. . . . . . . "1101394143"^^ . . . . . . . "Organic acid disorder"@en . . . . . . . . . . "289899" . "Organic aciduria,"@en . . . . . . . . "Le acidosi organiche sono un gruppo di patologie identificate da un abbassamento del pH ematico (acidosi), per , e da . L'acidosi \u00E8 quindi un sintomo, il sintomo preponderante di diverse patologie, e a queste ha dato il nome. In senso stretto si considerano \n* difetti della catena respiratoria \n* deficit di piruvato chinasi \n* deficit del ciclo di Krebs \n* deficit di gluconeogenesi. Nel senso pi\u00F9 lato, consideriamo anche \n* i difetti del metabolismo degli aminoacidi ramificati \n* i difetti della beta ossidazione degli acidi grassi."@it . . . 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"Acidosi organiche"@it . "Organic acidemia"@en . "14536832"^^ . . . . . . . . . "Organoazidopathie, Synonym: Organoazidurie, ist ein \u00DCberbegriff f\u00FCr eine Gruppe von Stoffwechselerkrankungen, die den Abbau von Aminos\u00E4uren oder Fetts\u00E4uren betreffen und \u00FCberwiegend einem autosomal rezessiven Erbgang folgen mit Anreicherung und Ausscheidung von organischen S\u00E4uren. Diese Krankheitsgruppe wird \u2013 historisch bedingt \u2013 nicht systematisch, sondern aufgrund der gemeinsamen Untersuchungstechnik Urinuntersuchung diagnostiziert."@de . . . . "Organic acidemia, is a term used to classify a group of metabolic disorders which disrupt normal amino acid metabolism, particularly branched-chain amino acids, causing a buildup of acids which are usually not present. The branched-chain amino acids include isoleucine, leucine and valine. Organic acids refer to the amino acids and certain odd-chained fatty acids which are affected by these disorders. The four main types of organic acidemia are: methylmalonic acidemia, propionic acidemia, isovaleric acidemia, and maple syrup urine disease."@en . "Acydurie organiczne (czasem u\u017Cywane s\u0105 tak\u017Ce nazwy: kwasice organiczne lub acydemie organiczne) \u2013 termin u\u017Cywany do okre\u015Blenia grupy genetycznie uwarunkowanych zaburze\u0144 metabolicznych, dotycz\u0105cych metabolizmu aminokwas\u00F3w, szczeg\u00F3lnie aminokwas\u00F3w rozga\u0142\u0119zionych (izoleucyna, leucyna i walina). Te schorzenia powoduj\u0105 nadmiern\u0105 produkcj\u0119 i nagromadzenie okre\u015Blonych kwas\u00F3w organicznych, kt\u00F3rych zazwyczaj u os\u00F3b zdrowych nie stwierdza si\u0119. Najcz\u0119\u015Bciej spotykane acydurie organiczne to: , , z\u0142o\u017Cona acyduria malonowa i metylomalonowa, i choroba syropu klonowego."@pl . . . . . . . . . "Acydurie organiczne (czasem u\u017Cywane s\u0105 tak\u017Ce nazwy: kwasice organiczne lub acydemie organiczne) \u2013 termin u\u017Cywany do okre\u015Blenia grupy genetycznie uwarunkowanych zaburze\u0144 metabolicznych, dotycz\u0105cych metabolizmu aminokwas\u00F3w, szczeg\u00F3lnie aminokwas\u00F3w rozga\u0142\u0119zionych (izoleucyna, leucyna i walina). Te schorzenia powoduj\u0105 nadmiern\u0105 produkcj\u0119 i nagromadzenie okre\u015Blonych kwas\u00F3w organicznych, kt\u00F3rych zazwyczaj u os\u00F3b zdrowych nie stwierdza si\u0119. Najcz\u0119\u015Bciej spotykane acydurie organiczne to: , , z\u0142o\u017Cona acyduria malonowa i metylomalonowa, i choroba syropu klonowego."@pl . . . . . . "Le acidosi organiche sono un gruppo di patologie identificate da un abbassamento del pH ematico (acidosi), per , e da . L'acidosi \u00E8 quindi un sintomo, il sintomo preponderante di diverse patologie, e a queste ha dato il nome. In senso stretto si considerano \n* difetti della catena respiratoria \n* deficit di piruvato chinasi \n* deficit del ciclo di Krebs \n* deficit di gluconeogenesi. Nel senso pi\u00F9 lato, consideriamo anche \n* i difetti del metabolismo degli aminoacidi ramificati \n* i difetti della beta ossidazione degli acidi grassi."@it . . . 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"Organoazidopathie"@de . . "\u0627\u062D\u0645\u0636\u0627\u0636 \u0627\u0644\u062F\u0645 \u0627\u0644\u0639\u0636\u0648\u064A"@ar . "Organoazidopathie, Synonym: Organoazidurie, ist ein \u00DCberbegriff f\u00FCr eine Gruppe von Stoffwechselerkrankungen, die den Abbau von Aminos\u00E4uren oder Fetts\u00E4uren betreffen und \u00FCberwiegend einem autosomal rezessiven Erbgang folgen mit Anreicherung und Ausscheidung von organischen S\u00E4uren. Diese Krankheitsgruppe wird \u2013 historisch bedingt \u2013 nicht systematisch, sondern aufgrund der gemeinsamen Untersuchungstechnik Urinuntersuchung diagnostiziert. Die organischen S\u00E4uren k\u00F6nnen als giftige Stoffwechselzwischenprodukte eine Intoxikation hervorrufen. Davon sind vorwiegend Harnstoffzyklus, Gluconeogenese, die mitochondriale Atmungskette und die renale Karnitinreabsorption betroffen, woraus sich die entsprechende klinische Symptomatik der Hyperammon\u00E4mie, Hypoglyk\u00E4mie, Laktatazidose, Ketose und des Karnitinmangels ergibt."@de . . "Organic acidemia, is a term used to classify a group of metabolic disorders which disrupt normal amino acid metabolism, particularly branched-chain amino acids, causing a buildup of acids which are usually not present. The branched-chain amino acids include isoleucine, leucine and valine. Organic acids refer to the amino acids and certain odd-chained fatty acids which are affected by these disorders. The four main types of organic acidemia are: methylmalonic acidemia, propionic acidemia, isovaleric acidemia, and maple syrup urine disease."@en . . . "Organic acidemia"@en . . . . . . . . . . "289899"^^ . "7359"^^ . . . . . . . . "Organic acidemia"@en . . . . . "Acydurie organiczne"@pl . .