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Elevated urine succinic acid level

MedGen UID:
1053921
•Concept ID:
CN377080
•
Finding
Synonym: Succinic acid high in urine
 
HPO: HP:0430137

Definition

The concentration of succinic acid in the urine, normalized for urine concentration, is above the upper limit of normal. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVElevated urine succinic acid level

Professional guidelines

PubMed

Succinic semialdehyde dehydrogenase deficiency of four Chinese patients and prenatal diagnosis for three fetuses.
Li X, Ding Y, Liu Y, Zhang Y, Song J, Wang Q, Li M, Qin Y, Huang S, Yang Y
Gene 2015 Dec 10;574(1):41-7. Epub 2015 Jul 26 doi: 10.1016/j.gene.2015.07.078. PMID: 26220405

Recent clinical studies

Etiology

Enterocystoplasty in children with neuropathic bladders: long-term follow-up.
López Pereira P, Moreno Valle JA, Espinosa L, Alonso Dorrego JM, Burgos Lucena L, Martínez Urrutia MJ, Lobato Romera R, Luz Picazo M, Viguer JM, Jaureguízar Monereo E
J Pediatr Urol 2008 Feb;4(1):27-31. Epub 2007 Sep 17 doi: 10.1016/j.jpurol.2007.07.009. PMID: 18631888
Followup study of renal function in children with reflux nephropathy after resolution of vesicoureteral reflux.
Konda R, Sakai K, Ota S, Takeda A, Orikasa S
J Urol 1997 Mar;157(3):975-9. PMID: 9072628

Diagnosis

Succinic semialdehyde dehydrogenase deficiency of four Chinese patients and prenatal diagnosis for three fetuses.
Li X, Ding Y, Liu Y, Zhang Y, Song J, Wang Q, Li M, Qin Y, Huang S, Yang Y
Gene 2015 Dec 10;574(1):41-7. Epub 2015 Jul 26 doi: 10.1016/j.gene.2015.07.078. PMID: 26220405
The first case of 4-hydroxybutyric aciduria in Japan.
Ishiguro Y, Kajita M, Aoshima T, Watanabe K, Kimura M, Yamaguchi S
Brain Dev 2001 Mar;23(2):128-30. doi: 10.1016/s0387-7604(01)00181-4. PMID: 11248463
Followup study of renal function in children with reflux nephropathy after resolution of vesicoureteral reflux.
Konda R, Sakai K, Ota S, Takeda A, Orikasa S
J Urol 1997 Mar;157(3):975-9. PMID: 9072628
Organic acid and acylcarnitine profiles of glutaric aciduria type I.
Matsumoto M, Matsumoto I, Shinka T, Kuhara T, Imamura H, Shimao S, Okada T
Acta Paediatr Jpn 1990 Feb;32(1):76-82. doi: 10.1111/j.1442-200x.1990.tb00787.x. PMID: 2109491
Identification and quantitation of urinary dicarboxylic acids as their dicyclohexyl esters in disease states by gas chromatography mass spectrometry.
Norman EJ, Berry HK, Denton MD
Biomed Mass Spectrom 1979 Dec;6(12):546-53. doi: 10.1002/bms.1200061203. PMID: 526567

Prognosis

Followup study of renal function in children with reflux nephropathy after resolution of vesicoureteral reflux.
Konda R, Sakai K, Ota S, Takeda A, Orikasa S
J Urol 1997 Mar;157(3):975-9. PMID: 9072628

Clinical prediction guides

Detection of γ-hydroxybutyric acid-related acids in blood plasma and urine: Extending the detection window of an exogenous γ-hydroxybutyric acid intake?
Küting T, Schneider B, Heidbreder A, Krämer M, Jarsiah P, Madea B, Hess C
Drug Test Anal 2021 Sep;13(9):1635-1649. Epub 2021 May 29 doi: 10.1002/dta.3097. PMID: 33991073
Novel mutations in a Chinese family with two patients with succinic semialdehyde dehydrogenase deficiency.
Chen XD, Lin YT, Jiang MY, Li XZ, Li D, Hu H, Liu L
Gynecol Endocrinol 2020 Oct;36(10):929-933. Epub 2020 Mar 30 doi: 10.1080/09513590.2020.1744555. PMID: 32223457
Followup study of renal function in children with reflux nephropathy after resolution of vesicoureteral reflux.
Konda R, Sakai K, Ota S, Takeda A, Orikasa S
J Urol 1997 Mar;157(3):975-9. PMID: 9072628
Urinary organic acid profiles of Reye's syndrome patients.
Harrington W, Liu AS, Lonsdale D, Igou D
Clin Chim Acta 1977 Feb 1;74(3):247-54. doi: 10.1016/0009-8981(77)90291-1. PMID: 832426
Gas chromatographic and mass spectrometric studies on urinary organic acids in a patient with congenital lactic acidosis due to pyruvate decarboxylase deficiency.
Chalmers RA, Lawson AM, Borud O
Clin Chim Acta 1977 Jun 1;77(2):117-24. doi: 10.1016/0009-8981(77)90018-3. PMID: 19171

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