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1. フェニルケトン尿症の概要overview of phenylketonuria [show details]
… or inadequate intake of amino acid mixture, total energy, and/or protein in relation to metabolic needs. Tyrosine is an essential amino acid in patients with PKU because… The mainstay of therapy in PKU remains dietary restriction of phenylalanine, although enzyme therapy using pegvaliase is approved for adult patients with PKU. Dietary therapy requires the use…
2. 先天性代謝異常：分類inborn errors of metabolism classification [show details]
… Of the 20 amino acids, 11 can be synthesized in vivo and 9 cannot. The nine that cannot be synthesized in vivo are called "essential amino acids."… CDG Ib (mannosephosphate isomerase [MPI]-CDG) is amenable to therapy with mannose.…
3. 乳児における腸管不全関連肝疾患intestinal failure associated liver disease in infants [show details]
… Even if the total dose of amino acids is not associated with IFALD, an imbalance in several conditionally essential amino acids may increase the risk.… generally recommended as a treatment for IFALD. Antioxidant therapy has been suggested as a therapeutic option in treating IFALD.…
4. 緩和ケアにおける食欲不振と悪液質の評価および管理assessment and management of anorexia and cachexia in palliative care [show details]
… depletion of both essential and nonessential amino acids, which may become conditionally essential. The following studies have evaluated supplementation with amino acids in cachectic patients:… sometimes can be successfully treated. Among patients with cancer, some treatments are associated with sarcopenia (androgen deprivation therapy,…
5. メープルシロップ尿症の概要overview of maple syrup urine disease [show details]
… The branched-chain amino acids, leucine, isoleucine, and valine, are essential amino acids with hydrophobic side chains and are important… reduction of branched-chain amino acids. Phenylbutyrate therapy reduced plasma levels of branched-chain amino acids in patients with intermediate MSUD ,…

Japanese Journal

腎不全アミノ酸輸液製剤投与中に意識障害をきたした慢性透析患者の２例

内田昌子,酒見隆信,池田裕次,馬場直樹,西原学宣,中城博見
日本透析医学会雑誌 28(3), 281-284, 1995
… 慢性腎不全患者の低蛋白食療法の欠点を補うために必須アミノ酸療法がある. …
NAID 130003721306

糖尿病性腎症による腎不全に対する必須アミノ酸療法の有効性の検討

星山俊潤,直克則,上田信行,久保田昌詞,向井光佐子,鮴谷佳和,七里元亮,鎌田武信
糖尿病 30(2), 155-160, 1987
… 晩期の糖尿病性腎症における必須アミノ酸療法の効果および適応時期についてrerrospectiveな検討を行った.対象は1) 必須アミノ酸非投与群 (n=6), 2) 必須アミノ酸療法有効群 (n=4), 3) 必須アミノ酸療法無効群 (n=8) の3群に分けられた.非投与群, 有効群および無効群の透析導入までの期間は, それぞれ, 9.4±3.2ヵ月, 16.5±4.4ヵ月, 4.3±3.4ヵ月であり, 有効群と非投与群の間に有意差を認めた.有 …
NAID 130004114858