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Elevated urine keto acid level

MedGen UID:
1782934
•Concept ID:
C5539608
•
Finding
HPO: HP:0033456

Definition

Increased amount of a keta acid in the urine. [from HPO]

Professional guidelines

PubMed

Guanidino compound analysis as a complementary diagnostic parameter for hyperargininemia: follow-up of guanidino compound levels during therapy.
Marescau B, De Deyn PP, Lowenthal A, Qureshi IA, Antonozzi I, Bachmann C, Cederbaum SD, Cerone R, Chamoles N, Colombo JP
Pediatr Res 1990 Mar;27(3):297-303. doi: 10.1203/00006450-199003000-00020. PMID: 1690873
Maple syrup urine disease: treatment of the acutely ill newborn.
Hammersen G, Wille L, Schmidt H, Lutz P, Bickel H
Eur J Pediatr 1978 Oct 12;129(3):157-65. doi: 10.1007/BF00442159. PMID: 699921

Recent clinical studies

Etiology

RNA interference therapy in acute hepatic porphyrias.
Yasuda M, Keel S, Balwani M
Blood 2023 Nov 9;142(19):1589-1599. doi: 10.1182/blood.2022018662. PMID: 37027823Free PMC Article
Metabolomics profile in acute respiratory distress syndrome by nuclear magnetic resonance spectroscopy in patients with community-acquired pneumonia.
Yan Y, Chen J, Liang Q, Zheng H, Ye Y, Nan W, Zhang X, Gao H, Li Y
Respir Res 2022 Jun 27;23(1):172. doi: 10.1186/s12931-022-02075-w. PMID: 35761396Free PMC Article
Phase 3 Trial of RNAi Therapeutic Givosiran for Acute Intermittent Porphyria.
Balwani M, Sardh E, Ventura P, Peiró PA, Rees DC, Stölzel U, Bissell DM, Bonkovsky HL, Windyga J, Anderson KE, Parker C, Silver SM, Keel SB, Wang JD, Stein PE, Harper P, Vassiliou D, Wang B, Phillips J, Ivanova A, Langendonk JG, Kauppinen R, Minder E, Horie Y, Penz C, Chen J, Liu S, Ko JJ, Sweetser MT, Garg P, Vaishnaw A, Kim JB, Simon AR, Gouya L; ENVISION Investigators
N Engl J Med 2020 Jun 11;382(24):2289-2301. doi: 10.1056/NEJMoa1913147. PMID: 32521132
Supplementation of arachidonic acid-enriched oil increases arachidonic acid contents in plasma phospholipids, but does not increase their metabolites and clinical parameters in Japanese healthy elderly individuals: a randomized controlled study.
Kakutani S, Ishikura Y, Tateishi N, Horikawa C, Tokuda H, Kontani M, Kawashima H, Sakakibara Y, Kiso Y, Shibata H, Morita I
Lipids Health Dis 2011 Dec 22;10:241. doi: 10.1186/1476-511X-10-241. PMID: 22188761Free PMC Article
Phenylalanine metabolism in uremic and normal man.
Jones MR, Kopple JD, Swendseid ME
Kidney Int 1978 Aug;14(2):169-79. doi: 10.1038/ki.1978.104. PMID: 357810

