Alternate Names : Stones – cystine, Cystine stones
Definition
Cystinuria is a condition passed down through families in which stones form in the kidney, ureter, and bladder.
See also: Nephrolithiasis
Overview, Causes, & Risk Factors
Cystinuria 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.
Pictures & Images
Female urinary tract
The female and male urinary tracts are relatively the same except for the length of the urethra.
Male urinary tract
The male and female urinary tracts are relatively the same except for the length of the urethra.
Cystinuria
Cystinuria is a disorder characterized by cystine stones in the kidney, ureter, and bladder. A genetic abnormality results in abnormal transport of amino acids in the kidney. The high levels of the amino acid cystine in the urine lead to stone formation. While this disease only accounts for 1-2 % of urinary tract stones in the general population, it is the most common cause among children.
Nephrolithiasis
Kidney stones result when urine becomes too concentrated and substances in the urine crystalize to form stones. Symptoms arise when the stones begin to move down the ureter causing intense pain. Kidney stones may form in the pelvis or calyces of the kidney or in the ureter.
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Review Date : 8/30/2009
Reviewed By : Louis S. Liou, MD, PhD, Assistant Professor of Urology, Department of Surgery, Boston University School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
