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What are the symptoms of cystinuria?
Severe cystinuria occurs when people are homozygous for the disease. For these individuals, the kidneys may excrete as much as 30 times the normal amount of cystine
What is the treatment for cystinuria?
The best treatment for cystinuria is to prevent stones from forming. Stones can be prevented by drinking enough liquid each day (about 5-7 qts) to produce at least 8 pts of urine,
What is the diagnosis for cystinuria?
When the urine contains extremely high amounts of cystine, yellow-brown hexagonal crystals are visible when a sample is examined under the microscope.
What is Nitroprusside?
They are two tests that a laboratory would perform on urine to screen for metabolic diseases. The first is to detect ketoaciduria and the second to detect cystinuria/homocystinuria.
What are the types of aminoaciduria?
There are several types of aminoaciduria, including cystinuria, Hartnup disease, and Lowe syndrome. Cystinuria involves the excessive excretion of cystine in the urine, while Hartnup disease is characterized by the impaired absorption of amino acids. Lowe syndrome is a rare genetic disorder that affects various systems in the body, including the kidneys.
What is phenylhydrazine-nitroprusside test?
They are two tests that a laboratory would perform on urine to screen for metabolic diseases. The first is to detect ketoaciduria and the second to detect cystinuria/homocystinuria.
What causes cystine crystal in the urine?
Cystine crystals in the urine are primarily caused by a genetic disorder known as cystinuria, which affects the kidneys' ability to reabsorb cystine, an amino acid. This leads to elevated levels of cystine in the urine, where it can precipitate and form crystals. Factors such as dehydration and urinary pH can also influence the formation of cystine crystals. Individuals with cystinuria are at an increased risk for kidney stone formation due to these crystals.
What is the test for sulphur containing amino acids?
The cyanide-nitroprusside test can be used to detect sulphur. The test detects sulphhydryl group compounds and is used to test urine in screening tests for the metabolic diseases, cystinuria and homocystinuria.
Cystinuria?
DefinitionCystinuria is a condition passed down through families in which stones form in the kidney, ureter, and bladder.See also: NephrolithiasisAlternative NamesStones - cystine; Cystine stonesCauses, incidence, and risk factorsCystinuria is an autosomal recessive disorder, so you must inherit the faulty gene from both parents in order to have symptoms.Cystinuria is caused by too much of an amino acid called cystine in the urine. After entering the kidneys, most cystine normally dissolves and goes back into the bloodstream. But people with cystinuria have a genetic defect that interferes with this process. As a result, cystine builds up in the urine and forms crystals or stones, which may get stuck in the kidneys, ureters, or bladder.About one in every 10,000 people have cystinuria. Cystine stones are most common in young adults under age 40. Less than 3% of urinary tract stones are cystine stones.SymptomsBlood in the urineFlank pain or pain in the side or back Usually on one side; rarely felt on both sidesOften severeMay get increasingly worse over daysPain may also be felt in the pelvis, groin, genitals, or between the upper abdomen and the back.Signs and testsThe disorder is usually diagnosed after an episode of kidney stones. Analysis of the stones shows they are made of cystine.Tests that may be done to detect stones and diagnose this condition include:Abdominal CT scan, MRI, or ultrasoundIntravenous pyelogram (IVP)Urinalysis (may show cystine crystals)24-hour urine collection (shows high levels of cystine)TreatmentThe goal of treatment is to relieve symptoms and prevent the development of more stones. A person with severe symptoms may need to be admitted to a hospital.Treatment involves drinking plenty of fluids, particularly water, so that large amounts of urine are produced. The patient should drink at least 6-8 glasses per day.In some cases, fluids may need to be given through a vein (by IV).Medications may be prescribed to help dissolve the cystine crystals. Eating less salt can also decrease cystine excretion and stone formation.Pain relievers may be needed to control pain in the kidney or bladder area associated with the passage of stones. The stones usually pass through the urine on their own. If they do not, surgery may be needed:Percutaneous nephrostolithotomy or nephrolithotomyUreteroscopy, for stones in the lower urinary tractExtracorporeal shock wavelithotripsy (ESWL). This procedure is not as successful for removal of cystine stones as it is for other types of stones)Expectations (prognosis)Cystinuria is a chronic, lifelong condition. Stones commonly return. However, the condition rarely results in kidney failure, and it does not affect other organs.ComplicationsBladder injuryfrom stoneUreteral obstructionKidney injuryfrom stoneKidney infectionUrinary tract infectionCalling your health care providerCall your health care provider if you have symptoms of urinary tract stones.PreventionThere is no known prevention for cystinuria. Any person with a known history of stones in the urinary tract should drink plenty of fluids to regularly produce a high amount of urine. This allows stones and crystals to leave the body before they become large enough to cause symptoms.ReferencesRogers A, Kalakish S, Desai RA, Assimos DG. Management of cystinuria.Urol Clin North Am. 2007;34(3).
