Aminoaciduria

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Aminoaciduria

Aminoaciduria occurs when the urine contains abnormally high amounts of amino acids.[1] In the healthy kidney, the glomeruli filter all amino acids out of the blood, and the renal tubules then reabsorb over 95% of the filtered amino acids back into the blood.[2]

In overflow aminoaciduria, abnormally high concentrations of amino acids in the blood plasma overwhelm the resorptive capacity of the renal tubules, resulting in high concentrations of amino acids in the urine.[3] This may be caused by congenital disorders of amino acid metabolism,[2] for example, phenylketonuria,[4] or may be secondary to liver disease.[2]

In renal aminoaciduria, the renal tubules are unable to reabsorb the filtered amino acids back into the blood, causing high concentrations of amino acids in the urine.[4] This may be caused by a defect in the transport proteins in the renal tubule, for example, as occurs in Hartnup disease,[4] or may be due to damage to the kidney tubule, for example, as occurs in Fanconi syndrome.[3]

References[edit]

  1. ^ "Aminoaciduria". Mosby's Medical Dictionary (9th ed.). Mosby. 2013. p. 79. ISBN 9780323112581.
  2. ^ a b c Schück, O (1984). "Amino acids excretion. Physiology and pathophysiology". Examination of Kidney Function. Translated by Cort, JH. Dordrecht: Springer Netherlands. p. 252. ISBN 9789400956605.
  3. ^ a b Crook, Martin Andrew (2012). "Chapter 27: Inborn errors of metabolism". Clinical biochemistry and metabolic medicine (8th ed.). London: Hodder Arnold. pp. 371–383. ISBN 9781444144154.
  4. ^ a b c Mundt, LA; Shanahan, K (2011). "Chapter 7: Urinary and metabolic diseases and related urinalysis findings. Aminoacidurias". Graff's textbook of routine urinalysis and body fluids (2nd ed.). Philadelphia: Wolters Kluwer/Lippincott Williams & Wilkins Health. pp. 203–210. ISBN 9781582558752.

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