Alkaptonuria is an inherited condition that causes urine to turn black when exposed to air.
Decrease homogenitisic acid formation by decreased phenylalanine and tyrosine. Essentially a PKU diet with decreased protein intake?
End rhymes for "hematuria": 1. acetonuria 2. acholuria 3. achromaturia 4. aciduria 5. adiposuria 6. albiduria 7. albinuria 8. albuminuria 9. alcaptonuria 10. alkalinuria 11. alkaluria 12. alkaptonuria 13. allantoinuria 14. alloxuria 15. ameburia 16. aminoaciduria 17. aminuria 18. ammoniuria 19. amylasuria 20. amylosuria 21. amyluria 22. anuria 23. archosauria 24. aspartylglycosaminuria 25. azoturia 26. bacilluria 27. bacteriuria 28. baruria 29. beeturia 30. bilirubinuria 31. biliuria 32. blennuria 33. bradyuria 34. calcariuria 35. calciuria 36. carbohydraturia 37. carboluria 38. centuria 39. chloriduria 40. chloruria 41. cholesteroluria 42. choleuria 43. choluria 44. chromaturia 45. chyluria 46. citrullinuria 47. creatinuria 48. crystalluria 49. curia 50. cylindruria 51. cystathioninuria 52. cystinuria 53. cyturia 54. dextrinuria 55. diastasuria 56. dinosauria 57. dysuria 58. enaliosauria 59. eosinophiluria 60. erythruria 61. etruria 62. fibrinuria 63. fructosuria 64. galactosuria 65. globulinuria 66. glucosuria 67. glutathionuria 68. glycosuria 69. haematocyturia 70. haematuria 71. haemoglobinuria 72. halisauria 73. hematocyturia 74. hematuria 75. hemoglobinuria 76. histidinuria 77. histonuria 78. holothuria 79. homaluria 80. homocitrullinuria 81. homocystinuria 82. hyalinuria 83. hydrothionuria 84. hypercalcinuria 85. hypercalciuria 86. hyperglycinuria 87. hyperglycosuria 88. hyperketonuria 89. hyperlysinuria 90. hyperoxaluria 91. hyperphosphaturia 92. hypersthenuria 93. hyperuricuria 94. hypoaldosteronuria 95. hypoazoturia 96. hypochloruria 97. hypocitraturia 98. hypophosphaturia 99. hyposthenuria 100. hypouricuria from: rhymezone.com
DefinitionAlkaptonuria is a rare condition in which a person's urine turns a dark brownish-black color when exposed to air.See also: Inborn error of metabolismAlternative NamesAKU; Alcaptonuria; Homogentisic acid oxidase deficiency; Alcaptonuric ochronosisCauses, incidence, and risk factorsA defect in the HGD gene causes alkaptonuria.The gene defect makes the body unable to properly break down certain amino acids (tyrosine and phenylalanine). As a result, a substance called homogentisic acid builds up in the skin and other body tissues. The acid leaves the body through the urine. The urine turns brownish-black when it mixes with air.Alkaptonuria is inherited, which means it is passed down from parents to their children. To get this disease, each of your parents must pass you a copy of the faulty HGD gene.SymptomsUrine in an infant's diaper may darken and can turn almost black after several hours. However, many persons with this condition may not know they have it until mid-adulthood (around age 40), when joint and other problems occur.Symptoms may include:Arthritis (especially of the spine) that gets worse over timeDarkening of the earDark spots on the white of the eye (sclera) and corneaSigns and testsA urine test (urinalysis) is done to test for alkaptonuria. If ferric chloride is added to the urine, it will turn the urine a black color in patients with this condition.TreatmentSome patients benefit from high-dose vitamin C. This has been shown to decrease the build-up of brown pigment in the cartilage and may slow the development of arthritis.Expectations (prognosis)The outcome is expected to be good.ComplicationsPeople with this condition also can get arthritis in adulthood. The build-up of homogentisic acid in the cartilage causes arthritis in about 50% of older adults with alkaptonuria.Homogentisic acid also can build up on the heart valves, especially the mitral valve. This can sometimes lead to the need for valve replacement.Coronary artery disease may develop earlier in people with alkaptonuria.Kidney stonesand prostate stones may be more common in people with alkaptonuria.Calling your health care providerCall your health care provider if you notice that your own urine or your child's urine becomes dark brown or black when it is exposed to air.PreventionThere is no know prevention.ReferencesChakrapani A, Holme E. Disorders of tyrosine metabolism. In: Fernandes J, Saudubray J-m, van den Berghe G, Walter JH, eds. Inborn Metabolic Diseases: Diagnosis and Treatment. 4th ed. New York, NY: Springer;2006:chap 18.