Diagnosis

Laboratory and metabolic investigations.
Morava E, Oglesbee D
Handb Clin Neurol 2023;194:167-172. doi: 10.1016/B978-0-12-821751-1.00012-9. PMID: 36813311
Metabolomics profile in acute respiratory distress syndrome by nuclear magnetic resonance spectroscopy in patients with community-acquired pneumonia.
Yan Y, Chen J, Liang Q, Zheng H, Ye Y, Nan W, Zhang X, Gao H, Li Y
Respir Res 2022 Jun 27;23(1):172. doi: 10.1186/s12931-022-02075-w. PMID: 35761396Free PMC Article
Spectrophotometric photodynamic diagnosis of prostate cancer cells excreted in voided urine using 5-aminolevulinic acid.
Nakai Y, Miyake M, Anai S, Hori S, Tatsumi Y, Morizawa Y, Onisi S, Tanaka N, Fujimoto K
Lasers Med Sci 2018 Sep;33(7):1557-1563. Epub 2018 May 4 doi: 10.1007/s10103-018-2519-4. PMID: 29725946
α-Ketoglutaramate: an overlooked metabolite of glutamine and a biomarker for hepatic encephalopathy and inborn errors of the urea cycle.
Cooper AJ, Kuhara T
Metab Brain Dis 2014 Dec;29(4):991-1006. Epub 2013 Nov 14 doi: 10.1007/s11011-013-9444-9. PMID: 24234505Free PMC Article
Maple syrup urine disease: clinical, EEG, and plasma amino acid correlations with a theoretical mechanism of acute neurotoxicity.
Korein J, Sansaricq C, Kalmijn M, Honig J, Lange B
Int J Neurosci 1994 Nov;79(1-2):21-45. doi: 10.3109/00207459408986065. PMID: 7744549

Therapy

Urinary prostanoids are elevated by anti-TNF and anti-IL6 receptor disease-modifying antirheumatic drugs but are not predictive of response to treatment in early rheumatoid arthritis.
Liu J, Idborg H, Korotkova M, Lend K, van Vollenhoven R, Lampa J, Rudin A, Nordström D, Gudbjornsson B, Gröndal G, Uhlig T, Hørslev-Petersen K, Lund Hetland M, Østergaard M, Nurmohamed M, Jakobsson PJ
Arthritis Res Ther 2024 Mar 5;26(1):61. doi: 10.1186/s13075-024-03295-9. PMID: 38444034Free PMC Article
Phase 3 Trial of RNAi Therapeutic Givosiran for Acute Intermittent Porphyria.
Balwani M, Sardh E, Ventura P, Peiró PA, Rees DC, Stölzel U, Bissell DM, Bonkovsky HL, Windyga J, Anderson KE, Parker C, Silver SM, Keel SB, Wang JD, Stein PE, Harper P, Vassiliou D, Wang B, Phillips J, Ivanova A, Langendonk JG, Kauppinen R, Minder E, Horie Y, Penz C, Chen J, Liu S, Ko JJ, Sweetser MT, Garg P, Vaishnaw A, Kim JB, Simon AR, Gouya L; ENVISION Investigators
N Engl J Med 2020 Jun 11;382(24):2289-2301. doi: 10.1056/NEJMoa1913147. PMID: 32521132
In vivo evidence that Agxt2 can regulate plasma levels of dimethylarginines in mice.
Kittel A, Maas R, König J, Mieth M, Weiss N, Jarzebska N, Hohenstein B, Martens-Lobenhoffer J, Bode-Böger SM, Rodionov RN
Biochem Biophys Res Commun 2013 Jan 4;430(1):84-9. Epub 2012 Nov 12 doi: 10.1016/j.bbrc.2012.11.008. PMID: 23154179
Supplementation of arachidonic acid-enriched oil increases arachidonic acid contents in plasma phospholipids, but does not increase their metabolites and clinical parameters in Japanese healthy elderly individuals: a randomized controlled study.
Kakutani S, Ishikura Y, Tateishi N, Horikawa C, Tokuda H, Kontani M, Kawashima H, Sakakibara Y, Kiso Y, Shibata H, Morita I
Lipids Health Dis 2011 Dec 22;10:241. doi: 10.1186/1476-511X-10-241. PMID: 22188761Free PMC Article
Phenylalanine metabolism in uremic and normal man.
Jones MR, Kopple JD, Swendseid ME
Kidney Int 1978 Aug;14(2):169-79. doi: 10.1038/ki.1978.104. PMID: 357810

