WordNet

simple and healthful and close to nature; "an organic lifestyle"
a fertilizer that is derived from animal or vegetable matter (同)organic fertilizer, organic fertiliser
being or relating to or derived from or having properties characteristic of living organisms; "organic life"; "organic growth"; "organic remains found in rock"
involving or affecting physiology or bodily organs; "an organic disease"
of or relating to foodstuff grown or raised without synthetic fertilizers or pesticides or hormones; "organic eggs"; "organic vegetables"; "organic chicken"
relating or belonging to the class of chemical compounds having a carbon basis; "hydrocarbons are organic compounds"
wind instrument whose sound is produced by means of pipes arranged in sets supplied with air from a bellows and controlled from a large complex musical keyboard (同)pipe_organ
a fully differentiated structural and functional unit in an animal that is specialized for some particular function
a government agency or instrument devoted to the performance of some specific function; "The Census Bureau is an organ of the Commerce Department"
a periodical that is published by a special interest group; "the organ of the communist party"
a blood disorder characterized by an increased concentration of hydrogen ions in the blood (which falls below 7 on the pH scale)

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有機体の,生物の,生物から生じた / (動植物の)器官の / 有機肥料を用いる(を用いて育った)・有機的な,組織的な,系統的な・有機質の
『オルガン』,パイプオルガン(pipe organ),リードオルガン(reed organ),電子オルガン(electronic organ),手回しオルガン(barrel organ) / (動植物の)『器官』 / (政党・会社などの)機関誌,機関紙 / 《しばしば複数形で》(行動・実施などの)組織,機関

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/03/17 21:42:22」(JST)

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Organic acidemia, also called organic aciduria, 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.^[1]

The branched-chain amino acids include isoleucine, leucine and valine.^[1] 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.^[1]

Diagnosis and Symptoms[edit]

Organic acidemias are usually diagnosed in infancy, characterized by urinary excretion of abnormal amounts or types of organic acids. The diagnosis is usually made by detecting an abnormal pattern of organic acids in a urine sample by gas chromatography-mass spectrometry. In some conditions, the urine is always abnormal, in others the characteristic substances are only present intermittently. Many of the organic acidemias are detectable by newborn screening with tandem mass spectrometry.^[2]

These disorders vary in their prognosis, from manageable to fatal, and usually affect more than one organ system, especially the central nervous system.

Neurological damage and developmental delay are common factors in diagnosis, with associated symptoms ranging from poor feeding to slow growth, lethargy, vomiting, dehydration, malnutrition, hypoglycemia, hypotonia, metabolic acidosis, ketoacidosis, hyperammonemia, and if left untreated, death.

Cause[edit]

Most of the organic acidemias result from defective autosomal genes for various enzymes important to amino acid metabolism. Neurological and physiological harm is caused by this impaired ability to synthesize a key enzyme required to break down a specific amino acid, or group of amino acids, resulting in acidemia and toxicity to specific organs systems. Most are inherited as autosomal recessive diseases.

Treatment[edit]

Treatment or management of organic acidemias vary. There are no effective treatments for all of the conditions, though treatment for some may include a limited protein/high carbohydrate diet, intravenous fluids, amino acid substitution, vitamin supplementation, carnitine, induced anabolism,^[3] and in some cases, tube-feeding.

External links[edit]

GeneReviews/UW/NIH entry on Organic acidemias

http://www.oaanews.org - Organic Acidemia Association - Parent Support Group for organic acid disorders.

References[edit]

^ ^a ^b ^c Ogier de Baulny H, Saudubray JM (2002). "Branched-chain organic acidurias". Semin Neonatol. 7 (1): 65–74. doi:10.1053/siny.2001.0087. PMID 12069539.
^ Dionisi-Vici C, Deodato F, Raschinger W, Rhead W, Wilcken B (2006). "Classical organic acidurias, propionic aciduria, methylmalonic aciduria, and isovaleric aciduria: long-term outcome and effects of expanded newborn screening using tandem mass spectrometry". J Inherit Metab Dis. 29 (2–3): 383–389. doi:10.1007/s10545-006-0278-z. PMID 16763906.
^ Saudubray JM, Ogier H, Charpentier C, Depondt E, Couda FX, Munnich A, Mitchell G, Rey F, Rey J, Frazal J (1984). "Hudson memorial lecture. Neonatal management of organic acidurias. Clinical update". J Inherit Metab Dis. 7 (Suppl. 1): 2–9. PMID 6434839.

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1. 有機酸血症 organic acidemias
2. 先天性代謝異常：分類 inborn errors of metabolism classification
3. 先天性代謝異常：代謝性救急疾患 inborn errors of metabolism metabolic emergencies
4. 先天性代謝異常：個々の障害の発見 inborn errors of metabolism identifying the specific disorder
5. 先天性代謝異常：疫学、病因、および臨床的特徴 inborn errors of metabolism epidemiology pathogenesis and clinical features

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