using a reactor in which at least two reaction regions are connected in series, the method being characterized by comprising: a step (B) of cyclically supplying at least a portion of a reaction liquid withdrawn from at least one reaction region to a first reaction region (I) in the reactor; and a step (b1) of further cyclically supplying at least a portion of the reaction liquid withdrawn from at least one reaction region to at least one reaction region other than the first reaction region. The method is also characterized in that an oxidizing agent is supplied to at least one reaction region in the reactor.

A method of preventing polymer tar build-up in ACH production of MAA and/or MMA is described. The method is characterised in that one or more surfactants are contacted with the hydrolysis and optional esterification stage reaction medium, the said surfactants are selected from: a) C.sub.10 to C.sub.30 alcohol ethoxylates with an average of 5 to 100 ethylene oxide units per molecule; b) alkyl, hydrogen, O[CH.sub.2CH.sub.2O].sub.xH and/or O[CH.sub.2CH.sub.2CH.sub.2O].sub.x H N-substituted alkylene di- or triamines with an average of 1 to 30 total ethylene oxide and propylene oxide repeating units per molecule and wherein x and x are from 1 to 30; and c) C.sub.10 to C.sub.30 alcohol ethoxylate, propoxylates with an average of 5 to 100 total propylene oxide and ethylene oxide units per molecule, which units may be in a random, block or alternating sequence or may be a combination thereof. The method is particularly useful for preventing build-up of oligomer and polymer tar-like deposits in reaction vessels, process equipment, pipework or other parts of the acetone cyanohydrin MMA and MAA production process.