What makes the kidneys an organ?
"Kidney stones are made of salts and minerals in the urine that stick together to form small "pebbles." Calcium stones are the most common type of kidney stone and occur in two major forms: calcium oxalate and calcium phosphate. Uric acid stones form when the urine is persistently acidic. Struvite stones result from kidney infections.
What conditions might predispose a person to kidney stones?
There are several types of kidney stones, but the most common are calcium oxalate stones, followed by calcium phosphate stones.A variety of foods contain oxalates, which are usually bound up with other compounds in the intestines. When oxalates make it to the kidney, they bind with calcium and can form stones."Calcium oxalate stonesThe most common type of kidney stone is composed of calcium oxalate crystals, occurring in about 75 to 80% of cases,[9] and the factors that promote the precipitation of crystals in the urine are associated with the development of these stones.Perhaps counter-intuitively, current evidence suggests that the consumption of low-calcium diets is associated with a higher overall risk for the development of kidney stones.[2] This is perhaps related to the role of calcium in binding ingested oxalate in the gastrointestinal tract. As the amount of calcium intake decreases, the amount of oxalate available for absorption into the bloodstream increases; this oxalate is then excreted in greater amounts into the urine by the kidneys. In the urine, oxalate is a very strong promoter of calcium oxalate precipitation, about 15 times stronger than calcium.Uric acid (urate)About 5-10% of all stones are formed from uric acid.[9] A risk factor is obesity.[21] Uric acid stones form in association with conditions that cause hyperuricosuria with or without high blood serum uric acid levels (hyperuricemia); and with acid/base metabolism disorders where the urine is excessively acidic (low pH) resulting in uric acid precipitation. A diagnosis of uric acid nephrolithiasis is supported if there is a radiolucent stone, a persistent undue urine acidity, and uric acid crystals in fresh urine samples.[22][edit] Other typesOther types of kidney stones are composed of struvite (magnesium, ammonium and phosphate); calcium phosphate; and cystine.Struvite stones are also known as infection stones, urease or triple-phosphate stones. About 10-15% of urinary calculi consist of struvite stones.[23] The formation of struvite stones is associated with the presence of urea-splitting bacteria,[24] most commonly Proteus mirabilis (but also Klebsiella, Serratia, Providencia species). These organisms are capable of splitting urea into ammonia, decreasing the acidity of the urine and resulting in favorable conditions for the formation of struvite stones. Struvite stones are always associated with urinary tract infections.The formation of calcium phosphate stones is associated with conditions such as hyperparathyroidism and renal tubular acidosis.Formation of cystine stones is uniquely associated with people suffering from cystinuria, who accumulate cystine in their urine. Cystinuria can be caused by Fanconi's syndrome.Urolithiasis has also been noted to occur in the setting of therapeutic drug use, with crystals of drug forming within the renal tract in some patients currently being treated with Indinavir, Sulfadiazine or Triamterene."
What rhymes with hematuria?
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