Prognosis

Urinary prostanoids are elevated by anti-TNF and anti-IL6 receptor disease-modifying antirheumatic drugs but are not predictive of response to treatment in early rheumatoid arthritis.
Liu J, Idborg H, Korotkova M, Lend K, van Vollenhoven R, Lampa J, Rudin A, Nordström D, Gudbjornsson B, Gröndal G, Uhlig T, Hørslev-Petersen K, Lund Hetland M, Østergaard M, Nurmohamed M, Jakobsson PJ
Arthritis Res Ther 2024 Mar 5;26(1):61. doi: 10.1186/s13075-024-03295-9. PMID: 38444034Free PMC Article
Metabolomics profile in acute respiratory distress syndrome by nuclear magnetic resonance spectroscopy in patients with community-acquired pneumonia.
Yan Y, Chen J, Liang Q, Zheng H, Ye Y, Nan W, Zhang X, Gao H, Li Y
Respir Res 2022 Jun 27;23(1):172. doi: 10.1186/s12931-022-02075-w. PMID: 35761396Free PMC Article
α-ketoglutarate is associated with delayed wound healing in diabetes.
Tan Q, Wang W, Yang C, Zhang J, Sun K, Luo HC, Mai LF, Lao Y, Yan L, Ren M
Clin Endocrinol (Oxf) 2016 Jul;85(1):54-61. Epub 2016 Mar 21 doi: 10.1111/cen.13047. PMID: 26921880
Genotoxicity evaluation in workers occupationally exposed to lead.
Grover P, Rekhadevi PV, Danadevi K, Vuyyuri SB, Mahboob M, Rahman MF
Int J Hyg Environ Health 2010 Mar;213(2):99-106. Epub 2010 Feb 12 doi: 10.1016/j.ijheh.2010.01.005. PMID: 20153251
Excretion measurement of porphyrins and their precursors after topical administration of 5-aminolaevulinic acid for fluorescence endoscopy in head and neck cancer.
Lippert BM, Gross U, Klein M, Külkens C, Klahr N, Brossmann P, Teymoortash A, Ney M, Doss MO, Werner JA
Res Commun Mol Pathol Pharmacol 2003;113-114:75-85. PMID: 15686109

Clinical prediction guides

Urinary prostanoids are elevated by anti-TNF and anti-IL6 receptor disease-modifying antirheumatic drugs but are not predictive of response to treatment in early rheumatoid arthritis.
Liu J, Idborg H, Korotkova M, Lend K, van Vollenhoven R, Lampa J, Rudin A, Nordström D, Gudbjornsson B, Gröndal G, Uhlig T, Hørslev-Petersen K, Lund Hetland M, Østergaard M, Nurmohamed M, Jakobsson PJ
Arthritis Res Ther 2024 Mar 5;26(1):61. doi: 10.1186/s13075-024-03295-9. PMID: 38444034Free PMC Article
Laboratory and metabolic investigations.
Morava E, Oglesbee D
Handb Clin Neurol 2023;194:167-172. doi: 10.1016/B978-0-12-821751-1.00012-9. PMID: 36813311
Phase 3 Trial of RNAi Therapeutic Givosiran for Acute Intermittent Porphyria.
Balwani M, Sardh E, Ventura P, Peiró PA, Rees DC, Stölzel U, Bissell DM, Bonkovsky HL, Windyga J, Anderson KE, Parker C, Silver SM, Keel SB, Wang JD, Stein PE, Harper P, Vassiliou D, Wang B, Phillips J, Ivanova A, Langendonk JG, Kauppinen R, Minder E, Horie Y, Penz C, Chen J, Liu S, Ko JJ, Sweetser MT, Garg P, Vaishnaw A, Kim JB, Simon AR, Gouya L; ENVISION Investigators
N Engl J Med 2020 Jun 11;382(24):2289-2301. doi: 10.1056/NEJMoa1913147. PMID: 32521132
α-ketoglutarate is associated with delayed wound healing in diabetes.
Tan Q, Wang W, Yang C, Zhang J, Sun K, Luo HC, Mai LF, Lao Y, Yan L, Ren M
Clin Endocrinol (Oxf) 2016 Jul;85(1):54-61. Epub 2016 Mar 21 doi: 10.1111/cen.13047. PMID: 26921880
Maple syrup urine disease: clinical, EEG, and plasma amino acid correlations with a theoretical mechanism of acute neurotoxicity.
Korein J, Sansaricq C, Kalmijn M, Honig J, Lange B
Int J Neurosci 1994 Nov;79(1-2):21-45. doi: 10.3109/00207459408986065. PMID: 7744549

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